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Sample records for bulbar palsy progressive

  1. Paralisia bulbar progressiva juvenil doença de Fazio-Londe: relato de caso Progressive bulbar palsy (Fazio-Londe disease: case report

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    Bianca Helena Brum Batista

    2002-09-01

    Full Text Available A paralisia bulbar progressiva, também denominada doença de Fazio-Londe, caracteriza-se pelo acometimento degenerativo progressivo de nervos cranianos bulbares em crianças. Foi descrita primeiramente por Fazio em 1892 e até a presente data somente 30 casos foram relatados na literatura. Acomete ambos os sexos, assumindo dois padrões clínicos, um de início precoce (idade Progressive bulbar palsy, also called Fazio -Londe disease, is characterized by progressive impairment of cranial nerves in children. It was first reported by Fazio in 1892 and until now only 30 cases have been published in the literature. Both sexes can be affected and clinical course can be divided on early (< 6 years age, predominance of respiratory symptoms and late course (6-20 years of age, predominance of motor symptoms on superior limbs. We report a 4 years old boy that started with intense stridor and respiratory distress, initially being diagnosed as an acute asthma attack. Clinical signs worsened and 12 months latter he already had impairment of cranial nerves V, VII, VIII, IX and X confirmed by clinical examination and neurophysiological evaluation.

  2. Myasthenia Gravis With Thymoma, Manifesting as AChR-Ab-Positive, Distinct Bulbar Palsy Accompanied by Dysgeusia: A Case Series and Review of Literature

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    Kai Zhu

    2018-04-01

    Full Text Available In this review, we summarized three cases of myasthenia gravis (MG with taste disorder and describe their clinical features in detail. Three MG patients presented with significant bulbar palsy symptoms, high AChR-Ab titers, and negative MuSK-Ab, were diagnosed with thymoma. Furthermore, we observed that dysgeusia could manifest earlier than the occurrence of typical MG symptoms, even predict a MG relapse or a myasthenic crisis in the course of MG. We believe that dysgeusia is a non-motor symptom of MG, which especially exists in MG patients with thymoma and serious bulbar palsy. Therefore, being alert to this symptom may facilitate the early diagnosis of MG and judge the progress of the disease.

  3. Acute Thyrotoxic Bulbar Myopathy with Encephalopathic Behaviour: An Uncommon Complication of Hyperthyroidism

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    Neeraja J. Boddu

    2013-01-01

    Full Text Available Objective. Acute thyrotoxic bulbar palsy is rare, severe, and rapidly progressive. We describe a case of thyrotoxicosis with bulbar palsy, encephalopathy, and pyramidal tract dysfunction. Case Report. 64-year-old white male with toxic multinodular goiter presented with rapid atrial fibrillation. He had mild tremor, normal cranial nerve examination, 4/5 strength in all extremities, normal reflexes, and down going plantars. TSH was low at 0.09 (normal: 0.34–5.6 uIU/mL, and free T4 was high at 5.22 (normal: 0.47–1.41 ng/dL. Despite optimal AV nodal blockade, he had persistent rapid atrial fibrillation. He later developed cervical dystonia, rigidity, clonus, dysarthria, dysphagia, vocal cord palsy, and absent gag reflex. Thyroid storm was suspected. Neuroimaging and cerebrospinal fluid cultures were nondiagnostic. Acetylcholine receptor antibodies were negative. Swallow ability was impaired with heavy secretions. Remarkable improvement in symptoms was noted after initiation of treatment for thyroid storm. Conclusion. Pyramidal tract symptoms and bulbar palsy may occur with thyrotoxicosis. Cranial nerve involvement and encephalopathy raise a question of primary brain mechanism causing bulbar palsy. This is reversible with prompt treatment of thyroid storm.

  4. Amyotrophic Lateral Sclerosis Regional Variants (Brachial Amyotrophic Diplegia, Leg Amyotrophic Diplegia, and Isolated Bulbar Amyotrophic Lateral Sclerosis).

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    Jawdat, Omar; Statland, Jeffrey M; Barohn, Richard J; Katz, Jonathan S; Dimachkie, Mazen M

    2015-11-01

    Amyotrophic lateral sclerosis (ALS), a rapidly progressive, invariably fatal disease, involves mixed upper and lower motor neurons in different spinal cord regions. Patients with bulbar onset progress more rapidly than patients with limb onset or with a lower motor neuron presentation. Recent descriptions of regional variants suggest some patients have ALS isolated to a single spinal region for many years, including brachial amyotrophic diplegia, leg amyotrophic diplegia, and isolated bulbar palsy. Clearer definitions of regional variants will have implications for prognosis, understanding the pathophysiology of ALS, identifying genetic factors related to slower disease progression, and future planning of clinical trials. Copyright © 2015 Elsevier Inc. All rights reserved.

  5. A Case of Myasthenia Gravis Presenting Solely With Bulbar Palsy Not Associated With Easy Fatigability.

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    Pinank R. Mer

    2015-12-01

    present case shows that it is important to consider MG even in cases presenting solely with progressive bulbar palsy without easy fatigability.

  6. Zolpidem in Progressive Supranuclear Palsy

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    Sandip K. Dash

    2013-01-01

    Full Text Available Progressive supranuclear palsy (PSP is a progressive neurodegenerative disorder, characterized by motor symptoms, postural instability, personality changes, and cognitive impairment. There is no effective treatment for this disorder. Reduced neurotransmission of GABA in the striatum and globus pallidus may contribute to the symptoms of motor and cognitive symptoms seen in PSP. Zolpidem is a GABA agonist of the benzodiazepine subreceptor BZ1. Here a nondiabetic, normotensive case of PSP is (Progressive Supranuclear Palsy described, which showed improvement in swallowing, speech, and gaze paresis after zolpidem therapy and possible mechanism of actions are discussed. However, more trials are needed with large number of patients to confirm the effectiveness of zolpidem in progressive supranuclear palsy.

  7. Disease: H00841 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available drome (BVVLS) [DS:H01903]; Fazio-Londe disease Infantile progressive bulbar palsy is a rare neurological disorder that occurs in chil...dren. Infantile progressive bulbar palsy presents as following two forms. The Brown

  8. The applause sign and neuropsychological profile in progressive supranuclear palsy and Parkinson's disease.

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    Somme, Johanne; Gómez-Esteban, Juan Carlos; Tijero, Beatriz; Berganzo, Koldo; Lezcano, Elena; Zarranz, Juan Jose

    2013-08-01

    The applause sign has been associated with various neurodegenerative diseases. We investigate its validity in the differential diagnosis of progressive supranuclear palsy and Parkinson's disease, and its relationship with neuropsychological tests. 23 patients with progressive supranuclear palsy and 106 patients with Parkinson's disease were included and administered the following scales: progressive supranuclear palsy rating scale, unified Parkinson's disease rating scale (UPDRS), mini-mental state examination (MMSE), frontal assessment battery (FAB), neuropsychiatric inventory and three-clap test. 73.9% with progressive supranuclear palsy and 21.7% with Parkinson's disease showed a positive applause sign. Only a positive applause sign, UPDRS II score and disease duration were found to be predictors of progressive supranuclear palsy. Both patient-groups showed statistically significant correlations between the applause sign and neuropsychological tests: in progressive supranuclear palsy patients MMSE correlation coefficient: 0.62 (p: 0.002) and FAB correlation coefficient: 0.48 (p: 0.02), and in Parkinson's disease patients MMSE correlation coefficient: 0.47 (pspecific to progressive supranuclear palsy and may also be observed in Parkinson's disease patients with altered cognition, and it's related to cortical frontal abnormalities such as language disorders and inhibitory control. Copyright © 2012 Elsevier B.V. All rights reserved.

  9. Kinematics of Disease Progression in Bulbar ALS

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    Yunusova, Yana; Green, Jordan R.; Lindstrom, Mary J.; Ball, Laura J.; Pattee, Gary L.; Zinman, Lorne

    2010-01-01

    The goal of this study was to investigate the deterioration of lip and jaw movements during speech longitudinally in three individuals diagnosed with bulbar amyotrophic lateral sclerosis (ALS). The study was motivated by the need to understand the relationship between physiologic changes in speech movements and clinical measures of speech…

  10. Progressive Supranuclear Palsy: an Update.

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    Armstrong, Melissa J

    2018-02-17

    Progressive supranuclear palsy (PSP) is a 4R tau neuropathologic entity. While historically defined by the presence of a vertical supranuclear gaze palsy and falls in the first symptomatic year, clinicopathologic studies identify alternate presenting phenotypes. This article reviews the new PSP diagnostic criteria, diagnostic approaches, and treatment strategies. The 2017 International Parkinson and Movement Disorder Society PSP criteria outline 14 core clinical features and 4 clinical clues that combine to diagnose one of eight PSP phenotypes with probable, possible, or suggestive certainty. Evidence supports the use of select imaging approaches in the classic PSP-Richardson syndrome phenotype. Recent trials of putative disease-modifying agents showed no benefit. The new PSP diagnostic criteria incorporating the range of presenting phenotypes have important implications for diagnosis and research. More work is needed to understand how diagnostic evaluations inform phenotype assessment and identify expected progression. Current treatment is symptomatic, but tau-based therapeutics are in active clinical trials.

  11. Genetics Home Reference: progressive supranuclear palsy

    Science.gov (United States)

    ... slow and slurred speech (dysarthria) and trouble swallowing (dysphagia). Most affected individuals also experience changes in personality ... UK): Treatment of Progressive Supranuclear Palsy Partners in Parkinson's: Movement Disorder Specialist Finder University of California, San ...

  12. A longitudinal study of motor, oculomotor and cognitive function in progressive supranuclear palsy.

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    Boyd C P Ghosh

    Full Text Available We studied the annual change in measures of motor, oculomotor and cognitive function in progressive supranuclear palsy. This had twin objectives, to assess the potential for clinical parameters to monitor disease progression in clinical trials and to illuminate the progression of pathophysiology.Twenty three patients with progressive supranuclear palsy (Richardson's syndrome were compared to 22 matched controls at baseline and 16 of these patients compared at baseline and one year using: the progressive supranuclear palsy rating scale; the unified Parkinson's disease rating scale; the revised Addenbrooke's cognitive examination; the frontal assessment battery; the cubes section of the visual object and space perception battery; the Hayling and Brixton executive tests; and saccadic latencies.Patients were significantly impaired in all domains at baseline. However, cognitive performance was maintained over a year on the majority of tests. The unified Parkinson's disease rating scale, saccadic latency and progressive supranuclear palsy rating scale deteriorated over a year, with the latter showing the largest change. Power estimates indicate that using the progressive supranuclear palsy rating scale as an outcome measure in a clinical trial would require 45 patients per arm, to identify a 50% reduction in rate of decline with 80% power.Motor, oculomotor and cognitive domains deteriorate at different rates in progressive supranuclear palsy. This may be due to differential degeneration of their respective cortical-subcortical circuits, and has major implications for the selection of outcome measures in clinical trials due to wide variation in sensitivity to annual rates of decline.

  13. Progressive supranuclear palsy syndrome induced by clebopride.

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    Campdelacreu, Jaume; Kumru, Hatice; Tolosa, Eduard; Valls-Solé, Josep; Benabarre, Antoni

    2004-04-01

    We report on a patient who presented with a progressive supranuclear palsy (PSP) syndrome while receiving clebopride (CLB), a prokinetic drug with central antidopaminergic properties. The clinical and neurophysiological signs progressively disappeared after CLB withdrawal. To our knowledge, this is the first published PSP-like syndrome attributable to an antidopaminergic drug. Copyright 2003 Movement Disorder Society

  14. Specific magnetic resonance imaging findings in patient with progressive supranuciear palsy

    International Nuclear Information System (INIS)

    Vuchkova, R.; Zlatareva, D.

    2013-01-01

    Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease. The clinical symptoms are vertical supranuclear palsy, gait and postural instability, cognitive deficit, Parkinsonism. It is not always possible to differentiate clinically PSP from other atypical Parkinsonian syndromes and from Parkinson disease. We present a case of 56-years old women with clinically suspected PSP. The typical magnetic resonance findings were of most importance in diagnosis. For diagnosing the disease by means of neuroimaging it is necessary to analyze the sagittal MR images what is achievable in daily routine practice without additional software and post processing. (authors)

  15. A protocol for identification of early bulbar signs in amyotrophic lateral sclerosis.

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    Ball, L J; Willis, A; Beukelman, D R; Pattee, G L

    2001-10-15

    The purpose of this project is to identify characteristics that may be of assistance in establishing the diagnosis and monitoring early progression of bulbar dysfunction in patients with Amyotrophic Lateral Sclerosis (ALS). Early identification of bulbar dysfunction would assist in clinical trials and management decisions. A database of 218 clinic visits of patients with ALS was developed and formed the basis for these analyses. As a framework for the description of our methodology, the Disablement Model [World Health Organization. WHO International classification of impairment, activity, and participation: beginner's guide. In: WHO, editor. Beta-1 draft for field trials; 1999] was utilized. Our data identified that the strongest early predictors of bulbar speech dysfunction include altered voice quality (laryngeal control), speaking rate, and communication effectiveness. A protocol for measuring these speech parameters was therefore undertaken. This paper presents the protocol used to measure these bulbar parameters.

  16. Genetics of Progressive Supranuclear Palsy

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    Sun Young Im

    2015-09-01

    Full Text Available Progressive supranuclear palsy (PSP is a neurodegenerative syndrome that is clinically characterized by progressive postural instability, supranuclear gaze palsy, parkinsonism and cognitive decline. Pathologically, diagnosis of PSP is based on characteristic features, such as neurofibrillary tangles, neutrophil threads, tau-positive astrocytes and their processes in basal ganglia and brainstem, and the accumulation of 4 repeat tau protein. PSP is generally recognized as a sporadic disorder; however, understanding of genetic background of PSP has been expanding rapidly. Here we review relevant publications to outline the genetics of PSP. Although only small number of familial PSP cases have been reported, the recognition of familial PSP has been increasing. In some familial cases of clinically probable PSP, PSP pathologies were confirmed based on NINDS neuropathological diagnostic criteria. Several mutations in MAPT, the gene that causes a form of familial frontotemporal lobar degeneration with tauopathy, have been identified in both sporadic and familial PSP cases. The H1 haplotype of MAPT is a risk haplotype for PSP, and within H1, a sub-haplotype (H1c is associated with PSP. A recent genome-wide association study on autopsyproven PSP revealed additional PSP risk alleles in STX6 and EIF2AK3. Several heredodegenerative parkinsonian disorders are referred to as PSP-look-alikes because their clinical phenotype, but not their pathology, mimics PSP. Due to the fast development of genomics and bioinformatics, more genetic factors related to PSP are expected to be discovered. Undoubtedly, these studies will provide a better understanding of the pathogenesis of PSP and clues for developing therapeutic strategies.

  17. Progressive supranuclear palsy presenting as primary lateral sclerosis but lacking parkinsonism, gaze palsy, aphasia, or dementia.

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    Nagao, Shigeto; Yokota, Osamu; Nanba, Reiko; Takata, Hiroshi; Haraguchi, Takashi; Ishizu, Hideki; Ikeda, Chikako; Takeda, Naoya; Oshima, Etsuko; Sakane, Katsuaki; Terada, Seishi; Ihara, Yuetsu; Uchitomi, Yosuke

    2012-12-15

    We report an autopsy case of progressive supranuclear palsy (PSP) that clinically showed only slowly progressive and symmetric upper motor neuron syndrome over a disease course of 12 years. A female patient initially exhibited dysarthria at the age of 65, followed by gait disturbance and dysphagia. Neurological examination at age 67 disclosed pseudobulbar palsy, spastic gait, hyperreflexia, and presence of bilateral Hoffmann and Babinski signs. However, muscle atrophy, weakness, evidence of denervation on electromyography, vertical gaze palsy, parkinsonism, gait freezing, aphasia, speech apraxia, or dementia was not noted throughout the course. She was clinically diagnosed as having motor neuron disease consistent with so-called primary lateral sclerosis. Pathological examination disclosed histopathological features of PSP, including argyrophilic and tau-positive tufted astrocytes, neurofibrillary tangles, coiled bodies, and thread-like processes in the motor cortex and superior frontal gyrus, and to a lesser degree, in the basal ganglia and brain stem nuclei. In addition, severe fibrillary gliosis was noted in the precentral gyrus and corticospinal tract, being consistent with upper motor neuron syndrome observed in this case. No TAR-DNA binding protein 43-positive lesion, FUS pathology, Bunina body, or Lewy body-like hyaline inclusion was noted in the motor cortex or lower motor neurons. These findings suggest that when tau pathology is prominent in the motor cortex but is minimal in the basal ganglia and brain stem nuclei, a PSP case can lack all classic clinical features of PSP and show only slowly progressive upper motor syndrome, consistent with clinical picture of primary lateral sclerosis. Copyright © 2012 Elsevier B.V. All rights reserved.

  18. Computed tomographic findings in progressive supranuclear palsy

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    Saitoh, H; Yoshii, F; Shinohara, Y

    1987-03-01

    CT findings of 6 patients with progressive supranuclear palsy (PSP) are described, with emphasis on their supratentorial changes in comparison with those of control subjects and patients with Parkinson's disease (PD). As estimated from CT films, the lateral ventricles, third ventricle and prepontine cistern were significantly enlarged in PSP patients compared with both controls and PD patients. It is suggested that the patients with PSP have not only infratentorial but also supratentorial lesions.

  19. Thirty-six Cases of Pseudobulbar Palsy Treated by Needling with Prompt and Deep Insertion

    Institute of Scientific and Technical Information of China (English)

    Chen Hong

    2006-01-01

    @@ Pseudobulbar palsy refers to bulbar paralysis due to the upper motor neuron injury, which is one of the severe complications of cerebrovascular diseases.The author has treated 36 cases of the disease with acupuncture by a prompt and deep insertion technique, and achieved satisfactory therapeutic results. A report follows.

  20. Spinal and bulbar muscular atrophy.

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    Lieberman, Andrew P

    2018-01-01

    Spinal and bulbar muscular atrophy (SBMA) is an adult-onset degenerative disorder of the neuromuscular system resulting in slowly progressive weakness and atrophy of the proximal limb and bulbar muscles. The disease is caused by the expansion of a CAG/glutamine tract in the amino-terminus of the androgen receptor. That SBMA exclusively affects males reflects the fact that critical pathogenic events are hormone-dependent. These include translocation of the polyglutamine androgen receptor from the cytoplasm to the nucleus and unfolding of the mutant protein. Studies of the pathology of SBMA subjects have revealed nuclear aggregates of the mutant androgen receptor, loss of lower motor neurons in the brainstem and spinal cord, and both neurogenic and myopathic changes in skeletal muscle. Mechanisms underlying disease pathogenesis include toxicity in both lower motor neurons and skeletal muscle, where effects on transcription, intracellular transport, and mitochondrial function have been documented. Therapies to treat SBMA patients remain largely supportive, although experimental approaches targeting androgen action or promoting degradation of the mutant androgen receptor protein or the encoding RNA are under active study. Copyright © 2018 Elsevier B.V. All rights reserved.

  1. Motor neuron disease in blacks

    African Journals Online (AJOL)

    1989-08-19

    Aug 19, 1989 ... amyotrophic lateral sclerosis (ALS) - 27 men (75%), 12 women (52%). Those with predominant affection of bulbar muscles, including those of LMN type, were classified as progressive bulbar palsy (BP - 5 men, 6 women) and those with predominant LMN involvement of limb and trunk muscles were labelled ...

  2. A protocol for comprehensive assessment of bulbar dysfunction in amyotrophic lateral sclerosis (ALS).

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    Yunusova, Yana; Green, Jordan R; Wang, Jun; Pattee, Gary; Zinman, Lorne

    2011-02-21

    Improved methods for assessing bulbar impairment are necessary for expediting diagnosis of bulbar dysfunction in ALS, for predicting disease progression across speech subsystems, and for addressing the critical need for sensitive outcome measures for ongoing experimental treatment trials. To address this need, we are obtaining longitudinal profiles of bulbar impairment in 100 individuals based on a comprehensive instrumentation-based assessment that yield objective measures. Using instrumental approaches to quantify speech-related behaviors is very important in a field that has primarily relied on subjective, auditory-perceptual forms of speech assessment(1). Our assessment protocol measures performance across all of the speech subsystems, which include respiratory, phonatory (laryngeal), resonatory (velopharyngeal), and articulatory. The articulatory subsystem is divided into the facial components (jaw and lip), and the tongue. Prior research has suggested that each speech subsystem responds differently to neurological diseases such as ALS. The current protocol is designed to test the performance of each speech subsystem as independently from other subsystems as possible. The speech subsystems are evaluated in the context of more global changes to speech performance. These speech system level variables include speaking rate and intelligibility of speech. The protocol requires specialized instrumentation, and commercial and custom software. The respiratory, phonatory, and resonatory subsystems are evaluated using pressure-flow (aerodynamic) and acoustic methods. The articulatory subsystem is assessed using 3D motion tracking techniques. The objective measures that are used to quantify bulbar impairment have been well established in the speech literature and show sensitivity to changes in bulbar function with disease progression. The result of the assessment is a comprehensive, across-subsystem performance profile for each participant. The profile, when compared to

  3. A case of atypical progressive supranuclear palsy

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    Spaccavento S

    2013-12-01

    Full Text Available Simona Spaccavento, Marina Del Prete, Angela Craca, Anna Loverre IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy Background: Progressive supranuclear palsy (PSP is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progressive apraxia of speech (AOS and aphasia. Aim: We aimed to investigate the clinical presentation of PSP, using a comprehensive multidimensional evaluation, and the disease response to various pharmacological treatments. Methods: A 72-year-old right-handed male, with 17 years education, who first presented with aphasia, AOS, depression, apathy, and postural instability at 69 years; a complete neuropsychological evaluation, tapping the different cognitive domains, was performed. Results: Testing revealed a moderate global cognitive deficit (Mini-Mental State Examination test score =20, low memory test scores (story recall, Rey’s 15-word Immediate and Delayed Recall, and poor phonemic and semantic fluency. The patient’s language was characterized by AOS, with slow speech rate, prolonged intervals between syllables and words, decreased articulatory accuracy, sound distortions, and anomia. Behavioral changes, such as depression, anxiety, apathy, and irritability, were reported. The neurological examination revealed supranuclear vertical gaze palsy, poor face miming, and a mild balance deficit. Magnetic resonance imaging showed only widespread cortical atrophy. Single photon emission computed tomography demonstrated left > right frontotemporal cortical abnormalities. After 6 months, a further neuropsychological assessment showed a progression in cognitive deficits, with additional attention deficits. The patient reported frequent falls, but the neurological deficits remained unchanged. Neuroimaging tests showed the same brain involvement. Conclusion: Our case highlights the heterogeneity of the clinical features in

  4. Horizontal gaze palsy with progressive scoliosis: CT and MR findings

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    Bomfim, Rodrigo C.; Tavora, Daniel G.F.; Nakayama, Mauro; Gama, Romulo L. [Sarah Network of Rehabilitation Hospitals, Department of Radiology, Ceara (Brazil)

    2009-02-15

    Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare congenital disorder characterized by absence of conjugate horizontal eye movements and progressive scoliosis developing in childhood and adolescence. We present a child with clinical and neuroimaging findings typical of HGPPS. CT and MRI of the brain demonstrated pons hypoplasia, absence of the facial colliculi, butterfly configuration of the medulla and a deep midline pontine cleft. We briefly discuss the imaging aspects of this rare entity in light of the current literature. (orig.)

  5. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy

    NARCIS (Netherlands)

    G. Hoglinger (Gunter); N.M. Melhem (Nadine); D. Dickson (Dennis); P.M.A. Sleiman (Patrick); L.-S. Wang; L. Klei (Lambertus); R. Rademakers (Rosa); R. de Silva (Rohan); I. Litvan (Irene); D.E. Riley (David); J.C. van Swieten (John); P. Heutink (Peter); Z.K. Wszolek (Zbigniew); R.J. Uitti (Ryan); J. Vandrovcova (Jana); H.I. Hurtig (Howard); R.G. Gross (Rachel); W. Maetzler (Walter); S. Goldwurm (Stefano); E. Tolosa; B. Borroni (Barbara); P. Pastor (Pau); L.B. Cantwell (Laura); M.R. Han; A. Dillman (Allissa); M.P. van der Brug (Marcel); J. Gibbs (Raphael); M.R. Cookson (Mark); D.G. Hernandez (Dena); A. Singleton (Andrew); M.J. Farrer (Matthew); C.-E. Yu (Changen); L.I. Golbe (Lawrence); T. Revesz (Tamas); J. Hardy (John); A.J. Lees (Andrew); B. Devlin (Bernie); H. Hakonarson (Hakon); U. Müller (Ulrich); G.D. Schellenberg (Gerard); R.L. Albin (Roger); E. Alonso (Elena); M. Apfelbacher (Manuela); S.E. Arnold (Steven); J. Avila (Jesús); T.G. Beach (Thomas); S. Beecher (Sherry); D. Berg (Daniela); T.D. Bird (Thomas); N. Bogdanović (Nenad); A.J.W. Boon (Andrea); Y. Bordelon (Yvette); A. Brice (Alexis); H. Budka (Herbert); M. Canesi (Margherita); W.Z. Chiu (Wang Zheng); R. Cilia (Roberto); C. Colosimo (Carlo); P.P. de Deyn (Peter); J.G. de Yebenes; L. Donker Kaat (Laura); R. Duara (Ranjan); A. Durr; S. Engelborghs (Sebastiaan); G. Fabbrini (Giovanni); N.A. Finch (Nicole); R. Flook (Robyn); M.P. Frosch (Matthew); C. Gaig; D. Galasko (Douglas); T. Gasser (Thomas); M. Gearing (Marla); E.T. Geller (Evan); B. Ghetti (Bernardino); N.R. Graff-Radford (Neill); M. Grossman (Murray); D.A. Hall (Deborah); L.-N. Hazrati; M. Höllerhage (Matthias); J. Jankovic (Joseph); J.L. Juncos (Jorge); A. Karydas (Anna); H.A. Kretzschmar (Hans); I. Leber (Isabelle); V.M.Y. Lee (Virginia); A.P. Lieberman (Andrew); K.E. Lyons (Kelly); C. Mariani (Claudio); E. Masliah (Eliezer); L.A. Massey (Luke); C.A. McLean (Catriona); N. Meucci (Nicoletta); B.L. Miller (Bruce); B. Mollenhauer (Brit); J.C. Möller (Jens); H. Morris (Huw); S.S. O'Sullivan (Sean); W. Oertel; D. Ottaviani (Donatella); A. Padovani (Alessandro); R. Pahwa (Rajesh); G. Pezzoli (Gianni); S. Pickering-Brown (Stuart); W. Poewe (Werner); A. Rabano (Alberto); A. Rajput (Alex); S.G. Reich (Stephen); G. Respondek (Gesine); S. Roeber (Sigrun); J.D. Rohrer (Jonathan Daniel); O.A. Ross (Owen); M. Rossor (Martin); G. Sacilotto (Giorgio); W.W. Seeley (William); K. Seppi (Klaus); L. Silveira-Moriyama (Laura); S. Spina (Salvatore); K. Srulijes (Karin); P. St. George-Hyslop (Peter); M. Stamelou (Maria); D.G. Standaert (David); S. Tesei (Silvana); W.W. Tourtellotte (Wallace); C. Trenkwalder (Claudia); C. Troakes (Claire); J.Q. Trojanowski (John); J.C. Troncoso (Juan); V.M. Deerlin (Vivianna); J.P.G. Vonsattel; G.K. Wenning (Gregor); C.L. White III (Charles); P. Winter (Pia); C. Zarow (Chris); A.L. Zecchinelli (Anna); A. Antonini (Angelo)

    2011-01-01

    textabstractProgressive supranuclear palsy (PSP) is a movement disorder with prominent tau neuropathology. Brain diseases with abnormal tau deposits are called tauopathies, the most common of which is Alzheimer's disease. Environmental causes of tauopathies include repetitive head trauma associated

  6. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy

    NARCIS (Netherlands)

    Hoglinger, G.U.; Melhem, N.M.; Dickson, D.W.; Sleiman, P.M.A.; Wang, L.S.; Klei, L.; Rademakers, R.; de Silva, R.; Litvan, I.; Riley, D.E.; van Swieten, J.C.; Heutink, P.; Wszolek, Z.K.; Uitti, R.J.; Vandrovcova, J.; Hurtig, H.I.; Gross, R.G.; Maetzler, W.; Goldwurm, S.; Tolosa, E.; Borroni, B.; Pastor, P.; Cantwell, L.B.; Han, M.R.; Dillman, A.; van der Brug, M.P.; Gibbs, J.R.; Cookson, M.R.; Hernandez, D.G.; Singleton, A.B.; Farrer, M.J.; Yu, C.E.; Golbe, L.I.; Revesz, T.; Hardy, J.; Lees, A.J.; Devlin, B.; Hakonarson, H.; Muller, U.; Schellenberg, G.D.

    2011-01-01

    Progressive supranuclear palsy (PSP) is a movement disorder with prominent tau neuropathology. Brain diseases with abnormal tau deposits are called tauopathies, the most common of which is Alzheimer's disease. Environmental causes of tauopathies include repetitive head trauma associated with some

  7. Head and Arm Tremor in X-linked Spinal and Bulbar Muscular Atrophy

    Directory of Open Access Journals (Sweden)

    Irene Aicua

    2014-10-01

    Full Text Available Background: X‐linked spinal and bulbar muscular atrophy (SBMA is a rare adult‐onset neuronopathy. Although tremor is known to occur in this disease, the number of reported cases of SBMA with tremor is rare, and the number with videotaped documentation is exceedingly rare. Our aim was to describe/document the characteristic signs of tremor in spinal and bulbar muscular atrophy.Case Report: We report a case of a 58‐year‐old male with a positive family history of tremor. On examination, the patient had jaw and hand tremors but he also exhibited gynecomastia, progressive bulbar paresis, and wasting and weakness primarily in the proximal limb muscles. The laboratory tests revealed an elevated creatine phosphokinase. Genetic testing was positive for X‐SBMA, with 42 CAG repeats.Discussion: Essential tremor is one of the most common movement disorders, yet it is important for clinicians to be aware of the presence of other distinguishing features that point to alternative diagnoses. The presence of action tremor associated with muscle atrophy and gynecomastia should lead to a suspicion of SBMA.

  8. Epidemiological survey of X-linked bulbar and spinal muscular atrophy, or Kennedy disease, in the province of Reggio Emilia, Italy.

    Science.gov (United States)

    Guidetti, D; Sabadini, R; Ferlini, A; Torrente, I

    2001-01-01

    Commencing with the work carried out during the epidemiological survey of amyotrophic lateral sclerosis in the period 1980-1992 and the pathology follow-up, we carried out a perspective incidence, prevalence and mortality survey of X-linked bulbar and spinal muscular atrophy (X-BSMA) in the province of Reggio Emilia in Northern Italy. Based on 11 patients (eight familial and three sporadic cases), the mean incidence per year for the period 1980 through 1997, as evaluated at the onset of symptoms, was 0.09 cases/100,000 for the total population and 0.19 cases/100,000 for the male population. On December 31, 1997, the prevalence rate was 1.6/100,000 for the total population and 3.3/100,000 for the male population. In the 18-year period of 1980-1997, the average yearly mortality rate was: 0.03 cases/100,000 per year for the total population and 0.06 cases/ 100,000 for the male population. The average age at onset was 44.8 +/- 10.1, and the average survival period was 27.3 +/- 2.3 years. The average age of the prevalence day was 58.9 +/- 14.9, and the average age at death was 71.3 +/- 4.7 years. Whereas the incidence rate of X-BSMA in the province of Reggio Emilia is 16 times lower that of amyotrophic lateral sclerosis (ALS), the incidence rate of progressive bulbar palsy in the male population is only slightly higher than X-BSMA; and the prevalence rate of ALS for males is two times the prevalence rate for X-BSMA, with overlapping of confidence intervals. X-BSMA is a rare disease, which is probably under-diagnosed, but due to the long survival period of this disease its frequency is not negligible. Because of the presence of sporadic cases or non-evident familial cases, it is appropriate to consider this diagnostic possibility in making a diagnosis of ALS in patients in whom lower motor neuron dysfunction or bulbar onset predominates.

  9. Factors Associated with Enhanced Gross Motor Progress in Children with Cerebral Palsy: A Register-Based Study.

    Science.gov (United States)

    Størvold, Gunfrid V; Jahnsen, Reidun B; Evensen, Kari Anne I; Romild, Ulla K; Bratberg, Grete H

    2018-05-01

    To examine associations between interventions and child characteristics; and enhanced gross motor progress in children with cerebral palsy (CP). Prospective cohort study based on 2048 assessments of 442 children (256 boys, 186 girls) aged 2-12 years registered in the Cerebral Palsy Follow-up Program and the Cerebral Palsy Register of Norway. Gross motor progress estimates were based on repeated measures of reference percentiles for the Gross Motor Function Measure (GMFM-66) in a linear mixed model. Mean follow-up time: 2.9 years. Intensive training was the only intervention factor associated with enhanced gross motor progress (mean 3.3 percentiles, 95% CI: 1.0, 5.5 per period of ≥3 sessions per week and/or participation in an intensive program). Gross motor function was on average 24.2 percentiles (95% CI: 15.2, 33.2) lower in children with intellectual disability compared with others. Except for eating problems (-10.5 percentiles 95% CI: -18.5, -2.4) and ankle contractures by age (-1.9 percentiles 95% CI: -3.6, -0.2) no other factors examined were associated with long-term gross motor progress. Intensive training was associated with enhanced gross motor progress over an average of 2.9 years in children with CP. Intellectual disability was a strong negative prognostic factor. Preventing ankle contractures appears important for gross motor progress.

  10. Pathogenesis-targeting therapeutics for spinal and bulbar muscular atrophy (SBMA).

    Science.gov (United States)

    Suzuki, Keisuke; Kastuno, Masahisa; Banno, Haruhiko; Sobue, Gen

    2009-08-01

    Spinal and bulbar muscular atrophy (SBMA) is an hereditary, adult-onset, lower motor neuron disease caused by an aberrant elongation of a trinucleotide CAG repeat, which encodes the polyglutamine tract, in the first exon of the androgen receptor (AR) gene. The main symptoms are slowly progressive muscle weakness and atrophy of bulbar, facial and limb muscles. The cardinal histopathological findings of SBMA are an extensive loss of lower motor neurons in the anterior horn of the spinal cord as well as in brainstem motor nuclei and intranuclear accumulations of mutant AR protein in the residual motor neurons. Androgen deprivation therapy rescues neuronal dysfunction in animal models of SBMA, suggesting that the molecular basis for motor neuron degeneration in this disorder is testosterone-dependent nuclear accumulation of the mutant AR. Suppression of disease progression by leuprorelin acetate has also been demonstrated in a phase 2 clinical trial. In addition, the clarification of pathophysiology leads to appearance of candidate drugs to treat this devastating disease: heat shock protein (HSP) inducer, Hsp90 inhibitor, and histone deacetylase inhibitor. Advances in basic and clinical research on SBMA are now paving the way for clinical application of pathogenesis-targeting therapeutics.

  11. Regional Cerebral Perfusion in Progressive Supranuclear Palsy

    International Nuclear Information System (INIS)

    Lee, Won Yong; Lee, Ki Hyeong; Yoon, Byung Woo; Lee, Sang Bok; Jeon, Beom S.; Lee, Kyung Han; Lee, Myung Chul

    1996-01-01

    Progressive supranuclear palsy (PSP) is a Parkinson-plus syndrome characterized clinically by supranuclear ophthalmoplegia, pseudobulbar palsy, axial rigidity, bradykinesia, postural instability and dementia. Presence of dementia and lack of cortical histopathology suggest the derangement of cortical function by pathological changes in subcortical structures in PSP, which is supported by the pattern of behavioral changes and measurement of brain metabolism using positron emission tomography. This study was done to examine whether there are specific changes of regional cerebral perfusion in PSP and whether there is a correlation between severity of motor abnormaility and degree of changes in cerebral perfusion. We measured regional cerebral perfusion indices in 5 cortical and 2 subcortical areas in 6 patients with a clinical diagnosis of PSP and 6 healthy age and sex matched controls using Tc-99m-HMPAO SPECT. Compared with age and sex matched controls, only superior frontal regional perfusion index was significantly decreased in PSP (p 0.05). We affirm the previous reports that perfusion in superior frontal cortex is decreased in PSP. Based on our results that there was no correlation between severity of motor abnormality and cerebral perfusion in the superior frontal cortex, nonmotoric symptoms including dementia needs to be looked at whether there is a correlation with the perfusion abnormality in superior frontal cortex

  12. Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options

    OpenAIRE

    Lamb, Ruth; Rohrer, Jonathan D.; Lees, Andrew J.; Morris, Huw R.

    2016-01-01

    Opinion statement There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case series, or expert opinion. Levodopa may provide some improvement in symptoms of Parkinsonism (specif...

  13. Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options

    OpenAIRE

    Lamb, R.; Rohrer, J. D.; Lees, A. J.; Morris, H. R.

    2016-01-01

    There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case series, or expert opinion. Levodopa may provide some improvement in symptoms of Parkinsonism (specifically bradykinesi...

  14. Accuracy of MR markers for differentiating Progressive Supranuclear Palsy from Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Stefano Zanigni

    2016-01-01

    Conclusion: Although several quantitative brain MR markers provided high diagnostic accuracy in differentiating Progressive Supranuclear Palsy-Richardson's Syndrome from Parkinson's disease, the morphometric assessment of midbrain area is the best single diagnostic marker and should be routinely included in the neuroradiological work-up of parkinsonian patients.

  15. Intrathecal baclofen pumps do not accelerate progression of scoliosis in quadriplegic spastic cerebral palsy.

    Science.gov (United States)

    Rushton, Paul R P; Nasto, Luigi A; Aujla, Ranjit K; Ammar, Amr; Grevitt, Michael P; Vloeberghs, Michael H

    2017-06-01

    To compare scoliosis progression in quadriplegic spastic cerebral palsy with and without intrathecal baclofen (ITB) pumps. A retrospective matched cohort study was conducted. Patients with quadriplegic spastic cerebral palsy, GMFCS level 5, treated with ITB pumps with follow-up >1 year were matched to comparable cases by age and baseline Cobb angle without ITB pumps. Annual and peak coronal curve progression, pelvic obliquity progression and need for spinal fusion were compared. ITB group: 25 patients (9 female), mean age at pump insertion 9.4 and Risser 0.9. Initial Cobb angle 25.6° and pelvic tilt 3.2°. Follow-up 4.3 (1.0-7.8) years. Cobb angle at follow-up 76.1° and pelvic tilt 18.9°. Non-ITB group: 25 patients (14 female), mean age at baseline 9.2 and Risser 1.0. Initial Cobb angle 29.7° and pelvic tilt 7.1°. Follow-up 3.5 (1.0-7.5) years. Cobb angle at follow-up 69.1° and pelvic tilt 21.0°. The two groups were statistically similar for baseline age, Cobb angle and Risser grade. Mean curve progression was 13.6°/year for the ITB group vs 12.6°/year for the non-ITB group (p = 0.39). Peak curve progression was similar between the groups. Pelvic tilt progression was comparable; ITB group 4.5°/year vs non-ITB 4.6°/year (p = 0.97). During follow-up 5 patients in the ITB group and 9 in the non-ITB group required spinal fusion surgery for curve progression (p = 0.35). Patients with quadriplegic spastic cerebral palsy with and without ITB pumps showed significant curve progression over time. ITB pumps do not appear to alter the natural history of curve progression in this population.

  16. Aspiration pneumonia and bronchopneumonia in progressive supranuclear palsy treated with qing fei tang: two case reports.

    Science.gov (United States)

    Nozaki, Ichiro; Kato-Motozaki, Yuko; Ikeda, Tokuhei; Takahashi, Kazuya; Tagami, Atsuro; Ishida, Chiho; Komai, Kiyonobu

    2015-03-26

    Qing fei tang, which is used for various respiratory diseases, is useful for reducing relapse of aspiration pneumonia and bronchopneumonia in stroke, but the effect remains unknown in Parkinson's syndrome. We report two cases of Japanese patients with progressive supranuclear palsy and relapsing aspiration pneumonia and bronchopneumonia, which was successfully prevented by qing fei tang. Two Japanese men with progressive supranuclear palsy and receiving total enteral feeding (patient one (66-years-old) and patient two (76-years-old)) had experienced recurrent aspiration pneumonia and bronchopneumonia, which was unresponsive to conventional therapy. The respiratory infection developed twice at intervals of two months in patient one, and nine times at almost monthly intervals in patient two. Thereafter, they were given qing fei tang. After administration of qing fei tang, the respiratory infection reoccurred only once; after 5.5 months for patient one, and six months for patient two. Both of our patients clearly showed a reduced incidence of respiratory infection. Both of our patients clearly showed a reduced incidence of respiratory infection after the administration of qing fei tang. Qing fei tang could be useful for the prevention of recurrent aspiration pneumonia and bronchopneumonia in progressive supranuclear palsy.

  17. Regional Cerebral Perfusion in Progressive Supranuclear Palsy

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Won Yong; Lee, Ki Hyeong; Yoon, Byung Woo; Lee, Sang Bok; Jeon, Beom S. [Samsung Medical Center, Seoul (Korea, Republic of); Lee, Kyung Han; Lee, Myung Chul [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1996-03-15

    Progressive supranuclear palsy (PSP) is a Parkinson-plus syndrome characterized clinically by supranuclear ophthalmoplegia, pseudobulbar palsy, axial rigidity, bradykinesia, postural instability and dementia. Presence of dementia and lack of cortical histopathology suggest the derangement of cortical function by pathological changes in subcortical structures in PSP, which is supported by the pattern of behavioral changes and measurement of brain metabolism using positron emission tomography. This study was done to examine whether there are specific changes of regional cerebral perfusion in PSP and whether there is a correlation between severity of motor abnormaility and degree of changes in cerebral perfusion. We measured regional cerebral perfusion indices in 5 cortical and 2 subcortical areas in 6 patients with a clinical diagnosis of PSP and 6 healthy age and sex matched controls using Tc-99m-HMPAO SPECT. Compared with age and sex matched controls, only superior frontal regional perfusion index was significantly decreased in PSP (p<0.05). There was no correlation between the severity of the motor abnormality and any of the regional cerebral perfusion indices (p>0.05). We affirm the previous reports that perfusion in superior frontal cortex is decreased in PSP. Based on our results that there was no correlation between severity of motor abnormality and cerebral perfusion in the superior frontal cortex, nonmotoric symptoms including dementia needs to be looked at whether there is a correlation with the perfusion abnormality in superior frontal cortex

  18. Review: Non-transecting bulbar urethroplasty using buccal mucosa ...

    African Journals Online (AJOL)

    Review: Non-transecting bulbar urethroplasty using buccal mucosa. S Bugeja, S Ivaz, AV Frost, DE Andrich, AR Mundy. Abstract. Augmentation urethroplasty using oral mucosal graft has become the standard surgical treatment of long bulbar strictures. In very tight strictures the urethral plate is narrowed to the extent that an ...

  19. Apparent diffusion coefficient measurements in progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

    Ohshita, T.; Oka, M.; Imon, Y.; Yamaguchi, S.; Mimori, Y.; Nakamura, S. [Hiroshima Univ. (Japan). School of Medicine

    2000-09-01

    We measured the apparent diffusion coefficient (ADC), using diffusion-weighted imaging (DWI) and signal intensity on T2-weighted MRI in the cerebral white matter of patients with progressive supranuclear palsy (PSP) and age-matched normal subjects. In PSP, ADC in the prefrontal and precentral white matter was significantly higher than in controls. There was no significant difference in signal intensity on T2-weighted images. The ADC did correlate with signal intensity. The distribution of the elevation of ADC may be the consequence of underlying pathological changes, such as neurofibrillary tangles or glial fibrillary tangles in the cortex. Our findings suggest that ADC measurement might be useful for demonstrating subtle neuropathological changes. (orig.)

  20. Download this PDF file

    African Journals Online (AJOL)

    AJNS WEBMASTERS

    1. Departments of Medicine (Neurology Division) & Dermatology, Govt. Medical College & Associated S.M.H.S. Hospital,. Srinagar, Kashmir, India. E-Mail Contact - PARVAIZ A. Shah : parvaizshah11 (at) gmail (dot) com. KEY WORDS: Amyotrophic lateral sclerosis, False positive , Myasthenia gravis ,Progressive bulbar palsy.

  1. Disruption of spatial organization and interjoint coordination in Parkinson's disease, progressive supranuclear palsy, and multiple system atrophy.

    Science.gov (United States)

    Leiguarda, R; Merello, M; Balej, J; Starkstein, S; Nogues, M; Marsden, C D

    2000-07-01

    Patients with basal ganglia diseases may exhibit ideomotor apraxia. To define the nature of the impairment of the action production system, we studied a repetitive gesture of slicing bread by three-dimensional computergraphic analysis in eight nondemented patients with Parkinson's disease in the "on" state, five with progressive supranuclear palsy and four with multiple system atrophy. Two patients with Parkinson's disease and two with progressive supranuclear palsy showed ideomotor apraxia for transitive movements on standard testing. A Selspott II system was used for kinematic analysis of wrist trajectories and angular motions of the shoulder and elbow joints. Patients with Parkinson's disease, progressive supranuclear palsy, and even some with multiple system atrophy exhibited kinematic deficits in the spatial precision of movement and velocity-curvature relationships; in addition, they failed to maintain proper angle/angle relationships and to apportion their relative joint amplitudes normally. Spatial disruption of wrist trajectories was more severe in patients with ideomotor apraxia. We posit that the basal ganglia are part of the parallel parieto-frontal circuits devoted to sensorimotor integration for object-oriented behavior. The severity and characteristics of spatial abnormalities of a transitive movement would therefore depend on the location and distribution of the pathologic process within these circuits.

  2. Cerebral hypometabolism in progressive supranuclear palsy studied with positron emission tomography

    International Nuclear Information System (INIS)

    Foster, N.L.; Gilman, S.; Berent, S.; Morin, E.M.; Brown, M.B.; Koeppe, R.A.

    1988-01-01

    Progressive supranuclear palsy (PSP) is characterized by supranuclear palsy of gaze, axial dystonia, bradykinesia, rigidity, and a progressive dementia. Pathological changes in this disorder are generally restricted to subcortical structures, yet the type and range of cognitive deficits suggest the involvement of many cerebral regions. We examined the extent of functional impairment to cerebral cortical and subcortical structures as measured by the level of glucose metabolic activity at rest. Fourteen patients with PSP were compared to 21 normal volunteers of similar age using 18F-2-fluoro-2-deoxy-D-glucose and positron emission tomography. Glucose metabolism was reduced in the caudate nucleus, putamen, thalamus, pons, and cerebral cortex, but not in the cerebellum in the patients with PSP as compared to the normal subjects. Analysis of individual brain regions revealed significant declines in cerebral glucose utilization in most regions throughout the cerebral cortex, particularly those in the superior half of the frontal lobe. Declines in the most affected regions of cerebral cortex were greater than those in any single subcortical structure. Although using conventional neuropathological techniques the cerebral cortex appears to be unaffected in PSP, significant and pervasive functional impairments in both cortical and subcortical structures are present. These observations help to account for the constellation of cognitive symptoms in individual patients with PSP and the difficulty encountered in identifying a characteristic psychometric profile for this group of patients

  3. Computed tomographic findings of progressive supranuclear palsy compared with Parkinson's disease

    Energy Technology Data Exchange (ETDEWEB)

    Yuki, Nobuhiro; Sato, Shuzo; Yuasa, Tatsuhiko; Ito, Jusuke; Miyatake, Tadashi [Niigata Univ. (Japan). School of Dentistry

    1990-10-01

    We investigated computed tomographic (CT) films of 4 pathologically documented cases of progressive supranuclear palsy (PSP) in which the clinical presentations were atypical and compared the findings with those of 15 patients with Parkinson's disease (PD). Dilatation of the third ventricle, atrophy of the midbrain tegmentum, and enlargement of the interpeduncular cistern toward the aqueduct were found to be the characteristic findings in PSP. Thus, radiological findings can be useful when the differential diagnosis between PSP and PD is clinically difficult. (author).

  4. Word-Retrieval in Two Cases of Progressive Supranuclear Palsy

    Directory of Open Access Journals (Sweden)

    Jennifer M. Gurd

    1997-01-01

    Full Text Available In this paper, we describe the detailed neuropsychological investigation of two cases of progressive supranuclear palsy (PSP and demonstrate word-finding difficulties associated with pervasive problems in word-retrieval. The pattern of deficits resembles that seen in Parkinson’s Disease (PD but is more severe, even in very mild PSP, and appears less amenable to cue facilitation. Performance on a variety of word-production tasks is compared, and experimental controls for motor effects on performance are included. The role of stimulus cues in speeded word-finding is addressed experimentally, as are central executive influences on task performance. This combines with functional brain-scan data from single photon emission computed tomography (SPECT which shows reduced frontal perfusion in one of the two cases.

  5. Improvement of balance after audio-biofeedback A 6-week intervention study in patients with progressive supranuclear palsy

    NARCIS (Netherlands)

    Nicolai, S.; Mirelman, A.; Herman, T.; Zijlstra, A.; Mancini, M.; Becker, C.; Lindeman, U.; Berg, D.; Maetzler, W.

    Progressive supranuclear palsy (PSP) is a neurodegenerative disease with no sufficient treatment options to date. The most devastating symptom is the loss of balance with consecutive falls Based on the observation that postural control improved in patients with vestibular dysfunction after

  6. Completed suicide in a case of clinically diagnosed progressive supranuclear palsy.

    Science.gov (United States)

    Wiener, Jennifer; Moran, Maria T; Haut, Marc W

    2015-08-01

    We present the clinical history and the cognitive and behavioral presentations of a male patient with suspected progressive supranuclear palsy (PSP) who fatally shot himself in the head. We believe his act of suicide was the consequence of impulsivity, rather than primary depression or mood disturbance. In cases of suspected PSP and other atypical parkinsonisms, health professionals must be aware of neurobehavioral risk factors for suicide attempts and completions to promote patient safety; however, the literature on this topic is sparse. Our case highlights the potentially lethal consequences of impulsivity and other neuropsychiatric symptoms in PSP and related syndromes.

  7. Characterization of dopaminergic dysfunction in familial progressive supranuclear palsy: an 18F-dopa PET study

    International Nuclear Information System (INIS)

    Tai, Y.F.; Ahsan, R.L.; Pavese, N.; Brooks, D.J.; Piccini, P.; Yebenes de, J.G.

    2007-01-01

    We analyzed 18 F-dopa PET data from 11 members of kindreds with familial progressive supranuclear palsy (PSP) to characterize their cerebral dopaminergic dysfunction. Three clinically-affected PSP patients showed reduced 18 F-dopa uptake in the striatum, orbitofrontal cortex and amygdala. One asymptomatic subject exhibited progressive putamen dopaminergic dysfunction. 60 % of subjects with abnormal 18 F-dopa scans developed PSP subsequently. This is the first in vivo documentation of cortical dopaminergic deficiency in PSP. Reduced striatal 18 F-dopa uptake in susceptible relatives may predict later clinical disease. (author)

  8. Bulbar ulcer and homeopathy. Ulcera bulbar y homeopatía. Úlcera bulbar y homeopatía.

    OpenAIRE

    Fabio S. Mangolini

    2003-01-01

    It is reported a case of bulbar ulcer healed after homeopathic treatment. This case serves to illustrate the efficacy of Homeopathy even when the patient concomitantly uses conventional remedies. The ulcer was anatomically healed notwithstanding H. pylori positive tests both before and after the treatment.

  9. Risk factors for emergence and progression of scoliosis in children with severe cerebral palsy : a systematic review

    NARCIS (Netherlands)

    Loeters, Marianne J. B.; Maathuis, Carel G. B.; Hadders-Algra, Mijna

    Aim Scoliosis is a frequently occurring and serious complication of severe cerebral palsy (CP). This systematic review aims to the assess the risk factors associated with the emergence and progression of scoliosis in children with CP functioning at level IV or V of the Gross Motor Function

  10. PET studies of brain energy metabolism in a model of subcortical dementia: progressive supranuclear Palsy

    International Nuclear Information System (INIS)

    Blin, J.; Baron, J.C.; Cambon, H.

    1988-01-01

    In 41 patients with clinically determined Progressive Supranuclear Palsy, a model of degenerative subcortical dementia, alterations in regional brain energy metabolism with respect to control subjects have been investigated using positron computed tomography and correlated to clinical and neuropsychological scores. A generalized significant reduction in brain metabolism was found, which predominated in the prefrontal cortex in accordance with, and statistically correlated to, the frontal neuropsychological score

  11. A bulbar artery pseudoaneurysm following traumatic urethral catheterization.

    Science.gov (United States)

    Bettez, Mathieu; Aubé, Melanie; Sherbiny, Mohamed El; Cabrera, Tatiana; Jednak, Roman

    2017-01-01

    Traumatic urethral catheterization may result in a number of serious complications. A rare occurrence is the development of a urethral pseudoaneurysm. We report the case of a 13-year-old male who required placement of a Foley catheter for an orthopedic surgical procedure. The Foley was misplaced in the bulbourethra, resulting in the development of a bulbar artery pseudoaneurysm. Profuse bleeding via the urethra was noted after removal of the catheter, and the patient experienced severe intermittent hematuria during the postoperative period. Cystoscopy revealed a pulsatile mass within the bulbourethra. Angiography confirmed a bulbar artery pseudoaneurysm, which was successfully embolized with resolution of bleeding.

  12. A bulbar artery pseudoaneurysm following traumatic urethral catheterization

    Science.gov (United States)

    Bettez, Mathieu; Aubé, Melanie; Sherbiny, Mohamed El; Cabrera, Tatiana; Jednak, Roman

    2017-01-01

    Traumatic urethral catheterization may result in a number of serious complications. A rare occurrence is the development of a urethral pseudoaneurysm. We report the case of a 13-year-old male who required placement of a Foley catheter for an orthopedic surgical procedure. The Foley was misplaced in the bulbourethra, resulting in the development of a bulbar artery pseudoaneurysm. Profuse bleeding via the urethra was noted after removal of the catheter, and the patient experienced severe intermittent hematuria during the postoperative period. Cystoscopy revealed a pulsatile mass within the bulbourethra. Angiography confirmed a bulbar artery pseudoaneurysm, which was successfully embolized with resolution of bleeding. PMID:28163815

  13. [Effective acupoints for bulbar paralysis by professor GAO Weibin].

    Science.gov (United States)

    Kang, Lianru; Zheng, Shuang

    2016-04-01

    Professor GAO Weibin academically advocates, based on basic theory of TCM and theories of different schools, modern science technology should be used for the methods and principles of acupuncture and Chinese medicine for neuropathy, so as to explore and summarize the rules, characteristics and advantages of TCM for nervous system disease, especially bulbar paralysis. During the treatment of bulbar paralysis, professor GAO creatively proposes the effective acupuncture points such as Gongxue, Tunyan-1, Tunyan-2, Fayin, Tiyan and Zhifanliu from the aspects of neuroanatomy, and analyzes their anatomical structure and action mechanism.

  14. [Advances in genetic research of cerebral palsy].

    Science.gov (United States)

    Wang, Fang-Fang; Luo, Rong; Qu, Yi; Mu, De-Zhi

    2017-09-01

    Cerebral palsy is a group of syndromes caused by non-progressive brain injury in the fetus or infant and can cause disabilities in childhood. Etiology of cerebral palsy has always been a hot topic for clinical scientists. More and more studies have shown that genetic factors are closely associated with the development of cerebral palsy. With the development and application of various molecular and biological techniques such as chromosome microarray analysis, genome-wide association study, and whole exome sequencing, new achievements have been made in the genetic research of cerebral palsy. Chromosome abnormalities, copy number variations, susceptibility genes, and single gene mutation associated with the development of cerebral palsy have been identified, which provides new opportunities for the research on the pathogenesis of cerebral palsy. This article reviews the advances in the genetic research on cerebral palsy in recent years.

  15. Different decision deficits impair response inhibition in progressive supranuclear palsy and Parkinson's disease.

    Science.gov (United States)

    Zhang, Jiaxiang; Rittman, Timothy; Nombela, Cristina; Fois, Alessandro; Coyle-Gilchrist, Ian; Barker, Roger A; Hughes, Laura E; Rowe, James B

    2016-01-01

    Progressive supranuclear palsy and Parkinson's disease have distinct underlying neuropathology, but both diseases affect cognitive function in addition to causing a movement disorder. They impair response inhibition and may lead to impulsivity, which can occur even in the presence of profound akinesia and rigidity. The current study examined the mechanisms of cognitive impairments underlying disinhibition, using horizontal saccadic latencies that obviate the impact of limb slowness on executing response decisions. Nineteen patients with clinically diagnosed progressive supranuclear palsy (Richardson's syndrome), 24 patients with clinically diagnosed Parkinson's disease and 26 healthy control subjects completed a saccadic Go/No-Go task with a head-mounted infrared saccadometer. Participants were cued on each trial to make a pro-saccade to a horizontal target or withhold their responses. Both patient groups had impaired behavioural performance, with more commission errors than controls. Mean saccadic latencies were similar between all three groups. We analysed behavioural responses as a binary decision between Go and No-Go choices. By using Bayesian parameter estimation, we fitted a hierarchical drift-diffusion model to individual participants' single trial data. The model decomposes saccadic latencies into parameters for the decision process: decision boundary, drift rate of accumulation, decision bias, and non-decision time. In a leave-one-out three-way classification analysis, the model parameters provided better discrimination between patients and controls than raw behavioural measures. Furthermore, the model revealed disease-specific deficits in the Go/No-Go decision process. Both patient groups had slower drift rate of accumulation, and shorter non-decision time than controls. But patients with progressive supranuclear palsy were strongly biased towards a pro-saccade decision boundary compared to Parkinson's patients and controls. This indicates a prepotency of

  16. NEYROPSYCHOLOGICAL CONSECUENCES OF CEREBRAL PALSY

    Directory of Open Access Journals (Sweden)

    ANA MARÍA NAVARRO MELENDRO

    2005-01-01

    Full Text Available Cerebral Palsy is defined as a movement alteration result of a non progressive damage witch is permanent in anencephalon that has not acquired its final maturation. Patients that suffer cerebral palsy present learning disabilities,that varies between being completely normal to severe as a consequence of memory, gnosis, praxis, perceptive andlanguage impairments. Nevertheless the consequences of this disease are not always predictable. This paper pretendsto make a description of the cognitive and behavioral deficits that overcomes along with the manifestation of thecerebral palsy and its possible treatment. We used a complete neuropsychological battery to evaluate a 7 years oldpatient who was diagnosed of cerebral palsy and spastic diplegia finding some cognitive impairment in fields such asmnesic, gnosic and attention processes.

  17. Brain Dopamine Transporter Binding and Glucose Metabolism in Progressive Supranuclear Palsy-Like Creutzfeldt-Jakob Disease

    Directory of Open Access Journals (Sweden)

    Eero Rissanen

    2014-01-01

    Full Text Available Here, we present a patient with Creutzfeldt-Jakob disease (CJD who developed initial symptoms mimicking progressive supranuclear palsy (PSP. Before the development of typical CJD symptoms, functional imaging supported a diagnosis of PSP when [123I]-FP-CIT-SPECT showed a defect in striatal dopamine transporter binding, while [18F]-fluorodeoxyglucose PET showed cortical hypometabolism suggestive of Lewy body dementia. However, the postmortem neuropathological examination was indicative of CJD only, without tau protein or Lewy body findings. This case demonstrates that CJD should be taken into account in rapidly progressing atypical cases of parkinsonism, even when functional imaging supports a diagnosis of a movement disorder.

  18. Effectiveness of functional progressive resistance exercise training on walking ability in children with cerebral palsy: A randomized controlled trial

    NARCIS (Netherlands)

    Scholtes, V.A.; Becher, J.G.; Janssen-Potten, Y.J.; Dekkers, H.; Smallenbroek, L.; Dallmeijer, A.J.

    2012-01-01

    The objective of the study was to evaluate the effectiveness of functional progressive resistance exercise (PRE) training on walking ability in children with cerebral palsy (CP).Fifty-one ambulant children with spastic CP (mean age 10 years 5 months, 29 boys) were randomized to an intervention (n=

  19. Non-transecting bulbar urethroplasty using buccal mucosa

    African Journals Online (AJOL)

    S. Bugeja

    S. Ivaz, A.V. Frost, D.E. Andrich, A.R. Mundy. University College London Hospital, Reconstructive Urology Unit, UK. Received 6 September 2015; accepted 30 September 2015. Available online 2 December 2015. KEYWORDS. Urethral stricture;. Bulbar urethroplasty;. Non-transecting;. Buccal mucosal graft. Abstract.

  20. Advancing functional dysconnectivity and atrophy in progressive supranuclear palsy

    Directory of Open Access Journals (Sweden)

    Jesse A. Brown

    2017-01-01

    Full Text Available Progressive supranuclear palsy syndrome (PSP-S results from neurodegeneration within a network of brainstem, subcortical, frontal and parietal cortical brain regions. It is unclear how network dysfunction progresses and relates to longitudinal atrophy and clinical decline. In this study, we evaluated patients with PSP-S (n = 12 and healthy control subjects (n = 20 at baseline and 6 months later. Subjects underwent structural MRI and task-free functional MRI (tf-fMRI scans and clinical evaluations at both time points. At baseline, voxel based morphometry (VBM revealed that patients with mild-to-moderate clinical symptoms showed structural atrophy in subcortex and brainstem, prefrontal cortex (PFC; supplementary motor area, paracingulate, dorsal and ventral medial PFC, and parietal cortex (precuneus. Tf-fMRI functional connectivity (FC was examined in a rostral midbrain tegmentum (rMT-anchored intrinsic connectivity network that is compromised in PSP-S. In healthy controls, this network contained a medial parietal module, a prefrontal-paralimbic module, and a subcortical-brainstem module. Baseline FC deficits in PSP-S were most severe in rMT network integrative hubs in the prefrontal-paralimbic and subcortical-brainstem modules. Longitudinally, patients with PSP-S had declining intermodular FC between the subcortical-brainstem and parietal modules, while progressive atrophy was observed in subcortical-brainstem regions (midbrain, pallidum and posterior frontal (perirolandic cortex. This suggested that later-stage subcortical-posterior cortical change may follow an earlier-stage subcortical-anterior cortical disease process. Clinically, patients with more severe baseline impairment showed greater subsequent prefrontal-parietal cortical FC declines and posterior frontal atrophy rates, while patients with more rapid longitudinal clinical decline showed coupled prefrontal-paralimbic FC decline. VBM and FC can augment disease monitoring in PSP

  1. Different decision deficits impair response inhibition in progressive supranuclear palsy and Parkinson’s disease

    Science.gov (United States)

    Rittman, Timothy; Nombela, Cristina; Fois, Alessandro; Coyle-Gilchrist, Ian; Barker, Roger A.; Hughes, Laura E.; Rowe, James B.

    2016-01-01

    Abstract Progressive supranuclear palsy and Parkinson’s disease have distinct underlying neuropathology, but both diseases affect cognitive function in addition to causing a movement disorder. They impair response inhibition and may lead to impulsivity, which can occur even in the presence of profound akinesia and rigidity. The current study examined the mechanisms of cognitive impairments underlying disinhibition, using horizontal saccadic latencies that obviate the impact of limb slowness on executing response decisions. Nineteen patients with clinically diagnosed progressive supranuclear palsy (Richardson’s syndrome), 24 patients with clinically diagnosed Parkinson’s disease and 26 healthy control subjects completed a saccadic Go/No-Go task with a head-mounted infrared saccadometer. Participants were cued on each trial to make a pro-saccade to a horizontal target or withhold their responses. Both patient groups had impaired behavioural performance, with more commission errors than controls. Mean saccadic latencies were similar between all three groups. We analysed behavioural responses as a binary decision between Go and No-Go choices. By using Bayesian parameter estimation, we fitted a hierarchical drift–diffusion model to individual participants’ single trial data. The model decomposes saccadic latencies into parameters for the decision process: decision boundary, drift rate of accumulation, decision bias, and non-decision time. In a leave-one-out three-way classification analysis, the model parameters provided better discrimination between patients and controls than raw behavioural measures. Furthermore, the model revealed disease-specific deficits in the Go/No-Go decision process. Both patient groups had slower drift rate of accumulation, and shorter non-decision time than controls. But patients with progressive supranuclear palsy were strongly biased towards a pro-saccade decision boundary compared to Parkinson’s patients and controls. This

  2. Bulbar impairment score and survival of stable amyotrophic lateral sclerosis patients after noninvasive ventilation initiation.

    Science.gov (United States)

    Sancho, Jesús; Martínez, Daniel; Bures, Enric; Díaz, José Luis; Ponz, Alejandro; Servera, Emilio

    2018-04-01

    There is general agreement that noninvasive ventilation (NIV) prolongs survival in amyotrophic lateral sclerosis (ALS) and that the main cause of NIV failure is the severity of bulbar dysfunction. However, there is no evidence that bulbar impairment is a contraindication for NIV. The aim of this study was to determine the effect of bulbar impairment on survival in ALS patients with NIV. ALS patients for whom NIV was indicated were included. Those patients who refused NIV were taken as the control group. 120 patients who underwent NIV and 20 who refused NIV were included. The NIV group presented longer survival (median 18.50 months, 95% CI 12.62-24.38 months) than the no-NIV group (3.00 months, 95% CI 0.82-5.18 months) (pNIV, adjusted for NIV failure, were severity of bulbar dysfunction (hazard ratio (HR) 0.5, 95% CI 0.92-0.97; p=0.001) and time spent with oxygen saturation measured by pulse oximetry NIV (HR 1.12, 95% CI 1.01-1.24; p=0.02). Severe bulbar impairment in ALS does not always prevent NIV from being used, but the severity of bulbar dysfunction at NIV initiation and %sleep S pO 2 NIV appear to be the main prognostic factors of NIV failure in ALS.

  3. Rapidly worsening bulbar symptoms in a patient with spinobulbar muscular atrophy

    Directory of Open Access Journals (Sweden)

    Montserrat Diaz-Abad

    2013-12-01

    Full Text Available X-linked spinobulbar muscular atrophy (Kennedy’s disease affects muscles and motor neurons, manifesting as weakness and wasting of bulbar, facial, and proximal limb muscles due to loss of anterior horn cells in the brain and spinal cord. We present the case of a patient with X-linked spinobulbar muscular atrophy with rapidly worsening bulbar symptoms caused by laryngopharyngeal irritation associated with a viral upper respiratory tract infection, seasonal allergies and laryngopharyngeal reflux, who dramatically improved with multimodality therapy.

  4. Efficacy of Āyurvedic treatment using Pañcakarma combined with balance exercises for disability and balance in progressive supranuclear palsy

    Science.gov (United States)

    Jindal, Nitin; Shamkuwar, Manoj K.; Kaur, Jaskirat; Berry, Sadhan

    2012-01-01

    A 55-year-old female presented at Department of Pañcakarma with diagnosis of progressive supranuclear palsy (PSP). For assessing disability, progressive supranuclear palsy rating scale (PSPRS) was used and balance was assessed by using Tetrax Interactive Balance System (IBS) posturography. Āyurvedic treatment was given along with Pañcakarma and balance exercises for 3 months. As part of Āyurvedic treatment, first Virecana karma was done with classical method and then Mātrā basti, Śirobasti, and other palliative treatment was given for 3 months. Amanatidine was not continued during Virecana karma but started thereafter. On comparison with pre-intervention scores, there was a significant improvement in the patient post-treatment. The features which mainly showed improvement were: Eye movements, spontaneous episodes of laughing, dysphagia, dysarthria, double vision, and neck rigidity. Balance showed significant improvement and there was a remarkable decrease in the postural sway. This case study may present new possibilities for treatment of neurodegenerative diseases by Āyurveda. PMID:23929996

  5. Cerebral blood flow, oxygen and glucose metabolism with PET in progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Otsuka, Makoto; Ichiya, Yuici; Kuwabara, Yasuo

    1989-01-01

    Cerebral blood flow, cerebral oxygen metabolic rate and cerebral glucose metabolic rate were measured with positron emission tomography (PET) in four patients with progressive supranuclear palsy (PSP). Decreased blood flow and hypometabolism of oxygen and glucose were found in both subcortical and cortical regions, particularly in the striatum including the head of the caudate nucleus and the frontal cortex. The coupling between blood flow and metabolism was preserved even in the regions which showed decreased blood flow and hypometabolism. These findings indicated the hypofunction, as revealed by decreased blood flow and hypometablolism on PET, both in the striatum and the frontal cortex, and which may underlie the pathophysiological mechanism of motor and mental disturbance in PSP. (author)

  6. Plasma Screening for Progranulin Mutations in Patients with Progressive Supranuclear Palsy and Corticobasal Syndromes.

    Science.gov (United States)

    Galimberti, Daniela; Bertram, Kelly; Formica, Alessandra; Fenoglio, Chiara; Cioffi, Sara M G; Arighi, Andrea; Scarpini, Elio; Colosimo, Carlo

    2016-05-04

    Progranulin gene (GRN) mutations are characterized by heterogeneous presentations. Corticobasal syndrome (CBS) is often associated with GRN mutations, whereas association with progressive supranuclear palsy syndrome (PSPS) is rare. Plasma progranulin levels were evaluated in 34 patients, including 19 with PSPS, 12 with CBS, and 3 with mixed signs, with the purpose to screen for the presence of causal mutations, associated with low levels. We found undetectable levels in a patient with CBS. Sequencing confirmed the presence of the Thr272fs deletion. Progranulin mutation screening is suggested in cases of CBS, even in the absence of positive family history for dementia and/or movement disorders.

  7. The Cerebral Palsy Research Registry: Development and Progress Toward National Collaboration in the United States

    Science.gov (United States)

    Hurley, Donna S.; Sukal-Moulton, Theresa; Msall, Michael E.; Gaebler-Spira, Deborah; Krosschell, Kristin J.; Dewald, Julius P.

    2011-01-01

    Cerebral palsy is the most common neurodevelopmental motor disability in children. The condition requires medical, educational, social, and rehabilitative resources throughout the life span. Several countries have developed population-based registries that serve the purpose of prospective longitudinal collection of etiologic, demographic, and functional severity. The United States has not created a comprehensive program to develop such a registry. Barriers have been large population size, poor interinstitution collaboration, and decentralized medical and social systems. The Cerebral Palsy Research Registry was created to fill the gap between population and clinical-based cerebral palsy registries and promote research in the field. This is accomplished by connecting persons with cerebral palsy, as well as their families, to a network of regional researchers. This article describes the development of an expandable cerebral palsy research registry, its current status, and the potential it has to affect families and persons with cerebral palsy in the United States and abroad. PMID:21677201

  8. Urethroplasty, by perineal approach, for bulbar and membranous urethral strictures in children and adolescents.

    Science.gov (United States)

    Shenfeld, Ofer Z; Gdor, Joshua; Katz, Ran; Gofrit, Ofer N; Pode, Dov; Landau, Ezekiel H

    2008-03-01

    To evaluate the safety and efficacy of urethroplasty for bulbar and membranous urethral strictures using the perineal approach in children and adolescents. Urethroplasty by the perineal approach is considered the best treatment for bulbar and membranous urethral strictures in adults. It is not as clear whether this also holds true in children, because the published data addressing this question are scant. We retrospectively reviewed our urethroplasty database to identify patients who had undergone urethroplasty using the perineal approach surgery at age 1 to 13 years (children) and 14 to 18 years (adolescents). A total of 14 patients who had undergone urethroplasty by the perineal approach were identified, including 5 children (mean age 10.8 years) and 9 adolescents (mean age 16.7 years). Of the 14 patients, 7 had membranous and 7 bulbar urethral strictures. The membranous strictures were all secondary to pelvic fractures. The bulbar strictures were "idiopathic" in 57%, traumatic in 29%, and secondary to hypospadias in 14%. All bulbar strictures had been previously treated for 2.5 years, on average, by repeated dilation or urethrotomy that failed. Anastomotic urethroplasty was used in 79% of the patients and tissue transfer techniques in the remainder. The mean follow-up was 30 months (range 12 to 54). Surgery was primarily successful in 93% of the patients, and subsequently successful in 100%. The mean maximal urinary flow increased from 2.65 mL/s preoperatively to 27.65 mL/s postoperatively. No significant complications occurred, and success was similar in both groups. In pediatric patients, as in adults, bulbar and membranous strictures can be treated successfully with urethroplasty using the perineal approach. These patients should probably not be treated "conservatively" with urethral dilation or endoscopic incision. Longer follow-up is needed to confirm that these good results are maintained as these patients cross into adulthood, especially for those who

  9. A unique case of isolated sebaceous adenoma of the bulbar conjunctiva

    Directory of Open Access Journals (Sweden)

    Hatice Deniz Ilhan

    Full Text Available ABSTRACT Our patient was a 34 year-old male who presented with a painless conjunctival mass that had developed 3 months before his first visit. On performing slit-lamp biomicroscopy, a lobulated pink-yellowish solid mobile mass was observed on the nasal bulbar conjunctival surface of his left eye. The tumor was excised, and histopathologic examination of the tumor revealed a sebaceous adenoma. Systemic examination was normal. No recurrence was observed during the 24-month follow-up period. Sebaceous adenoma of the bulbar conjunctiva is an extremely rare benign tumor, which may be observed to be isolated in the absence of malignancy.

  10. Therapeutic advances in multiple system atrophy and progressive supranuclear palsy.

    Science.gov (United States)

    Poewe, Werner; Mahlknecht, Philipp; Krismer, Florian

    2015-09-15

    Multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) are relentlessly progressive neurodegenerative diseases leading to severe disability and ultimately death within less than 10 y. Despite increasing efforts in basic and clinical research, effective therapies for these atypical parkinsonian disorders are lacking. Although earlier small clinical studies in MSA and PSP mainly focused on symptomatic treatment, advances in the understanding of the molecular underpinnings of these diseases and in the search for biomarkers have paved the way for the first large and well-designed clinical trials aiming at disease modification. Targets of intervention in these trials have included α-synuclein inclusion pathology in the case of MSA and tau-related mechanisms in PSP. Since 2013, four large randomized, placebo-controlled, double-blind disease-modification trials have been completed and published, using rasagiline (MSA), rifampicin (MSA), tideglusib (PSP), or davunetide (PSP). All of these failed to demonstrate signal efficacy with regard to the primary outcome measures. In addition, two randomized, placebo-controlled, double-blind trials have studied the efficacy of droxidopa in the symptomatic treatment of neurogenic orthostatic hypotension, including patients with MSA, with positive results in one trial. This review summarizes the design and the outcomes of these and other smaller trials published since 2013 and attempts to highlight priority areas of future therapeutic research in MSA and PSP. © 2015 International Parkinson and Movement Disorder Society. © 2015 International Parkinson and Movement Disorder Society.

  11. [Facial palsy].

    Science.gov (United States)

    Cavoy, R

    2013-09-01

    Facial palsy is a daily challenge for the clinicians. Determining whether facial nerve palsy is peripheral or central is a key step in the diagnosis. Central nervous lesions can give facial palsy which may be easily differentiated from peripheral palsy. The next question is the peripheral facial paralysis idiopathic or symptomatic. A good knowledge of anatomy of facial nerve is helpful. A structure approach is given to identify additional features that distinguish symptomatic facial palsy from idiopathic one. The main cause of peripheral facial palsies is idiopathic one, or Bell's palsy, which remains a diagnosis of exclusion. The most common cause of symptomatic peripheral facial palsy is Ramsay-Hunt syndrome. Early identification of symptomatic facial palsy is important because of often worst outcome and different management. The prognosis of Bell's palsy is on the whole favorable and is improved with a prompt tapering course of prednisone. In Ramsay-Hunt syndrome, an antiviral therapy is added along with prednisone. We also discussed of current treatment recommendations. We will review short and long term complications of peripheral facial palsy.

  12. Clinical analysis and study on the life quality of patients with radiation encephalopathy after radiotherapy of nasopharyngeal cancer in Guangdong province

    International Nuclear Information System (INIS)

    Tang Yamei; Zhang Yinyin; Xing Yigang; Liu Yimin; Li Yi; Guo Ling

    2005-01-01

    Objective: To investigate clinical manifestations and image features (MRI) of radiation encephalopathy (RE) in nasopharyngeal cancer (NPC) patients of Guangdong Province after radiotherapy and to evaluate the life quality of the patients as well as influencing factors in RE. Methods: The clinical data of 89 patients of RE were retrospectively reviewed. The life quality was evaluated using the LENT/SOMA scoring system in 33 RE and 34 NPC patients received radiotherapy but without RE, who served as the controls. Results: There is significant difference in morbidity in six months between the re-radiotherapy group and the first course radiotherapy group (P<0.05). The initial symptom was varied but mainly showed bulbar palsy. The most common symptoms consisted of bulbar palsy, limb paralysis, sensory abnormality and headache. The magnetic resonance (MR) image showed long T1 and long T2. Patients of the RE group had lower QOL score in the social domain, health and life level (P<0.05) compared with the controls. The results of bivariate correlation statistics shows that there are high correlations between some domain of QOL and headache/nervous system defect/bulbar palsy (P<0.05), indicating the influence of QOL after the onset of illness. Conclusions: The latency of RE is shorter after re-radiotherapy. Radiation encephalopathy negatively affecting some domain of life quality of the patient. The negative effect of bulbar palsy should be taken into account. (authors)

  13. Survival in amyotrophic lateral sclerosis with home mechanical ventilation: the impact of systematic respiratory assessment and bulbar involvement.

    Science.gov (United States)

    Farrero, Eva; Prats, Enric; Povedano, Mónica; Martinez-Matos, J Antonio; Manresa, Frederic; Escarrabill, Joan

    2005-06-01

    To analyze (1) the impact of a protocol of early respiratory evaluation of the indications for home mechanical ventilation (HMV) in patients with amyotrophic lateral sclerosis (ALS), and (2) the effects of the protocol and of bulbar involvement on the survival of patients receiving noninvasive ventilation (NIV). Retrospective study in a tertiary care referral center. HMV was indicated in 86 patients with ALS, with 22 patients (25%) presenting with intolerance to treatment associated with bulbar involvement. Treatment with HMV had been initiated in 15 of 64 patients prior to initiating the protocol (group A) and in the remaining 49 patients after protocol initiation (group B). In group A, the majority of patients began treatment with HMV during an acute episode requiring ICU admission (p = 0.001) and tracheal ventilation (p = 0.025), with a lower percentage of patients beginning HMV treatment without respiratory insufficiency (p = 0.013). No significant differences in survival rates were found between groups A and B among patients treated with NIV. Greater survival was observed in group B (p = 0.03) when patients with bulbar involvement were excluded (96%). Patients without bulbar involvement at the start of therapy with NIV presented a significantly better survival rate (p = 0.03). Multivariate analysis showed bulbar involvement to be an independent prognostic factor for survival (relative risk, 1.6; 95% confidence interval, 1.01 to 2.54; p = 0.04). No significant differences in survival were observed between patients with bulbar involvement following treatment with NIV and those with intolerance, except for the subgroup of patients who began NIV treatment with hypercapnia (p = 0.0002). Early systematic respiratory evaluation in patients with ALS is necessary to improve the results of HMV. Further studies are required to confirm the benefits of NIV treatment in patients with bulbar involvement, especially in the early stages.

  14. Anatomy of cerebellar nucleo-bulbar projections in the rat

    NARCIS (Netherlands)

    T.M. Teune (Thea)

    1999-01-01

    textabstractThe cerebellum is located caudal to the cerebral hemispheres and is connected to the rest of the brain by way of three "peduncles", that convey its afferent and efferent information. The cerebellum is functionally related to spinal, bulbar and cerebral motor systems. Its role in the

  15. Synergistic convergence and split pons in horizontal gaze palsy and progressive scoliosis in two sisters

    Directory of Open Access Journals (Sweden)

    Jain Nitin

    2011-01-01

    Full Text Available Synergistic convergence is an ocular motor anomaly where on attempted abduction or on attempted horizontal gaze, both the eyes converge. It has been related to peripheral causes such as congenital fibrosis of extraocular muscles (CFEOM, congenital cranial dysinnervation syndrome, ocular misinnervation or rarely central causes like horizontal gaze palsy with progressive scoliosis, brain stem dysplasia. We hereby report the occurrence of synergistic convergence in two sisters. Both of them also had kyphoscoliosis. Magnetic resonance imaging (MRI brain and spine in both the patients showed signs of brain stem dysplasia (split pons sign differing in degree (younger sister had more marked changes.

  16. Acute sixth nerve palsy in a young man, beware of the 'red herring'.

    LENUS (Irish Health Repository)

    O'Neill, E C

    2012-02-01

    BACKGROUND: Cranial nerve palsies has several etiologies including vascular insufficiency, neoplasm, trauma and inflammation. Isolated sixth nerve palsy is an extremely rare presenting feature of leukemia. AIM: We describe an unusual ocular presentation of a bilateral progressive sixth nerve palsy in a young male with a preceding head injury. CONCLUSION: Acquired sixth nerve palsies in young adults may be due to trauma but in the absence of a definitive history other systemic processes must be outruled. We describe a case of bilateral sixth nerve palsy in a patient with ALL with no obvious CNS involvement. Potential etiological mechanisms are discussed.

  17. FTLD-TDP with motor neuron disease, visuospatial impairment and a progressive supranuclear palsy-like syndrome: broadening the clinical phenotype of TDP-43 proteinopathies. A report of three cases

    Directory of Open Access Journals (Sweden)

    Holmerová Iva

    2011-05-01

    Full Text Available Abstract Background Frontotemporal lobar degeneration with ubiquitin and TDP-43 positive neuronal inclusions represents a novel entity (FTLD-TDP that may be associated with motor neuron disease (FTLD-MND; involvement of extrapyramidal and other systems has also been reported. Case presentation We present three cases with similar clinical symptoms, including Parkinsonism, supranuclear gaze palsy, visuospatial impairment and a behavioral variant of frontotemporal dementia, associated with either clinically possible or definite MND. Neuropathological examination revealed hallmarks of FTLD-TDP with major involvement of subcortical and, in particular, mesencephalic structures. These cases differed in onset and progression of clinical manifestations as well as distribution of histopathological changes in the brain and spinal cord. Two cases were sporadic, whereas the third case had a pathological variation in the progranulin gene 102 delC. Conclusions Association of a "progressive supranuclear palsy-like" syndrome with marked visuospatial impairment, motor neuron disease and early behavioral disturbances may represent a clinically distinct phenotype of FTLD-TDP. Our observations further support the concept that TDP-43 proteinopathies represent a spectrum of disorders, where preferential localization of pathogenetic inclusions and neuronal cell loss defines clinical phenotypes ranging from frontotemporal dementia with or without motor neuron disease, to corticobasal syndrome and to a progressive supranuclear palsy-like syndrome.

  18. Straddle injuries to the bulbar urethra: management and outcome in 53 patients

    Directory of Open Access Journals (Sweden)

    Mohammed Abd-Alla Elgammal

    2009-08-01

    Full Text Available Objective: To describe our experience with blunt injuries to the bulbar urethra and their late sequelae to identify factors that may affect patient outcome. Materials and Methods: A retrospective study was performed on 53 male patients who presented, between January 2001 and December 2005, with blunt traumatic injury to the bulbar urethra. The definitive diagnosis of urethral rupture was made by retrograde urethrography, where urethral rupture was classified into partial or complete. The minimum follow-up period was 3 years. The initial management was either suprapubic cystostomy or endoscopic urethral realignment over a urethral catheter using a cystoscope to pass a guide-wire over which the catheter was inserted. Stricture formation was managed by visual internal urethrotomy (VIU for passable strictures and urethroplasty (stricture excision and re-anastomosis for impassable strictures or recurrence after VIU. The follow-up period was three years. The results were analyzed by SPSS software (chi-square and Student's-t-test. Results: Stricture formation occurred in 19 of 22 patients (86% with complete urethral rupture and in 10 of 31 (32% with partial rupture (p < 0.001. Strictures occurred in 11 of 31 (35% patients treated initially with suprapubic cystostomy and in 18 of 22 (82% treated with primary urethral realignment (p < 0.001. The success rate after VIU was 15% (4 of 26 patients and after urethroplasty it was 96% (24 of 25 patients (p < 0.001. Conclusions: Suprapubic cystostomy is better than urethral realignment and catheterization as primary management after straddle injury to the bulbar urethra. Stricture excision and re-anastomosis is better than VIU as delayed management for strictures that develop after straddle injury to the bulbar urethra.

  19. A Clinical Study on 1 Case of Patient with Bilateral Simultaneous Bell's Palsy Treated by Hominis Placenta Herbal-Acupuncture

    Directory of Open Access Journals (Sweden)

    Kwon, Kang

    2003-06-01

    Full Text Available Objective : This study was carried out to investigate the progress of bilateral simultaneous facial palsy and the effect of Hominis Placenta herbal-acupunture and the other oriental medical therapies. Methods : We used two methods to research the progress of disease. 1. Diagnosis - Facial muscle test, Taste test, Hearing test, Photographies, Lab-finding 2. Treatment - Acupuncture, Herbal-acupuncture, Electroacupuncture, Herb-med Results : The onset of Rt. facial palsy was earlier than Lt. facial palsy 3days. The reaction on the treatment of Rt. facial palsy was more dull than Lt. facial palsy. In terms of treatment period, Rt. facial palsy was very longer than Lt. facial palsy. Conclusion : According to the above results, we discoveried that Hominis Placenta herbal-acupunture and the other oriental medical therapies had good influence on the bilateral simultaneous facial palsy. In the future, we should endeavor to know influence between Rt. and Lt. face in case of bilateral simultaneous Bell's palsy.

  20. Klinefelter′s syndrome associated with progressive muscular atrophy simulating Kennedy′s disease

    OpenAIRE

    Pedro Enrique Jiménez Caballero

    2012-01-01

    Kennedy's disease, an X-linked spinal and bulbar muscular atrophy, is characterized by loss of lower motor neurons. Mild sensory deficits, gynecomastia and infertility may be observed. Klinefelter's syndrome is a variation of sex chromosome disorder characterized by hypogonadism, gynecomastia and azoospermia, and the most frequent karyotype is XXY. A 55-year-old man who presented with slowly progressive and diffuse neurogenic muscle atrophy without bulbar or sensory symptoms. He also had Klin...

  1. Characteristics of Nonmotor Symptoms in Progressive Supranuclear Palsy

    Directory of Open Access Journals (Sweden)

    Ruwei Ou

    2016-01-01

    Full Text Available Objectives. To explore the clinical correlates of nonmotor symptoms (NMS in progressive supranuclear palsy (PSP and their differences from healthy controls and patients with Parkinson’s disease (PD. Methods. Twenty-seven PSP patients, 27 age- and gender-matched healthy controls (HC, and 27 age- and gender-matched PD patients were included for this case-control study. NMS were assessed using the Nonmotor Symptoms Scale (NMSS, including 9 domains. Results. All PSP patients reported NMS. The frequency and severity of “sleep/fatigue,” “mood/apathy,” “attention/memory,” “gastrointestinal,” “sexual dysfunction,” and “miscellaneous” domains in PSP group were significantly higher than those in HC group (P<0.05. The frequency of “mood/apathy,” “attention/memory,” and “sexual dysfunction” domains and the severity of “attention/memory” and “gastrointestinal” domains in PSP group were significantly higher than those in PD group (P<0.05. The “attention/memory” domain in PSP had a significant but weak-to-moderate correlation with age (R=0.387, P=0.046 and onset age (R=0.406, P=0.036. Conclusions. NMS are common in PSP patients. Patients with PSP seem to be subjected to more frequent and severe specific NMS compared to healthy aging subjects and PD patients. Older PSP patients and late-onset patients are likely to be subjected to cognitive decline.

  2. Surgical Repair of Bulbar Urethral Strictures: Advantages of Ventral, Dorsal, and Lateral Approaches and When to Choose Them

    Directory of Open Access Journals (Sweden)

    Krishnan Venkatesan

    2015-01-01

    Full Text Available Objectives. To review the available literature describing the three most common approaches for buccal mucosal graft (BMG augmentation during reconstruction of bulbar urethral strictures. Due to its excellent histological properties, buccal mucosa graft is now routinely used in urethral reconstruction. The best approach for the placement of such a graft remains controversial. Methods. PubMed search was conducted for available English literature describing outcomes of bulbar urethroplasty augmentation techniques using dorsal, ventral, and lateral approaches. Prospective and retrospective studies as well as meta-analyses and latest systematic reviews were included. Results. Most of the studies reviewed are of retrospective nature and majority described dorsal or ventral approaches. Medium- and long-term outcomes of all three approaches were comparable ranging between 80 and 88%. Conclusion. Various techniques of BMG augmentation urethroplasty have been described for repairs of bulbar urethral strictures. In this review, we describe and compare the three most common “competing” approaches for bulbar urethroplasty with utilization of BMG.

  3. Bell's Palsy Treatment

    Science.gov (United States)

    ... Stories Español Eye Health / Eye Health A-Z Bell's Palsy Sections What Is Bell's Palsy? Bell's Palsy Symptoms Bell's Palsy Treatment Bell's Palsy Treatment Leer en Español: Tratamiento de la parálisis ...

  4. Bell's Palsy Symptoms

    Science.gov (United States)

    ... Stories Español Eye Health / Eye Health A-Z Bell's Palsy Sections What Is Bell's Palsy? Bell's Palsy Symptoms Bell's Palsy Treatment Bell's Palsy Symptoms Leer en Español: Síntomas de la parálisis ...

  5. Short Segment Bulbar Urethral Strictures: Review of 48 Cases ...

    African Journals Online (AJOL)

    2017-09-14

    Sep 14, 2017 ... Fall astride injuries accounted for most strictures, 39(89.3%) of cases. The mean stricture lenght was 1.04 cm ± 0.49 and ... such as the elderly with impotence and proximal bulbar strictures in a selected group of patients, BAU ... BUS was diagnosed from history, physical examination, combined retrograde ...

  6. Klinefelter′s syndrome associated with progressive muscular atrophy simulating Kennedy′s disease

    Directory of Open Access Journals (Sweden)

    Pedro Enrique Jiménez Caballero

    2012-01-01

    Full Text Available Kennedy′s disease, an X-linked spinal and bulbar muscular atrophy, is characterized by loss of lower motor neurons. Mild sensory deficits, gynecomastia and infertility may be observed. Klinefelter′s syndrome is a variation of sex chromosome disorder characterized by hypogonadism, gynecomastia and azoospermia, and the most frequent karyotype is XXY. A 55-year-old man who presented with slowly progressive and diffuse neurogenic muscle atrophy without bulbar or sensory symptoms. He also had Klinefelter′s syndrome. Genetic study of Kennedy′s disease was normal. Our patient differs from those with Kennedy′s disease in the absence of bulbar and sensory symptoms. It is suggested that the X chromosome plays an important role in the biology of motor neurons.

  7. Physical fitness in children and adolescents with cerebral palsy

    NARCIS (Netherlands)

    Verschuren, O.W.

    2007-01-01

    Cerebral Palsy (CP) is a non-progressive condition; however, certain negative side effects such as a low muscle strength and cardio respiratory endurance can develop at later stages and can get progressively worse depending on the specifics of a person's condition. Children and adolescents with CP

  8. The effect of intrathecal baclofen treatment on activities of daily life in children and young adults with cerebral palsy and progressive neurological disorders.

    Science.gov (United States)

    Bonouvrié, Laura; Becher, Jules; Soudant, Dan; Buizer, Annemieke; van Ouwerkerk, Willem; Vles, Georges; Vermeulen, R Jeroen

    2016-07-01

    Intrathecal baclofen (ITB) treatment is applied in patients with spastic cerebral palsy (SCP), dystonic cerebral palsy (DCP) and progressive neurological disease (PND). Our aim was to investigate whether ITB treatment has a different effect on activities of daily life (ADL) in these groups. A retrospective and cross-sectional survey was conducted using a questionnaire to assess the qualitative effect of ITB (Likert scale) on different domains of functioning (mobility, personal care, communication, comfort) and satisfaction with the results. Groups were compared using non-parametric statistics. Questionnaires were completed for 68 patients (39 SCP, 13 DCP, 16 PND). Satisfaction scores were relatively high in all groups (7-8) and the positive effect on personal care and communication was similar in all groups. The PND group had the shortest follow-up and scored significantly less favourably for the effect on mobility and comfort. This is the first study to show that ITB treatment has similar effects on personal care and communication in stable and progressive neurological disease. The decrease in mobility in the PND group is likely due to the progressive nature of the disease. The different effect on comfort between groups is mainly due to the smaller effect on startles in the PND group. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  9. Brain hypermetabolism in amyotrophic lateral sclerosis: a FDG PET study in ALS of spinal and bulbar onset

    International Nuclear Information System (INIS)

    Cistaro, Angelina; Fania, Piercarlo; Consuelo Valentini, Maria; Carrara, Giovanna; Chio, Adriano; Calvo, Andrea; Moglia, Cristina; Montuschi, Anna; Nobili, Flavio; Morbelli, Silvia; Salmaso, Dario; Pagani, Marco

    2012-01-01

    To identify the neurobiological traits of amyotrophic lateral sclerosis (ALS) and to elucidate functional differences between ALS of spinal and bulbar onset. We hypothesized that glucose metabolism distribution might vary between groups. The study groups comprised 32 patients with ALS of either bulbar (n = 13) or spinal (n=19) onset and 22 subjects as controls. They were investigated by [ 18 F]fluorodeoxyglucose (FDG) positron emission tomography (FDG PET), comparing the patient groups with each other and with the controls by statistical parametric mapping. Highly significant relative increases in glucose metabolism distribution were found in the group comprising all 32 ALS patients as compared with the controls in the bilateral amygdalae, midbrain, pons and cerebellum. Relative hypermetabolism was also found in patients with spinal onset as compared with the controls in the right midbrain. In patients with bulbar onset compared with the controls and with patients with spinal onset, large relatively hypometabolic areas were found in the bilateral frontal cortex, right insula, anterior cingulate, precuneus and inferior parietal lobe. Patients with spinal onset had significantly higher scores in a neuropsychological test assessing verbal fluency compared with patients with bulbar onset. This large FDG PET investigation provided unprecedented evidence of relatively increased metabolism in the amygdalae, midbrain and pons in ALS patients as compared with control subjects, possibly due to local activation of astrocytes and microglia. Highly significant relative decreases in metabolism were found in large frontal and parietal regions in the bulbar onset patients as compared with the spinal onset patients and the controls, suggesting a differential metabolic and neuropsychological state between the two conditions. (orig.)

  10. Brain hypermetabolism in amyotrophic lateral sclerosis: a FDG PET study in ALS of spinal and bulbar onset

    Energy Technology Data Exchange (ETDEWEB)

    Cistaro, Angelina; Fania, Piercarlo [Positron Emission Tomography Center IRMET S.p.A, Turin (Italy); Consuelo Valentini, Maria; Carrara, Giovanna [CTO Hospital, Department of Neuroradiology, Turin (Italy); Chio, Adriano; Calvo, Andrea; Moglia, Cristina; Montuschi, Anna [University of Turin, Department of Neuroscience, ALS Center, Turin (Italy); Nobili, Flavio [University of Genoa, Department of Neurosciences, Clinical Neurophysiology Unit, Ophthalmology and Genetics, Genoa (Italy); Morbelli, Silvia [University of Genoa, Department of Internal Medicine, Nuclear Medicine Unit, Genoa (Italy); Salmaso, Dario [Institute of Cognitive Sciences and Technologies, CNR, Padua (Italy); Institute of Cognitive Sciences and Technologies, CNR, Rome (Italy); Pagani, Marco [Institute of Cognitive Sciences and Technologies, CNR, Padua (Italy); Karolinska Hospital, Department of Nuclear Medicine, Stockholm (Sweden); Institute of Cognitive Sciences and Technologies, CNR, Rome (Italy)

    2012-02-15

    To identify the neurobiological traits of amyotrophic lateral sclerosis (ALS) and to elucidate functional differences between ALS of spinal and bulbar onset. We hypothesized that glucose metabolism distribution might vary between groups. The study groups comprised 32 patients with ALS of either bulbar (n = 13) or spinal (n=19) onset and 22 subjects as controls. They were investigated by [{sup 18}F]fluorodeoxyglucose (FDG) positron emission tomography (FDG PET), comparing the patient groups with each other and with the controls by statistical parametric mapping. Highly significant relative increases in glucose metabolism distribution were found in the group comprising all 32 ALS patients as compared with the controls in the bilateral amygdalae, midbrain, pons and cerebellum. Relative hypermetabolism was also found in patients with spinal onset as compared with the controls in the right midbrain. In patients with bulbar onset compared with the controls and with patients with spinal onset, large relatively hypometabolic areas were found in the bilateral frontal cortex, right insula, anterior cingulate, precuneus and inferior parietal lobe. Patients with spinal onset had significantly higher scores in a neuropsychological test assessing verbal fluency compared with patients with bulbar onset. This large FDG PET investigation provided unprecedented evidence of relatively increased metabolism in the amygdalae, midbrain and pons in ALS patients as compared with control subjects, possibly due to local activation of astrocytes and microglia. Highly significant relative decreases in metabolism were found in large frontal and parietal regions in the bulbar onset patients as compared with the spinal onset patients and the controls, suggesting a differential metabolic and neuropsychological state between the two conditions. (orig.)

  11. Impaired perception of surface tilt in progressive supranuclear palsy.

    Directory of Open Access Journals (Sweden)

    Marian L Dale

    Full Text Available Progressive supranuclear palsy (PSP is characterized by early postural instability and backward falls. The mechanisms underlying backward postural instability in PSP are not understood. The aim of this study was to test the hypothesis that postural instability in PSP is a result of dysfunction in the perception of postural verticality.We gathered posturography data on 12 subjects with PSP to compare with 12 subjects with idiopathic Parkinson's Disease (PD and 12 healthy subjects. Objective tests of postural impairment included: dynamic sensory perception tests of gravity and of surface oscillations, postural responses to surface perturbations, the sensory organization test of postural sway under altered sensory conditions and limits of stability in stance.Perception of toes up (but not toes down surface tilt was reduced in subjects with PSP compared to both control subjects (p≤0.001 standing, p≤0.007 seated and subjects with PD (p≤0.03 standing, p≤0.04 seated. Subjects with PSP, PD and normal controls accurately perceived the direction of gravity when standing on a tilting surface. Unlike PD and control subjects, subjects with PSP exerted less postural corrective torque in response to toes up surface tilts.Difficulty perceiving backward tilt of the surface or body may account for backward falls and postural impairments in patients with PSP. These observations suggest that abnormal central integration of sensory inputs for perception of body and surface orientation contributes to the pathophysiology of postural instability in PSP.

  12. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy.

    Science.gov (United States)

    Höglinger, Günter U; Melhem, Nadine M; Dickson, Dennis W; Sleiman, Patrick M A; Wang, Li-San; Klei, Lambertus; Rademakers, Rosa; de Silva, Rohan; Litvan, Irene; Riley, David E; van Swieten, John C; Heutink, Peter; Wszolek, Zbigniew K; Uitti, Ryan J; Vandrovcova, Jana; Hurtig, Howard I; Gross, Rachel G; Maetzler, Walter; Goldwurm, Stefano; Tolosa, Eduardo; Borroni, Barbara; Pastor, Pau; Cantwell, Laura B; Han, Mi Ryung; Dillman, Allissa; van der Brug, Marcel P; Gibbs, J Raphael; Cookson, Mark R; Hernandez, Dena G; Singleton, Andrew B; Farrer, Matthew J; Yu, Chang-En; Golbe, Lawrence I; Revesz, Tamas; Hardy, John; Lees, Andrew J; Devlin, Bernie; Hakonarson, Hakon; Müller, Ulrich; Schellenberg, Gerard D

    2011-06-19

    Progressive supranuclear palsy (PSP) is a movement disorder with prominent tau neuropathology. Brain diseases with abnormal tau deposits are called tauopathies, the most common of which is Alzheimer's disease. Environmental causes of tauopathies include repetitive head trauma associated with some sports. To identify common genetic variation contributing to risk for tauopathies, we carried out a genome-wide association study of 1,114 individuals with PSP (cases) and 3,247 controls (stage 1) followed by a second stage in which we genotyped 1,051 cases and 3,560 controls for the stage 1 SNPs that yielded P ≤ 10(-3). We found significant previously unidentified signals (P < 5 × 10(-8)) associated with PSP risk at STX6, EIF2AK3 and MOBP. We confirmed two independent variants in MAPT affecting risk for PSP, one of which influences MAPT brain expression. The genes implicated encode proteins for vesicle-membrane fusion at the Golgi-endosomal interface, for the endoplasmic reticulum unfolded protein response and for a myelin structural component.

  13. Adynamic Graciloplasty With a Pedicled Gracilis Muscle Flap Wrapped Around Bulbar Urethra for Treatment of Male Acquired Urinary Incontinence.

    Science.gov (United States)

    Guo, Hailin; Sa, Yinglong; Xu, Yuemin; Wang, Lin; Fei, Xiaofang

    2016-05-01

    To evaluate the efficacy of adynamic gracilis urethral myoplasty with a pedicled gracilis muscle flap wrapped around bulbar urethra for treatment of male acquired urinary incontinence. Twenty-four patients with acquired urinary incontinence (8 after radical prostatectomy, 7 after transurethral resection of the prostate, and 9 after posterior urethroplasty) were included in our study. Eighteen of these patients (75.0%) had mild to moderate urinary incontinence, and 6 (25.0%) had severe urinary incontinence. All patients received adynamic gracilis urethral myoplasty with a pedicled gracilis muscle flap wrapped around bulbar urethra and had a close follow-up. The mean postoperative maximum urethral pressure after the gracilis muscle wrapped around bulbar urethra was significantly higher than that of the preoperative measurements (P urethra can raise the urethral pressure. Adynamic graciloplasty with a pedicled gracilis muscle flap wrapped around bulbar urethra is a safe and effective surgical option in the treatment of male patients with mild to moderate incontinence, but is not suitable for severe incontinence. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Progressive supranuclear palsy dopamine D2 receptor tomoscintigraphy to detect L-dopamine efficiency. Paralysies supra-nucleaires progressives. Quantification des recepteurs dopaminergiques D2 par tomoscintigraphie

    Energy Technology Data Exchange (ETDEWEB)

    Tranquart, F; Henry Le Bras, F; Toffol, B de; Autret, A; Guilloteau, D; Baulieu, J L [Hopital Bretonneau, 37 - Tours (France)

    1994-09-01

    Progressive supranuclear palsy (PSP) may sometimes be misdiagnosed as Parkinson's disease in its early stages, hence an early positive diagnosis of PSP based on dopamine D2 receptor density could be extremely valuable. In the present case report, the absence of dopamine D2 receptors was clearly demonstrated in the striatum using [sup 123]I-iodobenzamide (IBZM) tomoscintigraphy. This illustrates the potential use of IBZM tomoscintigraphy to identify Parkinson-like's disease presenting with decreased dopamine D2 receptor density; and hence to predict L-Dopa effectiveness. Further studies are needed to evaluate the value of IBZM tomoscintigraphy in the different Parkinson's like diseases. (authors). 11 refs., 2 figs.

  15. Abnormal cortical synaptic plasticity in primary motor area in progressive supranuclear palsy.

    Science.gov (United States)

    Conte, Antonella; Belvisi, Daniele; Bologna, Matteo; Ottaviani, Donatella; Fabbrini, Giovanni; Colosimo, Carlo; Williams, David R; Berardelli, Alfredo

    2012-03-01

    No study has yet investigated whether cortical plasticity in primary motor area (M1) is abnormal in patients with progressive supranuclear palsy (PSP). We studied M1 plasticity in 15 PSP patients and 15 age-matched healthy subjects. We used intermittent theta-burst stimulation (iTBS) to investigate long-term potentiation (LTP) and continuous TBS (cTBS) to investigate long-term depression (LTD)-like cortical plasticity in M1. Ten patients underwent iTBS again 1 year later. We also investigated short-interval intracortical inhibition (SICI) and intracortical facilitation (ICF) in M1 with paired-pulse transcranial magnetic stimulation, tested H reflex from upper limb flexor muscles before and after iTBS, and measured motor evoked potential (MEP) input-output (I/O) curves before and after iTBS. iTBS elicited a significantly larger MEP facilitation after iTBS in patients than in healthy subjects. Whereas in healthy subjects, cTBS inhibited MEP, in patients it significantly facilitated MEPs. In patients, SICI was reduced, whereas ICF was normal. H reflex size remained unchanged after iTBS. Patients had steeper MEP I/O slopes than healthy subjects at baseline and became even more steeper after iTBS only in patients. The iTBS-induced abnormal MEP facilitation in PSP persisted at 1-year follow-up. In conclusion, patients with PSP have abnormal M1 LTP/LTD-like plasticity. The enhanced LTP-like cortical synaptic plasticity parallels disease progression.

  16. Facial palsy in Melkersson-Rosenthal syndrome and Bell's palsy: familial history and recurrence tendency.

    Science.gov (United States)

    Sun, Baochun; Zhou, Chengyong; Han, Zeli

    2015-02-01

    The aim of this study was to compare genetic predilection and recurrence tendency between facial palsy in Melkersson-Rosenthal syndrome (MRS) and Bell's palsy We carried out an investigation on patients with facial palsy in MRS and those with Bell's palsy who visited the outpatient department in our hospital between February 2009 and February 2013. They were asked about familial history and whether it was the first episode, with the results recorded and compared. There were 16 patients with facial palsy in MRS and 860 patients with Bell's palsy involved in the study. Familial history was positive in 5 of 16 patients (31.3%) with facial palsy in MRS and 56 of 860 patients (6.5%) with Bell's palsy (P palsy in MRS and 88 of 860 cases (10.2%) with Bell's palsy had a history of facial palsy in the past (P Bell's palsy, facial palsy in MRS has an obvious genetic predilection and recurrence tendency. © The Author(s) 2014.

  17. Versatility of the ventral approach in bulbar urethroplasty using dorsal, ventral or dorsal plus ventral oral grafts

    OpenAIRE

    Palminteri, Enzo; Berdondini, Elisa; Fusco, Ferdinando; Nunzio, Cosimo De; Giannitsas, Kostas; Shokeir, Ahmed A.

    2012-01-01

    Objectives To investigate the versatility of the ventral urethrotomy approach in bulbar reconstruction with buccal mucosa (BM) grafts placed on the dorsal, ventral or dorsal plus ventral urethral surface. Patients and methods Between 1999 and 2008, 216 patients with bulbar strictures underwent BM graft urethroplasty using the ventral-sagittal urethrotomy approach. Of these patients, 32 (14.8%; mean stricture 3.2?cm, range 1.5?5) had a dorsal graft urethroplasty (DGU), 121 (56%; mean stricture...

  18. Bell's Palsy in Children: Role of the School Nurse in Early Recognition and Referral

    Science.gov (United States)

    Gordon, Shirley C.

    2008-01-01

    Bell's palsy is the most common condition affecting facial nerves. It is an acute, rapidly progressing, idiopathic, unilateral facial paralysis that is generally self-limiting and non-life threatening that occurs in all age groups (Okuwobi, Omole, & Griffith, 2003). The school nurse may be the first person to assess facial palsy and muscle…

  19. United Cerebral Palsy

    Science.gov (United States)

    ... your local affiliate Find your local affiliate United Cerebral Palsy United Cerebral Palsy (UCP) is a trusted resource for individuals with Cerebral Palsy and other disabilities and their networks. Individuals with ...

  20. Cerebral Palsy (For Teens)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Cerebral Palsy KidsHealth / For Teens / Cerebral Palsy What's in this ... do just what everyone else does. What Is Cerebral Palsy? Cerebral palsy (CP) is a disorder of the ...

  1. Bell's Palsy (For Teens)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Bell's Palsy KidsHealth / For Teens / Bell's Palsy What's in this ... Print en español Parálisis de Bell What Is Bell's Palsy? Bell's palsy is a temporary weakness or paralysis ...

  2. Vessel Sampling and Blood Flow Velocity Distribution With Vessel Diameter for Characterizing the Human Bulbar Conjunctival Microvasculature.

    Science.gov (United States)

    Wang, Liang; Yuan, Jin; Jiang, Hong; Yan, Wentao; Cintrón-Colón, Hector R; Perez, Victor L; DeBuc, Delia C; Feuer, William J; Wang, Jianhua

    2016-03-01

    This study determined (1) how many vessels (i.e., the vessel sampling) are needed to reliably characterize the bulbar conjunctival microvasculature and (2) if characteristic information can be obtained from the distribution histogram of the blood flow velocity and vessel diameter. Functional slitlamp biomicroscope was used to image hundreds of venules per subject. The bulbar conjunctiva in five healthy human subjects was imaged on six different locations in the temporal bulbar conjunctiva. The histograms of the diameter and velocity were plotted to examine whether the distribution was normal. Standard errors were calculated from the standard deviation and vessel sample size. The ratio of the standard error of the mean over the population mean was used to determine the sample size cutoff. The velocity was plotted as a function of the vessel diameter to display the distribution of the diameter and velocity. The results showed that the sampling size was approximately 15 vessels, which generated a standard error equivalent to 15% of the population mean from the total vessel population. The distributions of the diameter and velocity were not only unimodal, but also somewhat positively skewed and not normal. The blood flow velocity was related to the vessel diameter (r=0.23, Psampling size of the vessels and the distribution histogram of the blood flow velocity and vessel diameter, which may lead to a better understanding of the human microvascular system of the bulbar conjunctiva.

  3. Microvascular Cranial Nerve Palsy

    Science.gov (United States)

    ... Español Eye Health / Eye Health A-Z Microvascular Cranial Nerve Palsy Sections What Is Microvascular Cranial Nerve Palsy? ... Microvascular Cranial Nerve Palsy Treatment What Is Microvascular Cranial Nerve Palsy? Leer en Español: ¿Qué es una parálisis ...

  4. The prognostic value of concurrent phrenic nerve palsy in newborn babies with neonatal brachial plexus palsy.

    Science.gov (United States)

    Yoshida, Kiyoshi; Kawabata, Hidehiko

    2015-06-01

    To investigate the prognostic value of concurrent phrenic nerve palsy for predicting spontaneous motor recovery in neonatal brachial plexus palsy. We reviewed the records of 366 neonates with brachial plexus palsy. The clinical and follow-up data of patients with and without phrenic nerve palsy were compared. Of 366 newborn babies with neonatal brachial plexus palsy, 21 (6%) had concurrent phrenic nerve palsy. Sixteen of these neonates had upper-type palsy and 5 had total-type palsy. Poor spontaneous motor recovery was observed in 13 neonates with concurrent phrenic nerve palsy (62%) and in 129 without concurrent phrenic nerve palsy (39%). Among neonates born via vertex delivery, poor motor recovery was observed in 7 of 9 (78%) neonates with concurrent phrenic nerve palsy and 115 of 296 (39%) without concurrent phrenic nerve palsy. Concurrent phrenic nerve palsy in neonates with brachial plexus palsy has prognostic value in predicting poor spontaneous motor recovery of the brachial plexus, particularly after vertex delivery. Therapeutic IV. Copyright © 2015 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

  5. Radiation-induced cranial nerve palsy: hypoglossal nerve and vocal cord palsies

    International Nuclear Information System (INIS)

    Takimoto, Toru; Saito, Yasuo; Suzuki, Masayuki; Nishimura, Toshirou

    1991-01-01

    Cranial nerve palsies are an unexpected complication of radiotherapy for head and neck tumours. We present a case of this radiation-induced cranial palsy. An 18-year-old female with nasopharyngeal carcinoma developed a right hypoglossal nerve palsy 42 months after cancericidal doses of radiotherapy. In addition, she developed a bilateral vocal cord palsy 62 months after the therapy. Follow-up over four years has demonstrated no evidence of tumour recurrence and no sign of neurological improvement. (author)

  6. Horizontal gaze palsy and progressive scoliosis in a patient with congenital esotropia and inability to abduct. A case report.

    Science.gov (United States)

    Fernández-Vega Cueto, A; Rodríguez-Ezcurra, J J; Rodríguez-Maiztegui, I

    2016-12-01

    The case is presented on a 4- year-old child with congenital esotropia, limitation of abduction, cross-fixation, and thoracolumbar scoliosis. Genetic testing of ROBO3 gene confirmed the diagnosis of horizontal gaze palsy and scoliosis (HGPSS) DISCUSSION: HGPPS is a rare congenital disorder characterised by absence of conjugate horizontal eye movements and progressive scoliosis developed in childhood and adolescence. We highlight this motility disorder as a part of the differential diagnosis of early childhood esotropia with cross- fixation and limitation of abduction. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  7. Anastomotic fibrous ring as cause of stricture recurrence after bulbar onlay graft urethroplasty.

    Science.gov (United States)

    Barbagli, Guido; Guazzoni, Giorgio; Palminteri, Enzo; Lazzeri, Massimo

    2006-08-01

    We retrospectively reviewed patterns of failure after bulbar substitution urethroplasty. In particular we investigated the prevalence and location of anastomotic fibrous ring strictures occurring at the apical anastomoses between the graft and urethral plate after 3 types of onlay graft techniques. We reviewed the records of 107 patients who underwent bulbar urethroplasty between 1994 and 2004. Mean patient age was 44 years. Patients with lichen sclerosus, failed hypospadias repair or urethroplasty and panurethral strictures were excluded. A total of 45 patients underwent dorsal onlay skin graft urethroplasty, 50 underwent buccal mucosa onlay graft urethroplasty and 12 underwent augmented end-to-end urethroplasty. The clinical outcome was considered a success or failure at the time that any postoperative procedure was needed, including dilation. Mean followup was 74 months (range 12 to 130). Of 107 cases 85 (80%) were successful and 22 (20%) failed. Failure in 12 patients (11%) involved the whole grafted area and in 10 (9%) it involved the anastomotic site, which was distal and proximal in 5 each. Urethrography, urethral ultrasound and urethroscopy were fundamental for determining the difference between full-length and focal extension of re-stricture. Failures were treated with multistage urethroplasty in 12 cases, urethrotomy in 7 and 1-stage urethroplasty in 3. Of the patients 16 had a satisfactory final outcome and 6 underwent definitive perineal urinary diversion. The prevalence and location of anastomotic ring strictures after bulbar urethroplasty were uniformly distributed in after 3 surgical techniques using skin or buccal mucosa. Further studies are necessary to clarify the etiology of these fibrous ring strictures.

  8. Cerebral Palsy

    Science.gov (United States)

    Cerebral palsy is a group of disorders that affect a person's ability to move and to maintain balance ... do not get worse over time. People with cerebral palsy may have difficulty walking. They may also have ...

  9. Bell's Palsy

    Science.gov (United States)

    Bell's palsy is the most common cause of facial paralysis. It usually affects just one side of the ... become inflamed. You are most likely to get Bell's palsy if you are pregnant, diabetic or sick with ...

  10. La belle indifférence revisited: a case report on progressive supranuclear palsy misdiagnosed as conversion disorder

    Directory of Open Access Journals (Sweden)

    van Meerkerk-Aanen PJ

    2017-08-01

    Full Text Available Petra J van Meerkerk-Aanen,1 Lars de Vroege,1,2 David Khasho,1 Aziza Foruz,1 J Thies van Asseldonk,3 Christina M van der Feltz-Cornelis1,2 1Clinical Center of Excellence for Body, Mind, and Health, GGz Breburg, 2Department Tranzo, Tilburg School of Behavioral and Social Sciences, Tilburg University, 3Department of Neurology, Elisabeth Hospital, Tilburg, the Netherlands Background: Since the advent of computed tomography and magnetic resonance imaging scans, neurological disorders have less often been falsely labeled as conversion disorder (CD. However, misdiagnosis of a neurological disorder as CD still occurs, especially in cases with insidious onset. Misinterpretation of la belle indifférence may contribute to such misdiagnosis. Here, we describe a case of progressive supranuclear palsy/Richardson’s syndrome (PSPS misdiagnosed as a case of CD.Case: A 62-year-old woman consulted two different neurologists in 2012 because of falling spells since 2009 and was diagnosed with CD. She was referred to the Clinical Center of Excellence for Body, Mind, and Health for treatment of CD. After neurological examination, blood tests, and psychiatric examination, in which la belle indifférence and a history of incest were found, CD was confirmed. However, despite treatment for CD, the patient’s physical symptoms deteriorated over a year. After repeated physical and psychiatric examinations, neurocognitive assessment, and consultation with a third neurologist because of suspicion of neurological disease, the patient was diagnosed with PSPS.Conclusion: La belle indifférence may be a psychological sign in the context of CD, but it may also be an expression of lack of mimic due to Parkinsonism or of eye movement disorder in the context of neurological illness. A diagnosis of CD should not be considered definitive if no improvement occurs in terms of physical, mental, and cognitive symptoms despite appropriate therapy. In case of deterioration, neurological

  11. Long-term outcomes of external femoral derotation osteotomies in children with cerebral palsy.

    Science.gov (United States)

    Õunpuu, Sylvia; Solomito, Matthew; Bell, Katharine; Pierz, Kristan

    2017-07-01

    External femoral derotation osteotomy (FDO) is an orthopaedic intervention to correct increased femoral anteversion and associated excessive internal hip rotation and internal foot progression during gait in children with cerebral palsy. The resulting functional issues may include clearance problems and hip abductor lever-arm dysfunction. The purpose of this study was to evaluate long-term gait outcomes of FDO. Twenty ambulatory patients (27 sides) with cerebral palsy who underwent pre-operative (P0) and a one year post-operative (P1) gait analysis as part of the standard of care had a second post-operative analysis (P2) approximately 11 years post-surgical intervention. Mean hip rotation in stance showed statistically significant decreases in internal rotation at P1 post-surgical intervention that were maintained long-term (mean hip rotation P0: 21±9, P1: 0±9 and P2: 6±12 degrees internal). Similar results were seen with mean foot progression (P0: 9±16 degrees internal, P1: 14±13 degrees external, P2: 13±16 degrees external). However, 2/27 sides (9%) showed a recurrence of internal hip rotation of >15° at the 11year follow-up. The reasons for this recurrence could include age, surgical location and ongoing disease process all of which need to be further examined. We conclude that FDO can show long-term kinematic and functional benefits when performed in the prepubescent child with cerebral palsy in comparison to the natural progression of of hip rotation in cerebral palsy. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. Primary non-transecting bulbar urethroplasty long-term success rates are similar to transecting urethroplasty.

    Science.gov (United States)

    Anderson, Kirk M; Blakely, Stephen A; O'Donnell, Colin I; Nikolavsky, Dmitriy; Flynn, Brian J

    2017-01-01

    To review the long-term outcomes of transecting versus non-transecting urethroplasty to repair bulbar urethral strictures. A retrospective review was conducted of 342 patients who underwent anterior urethroplasty performed by a single surgeon from 2003 to 2014. Patients were excluded from further analysis if there had been prior urethroplasty, stricture location outside the bulbous urethra, or age urethroplasty. In the non-transecting group, surgical techniques used included non-transecting anastomotic urethroplasty and dorsal and/or ventral buccal grafting. The primary endpoint was stricture resolution in transecting vs. non-transecting bulbar urethroplasty. Success was defined as freedom from secondary procedures including dilation, urethrotomy, or repeat urethroplasty. One hundred and fifty-two patients met inclusion criteria. At a mean follow-up of 65 months (range: 10-138 months), stricture-free recurrence in the transecting and non-transecting groups was similar, 83% (n = 85/102) and 82% (n = 41/50), respectively (p = 0.84). Surgical technique (p = 0.91), stricture length (p = 0.8), and etiology (p = 0.6) did not affect stricture recurrence rate on multivariate analysis. There was no difference detected in time to stricture recurrence (p = 0.21). In this retrospective series, transecting and non-transecting primary bulbar urethroplasty resulted in similar long-term stricture resolution rate. Prospective studies are needed to determine what differences may present in outcomes related to sexual function and long-term success.

  13. Effects of Choto-san (Diao-Teng-San) on microcirculation of bulbar conjunctiva and hemorheological factors in patients with asymptomatic cerebral infarction

    OpenAIRE

    YANG, Qiao; Kita, Toshiaki; Hikiami, Hiroaki; Shimada, Yutaka; Itoh, Takashi; Terasawa, Katsutoshi

    1999-01-01

    In this study, the effects of Choto-san ( 釣藤散 ) on the microcirculation of bulbar conjunctiva in 16 patients with asymptornatic cerebral infarction were investigated with a video-microscopic system. After the administration of Choto-san for four weeks, variables of microcirculatory flow of the bulbar conjunctiva, that is, the internal diameter of vessels, flow velocity and flow volume rate were increased (p

  14. Neck Vibration Proprioceptive Postural Response Intact in Progressive Supranuclear Palsy unlike Idiopathic Parkinson’s Disease

    Directory of Open Access Journals (Sweden)

    Stefan Kammermeier

    2017-12-01

    Full Text Available Progressive supranuclear palsy (PSP and late-stage idiopathic Parkinson’s disease (IPD are neurodegenerative movement disorders resulting in different postural instability and falling symptoms. IPD falls occur usually forward in late stage, whereas PSP falls happen in early stages, mostly backward, unprovoked, and with high morbidity. Postural responses to sensory anteroposterior tilt illusion by bilateral dorsal neck vibration were probed in both groups versus healthy controls on a static recording posture platform. Three distinct anteroposterior body mass excursion peaks (P1–P3 were observed. 18 IPD subjects exhibited well-known excessive response amplitudes, whereas 21 PSP subjects’ responses remained unaltered to 22 control subjects. Neither IPD nor PSP showed response latency deficits, despite brainstem degeneration especially in PSP. The observed response patterns suggest that PSP brainstem pathology might spare the involved proprioceptive pathways and implies viability of neck vibration for possible biofeedback and augmentation therapy in PSP postural instability.

  15. Speech Movement Measures as Markers of Bulbar Disease in Amyotrophic Lateral Sclerosis

    Science.gov (United States)

    Shellikeri, Sanjana; Green, Jordan R.; Kulkarni, Madhura; Rong, Panying; Martino, Rosemary; Zinman, Lorne; Yunusova, Yana

    2016-01-01

    Purpose: The goal of this study was to identify the effects of amyotrophic lateral sclerosis (ALS) on tongue and jaw control, both cross-sectionally and longitudinally. The data were examined in the context of their utility as a diagnostic marker of bulbar disease. Method: Tongue and jaw movements were recorded cross-sectionally (n = 33…

  16. Effect of aerobic training in patients with spinal and bulbar muscular atrophy (Kennedy disease)

    DEFF Research Database (Denmark)

    Preisler, N; Andersen, G; Thøgersen, F

    2009-01-01

    ) or any of the other variables examined before and after training, and the patients with SBMA did not feel improvements in ADL. CONCLUSIONS: Frequent, moderate-intensity aerobic conditioning is of little beneficial effect in patients with spinal and bulbar muscular atrophy (SBMA). High levels of plasma......OBJECTIVE: We examined the effect of aerobic exercise in patients with spinal and bulbar muscular atrophy (SBMA). SBMA is caused by a defect androgen receptor. This defect causes motor neuron death, but considering the important function of androgens in muscle, it is possible that muscle damage...... measurements, lung function, plasma proteins, and hormones were evaluated before and after training. Evaluation of improvements in activities of daily living (ADL) was conducted after training. RESULTS: W(max) increased by 18%, and CS activity increased by 35%. There was no significant change in Vo(2max...

  17. Diffusion tensor analysis of corpus callosum in progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Ito, Shoichi; Makino, Takahiro; Shirai, Wakako; Hattori, Takamichi

    2008-01-01

    Progressive supranuclear palsy (PSP) is a neurodegenerative disease featuring parkinsonism, supranuclear ophthalmoplegia, dysphagia, and frontal lobe dysfunction. The corpus callosum which consists of many commissure fibers probably reflects cerebral cortical function. Several previous reports showed atrophy or diffusion abnormalities of anterior corpus callosum in PSP patients, but partitioning method used in these studies was based on data obtained in nonhuman primates. In this study, we performed a diffusion tensor analysis using a new partitioning method for the human corpus callosum. Seven consecutive patients with PSP were compared with 29 age-matched patients with Parkinson's Disease (PD) and 19 age-matched healthy control subjects. All subjects underwent diffusion tensor magnetic resonance imaging, and the corpus callosum was partitioned into five areas on the mid-sagittal plane according to a recently established topography of human corpus callosum (CC1-prefrontal area, CC2-premotor and supplementary motor area, CC3-motor area, CC4-sensory area, CC5-parietal, temporal, and occipital area). Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were measured in each area and differences between groups were analyzed. In the PSP group, FA values were significantly decreased in CC1 and CC2, and ADC values were significantly increased in CC1 and CC2. Receiver operating characteristic analysis showed excellent reliability of FA and ADC analyses of CC1 for differentiating PSP from PD. The anterior corpus callosum corresponding to the prefrontal, premotor, and supplementary motor cortices is affected in PSP patients. This analysis can be an additional test for further confirmation of the diagnosis of PSP

  18. Diffusion tensor analysis of corpus callosum in progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

    Ito, Shoichi; Makino, Takahiro; Shirai, Wakako; Hattori, Takamichi [Department of Neurology, Graduate School of Medicine, Chiba University (Japan)

    2008-11-15

    Progressive supranuclear palsy (PSP) is a neurodegenerative disease featuring parkinsonism, supranuclear ophthalmoplegia, dysphagia, and frontal lobe dysfunction. The corpus callosum which consists of many commissure fibers probably reflects cerebral cortical function. Several previous reports showed atrophy or diffusion abnormalities of anterior corpus callosum in PSP patients, but partitioning method used in these studies was based on data obtained in nonhuman primates. In this study, we performed a diffusion tensor analysis using a new partitioning method for the human corpus callosum. Seven consecutive patients with PSP were compared with 29 age-matched patients with Parkinson's Disease (PD) and 19 age-matched healthy control subjects. All subjects underwent diffusion tensor magnetic resonance imaging, and the corpus callosum was partitioned into five areas on the mid-sagittal plane according to a recently established topography of human corpus callosum (CC1-prefrontal area, CC2-premotor and supplementary motor area, CC3-motor area, CC4-sensory area, CC5-parietal, temporal, and occipital area). Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were measured in each area and differences between groups were analyzed. In the PSP group, FA values were significantly decreased in CC1 and CC2, and ADC values were significantly increased in CC1 and CC2. Receiver operating characteristic analysis showed excellent reliability of FA and ADC analyses of CC1 for differentiating PSP from PD. The anterior corpus callosum corresponding to the prefrontal, premotor, and supplementary motor cortices is affected in PSP patients. This analysis can be an additional test for further confirmation of the diagnosis of PSP.

  19. Reshaping of bulbar odor response by nasal flow rate in the rat.

    Directory of Open Access Journals (Sweden)

    Emmanuelle Courtiol

    2011-01-01

    Full Text Available The impact of respiratory dynamics on odor response has been poorly studied at the olfactory bulb level. However, it has been shown that sniffing in the behaving rodent is highly dynamic and varies both in frequency and flow rate. Bulbar odor response could vary with these sniffing parameter variations. Consequently, it is necessary to understand how nasal airflow can modify and shape odor response at the olfactory bulb level.To assess this question, we used a double cannulation and simulated nasal airflow protocol on anesthetized rats to uncouple nasal airflow from animal respiration. Both mitral/tufted cell extracellular unit activity and local field potentials (LFPs were recorded. We found that airflow changes in the normal range were sufficient to substantially reorganize the response of the olfactory bulb. In particular, cellular odor-evoked activities, LFP oscillations and spike phase-locking to LFPs were strongly modified by nasal flow rate.Our results indicate the importance of reconsidering the notion of odor coding as odor response at the bulbar level is ceaselessly modified by respiratory dynamics.

  20. Clinical practice guideline: Bell's palsy.

    Science.gov (United States)

    Baugh, Reginald F; Basura, Gregory J; Ishii, Lisa E; Schwartz, Seth R; Drumheller, Caitlin Murray; Burkholder, Rebecca; Deckard, Nathan A; Dawson, Cindy; Driscoll, Colin; Gillespie, M Boyd; Gurgel, Richard K; Halperin, John; Khalid, Ayesha N; Kumar, Kaparaboyna Ashok; Micco, Alan; Munsell, Debra; Rosenbaum, Steven; Vaughan, William

    2013-11-01

    Bell's palsy, named after the Scottish anatomist, Sir Charles Bell, is the most common acute mono-neuropathy, or disorder affecting a single nerve, and is the most common diagnosis associated with facial nerve weakness/paralysis. Bell's palsy is a rapid unilateral facial nerve paresis (weakness) or paralysis (complete loss of movement) of unknown cause. The condition leads to the partial or complete inability to voluntarily move facial muscles on the affected side of the face. Although typically self-limited, the facial paresis/paralysis that occurs in Bell's palsy may cause significant temporary oral incompetence and an inability to close the eyelid, leading to potential eye injury. Additional long-term poor outcomes do occur and can be devastating to the patient. Treatments are generally designed to improve facial function and facilitate recovery. There are myriad treatment options for Bell's palsy, and some controversy exists regarding the effectiveness of several of these options, and there are consequent variations in care. In addition, numerous diagnostic tests available are used in the evaluation of patients with Bell's palsy. Many of these tests are of questionable benefit in Bell's palsy. Furthermore, while patients with Bell's palsy enter the health care system with facial paresis/paralysis as a primary complaint, not all patients with facial paresis/paralysis have Bell's palsy. It is a concern that patients with alternative underlying etiologies may be misdiagnosed or have unnecessary delay in diagnosis. All of these quality concerns provide an important opportunity for improvement in the diagnosis and management of patients with Bell's palsy. The primary purpose of this guideline is to improve the accuracy of diagnosis for Bell's palsy, to improve the quality of care and outcomes for patients with Bell's palsy, and to decrease harmful variations in the evaluation and management of Bell's palsy. This guideline addresses these needs by encouraging

  1. A histopathological study of bulbar conjunctival flaps occurring in 2 contact lens wearers.

    Science.gov (United States)

    Markoulli, Maria; Francis, Ian C; Yong, Jim; Jalbert, Isabelle; Carnt, Nicole; Cole, Nerida; Papas, Eric

    2011-09-01

    To study the histopathology of paralimbal bulbar conjunctival flaps occurring secondary to soft contact lens wear. Slit-lamp biomicroscopy using sodium fluorescein, cobalt blue light, and a Wratten filter was used to observe the presence, location, and dimensions of bulbar conjunctival flaps presenting in a cohort of contact lens wearers. Two subjects who exhibited such flaps agreed to undergo conjunctival biopsy. Tissue samples, obtained from the region of the flap, and an adjacent unaffected area were processed by standard histopathological methods. In the first subject, analysis of the flap tissue showed even collagen distribution and overall normal histology. The flap of the second subject displayed a mild focal increase in collagen and mild degeneration of collagen, but no increase in elastic tissue. Conjunctival epithelium was normal in both cases. In these 2 subjects, conjunctival flap tissue either was normal or showed only minimal abnormality. There is insufficient evidence for significant pathological change on the time scale of this study.

  2. Birth Defects: Cerebral Palsy

    Science.gov (United States)

    ... Loss > Birth defects & other health conditions > Cerebral palsy Cerebral palsy E-mail to a friend Please fill in ... this page It's been added to your dashboard . Cerebral palsy (also called CP) is a group of conditions ...

  3. MIDBRAIN CATECHOLAMINERGIC NEURONS CO-EXPRESS α-SYNUCLEIN AND TAU IN PROGRESSIVE SUPRANUCLEAR PALSY

    Directory of Open Access Journals (Sweden)

    María Elena eErro Aguirre

    2015-03-01

    Full Text Available Objective: To analyze the frequency and distribution of α-synuclein deposits in progressive supranuclear palsy (PSP.Methods: The brains of 25 cases of pathologically confirmed PSP were evaluated with immunohistochemistry for α-synuclein and tau. Multiple immunofluorescent stains were applied to analyze the expression of tau and α-synuclein aggregates in catecholaminergic neurons. Patients’ clinical symptoms were retrospectively recorded. Results: Deposits α-synuclein in the form of typical Lewy bodies (LBs were only found in two PSP cases (8% that fulfilled the clinical subtype of PSP known as Richardson’s syndrome (RS. LBs were present in the locus ceruleus, substantia nigra pars compacta, basal forebrain, amygdala and cingulated cortex in a distribution mimicking that of Parkinson’s disease. Triple-immunolabeling revealed co-expression of α-synuclein and tau proteins in some tyrosine hydroxilase-positive neurons of the locus ceruleus and substantia nigra pars compacta.Conclusions: There is no apparent clinical correlation between the presence of LBs in PSP. Tau protein co-aggregate with α-synuclein in catecholaminergic neurons of PSP brains suggesting a synergistic interaction between the two proteins. This is in keeping with the current view of neurodegenerative disorders as ‘misfolded protein diseases’.

  4. Employees with Cerebral Palsy

    Science.gov (United States)

    ... Resources Home | Accommodation and Compliance Series: Employees with Cerebral Palsy (CP) By Eddie Whidden, MA Preface Introduction Information ... SOAR) at http://AskJAN.org/soar. Information about Cerebral Palsy (CP) What is CP? Cerebral palsy is a ...

  5. Recurrences of Bell's palsy.

    Science.gov (United States)

    Cirpaciu, D; Goanta, C M; Cirpaciu, M D

    2014-01-01

    Bell's palsy in known as the most common cause of facial paralysis, determined by the acute onset of lower motor neuron weakness of the facial nerve with no detectable cause. With a lifetime risk of 1 in 60 and an annual incidence of 11-40/100,000 population, the condition resolves completely in around 71% of the untreated cases. Clinical trials performed for Bell's palsy have reported some recurrences, ipsilateral or contralateral to the side affected in the primary episode of facial palsy. Only few data are found in the literature. Melkersson-Rosenthal is a rare neuromucocutaneous syndrome characterized by recurrent facial paralysis, fissured tongue (lingua plicata), orofacial edema. We attempted to analyze some clinical and epidemiologic aspects of recurrent idiopathic palsy, and to develop relevant correlations between the existing data in literature and those obtained in this study. This is a retrospective study carried out on a 10-years period for adults and a five-year period for children. A number of 185 patients aged between 4 and 70 years old were analyzed. 136 of them were adults and 49 were children. 22 of 185 patients with Bell's palsy (12%) had a recurrent partial or complete facial paralysis with one to six episodes of palsy. From this group of 22 cases, 5 patients were diagnosed with Melkersson-Rosenthal syndrome. The patients' age was between 4 and 70 years old, with a medium age of 27,6 years. In the group studied, fifteen patients, meaning 68%, were women and seven were men. The majority of patients in our group with more than two facial palsy episodes had at least one episode on the contralateral side. Our study found a significant incidence of recurrences of idiopathic facial palsy. Recurrent idiopathic facial palsy and Melkersson-Rosenthal syndrome is diagnosed more often in young females. Recurrence is more likely to occur in the first two years from the onset, which leads to the conclusion that we should have a follow up of patients

  6. Characteristics of sleep dysfunction and sleep - disordered breathing in amyotrophic lateral sclerosis patients

    Directory of Open Access Journals (Sweden)

    Fang WANG

    2017-10-01

    Full Text Available Objective To study the characteristics of sleep architecture and sleep - disordered breathing (SDB in patients with amyotrophic lateral sclerosis (ALS using polysomnography (PSG. Methods A total of 36 patients with ALS were recruited in this study. According to symptoms of medulla oblongata, the patients were divided into limb involvement group (N = 14 and bulbar palsy group (N = 22. Detailed record of the patients was made including general information and chief complaints of sleep dysfunction and SDB, which covered sleep initiation and maintenance disorders, arousals, difficulty in breathing and snoring, nocturnal polyuria, restless legs syndrome (RLS and muscle soreness. Appel Amyotrophic Lateral Sclerosis (AALS Scores were used to assess bulbar function, breathing function,myodynamia and limbs function. PSG was performed to monitor EEG, EOG, EMG, ECG, position, snore, gas flow of mouth and nose, chest breathing, pulse oxygen saturation (SpO2 and sleep-related parameters including total sleep time (TST, sleep efficiency (SE, sleep latency (SL, awakening times, percentage of different non-rapid eye movement (NREM and rapial eye movement (REM, and apnea hypopnea index (AHI. Pearson correlation analysis evaluated the relationship between AHI of REM, periodic limb movements (PLM and clinical information, AALS Scores. Results Bulbar palsy group had higher scores in AALS Scores (P = 0.007, bulbar function (P = 0.000 and breathing function (P = 0.000, and lower score in upper limb myodynamia (P = 0.016 than limb involvement group. Both 2 groups showed disturbed sleep architecture in the performance of sleep fragmentation. Bulbar palsy group had more awakening times (P = 0.027, lower percentage of REM sleep (P = 0.009 and less PLM (P = 0.020 than limb involvement group. The main respiratory event of 2 groups was hypopnea. Bulbar palsy group had higher AHI (P = 0.038 and AHI of REM and NREM (P = 0.031, 0.049 than limb involvement group. Pearson

  7. Peripheral facial palsy in children.

    Science.gov (United States)

    Yılmaz, Unsal; Cubukçu, Duygu; Yılmaz, Tuba Sevim; Akıncı, Gülçin; Ozcan, Muazzez; Güzel, Orkide

    2014-11-01

    The aim of this study is to evaluate the types and clinical characteristics of peripheral facial palsy in children. The hospital charts of children diagnosed with peripheral facial palsy were reviewed retrospectively. A total of 81 children (42 female and 39 male) with a mean age of 9.2 ± 4.3 years were included in the study. Causes of facial palsy were 65 (80.2%) idiopathic (Bell palsy) facial palsy, 9 (11.1%) otitis media/mastoiditis, and tumor, trauma, congenital facial palsy, chickenpox, Melkersson-Rosenthal syndrome, enlarged lymph nodes, and familial Mediterranean fever (each 1; 1.2%). Five (6.1%) patients had recurrent attacks. In patients with Bell palsy, female/male and right/left ratios were 36/29 and 35/30, respectively. Of them, 31 (47.7%) had a history of preceding infection. The overall rate of complete recovery was 98.4%. A wide variety of disorders can present with peripheral facial palsy in children. Therefore, careful investigation and differential diagnosis is essential. © The Author(s) 2013.

  8. History and evolution of dorsal onlay urethroplasty for bulbar urethral stricture repair using skin or buccal mucosal grafts.

    Science.gov (United States)

    Barbagli, G; Lazzeri, M

    2007-01-01

    OBJECTIVES. To illustrate the history and the evolution over time of bulbar dorsal onlay urethroplasty, comparing outcomes when using buccal mucosa or skin grafts. MATERIALS AND METHODS. Ninety-four patients underwent bulbar urethral reconstruction using two dorsal onlay techniques, namely augmented anastomotic urethroplasty and dorsal onlay graft urethroplasty. Preoperative evaluation included clinical history, physical examination, urine culture, residual urine measurement, uroflowmetry and urethrography. Thirty-four patients underwent augmented anastomotic urethroplasty using penile skin (10 cases) or buccal mucosa (24 cases) grafts. Sixty patients underwent dorsal onlay graft urethroplasty using penile skin (38 cases) or buccal mucosa (22 cases) grafts. Forty-eight out of 94 patients received skin grafts and 46 buccal mucosal grafts. RESULTS. Sixty-four (68%) out of 94 cases were successful, whereas 30 (32%) failed. The 34 augmented anastomotic urethroplasties provided successful outcomes in 24 cases (70.6%), but poor outcomes in 10 (29.4%) cases. The 60 dorsal onlay graft urethroplasty proved to be successful in 42 cases (70%), failing in 18 (30%) cases. Twenty-eight (58.3%) out of 48 penile skin grafts were successful and 20 (41.7%) failed. Thirty-six (78.3%) out of 46 buccal mucosa grafts were successful and 10 (21.7%) failed. The 30 failed cases were then treated with internal urethrotomy in 14 cases (46.7%), perineal urethrostomy in 8 cases (26.7%), two-stage repair in 4 cases (13.3%), and one-stage repair in 4 cases (13.3%). CONCLUSIONS. The dorsal onlay technique used for bulbar urethral stricture repair has changed over time. In our experience, the buccal mucosa seems to be the best substitute graft material for bulbar urethroplasty using dorsal approach.

  9. Outcomes of Direct Vision Internal Urethrotomy for Bulbar Urethral Strictures: Technique Modification with High Dose Triamcinolone Injection

    Directory of Open Access Journals (Sweden)

    Rishi Modh

    2015-01-01

    Full Text Available Objective. To evaluate the recurrence rate of bulbar urethral strictures managed with cold knife direct vision internal urethrotomy and high dose corticosteroid injection. Methods. 28 patients with bulbar urethral strictures underwent direct vision internal urethrotomy with high dose triamcinolone injection into the periurethral tissue and were followed up for recurrence. Results. Our cohort had a mean age of 60 years and average stricture length of 1.85 cm, and 71% underwent multiple previous urethral stricture procedures with an average of 5.7 procedures each. Our technique modification of high dose corticosteroid injection had a recurrence rate of 29% at a mean follow-up of 20 months with a low rate of urinary tract infections. In patients who failed treatment, mean time to stricture recurrence was 7 months. Patients who were successfully treated had significantly better International Prostate Symptom Scores at 6, 9, and 12 months. There was no significant difference in maximum flow velocity on Uroflowmetry at last follow-up but there was significant difference in length of follow-up (p=0.02. Conclusions. High dose corticosteroid injection at the time of direct vision internal urethrotomy is a safe and effective procedure to delay anatomical and symptomatic recurrence of bulbar urethral strictures, particularly in those who are poor candidates for urethroplasty.

  10. Outcomes of Direct Vision Internal Urethrotomy for Bulbar Urethral Strictures: Technique Modification with High Dose Triamcinolone Injection.

    Science.gov (United States)

    Modh, Rishi; Cai, Peter Y; Sheffield, Alyssa; Yeung, Lawrence L

    2015-01-01

    Objective. To evaluate the recurrence rate of bulbar urethral strictures managed with cold knife direct vision internal urethrotomy and high dose corticosteroid injection. Methods. 28 patients with bulbar urethral strictures underwent direct vision internal urethrotomy with high dose triamcinolone injection into the periurethral tissue and were followed up for recurrence. Results. Our cohort had a mean age of 60 years and average stricture length of 1.85 cm, and 71% underwent multiple previous urethral stricture procedures with an average of 5.7 procedures each. Our technique modification of high dose corticosteroid injection had a recurrence rate of 29% at a mean follow-up of 20 months with a low rate of urinary tract infections. In patients who failed treatment, mean time to stricture recurrence was 7 months. Patients who were successfully treated had significantly better International Prostate Symptom Scores at 6, 9, and 12 months. There was no significant difference in maximum flow velocity on Uroflowmetry at last follow-up but there was significant difference in length of follow-up (p = 0.02). Conclusions. High dose corticosteroid injection at the time of direct vision internal urethrotomy is a safe and effective procedure to delay anatomical and symptomatic recurrence of bulbar urethral strictures, particularly in those who are poor candidates for urethroplasty.

  11. [Etiologies of cerebral palsy and classical treatment possibilities].

    Science.gov (United States)

    Maurer, Ute

    2002-01-01

    Cerebral palsy is a non-progressive disorder of the developing brain with different etiologies in the pre-, peri- or postnatal period. The most important of these diseases is cystic periventricular leukomalacia (PVL), followed by intra- and periventricular hemorrhage, hypoxic-ischemic encephalopathy, vascular disorders, infections or brain malformations. The underlying cause is always a damage of the first motor neuron. Prevalence of cerebral palsy in Europe is 2-3 per 1000 live births with a broad spectrum in different birth weight groups. Our own data concerning only pre-term infants in the NICU with birth weight below 1500 g (VLBW) are between 10%-20%. Established classical treatment methods include physiotherapy (Bobath, Vojta, Hippotherapy), methods of speech and occupational therapists (Castillo-Morales, Sensory Integration) and other therapeutical concepts (Petö, Affolter, Frostig).

  12. The Efficacy of Bulbar Urethral Mobilization for Anastomotic Anterior Urethroplasty in a Case With Recurrent Anterior Urethral Stricture.

    Science.gov (United States)

    Fukui, Shinji; Aoki, Katsuya; Kaneko, Yoshiteru; Samma, Shoji; Fujimoto, Kiyohide

    2014-05-01

    A 2-month-old boy was diagnosed with febrile urinary tract infection. Voiding cystourethrography showed bulbar and anterior urethral strictures, and endoscopic internal urethrotomy was performed. He developed febrile urinary tract infection again and revealed the recurrence of the anterior urethral stricture. Consequently, endoscopic internal urethrotomy was performed 4 times. Because the anterior urethral stricture had not improved, he was referred to us. Anterior urethroplasty was performed when he was 5 years. After excision of the scarred portions of the urethra, the defect of the urethra was 20 mm. Transperineal bulbar urethral mobilization was performed, and a single-stage end-to-end anterior urethroplasty without tension could be performed simultaneously.

  13. Genetic linkage of autosomal dominant progressive supranuclear palsy to 1q31.1.

    Science.gov (United States)

    Ros, Raquel; Gómez Garre, Pilar; Hirano, Michio; Tai, Yen F; Ampuero, Israel; Vidal, Lídice; Rojo, Ana; Fontan, Aurora; Vazquez, Ana; Fanjul, Samira; Hernandez, Jaime; Cantarero, Susana; Hoenicka, Janet; Jones, Alison; Ahsan, R Laila; Pavese, Nicola; Piccini, Paola; Brooks, David J; Perez-Tur, Jordi; Nyggard, Torbjorn; de Yébenes, Justo G

    2005-05-01

    Progressive supranuclear palsy (PSP) is a disorder of unknown pathogenesis. Familial clusters of PSP have been reported related to mutations of protein tau. We report the linkage of a large Spanish family with typical autosomal dominant PSP to a new locus in chromosome 1. Four members of this family had typical PSP, confirmed by neuropathology in one case. At least five ancestors had similar disease. Other members of the family have incomplete phenotypes. The power of the linkage analysis was increased by detecting presymptomatic individuals with 18F-fluoro-dopa and 18F-deoxyglucose positron emission tomography. We screened the human genome with 340 polymorphic markers and we enriched the areas of interest with additional markers. The disease status was defined according to the clinical and positron emission tomography data. We excluded linkage to the tau gene in chromosome 17. PSP was linked, in this family, to one area of 3.4 cM in chromosome 1q31.1, with a maximal multipoint < OD score of +3.53. This area contains at least three genes, whose relevance in PSP is unknown. We expect to further define the gene responsible for PSP, which could help to understand the pathogenesis of this disease and to design effective treatment.

  14. Conjugate Gaze Palsies

    Science.gov (United States)

    ... version Home Brain, Spinal Cord, and Nerve Disorders Cranial Nerve Disorders Conjugate Gaze Palsies Horizontal gaze palsy Vertical ... Version. DOCTORS: Click here for the Professional Version Cranial Nerve Disorders Overview of the Cranial Nerves Internuclear Ophthalmoplegia ...

  15. Prevalence of Cerebral Palsy in Children (Under Five) in and ...

    African Journals Online (AJOL)

    Cerebral palsy (CP) is a non-progressive disorder of posture and movement due to brain damage/insult/lesion before birth, during delivery or in the perinatal period. It is a neurological disorder of childhood with significant medico-social implications. A retrospective hospital based cross sectional study was conducted to ...

  16. Cerebral blood flow SPECT may be helpful in establishing the diagnosis of progressive supranuclear palsy and corticobasal degeneration

    International Nuclear Information System (INIS)

    Slawek, J.; Lass, P.; Derejko, M.; Dubaniewicz, M.

    2001-01-01

    We present 4 cases, which illustrate the usefulness of neuroimaging studies in atypical forms of Parkinsonism. Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD) are rare neurodegenerative progressive disorders of the central nervous system of unknown cause. The clinical accuracy in this diagnosis is not very high even in centres specialising in movement disorders. Functional imaging can be helpful in diagnosing PSP and CBD. We present the results of cerebral blood flow (CBF) SPECT scanning in 2 patients with PSP and 2 patients with CBD. This was performed using a triple-head gammacamera and 99m Tc-HMPAO. In PSP patients a diffuse frontal perfusion deficit was seen, eventually with striatal and occipital hypoperfusion. CT/MRI was either normal or showed a diffuse cortical-subcortical atrophy. In CBD patients left fronto-parieto-temporal cortex and a striatal hypoperfusion were shown. CT scanning was normal in one case and showed an asymmetrical temporo-parietal atrophy in second one. The pattern of diffuse frontal perfusions deficit in PSP and asymmetrical, contralateral to symptoms of CBD, cortico-subcortical hypoperfusion may be helpful in establishing the correct diagnosis. (author)

  17. Pediatric Bulbar and Posterior Urethral Injuries: Operative Outcomes and Long-Term Follow-Up.

    Science.gov (United States)

    Trachta, Jan; Moravek, Jiri; Kriz, Jan; Padr, Radek; Skaba, Richard

    2016-02-01

    The aim of this study was to analyze complications and outcomes of end-to-end urethral anastomosis performed for posttraumatic bulbar strictures or posterior urethral injuries in pediatric patients. The records of 15 boys, age 18 years and below, admitted to our tertiary trauma center with urethral injuries from 1989 to 2014 were reviewed retrospectively. Out of these 15 boys, 7 were excluded (2 for iatrogenic trauma, 2 for minor straddle injuries who were not operated on, 2 for incomplete records, and 1 lost to follow-up) and 8 analyzed patients were operated for bulbar or posterior urethral injury. The mean follow-up after the operation was 4.5 years (range 0.5-10). To obtain up-to-date follow-up information, all the analyzed patients were contacted by a letter and telephone in January 2015 and asked about lower urinary tract or erectile dysfunction (ED) using the International Index of Erectile Function-5 questionnaire. Mean age at the time of injury was 12.3 years (range 5-17). Four patients with pelvic fracture had complete posterior urethra disruption, three patients after straddle injury developed obliterating stricture of the bulbar urethra and one patient had torn his bulbar urethra apart by a sharp hook. Except for the immediate exploration of the open perineal wound, all patients were operated via perineal approach 1 to 6 months after initial suprapubic catheter insertion. Five patients needed a cystotomy to identify the proximal urethral stump by a probe, and two patients had partial pubectomy to gain urethral length. Postoperative complications included stricture in anastomosis in six patients (all reoperated, four more than once including attempts of endoscopic internal urethrotomy). Six days after surgery, one patient developed massive external bleeding around a permanent urinary catheter due to a posttraumatic ruptured arterial aneurysm that was later stopped by urgent angiography and coil insertion. After discharge, three patients had transient

  18. Neutrophil-to-lymphocyte ratio as a novel-potential marker for predicting prognosis of Bell palsy.

    Science.gov (United States)

    Bucak, Abdulkadir; Ulu, Sahin; Oruc, Serdar; Yucedag, Fatih; Tekin, Mustafa Said; Karakaya, Fatıma; Aycicek, Abdullah

    2014-07-01

    Bell palsy can be defined as an idiopathic, acute, facial nerve palsy. Although the pathogenesis of Bell palsy is not fully understood, inflammation seems to play important role. Neutrophil-to-lymphocyte (NLR) ratio was defined as a novel potential marker to determine inflammation and it is routinely measured in peripheral blood. Our goal was to investigate the relationship between Bell palsy and inflammation by using NLR. Retrospective study. The 54 patients who were followed up for Bell palsy for a period of 1 to 3 years, along with 45 age- and sex-matched controls, were included in the study. An automated blood cell counter was used for NLR measurements. All patients were treated with prednisone, 1 mg/kg per day with a progressive dose reduction. Patients were classified according to the House-Brackmann grading system at posttreatment period. Those with House-Brackmann grade I and grade II were regarded as satisfactory recovery; and those with House-Brackmann grade III to grade VI were regarded as nonsatisfactory recovery. The mean NLR and neutrophil values in patients with Bell palsy were significantly higher than in the control group (P=0.001 and PBell palsy and its prognosis. Our result suggest that while evaluating Bell palsy patients, NLR might be taken into account as a novel potential marker to predict the patients' prognosis. 3b. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  19. A rare cause of facial nerve palsy in children : Hyperostosis corticalis generalisata (Van Buchem disease). Three new pediatric cases and a literature review

    NARCIS (Netherlands)

    van Egmond, M. E.; Dikkers, F. G.; Boot, A. M.; van Lierop, A. H. J. M.; Papapoulos, S. E.; Brouwer, O. F.

    2012-01-01

    Differential diagnosis of facial nerve palsy in children is extensive. We report on three pediatric cases presenting with facial nerve palsy caused by hyperostosis corticalis generalisata (Van Buchem disease). This autosomal recessive disease is characterized by progressive bone overgrowth, with

  20. Nanomedicine in cerebral palsy

    Science.gov (United States)

    Balakrishnan, Bindu; Nance, Elizabeth; Johnston, Michael V; Kannan, Rangaramanujam; Kannan, Sujatha

    2013-01-01

    Cerebral palsy is a chronic childhood disorder that can have diverse etiologies. Injury to the developing brain that occurs either in utero or soon after birth can result in the motor, sensory, and cognitive deficits seen in cerebral palsy. Although the etiologies for cerebral palsy are variable, neuroinflammation plays a key role in the pathophysiology of the brain injury irrespective of the etiology. Currently, there is no effective cure for cerebral palsy. Nanomedicine offers a new frontier in the development of therapies for prevention and treatment of brain injury resulting in cerebral palsy. Nanomaterials such as dendrimers provide opportunities for the targeted delivery of multiple drugs that can mitigate several pathways involved in injury and can be delivered specifically to the cells that are responsible for neuroinflammation and injury. These materials also offer the opportunity to deliver agents that would promote repair and regeneration in the brain, resulting not only in attenuation of injury, but also enabling normal growth. In this review, the current advances in nanotechnology for treatment of brain injury are discussed with specific relevance to cerebral palsy. Future directions that would facilitate clinical translation in neonates and children are also addressed. PMID:24204146

  1. The Efficacy of Bulbar Urethral Mobilization for Anastomotic Anterior Urethroplasty in a Case With Recurrent Anterior Urethral Stricture

    Directory of Open Access Journals (Sweden)

    Shinji Fukui

    2014-05-01

    Full Text Available A 2-month-old boy was diagnosed with febrile urinary tract infection. Voiding cystourethrography showed bulbar and anterior urethral strictures, and endoscopic internal urethrotomy was performed. He developed febrile urinary tract infection again and revealed the recurrence of the anterior urethral stricture. Consequently, endoscopic internal urethrotomy was performed 4 times. Because the anterior urethral stricture had not improved, he was referred to us. Anterior urethroplasty was performed when he was 5 years. After excision of the scarred portions of the urethra, the defect of the urethra was 20 mm. Transperineal bulbar urethral mobilization was performed, and a single-stage end-to-end anterior urethroplasty without tension could be performed simultaneously.

  2. [Bell's palsy].

    Science.gov (United States)

    Prud'hon, S; Kubis, N

    2018-03-30

    Idiopathic peripheral facial palsy, also named Bell's palsy, is the most common cause of peripheral facial palsy in adults. Although it is considered as a benign condition, its social and psychological impact can be dramatic, especially in the case of incomplete recovery. The main pathophysiological hypothesis is the reactivation of HSV 1 virus in the geniculate ganglia, leading to nerve edema and its compression through the petrosal bone. Patients experience an acute (less than 24 hours) motor deficit involving ipsilateral muscles of the upper and lower face and reaching its peak within the first three days. Frequently, symptoms are preceded or accompanied by retro-auricular pain and/or ipsilateral face numbness. Diagnosis is usually clinical but one should look for negative signs to eliminate central facial palsy or peripheral facial palsy secondary to infectious, neoplastic or autoimmune diseases. About 75% of the patients will experience spontaneous full recovery, this rate can be improved with oral corticotherapy when introduced within the first 72 hours. To date, no benefit has been demonstrated by adding an antiviral treatment. Hemifacial spasms (involuntary muscles contractions of the hemiface) or syncinesia (involuntary muscles contractions elicited by voluntary ones, due to aberrant reinnervation) may complicate the disease's course. Electroneuromyography can be useful at different stages: it can first reveal the early conduction bloc, then estimate the axonal loss, then bring evidence of the reinnervation process and, lastly, help for the diagnosis of complications. Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  3. Versatility of the ventral approach in bulbar urethroplasty using dorsal, ventral or dorsal plus ventral oral grafts.

    Science.gov (United States)

    Palminteri, Enzo; Berdondini, Elisa; Fusco, Ferdinando; De Nunzio, Cosimo; Giannitsas, Kostas; Shokeir, Ahmed A

    2012-06-01

    To investigate the versatility of the ventral urethrotomy approach in bulbar reconstruction with buccal mucosa (BM) grafts placed on the dorsal, ventral or dorsal plus ventral urethral surface. Between 1999 and 2008, 216 patients with bulbar strictures underwent BM graft urethroplasty using the ventral-sagittal urethrotomy approach. Of these patients, 32 (14.8%; mean stricture 3.2 cm, range 1.5-5) had a dorsal graft urethroplasty (DGU), 121 (56%; mean stricture 3.7, range 1.5-8) a ventral graft urethroplasty (VGU), and 63 (29.2%; mean stricture 3.4, range 1.5-10) a dorsal plus ventral graft urethroplasty (DVGU). The strictured urethra was opened by a ventral-sagittal urethrotomy and BM graft was inserted dorsally or ventrally or dorsal plus ventral to augment the urethral plate. The median follow-up was 37 months. The overall 5-year actuarial success rate was 91.4%. The 5-year actuarial success rates were 87.8%, 95.5% and 86.3% for the DGU, VGU and DVGU, respectively. There were no statistically significant differences among the three groups. Success rates decreased significantly only with a stricture length of >4 cm. In BM graft bulbar urethroplasties the ventral urethrotomy access is simple and versatile, allowing an intraoperative choice of dorsal, ventral or combined dorsal and ventral grafting, with comparable success rates.

  4. Comparison of cold-knife optical internal urethrotomy and holmium:YAG laser internal urethrotomy in bulbar urethral strictures

    Science.gov (United States)

    Yenice, Mustafa Gurkan; Sam, Emre; Colakoglu, Yunus; Atar, Feyzi Arda; Sahin, Selcuk; Simsek, Abdulmuttalip; Tugcu, Volkan

    2017-01-01

    Introduction To compare the results of cold-knife optical internal urethrotomy (OIU) and Holmium:YAG laser internal urethrotomy (HIU) in primary bulbar urethral strictures. Material and methods A total of 63 patients diagnosed with primary bulbar urethral stricture between August 2014 and September 2015 were assigned to the OIU (n = 29) and HIU (n = 34) groups. The demographic variables, biochemistry panels, and preoperative and postoperative uroflowmetry results including the maximum flow rate (Qmax) and mean flow rate (Qmean) values, retrograde urethrography, and diagnostic flexible urethroscopy findings were recorded prospectively. Demographic features and preoperative values were not statistically different between groups (p >0.05). Mean surgical times were 18.4 ±2.3 min for OIU and 21.9 ±3.8 min for HIU groups, which was statistically significant (p 0.05). There was no recurrence in the first 3 months in either group. The urethral stricture recurrence rate up to month 12 was not statistically significant for the OIU group (n = 6, 20.7%) as compared to the HIU group (n = 11, 32.4%; p = 0.299). At follow-up, the SFR and IFR was 96% and 88% at 3-months, and 82% and 71% at 12-months, respectively (p method to OIU, and it has similar success rates in the treatment of short segment bulbar urethral strictures. PMID:29732217

  5. Treatment for long bulbar urethral strictures with membranous involvement using urethroplasty with oral mucosa graft.

    Science.gov (United States)

    Gimbernat, H; Arance, I; Redondo, C; Meilán, E; Andrés, G; Angulo, J C

    2014-10-01

    Urethroplasty with oral mucosa grafting is the most popular technique for treating nontraumatic bulbar urethral strictures; however, cases involving the membranous portion are usually treated using progressive perineal anastomotic urethroplasty. We assessed the feasibility of performing dorsal (or ventral) graft urethroplasty on bulbar urethral strictures with mainly membranous involvement using a modified Barbagli technique. This was a prospective study of 14 patients with bulbomembranous urethral strictures who underwent dilation urethroplasty with oral mucosa graft between 2005 and 2013, performed using a modified technique Barbagli, with proximal anchoring of the graft and securing of the graft to the tunica cavernosa in 12 cases (85.7%) and ventrally in 2 (14.3%). The minimum follow-up time was 1 year. We evaluated the subjective (patient satisfaction) and objective (maximum flow [Qmax] and postvoid residual volume [PVRV], preoperative and postoperative) results and complications. Failure was defined as the need for any postoperative instrumentation. A total of 14 patients (median age, 64+13 years) underwent surgery. The main antecedent of note was transurethral resection of the prostate in 9 cases (64.3%). The median length of the stenosis was 45+26.5mm. Prior to surgery, 50% of the patients had been subjected to dilatations and 4% to endoscopic urethrotomy. The mean surgical time and hospital stay were was 177+76min and 1.5+1 day, respectively. The preoperative Qmax and PVRV values were 4.5+4.45mL/sec and 212.5+130 cc, respectively. The postoperative values were 15.15+7.2mL/sec and 6+21.5cc, respectively (Purethroplasty with free oral mucosa grafts represents a viable alternative for patients with nontraumatic etiology and little fibrosis. The dilation of the urethral lumen achieves good results with minimum failure rates and little probability of complications. For many of these patients, the length of the stricture is too long to perform the tension

  6. Abnormal Resting-State Functional Connectivity in Progressive Supranuclear Palsy and Corticobasal Syndrome

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    Komal Bharti

    2017-06-01

    Full Text Available BackgroundPathological and MRI-based evidence suggests that multiple brain structures are likely to be involved in functional disconnection between brain areas. Few studies have investigated resting-state functional connectivity (rsFC in progressive supranuclear palsy (PSP and corticobasal syndrome (CBS. In this study, we investigated within- and between-network rsFC abnormalities in these two conditions.MethodsTwenty patients with PSP, 11 patients with CBS, and 16 healthy subjects (HS underwent a resting-state fMRI study. Resting-state networks (RSNs were extracted to evaluate within- and between-network rsFC using the Melodic and FSLNets software packages.ResultsIncreased within-network rsFC was observed in both PSP and CBS patients, with a larger number of RSNs being involved in CBS. Within-network cerebellar rsFC positively correlated with mini-mental state examination scores in patients with PSP. Compared to healthy volunteers, PSP and CBS patients exhibit reduced functional connectivity between the lateral visual and auditory RSNs, with PSP patients additionally showing lower functional connectivity between the cerebellar and insular RSNs. Moreover, rsFC between the salience and executive-control RSNs was increased in patients with CBS compared to HS.ConclusionThis study provides evidence of functional brain reorganization in both PSP and CBS. Increased within-network rsFC could represent a higher degree of synchronization in damaged brain areas, while between-network rsFC abnormalities may mainly reflect degeneration of long-range white matter fibers.

  7. Nelson′s syndrome presenting as bilateral oculomotor palsy

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    Abhay Gundgurthi

    2013-01-01

    Full Text Available Nelson′s syndrome refers to a clinical spectrum arising from progressive enlargement of pituitary adenoma and elevated adrenocorticotrophic hormone after total bilateral adrenalectomy for Cushing′s disease comprising of hyperpigmentation, visual field defects which can be life threatening. We report here a 50-year male presenting with rapid onset of Nelson′s syndrome with an unusual finding of bilateral oculomotor palsy mistakenly treated as ocular myasthenia.

  8. A follow-up study on brainstem atrophy in progressive supranuclear palsy. When does brain MRI contribute to the differential diagnosis between progressive supranuclear palsy and Parkinson disease?

    International Nuclear Information System (INIS)

    Yamamoto, Toshiyuki; Oya, Yasushi; Ogawa, Masafumi; Ogata, Katsuhisa; Kawai, Mitsuru

    2003-01-01

    The objective of this study was to investigate when the MRI can discriminate progressive supranuclear palsy (PSP) from Parkinson disease (PD). We obtained the following parameters using T1-weighted axial images of the midbrain and the middle pons from 40 studies of 17 PSP patients and 26 studies of 26 PD patients; anteroposterior diameter of the pons (Pons AP), anteroposterior length of the pontine tegmentum (Pons T), anteroposterior diameter of the midbrain (Midbrain AP), ratio of anteroposterior diameter of the pontine base to that of the pontine tegmentum (Pons B/T ratio), ratio of the sum of the bilateral crus cerebri widths to the midbrain AP (Midbrain [Cr+Cl]/AP ratio). All the PSP patients were studied more than twice and each study was classified into four groups according to the duration of the illness; less than 24 months (7 studies), from 24 to 47 months (10 studies), from 48 to 71 months (14 studies) and 72 months or longer (9 studies). The first MRI studies (duration of disease 39.9±22.1 months) were compared with the second studies (duration of disease 67.6±31.6 months) in 17 PSP patients. Pons AP, Pons T, and Midbrain AP were significantly smaller in the second studies, indicating progressive pontine and midbrain atrophy. Compared with the PD group, the PSP group showed significant atrophy four years after onset of the disease in Pons AP and two years in Pons T. Pons B/T ratio, and Midbrain [Cr+Cl]/AP ratio were significantly smaller in the PSP group two years after onset, suggesting midbrain and pontine tegmentum atrophy. The diagnostic MRI criteria of Pons B/T ratio more than four and Midbrain [Cr+Cl]/AP ratio more than two each showed accuracy in PSP of more than 70% two years after onset. Discrimination of PSP from PD was difficult during the first two years after onset. The atrophic process in the midbrain and the pontine tegmentum may precede that of the pontine base. Pons B/T ratio and Midbrain [Cr+Cl]/AP ratio presented here are potentially

  9. Pathogenesis of cerebral palsy through the prism of immune regulation of nervous tissue homeostasis: literature review.

    Science.gov (United States)

    Lisovska, Natalya; Daribayev, Zholtay; Lisovskyy, Yevgeny; Kussainova, Kenzhe; Austin, Lana; Bulekbayeva, Sholpan

    2016-11-01

    The cerebral palsy is highly actual issue of pediatrics, causing significant neurological disability. Though the great progress in the neuroscience has been recently achieved, the pathogenesis of cerebral palsy is still poorly understood. In this work, we reviewed available experimental and clinical data concerning the role of immune cells in pathogenesis of cerebral palsy. Maintaining of homeostasis in nervous tissue and its transformation in case of periventricular leukomalacia were analyzed. The reviewed data demonstrate involvement of immune regulatory cells in the formation of nervous tissue imbalance and chronicity of inborn brain damage. The supported opinion, that periventricular leukomalacia is not a static phenomenon, but developing process, encourages our optimism about the possibility of its correction. The further studies of changes of the nervous and immune systems in cerebral palsy are needed to create fundamentally new directions of the specific therapy and individual schemes of rehabilitation.

  10. Therapeutic interventions in cerebral palsy.

    Science.gov (United States)

    Patel, Dilip R

    2005-11-01

    Various therapeutic interventions have been used in the management of children with cerebral palsy. Traditional physiotherapy and occupational therapy are widely used interventions and have been shown to be of benefit in the treatment of cerebral palsy. Evidence in support of the effectiveness of the neurodevelopmental treatment is equivocal at best. There is evidence to support the use and effectiveness of neuromuscular electrical stimulation in children with cerebral palsy. The effectiveness of many other interventions used in the treatment of cerebral palsy has not been clearly established based on well-controlled trials. These include: sensory integration, body-weight support treadmill training, conductive education, constraint-induced therapy, hyperbaric oxygen therapy, and the Vojta method. This article provides an overview of salient aspects of popular interventions used in the management of children with cerebral palsy.

  11. Facial nerve palsy due to birth trauma

    Science.gov (United States)

    Seventh cranial nerve palsy due to birth trauma; Facial palsy - birth trauma; Facial palsy - neonate; Facial palsy - infant ... An infant's facial nerve is also called the seventh cranial nerve. It can be damaged just before or at the time of delivery. ...

  12. Nanomedicine in cerebral palsy

    Directory of Open Access Journals (Sweden)

    Balakrishnan B

    2013-11-01

    Full Text Available Bindu Balakrishnan,1 Elizabeth Nance,1 Michael V Johnston,2 Rangaramanujam Kannan,3 Sujatha Kannan1 1Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University; Baltimore, MD, USA; 2Department of Neurology and Pediatrics, Kennedy Krieger Institute, Baltimore, MD, USA; 3Department of Ophthalmology, Center for Nanomedicine, Johns Hopkins University, Baltimore, MD, USA Abstract: Cerebral palsy is a chronic childhood disorder that can have diverse etiologies. Injury to the developing brain that occurs either in utero or soon after birth can result in the motor, sensory, and cognitive deficits seen in cerebral palsy. Although the etiologies for cerebral palsy are variable, neuroinflammation plays a key role in the pathophysiology of the brain injury irrespective of the etiology. Currently, there is no effective cure for cerebral palsy. Nanomedicine offers a new frontier in the development of therapies for prevention and treatment of brain injury resulting in cerebral palsy. Nanomaterials such as dendrimers provide opportunities for the targeted delivery of multiple drugs that can mitigate several pathways involved in injury and can be delivered specifically to the cells that are responsible for neuroinflammation and injury. These materials also offer the opportunity to deliver agents that would promote repair and regeneration in the brain, resulting not only in attenuation of injury, but also enabling normal growth. In this review, the current advances in nanotechnology for treatment of brain injury are discussed with specific relevance to cerebral palsy. Future directions that would facilitate clinical translation in neonates and children are also addressed. Keywords: dendrimer, cerebral palsy, neuroinflammation, nanoparticle, neonatal brain injury, G4OH-PAMAM

  13. Pedicled rectus abdominis muscle and fascia flap sling the bulbar urethra for treatment for male-acquired urinary incontinence: report of ten cases.

    Science.gov (United States)

    Xu, Yue-Min; Zhang, Xin-Ru; Xie, Hong; Song, Lu-Jie; Feng, Chao; Fei, Xiao-Fang

    2014-03-01

    Male urinary incontinence is relatively common complication of radical prostatectomy and of posterior urethroplasty following traumatic pelvic fracture. Here, we investigate the use of pedicled rectus abdominis muscle and fascia flap sling of the bulbar urethra for treatment for male-acquired urinary incontinence. Ten patients with acquired urinary incontinence were included in the study. Urinary incontinence was secondary to TURP in three patients and was secondary to posterior urethroplasty performed following traumatic pelvic fracture in seven patients. Pedicled rectus abdominalis muscle and fascial flaps, approximately 2.5 cm wide and 15 cm long, were isolated. The flaps were inserted into a perineal incision through a subcutaneous tunnel. The free end of the flap was sectioned to form two muscle strips, each 3 cm in length, and inserted into the space between bulbar urethra and corpus cavernosa. After adequate sling tension had been achieved, the two strips of muscle were anastomosed around the bulbar urethra using a 2-zero polyglactin suture. The patients were followed up for between 12 and 82 months (mean 42.8 months). Complete continence was achieved with good voiding in seven of the 10 patients. In other three patients achieved good voiding following catheter removal, but incontinence was only moderately improved. A pedicled rectus muscle fascial sling of the bulbar urethra is an effective and safe treatment for male patients with mild to moderate acquired urinary incontinence, but it may not be suitable for severe incontinence or for patients with weak rectus abdominalis muscles.

  14. The diagnostic yield of neuroimaging in sixth nerve palsy - Sankara Nethralaya Abducens Palsy Study (SNAPS: Report 1

    Directory of Open Access Journals (Sweden)

    Akshay Gopinathan Nair

    2014-01-01

    Full Text Available Aims: The aim was to assess the etiology of sixth nerve palsy and on the basis of our data, to formulate a diagnostic algorithm for the management in sixth nerve palsy. Design: Retrospective chart review. Results: Of the 104 neurologically isolated cases, 9 cases were attributable to trauma, and 95 (86.36% cases were classified as nontraumatic, neurologically isolated cases. Of the 95 nontraumatic, isolated cases of sixth nerve palsy, 52 cases were associated with vasculopathic risk factors, namely diabetes and hypertension and were classified as vasculopathic sixth nerve palsy (54.7%, and those with a history of sixth nerve palsy from birth (6 cases were classified as congenital sixth nerve palsy (6.3%. Of the rest, neuroimaging alone yielded a cause in 18 of the 37 cases (48.64%. Of the other 19 cases where neuroimaging did not yield a cause, 6 cases were attributed to preceding history of infection (3 upper respiratory tract infection and 3 viral illnesses, 2 cases of sixth nerve palsy were found to be a false localizing sign in idiopathic intracranial hypertension and in 11 cases, the cause was undetermined. In these idiopathic cases of isolated sixth nerve palsy, neuroimaging yielded no positive findings. Conclusions: In the absence of risk factors, a suggestive history, or positive laboratory and clinical findings, neuroimaging can serve as a useful diagnostic tool in identifying the exact cause of sixth nerve palsy. Furthermore, we recommend an algorithm to assess the need for neuroimaging in sixth nerve palsy.

  15. Multi-disciplinary clinical protocol for the diagnosis of bulbar amyotrophic lateral sclerosis.

    Science.gov (United States)

    Chiaramonte, Rita; Di Luciano, Carmela; Chiaramonte, Ignazio; Serra, Agostino; Bonfiglio, Marco

    2018-04-23

    The objective of this study was to examine the role of different specialists in the diagnosis of amyotrophic lateral sclerosis (ALS), to understand changes in verbal expression and phonation, respiratory dynamics and swallowing that occurred rapidly over a short period of time. 22 patients with bulbar ALS were submitted for voice assessment, ENT evaluation, Multi-Dimensional Voice Program (MDVP), spectrogram, electroglottography, fiberoptic endoscopic evaluation of swallowing. In the early stage of the disease, the oral tract and velopharyngeal port were involved. Three months after the initial symptoms, most of the patients presented hoarseness, breathy voice, dysarthria, pitch modulation problems and difficulties in pronunciation of explosive, velar and lingual consonants. Values of MDVP were altered. Spectrogram showed an additional formant, due to nasal resonance. Electroglottography showed periodic oscillation of the vocal folds only during short vocal cycle. Swallowing was characterized by weakness and incoordination of oro-pharyngeal muscles with penetration or aspiration. A specific multidisciplinary clinical protocol was designed to report vocal parameters and swallowing disorders that changed more quickly in bulbar ALS patients. Furthermore, the patients were stratified according to involvement of pharyngeal structures, and severity index. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Environmental and occupational risk factors for progressive supranuclear palsy: Case-control study.

    Science.gov (United States)

    Litvan, Irene; Lees, Peter S J; Cunningham, Christopher R; Rai, Shesh N; Cambon, Alexander C; Standaert, David G; Marras, Connie; Juncos, Jorge; Riley, David; Reich, Stephen; Hall, Deborah; Kluger, Benzi; Bordelon, Yvette; Shprecher, David R

    2016-05-01

    The cause of progressive supranuclear palsy (PSP) is largely unknown. Based on evidence for impaired mitochondrial activity in PSP, we hypothesized that the disease may be related to exposure to environmental toxins, some of which are mitochondrial inhibitors. This multicenter case-control study included 284 incident PSP cases of 350 cases and 284 age-, sex-, and race-matched controls primarily from the same geographical areas. All subjects were administered standardized interviews to obtain data on demographics, residential history, and lifetime occupational history. An industrial hygienist and a toxicologist unaware of case status assessed occupational histories to estimate past exposure to metals, pesticides, organic solvents, and other chemicals. Cases and controls were similar on demographic factors. In unadjusted analyses, PSP was associated with lower education, lower income, more smoking pack-years, more years of drinking well water, more years living on a farm, more years living 1 mile from an agricultural region, more transportation jobs, and more jobs with exposure to metals in general. However, in adjusted models, only more years of drinking well water was significantly associated with PSP. There was an inverse association with having a college degree. We did not find evidence for a specific causative chemical exposure; higher number of years of drinking well water is a risk factor for PSP. This result remained significant after adjusting for income, smoking, education and occupational exposures. This is the first case-control study to demonstrate PSP is associated with environmental factors. © 2016 International Parkinson and Movement Disorder Society. © 2016 International Parkinson and Movement Disorder Society.

  17. Functional MRI, DTI and neurophysiology in horizontal gaze palsy with progressive scoliosis

    Energy Technology Data Exchange (ETDEWEB)

    Haller, Sven; Wetzel, Stephan G. [University Hospital Basel, Institute of Radiology, Department of Neuroradiology, Basel (Switzerland); Luetschg, Juerg [University Children' s Hospital (UKBB), Basel (Switzerland)

    2008-05-15

    Horizontal gaze palsy with progressive scoliosis (HGPPS) is an autosomal recessive disease due to a mutation in the ROBO3 gene. This rare disease is of particular interest because the absence, or at least reduction, of crossing of the ascending lemniscal and descending corticospinal tracts in the medulla predicts abnormal ipsilateral sensory and motor systems. We evaluated the use of functional magnetic resonance imaging (fMRI) for the first time in this disease and compared it to diffusion tensor imaging (DTI) tractography and neurophysiological findings in the same patient with genetically confirmed ROBO3 mutation. As expected, motor fMRI, somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP) were dominantly ipsilateral to the stimulation side. DTI tractography revealed ipsilateral ascending and descending connectivity in the brainstem yet normal interhemispheric connections in the corpus callosum. Auditory fMRI revealed bilateral auditory activation to monaural left-sided auditory stimulation. No significant cortical activation was observed after monaural right-sided stimulation, a hearing defect having been excluded. Prosaccades fMRI showed no activations in the eye-movement network. Motor fMRI confirmed the established findings of DTI and neurophysiology in the same patient. In suspected HGPPS, any technique appears appropriate for further investigation. Auditory fMRI suggests that a monaural auditory system with bilateral auditory activations might be a physiological advantage as compared to a binaural yet only unilateral auditory system, in analogy to anisometropic amblyopia. Moving-head fMRI studies in the future might show whether the compensatory head movements instead of normal eye movements activate the eye-movement network. (orig.)

  18. Functional MRI, DTI and neurophysiology in horizontal gaze palsy with progressive scoliosis

    International Nuclear Information System (INIS)

    Haller, Sven; Wetzel, Stephan G.; Luetschg, Juerg

    2008-01-01

    Horizontal gaze palsy with progressive scoliosis (HGPPS) is an autosomal recessive disease due to a mutation in the ROBO3 gene. This rare disease is of particular interest because the absence, or at least reduction, of crossing of the ascending lemniscal and descending corticospinal tracts in the medulla predicts abnormal ipsilateral sensory and motor systems. We evaluated the use of functional magnetic resonance imaging (fMRI) for the first time in this disease and compared it to diffusion tensor imaging (DTI) tractography and neurophysiological findings in the same patient with genetically confirmed ROBO3 mutation. As expected, motor fMRI, somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP) were dominantly ipsilateral to the stimulation side. DTI tractography revealed ipsilateral ascending and descending connectivity in the brainstem yet normal interhemispheric connections in the corpus callosum. Auditory fMRI revealed bilateral auditory activation to monaural left-sided auditory stimulation. No significant cortical activation was observed after monaural right-sided stimulation, a hearing defect having been excluded. Prosaccades fMRI showed no activations in the eye-movement network. Motor fMRI confirmed the established findings of DTI and neurophysiology in the same patient. In suspected HGPPS, any technique appears appropriate for further investigation. Auditory fMRI suggests that a monaural auditory system with bilateral auditory activations might be a physiological advantage as compared to a binaural yet only unilateral auditory system, in analogy to anisometropic amblyopia. Moving-head fMRI studies in the future might show whether the compensatory head movements instead of normal eye movements activate the eye-movement network. (orig.)

  19. H MR spectroscopy in Parkinson's disease and progressive supranuclear palsy : preliminary study

    International Nuclear Information System (INIS)

    Chang, Kee Hyun; Jeon, Beom Seok; Song, In Chan; Kim, Dong Sung; Min, Kwan Hong; Han, Moon Hee; Kang, Sa Ouk; Min, Byoung Goo; Han, Man Chung

    1996-01-01

    To determine whether h magnetic resonance spectroscopy (MRS) is useful in differentiating idiopathic Parkinson's disease (IPD) from progressive supranuclear palsy (PSP), based on metabolite ratios. Using a 1.5 T MR Unit, single voxel H MRS using STEAM with a TR of 2000 ms and a TE of 135ms was performed in seven PD and eight PSP patients. Five age-matched volunteers(mean age, 63 years) and another five younger healthy volunteers(mean age, 30 tears) were studied as normal controls. The regions of interest were the putamen and pallidum, with a size of 2X2X2 cm. After measuring the spectral intensities of each metabolite (N-acetylaspartate=NAA, choline=Cho, creatine=Cr and lactate), relative peak height ratios of NAA/Cr, Cho/Cr and NAA/ Cho, and lactate levels among four groups were compared. NAA/ Cho and NAA/ Cr ratios were statistically lower in the PSP group than the IPD group (1.21 ± 0.26 versus 1.45 ± 0.20, and 1.26 ± 0.23 versus 1.38 ± 0.19, respectively : p 0.05). Cho/Cr ratios were not different among four groups. Lactate was not detected in any patients. NAA/Cho and NAA/Cr ratios in the corpus stratum were significantly lower in the PSP group than in the age-matched control and IPD groups. These results suggest that loss of neuron cells in the corpus stratum is more prominent in PSP than in IPD, and that NAA/Cho and NAA/Cr ratios may help in differential diagnosis of IPD and PSP

  20. Functional MRI, DTI and neurophysiology in horizontal gaze palsy with progressive scoliosis.

    Science.gov (United States)

    Haller, Sven; Wetzel, Stephan G; Lütschg, Jürg

    2008-05-01

    Horizontal gaze palsy with progressive scoliosis (HGPPS) is an autosomal recessive disease due to a mutation in the ROBO3 gene. This rare disease is of particular interest because the absence, or at least reduction, of crossing of the ascending lemniscal and descending corticospinal tracts in the medulla predicts abnormal ipsilateral sensory and motor systems. We evaluated the use of functional magnetic resonance imaging (fMRI) for the first time in this disease and compared it to diffusion tensor imaging (DTI) tractography and neurophysiological findings in the same patient with genetically confirmed ROBO3 mutation. As expected, motor fMRI, somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP) were dominantly ipsilateral to the stimulation side. DTI tractography revealed ipsilateral ascending and descending connectivity in the brainstem yet normal interhemispheric connections in the corpus callosum. Auditory fMRI revealed bilateral auditory activation to monaural left-sided auditory stimulation. No significant cortical activation was observed after monaural right-sided stimulation, a hearing defect having been excluded. Prosaccades fMRI showed no activations in the eye-movement network. Motor fMRI confirmed the established findings of DTI and neurophysiology in the same patient. In suspected HGPPS, any technique appears appropriate for further investigation. Auditory fMRI suggests that a monaural auditory system with bilateral auditory activations might be a physiological advantage as compared to a binaural yet only unilateral auditory system, in analogy to anisometropic amblyopia. Moving-head fMRI studies in the future might show whether the compensatory head movements instead of normal eye movements activate the eye-movement network.

  1. Neuropathology and Therapeutic Intervention in Spinal and Bulbar Muscular Atrophy

    Directory of Open Access Journals (Sweden)

    Haruhiko Banno

    2009-03-01

    Full Text Available Spinal and bulbar muscular atrophy (SBMA is a hereditary motor neuron disease caused by the expansion of a polyglutamine tract in the androgen receptor (AR. The histopathological finding in SBMA is loss of lower motor neurons in the anterior horn of the spinal cord as well as in the brainstem motor nuclei. Animal studies have revealed that the pathogenesis of SBMA depends on the level of serum testosterone, and that androgen deprivation mitigates neurodegeneration through inhibition of nuclear accumulation of the pathogenic AR. Heat shock proteins, ubiquitin-proteasome system and transcriptional regulation are also potential targets of therapy development for SBMA.

  2. What makes children with cerebral palsy vulnerable to malnutrition? Findings from the Bangladesh cerebral palsy register (BCPR).

    Science.gov (United States)

    Jahan, Israt; Muhit, Mohammad; Karim, Tasneem; Smithers-Sheedy, Hayley; Novak, Iona; Jones, Cheryl; Badawi, Nadia; Khandaker, Gulam

    2018-04-16

    To assess the nutritional status and underlying risk factors for malnutrition among children with cerebral palsy in rural Bangladesh. We used data from the Bangladesh Cerebral Palsy Register; a prospective population based surveillance of children with cerebral palsy aged 0-18 years in a rural subdistrict of Bangladesh (i.e., Shahjadpur). Socio-demographic, clinical and anthropometric measurements were collected using Bangladesh Cerebral Palsy Register record form. Z scores were calculated using World Health Organization Anthro and World Health Organization AnthroPlus software. A total of 726 children with cerebral palsy were registered into the Bangladesh Cerebral Palsy Register (mean age 7.6 years, standard deviation 4.5, 38.1% female) between January 2015 and December 2016. More than two-third of children were underweight (70.0%) and stunted (73.1%). Mean z score for weight for age, height for age and weight for height were -2.8 (standard deviation 1.8), -3.1 (standard deviation 2.2) and -1.2 (standard deviation 2.3) respectively. Moderate to severe undernutrition (i.e., both underweight and stunting) were significantly associated with age, monthly family income, gross motor functional classification system and neurological type of cerebral palsy. The burden of undernutrition is high among children with cerebral palsy in rural Bangladesh which is augmented by both poverty and clinical severity. Enhancing clinical nutritional services for children with cerebral palsy should be a public health priority in Bangladesh. Implications for Rehabilitation Population-based surveillance data on nutritional status of children with cerebral palsy in Bangladesh indicates substantially high burden of malnutrition among children with CP in rural Bangladesh. Children with severe form of cerebral palsy, for example, higher Gross Motor Function Classification System (GMFCS) level, tri/quadriplegic cerebral palsy presents the highest proportion of severe malnutrition; hence, these

  3. Clinical Approach to Supranuclear Brainstem Saccadic Gaze Palsies

    Directory of Open Access Journals (Sweden)

    Alexandra Lloyd-Smith Sequeira

    2017-08-01

    Full Text Available Failure of brainstem supranuclear centers for saccadic eye movements results in the clinical presence of a brainstem-mediated supranuclear saccadic gaze palsy (SGP, which is manifested as slowing of saccades with or without range of motion limitation of eye movements and as loss of quick phases of optokinetic nystagmus. Limitation in the range of motion of eye movements is typically worse with saccades than with smooth pursuit and is overcome with vestibular–ocular reflexive eye movements. The differential diagnosis of SGPs is broad, although acute-onset SGP is most often from brainstem infarction and chronic vertical SGP is most commonly caused by the neurodegenerative condition progressive supranuclear palsy. In this review, we discuss the brainstem anatomy and physiology of the brainstem saccade-generating network; we discuss the clinical features of SGPs, with an emphasis on insights from quantitative ocular motor recordings; and we consider the broad differential diagnosis of SGPs.

  4. Bell's palsy before Bell: Cornelis Stalpart van der Wiel's observation of Bell's palsy in 1683.

    Science.gov (United States)

    van de Graaf, Robert C; Nicolai, Jean-Philippe A

    2005-11-01

    Bell's palsy is named after Sir Charles Bell (1774-1842), who has long been considered to be the first to describe idiopathic facial paralysis in the early 19th century. However, it was discovered that Nicolaus Anton Friedreich (1761-1836) and James Douglas (1675-1742) preceded him in the 18th century. Recently, an even earlier account of Bell's palsy was found, as observed by Cornelis Stalpart van der Wiel (1620-1702) from The Hague, The Netherlands in 1683. Because our current knowledge of the history of Bell's palsy before Bell is limited to a few documents, it is interesting to discuss Stalpart van der Wiel's description and determine its additional value for the history of Bell's palsy. It is concluded that Cornelis Stalpart van der Wiel was the first to record Bell's palsy in 1683. His manuscript provides clues for future historical research.

  5. Rehabilitation in progressive supranuclear palsy: Effectiveness of two multidisciplinary treatments.

    Directory of Open Access Journals (Sweden)

    Ilaria Clerici

    Full Text Available to date, there are no medical or surgical treatments for progressive supranuclear palsy (PSP. It is possible to speculate that patients with PSP could benefit from rehabilitative treatments designed for Parkinson's disease, including the use of robot-assisted walking training.to evaluate whether the use of the robotic device Lokomat® is superior in PSP patients to the use of treadmill with visual cues and auditory feedbacks (treadmill-plus in the context of an aerobic, multidisciplinary, intensive, motor-cognitive and goal-based rehabilitation treatment (MIRT conceived for Parkinsonian patients.we enrolled twenty-four PSP patients. Twelve subjects underwent a 4-week MIRT exploiting the use of the treadmill-plus (MIRT group. Twelve subjects underwent the same treatment, but replacing the treadmill-plus with Lokomat® (MIRT-Lokomat group. Subjects were evaluated with clinical and functional scales at admission and discharge. The primary outcomes were the total PSP Rating Scale (PSPRS score and its "limb" and "gait" sub-scores. Secondary outcomes were Berg Balance Scale (BBS, Six Minutes Walking test (6MWT and the number of falls.total PSPRS, PSPRS-gait sub-score, BBS, 6MWT and number of falls improved significantly in both groups (p ≤ 0.003 all, except 6MWT, p = 0.032 and p = 0.018 in MIRT-Lokomat and MIRT group respectively. The PSPRS-limb sub-score improved significantly only in the MIRT group (p = 0.002. A significant difference between groups was observed only for total PSPRS, indicating a slightly better improvement for patients in the MIRT group (p = 0.047. No differences between groups were revealed for the other outcomes, indicating that the effect of rehabilitation was similar in both groups.Lokomat® training, in comparison with treadmill-plus training, does not provide further benefits in PSP patients undergoing MIRT. Our findings suggest the usefulness of an aerobic, multidisciplinary, intensive, motor-cognitive and goal

  6. Silencing neuronal mutant androgen receptor in a mouse model of spinal and bulbar muscular atrophy.

    Science.gov (United States)

    Sahashi, Kentaro; Katsuno, Masahisa; Hung, Gene; Adachi, Hiroaki; Kondo, Naohide; Nakatsuji, Hideaki; Tohnai, Genki; Iida, Madoka; Bennett, C Frank; Sobue, Gen

    2015-11-01

    Spinal and bulbar muscular atrophy (SBMA), an adult-onset neurodegenerative disease that affects males, results from a CAG triplet repeat/polyglutamine expansions in the androgen receptor (AR) gene. Patients develop progressive muscular weakness and atrophy, and no effective therapy is currently available. The tissue-specific pathogenesis, especially relative pathological contributions between degenerative motor neurons and muscles, remains inconclusive. Though peripheral pathology in skeletal muscle caused by toxic AR protein has been recently reported to play a pivotal role in the pathogenesis of SBMA using mouse models, the role of motor neuron degeneration in SBMA has not been rigorously investigated. Here, we exploited synthetic antisense oligonucleotides to inhibit the RNA levels of mutant AR in the central nervous system (CNS) and explore its therapeutic effects in our SBMA mouse model that harbors a mutant AR gene with 97 CAG expansions and characteristic SBMA-like neurogenic phenotypes. A single intracerebroventricular administration of the antisense oligonucleotides in the presymptomatic phase efficiently suppressed the mutant gene expression in the CNS, and delayed the onset and progression of motor dysfunction, improved body weight gain and survival with the amelioration of neuronal histopathology in motor units such as spinal motor neurons, neuromuscular junctions and skeletal muscle. These findings highlight the importance of the neurotoxicity of mutant AR protein in motor neurons as a therapeutic target. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  7. Radiation-induced cranial nerve palsy and its causative factors in nasopharyngeal carcinoma

    International Nuclear Information System (INIS)

    Kong Lin; Zhang Youwang; Wu Yongru; Guo Xiaomao; Li Longgen

    2005-01-01

    Objective: To analyze the incidence and causative factors of radiation-induced cranial nerve palsy in patients with nasopharyngeal carcinoma (NPC). Methods: 512 NPC patients who underwent radiotherapy from January 1, 1989 to December 31, 1990 and from January 1, 1994 to December 31, 1995 were retrospectively analyzed. According to Fuzhou' 92 NPC Staging Classification, there are 31 patients in stage I, 212 in stage II, 198 in stage III and 71 in stage IV. All patients were treated by 60 Co or 6 MV X-ray with faciocervical fields or pre-auricular fields to primary area. Some patients were boosted by post-auricular fields or cranial fields. The median dose to the nasopharyngeal region was 7130 cGy by external beam radiotherapy. Thirty-four patients were boosted by brachytherapy. The medial dose to cervical lymph nodes was 6410 cGy as definitive treatment and 5480 cGy as prophylactic treatment. 101 patients were treated with combined chemotherapy. Results: The median follow-up was 6.7 years . Radiation-induced cranial nerve palsies occurred in 81 among the 512 patients. The 5- and 10-year cumulative incidences were 10.3%, 25.4%, respectively. The most common affected nerve was XII. On multivariates analysis, cranial nerve invasion before radiation, chemotherapy, dose to the nasopharyngeal region and age were the independent factors of radiation-induced cranial nerve palsy on nerve I-VII, while the N stage and the radiation fields were independent factors on nerve IX-XII. The cumulative incidence of cranial nerve I-VII palsies increased in patients with cranial nerve invasion, chemotherapy and the dose to the nasopharyngeal region (>7000 cGy). The cumulative incidence of cranial nerve IX- XII palsies increased in patients with advanced N stage. Patients in the first group of treatment field had the highest risk to progress cranial nerve IX-XII palsies, followed by the second group, and the third group had the lowest risk. Only 1 in 34 patients with brachytherapy

  8. Isolated trochlear nerve palsy with midbrain hemorrhage

    Directory of Open Access Journals (Sweden)

    Raghavendra S

    2010-01-01

    Full Text Available Midbrain hemorrhage causing isolated fourth nerve palsy is extremely rare. Idiopathic, traumatic and congenital abnormalities are the most common causes of fourth nerve palsy. We report acute isolated fourth nerve palsy in an 18-year-old lady due to a midbrain hemorrhage probably due to a midbrain cavernoma. The case highlights the need for neuroimaging in selected cases of isolated trochlear nerve palsy.

  9. Neurocysticercosis presenting as pseudobulbar palsy

    Directory of Open Access Journals (Sweden)

    Arinaganahalli Subbanna Praveen Kumar

    2014-01-01

    Full Text Available Neurocysticercosis (NCC is the most common helminthic infestation of the central nervous system (CNS and a leading cause of acquired epilepsy worldwide. The common manifestations of NCC are seizures and headache. The NCC as a cause of pseudobulbar palsy is very unusual and not reported yet in the literature. A pseudobulbar palsy can occur in any disorder that causes bilateral corticobulbar disease. The common etiologies of pseudobulbar palsy are vascular, demyelinative, or motor neuron disease. We report a 38-year-old female patient who presented with partial seizures and pseudobulbar palsy. The MRI brain showed multiple small cysts with scolex in both the cerebral hemispheres and a giant intraparenchymal cyst. Our patient responded well to standard treatment of neurocysticercosis and antiepileptics.

  10. Retinal degeneration in progressive supranuclear palsy measured by optical coherence tomography and scanning laser polarimetry.

    Science.gov (United States)

    Stemplewitz, Birthe; Kromer, Robert; Vettorazzi, Eik; Hidding, Ute; Frings, Andreas; Buhmann, Carsten

    2017-07-13

    This cross-sectional study compared the retinal morphology between patients with progressive supranuclear palsy (PSP) and healthy controls. (The retinal nerve fiber layer (RNFL) around the optic disc and the retina in the macular area of 22 PSP patients and 151 controls were investigated by spectral domain optical coherence tomography (SD-OCT). Additionally, the RNFL and the nerve fiber index (NFI) were measured by scanning laser polarimetry (SLP). Results of RNFL measurements with SD-OCT and SLP were compared to assess diagnostic discriminatory power. Applying OCT, PSP patients showed a smaller RNFL thickness in the inferior nasal and inferior temporal areas. The macular volume and the thickness of the majority of macular sectors were reduced compared to controls. SLP data showed a thinner RNFL thickness and an increase in the NFI in PSP patients. Sensitivity and specificity to discriminate PSP patients from controls were higher applying SLP than SD-OCT. Retinal changes did not correlate with disease duration or severity in any OCT or SLP measurement. PSP seems to be associated with reduced thickness and volume of the macula and reduction of the RNFL, independent of disease duration or severity. Retinal imaging with SD-OCT and SLP might become an additional tool in PSP diagnosis.

  11. The Brown-Vialetto-Van Laere and Fazio Londe syndrome revisited: natural history, genetics, treatment and future perspectives

    NARCIS (Netherlands)

    Bosch, Annet M.; Stroek, Kevin; Abeling, Nico G.; Waterham, Hans R.; Ijlst, Lodewijk; Wanders, Ronald J. A.

    2012-01-01

    The Brown-Vialetto-Van Laere syndrome is a rare neurological disorder which may present at all ages with sensorineural deafness, bulbar palsy and respiratory compromise. Fazio-Londe syndrome is considered to be the same disease entity. Recently it was demonstrated that in some patients the disease

  12. Multi-institutional Outcomes of Endoscopic Management of Stricture Recurrence after Bulbar Urethroplasty.

    Science.gov (United States)

    Sukumar, Shyam; Elliott, Sean P; Myers, Jeremy B; Voelzke, Bryan B; Smith, Thomas G; Carolan, Alexandra Mc; Maidaa, Michael; Vanni, Alex J; Breyer, Benjamin N; Erickson, Bradley A

    2018-05-03

    Approximately 10-20% of patients will have a recurrence after urethroplasty. Initial management of these recurrences is often with urethral dilation (UD) or direct vision internal urethrotomy (DVIU). In the current study, we describe outcomes of endoscopic management of stricture recurrence after bulbar urethroplasty. We retrospectively reviewed bulbar urethroplasty data from 5 surgeons from the Trauma and Urologic Reconstruction Network of Surgeons. Men who underwent UD or DVIU for urethroplasty recurrence were identified. Recurrence was defined as inability to pass a 17Fr cystoscope through the area of reconstruction. The primary outcome was the success rate of recurrence management. Comparisons were made between UD and DVIU and then between endoscopic management of recurrences after excision and primary anastomosis urethroplasty (EPA) versus substitutional repairs using time-to-event statistics. There were 53 men with recurrence that were initially managed endoscopically. Median time to urethral stricture recurrence after urethroplasty was noted to be 5 months. At a median follow-up of 5 months, overall success was 42%. Success after UD (n=1/10, 10%) was significantly lower than after DVIU (n=21/43, 49%; p urethroplasty. DVIU is more successful for patients with a recurrence after a substitution urethroplasty compared to after EPA, perhaps indicating a different mechanism of recurrence for EPA (ischemic) versus substitution urethroplasty (non-ischemic). Copyright © 2018 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  13. Neurorehabilitation with versus without resistance training after botulinum toxin treatment in children with cerebral palsy

    DEFF Research Database (Denmark)

    Bandholm, Thomas Quaade; Jensen, Bente Rona; Nielsen, Lone M

    2012-01-01

    Objective: To compare the effects of physical rehabilitation with (PRT) and without (CON) progressive resistance training following treatment of spastic plantarflexors with botulinum toxin type A (BoNT) in children with cerebral palsy (CP). Methods: Fourteen children with CP performed supervised...

  14. Eagle's syndrome with facial palsy

    Directory of Open Access Journals (Sweden)

    Mohammed Al-Hashim

    2017-01-01

    Full Text Available Eagle's syndrome (ES is a rare disease in which the styloid process is elongated and compressing adjacent structures. We describe a rare presentation of ES in which the patient presented with facial palsy. Facial palsy as a presentation of ES is very rare. A review of the English literature revealed only one previously reported case. Our case is a 39-year-old male who presented with left facial palsy. He also reported a 9-year history of the classical symptoms of ES. A computed tomography scan with three-dimensional reconstruction confirmed the diagnoses. He was started on conservative management but without significant improvement. Surgical intervention was offered, but the patient refused. It is important for otolaryngologists, dentists, and other specialists who deal with head and neck problems to be able to recognize ES despite its rarity. Although the patient responded to a treatment similar to that of Bell's palsy because of the clinical features and imaging, ES was most likely the cause of his facial palsy.

  15. The Disturbance of Gaze in Progressive Supranuclear Palsy (PSP: Implications for Pathogenesis

    Directory of Open Access Journals (Sweden)

    Athena L Chen

    2010-12-01

    Full Text Available Progressive supranuclear palsy (PSP is a disease of later life that is currently regarded as a form of neurodegenerative tauopathy. Disturbance of gaze is a cardinal clinical feature of PSP that often helps clinicians to establish the diagnosis. Since the neurobiology of gaze control is now well understood, it is possible to use eye movements as investigational tools to understand aspects of the pathogenesis of PSP. In this review, we summarize each disorder of gaze control that occurs in PSP, drawing on our studies of fifty patients, and on reports from other laboratories that have measured the disturbances of eye movements. When these gaze disorders are approached by considering each functional class of eye movements and its neurobiological basis, a distinct pattern of eye movement deficits emerges that provides insight into the pathogenesis of PSP. Although some aspects of all forms of eye movements are affected in PSP, the predominant defects concern vertical saccades (slow and hypometric, both up and down, impaired vergence, and inability to modulate the linear vestibulo-ocular reflex appropriately for viewing distance. These vertical and vergence eye movements habitually work in concert to enable visuomotor skills that are important during locomotion with the hands free. Taken with the prominent early feature of falls, these findings suggest that PSP tauopathy impairs a recently-evolved neural system concerned with bipedal locomotion in an erect posture and frequent gaze shifts between the distant environment and proximate hands. This approach provides a conceptual framework that can be used to address the nosological challenge posed by overlapping clinical and neuropathological features of neurodegenerative tauopathies.

  16. MR imaging of cerebral palsy

    International Nuclear Information System (INIS)

    Saginoya, Toshiyuki; Yamaguchi, Keiichiro; Kuniyoshi, Kazuhide

    1996-01-01

    We evaluated 35 patients with cerebral palsy on the basis of MR imaging findings in the brain. The types of palsy were spastic quadriplegia (n=11), spastic diplegia (n=9), spastic hemiplegia (n=2), double hemiplegia (n=1), athetosis (n=10) and mixed (n=2). Of all patients, 28 (80%) generated abnormal findings. In spastic quadriplegia, although eight cases revealed severe brain damage, two cases showed no abnormal findings in the brain. One of the three had cervical cord compression caused by atlanto-axial subluxation. In spastic diplegia, the findings were divided according to whether the patient was born at term or preterm. If the patient had been born prematurely, the findings showed periventricular leukomalacia and abnormally high intensity in the posterior limbs of the internal capsule on T2-weighted images. MR imaging in spastic hemiplegia revealed cerebral infarction. In the athetoid type, half of all cases showed either no abnormal findings or slight widening of the lateral ventricle. Three cases showed abnormal signals of the basal ganglia. The reason why athetoid-type palsy did not show severe abnormality is unknown. We believe that MR imaging is a useful diagnostic modality to detect damage in the brain in cerebral palsy and plays an important role in the differentiation of cerebral palsy from the spastic palsy disease. (author)

  17. MR imaging of cerebral palsy

    Energy Technology Data Exchange (ETDEWEB)

    Saginoya, Toshiyuki [Urasoe General Hospital, Okinawa (Japan); Yamaguchi, Keiichiro; Kuniyoshi, Kazuhide [and others

    1996-06-01

    We evaluated 35 patients with cerebral palsy on the basis of MR imaging findings in the brain. The types of palsy were spastic quadriplegia (n=11), spastic diplegia (n=9), spastic hemiplegia (n=2), double hemiplegia (n=1), athetosis (n=10) and mixed (n=2). Of all patients, 28 (80%) generated abnormal findings. In spastic quadriplegia, although eight cases revealed severe brain damage, two cases showed no abnormal findings in the brain. One of the three had cervical cord compression caused by atlanto-axial subluxation. In spastic diplegia, the findings were divided according to whether the patient was born at term or preterm. If the patient had been born prematurely, the findings showed periventricular leukomalacia and abnormally high intensity in the posterior limbs of the internal capsule on T2-weighted images. MR imaging in spastic hemiplegia revealed cerebral infarction. In the athetoid type, half of all cases showed either no abnormal findings or slight widening of the lateral ventricle. Three cases showed abnormal signals of the basal ganglia. The reason why athetoid-type palsy did not show severe abnormality is unknown. We believe that MR imaging is a useful diagnostic modality to detect damage in the brain in cerebral palsy and plays an important role in the differentiation of cerebral palsy from the spastic palsy disease. (author)

  18. Unusual cause of brachial palsy with diaphragmatic palsy.

    Science.gov (United States)

    Gupta, Vishal; Pandita, Aakash; Panghal, Astha; Hassan, Neha

    2018-05-12

    We report a preterm neonate born with respiratory distress. The neonate was found to have diaphragmatic palsy and brachial palsy. The neonate was born by caesarean section and there was no history of birth trauma. On examination, there was bilateral congenital talipes equinovarus and a scar was present on the forearm. The mother had a history of chickenpox during the 16 weeks of pregnancy for which no treatment was sought. On investigation, PCR for varicella was found to be positive in the neonate. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  19. Clinical practice guideline: Bell's Palsy executive summary.

    Science.gov (United States)

    Baugh, Reginald F; Basura, Gregory J; Ishii, Lisa E; Schwartz, Seth R; Drumheller, Caitlin Murray; Burkholder, Rebecca; Deckard, Nathan A; Dawson, Cindy; Driscoll, Colin; Gillespie, M Boyd; Gurgel, Richard K; Halperin, John; Khalid, Ayesha N; Kumar, Kaparaboyna Ashok; Micco, Alan; Munsell, Debra; Rosenbaum, Steven; Vaughan, William

    2013-11-01

    The American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF) has published a supplement to this issue featuring the new Clinical Practice Guideline: Bell's Palsy. To assist in implementing the guideline recommendations, this article summarizes the rationale, purpose, and key action statements. The 11 recommendations developed encourage accurate and efficient diagnosis and treatment and, when applicable, facilitate patient follow-up to address the management of long-term sequelae or evaluation of new or worsening symptoms not indicative of Bell's palsy. There are myriad treatment options for Bell's palsy; some controversy exists regarding the effectiveness of several of these options, and there are consequent variations in care. In addition, there are numerous diagnostic tests available that are used in the evaluation of patients with Bell's palsy. Many of these tests are of questionable benefit in Bell's palsy. Furthermore, while patients with Bell's palsy enter the health care system with facial paresis/paralysis as a primary complaint, not all patients with facial paresis/paralysis have Bell's palsy. It is a concern that patients with alternative underlying etiologies may be misdiagnosed or have an unnecessary delay in diagnosis. All of these quality concerns provide an important opportunity for improvement in the diagnosis and management of patients with Bell's palsy.

  20. Biofeedback Therapy Effect on Facial Nerve Palsy and Prevention of Synkinesis

    Directory of Open Access Journals (Sweden)

    Abbas ali Pour-Momeny

    2011-04-01

    Full Text Available Objective: Synkiesia is a sequel of facial nerve palsy. It usually begins 3-4 months after axonal regeneration and progressed up to two years afterward. Treatment of synkinesia is very difficult and sometimes impossible.The aim of our study is find a better procedure to treat facial nerve palsy and prevent synkinesia. Materials and Methods: Twenty nine patients with facial nerve palsy were selected by electrodiagnosis tests. They were divided in two groups. The experimental group was treated by biofeedback electromyography and the second group was treated by common physiotherapy. The evaluation of all patients was done by Photoshop assessment and facial grading scale before and after treatment. Result: After the treatment, a significant general improvement was observed in both groups (p<0.05, but in experimental group (biofeedback showed better result than the other one. The number of patients with synkinesia as well as the severity of their synkinesis in experimental group were lesser than the other one. Conclusion: Biofeedback therapy is more efficient than common physiotherapy.By using this approach, control and reducing synkinesia is more feasible. Assessment by Pohotoshop procedure showed better accuracy than facial grading scale.

  1. Progressive multifocal leukoencephalopathy limited to the brain stem

    Energy Technology Data Exchange (ETDEWEB)

    Kastrup, O.; Maschke, M.; Diener, H.C. [Neurologische Universitaetsklinik, University of Essen (Germany); Wanke, I. [Department of Neuroradiology, University of Essen (Germany)

    2002-03-01

    Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating slow-virus encephalitis caused by the JC polyomavirus in 2-5% of patients with AIDS. MRI typically shows multiple lesions in the cerebral hemispheres. We present a rare case of rapidly evolving and lethal PML with a severe bulbar syndrome and spastic tetraparesis in a patient with AIDS. MRI showed high-signal lesions on T2-weighted images confined to the brain stem, extending from the medulla oblongata to the midbrain. JC virus polymerase chain reaction in cerebrospinal fluid was positive, and neuropathology showed the findings of PML. This case was also notable because of the rapid progression despite improved immune status with antiretroviral therapy. (orig.)

  2. Surgical management of third nerve palsy

    Directory of Open Access Journals (Sweden)

    Anupam Singh

    2016-01-01

    Full Text Available Third nerve paralysis has been known to be associated with a wide spectrum of presentation and other associated factors such as the presence of ptosis, pupillary involvement, amblyopia, aberrant regeneration, poor bell′s phenomenon, superior oblique (SO overaction, and lateral rectus (LR contracture. Correction of strabismus due to third nerve palsy can be complex as four out of the six extraocular muscles are involved and therefore should be approached differently. Third nerve palsy can be congenital or acquired. The common causes of isolated third nerve palsy in children are congenital (43%, trauma (20%, inflammation (13%, aneurysm (7%, and ophthalmoplegic migraine. Whereas, in adult population, common etiologies are vasculopathic disorders (diabetes mellitus, hypertension, aneurysm, and trauma. Treatment can be both nonsurgical and surgical. As nonsurgical modalities are not of much help, surgery remains the main-stay of treatment. Surgical strategies are different for complete and partial third nerve palsy. Surgery for complete third nerve palsy may involve supra-maximal recession - resection of the recti. This may be combined with SO transposition and augmented by surgery on the other eye. For partial third nerve, palsy surgery is determined according to nature and extent of involvement of extraocular muscles.

  3. Surgical management of third nerve palsy

    Science.gov (United States)

    Singh, Anupam; Bahuguna, Chirag; Nagpal, Ritu; Kumar, Barun

    2016-01-01

    Third nerve paralysis has been known to be associated with a wide spectrum of presentation and other associated factors such as the presence of ptosis, pupillary involvement, amblyopia, aberrant regeneration, poor bell's phenomenon, superior oblique (SO) overaction, and lateral rectus (LR) contracture. Correction of strabismus due to third nerve palsy can be complex as four out of the six extraocular muscles are involved and therefore should be approached differently. Third nerve palsy can be congenital or acquired. The common causes of isolated third nerve palsy in children are congenital (43%), trauma (20%), inflammation (13%), aneurysm (7%), and ophthalmoplegic migraine. Whereas, in adult population, common etiologies are vasculopathic disorders (diabetes mellitus, hypertension), aneurysm, and trauma. Treatment can be both nonsurgical and surgical. As nonsurgical modalities are not of much help, surgery remains the main-stay of treatment. Surgical strategies are different for complete and partial third nerve palsy. Surgery for complete third nerve palsy may involve supra-maximal recession - resection of the recti. This may be combined with SO transposition and augmented by surgery on the other eye. For partial third nerve, palsy surgery is determined according to nature and extent of involvement of extraocular muscles. PMID:27433033

  4. A COMPREHENSIVE ANALYSIS OF ISOLATED INFRANUCLEAR ABDUCENS NERVE PALSY IN A TERTIARY EYE CARE CENTRE

    Directory of Open Access Journals (Sweden)

    G. Dhamodara

    2017-01-01

    Full Text Available BACKGROUND A comprehensive analysis of the aetiology and clinical profile of isolated infranuclear abducens nerve palsy in a tertiary eye care centre. MATERIALS AND METHODS A hospital-based retrospective case series analysis of 90 isolated infranuclear neurogenic abducens nerve palsies. Documentation included age, gender, presenting complaints, history of diabetes mellitus, hypertension, mode of onset, progression of the disease, treatment given and recovery rate was evaluated. Detailed ophthalmic evaluation of both eyes including anterior segment examination, extraocular movements, diplopia charting and Hess charting. Thorough central nervous system examination and systemic examination was done. Inclusion Criteria- All isolated infranuclear neurogenic lesions of abducens nerve palsy. Exclusion Criteria- Conditions like supranuclear lesions, myasthenia, orbital inflammation and myopathies, false localising sign of abducens nerve palsy were excluded by appropriate testing and investigations. RESULTS Total cases were 90 patients. Mean age of presentation was between 3rd to 5th decades with male preponderance. Commonest presenting symptom was diplopia (71.1%, commonest cause being idiopathic neuritis (48%, diabetes mellitus (20%, hypertension (15%, trauma (10% and others (7%. CONCLUSION In our study, isolated infranuclear abducens nerve palsy with nonspecific aetiology predominantly affecting males of 3 rd to 5 th decade with variable recovery rates were seen. Hence, careful clinical examination in all cases is essential with close follow up on a long-time basis.

  5. Disease-specific structural changes in thalamus and dentatorubrothalamic tract in progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

    Surova, Yulia; Hall, Sara; Widner, Haakan [Lund University, Department of Clinical Sciences, Lund (Sweden); Skaane University Hospital, Department of Neurology, Lund (Sweden); Nilsson, Markus [Lund University, Lund University Bioimaging Center, Lund (Sweden); Laett, Jimmy [Skaane University Hospital, Center for Medical Imaging and Physiology, Lund (Sweden); Lampinen, Bjoern [Lund University, Department of Medical Radiation Physics, Lund (Sweden); Lindberg, Olof [Malmoe, Lund University, Department of Clinical Sciences, Malmoe (Sweden); Nilsson, Christer [Skaane University Hospital, Department of Neurology, Lund (Sweden); Malmoe, Lund University, Department of Clinical Sciences, Malmoe (Sweden); Westen, Danielle van [Lund University, Department of Clinical Sciences, Lund (Sweden); Skaane University Hospital, Center for Medical Imaging and Physiology, Lund (Sweden); Hansson, Oskar [Malmoe, Lund University, Department of Clinical Sciences, Malmoe (Sweden); Skaane University Hospital, Memory Clinic, Lund (Sweden)

    2015-11-15

    The aim of this study is to identify disease-specific changes of the thalamus, basal ganglia, pons, and midbrain in patients with progressive supranuclear palsy (PSP), Parkinson's disease (PD), and multiple system atrophy with predominant parkinsonism (MSA-P) using diffusion tensor imaging and volumetric analysis. MRI diffusion and volumetric data were acquired in a derivation of 30 controls and 8 patients with PSP and a validation cohort comprised of controls (n = 21) and patients with PSP (n = 27), PD (n = 10), and MSA-P (n = 11). Analysis was performed using regions of interest (ROI), tract-based spatial statistic (TBSS), and tractography and results compared between diagnostic groups. In the derivation cohort, we observed increased mean diffusivity (MD) in the thalamus, superior cerebellar peduncle, and the midbrain in PSP compared to controls. Furthermore, volumetric analysis showed reduced thalamic volumes in PSP. In the validation cohort, the observations of increased MD were replicated by ROI-based analysis and in the thalamus by TBSS-based analysis. Such differences were not found for patients with PD in any of the cohorts. Tractography of the dentatorubrothalamic tract (DRTT) showed increased MD in PSP patients from both cohorts compared to controls and in the validation cohort in PSP compared to PD and MSA patients. Increased MD in the thalamus and along the DRTT correlated with disease stage and motor function in PSP. Patients with PSP, but not PD or MSA-P, exhibit signs of structural abnormalities in the thalamus and in the DRTT. These changes are associated with disease stage and impaired motor function. (orig.)

  6. Disease-specific structural changes in thalamus and dentatorubrothalamic tract in progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Surova, Yulia; Hall, Sara; Widner, Haakan; Nilsson, Markus; Laett, Jimmy; Lampinen, Bjoern; Lindberg, Olof; Nilsson, Christer; Westen, Danielle van; Hansson, Oskar

    2015-01-01

    The aim of this study is to identify disease-specific changes of the thalamus, basal ganglia, pons, and midbrain in patients with progressive supranuclear palsy (PSP), Parkinson's disease (PD), and multiple system atrophy with predominant parkinsonism (MSA-P) using diffusion tensor imaging and volumetric analysis. MRI diffusion and volumetric data were acquired in a derivation of 30 controls and 8 patients with PSP and a validation cohort comprised of controls (n = 21) and patients with PSP (n = 27), PD (n = 10), and MSA-P (n = 11). Analysis was performed using regions of interest (ROI), tract-based spatial statistic (TBSS), and tractography and results compared between diagnostic groups. In the derivation cohort, we observed increased mean diffusivity (MD) in the thalamus, superior cerebellar peduncle, and the midbrain in PSP compared to controls. Furthermore, volumetric analysis showed reduced thalamic volumes in PSP. In the validation cohort, the observations of increased MD were replicated by ROI-based analysis and in the thalamus by TBSS-based analysis. Such differences were not found for patients with PD in any of the cohorts. Tractography of the dentatorubrothalamic tract (DRTT) showed increased MD in PSP patients from both cohorts compared to controls and in the validation cohort in PSP compared to PD and MSA patients. Increased MD in the thalamus and along the DRTT correlated with disease stage and motor function in PSP. Patients with PSP, but not PD or MSA-P, exhibit signs of structural abnormalities in the thalamus and in the DRTT. These changes are associated with disease stage and impaired motor function. (orig.)

  7. A Case Study: Effect of Progressive Resistance and Balance Training on Upper Trunk Muscle Strength of Children with Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Mehrnoush Ismailiyan

    2016-04-01

    Conclusion The results of this study showed that 8 weeks of progressive resistance and balance training (in combination has increased muscle strength in children with cerebral palsy. The present research showed that resistance and balanced trainings have significant effects on muscle strength of children with CP. It seems that these practices have been effective, especially for the wrist flexor and elbow flexor muscles. It can be said that the increase in the muscles of children with CP was due to practice principle along with increase in neuronal compatibility. One of the important points in the effectiveness of resistance training is the intensity of training. The results showed that resistance and balanced trainings increase the muscle strength of children with CP. This power could be partly due to increase in muscle volume and partly due to anabolic hormones.

  8. [Differential diagnosis and atypical subsets of amyotrophic lateral sclerosis].

    Science.gov (United States)

    Pradat, P-F; Bruneteau, G

    2006-06-01

    Amyotrophic lateral sclerosis (ALS) is a progressive degeneration of upper and lower motor neurons. In the absence of any validated biological marker, the diagnosis of ALS depends upon recognition of characteristic symptoms and signs together with supportive electrophysiological findings. The diagnosis of ALS is easy to recognize in its fully developed form but during the early stages both false positive and false negative diagnoses are common. In clinical practice, diagnostic difficulties mostly arise with patients who present either with only upper motor neuron, or with only lower motor neuron signs. It may be difficult to distinguish ALS with clinically predominant lower motor neuron involvement from alternative diagnoses including spinal atrophies of adult onset, Kennedy's disease, inclusion body myositis and motor neuropathies with conduction blocks. The diagnosis of ALS related syndromes (progressive muscular atrophy, primary lateral sclerosis and progressive bulbar palsy) requires the elimination of alternate diagnoses. This paper reviews the main characteristics of diseases mimicking ALS and the atypical subsets of ALS.

  9. Idiopathic Non-traumatic Facial Nerve Palsy (Bell’s Palsy) in Neonates; An Atypical Age and Management Dilemma

    Science.gov (United States)

    Khair, Abdulhafeez M.; Ibrahim, Khalid

    2018-01-01

    Idiopathic (Bell’s) palsy is the commonest cause of unilateral facial paralysis in children. Although being idiopathic by definition, possible infectious, inflammatory, and ischemic triggers have been suggested. Bell’s palsy is thought to be responsible for up to three-fourths of cases of acute unilateral facial paralysis worldwide. The diagnosis has to be reached after other causes of acute peripheral palsy have been excluded. However, it is rarely described in neonates and young infants. Steroids may have some role in treatment, but antiviral therapies have doubtful evidence of benefit. Prognosis is good, though residual dysfunction is occasionally encountered. We report the case of a two-week-old neonate with no prior illnesses who presented with acute left facial palsy. Clinical findings and normal brain imaging were consistent with the diagnosis of Bell’s palsy. The patient had a good response to oral steroids. PMID:29468002

  10. Idiopathic Non-traumatic Facial Nerve Palsy (Bell’s Palsy in Neonates; An Atypical Age and Management Dilemma

    Directory of Open Access Journals (Sweden)

    Abdulhafeez M. Khair

    2018-01-01

    Full Text Available Idiopathic (Bell’s palsy is the commonest cause of unilateral facial paralysis in children. Although being idiopathic by definition, possible infectious, inflammatory, and ischemic triggers have been suggested. Bell’s palsy is thought to be responsible for up to three-fourths of cases of acute unilateral facial paralysis worldwide. The diagnosis has to be reached after other causes of acute peripheral palsy have been excluded. However, it is rarely described in neonates and young infants. Steroids may have some role in treatment, but antiviral therapies have doubtful evidence of benefit. Prognosis is good, though residual dysfunction is occasionally encountered. We report the case of a two-week-old neonate with no prior illnesses who presented with acute left facial palsy. Clinical findings and normal brain imaging were consistent with the diagnosis of Bell’s palsy. The patient had a good response to oral steroids.

  11. Sarcoma de Kaposi en conjuntiva bulbar

    Directory of Open Access Journals (Sweden)

    Ileana Agramonte Centelles

    Full Text Available Paciente masculino de 29 años de edad, raza blanca, soltero, profesor universitario, con antecedentes de padecer crisis de epilepsia tratado con fenitoína y actualmente controlado, menciona que desde hace aproximadamente 4 semanas comenzó con ojo rojo y molestias oculares del ojo derecho, por lo cual acudió a su área de salud donde fue tratado como cuadro de conjuntivitis. No mostró mejoría alguna, sino empeoramiento del cuadro clínico, y observó un enrojecimiento ocular intenso en el ángulo interno de dicho ojo que se fue extendiendo, acompañado de ligera fotofobia. Por la tórpida evolución del cuadro decidió acudir a nuestra institución por lo cual fue remitido a la Consulta de Oculoplastia. También refirió que desde hacía dos meses había presentado anorexia, dificultad al comer, así como pérdida de peso, por lo cual se decidió comenzar estudio y tratamiento. Se decidió realizar la resección de la masa tumoral en conjuntiva bulbar y se envió para estudio anatomopatológico. El resultado fue compatible con un sarcoma de Kaposi.

  12. Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options.

    Science.gov (United States)

    Lamb, Ruth; Rohrer, Jonathan D; Lees, Andrew J; Morris, Huw R

    2016-09-01

    There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case series, or expert opinion. Levodopa may provide some improvement in symptoms of Parkinsonism (specifically bradykinesia and rigidity) in PSP and CBD; however, evidence is conflicting and where present, benefits are often negligible and short lived. In fact, "poor" response to levodopa forms part of the NINDS-SPSP criteria for the diagnosis of PSP and consensus criteria for the diagnosis of CBD (Lang Mov Disord. 20 Suppl 1:S83-91, 2005; Litvan et al. Neurology. 48:119-25, 1997; Armstrong et al. Neurology. 80(5):496-503, 2013). There is some evidence that intrasalivery gland botulinum toxin is useful in managing problematic sialorrhea and that intramuscular botulinum toxin and baclofen are helpful in reducing dystonia, including blepharospasm. Benzodiazepines may also be useful in managing dystonia. Myoclonus may be managed using levetiracetam and benzodiazepines. Pharmacological agents licensed for Alzheimer's disease (such as acetylcholinesterase inhibitors and N-Methyl-D-aspartate receptor antagonists) have been used off-label in PSP, CBD, and other tauopathies with the aim of improving cognition; however, there is limited evidence that they are effective and risk of adverse effects may outweigh benefits. The use of atypical antipsychotics for behavioural symptoms is not recommended in the elderly or those with demetia associated conditions and most antipsychotics will worsen Parkinsonism. Antidepressants may be useful for behavioral symptoms and depression but are often poorly tolerated due to adverse effects. In the absence of an effective drug treatment to target the underlying cause of

  13. Auditory Selective Attention in Cerebral-Palsied Individuals.

    Science.gov (United States)

    Laraway, Lee Ann

    1985-01-01

    To examine differences between auditory selective attention abilities of normal and cerebral-palsied individuals, 23 cerebral-palsied and 23 normal subjects (5-21) were asked to repeat a series of 30 items in presence of intermittent white noise. Results indicated that cerebral-palsied individuals perform significantly more poorly when the…

  14. Feasibility and test-retest reliability of measuring lower-limb strength in young children with cerebral palsy

    NARCIS (Netherlands)

    Van Vulpen, L. F.; de Groot, Sonja; Becher, J. G.; De Wolf, G. S.; Dallmeijer, A. J.

    2013-01-01

    BACKGROUND: Quantifying leg muscle strength in young children with cerebral palsy (CP) is essential for identifying muscle groups for treatment and for monitoring progress. AIM: To study the feasibility, intratester reliability and the optimal test design (number of test occasions and repetitions)

  15. Feasibility and test-retest reliability of measuring lower‑limb strength in young children with cerebral palsy

    NARCIS (Netherlands)

    van Vulpen, L. F.; de Groot, S.; Becher, J. G.; de Wolf, G. S.; Dallmeijer, A. J.

    2013-01-01

    Quantifying leg muscle strength in young children with cerebral palsy (CP) is essential for identifying muscle groups for treatment and for monitoring progress. To study the feasibility, intratester reliability and the optimal test design (number of test occasions and repetitions) of measuring

  16. Neuroevolutional Approach to Cerebral Palsy and Speech.

    Science.gov (United States)

    Mysak, Edward D.

    Intended for cerebral palsy specialists, the book emphasizes the contribution that a neuroevolutional approach to therapy can make to habilitation goals of the child with cerebral palsy and applies the basic principles of the Bobath approach to therapy. The first section discusses cerebral palsy as a reflection of disturbed neuro-ontogenisis and…

  17. Enhanced MRI in patients with facial palsy

    International Nuclear Information System (INIS)

    Yanagida, Masahiro; Kato, Tsutomu; Ushiro, Koichi; Kitajiri, Masanori; Yamashita, Toshio; Kumazawa, Tadami; Tanaka, Yoshimasa

    1991-01-01

    We performed Gd-DTPA-enhanced magnetic resonance imaging (MRI) examinations at several stages in 40 patients with peripheral facial nerve palsy (Bell's palsy and Ramsay-Hunt syndrome). In 38 of the 40 patients, one and more enhanced region could be seen in certain portion of the facial nerve in the temporal bone on the affected side, whereas no enhanced regions were seen on the intact side. Correlations between the timing of the MRI examination and the location of the enhanced regions were analysed. In all 6 patients examined by MRI within 5 days after the onset of facial nerve palsy, enhanced regions were present in the meatal portion. In 3 of the 8 patients (38%) examined by MRI 6 to 10 days after the onset of facial palsy, enhanced areas were seen in both the meatal and labyrinthine portions. In 8 of the 9 patients (89%) tested 11 to 20 days after the onset of palsy, the vertical portion was enhanced. In the 12 patients examined by MRI 21 to 40 days after the onset of facial nerve palsy, the meatal portion was not enhanced while the labyrinthine portion, the horizontal portion and the vertical portion were enhanced in 5 (42%), 8 (67%) and 11 (92%), respectively. Enhancement in the vertical portion was observed in all 5 patients examined more than 41 days after the onset of facial palsy. These results suggest that the central portion of the facial nerve in the temporal bone tends to be enhanced in the early stage of facial nerve palsy, while the peripheral portion is enhanced in the late stage. These changes of Gd-DTPA enhanced regions in the facial nerve may suggest dromic degeneration of the facial nerve in peripheral facial nerve palsy. (author)

  18. Frequency of joined disabilities of children with cerebral palsy in Tuzla canton

    Directory of Open Access Journals (Sweden)

    Mirela Babajić

    2013-12-01

    Full Text Available Introduction: Cerebral palsy (CP connotes a group of non-progressive, but often variable symptoms of motor impairment of movement and posture, as well as other impairments which are a consequenceof anomalies or brain impairment in different phases of its development. CP is a pathological condition characterised in the fi rst place by motor function impairment to which other disorders such as: visual andhearing impairment, intellectual defi cit, emotional problems, behaviour disorder, speech disorder, epileptic seizure and similar can join. The aim of this study is to determine frequency of joined disabilities ofchildren with cerebral palsy in Tuzla Canton.Methods: The research covers a total sample of 48 examinees, chronological age from 2-19 years, in Tuzla Canton. Research instrument was a Structural Questionnaire for the parents of children and adolescentswith cerebral palsy. Research data were processed by nonparametric statistics method. Basic statistical parameters of frequency and percentages were calculated, and tabular presentation was made.Results: After classification of examinees as per frequency of joined disabilities was done, work results have shown that speech impairment occurred with 35.4 % of children, visual impairment 33.3 %, epilepsy29.3 %, whereas hearing impairment occurred with 2 % of children.Conclusion: In research of frequency of joined disabilities of children with cerebral palsy in Tuzla Canton, most expressed are speech and visual disorders with children, then epilepsy, whereas a small percentageof children are with hearing disorder.

  19. Holmium laser vs. conventional (cold knife) direct visual internal urethrotomy for short-segment bulbar urethral stricture: Outcome analysis.

    Science.gov (United States)

    Jhanwar, Ankur; Kumar, Manoj; Sankhwar, Satya Narayan; Prakash, Gaurav

    2016-01-01

    Our goal was to analyze the outcome between holmium laser and cold knife direct visual internal urethrotomy (DVIU) for short-segment bulbar urethral stricture. We conducted a prospective study comprised of 112 male patients seen from June 2013 to December 2014. Inclusion criterion was short-segment bulbar urethral stricture (≤1.5cm). Exclusion criteria were prior intervention/urethroplasty, pan-anterior urethral strictures, posterior stenosis, urinary tract infection, and those who lost to followup. Patients were divided into two groups; Group A (n=58) included cold knife DVIU and group B (n=54) included holmium laser endourethrotomy patients. Patient followup included uroflowmetry at postoperative Day 3, as well as at three months and six months. Baseline demographics were comparable in both groups. A total of 107 patients met the inclusion criteria and five patients were excluded due to inadequate followup. Mean stricture length was 1.31 ± 0.252 cm (p=0.53) and 1.34 ± 0.251 cm in Groups A and B, respectively. Mean operating time in Group A was 16.3 ± 1.78 min and in Group B was 20.96 ± 2.23 min (p=0.0001). Five patients in Group A had bleeding after the procedure that was managed conservatively by applying perineal compression. Three patients in Group B had fluid extravasation postoperatively. Qmax (ml/s) was found to be statistically insignificant between the two groups at all followups. Both holmium laser and cold knife urethrotomy are safe and equally effective in treating short-segment bulbar urethral strictures in terms of outcome and complication rate. However, holmium laser requires more expertise and is a costly alternative.

  20. [Case of surgical treatment for giant hemangioblastoma in the dorsal medulla oblongata].

    Science.gov (United States)

    Kamoshima, Yuuta; Terasaka, Shunsuke; Shimoda, Yusuke; Kobayashi, Hiroyuki; Kuroda, Satoshi; Asano, Takeshi; Yamaguchi, Shigeru; Murata, Junichi; Houkin, Kiyohiro

    2012-03-01

    Hemangioblastoma in the medulla oblongata is a relatively rare tumor. We present the case of a giant hemangioblastoma occurring in the dorsal medulla oblongata. A 33-year-old man with no neurological symptoms was diagnosed with a hemangioblastoma in the dorsal medulla oblongata, and opted for observation in the outpatient department. After 22 months of observation time, MRI scans showed rapid local tumor progression and obstructive hydrocephalus. At this point, he presented with mild dysphagia as a preoperative neurological deficit. Total surgical removal of the tumor was performed after temporary ventricle drainage and preoperative embolization of the feeding artery. Postoperatively, he became fully conscious but developed bulbar palsy followed by tracheostomy. During the 12 months of postoperative follow-up, severe dysphagia was still present.

  1. DIABETES MELLITUS AND BELL’S PALSY IN IRANIAN POPULATION

    Directory of Open Access Journals (Sweden)

    A. R Karimi-Yazdi

    2008-08-01

    Full Text Available "nDuring last decades many researchers have focused on the conditions associated with Bell's palsy including diabetes mellitus, hypertension, and viral infections. This study was performed to evaluate correlation of diabetes mellitus and Bell's palsy and some relevant features not discussed in the literature in an Iranian population. The presence of diabetes mellitus was evaluated in a total number of 275 subjects (75 patients with Bell's palsy and 200 control subjects. Diabetes mellitus was noted in 10 (13.3% patients with Bell's palsy among which 6 case were diagnosed as new cases of diabetes. Previous history of Bell's palsy was present in 10.67% of the subjects with Bell's palsy. Symptoms of other cranial nerves revealed higher figures in Bell's palsy patients with underlying diabetes. Such studies in developing countries may reveal some unknown features of the disease. This study confirms the correlation of diabetes mellitus and Bell's palsy for the first time in an Iranian population. The results also suggest that diabetic patients with Bell's palsy suffer from more cranial nerve symptoms. We offer screening tests of diabetes as a routine process in the management of Bell's palsy especially in developing countries.

  2. Monocular Elevation Deficiency - Double Elevator Palsy

    Science.gov (United States)

    ... Español Condiciones Chinese Conditions Monocular Elevation Deficiency/ Double Elevator Palsy En Español Read in Chinese What is monocular elevation deficiency (Double Elevator Palsy)? Monocular Elevation Deficiency, also known by the ...

  3. Delayed facial nerve decompression for Bell's palsy.

    Science.gov (United States)

    Kim, Sang Hoon; Jung, Junyang; Lee, Jong Ha; Byun, Jae Yong; Park, Moon Suh; Yeo, Seung Geun

    2016-07-01

    Incomplete recovery of facial motor function continues to be long-term sequelae in some patients with Bell's palsy. The purpose of this study was to investigate the efficacy of transmastoid facial nerve decompression after steroid and antiviral treatment in patients with late stage Bell's palsy. Twelve patients underwent surgical decompression for Bell's palsy 21-70 days after onset, whereas 22 patients were followed up after steroid and antiviral therapy without decompression. Surgical criteria included greater than 90 % degeneration on electroneuronography and no voluntary electromyography potentials. This study was a retrospective study of electrodiagnostic data and medical chart review between 2006 and 2013. Recovery from facial palsy was assessed using the House-Brackmann grading system. Final recovery rate did not differ significantly in the two groups; however, all patients in the decompression group recovered to at least House-Brackmann grade III at final follow-up. Although postoperative hearing threshold was increased in both groups, there was no significant between group difference in hearing threshold. Transmastoid decompression of the facial nerve in patients with severe late stage Bell's palsy at risk for a poor facial nerve outcome reduced severe complications of facial palsy with minimal morbidity.

  4. Divergence Palsy due to Divalproex and Oxcarbazepine.

    Science.gov (United States)

    Bouffard, Marc Albert; Caplan, Louis R; Torun, Nurhan

    This case series is the first to describe divergence palsy as an adverse effect of antiepileptic drug use. Diplopia is a common adverse effect of antiepileptic drugs, but no explanatory motility deficit has ever been reported. We present 2 patients, 1 on oxcarbazepine and 1 on divalproex, each with a normal examination result between spells and divergency palsy when symptomatic. Discontinuation of the antiepileptic medication led to resolution of the episodes in both cases. Rechallenge with the offending agent after washout in one patient resulted in recurrence of diplopia and divergence palsy, both resolving after subsequent withdrawal of the antiepileptic. Antiepileptic drugs may cause divergence palsy.

  5. Postural Stabilization Differences in Idiopathic Parkinson’s Disease and Progressive Supranuclear Palsy during Self-Triggered Fast Forward Weight Lifting

    Directory of Open Access Journals (Sweden)

    Stefan Kammermeier

    2018-01-01

    Full Text Available Progressive supranuclear palsy (PSP and late-stage idiopathic Parkinson’s disease (IPD are neurodegenerative movement disorders resulting in different postural instability and falling symptoms. IPD falls occur usually forward in late stage, whereas PSP falls happen in early stages, mostly backward, unprovoked, and with high morbidity. Self-triggered, weighted movements appear to provoke falls in IPD, but not in PSP. Repeated self-triggered lifting of a 0.5–1-kg weight (<2% of body weight with the dominant hand was performed in 17 PSP, 15 IPD with falling history, and 16 controls on a posturography platform. PSP showed excessive force scaling of weight and body motion with high-frequency multiaxial body sway, whereas IPD presented a delayed-onset forward body displacement. Differences in center of mass displacement apparent at very small weights indicate that both syndromes decompensate postural control already within stability limits. PSP may be subject to specific postural system devolution. IPD are susceptible to delayed forward falling. Differential physiotherapy strategies are suggested.

  6. Clinical studies on Bell's palsy

    International Nuclear Information System (INIS)

    Yamada, Yoshio

    1985-01-01

    The purpose of this study was to evaluate the diagnostic value of the salivary gland scintigraphy using sup(99m)Tc-pertechnetate for the prognosis of Bell's palsy. The salivary gland scintigraphy was performed in 40 patients with Bell's palsy and 15 normal subjects. After intravenous injection of 10 mCt of sup(99m)Tc-pertechnetate, sequential scintigrams were taken with a scintillation camera every one minute for 25 minutes. At 15 minutes after injection, both of normal subjects and patients were given ascorbic acid to stimulate the secretion of saliva. By the present method, the time activity curve was examined for the regions of interest over the parotid and submandibular glands and backgrounds. In normal subjects, values of the concentration and excretory ratio between the right and left sides of the parotid and submandibular glands were more than 80%. Some patients with Bell's palsy showed a decreased concentration and/or excretory ratio less than 80% between the normal and affected sides of the parotid glands. This suggests a functional involvement of the facial nerve in the salivary secretion from the parotid glands. On examination within 10 days of the onset of Bell's palsy, 31 cases with complete recovery showed values of the concentration ratio and/or excretory ratio more than 80% between the normal and affected sides of the submandibular glands. In contrast, 9 cases with imcomplete recovery showed low values of the concentration ratio and excretory ratio less than 80%. In the latter, more active treatments such as decompression operation should be considerd in the early stage of the palsy. The salivary scintigraphy using sup(99m)Tc-pertechnetate is more valuable as an early prognostic indicator for Bell's palsy compared with other prognostic tests such as the lid vibration test, the stapedial reflex test, the electrogustometry, the nerve excitability test and the evoked electromyography. (author)

  7. The early markers for later dyskinetic cerebral palsy are different from those for spastic cerebral palsy

    NARCIS (Netherlands)

    Einspieler, C; Cioni, G; Paolicelli, PB; Bos, AF; Dressler, A; Ferrari, F; Roversi, MF; Prechtl, HFR

    Qualitative abnormalities of spontaneous motor activity in new-borns and young infants are early predictive markers for later spastic cerebral palsy. Aim of this research was to identify which motor patterns may be specific for later dyskinetic cerebral palsy. In a large, prospectively performed

  8. Pattern of facial palsy in a typical Nigerian specialist hospital.

    Science.gov (United States)

    Lamina, S; Hanif, S

    2012-12-01

    Data on incidence of facial palsy is generally lacking in Nigeria. To assess six years' incidence of facial palsy in Murtala Muhammed Specialist Hospital (MMSH), Kano, Nigeria. The records of patients diagnosed as facial problems between January 2000 and December 2005 were scrutinized. Data on diagnosis, age, sex, side affected, occupation and causes were obtained. A total number of 698 patients with facial problems were recorded. Five hundred and ninety four (85%) were diagnosed as facial palsy. Out of the diagnosed facial palsy, males (56.2%) had a higher incidence than females; 20-34 years age group (40.3%) had a greater prevalence; the commonest cause of facial palsy was found out to be Idiopathic (39.1%) and was most common among business men (31.6%). Right sided facial palsy (52.2%) was predominant. Incidence of facial palsy was highest in 2003 (25.3%) and decreased from 2004. It was concluded that the incidence of facial palsy was high and Bell's palsy remains the most common causes of facial (nerve) paralysis.

  9. Validation of a Cerebral Palsy Register

    DEFF Research Database (Denmark)

    Topp, Monica; Langhoff-Roos, Jens; Uldall, P.

    1997-01-01

    OBJECTIVES: To analyse completeness and validity of data in the Cerebral Palsy Register in Denmark, 1979-1982. METHODS: Completeness has been assessed by comparing data from The Danish National Patient Register (DNPR) with the cases included in the Cerebral Palsy Register (CPR). Agreement between......, but gestational age was subject to a systematic error, and urinary infections in pregnancy (kappa = 0.43) and placental abruption (kappa = 0.52) were seriously under-reported in the CPR. CONCLUSIONS: Completeness of the Cerebral Palsy Register in Denmark, 1979-1982, has been assessed to maximal 85%, emphasizing...

  10. An evaluation of the sling surgical method of the bulbar urethra in the treatment of men's stress urinary incontinence at Shohadaye Ashayer Teaching Hospital in 2008.

    Science.gov (United States)

    Heidari, Mohammad; Khorramabadi, Manoochehr Shams

    2012-11-01

    To evaluate the utility and efficacy of bulbar urethera sling in the management of sphincter insufficiency that usually occurs after prostate surgery or posterior urethral injuries and may lead to moderate to severe stress incontinence. A total of 30 patients underwent sling surgery with rectus fascia in a four-year period at the Shohadaye Ashayer Teaching Hospital in Iran. Urinary incontinence occurred in 8 patients after open prostatectomy, in 12 patients after prostatectomy through urethra, in 8 patients after radical prostatectomy. For the purpose of the study, 2 patients in whom incontinence occurred after pelvic fracture were excluded. The 28 patients were followed up for a one-year period after the operation. All patients had incontinence from one to six years. After hospitalisation, an 18 gauge Foley's catheter was introduced in the urethra in every patient. The perineum was incised longitudinally, and the bulbar urethra was freed and a 2x7 cm span of rectus fascia was separated and placed under the bulbar urethra. Treatment was defined as use of one or no pad per day and recovery, as a reduction of at least 50% in the number of the used pads after sling operation. After operation, all patients suffering from moderate to severe stress incontinence were treated with 0-1 pad per day. Four patients were unable to urinate; in 2 patients the sling was modified and loosened, and in two others dilatation resolved their problems. Bulbar urethra sling can be carried out in moderate to severe urinary incontinence treatment in any hospital at a modest cost with satisfactory results.

  11. Alternative promoter usage generates novel shorter MAPT mRNA transcripts in Alzheimer's disease and progressive supranuclear palsy brains.

    Science.gov (United States)

    Huin, Vincent; Buée, Luc; Behal, Hélène; Labreuche, Julien; Sablonnière, Bernard; Dhaenens, Claire-Marie

    2017-10-03

    Alternative promoter usage is an important mechanism for transcriptome diversity and the regulation of gene expression. Indeed, this alternative usage may influence tissue/subcellular specificity, protein translation and function of the proteins. The existence of an alternative promoter for MAPT gene was considered for a long time to explain differential tissue specificity and differential response to transcription and growth factors between mRNA transcripts. The alternative promoter usage could explain partly the different tau proteins expression patterns observed in tauopathies. Here, we report on our discovery of a functional alternative promoter for MAPT, located upstream of the gene's second exon (exon 1). By analyzing genome databases and brain tissue from control individuals and patients with Alzheimer's disease or progressive supranuclear palsy, we identified novel shorter transcripts derived from this alternative promoter. These transcripts are increased in patients' brain tissue as assessed by 5'RACE-PCR and qPCR. We suggest that these new MAPT isoforms can be translated into normal or amino-terminal-truncated tau proteins. We further suggest that activation of MAPT's alternative promoter under pathological conditions leads to the production of truncated proteins, changes in protein localization and function, and thus neurodegeneration.

  12. Comprehensive visual impairment evaluation for cerebral palsy children

    Directory of Open Access Journals (Sweden)

    Ping Wang

    2015-01-01

    Full Text Available AIM: To evaluate the visual impairment in cerebral palsy children with series objective indicators, and conclude their clinical features of visual function.METHODS: Objective tests including following pursuing test, optokinetic nystagmus(OKNdrum test, refractive error examination, fundus examination, ocular deviation examination, pattern visual evoked potential(P-VEPtests and brain magnetic resonance imaging(MRIwere carried out in 43 cerebral palsy children(86 eyeswith ocular visual dysfunction; The visual impairment data of the cerebral palsy children were collected, and the clinical features and possible mechanism were analyzed.RESULTS: 1. Of the 43 cerebral palsy children(86 eyeswith the visual impairment presented diversified, 25(50 eyes, 58.1%of refractive error, 24(48 eyes, 55.8%of strabismus, 12(24 eyes, 27.9%with nystagmus, 19(38 eyes, 44.2%of optical nerve atrophy or hyperplasia, 35(70 eyes, 81.4%of VEP abnormality. Among children with spastic cerebral palsy, the incidence of visual impairment was statistically significant difference compared with other groups(PP>0.05, no nystagmus in patients with severe occipital cortex damage.CONCLUSION: Cerebral palsy children were usually with visual impairment, and presented with special clinical features; Comprehensive objective visual tests are accurate and reliable for evaluation of the visual function in cerebral palsy children.

  13. Synergic prodegradative activity of Bicalutamide and trehalose on the mutant androgen receptor responsible for spinal and bulbar muscular atrophy

    NARCIS (Netherlands)

    Giorgetti, Elise; Rusmini, Paola; Crippa, Valeria; Cristofani, Riccardo; Boncoraglio, Alessandra; Cicardi, Maria E.; Galbiati, Mariarita; Poletti, Angelo

    2015-01-01

    Spinal and bulbar muscular atrophy (SBMA) is an X-linked motoneuron disease due to a CAG triplet-repeat expansion in the androgen receptor (AR) gene, which is translated into an elongated polyglutamine (polyQ) tract in AR protein (ARpolyQ). ARpolyQ toxicity is activated by the AR ligand testosterone

  14. Magnetisation transfer measurements of the subcortical grey and white matter in Parkinson's disease with and without dementia and in progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Hanyu, H.; Asano, T.; Sakurai, H.; Takasaki, M.; Shindo, H.; Abe, K.

    2001-01-01

    We measured the magnetisation transfer ratio (MTR) in the subcortical grey and white matter of 11 patients with idiopathic Parkinson's disease (PD) without dementia, six with PD with dementia (PDD), six with progressive supranuclear palsy (PSP), and 12 elderly control subjects to assess regional differences in structural brain damage. There were no significant differences in MTR in any region between PD and controls. However, patients with PDD had significantly lower MTR in the subcortical white matter, including the frontal white matter and the genu of the corpus callosum than the controls, whereas PSP had significantly lower MTR in the subcortical grey matter, including the putamen, globus pallidus and thalamus, in addition to the subcortical white matter. This suggests that regional patterns of structural brain damage can be detected using the magnetisation transfer technique. Measurement of MTR in the subcortical grey and white matter may be useful in differential diagnosis. (orig.)

  15. Bell's Palsy

    Science.gov (United States)

    ... have been linked to Bell's palsy include the virus that causes: Cold sores and genital herpes (herpes simplex) Chickenpox and shingles (herpes zoster) Infectious mononucleosis (Epstein-Barr) Cytomegalovirus infections Respiratory illnesses (adenovirus) German measles (rubella) ...

  16. Bell's Palsy

    Science.gov (United States)

    ... or paralysis. Bell's palsy is named for Sir Charles Bell, a 19th century Scottish surgeon who described ... confirm diagnosis of the disorder. Generally, a physician will examine the individual for upper and lower facial ...

  17. Case report of a patient with peripheral facial nerve palsy

    OpenAIRE

    Rysová, Jana

    2013-01-01

    Title of bachelor's thesis: Case report of a patient with peripheral facial nerve palsy Summary: Teoretical part of bachelor's thesis contains theoretical foundation of peripheral facial nerve palsy. Practical part of bachelor's thesis contains physiotherapeutic case report of patient with peripheral facial nerve palsy. Key words: peripheral facial nerve palsy, casuistry, rehabilitation

  18. Plasma Fibrinogen in Patients With Bell Palsy.

    Science.gov (United States)

    Zhao, Hua; Zhang, Xin; Tang, Yinda; Li, Shiting

    2016-10-01

    To determine the plasma fibrinogen level in patients with Bell palsy and explore the significances of it in Bell palsy. One hundred five consecutive patients with facial paralysis were divided into 3 groups: group I (Bell palsy), group II (temporal bone fractures), and group III (facial nerve schwannoma). In addition, 22 volunteers were defined as control group. Two milliliters fasting venous blood from elbow was collected, and was evaluated by CA-7000 Full-Automatic Coagulation Analyzer. The plasma fibrinogen concentration was significantly higher in the group of patients with Bell palsy (HB IV-VI) than that in the control group (P 0.05); similarly, there was also no marked difference between group III and control group (P >0.05). In group I, the plasma fibrinogen levels became higher with the HB grading increase. The plasma fibrinogen level of HB-VI was highest. Plasma fibrinogen has an important clinical meaning in Bell palsy, which should be used as routine examination items. Defibrinogen in treatment for patients with high plasma fibrinogen content also should be suggested.

  19. Autopsy case of undiagnosed gangliocytoma in the medulla oblongata complicated with cerebral palsy.

    Science.gov (United States)

    Takahashi, Motonori; Kondo, Takeshi; Morichika, Mai; Kuse, Azumi; Nakagawa, Kanako; Asano, Migiwa; Ueno, Yasuhiro

    2016-03-01

    A Japanese man in his 30s who had congenital cerebral palsy was found unresponsive in bed. His death was confirmed after resuscitation attempts. He had a history of occasional falling (despite the use of walking sticks and a wheelchair) owing to a slowly progressive gait disturbance, and had a medical examination without full neurological re-examination. Autopsy revealed gangliocytoma in the medulla oblongata, which was diagnosed as the cause of death. Although gangliocytoma is a well-differentiated benign tumor, the almost total replacement of the medulla oblongata by the tumor cells was assumed to result in ataxia via the olivocerebellar tract and secondary cerebellar atrophy, followed by central hypoventilation and death of the patient. The symptoms caused by gangliocytoma may be overlooked owing to long-standing cerebral palsy. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  20. A Novel Mobility Device to Improve Walking for a Child With Cerebral Palsy.

    Science.gov (United States)

    Fergus, Andrea

    2017-10-01

    To describe the use and outcomes associated with the Upsee in conjunction with Kinesiotape for a child with cerebral palsy. The Upsee and Kinesiotaping were implemented for 24 weeks with a 31-month-old child with cerebral palsy, Gross Motor Function Classification System level III. She progressed from walking with maximal assistance and extensive gait deviations to walking with supervision with a walker on level surfaces with improved gait. Genu recurvatum, heel strike, scissoring, hip extension, foot placement, step length, and stiff knee in swing improved on the basis of videotaped analyses. The Gross Motor Function Measure-66 improved by 11.4. The Upsee is a clinically feasible approach for gait impairments in children through providing increased opportunities for walking while supporting biomechanical alignment. Upsee effectiveness with and without taping is an area for future study.

  1. Comparative efficacy of progressive resistance exercise and ...

    African Journals Online (AJOL)

    Background: Progressive Resistance Exercise (PRE) and Biomechanical Ankle Platform System (BAPS) are two of the protocols available in managing children with Cerebral Palsy (CP). The comparative effects of these modalities on selected functional indices of ambulatory type CP were the focus of this study. Methods: ...

  2. 11 Things to Know about Cerebral Palsy

    Science.gov (United States)

    ... Button Past Emails 11 Things to Know about Cerebral Palsy Language: English (US) Español (Spanish) Recommend on Facebook Tweet Share Compartir Cerebral palsy (CP) is the most common motor disability in ...

  3. [Rapidly progressive compromise of cranial pairs as neurosyphilis manifestation].

    Science.gov (United States)

    Baccaro, Fernando; Moldes, Sofía; Novelli Poisson, Paola; Arduin, Julieta; Valerga, Mario

    2012-01-01

    Syphilis remains a common disease throughout the world, being neurosyphilis a relatively common manifestation. A case of a 34 years old male with HIV and neurosyphilis is presented, characterized by a clinical course evidenced by progressive palsy of cranial nerves. This case is unusual and a rare presentation of progressive cranial involvement with swallowing deficit, have found no similar data in the literature.

  4. Effects of Robot Assisted Gait Training in Progressive Supranuclear Palsy (PSP: a preliminary report.

    Directory of Open Access Journals (Sweden)

    Patrizio eSale

    2014-04-01

    Full Text Available Background and Purpose: Progressive supranuclear palsy (PSP is a rare neurodegenerative disease clinically characterized by prominent axial extrapyramidal motor symptoms with frequent falls. Over the last years the introduction of robotic technologies to recover lower limb function has been greatly employed in the rehabilitative practice. This observational trial is aimed at investigating the feasibility, the effectiveness and the efficacy of end-effector robot training in people with PSP.Method: Pilot observational trial.Participants: Five cognitively intact participants with PSP and gait disorders.Interventions: Patients were submitted to a rehabilitative program of robot-assisted walking sessions for 45 minutes, 5 times a week for 4 weeks.Main outcome measures: The spatiotemporal parameters at the beginning (T0 and at the end of treatment (T1 were recorded by a gait analysis laboratory.Results: Robot training was feasible, acceptable and safe and all participants completed the prescribed training sessions. All patients showed an improvement in the gait index (Mean velocity, Cadence, Step length and Step width (T0 versus T1.Conclusions: Robot training is a feasible and safe form of rehabilitation for cognitively intact people with PSP. This innovative approach can contribute to improve lower limb motor recovery. The focus on gait recovery is another quality that makes this research important for clinical practice. On the whole, the simplicity of treatment, the lack of side effects and the positive results in the patients support the recommendation to extend the trials of this treatment. Further investigation regarding the effectiveness of robot training in time is necessary.Trial registration: ClinicalTrials.gov NCT01668407.

  5. Simultaneous Bilateral Bell's palsy in a Nigerian man | Owolabi ...

    African Journals Online (AJOL)

    Bilateral Bellfs palsy occurring simultaneously is exceedingly rare. We report a case of a 27.year.old Nigerian man with bilateral Bellfs palsy, that occurred simultaneously, who had remarkable response to steroid and physiotherapy. We emphasized the importance of considering Bellfs palsy and the various differential ...

  6. The Big Bluff of Amyotrophic Lateral Sclerosis Diagnosis: The Role of Neurodegenerative Disease Mimics.

    Science.gov (United States)

    Bicchi, Ilaria; Emiliani, Carla; Vescovi, Angelo; Martino, Sabata

    2015-01-01

    Neurodegenerative diseases include a significant number of pathologies affecting the nervous system. Generally, the primary cause of each disease is specific; however, recently, it was shown that they may be correlated at molecular level. This aspect, together with the exhibition of similar symptoms, renders the diagnosis of these disorders difficult. Amyotrophic lateral sclerosis is one of these pathologies. Herein, we report several cases of amyotrophic lateral sclerosis misdiagnosed as a consequence of features that are common to several neurodegenerative diseases, such as Parkinson's, Huntington's and Alzheimer's disease, spinal muscular atrophy, progressive bulbar palsy, spastic paraplegia and frontotemporal dementia, and mostly with the lysosomal storage disorder GM2 gangliosidosis. Overall reports highlight that the differential diagnosis for amyotrophic lateral sclerosis should include correlated mechanisms. © 2015 S. Karger AG, Basel.

  7. Bell palsy in lyme disease-endemic regions of canada: a cautionary case of occult bilateral peripheral facial nerve palsy due to Lyme disease.

    Science.gov (United States)

    Ho, Karen; Melanson, Michel; Desai, Jamsheed A

    2012-09-01

    Lyme disease caused by the spirochete Borrelia burgdorferi is a multisystem disorder characterized by three clinical stages: dermatologic, neurologic, and rheumatologic. The number of known Lyme disease-endemic areas in Canada is increasing as the range of the vector Ixodes scapularis expands into the eastern and central provinces. Southern Ontario, Nova Scotia, southern Manitoba, New Brunswick, and southern Quebec are now considered Lyme disease-endemic regions in Canada. The use of field surveillance to map risk and endemic regions suggests that these geographic areas are growing, in part due to the effects of climate warming. Peripheral facial nerve palsy is the most common neurologic abnormality in the second stage of Lyme borreliosis, with up to 25% of Bell palsy (idiopathic peripheral facial nerve palsy) occurring due to Lyme disease. Here we present a case of occult bilateral facial nerve palsy due to Lyme disease initially diagnosed as Bell palsy. In Lyme disease-endemic regions of Canada, patients presenting with unilateral or bilateral peripheral facial nerve palsy should be evaluated for Lyme disease with serologic testing to avoid misdiagnosis. Serologic testing should not delay initiation of appropriate treatment for presumed Bell palsy.

  8. Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy

    NARCIS (Netherlands)

    Bailey, CK; Andriola, IFM; Kampinga, HH; Merry, DE

    2002-01-01

    Spinal and bulbar muscular atrophy (SBMA) is one of a growing number of neurodegenerative diseases caused by a polyglutamine-encoding CAG trinucleotide repeat expansion, and is caused by an expansion within exon 1 of the androgen receptor (AR) gene. The family of polyglutamine diseases is

  9. Immediate Postoperative Bell's Palsy: Viral Etiology or Post-Traumatic Phenomena?

    Directory of Open Access Journals (Sweden)

    Mohammad Ghasem Shams

    2010-12-01

    Full Text Available Introduction: Bell’s palsy is a sudden unilateral paralysis of the facial nerve. Postoperative Bell’s palsy following surgery is rare. It occurs in less than 1% of operations. The hypothesis: We premise that the main cause of immediate postoperative Bell's palsy is latent herpes viruses (herpes simplex virus type 1 and herpes zoster virus, which are reactivated from cranial nerve ganglia. Inflammation of the nerve initially results in a reversible neurapraxia, but ultimately Wallerian degeneration ensues. The palsy is often sudden in onset and evolves rapidly, with maximal facial weakness developing within two days. Associated symptoms of-ten seen in idiopathic Bell’s palsy are tearing problems, hyperacusis and altered taste.Evaluation of the hypothesis: Facial paralysis presenting postoperatively is distressing and poses a diagnostic chal-lenge. A complete interruption of the facial nerve at the sty-lomastoid foramen paralyzes all the muscles of facial expression. Taste sensation may be lost unilaterally and hye-racusis may be present. Idiopathic Bell’s palsy is due to inflammation of the facial nerve in the facial canal. Bell’s palsy may also occur from lesions that invade the temporal bone (carotid body, cholesteatoma, dermoid cyst, acoustic neu-romas. Although traumatic Bell’s palsy cannot be ruled out, it seems logic to postulate that the main cause of immediate postoperative Bell's palsy is latent herpes viruses.

  10. Association of Lead Levels and Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Neha Bansal MD

    2017-03-01

    Full Text Available Background: Cerebral palsy is a common motor disability in childhood. Raised lead levels affect cognition. Children with cerebral palsy may have raised lead levels, further impairing their residual cognitive motor and behavioral abilities. Environmental exposure and abnormal eating habits may lead to increased lead levels. Aims and Objectives: To measure blood lead levels in children with cerebral palsy and compare them with healthy neurologically normal children. To correlate blood lead levels with environmental factors. Material and Methods: Design: Prospective case-control study. Setting: Tertiary care hospital. Participants: Cases comprised 34 children with cerebral palsy, and controls comprised 34 neurologically normal, age- and sex-matched children. Methods: Clinical and demographic details were recorded as per proforma. Detailed environmental history was recorded to know the source of exposure to lead. These children were investigated and treated as per protocol. Venous blood was collected in ethylenediaminetetraacetic acid vials for analysis of blood lead levels. Lead levels were estimated by Schimadzu Flame AA-6800 (atomic absorption spectrophotometer. Data were analyzed using SPSS version 17. P < .05 was taken as significant. Results: Mean blood lead levels were 9.20 ± 8.31 µg/dL in cerebral palsy cases and 2.89 ± 3.04 µg/dL in their controls (P < .001. Among children with cerebral palsy, 19 (55.88% children had blood lead levels ≥5 µg/dL. Lead levels in children with pica were 12.33 ± 10.02 µg/dL in comparison to children with no history of pica, 6.70 ± 4.60 µg/dL (P = .029. No correlation was found between hemoglobin and blood lead levels in cases and controls. Conclusion: In our study, blood lead levels are raised in children with cerebral palsy. However, further studies are required to show effects of raised levels in these children.

  11. The Danish Cerebral Palsy Follow-up Program

    DEFF Research Database (Denmark)

    Rasmussen, Helle Mätzke; Nordbye-Nielsen, Kirsten; Møller-Madsen, Bjarne

    2016-01-01

    AIM OF DATABASE: The Danish Cerebral Palsy Follow-up Program is a combined follow-up program and national clinical quality database that aims to monitor and improve the quality of health care for children with cerebral palsy (CP). STUDY POPULATION: The database includes children with CP aged 0...... indicators in three of five regions in Denmark comprising 432 children with CP, corresponding to a coverage of 82% of the expected population. CONCLUSION: The Danish Cerebral Palsy Follow-up Program is currently under development as a national clinical quality database in Denmark. The database holds...

  12. Racial, Ethnic, and Socioeconomic Disparities in the Prevalence of Cerebral Palsy

    Science.gov (United States)

    Xing, Guibo; Fuentes-Afflick, Elena; Danielson, Beate; Smith, Lloyd H.; Gilbert, William M.

    2011-01-01

    OBJECTIVE: Racial and ethnic disparities in cerebral palsy have been documented, but the underlying mechanism is poorly understood. We determined whether low birth weight accounts for ethnic disparities in the prevalence of cerebral palsy and whether socioeconomic factors impact cerebral palsy within racial and ethnic groups. METHODS: In a retrospective cohort of 6.2 million births in California between 1991 and 2001, we compared maternal and infant characteristics among 8397 infants with cerebral palsy who qualified for services from the California Department of Health Services and unaffected infants. RESULTS: Overall, black infants were 29% more likely to have cerebral palsy than white infants (relative risk: 1.29 [95% confidence interval: 1.19–1.39]). However, black infants who were very low or moderately low birth weight were 21% to 29% less likely to have cerebral palsy than white infants of comparable birth weight. After we adjusted for birth weight, there was no difference in the risk of cerebral palsy between black and white infants. In multivariate analyses, women of all ethnicities who did not receive any prenatal care were twice as likely to have infants with cerebral palsy relative to women with an early onset of prenatal care. Maternal education was associated with cerebral palsy in a dose-response fashion among white and Hispanic women. Hispanic adolescent mothers (aged cerebral palsy. CONCLUSIONS: The increased risk of cerebral palsy among black infants is primarily related to their higher risk of low birth weight. Understanding how educational attainment and use of prenatal care impact the risk of cerebral palsy may inform new prevention strategies. PMID:21339278

  13. An unusual cause of orthopnoea-hashimoto's thyroiditis presenting as bilateral diaphragmatic palsy

    Directory of Open Access Journals (Sweden)

    N.K. Thulaseedharan, MBBS, MD(General Medicine

    2017-01-01

    Full Text Available We report a case of 36 yr old male without any comorbidities, who presented with a history of gradually progressive dyspnoea and orthopnoea for 6 months. Physical examination revealed bradycardia, paradoxical respiration suggestive of bilateral diaphragmatic palsy. Fluoroscopy demonstrated the presence of bilateral diaphragmatic paralysis. Etiological work up showed evidence of autoimmune hypothyroidism due to hashimoto's thyroiditis. Other possibilities were ruled out with appropriate tests. He was started on thyroxine and showed symptomatic improvement.

  14. Beyond the midbrain atrophy: wide spectrum of structural MRI finding in cases of pathologically proven progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Sakurai, Keita; Tokumaru, Aya M.; Shimoji, Keigo; Murayama, Shigeo; Kanemaru, Kazutomi; Morimoto, Satoru; Aiba, Ikuko; Nakagawa, Motoo; Ozawa, Yoshiyuki; Shimohira, Masashi; Shibamoto, Yuta; Matsukawa, Noriyuki; Hashizume, Yoshio

    2017-01-01

    Recently, it has been recognized that pathologically proven progressive supranuclear palsy (PSP) cases are classified into various clinical subtypes with non-uniform symptoms and imaging findings. This article reviews essential imaging findings, general information, and advanced magnetic resonance imaging (MRI) techniques for PSP and presents these MRI findings of pathologically proven typical and atypical PSP cases for educational purposes. With the review of literatures, notably including atypical pathologically proven PSP cases, MRI and clinical information of 15 pathologically proven typical and atypical PSP cases were retrospectively evaluated. In addition to typical symptoms, PSP patients can exhibit atypical symptoms including levodopa-responsive parkinsonism, pure akinesia, non-fluent aphasia, corticobasal syndrome, and predominant cerebellar ataxia. As well as clinical symptoms, the degree of midbrain atrophy, a well-known imaging hallmark, is not consistent in atypical PSP cases. This fact has important implications for the limitation of midbrain atrophy as a diagnostic imaging biomarker of PSP pathology. Additional evaluation of other imaging findings including various regional atrophies of the globus pallidus, frontal lobe, cerebral peduncle, and superior cerebellar peduncle is essential for the diagnosis of atypical PSP cases. It is necessary for radiologists to recognize the wide clinical and radiological spectra of typical and atypical PSP cases. (orig.)

  15. Beyond the midbrain atrophy: wide spectrum of structural MRI finding in cases of pathologically proven progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

    Sakurai, Keita; Tokumaru, Aya M.; Shimoji, Keigo [Tokyo Metropolitan Medical Center of Gerontology, Department of Diagnostic Radiology, Tokyo (Japan); Murayama, Shigeo; Kanemaru, Kazutomi; Morimoto, Satoru [Tokyo Metropolitan Geriatric Hospital, Department of Neurology, Tokyo (Japan); Aiba, Ikuko [National Hospital Organization Higashi Nagoya National Hospital, Department of Neurology, Nagoya (Japan); Nakagawa, Motoo; Ozawa, Yoshiyuki; Shimohira, Masashi; Shibamoto, Yuta [Nagoya City University Graduate School of Medical Sciences, Department of Radiology, Nagoya (Japan); Matsukawa, Noriyuki [Nagoya City University Graduate School of Medical Sciences, Department of Neurology and Neuroscience, Nagoya (Japan); Hashizume, Yoshio [Fukushimura Hospital, Choju Medical Institute, Toyohashi (Japan)

    2017-05-15

    Recently, it has been recognized that pathologically proven progressive supranuclear palsy (PSP) cases are classified into various clinical subtypes with non-uniform symptoms and imaging findings. This article reviews essential imaging findings, general information, and advanced magnetic resonance imaging (MRI) techniques for PSP and presents these MRI findings of pathologically proven typical and atypical PSP cases for educational purposes. With the review of literatures, notably including atypical pathologically proven PSP cases, MRI and clinical information of 15 pathologically proven typical and atypical PSP cases were retrospectively evaluated. In addition to typical symptoms, PSP patients can exhibit atypical symptoms including levodopa-responsive parkinsonism, pure akinesia, non-fluent aphasia, corticobasal syndrome, and predominant cerebellar ataxia. As well as clinical symptoms, the degree of midbrain atrophy, a well-known imaging hallmark, is not consistent in atypical PSP cases. This fact has important implications for the limitation of midbrain atrophy as a diagnostic imaging biomarker of PSP pathology. Additional evaluation of other imaging findings including various regional atrophies of the globus pallidus, frontal lobe, cerebral peduncle, and superior cerebellar peduncle is essential for the diagnosis of atypical PSP cases. It is necessary for radiologists to recognize the wide clinical and radiological spectra of typical and atypical PSP cases. (orig.)

  16. Automatic classification of patients with idiopathic Parkinson's disease and progressive supranuclear palsy using diffusion MRI datasets

    Science.gov (United States)

    Talai, Sahand; Boelmans, Kai; Sedlacik, Jan; Forkert, Nils D.

    2017-03-01

    Parkinsonian syndromes encompass a spectrum of neurodegenerative diseases, which can be classified into various subtypes. The differentiation of these subtypes is typically conducted based on clinical criteria. Due to the overlap of intra-syndrome symptoms, the accurate differential diagnosis based on clinical guidelines remains a challenge with failure rates up to 25%. The aim of this study is to present an image-based classification method of patients with Parkinson's disease (PD) and patients with progressive supranuclear palsy (PSP), an atypical variant of PD. Therefore, apparent diffusion coefficient (ADC) parameter maps were calculated based on diffusion-tensor magnetic resonance imaging (MRI) datasets. Mean ADC values were determined in 82 brain regions using an atlas-based approach. The extracted mean ADC values for each patient were then used as features for classification using a linear kernel support vector machine classifier. To increase the classification accuracy, a feature selection was performed, which resulted in the top 17 attributes to be used as the final input features. A leave-one-out cross validation based on 56 PD and 21 PSP subjects revealed that the proposed method is capable of differentiating PD and PSP patients with an accuracy of 94.8%. In conclusion, the classification of PD and PSP patients based on ADC features obtained from diffusion MRI datasets is a promising new approach for the differentiation of Parkinsonian syndromes in the broader context of decision support systems.

  17. The Efficacy of Bulbar Urethral Mobilization for Anastomotic Anterior Urethroplasty in a Case With Recurrent Anterior Urethral Stricture

    OpenAIRE

    Fukui, Shinji; Aoki, Katsuya; Kaneko, Yoshiteru; Samma, Shoji; Fujimoto, Kiyohide

    2014-01-01

    A 2-month-old boy was diagnosed with febrile urinary tract infection. Voiding cystourethrography showed bulbar and anterior urethral strictures, and endoscopic internal urethrotomy was performed. He developed febrile urinary tract infection again and revealed the recurrence of the anterior urethral stricture. Consequently, endoscopic internal urethrotomy was performed 4 times. Because the anterior urethral stricture had not improved, he was referred to us. Anterior urethroplasty was performed...

  18. Cortical thickness, surface area and volume measures in Parkinson's disease, multiple system atrophy and progressive supranuclear palsy.

    Directory of Open Access Journals (Sweden)

    Amanda Worker

    Full Text Available Parkinson's disease (PD, Multiple System Atrophy (MSA and Progressive Supranuclear Palsy (PSP are neurodegenerative diseases that can be difficult to distinguish clinically. The objective of the current study was to use surface-based analysis techniques to assess cortical thickness, surface area and grey matter volume to identify unique morphological patterns of cortical atrophy in PD, MSA and PSP and to relate these patterns of change to disease duration and clinical features.High resolution 3D T1-weighted MRI volumes were acquired from 14 PD patients, 18 MSA, 14 PSP and 19 healthy control participants. Cortical thickness, surface area and volume analyses were carried out using the automated surface-based analysis package FreeSurfer (version 5.1.0. Measures of disease severity and duration were assessed for correlation with cortical morphometric changes in each clinical group.Results show that in PSP, widespread cortical thinning and volume loss occurs within the frontal lobe, particularly the superior frontal gyrus. In addition, PSP patients also displayed increased surface area in the pericalcarine. In comparison, PD and MSA did not display significant changes in cortical morphology.These results demonstrate that patients with clinically established PSP exhibit distinct patterns of cortical atrophy, particularly affecting the frontal lobe. These results could be used in the future to develop a useful clinical application of MRI to distinguish PSP patients from PD and MSA patients.

  19. Olfaction and Pheromones: Uncanonical Sensory Influences and Bulbar Interactions

    Directory of Open Access Journals (Sweden)

    Víctor Vargas-Barroso

    2017-11-01

    Full Text Available The rodent main and accessory olfactory systems (AOS are considered functionally and anatomically segregated information-processing pathways. Each system is devoted to the detection of volatile odorants and pheromones, respectively. However, a growing number of evidences supports a cooperative interaction between them. For instance, at least four non-canonical receptor families (i.e., different from olfactory and vomeronasal receptor families have been recently discovered. These atypical receptor families are expressed in the sensory organs of the nasal cavity and furnish parallel processing-pathways that detect specific stimuli and mediate specific behaviors as well. Aside from the receptor and functional diversity of these sensory modalities, they converge into a poorly understood bulbar area at the intersection of the main- main olfactory bulb (MOB and accessory olfactory bulb (AOB that has been termed olfactory limbus (OL. Given the intimate association the OL with specialized glomeruli (i.e., necklace and modified glomeruli receiving uncanonical sensory afferences and its interactions with the MOB and AOB, the possibility that OL is a site of non-olfactory and atypical vomeronasal sensory decoding is discussed.

  20. Prevalence of cerebral palsy in Uganda: a population-based study.

    Science.gov (United States)

    Kakooza-Mwesige, Angelina; Andrews, Carin; Peterson, Stefan; Wabwire Mangen, Fred; Eliasson, Ann Christin; Forssberg, Hans

    2017-12-01

    Few population-based studies of cerebral palsy have been done in low-income and middle-income countries. We aimed to examine cerebral palsy prevalence and subtypes, functional impairments, and presumed time of injury in children in Uganda. In this population-based study, we used a nested, three-stage, cross-sectional method (Iganga-Mayuge Health and Demographic Surveillance System [HDSS]) to screen for cerebral palsy in children aged 2-17 years in a rural eastern Uganda district. A specialist team confirmed the diagnosis and determined the subtype, motor function (according to the Gross Motor Function Classification System [GMFCS]), and possible time of brain injury for each child. Triangulation and interviews with key village informants were used to identify additional cases of suspected cerebral palsy. We estimated crude and adjusted cerebral palsy prevalence. We did χ 2 analyses to examine differences between the group screened at stage 1 and the entire population and regression analyses to investigate associations between the number of cases and age, GMFCS level, subtype, and time of injury. We used data from the March 1, 2015, to June 30, 2015, surveillance round of the Iganga-Mayuge HDSS. 31 756 children were screened for cerebral palsy, which was confirmed in 86 (19%) of 442 children who screened positive in the first screening stage. The crude cerebral palsy prevalence was 2·7 (95% CI 2·2-3·3) per 1000 children, and prevalence increased to 2·9 (2·4-3·6) per 1000 children after adjustment for attrition. The prevalence was lower in older (8-17 years) than in younger (cerebral palsy was the most common subtype (45 [46%] of 97 children) followed by bilateral cerebral palsy (39 [40%] of 97 children). 14 (27%) of 51 children aged 2-7 years had severe cerebral palsy (GMFCS levels 4-5) compared with only five (12%) of 42 children aged 8-17 years. Few children (two [2%] of 97) diagnosed with cerebral palsy were born preterm. Post-neonatal events were the

  1. Isolated abducens nerve palsy with hyperhomocysteinemia: Association and outcomes

    Directory of Open Access Journals (Sweden)

    Virender Sachdeva

    2013-01-01

    Full Text Available Ischemic abducens nerve palsy usually presents as isolated cranial nerve palsy in the middle aged and elderly patients with known risk factors such as diabetes mellitus, hypertension, dyslipidemia, carotid artery disease, etc., In this report, we describe four patients with isolated abducens nerve palsy who presented with an acute onset diplopia whose detailed history and examination were suggestive of an ischemic etiology. Detailed systemic and laboratory evaluation revealed hyperhomocysteinemia as the only potential risk factor. To the best of our knowledge this is the first report of association of hyperhomocysteinemia and isolated abducens nerve palsy.

  2. Magnetic resonance imaging in congenital superior oblique palsy

    International Nuclear Information System (INIS)

    Sato, Miho; Kondo, Nagako; Awaya, Shinobu; Nomura, Hideki; Yagasaki, Teiji.

    1996-01-01

    MRI examinations were carried out on the defined congenital superior oblique palsy in order to distinguish the congenital and acquired palsies. Subjects were 19 patients diagnosed as congenital and their MRI images of 3 or 5 mm-thick coronary slice were taken. The volume of the oblique muscle was calculated from the images and a comparison was made between the diseased and healthy normal sides. The oblique muscle volume at the diseased side was found reduced in most of congenital superior oblique palsy patients. The reduction was observed even at childhood and was thus considered to be a malformation. Further, it is conceivable that the palsy could be caused by the abnormality in the central nervous system as well as by the present anatomical abnormality. (K.H.)

  3. Pretreatment Hematologic Findings as Novel Predictive Markers for Facial Palsy Prognosis.

    Science.gov (United States)

    Wasano, Koichiro; Kawasaki, Taiji; Yamamoto, Sayuri; Tomisato, Shuta; Shinden, Seiichi; Ishikawa, Toru; Minami, Shujiro; Wakabayashi, Takeshi; Ogawa, Kaoru

    2016-10-01

    To examine the relationship between prognosis of 2 different facial palsies and pretreatment hematologic laboratory values. Multicenter case series with chart review. Three tertiary care hospitals. We examined the clinical records of 468 facial palsy patients who were treated with an antiviral drug in combination with either oral or intravenous corticosteroids in participating hospitals between 2010 and 2014. Patients were divided into a Bell's palsy group or a Hunt's palsy group. We used the Yanagihara facial nerve grading system to grade the severity of facial palsy. "Recovery" from facial palsy was defined as achieving a Yanagihara score ≥36 points within 6 months of onset and having no accompanying facial contracture or synkinesis. We collected information about pretreatment hematologic findings, demographic data, and electrophysiologic test results of the Bell and Hunt group patients who recovered and those who did not. We then compared these data across the 2 palsy groups. In the Bell's palsy group, recovered and unrecovered patients differed significantly in age, sex, electroneuronography score, stapedial muscle reflex, neutrophil rate, lymphocyte rate, neutrophil-to-lymphocyte ratio, and initial Yanagihara score. In the Hunt's palsy group, recovered and unrecovered patients differed in age, electroneuronography score, stapedial muscle reflex, monocyte rate, platelet count, mean corpuscular volume, and initial Yanagihara score. Pretreatment hematologic findings, which reflect the severity of inflammation and bone marrow dysfunction caused by a virus infection, are useful for predicting the prognosis of facial palsy. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.

  4. USE OF BOTULINUM TOXIN TYPE A IN THE TREATMENT OF SPASTICITY IN CHILDREN WITH CEREBRAL PALSY

    Directory of Open Access Journals (Sweden)

    Ljiljana Lazić

    2011-06-01

    Full Text Available Cerebral palsy has an incidence of about 1-2 per 1000 live births, and in spite of the progress of neonatal medicine, it seems that the incidence will not subside in the near future. The most important characteristic of cerebral palsy is movement abnormality: spasticity, chorea, athetosis, ataxia, dystonia, as well as their different combinations. About 70% of children who suffer from cerebral palsy also suffer from some form of spasticity. Spasticity is a type of muscle hypertonicity characterized by rapid increase in resistance to passive stretching of muscles. The interest for botulinum toxin application in the treatment of spasticity has dramatically increased in the last 10 years. Botulinum toxin is the most powerful neurotoxin that is found in nature. It is produced by anaerobic bacteria – clostridium botulinum. It is produced in eight serotypes of which type A is the most commonly used. Botulinum toxin blocks neuromuscular transmission and causes irreversible weakness of the treated muscle. It has been used since 1993 in the treatment of cerebral palsy in children. The toxin effect is permanent and it results in irreversible denervation. Functional recovery is possible after 2-4 months, due to sprouting of nerve endings and the formation of new synaptic contacts. Treatment with botulinum toxin is safe. Adverse effects are rare, temporary and completely reversible. Application of botulinum toxin prevents or reduces contractures and deformities, and thus delays or avoids surgical treatment. Yet, physical therapy, which prolongs and improves the effects of botulinum toxin, remains an essential and most important form of therapy in the treatment of children with cerebral palsy.

  5. A general practice approach to Bell's palsy.

    Science.gov (United States)

    Phan, Nga T; Panizza, Benedict; Wallwork, Benjamin

    2016-11-01

    Bell's palsy is characterised by an acute onset of unilateral, lower motor neuron weakness of the facial nerve in the absence of an identifiable cause. Establishing the correct diagnosis is imperative and choosing the correct treatment options can optimise the likelihood of recovery. This article summarises our understanding of Bell's palsy and the evidence-based management options available for adult patients. The basic assessment should include a thorough history and physical examination as the diagnosis of Bell's palsy is based on exclusion. For confirmed cases of Bell's palsy, corticosteroids are the mainstay of treatment and should be initiated within 72 hours of symptom onset. Antiviral therapy in combination with corticosteroid therapy may confer a small benefit and may be offered on the basis of shared decision making. Currently, no recommendations can be made for acupuncture, physical therapy, electrotherapy or surgical decompression because well-designed studies are lacking and available data are of low quality.

  6. Vocal cord palsy: An uncommon presenting feature of myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Sethi Prahlad

    2011-01-01

    Full Text Available Vocal cord palsy can have myriad causes. Unilateral vocal cord palsy is common and frequently asymptomatic. Trauma, head, neck and mediastinal tumors as well as cerebrovascular accidents have been implicated in causing unilateral vocal cord palsy. Viral neuronitis accounts for most idiopathic cases. Bilateral vocal cord palsy, on the other hand, is much less common and is a potentially life-threatening condition. Myasthenia gravis, an autoimmune disorder caused by antibodies targeting the post-synaptic acetylcholine receptor, has been infrequently implicated in its causation. We report here a case of bilateral vocal cord palsy developing in a 68-year-old man with no prior history of myasthenia gravis 2 months after he was operated on for diverticulitis of the large intestine. Delay in considering the diagnosis led to endotracheal intubation and prolonged mechanical ventilation with attendant complications. Our case adds to the existing literature implicating myasthenia gravis as an infrequent cause of bilateral vocal cord palsy. Our case is unusual as, in our patient, acute-onset respiratory distress and stridor due to bilateral vocal cord palsy was the first manifestation of a myasthenic syndrome.

  7. Cerebral Palsy: Still A Social Problem

    Directory of Open Access Journals (Sweden)

    Angom Bisharda

    1997-08-01

    Full Text Available Research Problem: What arc the social aspects of cerebral palsy?Objective: To determine the extent and severity of neuromuscular involvement in cases of cerebral palsy and to find out the associated defects among these children.Study Design: Cross sectional study.Setting: Tertiary care hospital, outdoor patients.Participants: Children in the age group of 0 - 12 years.Sample Size: 120 children suffering from cerebral palsy.Study Variables: Social factors, neuromuscular involvement.Statistical Analysis: By proportionsResult: Out of 120 cases, maximum number of cases (66.6% were in the age group of 1- 4 years. 83 cases ( 69.16% were males. Among the various types, spastic type was the commonest (87.5%. Of these spastic cases, 52 (49.52% had quadriplegia. No case of tremor and rigidity was seen. Delayed milestones was the commonest associated disorder, seen in 107 (89.16% cases, followed by speech defect in 58(48.3% cases, visual defect in 34(28.3% cases and convulsions in 24 (20.0% cases. Hearing defect was seen in 5 cases (4.16% only.Conclusion: More concerted efforts arc required to identify children with cerebral palsy and rehabilitate them for the betterment of society.

  8. Cerebral Palsy: Still A Social Problem

    Directory of Open Access Journals (Sweden)

    Angom Bisharda

    1997-08-01

    Full Text Available Research Problem: What arc the social aspects of cerebral palsy? Objective: To determine the extent and severity of neuromuscular involvement in cases of cerebral palsy and to find out the associated defects among these children. Study Design: Cross sectional study. Setting: Tertiary care hospital, outdoor patients. Participants: Children in the age group of 0 - 12 years. Sample Size: 120 children suffering from cerebral palsy. Study Variables: Social factors, neuromuscular involvement. Statistical Analysis: By proportions Result: Out of 120 cases, maximum number of cases (66.6% were in the age group of 1- 4 years. 83 cases ( 69.16% were males. Among the various types, spastic type was the commonest (87.5%. Of these spastic cases, 52 (49.52% had quadriplegia. No case of tremor and rigidity was seen. Delayed milestones was the commonest associated disorder, seen in 107 (89.16% cases, followed by speech defect in 58(48.3% cases, visual defect in 34(28.3% cases and convulsions in 24 (20.0% cases. Hearing defect was seen in 5 cases (4.16% only. Conclusion: More concerted efforts arc required to identify children with cerebral palsy and rehabilitate them for the betterment of society.

  9. The MM-CGI Cerebral Palsy: modification and pretesting of an instrument to measure anticipatory grief in parents whose child has cerebral palsy.

    Science.gov (United States)

    Al-Gamal, Ekhlas; Long, Tony

    2014-07-01

    To establish the potential of a modified version of the MM-CGI Childhood Cancer to assess anticipatory grief in parents of children with cerebral palsy, to amend the existing scale for use with the specific patient group, to test the psychometric properties of the modified version (MM-CGI Cerebral Palsy) and to review the clinical potential of the new scale. Parents of children with cerebral palsy may experience reactions similar to parents of children with other enduring or life-limiting conditions, and anticipatory grief may be one such psychological reaction. While the burden of caring is sometimes balanced by positive perceptions of the child, which enhance coping ability, for many parents the outcome is damage to their physical and mental health and impaired family functioning. A cross-sectional, descriptive, correlational design. The MM-CGI Cerebral Palsy was administered in structured interviews with 204 parents. Standardised measures of caregivers' depression, stress and perceived social support were also administered. Mothers and fathers were recruited from healthcare centres and schools for special education. Cronbach's alpha was used to assess internal consistency, and Pearson's product-moment correlation was used to assess construct validity. The subscales were each found to measure a single dimension of anticipatory grief, and significant correlations were established with existing instruments. The instrument demonstrated excellent internal consistency reliability and good construct validity. The MM-CGI Cerebral Palsy could be useful for diagnosing anticipatory grief among parents of children with cerebral palsy. This preliminary work moves the programme on to testing in intervention studies. In the absence of an existing measure for the assessment of anticipatory grief, specifically in parents of children with cerebral palsy, the MM-CGI Cerebral Palsy could prove to be an effective assessment tool for clinicians and researchers. © 2013 John Wiley

  10. [Research on brain white matter network in cerebral palsy infant].

    Science.gov (United States)

    Li, Jun; Yang, Cheng; Wang, Yuanjun; Nie, Shengdong

    2017-10-01

    Present study used diffusion tensor image and tractography to construct brain white matter networks of 15 cerebral palsy infants and 30 healthy infants that matched for age and gender. After white matter network analysis, we found that both cerebral palsy and healthy infants had a small-world topology in white matter network, but cerebral palsy infants exhibited abnormal topological organization: increased shortest path length but decreased normalize clustering coefficient, global efficiency and local efficiency. Furthermore, we also found that white matter network hub regions were located in the left cuneus, precuneus, and left posterior cingulate gyrus. However, some abnormal nodes existed in the frontal, temporal, occipital and parietal lobes of cerebral palsy infants. These results indicated that the white matter networks for cerebral palsy infants were disrupted, which was consistent with previous studies about the abnormal brain white matter areas. This work could help us further study the pathogenesis of cerebral palsy infants.

  11. OCULAR FINDINGS IN CHILDREN WITH CEREBRAL PALSY ...

    African Journals Online (AJOL)

    Cerebral palsy is commonly associated with ocular abnormalities which often impact on their development and education. There is paucity of studies on this in Nigeria. We decided to study/ determine the prevalence of ocular abnormalities among children with cerebral palsy that attended the neurology clinic of University of ...

  12. Radiation-induced cranial nerve palsy

    International Nuclear Information System (INIS)

    Berger, P.S.; Bataini, J.P.

    1977-01-01

    Twenty-five patients with 35 cranial nerve palsies were seen at the Fondation Curie during follow-up after radical radiotherapy for head and neck tumors. The twelfth nerve was involved in 19 cases, the tenth in nine, and the eleventh in five; the fifth and second nerves were involved once each and in the same patient. The twelfth nerve was involved alone in 16 patients and the tenth nerve alone in three, with multiple nerves involved in the remaining six patients. The palsy was noted from 12 to 145 months after diagnosis of the tumor. The latency period could be correlated with dose so that the least square fit equation representing NSD vs delay is NSD = 2598--Delay (in months) x 4.6, with a correlation coefficient of -0.58. The distinction between tumor recurrence and radiation-induced nerve palsy is critical. It can often be inferred from the latency period but must be confirmed by observation over a period of time

  13. MRI comparative study of the progressive supranuclear palsy, striatonigral degeneration and Parkinson disease

    International Nuclear Information System (INIS)

    Wu Wulin; Lou Mingwu; Wang Xiaoyi; Liao Weihua

    2009-01-01

    Objective: To provide a reliable differential diagnosis among the progressive supranuclear palsy (PSP), striatonigral degeneration (SND) and Parkinson disease (PD). Methods: Conventional MRI data in clinically proved PSP (8 cases), SND (9 cases), PD (12 cases) and 12 normal controls were retrospectively analyzed. Midbrain area, pons area, pons diameter and middle cerebellar peduncles width were measured. The ratio of pons area over midbrain area (pons area / midbrain area) was calculated in all patients and normal controls. Then one way ANOVA and a Kruskal-Wallis H test were used for statistical analysis. Results: Midbrain area of the PSP group [(85.8 ± 12.0 mm 2 ] was the smallest, and there was statistically significant difference (P 2 ], PD group [(133.5 ± 10.8)mm 2 ] and the control group [(135.9 ± 7.5) mm 2 ]. There was no overlapping in the distribution of midbrain area between the PSP group (66.0-98.2 mm 2 ) and SND, PD and normal groups (116.2-142.1, 110.8-146.2 and 121.7-145.8 mm 2 ). The pons area-midbrain area ratio (P/M) of the PSP group (5.9 ± 0.8) was the largest in four groups, and there was statistically significant difference (P 2 , (18.6 ± 2.0) mm and (12.9 ± 2.4) mm] in all groups, and significant difference (P 2 , (22.7 ± 1.7) mm and (16.3 ± 1.1) mm], PD group [3.8 ± 0.3, (500.2 ± 25.8) mm 2 , (23.7 ± 1.0) mm and (16.8 ± 1.1) mm] and the control group [3.8 ± 0.3, (508.8 ± 20.6) mm 2 , (23.2 ± 1.2) mm and (16.4 ± 0.9) mm]. But the PD group and control group had no statistically significant difference in the midbrain area, pons area, pons diameter, P/M and middle cerebellar peduncles width (P > 0.05). Conclusion: MRI measurements helps in the differential diagnosis among PSP, SND and PD. (authors)

  14. Quantitative Assessment of Knee Progression Angle During Gait in Children With Cerebral Palsy.

    Science.gov (United States)

    Davids, Jon R; Cung, Nina Q; Pomeroy, Robin; Schultz, Brooke; Torburn, Leslie; Kulkarni, Vedant A; Brown, Sean; Bagley, Anita M

    2018-04-01

    Abnormal hip rotation is a common deviation in children with cerebral palsy (CP). Clinicians typically assess hip rotation during gait by observing the direction that the patella points relative to the path of walking, which is referred to as the knee progression angle (KPA). Two kinematic methods for calculating the KPA are compared with each other. Video-based qualitative assessment of KPA is compared with the quantitative methods to determine reliability and validity. The KPA was calculated by both direct and indirect methods for 32 typically developing (TD) children and a convenience cohort of 43 children with hemiplegic type CP. An additional convenience cohort of 26 children with hemiplegic type CP was selected for qualitative assessment of KPA, performed by 3 experienced clinicians, using 3 categories (internal, >10 degrees; neutral, -10 to 10 degrees; and external, >-10 degrees). Root mean square (RMS) analysis comparing the direct and indirect KPAs was 1.14+0.43 degrees for TD children, and 1.75+1.54 degrees for the affected side of children with CP. The difference in RMS among the 2 groups was statistically, but not clinically, significant (P=0.019). Intraclass correlation coefficient revealed excellent agreement between the direct and indirect methods of KPA for TD and CP children (0.996 and 0.992, respectively; P<0.001).For the qualitative assessment of KPA there was complete agreement among all examiners for 17 of 26 cases (65%). Direct KPA matched for 49 of 78 observations (63%) and indirect KPA matched for 52 of 78 observations (67%). The RMS analysis of direct and indirect methods for KPA was statistically but not clinically significant, which supports the use of either method based upon availability. Video-based qualitative assessment of KPA showed moderate reliability and validity. The differences between observed and calculated KPA indicate the need for caution when relying on visual assessments for clinical interpretation, and demonstrate the

  15. Cranial nerve palsies

    International Nuclear Information System (INIS)

    Ruggieri, P.; Adelizzi, J.; Modic, M.T.; Ross, J.S.; Tkach, J.; Masaryk, T.J.

    1990-01-01

    This paper evaluates the utility of multiplanar reconstructions (MPRs) of three-dimensional (3D) MR angiography data sets in the examination of patients with cranial nerve palsies. The authors hypothesis was that 3D data could be reformatted to highlight the intricate spatial relationships of vessels to adjacent neural tissues by taking advantage of the high vessel-parenchyma contrast in high-resolution 3D time-of-flight sequences. Twenty patients with cranial nerve palsies and 10 asymptomatic patients were examined with coronal T1-weighted and axial T2-weighted imaging plus a gadolinium-enhanced 3D MRA sequence (40/7/15 degrees, axial 60-mm volume, 0.9-mm isotropic resolution). Cranial nerves II-VIII were subsequently evaluated on axial and reformatted coronal and/or sagittal images

  16. Probability of walking in children with cerebral palsy in Europe

    DEFF Research Database (Denmark)

    Beckung, E.; Hagberg, G.; Uldall, P.

    2008-01-01

    cerebral palsy, as well as to IQ level, active epilepsy, and severe visual and hearing impairment. Severe cerebral palsy, defined as both the inability to walk and an IQ of ...OBJECTIVES: The purpose of this work was to describe walking ability in children with cerebral palsy from the Surveillance of Cerebral Palsy in Europe common database through 21 years and to examine the association between walking ability and predicting factors. PATIENTS AND METHODS: Anonymous data...... on 10042 children with cerebral palsy born between 1976 and 1996 were gathered from 14 European centers; 9012 patients were eligible for the analyses. RESULTS: Unaided walking as the primary way of walking at 5 years of age was reported for 54%, walking with assistive devices was reported for 16...

  17. Aggressive osteoblastoma in mastoid process of temporal bone with facial palsy

    Directory of Open Access Journals (Sweden)

    Manoj Jain

    2013-01-01

    Full Text Available Osteoblastoma is an uncommon primary bone tumor with a predilection for posterior elements of spine. Its occurrence in temporal bone and middle ear is extremely rare. Clinical symptoms are non-specific and cranial nerve involvement is uncommon. The cytomorphological features of osteoblastoma are not very well defined and the experience is limited to only few reports. We report an interesting and rare case of aggressive osteoblastoma, with progressive hearing loss and facial palsy, involving the mastoid process of temporal bone and middle ear along with the description of cyto-morphological features.

  18. Anastomotic Urethroplasty for an Obstructing Calculus Within a Bulbar Urethral Diverticulum and Urethral Stricture

    Directory of Open Access Journals (Sweden)

    Pooya Banapour

    2017-01-01

    Full Text Available A 61-year-old male with prior history of endoscopic urethral calculus removal presented to the emergency room with urinary retention and a palpable perineal mass. A CT showed a large calcification within the bulbar urethra. After multiple unsuccessful attempts at foley catheter insertion, the urology service was consulted. The patient was taken to the operating room where an obstructing urethral calculus with associated urethral stricture was visualized on cystoscopy. We present an exceedingly rare case of recurrent urethrolithiasis with associated urethral stricture managed with initial suprapubic tube and delayed primary end-to-end urethroplasty, excision of urethral stricture and urethral diverticulectomy.

  19. Facial palsy in children: emergency department management and outcome.

    Science.gov (United States)

    Wang, Cheng-Hsien; Chang, Yu-Che; Shih, Hong-Mo; Chen, Chun-Yu; Chen, Jih-Chang

    2010-02-01

    To describe the characteristics of children who present to an emergency department (ED) with facial palsy and determine the association of outcome with etiology, degree of initial paralysis, and ED management. This was a retrospective cohort study of children who presented to an ED with facial nerve paralysis (FNP). There were 85 patients with a mean age of 8.0 (SD, 6.1) years; 60% (n = 51) of the patients were male, and 65.9% (n = 56) were admitted to the hospital. Bell palsy (50.6%) was the most common etiology followed by infectious (22.4%), traumatic (16.5%), congenital (7.1%), and neoplastic etiologies (3.5%). Patients with Bell palsy had shorter recovery times (P = 0.049), and traumatic cases required a longer time for recovery (P = 0.016). Acute otitis media (AOM)-related pediatric FNP had shorter recovery times than non-AOM-related cases (P = 0.005) in infectious group. Patients given steroid therapy did not have a shorter recovery time (P = 0.237) or a better recovery (P = 0.269). There was no difference in recovery rate of pediatric patients with Bell palsy between hospitalization or not (P = 0.952). Bell palsy, infection, and trauma were most common etiologies of pediatric FNP. Recovery times were shorter in pediatric patients with Bell palsy and AOM-related FNP, whereas recovery took longer in traumatic cases. Steroid therapy did not seem beneficial for pediatric FNP. Hospitalization is not indicated for pediatric patients with Bell palsy.

  20. Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a rare cause of acute bulbar dysfunction in children = Variante faringo-cérvico-braquial da síndrome de Guillain-Barré: uma causa rara de disfunção bulbar aguda em crianças

    Directory of Open Access Journals (Sweden)

    Coelho, Joana

    2014-01-01

    Conclusões: Apesar da variante faringo-cervico-braquial ser pouco frequente em idade pediátrica, é um diagnóstico que deve ser considerado perante uma criança com disfunção bulbar aguda, pois a identificação precoce permite instituir rapidamente medidas terapêuticas que podem evitar a morte

  1. The evolution of primary progressive apraxia of speech.

    Science.gov (United States)

    Josephs, Keith A; Duffy, Joseph R; Strand, Edythe A; Machulda, Mary M; Senjem, Matthew L; Gunter, Jeffrey L; Schwarz, Christopher G; Reid, Robert I; Spychalla, Anthony J; Lowe, Val J; Jack, Clifford R; Whitwell, Jennifer L

    2014-10-01

    Primary progressive apraxia of speech is a recently described neurodegenerative disorder in which patients present with an isolated apraxia of speech and show focal degeneration of superior premotor cortex. Little is known about how these individuals progress over time, making it difficult to provide prognostic estimates. Thirteen subjects with primary progressive apraxia of speech underwent two serial comprehensive clinical and neuroimaging evaluations 2.4 years apart [median age of onset = 67 years (range: 49-76), seven females]. All underwent detailed speech and language, neurological and neuropsychological assessments, and magnetic resonance imaging, diffusion tensor imaging and (18)F-fluorodeoxyglucose positron emission tomography at both baseline and follow-up. Rates of change of whole brain, ventricle, and midbrain volumes were calculated using the boundary-shift integral and atlas-based parcellation, and rates of regional grey matter atrophy were assessed using tensor-based morphometry. White matter tract degeneration was assessed on diffusion-tensor imaging at each time-point. Patterns of hypometabolism were assessed at the single subject-level. Neuroimaging findings were compared with a cohort of 20 age, gender, and scan-interval matched healthy controls. All subjects developed extrapyramidal signs. In eight subjects the apraxia of speech remained the predominant feature. In the other five there was a striking progression of symptoms that had evolved into a progressive supranuclear palsy-like syndrome; they showed a combination of severe parkinsonism, near mutism, dysphagia with choking, vertical supranuclear gaze palsy or slowing, balance difficulties with falls and urinary incontinence, and one was wheelchair bound. Rates of whole brain atrophy (1.5% per year; controls = 0.4% per year), ventricular expansion (8.0% per year; controls = 3.3% per year) and midbrain atrophy (1.5% per year; controls = 0.1% per year) were elevated (P ≤ 0.001) in all 13

  2. The relation of breech presentation at term to cerebral palsy

    DEFF Research Database (Denmark)

    Krebs, L; Topp, M; Langhoff-Roos, J

    1999-01-01

    OBJECTIVE: To examine the relation between breech delivery and cerebral palsy, considering the influence of intrauterine growth, low Apgar score at birth, and mode of delivery. DESIGN: Register-based, case-control study. POPULATION: A cohort of infants with cerebral palsy born between 1979 and 1986...... in East Denmark, identified by linkage of the cerebral palsy register with the national birth register. Discharge letters from births of breech infants with cerebral palsy were reviewed. MAIN OUTCOME MEASURES: Presentation, mode of delivery, gestational age, birthweight, Apgar score, type of cerebral...

  3. Internal Urethrotomy With Intralesional Mitomycin C: An Effective Option for Endoscopic Management of Recurrent Bulbar and Bulbomembranous Urethral Strictures.

    Science.gov (United States)

    Farrell, M Ryan; Lawrenz, Cedric W; Levine, Laurence A

    2017-12-01

    To describe our experience with direct visual internal urethrotomy (DVIU) and mitomycin C (MMC) for recurrent bulbar and bulbomembranous urethral strictures of radiation and non-radiation-induced etiologies. We reviewed our database of consecutive patients presenting to our tertiary care institution with recurrent bulbar and bulbomembranous urethral strictures who underwent DVIU with MMC from 2011 to 2016. Patients were stratified by radiation-induced strictures (RIS) vs non-RIS. Cold-knife incisions were made at 12-, 3-, and 9-o'clock positions followed by intralesional injection of 10 mL MMC (0.4 mg/mL) in 0.2-0.4 mL aliquots and 1 month of postoperative daily clean intermittent catheterization (CIC). All 44 patients (RIS n = 18, non-RIS n = 26) failed prior endoscopic management or urethroplasty. Median stricture length was 2.0 cm (interquartile range [IQR] 1.0-2.5). Over a median follow-up of 25.8 months (IQR 12.9-47.2), 75.0% of patients (33/44) required no additional surgical intervention (RIS 12/18, 66.7%; non-RIS 21/26, 80.8%). Median time to stricture recurrence among those who recurred was 10.7 months (IQR 3.9-17.6; RIS 9.4 months, IQR 3.5-17.6; non-RIS 11.2 months, IQR 8.0-25.6). Four patients (RIS n = 2, non-RIS n = 2) elected to undergo urethroplasty for recurrence. A second DVIU with MMC was performed in the remaining recurrences (n = 7) with no further surgical intervention required in 37 of 40 of patients (92.5%) overall (RIS 14/16, 87.5%; non-RIS 23/24, 95.8%). No long-term complications were attributable to MMC. DVIU with MMC and short-term CIC for recurrent, short, bulbar and bulbomembranous urethral strictures is a safe endoscopic modality with promising early results. This approach may be useful for patients who are suboptimal candidates for open reconstruction. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Clinical significance of the corpus callosum in cerebral palsy

    International Nuclear Information System (INIS)

    Lee, Eun Ja; Kim, Ji Chang; Kim, Jong Chul; And Others

    2000-01-01

    To evaluate, using magnetic resonance (MR) imaging, the clinal significance of the corpus callosum by measuring the size of various portions of the corpus callosum in children with cerebral palsy, and in paired controls. Fifty-two children (30 boys and 22 girls aged between six and 96 (median, 19) months) in whom cerebral palsy was clinically diagnosed underwent MR imaging. There were 23 term patients and 29 preterm, and the control group was selected by age and sex matching. Clinal subtypes of cerebral palsy were classified as hemiplegia (n=14), spastic diplegia (n=22), or spastic quadriplegia (n=16), and according to the severity of motor palsy, the condition was also classified as mild (n=26), moderate (n=13), or severe (n=13). In addition to the length and height of the corpus callosum, the thickness of its genu, body, transitional zone and splenium, as seen on midsagittal T1-weighted MR images, were also measured. Differences in the measured values of the two groups were statistically analysed and differences in the size of the corpus callosum according to the clinical severity and subtypes of cerebral palsy, and gestational age, were also assessed. Except for height, the measured values of the corpus callosum in patients with cerebral palsy were significantly less than those of the control group (p less than 0.05). Its size decreased according to the severity of motor palsy. Compared with term patients, the corpus callosum in preterm patients was considerably smaller (p less than 0.05). There was statistically significant correlation between the severity of motor palsy and the size of the corpus callosum. Quantitative evaluation of the corpus callosum might be a good indicator of neurologic prognosis, and a sensitive marker for assessing the extent of brain injury

  5. Clinical significance of the corpus callosum in cerebral palsy

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eun Ja; Kim, Ji Chang [The Catholic University of Korea, Seoul (Korea, Republic of); Kim, Jong Chul [School of Medicine, Chungnam National University, Taejon (Korea, Republic of); And Others

    2000-10-01

    To evaluate, using magnetic resonance (MR) imaging, the clinal significance of the corpus callosum by measuring the size of various portions of the corpus callosum in children with cerebral palsy, and in paired controls. Fifty-two children (30 boys and 22 girls aged between six and 96 (median, 19) months) in whom cerebral palsy was clinically diagnosed underwent MR imaging. There were 23 term patients and 29 preterm, and the control group was selected by age and sex matching. Clinal subtypes of cerebral palsy were classified as hemiplegia (n=14), spastic diplegia (n=22), or spastic quadriplegia (n=16), and according to the severity of motor palsy, the condition was also classified as mild (n=26), moderate (n=13), or severe (n=13). In addition to the length and height of the corpus callosum, the thickness of its genu, body, transitional zone and splenium, as seen on midsagittal T1-weighted MR images, were also measured. Differences in the measured values of the two groups were statistically analysed and differences in the size of the corpus callosum according to the clinical severity and subtypes of cerebral palsy, and gestational age, were also assessed. Except for height, the measured values of the corpus callosum in patients with cerebral palsy were significantly less than those of the control group (p less than 0.05). Its size decreased according to the severity of motor palsy. Compared with term patients, the corpus callosum in preterm patients was considerably smaller (p less than 0.05). There was statistically significant correlation between the severity of motor palsy and the size of the corpus callosum. Quantitative evaluation of the corpus callosum might be a good indicator of neurologic prognosis, and a sensitive marker for assessing the extent of brain injury.

  6. Gastrostomy tube feeding of children with cerebral palsy

    DEFF Research Database (Denmark)

    Dahlseng, Magnus O; Andersen, Guro L; DA Graca Andrada, Maria

    2012-01-01

    To compare the prevalence of gastrostomy tube feeding (GTF) of children with cerebral palsy (CP) in six European countries.......To compare the prevalence of gastrostomy tube feeding (GTF) of children with cerebral palsy (CP) in six European countries....

  7. Mobility Experiences of Adolescents with Cerebral Palsy

    Science.gov (United States)

    Palisano, Robert J.; Shimmell, Lorie J.; Stewart, Debra; Lawless, John J.; Rosenbaum, Peter L.; Russell, Dianne J.

    2009-01-01

    The purpose of this study was to describe how youth with cerebral palsy experience mobility in their daily lives using a phenomenological approach. The participants were 10 youth with cerebral palsy, 17 to 20 years of age, selected using purposeful sampling with maximum variation strategies. A total of 14 interviews were completed. Transcripts…

  8. Multiple cranial nerve palsies complicating tympanomastoiditis: case ...

    African Journals Online (AJOL)

    Otitis media either acute or chronic, is not uncommon in childhood. Multiple cranial nerve palsies occuring as a complication of either form of otitis media is unusual. A case of a nine year old boy with chronic suppurative otitis media with associated mastoiditis complicated with ipsilateral multiple cranial nerve palsies is ...

  9. Risk Factors For Epilepsy In Children With Cerebral Palsy | Lagunju ...

    African Journals Online (AJOL)

    Epilepsy is said to occur in 15-90% of children with cerebral palsy and this poses additional economic and psychological stress on affected children and their families. Objectives To describe the risk factors for epilepsy in children with cerebral palsy. Methods One hundred and seventy six children with cerebral palsy seen at ...

  10. Progressive supranuclear palsy: neuronal and glial cytoskeletal pathology in the higher order processing autonomic nuclei of the lower brainstem.

    Science.gov (United States)

    Rüb, U; Del Tredici, K; Schultz, C; de Vos, R A I; Jansen Steur, E N H; Arai, K; Braak, H

    2002-02-01

    The medial and lateral parabrachial nuclei (MPB, LPB), the gigantocellular reticular nucleus (GI), the raphes magnus (RMG) and raphes obscurus nuclei (ROB), as well as the intermediate reticular zone (IRZ) represent pivotal subordinate brainstem centres, all of which control autonomic functions. In this study, we investigated the occurrence and severity of the neuronal and glial cytoskeletal pathology in these six brainstem nuclei from 17 individuals with clinically diagnosed and neuropathologically confirmed progressive supranuclear palsy (PSP). The association between the severity of the pathology and the duration of the disease was investigated by means of correlation analysis. The brainstem nuclei in all of the PSP cases were affected by the neuronal cytoskeletal pathology, with the IRZ and GI regularly showing severe involvement, the MPB, RMG, and ROB marked involvement, and the LPB mild involvement. In the six nuclear greys studied, glial cells undergo alterations of their cytoskeleton on an irregular basis, whereby diseased oligodendrocytes predominantly presented as coiled bodies and affected astrocytes as thorn-shaped astrocytes. In all six nuclei, the severity of the neuronal or glial cytoskeletal pathology showed no correlation with the duration of PSP. In view of their functional role, the neuronal pathology in the nuclei studied offers a possible explanation for the autonomic dysfunctions that eventually develop in the course of PSP.

  11. Olfactory impairment in the rotenone model of Parkinson's disease is associated with bulbar dopaminergic D2 activity after REM sleep deprivation

    Directory of Open Access Journals (Sweden)

    Laís Soares Rodrigues

    2014-12-01

    Full Text Available Olfactory and rapid eye movement (REM sleep deficits are commonly found in untreated subjects with a recent diagnosis of Parkinson's disease (PD. Besides different studies reported declines in olfactory performances during a short period of sleep deprivation. Mechanisms underlying these clinical manifestations are poorly understood although the impairment in the dopamine (DA neurotransmission in the olfactory bulb and in the nigrostriatal pathway may have important roles in olfactory as well as in REM sleep disturbances. Therefore, we have led to the hypothesis that a modulation of the dopaminergic D2 receptors in the olfactory bulb could provide a more comprehensive understanding of the olfactory deficits in PD and after a short period of REM sleep deprivation (REMSD. We decided to investigate the olfactory, neurochemical and histological alterations generated by the administration of piribedil (a selective D2 agonist or raclopride (a selective D2 antagonist, within the glomerular layer of the olfactory bulb, in rats submitted to intranigral rotenone and REMSD. Our findings provided a remarkable evidence of the occurrence of a negative correlation (r = - 0.52, P = 0.04 between the number of periglomerular TH-ir neurons and the bulbar levels of DA in the rotenone, but not sham groups. A significant positive correlation (r = 0.34, P = 0.03 was observed between nigral DA and olfactory discrimination index (DI, for the sham groups, indicating that increased DA levels in the substantia nigra pars compacta (SNpc are associated to enhanced olfactory discrimination performance. Also, increased levels in bulbar and striatal DA induced by piribedil in the rotenone control and rotenone REMSD groups were consistent with reduced amounts of DI. The present evidence reinforce that DA produced by periglomerular neurons, and particularly the bulbar dopaminergic D2 receptors, are essential participants in the olfactory discrimination processes, as well as SNpc

  12. Facial Palsy Following Embolization of a Juvenile Nasopharyngeal Angiofibroma.

    Science.gov (United States)

    Tawfik, Kareem O; Harmon, Jeffrey J; Walters, Zoe; Samy, Ravi; de Alarcon, Alessandro; Stevens, Shawn M; Abruzzo, Todd

    2018-05-01

    To describe a case of the rare complication of facial palsy following preoperative embolization of a juvenile nasopharyngeal angiofibroma (JNA). To illustrate the vascular supply to the facial nerve and as a result, highlight the etiology of the facial nerve palsy. The angiography and magnetic resonance (MR) imaging of a case of facial palsy following preoperative embolization of a JNA is reviewed. A 13-year-old male developed left-sided facial palsy following preoperative embolization of a left-sided JNA. Evaluation of MR imaging studies and retrospective review of the angiographic data suggested errant embolization of particles into the petrosquamosal branch of the middle meningeal artery (MMA), a branch of the internal maxillary artery (IMA), through collateral vasculature. The petrosquamosal branch of the MMA is the predominant blood supply to the facial nerve in the facial canal. The facial palsy resolved since complete infarction of the nerve was likely prevented by collateral blood supply from the stylomastoid artery. Facial palsy is a potential complication of embolization of the IMA, a branch of the external carotid artery (ECA). This is secondary to ischemia of the facial nerve due to embolization of its vascular supply. Clinicians should be aware of this potential complication and counsel patients accordingly prior to embolization for JNA.

  13. [Bell's palsy and facial pain associated with toxocara infection].

    Science.gov (United States)

    Bachtiar, Arian; Auer, Herbert; Finsterer, Josef

    2012-10-01

    Toxocarosis involving cranial nerves is extremely rare and almost exclusively concerns the optic nerve. Toxocarosis involving the seventh cranial nerve has not been reported. A 33y male developed left-sided Bell's palsy two days after left-sided otalgia 6y before. Despite extensive diagnostic work-up at that time the cause of Bell's palsy remained unknown. During the following years Bell's palsy slightly improved but retromandibular pain remained almost unchanged and he developed enlarged lymph nodes along the jugular veins, submandibularly, and in the trigonum caroticum. Re-evaluation 6y later revealed an increased titer of serum antibodies against Toxocara canis and a positive Westernblot for Toxocara canis ES-antigen. Despite absent eosinophilia in the serum, toxocarosis was diagnosed and a therapy with albendazole initiated, with benefit for retromandibular pain, but hardly for Bell's palsy or enlarged lymph nodes. CSF investigations after albendazole revealed a positive Westernblot for antibodies against toxocara but absent pleocytosis or eosinophilia, and negative PCR for Toxocara canis. Visceral larva migrans due to Toxocara canis may be associated with Bell's palsy, retromandibular pain, and lymphadenopathy. A causal relation between Bell's palsy and the helminthosis remains speculative. Adequate therapy years after onset of the infestation may be of limited benefit.

  14. Case report of physiotherapy care in patient with facial nervus palsy.

    OpenAIRE

    Zachová, Lenka

    2012-01-01

    Title: Case report of physiotherapy care in patient with facial nervus peripheral palsy. Objective: Summary of theoretical knowledge and working out the case report of patient diagnosed with peripheral palsy of right facial nerve. Methode and result: This thesis comprehensively summarizes the findings of peripheral facial nerve palsy and its treatment, especially with physiotherapeutic methods. It gives a wide view at the issue of physiotherapy in facial nerve palsy which is a symptom of many...

  15. Clinical Studies on Herbal Acupuncture Therapy in Peripheral Facial Palsy

    Directory of Open Access Journals (Sweden)

    Shin, Min-Seop

    2001-06-01

    Full Text Available Objectives : The treatment of Bell's palsy must be divided into three states(acute, subacute and healing state. 41 cases of the patient suffering from Bell's palsy were treated and observed from january 2000 to July 2001. The usage of herbal acupunctures on that disease have been effective. So I propose a method of herbal acupunctures on Bell's palsy. Methods : By the states(acute, subacute and healing state of Bell's palsy, SY(消炎 herbal acupuncture is used at the acute state, Hominis Placenta(紫河車 at the subacute, JGH(中氣下陷 at the healing state. Results : 1. At the acute state, SY(消炎 herbal acupuncture is effective to postauricular pain. 2. At the subacute state, Hominis Placenta(紫河車 herbal acupuncture is effective to decreasing pain and improving symptoms. 3. By the states(acute, subacute and healing state of Bell's palsy, SY(消炎, Hominis Placenta(紫河車 and JGH(中氣下陷 herbal acupuncture is effective to improving symptoms of Bell's palsy.

  16. Subcortical frontal lesions on MRI in patients with motor neurone disease

    International Nuclear Information System (INIS)

    Andreadou, E.; Sgouropoulos, P.; Varelas, P.; Papageorgiou, C.; Gouliamos, A.

    1998-01-01

    MRI was performed in 32 patients with motor neurone disease (26 men and 6 women, aged 40-77 years) and in a control group of 21 subjects. Of the patients studied, 19 had definite and 11 probable amyotrophic lateral sclerosis (ALS) and two had progressive bulbar palsy. In 10 patients there were asymmetrical bilateral foci of increased signal intensity on proton-density and T 2 -weighted images, confined to the white matter. Two patients had only cortical frontal atrophy and slightly increased ventricular size, whereas 20 had normal MRI. The focal lesions were not confined to corticospinal tracts, but were also observed in subcortical frontal areas. While the lesions along the corticospinal tracts correspond to pyramidal tract degeneration, the subcortical foci correlate with degeneration of the frontal bundles and indicate generalised involvement of the central nervous system. (orig.)

  17. Peripheral nerve involvement in Bell's palsy

    Directory of Open Access Journals (Sweden)

    J. A. Bueri

    1984-12-01

    Full Text Available A group of patients with Bell's palsy were studied in order to disclose the presence of subclinical peripheral nerve involvement. 20 patients, 8 male and 12 female, with recent Bell's palsy as their unique disease were examined, in all cases other causes of polyneuropathy were ruled out. Patients were investigated with CSF examination, facial nerve latencies in the affected and in the sound sides, and maximal motor nerve conduction velocities, as well as motor terminal latencies from the right median and peroneal nerves. CSF laboratory examination was normal in all cases. Facial nerve latencies were abnormal in all patients in the affected side, and they differed significantly from those of control group in the clinically sound side. Half of the patients showed abnormal values in the maximal motor nerve conduction velocities and motor terminal latencies of the right median and peroneal nerves. These results agree with previous reports which have pointed out that other cranial nerves may be affected in Bell's palsy. However, we have found a higher frequency of peripheral nerve involvement in this entity. These findings, support the hypothesis that in some patients Bell's palsy is the component of a more widespread disease, affecting other cranial and peripheral nerves.

  18. The history of facial palsy and spasm

    Science.gov (United States)

    Sajadi, Mohamad-Reza M.; Tabatabaie, Seyed Mahmoud

    2011-01-01

    Although Sir Charles Bell was the first to provide the anatomic basis for the condition that bears his name, in recent years researchers have shown that other European physicians provided earlier clinical descriptions of peripheral cranial nerve 7 palsy. In this article, we describe the history of facial distortion by Greek, Roman, and Persian physicians, culminating in Razi's detailed description in al-Hawi. Razi distinguished facial muscle spasm from paralysis, distinguished central from peripheral lesions, gave the earliest description of loss of forehead wrinkling, and gave the earliest known description of bilateral facial palsy. In doing so, he accurately described the clinical hallmarks of a condition that we recognize as Bell palsy. PMID:21747074

  19. Peripheral facial palsy: Speech, communication and oral motor function.

    Science.gov (United States)

    Movérare, T; Lohmander, A; Hultcrantz, M; Sjögreen, L

    2017-02-01

    The aim of the present study was to examine the effect of acquired unilateral peripheral facial palsy on speech, communication and oral functions and to study the relationship between the degree of facial palsy and articulation, saliva control, eating ability and lip force. In this descriptive study, 27 patients (15 men and 12 women, mean age 48years) with unilateral peripheral facial palsy were included if they were graded under 70 on the Sunnybrook Facial Grading System. The assessment was carried out in connection with customary visits to the ENT Clinic and comprised lip force, articulation and intelligibility, together with perceived ability to communicate and ability to eat and control saliva conducted through self-response questionnaires. The patients with unilateral facial palsy had significantly lower lip force, poorer articulation and ability to eat and control saliva compared with reference data in healthy populations. The degree of facial palsy correlated significantly with lip force but not with articulation, intelligibility, perceived communication ability or reported ability to eat and control saliva. Acquired peripheral facial palsy may affect communication and the ability to eat and control saliva. Physicians should be aware that there is no direct correlation between the degree of facial palsy and the possible effect on communication, eating ability and saliva control. Physicians are therefore recommended to ask specific questions relating to problems with these functions during customary medical visits and offer possible intervention by a speech-language pathologist or a physiotherapist. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  20. Prognosis and MRI findings in patients with peripheral facial palsy

    International Nuclear Information System (INIS)

    Mineta, Masayuki; Saitoh, Yasuhiro; Yoshikawa, Daihei; Yamada, Tomonori; Aburano, Tamio; Matoba, Mitsuaki.

    1997-01-01

    We examined a series of 21 peripheral facial palsy patients attempted to ( 17 Bell's palsy, 4 Hunt syndrome) with Gd-DTPA-enhanced MRI and attempted to determine the relation between prognosis and MRI findings. We divided patients into two groups based on facial palsy scores of Japanese facial nerve research; a good group (G-Group) and a bad group (B-group). The G-group scored over 20 points on the 20th day after the first visit and the B-group under 20 points. G-group consisted of 9 Bell's palsy and 1 Hunt syndrome patients, and the B-group of 8 Bell's palsy and 3 Hunt syndrome patients. The averaged facial palsy score of both groups was analyzed every week during 4 weeks. Recovery from the palsy was better in the G-group than the B-group (P<0.05); the scores at the 4th week of the G- and B-groups were 32.6±15.2 and 7.8±7.4, respectively. The MRI findings of both groups were examined retrospectively. Nine of 10 G-group and nine of 11 B-group patients had abnormal contrast enhancement. The result of enhanced facial nerve segment was as follows: G-group, auditory canal 1, labyrinthine/geniculate 7, tympanic 7, mastoid 7: B-group, auditory canal 2, labyrinthine/geniculate 8, tympanic 8, mastoid 7. Our results indicate no relation between the prognosis and the MRI findings. Therefore, it is impossible to predict the prognosis of facial palsy from the results of Gd-DTPA-enhanced MRI. (author)

  1. Prognosis and MRI findings in patients with peripheral facial palsy

    Energy Technology Data Exchange (ETDEWEB)

    Mineta, Masayuki; Saitoh, Yasuhiro; Yoshikawa, Daihei; Yamada, Tomonori; Aburano, Tamio [Asahikawa Medical College, Hokkaido (Japan); Matoba, Mitsuaki

    1997-02-01

    We examined a series of 21 peripheral facial palsy patients attempted to ( 17 Bell`s palsy, 4 Hunt syndrome) with Gd-DTPA-enhanced MRI and attempted to determine the relation between prognosis and MRI findings. We divided patients into two groups based on facial palsy scores of Japanese facial nerve research; a good group (G-Group) and a bad group (B-group). The G-group scored over 20 points on the 20th day after the first visit and the B-group under 20 points. G-group consisted of 9 Bell`s palsy and 1 Hunt syndrome patients, and the B-group of 8 Bell`s palsy and 3 Hunt syndrome patients. The averaged facial palsy score of both groups was analyzed every week during 4 weeks. Recovery from the palsy was better in the G-group than the B-group (P<0.05); the scores at the 4th week of the G- and B-groups were 32.6{+-}15.2 and 7.8{+-}7.4, respectively. The MRI findings of both groups were examined retrospectively. Nine of 10 G-group and nine of 11 B-group patients had abnormal contrast enhancement. The result of enhanced facial nerve segment was as follows: G-group, auditory canal 1, labyrinthine/geniculate 7, tympanic 7, mastoid 7: B-group, auditory canal 2, labyrinthine/geniculate 8, tympanic 8, mastoid 7. Our results indicate no relation between the prognosis and the MRI findings. Therefore, it is impossible to predict the prognosis of facial palsy from the results of Gd-DTPA-enhanced MRI. (author)

  2. Cerebral Palsy Checklist: Babies & Preschoolers (Birth to age 5)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Cerebral Palsy Checklist: Babies & Preschoolers KidsHealth / For Parents / Cerebral Palsy Checklist: Babies & Preschoolers What's in this article? Step ...

  3. Management of peripheral facial nerve palsy

    OpenAIRE

    Finsterer, Josef

    2008-01-01

    Peripheral facial nerve palsy (FNP) may (secondary FNP) or may not have a detectable cause (Bell?s palsy). Three quarters of peripheral FNP are primary and one quarter secondary. The most prevalent causes of secondary FNP are systemic viral infections, trauma, surgery, diabetes, local infections, tumor, immunological disorders, or drugs. The diagnosis of FNP relies upon the presence of typical symptoms and signs, blood chemical investigations, cerebro-spinal-fluid-investigations, X-ray of the...

  4. [Acute palsy of twelfth cranial nerve].

    Science.gov (United States)

    Munoz del Castillo, F; Molina Nieto, T; De la Riva Aguilar, A; Triviño Tarradas, F; Bravo-Rodríguez, F; Ramos Jurado, A

    2005-01-01

    The hypoglossal nerve or Twelfth-nerve palsy is a rare damage with different causes: tumors or metastases in skull base, cervicals tumors, schwannoma, dissection or aneurysm carotid arteries, stroke, trauma, idiopathic cause, radiation, infections (mononucleosis) or multiple cranial neuropathy. Tumors were responsible for nearly half of the cases in different studies. We studied a female with hypoglossal nerve acute palsy. We made a differential diagnostic with others causes and a review of the literature.

  5. Public health issues related to infection in pregnancy and cerebral palsy

    DEFF Research Database (Denmark)

    Schendel, Diana E.; Schuchat, Anne; Thorsen, Poul

    2002-01-01

    Cerebral palsy is the most common neuromotor developmental disability of childhood, affecting as many as 8,000 to 12,000 children born in the U.S. each year (corresponding to a prevalence rate of between 2 and 3 per 1000 children). Recent improvements in neonatal care have not resulted in a decline...... in the overall prevalence of cerebral palsy and, in fact, greater numbers of very preterm/very low birth weight infants are surviving with cerebral palsy and other developmental problems. Infection in pregnancy may be an important cause of the disorder. In preterm infants, there appears to be about a 2-fold...... increased risk for cerebral palsy from chorioamnionitis, and in term infants the estimated increased risk is about 4-fold. Provisionally, chorioamnionitis might account for 12% of spastic cerebral palsy in term infants and 28% of cerebral palsy in preterm infants. Studies of biochemical markers of fetal...

  6. Cerebral Palsy Checklist: Teens & Young Adult (13 to 21)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Cerebral Palsy Checklist: Teens & Young Adults KidsHealth / For Parents / Cerebral Palsy Checklist: Teens & Young Adults What's in this article? ...

  7. Association of Lead Levels and Cerebral Palsy

    Science.gov (United States)

    Bansal, Neha; Aggarwal, Anju; Faridi, M. M. A.; Sharma, Tusha; Baneerjee, B. D.

    2017-01-01

    Background: Cerebral palsy is a common motor disability in childhood. Raised lead levels affect cognition. Children with cerebral palsy may have raised lead levels, further impairing their residual cognitive motor and behavioral abilities. Environmental exposure and abnormal eating habits may lead to increased lead levels. Aims and Objectives: To measure blood lead levels in children with cerebral palsy and compare them with healthy neurologically normal children. To correlate blood lead levels with environmental factors. Material and Methods: Design: Prospective case-control study. Setting: Tertiary care hospital. Participants: Cases comprised 34 children with cerebral palsy, and controls comprised 34 neurologically normal, age- and sex-matched children. Methods: Clinical and demographic details were recorded as per proforma. Detailed environmental history was recorded to know the source of exposure to lead. These children were investigated and treated as per protocol. Venous blood was collected in ethylenediaminetetraacetic acid vials for analysis of blood lead levels. Lead levels were estimated by Schimadzu Flame AA-6800 (atomic absorption spectrophotometer). Data were analyzed using SPSS version 17. P pica were 12.33 ± 10.02 µg/dL in comparison to children with no history of pica, 6.70 ± 4.60 µg/dL (P = .029). No correlation was found between hemoglobin and blood lead levels in cases and controls. Conclusion: In our study, blood lead levels are raised in children with cerebral palsy. However, further studies are required to show effects of raised levels in these children. PMID:28491920

  8. Common questions about Bell palsy.

    Science.gov (United States)

    Albers, Janet R; Tamang, Stephen

    2014-02-01

    Bell palsy is an acute affliction of the facial nerve, resulting in sudden paralysis or weakness of the muscles on one side of the face. Testing patients with unilateral facial paralysis for diabetes mellitus or Lyme disease is not routinely recommended. Patients with Lyme disease typically present with additional manifestations, such as arthritis, rash, or facial swelling. Diabetes may be a comorbidity of Bell palsy, but testing is not needed in the absence of other indications, such as hypertension. In patients with atypical symptoms, magnetic resonance imaging with contrast enhancement can be used to rule out cranial mass effect and to add prognostic value. Steroids improve resolution of symptoms in patients with Bell palsy and remain the preferred treatment. Antiviral agents have a limited role, and may improve outcomes when combined with steroids in patients with severe symptoms. When facial paralysis is prolonged, surgery may be indicated to prevent ocular desiccation secondary to incomplete eyelid closure. Facial nerve decompression is rarely indicated or performed. Physical therapy modalities, including electrostimulation, exercise, and massage, are neither beneficial nor harmful.

  9. Bell's palsy: aetiology, clinical features and multidisciplinary care.

    Science.gov (United States)

    Eviston, Timothy J; Croxson, Glen R; Kennedy, Peter G E; Hadlock, Tessa; Krishnan, Arun V

    2015-12-01

    Bell's palsy is a common cranial neuropathy causing acute unilateral lower motor neuron facial paralysis. Immune, infective and ischaemic mechanisms are all potential contributors to the development of Bell's palsy, but the precise cause remains unclear. Advancements in the understanding of intra-axonal signal molecules and the molecular mechanisms underpinning Wallerian degeneration may further delineate its pathogenesis along with in vitro studies of virus-axon interactions. Recently published guidelines for the acute treatment of Bell's palsy advocate for steroid monotherapy, although controversy exists over whether combined corticosteroids and antivirals may possibly have a beneficial role in select cases of severe Bell's palsy. For those with longstanding sequaelae from incomplete recovery, aesthetic, functional (nasal patency, eye closure, speech and swallowing) and psychological considerations need to be addressed by the treating team. Increasingly, multidisciplinary collaboration between interested clinicians from a wide variety of subspecialties has proven effective. A patient centred approach utilising physiotherapy, targeted botulinum toxin injection and selective surgical intervention has reduced the burden of long-term disability in facial palsy. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  10. [Cranial nerve palsy caused by tumours of the head and neck

    NARCIS (Netherlands)

    Delsing, C.P.; Verbist, B.M.; Hoogen, F.J.A. van den

    2013-01-01

    Cranial nerve palsy is a diagnostic guiding symptom, but often goes unrecognized. The differential diagnosis includes a variety of diseases, including malignant tumours of the head and neck. Here we describe three cases of cranial nerve palsy. In two of the cases the palsy was recognized following

  11. Botulinum toxin treatment for facial palsy: A systematic review.

    Science.gov (United States)

    Cooper, Lilli; Lui, Michael; Nduka, Charles

    2017-06-01

    Facial palsy may be complicated by ipsilateral synkinesis or contralateral hyperkinesis. Botulinum toxin is increasingly used in the management of facial palsy; however, the optimum dose, treatment interval, adjunct therapy and performance as compared with alternative treatments have not been well established. This study aimed to systematically review the evidence for the use of botulinum toxin in facial palsy. The Cochrane central register of controlled trials (CENTRAL), MEDLINE(R) (1946 to September 2015) and Embase Classic + Embase (1947 to September 2015) were searched for randomised studies using botulinum toxin in facial palsy. Forty-seven studies were identified, and three included. Their physical and patient-reported outcomes are described, and observations and cautions are discussed. Facial asymmetry has a strong correlation to subjective domains such as impairment in social interaction and perception of self-image and appearance. Botulinum toxin injections represent a minimally invasive technique that is helpful in restoring facial symmetry at rest and during movement in chronic, and potentially acute, facial palsy. Botulinum toxin in combination with physical therapy may be particularly helpful. Currently, there is a paucity of data; areas for further research are suggested. A strong body of evidence may allow botulinum toxin treatment to be nationally standardised and recommended in the management of facial palsy. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  12. Is hemiplegic cerebral palsy equivalent to amblyopia of the corticospinal system?

    Science.gov (United States)

    Eyre, Janet A; Smith, Martin; Dabydeen, Lyvia; Clowry, Gavin J; Petacchi, Eliza; Battini, Roberta; Guzzetta, Andrea; Cioni, Giovanni

    2007-11-01

    Subjects with severe hemiplegic cerebral palsy have increased ipsilateral corticospinal projections from their noninfarcted cortex. We investigated whether their severe impairment might, in part, be caused by activity-dependent, competitive displacement of surviving contralateral corticospinal projections from the affected cortex by more active ipsilateral corticospinal projections from the nonaffected cortex, thereby compounding the impairment. Transcranial magnetic stimulation (TMS) characterized corticospinal tract development from each hemisphere over the first 2 years in 32 healthy children, 14 children with unilateral stroke, and 25 with bilateral lesions. Magnetic resonance imaging and anatomic studies compared corticospinal tract growth in 13 patients with perinatal stroke with 46 healthy subjects. Infants with unilateral lesions initially had responses after TMS of the affected cortex, which became progressively more abnormal, and seven were eventually lost. There was associated hypertrophy of the ipsilateral corticospinal axons projecting from the noninfarcted cortex. Magnetic resonance imaging and anatomic studies demonstrated hypertrophy of the corticospinal tract from the noninfarcted hemisphere. TMS findings soon after the stroke did not predict impairment; subsequent loss of responses and hypertrophy of ipsilateral corticospinal axons from the noninfarcted cortex predicted severe impairment at 2 years. Infants with bilateral lesions maintained responses to TMS from both hemispheres with a normal pattern of development. Rather than representing "reparative plasticity," increased ipsilateral projections from the noninfarcted cortex compound disability by competitively displacing surviving contralateral corticospinal projections from the infarcted cortex. This may provide a pathophysiological explanation for why signs of hemiplegic cerebral palsy appear late and progress over the first 2 years of life.

  13. Genomic analysis identifies masqueraders of full-term cerebral palsy.

    Science.gov (United States)

    Takezawa, Yusuke; Kikuchi, Atsuo; Haginoya, Kazuhiro; Niihori, Tetsuya; Numata-Uematsu, Yurika; Inui, Takehiko; Yamamura-Suzuki, Saeko; Miyabayashi, Takuya; Anzai, Mai; Suzuki-Muromoto, Sato; Okubo, Yukimune; Endo, Wakaba; Togashi, Noriko; Kobayashi, Yasuko; Onuma, Akira; Funayama, Ryo; Shirota, Matsuyuki; Nakayama, Keiko; Aoki, Yoko; Kure, Shigeo

    2018-05-01

    Cerebral palsy is a common, heterogeneous neurodevelopmental disorder that causes movement and postural disabilities. Recent studies have suggested genetic diseases can be misdiagnosed as cerebral palsy. We hypothesized that two simple criteria, that is, full-term births and nonspecific brain MRI findings, are keys to extracting masqueraders among cerebral palsy cases due to the following: (1) preterm infants are susceptible to multiple environmental factors and therefore demonstrate an increased risk of cerebral palsy and (2) brain MRI assessment is essential for excluding environmental causes and other particular disorders. A total of 107 patients-all full-term births-without specific findings on brain MRI were identified among 897 patients diagnosed with cerebral palsy who were followed at our center. DNA samples were available for 17 of the 107 cases for trio whole-exome sequencing and array comparative genomic hybridization. We prioritized variants in genes known to be relevant in neurodevelopmental diseases and evaluated their pathogenicity according to the American College of Medical Genetics guidelines. Pathogenic/likely pathogenic candidate variants were identified in 9 of 17 cases (52.9%) within eight genes: CTNNB1 , CYP2U1 , SPAST , GNAO1 , CACNA1A , AMPD2 , STXBP1 , and SCN2A . Five identified variants had previously been reported. No pathogenic copy number variations were identified. The AMPD2 missense variant and the splice-site variants in CTNNB1 and AMPD2 were validated by in vitro functional experiments. The high rate of detecting causative genetic variants (52.9%) suggests that patients diagnosed with cerebral palsy in full-term births without specific MRI findings may include genetic diseases masquerading as cerebral palsy.

  14. Contemporary management of Bell palsy.

    Science.gov (United States)

    Jowett, Nate; Hadlock, Tessa A

    2015-04-01

    Bell palsy (BP) is the most common diagnosis in acute and chronic facial palsy. Although most patients fully recover, more than one-quarter will have residual dysfunction. Of these, nearly half will demonstrate severe limitations in facial expression. Though significant attention has been paid to acute management and prognosis, a paucity of literature exists addressing management of the long-term sequelae of BP. This article describes contemporary use of physical therapy, injectables, and static and dynamic surgical procedures in facial reanimation of acute and chronic BP. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  15. Pediatric Cerebral Palsy in Africa: Where Are We?

    Science.gov (United States)

    Donald, Kirsten A; Kakooza, Angelina M; Wammanda, Robinson D; Mallewa, Macpherson; Samia, Pauline; Babakir, Haydar; Bearden, David; Majnemer, Annette; Fehlings, Darcy; Shevell, Michael; Chugani, Harry; Wilmshurst, Jo M

    2015-07-01

    Cerebral palsy is the most common cause of physical disability in children worldwide. However, little is reported on this condition in the African context. Doctors from 22 countries in Africa, and representatives from a further 5 countries outside Africa, met to discuss the challenges in the evaluation and management of children with cerebral palsy in Africa and to propose service needs and further research. Basic care is limited by the poor availability of diagnostic facilities or medical personnel with experience and expertise in managing cerebral palsy, exacerbated by lack of available interventions such as medications, surgical procedures, or even regular therapy input. Relevant guidelines are lacking. In order to guide services for children with existing disabilities, to effectively target the main etiologies and to develop preventive strategies for the continent, research priorities must include multicenter collaborative studies looking at the prevalence, risk factors, and treatment of cerebral palsy. © The Author(s) 2014.

  16. Sharing Experience dan Resiliensi: Studi atas Facebook Group Orang Tua Anak Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Safrina Rofasita

    2017-06-01

    [Orang tua yang mendapati anaknya terfonis sebagai anak Cerebral Palsy mengalami kedukaan mendalam yang mengakibatkan ketidakpercayaan diri, dan putus asa. Hal itu diakibatkan ketahanan terhadap stres (resiliensi rendah, oleh karena itu orang tua mengikuti sharing experiences penyandang Cerebral Palsy melalui Facebook Group orang tua anak Cerebral Palsy. Penelitian ini bertujuan menjawab pertanyaan adakah pengaruh sharing experiences penyandang Cerebral Palsy terhadap resiliensi orang tua anak Cerebral Palsy yang terhimpun dalam Facebook Group Orang Tua Anak Cerebral Palsy. Penelitian menggunakan methode kombinasi antara kuantitatif dan kualitatif. Penelitian menemukan bahwa Facebook Group berpengaruh pada peningkatan resiliensi orang tua anak cerebal palcy karena mereka mendapatkan pengetahuan dan informasi tambahan dari forum itu.

  17. Bilateral Bell palsy as a presenting sign of preeclampsia.

    Science.gov (United States)

    Vogell, Alison; Boelig, Rupsa C; Skora, Joanna; Baxter, Jason K

    2014-08-01

    Bell palsy is a facial nerve neuropathy that is a rare disorder but occurs at higher frequency in pregnancy. Almost 30% of cases are associated with preeclampsia or gestational hypertension. Bilateral Bell palsy occurs in only 0.3%-2.0% of cases of facial paralysis, has a poorer prognosis for recovery, and may be associated with a systemic disorder. We describe a case of a 24-year-old primigravid woman with a twin gestation at 35 weeks diagnosed initially with bilateral facial palsy and subsequently with preeclampsia. She then developed partial hemolysis, elevated liver enzymes, and low platelet count syndrome, prompting the diagnosis of severe preeclampsia, and was delivered. Bilateral facial palsy is a rare entity in pregnancy that may be the first sign of preeclampsia and suggests increased severity of disease, warranting close monitoring.

  18. Cranial ultrasound findings in preterm infants predict the development of cerebral palsy

    DEFF Research Database (Denmark)

    Skovgaard, Ann Lawaetz; Zachariassen, Gitte

    2017-01-01

    record review. The cohort consisted of very preterm born children (gestational age ≤ 32 + 0) born from 2004 to 2008. For each infant, we obtained results from all cranial ultrasounds performed during hospitalisation. In 2014, patient records were evaluated for cerebral palsy, Gross Motor Function...... haemorrhagic infarction (PVHI), of whom two developed cerebral palsy. Nine children were diagnosed with periventricular leukomalacia (PVL), of whom six developed cerebral palsy. Cerebral palsy was detected in 14 children (6.4%), and one (0.5%) child was in need of a hearing assistive device. Severe brain...... injury (GMH-IVH3, PVHI or PVL) (p = 0.000) and being of male gender (p = 0.03) were associated with cerebral palsy in childhood. Conclusion: Severe brain injuries detected by neonatal cranial ultrasound in very preterm infants is associated with development of cerebral palsy in childhood....

  19. A COMPARATIVE STUDY ON EFFECTIVENESS OF OPEN VERSUS CLOSED KINETIC CHAIN EXERCISES TO IMPROVE GAIT IN SPASTIC DIPLEGIC CEREBRAL PALSY

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    Trishna Saikia Baruah

    2016-04-01

    Full Text Available Background: Cerebral Palsy (CP describes a non- progressive but not unchanging disorder of movement and posture due to an insult to or anomaly of the developing brain. People with spastic diplegia typically walk slowly and have difficulties in performing activities such as walking running or jumping. Children with spastic diplegic cerebral palsy are relied more on cadence to increase speed. Hence, the purpose of this study is to compare the effectiveness of open and closed kinetic chain (OKC and CKC exercises in improving gait in spastic diplegic cerebral palsy. Methods: 30 children with spastic diplegic cerebral palsy of both genders with age 4-12 years was taken. Cadence and distance covered in 1Minute Walk Test was calculated before and after the test. The intervention for group A was CKC exercises and group B was OKC exercises for 3 days a week for 6 weeks and each session lasted for 30-45 minutes was given for both the groups. Results: Paired t-test was performed to find effectiveness of CKC and OKC improving gait in spastic diplegic CP to see the difference of means of 1minute walk, t = 10.789 which is significant (p = 0.000 and for cadence, t = 3.37 which is highly significant (p = 0.00 implying that cadence and distance covered in1minute walk was more with CKC exercises. Conclusion: Based on the result it is concluded that CKC exercises are effective in improving gait than OKC exercises in spastic diplegic cerebral palsy.

  20. Localization of Basal Ganglia and Thalamic Damage in Dyskinetic Cerebral Palsy.

    Science.gov (United States)

    Aravamuthan, Bhooma R; Waugh, Jeff L

    2016-01-01

    Dyskinetic cerebral palsy affects 15%-20% of patients with cerebral palsy. Basal ganglia injury is associated with dyskinetic cerebral palsy, but the patterns of injury within the basal ganglia predisposing to dyskinetic cerebral palsy are unknown, making treatment difficult. For example, deep brain stimulation of the globus pallidus interna improves dystonia in only 40% of patients with dyskinetic cerebral palsy. Basal ganglia injury heterogeneity may explain this variability. To investigate this, we conducted a qualitative systematic review of basal ganglia and thalamic damage in dyskinetic cerebral palsy. Reviews and articles primarily addressing genetic or toxic causes of cerebral palsy were excluded yielding 22 studies (304 subjects). Thirteen studies specified the involved basal ganglia nuclei (subthalamic nucleus, caudate, putamen, globus pallidus, or lentiform nuclei, comprised by the putamen and globus pallidus). Studies investigating the lentiform nuclei (without distinguishing between the putamen and globus pallidus) showed that all subjects (19 of 19) had lentiform nuclei damage. Studies simultaneously but independently investigating the putamen and globus pallidus also showed that all subjects (35 of 35) had lentiform nuclei damage (i.e., putamen or globus pallidus damage); this was followed in frequency by damage to the putamen alone (70 of 101, 69%), the subthalamic nucleus (17 of 25, 68%), the thalamus (88 of 142, 62%), the globus pallidus (7/35, 20%), and the caudate (6 of 47, 13%). Globus pallidus damage was almost always coincident with putaminal damage. Noting consistent involvement of the lentiform nuclei in dyskinetic cerebral palsy, these results could suggest two groups of patients with dyskinetic cerebral palsy: those with putamen-predominant damage and those with panlenticular damage involving both the putamen and the globus pallidus. Differentiating between these groups could help predict response to therapies such as deep brain

  1. Facial nerve palsy: Evaluation by contrast-enhanced MR imaging

    International Nuclear Information System (INIS)

    Kinoshita, T.; Ishii, K.; Okitsu, T.; Okudera, T.; Ogawa, T.

    2001-01-01

    AIM: The purpose of this study was to investigate the value of contrast-enhanced magnetic resonance (MR) imaging in patients with peripheral facial nerve palsy. MATERIALS AND METHODS: MR imaging was performed in 147 patients with facial nerve palsy, using a 1.0 T unit. All of 147 patients were evaluated by contrast-enhanced MR imaging and the pattern of enhancement was compared with that in 300 control subjects evaluated for suspected acoustic neurinoma. RESULTS: The intrameatal and labyrinthine segments of the normal facial nerve did not show enhancement, whereas enhancement of the distal intrameatal segment and the labyrinthine segment was respectively found in 67% and 43% of patients with Bell's palsy. The geniculate ganglion or the tympanic-mastoid segment was enhanced in 21% of normal controls versus 91% of patients with Bell's palsy. Abnormal enhancement of the non-paralyzed facial nerve was found in a patient with bilateral temporal bone fracture. CONCLUSION: Enhancement of the distal intrameatal and labyrinthine segments is specific for facial nerve palsy. Contrast-enhanced MR imaging can reveal inflammatory facial nerve lesions and traumatic nerve injury, including clinically silent damage in trauma. Kinoshita T. et al. (2001)

  2. Facial nerve palsy: Evaluation by contrast-enhanced MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Kinoshita, T.; Ishii, K.; Okitsu, T.; Okudera, T.; Ogawa, T

    2001-11-01

    AIM: The purpose of this study was to investigate the value of contrast-enhanced magnetic resonance (MR) imaging in patients with peripheral facial nerve palsy. MATERIALS AND METHODS: MR imaging was performed in 147 patients with facial nerve palsy, using a 1.0 T unit. All of 147 patients were evaluated by contrast-enhanced MR imaging and the pattern of enhancement was compared with that in 300 control subjects evaluated for suspected acoustic neurinoma. RESULTS: The intrameatal and labyrinthine segments of the normal facial nerve did not show enhancement, whereas enhancement of the distal intrameatal segment and the labyrinthine segment was respectively found in 67% and 43% of patients with Bell's palsy. The geniculate ganglion or the tympanic-mastoid segment was enhanced in 21% of normal controls versus 91% of patients with Bell's palsy. Abnormal enhancement of the non-paralyzed facial nerve was found in a patient with bilateral temporal bone fracture. CONCLUSION: Enhancement of the distal intrameatal and labyrinthine segments is specific for facial nerve palsy. Contrast-enhanced MR imaging can reveal inflammatory facial nerve lesions and traumatic nerve injury, including clinically silent damage in trauma. Kinoshita T. et al. (2001)

  3. Cerebral Palsy. Fact Sheet = La Paralisis Cerebral. Hojas Informativas Sobre Discapacidades.

    Science.gov (United States)

    National Information Center for Children and Youth with Disabilities, Washington, DC.

    This fact sheet on cerebral palsy is written in both English and Spanish. First, it provides a definition of cerebral palsy and considers various causes (e.g., an insufficient amount of oxygen reaching the fetal or newborn brain). The fact sheet then offers incidence figures and explains characteristics of the three main types of cerebral palsy:…

  4. Role of the tau gene region chromosome inversion in progressive supranuclear palsy, corticobasal degeneration, and related disorders.

    Science.gov (United States)

    Webb, Amy; Miller, Bruce; Bonasera, Stephen; Boxer, Adam; Karydas, Anna; Wilhelmsen, Kirk C

    2008-11-01

    An inverted region on chromosome 17 has been previously linked to many Pick complex diseases. Due to the inversion, an exact causal locus has been difficult to identify, but the microtubule-associated protein tau gene is a likely candidate gene for its involvement in these diseases with tau inclusion. To search for variants that confer susceptibility to 4 tauopathies and clinically related disorders. Genomewide association study. University research laboratory. A total of 231 samples were genotyped from an unrelated white population of patients with progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), frontotemporal dementia, and frontotemporal dementia with amyotrophy. Unaffected individuals from the same population were used as controls. The results from an inverted region of chromosome 17 that contains the MAPT gene. Genotypes of cases and controls were compared using a Fisher exact test on a marker-by-marker basis. Haplotypes were determined by visually inspecting genotypes. Comparing any particular disease and controls, the association was constant across the inverted chromosome segment. Significant associations were seen for PSP and PSP combined with CBD. Of the 2 haplotypes seen in the region, H1 was overrepresented in PSP and CBD cases compared with controls. As expected, the markers are highly correlated and the association is seen across the entire region, which makes it difficult to narrow down a disease-causing variant or even a possible candidate gene. However, considering the pathologic abnormalities of these diseases and the involvement of tau mutations seen in familial forms, the MAPT gene represents the most likely cause driving the association.

  5. La belle indifférence revisited: a case report on progressive supranuclear palsy misdiagnosed as conversion disorder.

    Science.gov (United States)

    van Meerkerk-Aanen, Petra J; de Vroege, Lars; Khasho, David; Foruz, Aziza; van Asseldonk, J Thies; van der Feltz-Cornelis, Christina M

    2017-01-01

    Since the advent of computed tomography and magnetic resonance imaging scans, neurological disorders have less often been falsely labeled as conversion disorder (CD). However, misdiagnosis of a neurological disorder as CD still occurs, especially in cases with insidious onset. Misinterpretation of la belle indifférence may contribute to such misdiagnosis. Here, we describe a case of progressive supranuclear palsy/Richardson's syndrome (PSPS) misdiagnosed as a case of CD. A 62-year-old woman consulted two different neurologists in 2012 because of falling spells since 2009 and was diagnosed with CD. She was referred to the Clinical Center of Excellence for Body, Mind, and Health for treatment of CD. After neurological examination, blood tests, and psychiatric examination, in which la belle indifférence and a history of incest were found, CD was confirmed. However, despite treatment for CD, the patient's physical symptoms deteriorated over a year. After repeated physical and psychiatric examinations, neurocognitive assessment, and consultation with a third neurologist because of suspicion of neurological disease, the patient was diagnosed with PSPS. La belle indifférence may be a psychological sign in the context of CD, but it may also be an expression of lack of mimic due to Parkinsonism or of eye movement disorder in the context of neurological illness. A diagnosis of CD should not be considered definitive if no improvement occurs in terms of physical, mental, and cognitive symptoms despite appropriate therapy. In case of deterioration, neurological reexamination and reinterpretation of la belle indifférence should be considered.

  6. Evaluation of Functional Disability in ALS

    Directory of Open Access Journals (Sweden)

    Akbar Soltan-Zadeh

    2002-06-01

    Full Text Available Objective: Amyotrophic Lateral Sclerosis (ALS is a progressive degenerative fatal disorder of motor neuron. In this research course of functional disability and possible underlying factors affecting disability were studied. Materials & Methods: First 59 patients with definite ALS were selected and for each patient ALSFRS (ALS Functional Rating Scale was determined at Initial and the end of a six month period. Results: During this period 9 patients expired. Bulbar onset localization showed a more rapid course than non-bulbar onset group. Conclusion: There was no significant relationship between the age group or gender with progression of disability. In expired group, mean survival in bulbar onset and older patients were significantly less than those with non-bulbar onset and younger ones. There was no significant relationship between survival and gender.

  7. Long-Lasting Cranial Nerve III Palsy as a Presenting Feature of Chronic Inflammatory Demyelinating Polyneuropathy

    Directory of Open Access Journals (Sweden)

    Rossella Spataro

    2015-01-01

    Full Text Available We describe a patient with chronic inflammatory demyelinating polyneuropathy (CIDP in which an adduction deficit and ptosis in the left eye presented several years before the polyneuropathy. A 52-year-old man presented with a 14-year history of unremitting diplopia, adduction deficit, and ptosis in the left eye. At the age of 45 a mild bilateral foot drop and impaired sensation in the four limbs appeared, with these symptoms showing a progressive course. The diagnostic workup included EMG/ENG which demonstrated reduced conduction velocity with bilateral and symmetrical sensory and motor involvement. Cerebrospinal fluid studies revealed a cytoalbuminologic dissociation. A prolonged treatment with corticosteroids allowed a significant improvement of the limb weakness. Diplopia and ptosis remained unchanged. This unusual form of CIDP presented as a long-lasting isolated cranial nerve palsy. A diagnostic workup for CIDP should therefore be performed in those patients in which an isolated and unremitting cranial nerve palsy cannot be explained by common causes.

  8. Bell's palsy: excluding serious illness in urgent and emergency care settings.

    Science.gov (United States)

    Mower, Sean

    2017-04-13

    Bell's palsy is a relatively benign condition that affects about 20 in every 100,000 patients a year, and in most cases the signs and symptoms resolve fully within around six months. The defining characteristic of the condition is a unilateral facial palsy, but this is also apparent in other conditions with a more serious prognosis, including strokes, some viral infections and tumours. This article reviews the literature on recognition of Bell's palsy, examines the underlying pathology, and compares it with other conditions associated with facial palsy. The article critically analyses the evidence and guidelines to identify best practice, and considers areas for improvement. Finally, it discusses how this information can be incorporated into practice, and provides guidance for clinicians on differentiating between conditions in which patients present with facial palsy to ensure they are managed appropriately.

  9. Surgical tips and tricks during urethroplasty for bulbar urethral strictures focusing on accurate localisation of the stricture: results from a tertiary centre.

    Science.gov (United States)

    Kuo, Tricia L C; Venugopal, Suresh; Inman, Richard D; Chapple, Christopher R

    2015-04-01

    There are several techniques for characterising and localising an anterior urethral stricture, such as preoperative retrograde urethrography, ultrasonography, and endoscopy. However, these techniques have some limitations. The final determinant is intraoperative assessment, as this yields the most information and defines what surgical procedure is undertaken. We present our intraoperative approach for localising and operating on a urethral stricture, with assessment of outcomes. A retrospective review of urethral strictures operated was carried out. All patients had a bulbar or bulbomembranous urethroplasty. All patients were referred to a tertiary centre and operated on by two urethral reconstructive surgeons. Intraoperative identification of the stricture was performed by cystoscopy. The location of the stricture is demonstrated externally on the urethra by external transillumination of the urethra and comparison with the endoscopic picture. This is combined with accurate placement of a suture through the urethra, at the distal extremity of the stricture, verified precisely by endoscopy. Clinical data were collected in a dedicated database. Intraoperative details and postoperative follow-up data for each patient were recorded and analysed. A descriptive data analysis was performed. A representative group of 35 male patients who had surgery for bulbar stricture was randomly selected from January 2010 to December 2013. Mean follow-up was 13.8 mo (range 2-43 mo). Mean age was 46.5 yr (range 17-70 yr). Three patients had undergone previous urethroplasty and 26 patients had previous urethrotomy or dilatation. All patients had preoperative retrograde urethrography and most (85.7%) had endoscopic assessment. The majority of patients (48.6%) had a stricture length of >2-7 cm and 45.7% of patients required a buccal mucosa graft. There were no intraoperative complications. Postoperatively, two patients had a urinary tract infection. All patients were assessed

  10. Cerebral palsy litigation: change course or abandon ship.

    Science.gov (United States)

    Sartwelle, Thomas P; Johnston, James C

    2015-06-01

    The cardinal driver of cerebral palsy litigation is electronic fetal monitoring, which has continued unabated for 40 years. Electronic fetal monitoring, however, is based on 19th-century childbirth myths, a virtually nonexistent scientific foundation, and has a false positive rate exceeding 99%. It has not affected the incidence of cerebral palsy. Electronic fetal monitoring has, however, increased the cesarian section rate, with the expected increase in mortality and morbidity risks to mothers and babies alike. This article explains why electronic fetal monitoring remains endorsed as efficacious in the worlds' labor rooms and courtrooms despite being such a feeble medical modality. It also reviews the reasons professional organizations have failed to condemn the use of electronic fetal monitoring in courtrooms. The failures of tort reform, special cerebral palsy courts, and damage limits to stem the escalating litigation are discussed. Finally, the authors propose using a currently available evidence rule-the Daubert doctrine that excludes "junk science" from the courtroom-as the beginning of the end to cerebral palsy litigation and electronic fetal monitoring's 40-year masquerade as science. © The Author(s) 2014.

  11. Bell's Palsy (For Kids)

    Science.gov (United States)

    ... palsy was named after a Scottish doctor, Sir Charles Bell, who studied the two facial nerves that ... who focuses on how the nervous system works — will do a test called electromyography (say: eh-lek- ...

  12. Tinjauan Anatomi Klinik dan Manajemen Bell's Palsy

    Directory of Open Access Journals (Sweden)

    Nur Mujaddidah

    2017-07-01

    Full Text Available Bell's Palsy is a peripheral facial nerve weakness (facial nerve with acute onset on one side of the face. This condition causes the inability of the patient to move half of his face consciously (volunter on the affected side. The Bell's Palsy incidence is 20-30 cases out of 100.000 people and accounts for 60-70% of all cases of unilateral facial paralysis. The disease is self-limited, but causes great suffering for patients who are not treated properly. Controversy in the management is still debated, and the cause is still unknown. The underlying hypothesis is ischemic, vascular, viral, bacterial, hereditary, and immunologic. Therapy done so far is to improve facial nerve function and healing process. The management of the therapy used will be closely related to the structure of the anatomy and its functions and associated abnormalities. The modalities of Bell's Palsy therapy are with corticosteroids and antivirals, facial exercises, electrostimulation, physiotherapy and decompression operations. Approximately 80-90% of patients with Bell's palsy recover completely within 6 months, even in 50-60% of cases improved within 3 weeks. Approximately 10% experienced persistent facial muscle asymmetry, and 5% experienced severe sequelae, and 8% of cases were recurrent.

  13. Antenatal and intrapartum interventions for preventing cerebral palsy: an overview of Cochrane systematic reviews.

    Science.gov (United States)

    Shepherd, Emily; Salam, Rehana A; Middleton, Philippa; Makrides, Maria; McIntyre, Sarah; Badawi, Nadia; Crowther, Caroline A

    2017-08-08

    Cerebral palsy is an umbrella term encompassing disorders of movement and posture, attributed to non-progressive disturbances occurring in the developing fetal or infant brain. As there are diverse risk factors and causes, no one strategy will prevent all cerebral palsy. Therefore, there is a need to systematically consider all potentially relevant interventions for their contribution to prevention. To summarise the evidence from Cochrane reviews regarding the effects of antenatal and intrapartum interventions for preventing cerebral palsy. We searched the Cochrane Database of Systematic Reviews on 7 August 2016, for reviews of antenatal or intrapartum interventions reporting on cerebral palsy. Two authors assessed reviews for inclusion, extracted data, assessed review quality, using AMSTAR and ROBIS, and quality of the evidence, using the GRADE approach. We organised reviews by topic, and summarised findings in text and tables. We categorised interventions as effective (high-quality evidence of effectiveness); possibly effective (moderate-quality evidence of effectiveness); ineffective (high-quality evidence of harm or of lack of effectiveness); probably ineffective (moderate-quality evidence of harm or of lack of effectiveness); and no conclusions possible (low- to very low-quality evidence). We included 15 Cochrane reviews. A further 62 reviews pre-specified the outcome cerebral palsy in their methods, but none of the included randomised controlled trials (RCTs) reported this outcome. The included reviews were high quality and at low risk of bias. They included 279 RCTs; data for cerebral palsy were available from 27 (10%) RCTs, involving 32,490 children. They considered interventions for: treating mild to moderate hypertension (two) and pre-eclampsia (two); diagnosing and preventing fetal compromise in labour (one); preventing preterm birth (four); preterm fetal maturation or neuroprotection (five); and managing preterm fetal compromise (one). Quality of

  14. The Clinical Study on Bell's Palsy Patients with TCD Measurement

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    Lee Hyun

    2003-06-01

    Full Text Available Objective : This study was carried to make out the connection between cerebral artery blood flow velocity and ischemic theory that presumed the cause of Bell's palsy. Method : We measured cerebral artery blood flow velocity each external carotid artery, internal carotid artery, common carotid artery, siphon, superficial temporal artery by TCD to 20 patients who diagnosed as facial nerve palsy from march 2001 to July 2001 and all objectives devided two groups as palsy side. A group is right side facial nerve palsy and B group is left facial nerve palsy. Results : 1. There is no effective change of blood flow in external carotid artery either A, B group. 2. There is no effective change of blood flow in internal carotid artery either A, B group. 3. There is no effective change of blood flow in common carotid artery either A, B group. 4. There is no effective change of blood flow in siphon artery either A, B group. 5. There is no effective change of blood flow in superficial temporal artery either A, B group.

  15. Intraneural metastasis of gastric carcinoma leads to sciatic nerve palsy

    International Nuclear Information System (INIS)

    Ichikawa, Jiro; Matsumoto, Seiichi; Shimoji, Takashi; Tanizawa, Taisuke; Gokita, Tabu; Hayakawa, Keiko; Aoki, Kaoru; Ina, Saori; Kanda, Hiroaki

    2012-01-01

    Soft tissue metastases, in particular intraneural metastasis, from any carcinomas seldom occur. To our knowledge, no case of sciatic nerve palsy due to intraneural metastasis of gastric carcinoma is reported in the literature. A case is reported of a 82-year old woman with sciatic nerve palsy with intraneural metastasis of gastric carcinoma. Although she had undergone partial gastrectomy with T2b, N0, M0 two years ago and primary site was cured, she developed sciatic nerve palsy from the carcinoma metastasis directly to the nerve. Operative resection and Histological examination revealed poorly differentiated adenocarcinoma, the same as her primary site adenocarcinoma. Sciatica is usually caused by a herniated disc or spinal canal stenosis. Sciatic nerve palsy may be caused by nondiscogenic etiologies that may be either intrapelvic or extrapelvic. It is important to image the entire course of the nerve to distinguish these etiologies quickly. The longer the nerve compression the less likely a palsy will recover. Surgery is a good intervention that simultaneously obtains a tissue diagnosis and decompresses the nerve

  16. Association between type of cerebral palsy and the cognitive levels

    Directory of Open Access Journals (Sweden)

    Ratna Dewi Kusumaningrum

    2009-07-01

    Conclusion Our data showed that most patients with cerebral palsy had mental retardation of several cognitive level but there was no significant association between each type of cerebral palsy with cognitive levels.

  17. CT findings of cerebral palsy and behaviour development

    International Nuclear Information System (INIS)

    Sakamoto, Zenji

    1987-01-01

    It is well recognized that CT scan is very useful in the early diagnosis of cerebral palsy. The author has studied this time the CT scan findings of cerebral palsy children in their relations to the type of palsy, cause of palsy, complications in the central nervous system, and prognosis of behaviour development, in order to predict the prognosis of behaviour development. Dilatation of the contralateral cerebral ventricle was found in 82 % of hemiplegic type. Abnormal EEG was found in 73 %, but their behaviour development was satisfactory, with good development of speech regardless to the side of palsy. This might be helped by compensational function of the brain due to plasticity. Diplegia presented bilateral moderate dilatation of ventricles with favorable prognosis. Tetraplegia was caused mostly by asphyxia or congenital anomaly and revealed marked dilatation of ventricles or severe cortical atrophy. Some cases presented diffuse cortical low-density, often associated with abnormal EEG, and their prognosis was worst. Athetosis had normal CT finding or mild ventricular dilatation, but all cases of ataxia presented normal CT findings. Hypotonia had mild ventricular dilatation. Two of three mixed type cases had normal CT findings and another had mild ventricular dilatation. No correlation was found between ventricular dilatation and behaviour development, but statistically significant difference was found in the cases with 30 % or more Evans' ratio (P < 0.05). Prognosis of severe ventricular dilatation cases was poor. (author)

  18. [Isolated palsy of the hypoglossal nerve complicating infectious mononucleosis].

    Science.gov (United States)

    Carra-Dallière, C; Mernes, R; Juntas-Morales, R

    2011-01-01

    Neurological complications of infectious mononucleosis are rare. Various disorders have been described: meningitis, encephalitis, peripheral neuropathy. Isolated cranial nerve palsy has rarely been reported. A 16-year-old man was admitted for isolated and unilateral hypoglossal nerve palsy, four weeks after infectious mononucleosis. Cerebral MRI, cerebrospinal fluid study and electromyography were normal. IgM anti-VCA were positive. Two months later, without treatment, the tongue had almost fully recovered. To the best of our knowledge, only seven cases of isolated palsy of the hypoglossal nerve complicating infectious mononucleosis have been previously reported. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  19. Subcortical frontal lesions on MRI in patients with motor neurone disease

    Energy Technology Data Exchange (ETDEWEB)

    Andreadou, E.; Sgouropoulos, P.; Varelas, P.; Papageorgiou, C. [Eginition Hospital, Athens (Greece); Gouliamos, A. [Department of Radiology, CT/MRI Unit, Areteion Hospital, University of Athens (Greece)

    1998-05-01

    MRI was performed in 32 patients with motor neurone disease (26 men and 6 women, aged 40-77 years) and in a control group of 21 subjects. Of the patients studied, 19 had definite and 11 probable amyotrophic lateral sclerosis (ALS) and two had progressive bulbar palsy. In 10 patients there were asymmetrical bilateral foci of increased signal intensity on proton-density and T{sub 2}-weighted images, confined to the white matter. Two patients had only cortical frontal atrophy and slightly increased ventricular size, whereas 20 had normal MRI. The focal lesions were not confined to corticospinal tracts, but were also observed in subcortical frontal areas. While the lesions along the corticospinal tracts correspond to pyramidal tract degeneration, the subcortical foci correlate with degeneration of the frontal bundles and indicate generalised involvement of the central nervous system. (orig.) With 3 figs., 2 tabs., 25 refs.

  20. Increasing rates of cerebral palsy across the severity spectrum in north-east England 1964-1993. The North of England Collaborative Cerebral Palsy Survey.

    Science.gov (United States)

    Colver, A F; Gibson, M; Hey, E N; Jarvis, S N; Mackie, P C; Richmond, S

    2000-07-01

    To report epidemiological trends in cerebral palsy including analyses by severity. Descriptive longitudinal study in north-east England. Every child with suspected cerebral palsy was examined by a developmental paediatrician to confirm the diagnosis. Severity of impact of disability was derived from a parent completed questionnaire already developed and validated for this purpose. All children with cerebral palsy, not associated with any known postneonatal insult, born 1964-1993 to mothers resident at the time of birth in the study area. Cerebral palsy rates by year, birth weight, and severity. Severity of 30% and above defines the more reliably ascertained cases; children who died before assessment at around 6 years of age are included in the most severe group (70% and above). 584 cases of cerebral palsy were ascertained, yielding a rate that rose from 1.68 per 1000 neonatal survivors during 1964-1968 to 2.45 during 1989-1993 (rise = 0.77; 95% confidence interval 0.2-1.3). For the more reliably ascertained cases there was a twofold increase in rate from 0.98 to 1.96 (rise = 0.98; 95% confidence interval 0.5-1.4). By birth weight, increases in rates were from 29.8 to 74.2 per 1000 neonatal survivors rate of cerebral palsy has risen in spite of falling perinatal and neonatal mortality rates, a rise that is even more pronounced when the mildest and least reliably ascertained are excluded. The effect of modern care seems to be that many babies rates, and to monitor changes in the distribution of severity.

  1. Bulbar urethroplasty using the dorsal approach: current techniques

    Directory of Open Access Journals (Sweden)

    Barbagli Guido

    2003-01-01

    Full Text Available INTRODUCTION: The use of flaps or grafts is mandatory in patients with longer and complex strictures. In 1995-96 we described a new dorsal onlay graft urethroplasty. Over time, our original technique was better defined and changed. Now this procedure (also named Barbagli technique has been greeted with a fair amount of enthusiasm in Europe and in the United States. SURGICAL TECHNIQUE: The patient is placed in normal lithotomy position, and a midline perineo-scrotal incision is made. The bulbar urethra is then free from the bulbo-cavernous muscles, and is dissected from the corpora cavernosa. The urethra is completely mobilized from the corpora cavernosa, it is rotated 180 degrees, and is incised along its dorsal surface. The graft (preputial skin or buccal mucosa or the flap is fixed and quilted to the tunica albuginea of the corporal bodies. The right mucosal margin of the opened urethra is sutured to the right side of the patch-graft. The urethra is rotated back into its original position. The left urethral margin is sutured to the left side of the patch graft and to the corporal bodies, and the grafted area is entirely covered by the urethral plate. The bulbo-cavernous muscles are approximated over the grafted area. A 16F silicone Foley catheter is left in place. COMMENTS: Dorsal onlay graft urethroplasty is a versatile procedure that may be combined with various substitute materials like preputial skin, buccal mucosa grafts or pedicled flaps.

  2. Nongoitrous autoimmune thyroiditis with facial palsy

    Directory of Open Access Journals (Sweden)

    Hyung Jik Lee

    2013-12-01

    Full Text Available We report a case of severe hypothyroidism with nongoitrous, autoimmune thyroiditis and pituitary hyperplasia in a 13-year-old boy, who presented with sudden palsy on the left side of his face. Prednisolone and antiviral medication was administered. However, the facial palsy did not improve completely. The medications were replaced with thyroxine, and the facial palsy recovered. Endocrinological testing showed severe hypothyroidism as follows: thyroid stimulating hormone (TSH level >100 µIU/mL, T4 of 1.04 µg/dL, T3 of 0.31 ng/mL, and free T4 of 0.07 ng/dL. Level of serum antithyroid peroxidase antibodies was 1,933.39 IU/mL, and that of antithyroglobulin antibodies was 848.16 IU/mL. Level of TSH receptor antibodies was >40 IU/L. Bioassay result for TSH receptor stimulating antibodies was negative. Thyroid sonography revealed no increase in the size or vascularity of the bilateral gland. Thyroid scintigraphy with 99mTc showed decreased uptake, and magnetic resonance imaging demonstrated an enlarged pituitary gland.

  3. Hypopituitarism in children with cerebral palsy.

    Science.gov (United States)

    Uday, Suma; Shaw, Nick; Krone, Ruth; Kirk, Jeremy

    2017-06-01

    Poor growth and delayed puberty in children with cerebral palsy is frequently felt to be related to malnutrition. Although growth hormone deficiency is commonly described in these children, multiple pituitary hormone deficiency (MPHD) has not been previously reported. We present a series of four children with cerebral palsy who were born before 29 weeks gestation who were referred to the regional endocrinology service, three for delayed puberty and one for short stature, in whom investigations identified MPHD. All patients had a height well below -2 standard deviation score (2nd centile) at presentation and three who had MRI scans had an ectopic posterior pituitary gland. We therefore recommend that the possibility of MPHD should be considered in all children with cerebral palsy and poor growth or delayed puberty. Early diagnosis and treatment is essential to maximise growth and prevent associated morbidity and mortality. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  4. Health-related physical fitness for children with cerebral palsy

    Science.gov (United States)

    Maltais, Désirée B.; Wiart, Lesley; Fowler, Eileen; Verschuren, Olaf; Damiano, Diane L.

    2014-01-01

    Low levels of physical activity are a global health concern for all children. Children with cerebral palsy have even lower physical activity levels than their typically developing peers. Low levels of physical activity, and thus an increased risk for related chronic diseases, are associated with deficits in health-related physical fitness. Recent research has provided therapists with the resources to effectively perform physical fitness testing and physical activity training in clinical settings with children who have cerebral palsy, although most testing and training data to date pertains to those who walk. Nevertheless, based on the present evidence, all children with cerebral palsy should engage, to the extent they are able, in aerobic, anaerobic and muscle strengthening activities. Future research is required to determine the best ways to evaluate health-related physical fitness in non-ambulatory children with cerebral palsy and foster long-term changes in physical activity behavior in all children with this condition. PMID:24820339

  5. Multiple Cranial Neuropathies Without Limb Involvements: Guillain-Barre Syndrome Variant?

    OpenAIRE

    Yu, Ju Young; Jung, Han Young; Kim, Chang Hwan; Kim, Hyo Sang; Kim, Myeong Ok

    2013-01-01

    Acute multiple cranial neuropathies are considered as variant of Guillain-Barre syndrome, which are immune-mediated diseases triggered by various cases. It is a rare disease which is related to infectious, inflammatory or systemic diseases. According to previous case reports, those affected can exhibit almost bilateral facial nerve palsy, then followed by bulbar dysfunctions (cranial nerves IX and X) accompanied by limb weakness and walking difficulties due to motor and/or sensory dysfunction...

  6. Bladder and Bowel Control in Children with Cerebral Palsy: Case-Control Study

    Science.gov (United States)

    Ozturk, Mustafa; Oktem, Faruk; Kisioglu, Nesimi; Demirci, Mustafa; Altuntas, Irfan; Kutluhan, Suleyman; Dogan, Malik

    2006-01-01

    Aim To determine the age of development of bladder and bowel control and the frequency of enuresis, encopresis, and urinary infections in children with cerebral palsy. Methods The study included 45 children with cerebral palsy who regularly attended a rehabilitation center in Isparta, Turkey, and two groups of age- and sex-matched children, 37 siblings of the children with cerebral palsy and 37 healthy children. Demographic data and information on the age of development of total bladder and bowel control and presence of possible urinary symptoms in children were collected from their caregivers by use of a questionnaire. Frequency of enuresis and encopresis was estimated among the children aged ≥5 years. A mid-way urinary sample was obtained from 40, 22, and 21 children in the cerebral palsy, siblings, and healthy children, respectively. Results The mean age of nighttime bladder and bowel control development was 47 months (95% confidence interval [CI], 35-58) and 45 (36-55) months, respectively, for the children with cerebral palsy, 35 months (95% CI, 24-46) and 26 months (95% CI, 24-28), respectively, for their siblings, and 27 months (95% CI, 22-33) and 25 months (95% CI, 23-27) months, respectively, for the healthy children. Among the children aged ≥5 years, enuresis was present in 11 of 34 children with cerebral palsy, 7 of 30 siblings, and 4 of 30 healthy children (P = 0.200), whereas encopresis was present in 5 children with cerebral palsy, one sibling, and one healthy child. Constipation was significantly more present in chidlren with cerebral palsy than in other two groups (P<0.001). Urine culture was positive in 13 children with cerebral palsy, 1 sibling, and 2 healthy chidlren (P = 0.024). There were no significant differences in other urinary symptoms and laboratory findings among the three groups. Conclusion The children with cerebral palsy gained bladder and bowel control at older age in comparison with their siblings and healthy children

  7. Storytelling: Enhancing Vocabularies For Cerebral Palsy Students

    OpenAIRE

    Aprilina, Raita Gina

    2015-01-01

    This paper reported on a study concerned with teaching vocabulary using storytelling technique in one of SLBs in Bandung. This study aimed to find out the cerebral palsy students' ability in English vocabulary before and after the treatment, and to find out whether storytelling significantly improved English vocabulary of students with cerebral palsy. This study used an experimental method with single subject research with A-B-A design which involved two participants. This study revealed that...

  8. Peripheral facial weakness (Bell's palsy).

    Science.gov (United States)

    Basić-Kes, Vanja; Dobrota, Vesna Dermanović; Cesarik, Marijan; Matovina, Lucija Zadro; Madzar, Zrinko; Zavoreo, Iris; Demarin, Vida

    2013-06-01

    Peripheral facial weakness is a facial nerve damage that results in muscle weakness on one side of the face. It may be idiopathic (Bell's palsy) or may have a detectable cause. Almost 80% of peripheral facial weakness cases are primary and the rest of them are secondary. The most frequent causes of secondary peripheral facial weakness are systemic viral infections, trauma, surgery, diabetes, local infections, tumor, immune disorders, drugs, degenerative diseases of the central nervous system, etc. The diagnosis relies upon the presence of typical signs and symptoms, blood chemistry tests, cerebrospinal fluid investigations, nerve conduction studies and neuroimaging methods (cerebral MRI, x-ray of the skull and mastoid). Treatment of secondary peripheral facial weakness is based on therapy for the underlying disorder, unlike the treatment of Bell's palsy that is controversial due to the lack of large, randomized, controlled, prospective studies. There are some indications that steroids or antiviral agents are beneficial but there are also studies that show no beneficial effect. Additional treatments include eye protection, physiotherapy, acupuncture, botulinum toxin, or surgery. Bell's palsy has a benign prognosis with complete recovery in about 80% of patients, 15% experience some mode of permanent nerve damage and severe consequences remain in 5% of patients.

  9. Neuro-ophthalmological approach to facial nerve palsy.

    Science.gov (United States)

    Portelinha, Joana; Passarinho, Maria Picoto; Costa, João Marques

    2015-01-01

    Facial nerve palsy is associated with significant morbidity and can have different etiologies. The most common causes are Bell's palsy, Ramsay-Hunt syndrome and trauma, including surgical trauma. Incidence varies between 17 and 35 cases per 100,000. Initial evaluation should include accurate clinical history, followed by a comprehensive investigation of the head and neck, including ophthalmological, otological, oral and neurological examination, to exclude secondary causes. Routine laboratory testing and diagnostic imaging is not indicated in patients with new-onset Bell's palsy, but should be performed in patients with risk factors, atypical cases or in any case without resolution within 4 months. Many factors are involved in determining the appropriate treatment of these patients: the underlying cause, expected duration of nerve dysfunction, anatomical manifestations, severity of symptoms and objective clinical findings. Systemic steroids should be offered to patients with new-onset Bell's palsy to increase the chance of facial nerve recovery and reduce synkinesis. Ophthalmologists play a pivotal role in the multidisciplinary team involved in the evaluation and rehabilitation of these patients. In the acute phase, the main priority should be to ensure adequate corneal protection. Treatment depends on the degree of nerve lesion and on the risk of the corneal damage based on the amount of lagophthalmos, the quality of Bell's phenomenon, the presence or absence of corneal sensitivity and the degree of lid retraction. The main therapy is intensive lubrication. Other treatments include: taping the eyelid overnight, botulinum toxin injection, tarsorrhaphy, eyelid weight implants, scleral contact lenses and palpebral spring. Once the cornea is protected, longer term planning for eyelid and facial rehabilitation may take place. Spontaneous complete recovery of Bell's palsy occurs in up to 70% of cases. Long-term complications include aberrant regeneration with

  10. The Clinical Study about Honilirls Placenta Herbal Acupuncture on Bell's Palsy

    Directory of Open Access Journals (Sweden)

    Lee Chae-Woo

    2005-12-01

    Full Text Available Objectives : This study has been designed and performed to identify the effect on Bell's palsy according to the injection of herbal medicine induced from the Hominis Placenta. Methods : We measured the facial palsy changes of the patients who were admitted for Bell's palsy in the Oriental Medical hospital of Dongeui medical center from 03-01-2004 to 07-31-2004. Bell's palsy patients were divided into two groups. One group(A group was injected with Hominis Placenta herbal acupuncture. The other group(B group was injected with normal saline. Then effects of these treatment was evaluated by Yanagihara's unweighted grading system. Results : A group was marked more higher than B group in treatment outcome. we discovered that it is significant differences between two groups after 4 week. Conclusions : These results provided that A group is more effective than B group. For clearly proving the effect of Hominis Placenta herbal acupuncture on Bell's palsy, it is need more sample's number and more treatment's duration.

  11. ocular findings in children with cerebral palsy attending a tertiary

    African Journals Online (AJOL)

    ocular abnormalities among children with cerebral palsy that attended the neurology clinic of University of. Ilorin Teaching ... recognize faces or hand-held toys (Chen, Weinberg and Catalano ... palsy that is also blind/visually impaired pose a.

  12. Pain symptoms in patients with severe cerebral palsy: Prevalence ...

    African Journals Online (AJOL)

    Purpose: To evaluate the presence of pain in patients diagnosed with severe cerebral palsy (CP) according to the degree of motor function impairment. Methods: A cross-sectional study was conducted on students of the Association of Parents and Friends of Exceptional Children (APAE) diagnosed with cerebral palsy and ...

  13. Enhanced MRI in patients with facial palsy; Study of time-related enhancement

    Energy Technology Data Exchange (ETDEWEB)

    Yanagida, Masahiro; Kato, Tsutomu; Ushiro, Koichi; Kitajiri, Masanori; Yamashita, Toshio; Kumazawa, Tadami; Tanaka, Yoshimasa (Kansai Medical School, Moriguchi, Osaka (Japan))

    1991-03-01

    We performed Gd-DTPA-enhanced magnetic resonance imaging (MRI) examinations at several stages in 40 patients with peripheral facial nerve palsy (Bell's palsy and Ramsay-Hunt syndrome). In 38 of the 40 patients, one and more enhanced region could be seen in certain portion of the facial nerve in the temporal bone on the affected side, whereas no enhanced regions were seen on the intact side. Correlations between the timing of the MRI examination and the location of the enhanced regions were analysed. In all 6 patients examined by MRI within 5 days after the onset of facial nerve palsy, enhanced regions were present in the meatal portion. In 3 of the 8 patients (38%) examined by MRI 6 to 10 days after the onset of facial palsy, enhanced areas were seen in both the meatal and labyrinthine portions. In 8 of the 9 patients (89%) tested 11 to 20 days after the onset of palsy, the vertical portion was enhanced. In the 12 patients examined by MRI 21 to 40 days after the onset of facial nerve palsy, the meatal portion was not enhanced while the labyrinthine portion, the horizontal portion and the vertical portion were enhanced in 5 (42%), 8 (67%) and 11 (92%), respectively. Enhancement in the vertical portion was observed in all 5 patients examined more than 41 days after the onset of facial palsy. These results suggest that the central portion of the facial nerve in the temporal bone tends to be enhanced in the early stage of facial nerve palsy, while the peripheral portion is enhanced in the late stage. These changes of Gd-DTPA enhanced regions in the facial nerve may suggest dromic degeneration of the facial nerve in peripheral facial nerve palsy. (author).

  14. Anastomic urethroplasty in bulbar urethral stricture. 13 years experience in a department of urology.

    Science.gov (United States)

    Sáez-Barranquero, F; Herrera-Imbroda, B; Yáñez-Gálvez, A; Sánchez-Soler, N; Castillo-Gallardo, E; Cantero-Mellado, J A; Julve-Villalta, E; Machuca-Santa Cruz, F J

    2016-01-01

    Urethral stenosis is a common disease in the clinical practice of urology, with a major impact on the quality of life of patients. The anastomotic urethroplasty is a technique with very precise indications usually membranous or bulbar urethra stenosis with a length of 3 cm or up to 7 cm when it is secondary to urethral disruptions (no stenosis) after pelvic trauma. We review anastomotic urethroplasty performed in our department between 2002 and 2015. A retrospective, descriptive and inferential analysis on 107 patients out of 482 treated with Anastomotic urethroplasty by urethral strictures at the Urology Department of the Hospital "Virgen de la Victoria" (Malaga) from January 2002 to September 2015, establishing effectiveness and safety of the technique, as well as factors that might influence the results. The main diagnostic method was retrograde urethrography and voiding cystourethrography in 100% of patients undergoing surgery, using voiding uroflowmetry for subsequent monitoring. The definition of success was a postoperative flowmetry with Qmax>15 ml/s, and in case of lower flow, we perform a cystoscopy to verify recurrence of stenosis or exclude other pathology. The median age was 42 years, with a mean follow up of 59 months. The length of stenosis valued by retrograde urethrography and voiding cystourethrography was in 91.6% of cases of >1 cm and urethroplasty was the initial treatment, followed in frequency by direct vision internal urethrotomy 9.3%. In the case of comorbidities associated with treatment with anastomotic urethroplasty it was observed that only Diabetes Mellitus had a tendency to statistical significance, with p=0.092, not demonstrating such significance in the case of hypertension or when the subject presented Diabetes Mellitus together with hypertension. Finally, the intervention was successful in 102 cases (95.3%), with only 5 cases (4.7%) where it failed, 4 of them treated with a new Anastomotic urethroplasty, with resolution of the

  15. [18F]-THK5351 PET Correlates with Topology and Symptom Severity in Progressive Supranuclear Palsy

    Directory of Open Access Journals (Sweden)

    Matthias Brendel

    2018-01-01

    Full Text Available Progressive supranuclear palsy (PSP is a neurodegenerative movement disorder characterized by deposition of fibrillar aggregates of 4R tau-protein in neurons and glial cells of the brain. These deposits are a key neuropathological finding, allowing a diagnosis of “definite PSP,” which is usually established post mortem. To date criteria for clinical diagnosis of PSP in vivo do not include biomarkers of tau pathology. For intervention trials, it is increasingly important to (i establish biomarkers for an early diagnosis and (ii to develop biomarkers that correlate with disease progression of PSP. [18F]-THK5351 is a novel PET-ligand that may afford in vivo visualization and quantification of tau-related alterations. We investigated binding characteristics of [18F]-THK5351 in patients with clinically diagnosed PSP and correlate tracer uptake with clinical findings. Eleven patients (68.4 ± 7.4 year; N = 6 female with probable PSP according to current clinical criteria and nine healthy controls (71.7 ± 7.2 year; N = 4 female underwent [18F]-THK5351 PET scanning. Voxel-wise statistical parametric comparison and volume-of-interest based quantification of standardized-uptake-values (SUV were conducted using the cerebellar cortex as reference region. We correlated disease severity as measured with the help of the PSP Rating Scale (PSPRS as well as several other clinical parameters with the individual PET findings. By voxel-wise mapping of [18F]-THK5351 uptake in the patient group we delineated typical distribution patterns that fit to known tau topology for PSP post mortem. Quantitative analysis indicated the strongest discrimination between PSP patients and healthy controls based on tracer uptake in the midbrain (+35%; p = 3.01E-7; Cohen's d: 4.0, followed by the globus pallidus, frontal cortex, and medulla oblongata. Midbrain [18F]-THK5351 uptake correlated well with clinical severity as measured by PSPRS (R = 0.66; p = 0.026. OCT and MRI

  16. The Association Between Maternal Age and Cerebral Palsy Risk Factors.

    Science.gov (United States)

    Schneider, Rilla E; Ng, Pamela; Zhang, Xun; Andersen, John; Buckley, David; Fehlings, Darcy; Kirton, Adam; Wood, Ellen; van Rensburg, Esias; Shevell, Michael I; Oskoui, Maryam

    2018-05-01

    Advanced maternal age is associated with higher frequencies of antenatal and perinatal conditions, as well as a higher risk of cerebral palsy in offspring. We explore the association between maternal age and specific cerebral palsy risk factors. Data were extracted from the Canadian Cerebral Palsy Registry. Maternal age was categorized as ≥35 years of age and less than 20 years of age at the time of birth. Chi-square and multivariate logistic regressions were performed to calculate odds ratios and their 95% confidence intervals. The final sample consisted of 1391 children with cerebral palsy, with 19% of children having mothers aged 35 or older and 4% of children having mothers below the age of 20. Univariate analyses showed that mothers aged 35 or older were more likely to have gestational diabetes (odds ratio 1.9, 95% confidence interval 1.3 to 2.8), to have a history of miscarriage (odds ratio 1.8, 95% confidence interval 1.3 to 2.4), to have undergone fertility treatments (odds ratio 2.4, 95% confidence interval 1.5 to 3.9), and to have delivered by Caesarean section (odds ratio 1.6, 95% confidence interval 1.2 to 2.2). These findings were supported by multivariate analyses. Children with mothers below the age of 20 were more likely to have a congenital malformation (odds ratio 2.4, 95% confidence interval 1.4 to 4.2), which is also supported by multivariate analysis. The risk factor profiles of children with cerebral palsy vary by maternal age. Future studies are warranted to further our understanding of the compound causal pathways leading to cerebral palsy and the observed greater prevalence of cerebral palsy with increasing maternal age. Copyright © 2018 Elsevier Inc. All rights reserved.

  17. Quality of Arithmetic Education for Children with Cerebral Palsy

    Science.gov (United States)

    Jenks, Kathleen M.; de Moor, Jan; van Lieshout, Ernest C. D. M.; Withagen, Floortje

    2010-01-01

    The aim of this exploratory study was to investigate the quality of arithmetic education for children with cerebral palsy. The use of individual educational plans, amount of arithmetic instruction time, arithmetic instructional grouping, and type of arithmetic teaching method were explored in three groups: children with cerebral palsy (CP) in…

  18. De novo point mutations in patients diagnosed with ataxic cerebral palsy.

    Science.gov (United States)

    Parolin Schnekenberg, Ricardo; Perkins, Emma M; Miller, Jack W; Davies, Wayne I L; D'Adamo, Maria Cristina; Pessia, Mauro; Fawcett, Katherine A; Sims, David; Gillard, Elodie; Hudspith, Karl; Skehel, Paul; Williams, Jonathan; O'Regan, Mary; Jayawant, Sandeep; Jefferson, Rosalind; Hughes, Sarah; Lustenberger, Andrea; Ragoussis, Jiannis; Jackson, Mandy; Tucker, Stephen J; Németh, Andrea H

    2015-07-01

    Cerebral palsy is a sporadic disorder with multiple likely aetiologies, but frequently considered to be caused by birth asphyxia. Genetic investigations are rarely performed in patients with cerebral palsy and there is little proven evidence of genetic causes. As part of a large project investigating children with ataxia, we identified four patients in our cohort with a diagnosis of ataxic cerebral palsy. They were investigated using either targeted next generation sequencing or trio-based exome sequencing and were found to have mutations in three different genes, KCNC3, ITPR1 and SPTBN2. All the mutations were de novo and associated with increased paternal age. The mutations were shown to be pathogenic using a combination of bioinformatics analysis and in vitro model systems. This work is the first to report that the ataxic subtype of cerebral palsy can be caused by de novo dominant point mutations, which explains the sporadic nature of these cases. We conclude that at least some subtypes of cerebral palsy may be caused by de novo genetic mutations and patients with a clinical diagnosis of cerebral palsy should be genetically investigated before causation is ascribed to perinatal asphyxia or other aetiologies. © The Author (2015). Published by Oxford University Press on behalf of the Guarantors of Brain.

  19. Laser Phototherapy As Modality of Clinical Treatment in Bell's Palsy

    Science.gov (United States)

    Marques, A. M. C.; Soares, L. G. P.; Marques, R. C.; Pinheiro, A. L. B.; Dent, M.

    2011-08-01

    Bell's palsy is defined as a peripheral facial nerve palsy, idiophatic, and sudden onset and is considered the most common cause of this pathology. It is caused by damage to cranial nerves VII, resulting in complete or partial paralysis of the facial mimic. May be associated with taste disturbances, salivation, tearing and hyperacusis. It is diagnosed after ruling out all possible etiologies, because its cause is not fully understood.Some researches shows that herpes virus may cause this type of palsy due to reactivation of the virus or by imunnomediated post-viral nerve demielinization. Physical therapy, corticosteroids and antiviral therapy have become the most widely accepted treatments for Bell's palsy. Therapy with low-level laser (LLLT) may induce the metabolism of injured nerve tissue for the production of proteins associated with its growth and to improve nerve regeneration. The success of the treatment of Bell's palsy by using laser phototherapy isolated or in association with other therapeutic approach has been reported on the literature. In most cases, the recovery occurs without uneventfully (complications), the acute illness is not associated with serious disorders. We will present a clinical approach for treating this condition.

  20. Motor learning curve and long-term effectiveness of modified constraint-induced movement therapy in children with unilateral cerebral palsy: a randomized controlled trial

    NARCIS (Netherlands)

    Geerdink-van Nistelrooij, Y.A. van; Aarts, P.; Geurts, A.C.H.

    2013-01-01

    The goal of this study was to determine the progression of manual dexterity during 6 weeks (54h) (modified) constraint-induced movement therapy ((m)CIMT) followed by 2 weeks (18h) bimanual training (BiT) in children with unilateral spastic cerebral palsy (CP), to establish whether and when a maximal

  1. Increased risk of peripheral arterial occlusive disease in patients with Bell's palsy using population data.

    Directory of Open Access Journals (Sweden)

    Li-Syue Liou

    Full Text Available This population-based cohort study investigated the risk of developing peripheral arterial occlusive disease (PAOD in patients with Bell's palsy.We used longitudinal claims data of health insurance of Taiwan to identify 5,152 patients with Bell's palsy newly diagnosed in 2000-2010 and a control cohort of 20,608 patients without Bell's palsy matched by propensity score. Incidence and hazard ratio (HR of PAOD were assessed by the end of 2013.The incidence of PAOD was approximately 1.5 times greater in the Bell's palsy group than in the non-Bell's palsy controls (7.75 vs. 4.99 per 1000 person-years. The Cox proportional hazards regression analysis measured adjusted HR was 1.54 (95% confidence interval (CI = 1.35-1.76 for the Bell's palsy group compared to the non-Bell's palsy group, after adjusting for sex, age, occupation, income and comorbidities. Men were at higher risk of PAOD than women in the Bell's palsy group, but not in the controls. The incidence of PAOD increased with age in both groups, but the Bell's palsy group to control group HR of PAOD decreased as age increased. The systemic steroid treatment reduced 13% of PAOD hazard for Bell's palsy patients, compared to those without the treatment, but not significant.Bell's palsy appears to be associated with an increased risk of developing PAOD. Further pathophysiologic, histopathology and immunologic research is required to explore the underlying biologic mechanism.

  2. Risk Factors for Malnutrition Among Children With Cerebral Palsy in Botswana.

    Science.gov (United States)

    Johnson, Allison; Gambrah-Sampaney, Claudia; Khurana, Esha; Baier, James; Baranov, Esther; Monokwane, Baphaleng; Bearden, David R

    2017-05-01

    Children with cerebral palsy in low-resource settings are at high risk of malnutrition, which further increases their risk of poor health outcomes. However, there are few available data on specific risk factors for malnutrition among children with cerebral palsy in the developing world. We performed a case-control study among children with cerebral palsy receiving care at a tertiary care hospital in Gaborone, Botswana. Children with cerebral palsy and malnutrition were identified according to World Health Organization growth curves and compared with subjects with cerebral palsy without malnutrition. Risk factors for malnutrition were identified using multivariable logistic regression models. These risk factors were then used to generate a Malnutrition Risk Score, and Receiver Operating Characteristic curves were used to identify optimal cutoffs to identify subjects at high risk of malnutrition. We identified 61 children with cerebral palsy, 26 of whom (43%) met criteria for malnutrition. Nonambulatory status (odds ratio 13.8, 95% confidence interval [CI] 3.8-50.1, P malnutrition. A Malnutrition Risk Score was constructed based on these risk factors, and receiver operating characteristic curve analysis demonstrated excellent performance characteristics of this score (area under the curve 0.92, 95% CI 0.89-0.94). Malnutrition is common among children with cerebral palsy in Botswana, and a simple risk score may help identify children with the highest risk. Further studies are needed to validate this screening tool and to determine optimal nutritional interventions in this population. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Diagnosis and management of patients with Bell's palsy.

    Science.gov (United States)

    Mooney, Tracy

    Bell's palsy (idiopathic facial paralysis) is the most common cause of acute unilateral facial nerve paralysis. Although it is usually a self-limiting condition, it can be distressing for the patient. Many people who experience one-sided facial paralysis fear that it is a symptom of stroke. However, there are subtle differences between Bell's palsy and stroke. This article discusses potential causes of the condition and identifies the differences between Bell's palsy and stroke. In addition, appropriate strategies for the care of patients with the condition are suggested. Management includes antiviral medication, corticosteroid therapy, eye care, botulinum toxin type A injection, physiotherapy, surgery and acupuncture. Psychological and emotional care of these patients is also important because any facial disability caused by facial nerve paralysis can result in anxiety and stress.

  4. The neuropathology of hereditary congenital facial palsy vs Mobius syndrome.

    NARCIS (Netherlands)

    Verzijl, H.T.F.M.; Zwaag, B. van der; Lammens, M.M.Y.; Donkelaar, H.J. ten; Padberg, G.W.A.M.

    2005-01-01

    OBJECTIVE: To characterize the neuropathology of hereditary congenital facial palsy. METHODS: The authors compared brainstem pathology of three members of one family with autosomal dominant congenital facial palsy to that in three age-matched controls. The neuropathologic findings of the familial

  5. The First Experience of Triple Nerve Transfer in Proximal Radial Nerve Palsy.

    Science.gov (United States)

    Emamhadi, Mohammadreza; Andalib, Sasan

    2018-01-01

    Injury to distal portion of posterior cord of brachial plexus leads to palsy of radial and axillary nerves. Symptoms are usually motor deficits of the deltoid muscle; triceps brachii muscle; and extensor muscles of the wrist, thumb, and fingers. Tendon transfers, nerve grafts, and nerve transfers are options for surgical treatment of proximal radial nerve palsy to restore some motor functions. Tendon transfer is painful, requires a long immobilization, and decreases donor muscle strength; nevertheless, nerve transfer produces promising outcomes. We present a patient with proximal radial nerve palsy following a blunt injury undergoing triple nerve transfer. The patient was involved in a motorcycle accident with complete palsy of the radial and axillary nerves. After 6 months, on admission, he showed spontaneous recovery of axillary nerve palsy, but radial nerve palsy remained. We performed triple nerve transfer, fascicle of ulnar nerve to long head of the triceps branch of radial nerve, flexor digitorum superficialis branch of median nerve to extensor carpi radialis brevis branch of radial nerve, and flexor carpi radialis branch of median nerve to posterior interosseous nerve, for restoration of elbow, wrist, and finger extensions, respectively. Our experience confirmed functional elbow, wrist, and finger extensions in the patient. Triple nerve transfer restores functions of the upper limb in patients with debilitating radial nerve palsy after blunt injuries. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. Validation of mobile eye tracking as novel and efficient means for differentiating progressive supranuclear palsy from Parkinson’s disease

    Directory of Open Access Journals (Sweden)

    Svenja eMarx

    2012-12-01

    Full Text Available Background: The decreased ability to carry out vertical saccades is a key symptom of Progressive Supranuclear Palsy (PSP. Objective measurement devices can help to reliably detect subtle eye-movement disturbances to improve sensitivity and specificity of the clinical diagnosis. The present study aims at transferring findings from restricted stationary video-oculography to a wearable head-mounted device, which can be readily applied in clinical practice.Methods: We investigated the eye movements in 10 possible or probable PSP patients, 11 Parkinson’s disease (PD patients and 10 age-matched healthy controls (HC using a mobile, gaze-driven video camera setup (EyeSeeCam. Ocular movements were analyzed during a standardized fixation protocol and in an unrestricted real-life scenario while walking along a corridor.Results: The EyeSeeCam detected prominent impairment of both saccade velocity and amplitude in PSP patients, differentiating them from PD and HCs. Differences were particularly evident for saccades in the vertical plane, and stronger for saccades than for other eye movements. Differences were more pronounced during the standardized protocol than in the real-life scenario. Conclusions: Combined analysis of saccade velocity and saccade amplitude during the fixation protocol with the EyeSeeCam provides a simple, rapid (< 20s and reliable tool to differentiate clinically established PSP patients from PD and HCs. As such, our findings prepare the ground for using wearable eye-tracking in patients with uncertain diagnoses.

  7. The asymmetric facial skin perfusion distribution of Bell's palsy discovered by laser speckle imaging technology.

    Science.gov (United States)

    Cui, Han; Chen, Yi; Zhong, Weizheng; Yu, Haibo; Li, Zhifeng; He, Yuhai; Yu, Wenlong; Jin, Lei

    2016-01-01

    Bell's palsy is a kind of peripheral neural disease that cause abrupt onset of unilateral facial weakness. In the pathologic study, it was evidenced that ischemia of facial nerve at the affected side of face existed in Bell's palsy patients. Since the direction of facial nerve blood flow is primarily proximal to distal, facial skin microcirculation would also be affected after the onset of Bell's palsy. Therefore, monitoring the full area of facial skin microcirculation would help to identify the condition of Bell's palsy patients. In this study, a non-invasive, real time and full field imaging technology - laser speckle imaging (LSI) technology was applied for measuring facial skin blood perfusion distribution of Bell's palsy patients. 85 participants with different stage of Bell's palsy were included. Results showed that Bell's palsy patients' facial skin perfusion of affected side was lower than that of the normal side at the region of eyelid, and that the asymmetric distribution of the facial skin perfusion between two sides of eyelid is positively related to the stage of the disease (P Bell's palsy patients, and we discovered that the facial skin blood perfusion could reflect the stage of Bell's palsy, which suggested that microcirculation should be investigated in patients with this neurological deficit. It was also suggested LSI as potential diagnostic tool for Bell's palsy.

  8. Increased risk of peripheral arterial occlusive disease in patients with Bell's palsy using population data

    Science.gov (United States)

    Liou, Li-Syue; Chang, Chih-Ya; Chen, Hsuan-Ju; Tseng, Chun-Hung; Chen, Cheng-Yu

    2017-01-01

    Objective This population-based cohort study investigated the risk of developing peripheral arterial occlusive disease (PAOD) in patients with Bell’s palsy. Methods We used longitudinal claims data of health insurance of Taiwan to identify 5,152 patients with Bell’s palsy newly diagnosed in 2000–2010 and a control cohort of 20,608 patients without Bell’s palsy matched by propensity score. Incidence and hazard ratio (HR) of PAOD were assessed by the end of 2013. Results The incidence of PAOD was approximately 1.5 times greater in the Bell’s palsy group than in the non-Bell’s palsy controls (7.75 vs. 4.99 per 1000 person-years). The Cox proportional hazards regression analysis measured adjusted HR was 1.54 (95% confidence interval (CI) = 1.35–1.76) for the Bell’s palsy group compared to the non-Bell’s palsy group, after adjusting for sex, age, occupation, income and comorbidities. Men were at higher risk of PAOD than women in the Bell’s palsy group, but not in the controls. The incidence of PAOD increased with age in both groups, but the Bell’s palsy group to control group HR of PAOD decreased as age increased. The systemic steroid treatment reduced 13% of PAOD hazard for Bell’s palsy patients, compared to those without the treatment, but not significant. Conclusions Bell’s palsy appears to be associated with an increased risk of developing PAOD. Further pathophysiologic, histopathology and immunologic research is required to explore the underlying biologic mechanism. PMID:29216223

  9. European study of frequency of participation of adolescents with and without cerebral palsy

    DEFF Research Database (Denmark)

    Michelsen, Susan I; Flachs, Esben M; Damsgaard, Mogens T

    2014-01-01

    Children with cerebral palsy participate less in everyday activities than children in the general populations. During adolescence, rapid physical and psychological changes occur which may be more difficult for adolescents with impairments. Within the European SPARCLE project we measured frequency...... respectively linear and logistic regression. Adolescents with cerebral palsy spent less time with friends and had less autonomy in their daily life than adolescents in the general populations. Adolescents with cerebral palsy participated much less in sport but played electronic games at least as often...... populations. Regional variation existed. For example adolescents with cerebral palsy in central Italy were most disadvantaged according to decisional autonomy, while adolescents with cerebral palsy in east Denmark and northern England played sports as often as their general populations. Participation...

  10. Epidemiology of cerebral palsy in Southern Denmark

    DEFF Research Database (Denmark)

    Frøslev-Friis, Christina; Dunkhase-Heinl, Ulrike; Andersen, Johnny Dohn Holmgren

    2015-01-01

    INTRODUCTION: The aim of this study was to describe the prevalence, subtypes, severity and neuroimaging findings of cerebral palsy (CP) in a cohort of children born in Southern Denmark. Risk factors were analysed and aetiology considered. METHODS: A population-based cohort study covering 17...... prevention of CP is possible if the numbers of preterm births and multiple pregnancies can be reduced. FUNDING: The Danish Cerebral Palsy Follow-up Programme is supported by the foundation "Ludvig og Sara Elsass Fond". TRIAL REGISTRATION: 2008-58-0034....

  11. Tinjauan Anatomi Klinik dan Manajemen Bell's Palsy

    OpenAIRE

    Nur Mujaddidah

    2017-01-01

    Bell's Palsy is a peripheral facial nerve weakness (facial nerve) with acute onset on one side of the face. This condition causes the inability of the patient to move half of his face consciously (volunter) on the affected side. The Bell's Palsy incidence is 20-30 cases out of 100.000 people and accounts for 60-70% of all cases of unilateral facial paralysis. The disease is self-limited, but causes great suffering for patients who are not treated properly. Controversy in the management is sti...

  12. Role of nitric oxide in the onset of facial nerve palsy by HSV-1 infection.

    Science.gov (United States)

    Hato, Naohito; Kohno, Hisashi; Yamada, Hiroyuki; Takahashi, Hirotaka; Gyo, Kiyofumi

    2013-12-01

    Although herpes simplex virus type 1 (HSV-1) is a causative agent of Bell palsy, the precise mechanism of the paralysis remains unknown. It is necessary to investigate the pathogenesis and treatment of Bell palsy due to HSV-1 infection. This study elucidated the role of nitric oxide (NO) in the incidence of facial nerve paralysis caused by HSV-1 in mice and to evaluate the possible role of edaravone, a free radical scavenger, in preventing the paralysis. Sixty-two mice served as animal models of Bell palsy in this laboratory study conducted at an academic institution. Levels of NO in the facial nerve were measured using high-performance liquid chromatography and absorption photometry. The incidence of facial palsy was assessed following administration of edaravone immediately after HSV-1 inoculation and daily for 11 days thereafter. The ratio of NO (inoculated side to control side) and incidence of facial palsy. RESULTS Before the onset of facial palsy, no substantial difference in the NO level was noted between the HSV-1-inoculated side and the control side. When facial palsy occurred, usually at 7 days after inoculation, the NO level was significantly higher on the inoculated side than on the control side. Following recovery from the palsy, the high NO level of the inoculated side decreased. No increase in the NO level was observed in animals without transient facial palsy. When edaravone was administered, the incidence of facial palsy decreased significantly. These findings suggest that NO produced by inducible NO synthase in the facial nerve plays an important role in the onset of facial palsy caused by HSV-1 infection, which is considered a causative virus of Bell palsy. Hato and colleagues elucidate the role of nitric oxide in HSV-1–related facial nerve paralysis in mice and evaluate the role of edaravone, a free radical scavenger, in preventing the paralysis.

  13. Differential distribution of striatal [123I]β-CIT in Parkinson's disease and progressive supranuclear palsy, evaluated with single-photon emission tomography

    International Nuclear Information System (INIS)

    Messa, C.; Volonte, M.A.; Fazio, F.; Zito, F.; Carpinelli, A.; D'Amico, A.; Rizzo, G.; Moresco, R.M.; Paulesu, E.; Franceschi, M.; Lucignani, G.

    1998-01-01

    Functional imaging of the presynaptic dopaminergic activity using single-photon emission tomography (SPET) and iodine-123 labelled 2-β-carboxymethoxy-3-β-(4-iodophenyl)tropane ([ 123 I]β-CIT) is important for the assessment of disease severity and progression in patients with Parkinson's disease (PD). However, its capability to discriminate between different extrapyramidal disorders has not yet been assessed. The aim of this study was to evaluate the possibility of differentiating patients with PD and with progressive supranuclear palsy (PSP) by means of this method. The distribution of [ 123 I]β-CIT in the basal ganglia was assessed in six normal subjects, 13 petients with PD and five patients with PSP in whom the disease was mild. SPET images were obtained 24±2 h after i.v. injection of the tracer using a brain-dedicated system (CERASPECT). MR and SPET images were co-registered in four normal subjects and used to define a standard set of 16 circular regions of interest (ROIs) on the slice showing the highest striatal activity. The basal ganglia ROIs corresponded to (1) the head of caudate, (2) a region of transition between the head of caudate and the anterior putamen, (3) the anterior putamen and (4) the posterior putamen. A ratio of specific to non-displaceable striatal uptake was calculated normalising the activity of the basal ganglia ROIs to that of the occipital cortex (V3''). ANOVA revealed a global reduction of V3'' in all ROIs of PD and PSP patients compared with normal controls (P 123 I]β-CIT distribution in discrete striatal areas provides information on the relative caudate-putamen damage, with different values being obtained in patients clinically diagnosed as having either PD or PSP. (orig.)

  14. Increased risk of Bell palsy in patients with migraine: a nationwide cohort study.

    Science.gov (United States)

    Peng, Kuan-Po; Chen, Yung-Tai; Fuh, Jong-Ling; Tang, Chao-Hsiun; Wang, Shuu-Jiun

    2015-01-13

    To evaluate the association between migraine and Bell palsy and to examine the effects of age, sex, migraine subtype, and comorbid risk factors for Bell palsy. This nationwide cohort study was conducted using data from the Taiwan National Health Insurance Research Database. Subjects aged 18 years or older with neurologist-diagnosed migraine from 2005 to 2009 were included. A nonheadache age- and propensity score-matched control cohort was selected for comparison. All subjects were followed until the end of 2010, death, or the occurrence of a Bell palsy event. Cox proportional hazards regression was used to calculate the adjusted hazard ratios and 95% confidence intervals to compare the risk of Bell palsy between groups. Both cohorts (n = 136,704 each) were followed for a mean of 3.2 years. During the follow-up period, 671 patients (424,372 person-years) in the migraine cohort and 365 matched control subjects (438,677 person-years) were newly diagnosed with Bell palsy (incidence rates, 158.1 and 83.2/100,000 person-years, respectively). The adjusted hazard ratio for Bell palsy was 1.91 (95% confidence interval, 1.68-2.17; p Bell palsy remained significant in sensitivity analyses, and tests of interaction failed to reach significance in all subgroup analyses. Migraine is a previously unidentified risk factor for Bell palsy. The association between these 2 conditions suggests a linked disease mechanism, which is worthy of further exploration. © 2014 American Academy of Neurology.

  15. Volumetric Magnetic Resonance Imaging Study of Brain and Cerebellum in Children with Cerebral Palsy.

    Science.gov (United States)

    Kułak, Piotr; Maciorkowska, Elżbieta; Gościk, Elżbieta

    2016-01-01

    Introduction. Quantitative magnetic resonance imaging (MRI) studies are rarely used in the diagnosis of patients with cerebral palsy. The aim of present study was to assess the relationships between the volumetric MRI and clinical findings in children with cerebral palsy compared to control subjects. Materials and Methods. Eighty-two children with cerebral palsy and 90 age- and sex-matched healthy controls were collected. Results. The dominant changes identified on MRI scans in children with cerebral palsy were periventricular leukomalacia (42%) and posthemorrhagic hydrocephalus (21%). The total brain and cerebellum volumes in children with cerebral palsy were significantly reduced in comparison to controls. Significant grey matter volume reduction was found in the total brain in children with cerebral palsy compared with the control subjects. Positive correlations between the age of the children of both groups and the grey matter volumes in the total brain were found. Negative relationship between width of third ventricle and speech development was found in the patients. Positive correlations were noted between the ventricles enlargement and motor dysfunction and mental retardation in children with cerebral palsy. Conclusions. By using the voxel-based morphometry, the total brain, cerebellum, and grey matter volumes were significantly reduced in children with cerebral palsy.

  16. [Surgical treatment in otogenic facial nerve palsy].

    Science.gov (United States)

    Feng, Guo-Dong; Gao, Zhi-Qiang; Zhai, Meng-Yao; Lü, Wei; Qi, Fang; Jiang, Hong; Zha, Yang; Shen, Peng

    2008-06-01

    To study the character of facial nerve palsy due to four different auris diseases including chronic otitis media, Hunt syndrome, tumor and physical or chemical factors, and to discuss the principles of the surgical management of otogenic facial nerve palsy. The clinical characters of 24 patients with otogenic facial nerve palsy because of the four different auris diseases were retrospectively analyzed, all the cases were performed surgical management from October 1991 to March 2007. Facial nerve function was evaluated with House-Brackmann (HB) grading system. The 24 patients including 10 males and 14 females were analysis, of whom 12 cases due to cholesteatoma, 3 cases due to chronic otitis media, 3 cases due to Hunt syndrome, 2 cases resulted from acute otitis media, 2 cases due to physical or chemical factors and 2 cases due to tumor. All cases were treated with operations included facial nerve decompression, lesion resection with facial nerve decompression and lesion resection without facial nerve decompression, 1 patient's facial nerve was resected because of the tumor. According to HB grade system, I degree recovery was attained in 4 cases, while II degree in 10 cases, III degree in 6 cases, IV degree in 2 cases, V degree in 2 cases and VI degree in 1 case. Removing the lesions completely was the basic factor to the surgery of otogenic facial palsy, moreover, it was important to have facial nerve decompression soon after lesion removal.

  17. Sleep disorders in children with cerebral palsy: An integrative review.

    Science.gov (United States)

    Lélis, Ana Luíza P A; Cardoso, Maria Vera L M; Hall, Wendy A

    2016-12-01

    Sleep disorders are more prevalent in children with cerebral palsy. The review aimed to identify and synthesize information about the nature of sleep disorders and their related factors in children with cerebral palsy. We performed an electronic search by using the search terms sleep/child*, and sleep/cerebral palsy in the following databases: Latin American literature on health sciences, SCOPUS, medical publications, cumulative index to nursing and allied health literature, psycinfo, worldcat, web of science, and the Cochrane library. The selection criteria were studies: available in Portuguese, English or Spanish and published between 2004 and 2014, with results addressing sleep disorders in children (ages 0-18 y) with a diagnosis of cerebral palsy. 36,361 abstracts were identified. Of those, 37 papers were selected, and 25 excluded. Twelve papers were incorporated in the study sample: eight quantitative studies, three reviews, and one case study. Eleven types of sleep disorders were identified, such as difficult morning awakening, insomnia, nightmares, difficulties in initiating and maintaining nighttime sleep (night waking), and sleep anxiety. Twenty-one factors were linked to sleep disorders, which we classified as intrinsic factors associated with common comorbidities accompanying cerebral palsy, and extrinsic aspects, specifically environmental and socio-familial variables, and clinical-surgical and pharmacological interventions. Copyright © 2015 Elsevier Ltd. All rights reserved.

  18. Bell palsy in a neonate with rapid response to oral corticosteroids: a case report.

    Science.gov (United States)

    Saini, Arushi; Singhi, Pratibha; Sodhi, K S; Gupta, Ajit

    2013-04-01

    Idiopathic facial nerve palsy, also known as Bell palsy is rare in the neonatal age group. Other more common causes such as birth trauma; infections, especially otitis media; and congenital malformations need to be excluded. We present here a 4-week-old neonate with Bell palsy who responded rapidly to oral corticosteroids. Such an early presentation of idiopathic facial nerve palsy and use of corticosteroids in neonates is scarcely reported in the literature.

  19. Computed tomographic (CT) scans in cerebral palsy (CP)

    International Nuclear Information System (INIS)

    Kolawole, T.M.; Patel, P.J.; Mahdi, A.H.

    1989-01-01

    The CT findings in 120 cerebral palsied children are analysed. The 72.5% positive findings are correlated with the clinical types, as well as the aetiological basis for the cerebral palsy. The spastic type, 83.3% of the total number of children, had the highest positive findings. The yield was increased in children with seizures (91.3%) and those in the postnatal group (90%), as well as those with birth trauma and neonatal asphyxia (94%). The findings were those of atrophy in 30.8%, hydrocephalus, in 10%, infarct in 11.6%, porencephaly in 8.3% and others. The atropic changes and their patterns are explained. Treatable lesions, such as tumour, hydrocephalus, subdural haematoma, porencephaly and hygroma were identified in 22.5% of cases. It is concluded that CT scan is definitely efficacious in the management of cerebral palsied children. (orig.)

  20. Catheter versus non-catheter angiography in isolated third nerve palsy

    International Nuclear Information System (INIS)

    Lee, A.G.

    2007-01-01

    To discuss the controversies surrounding the indications for catheter angiography versus non-catheter and less invasive angiography techniques (e.g. magnetic resonance angiography (MRA) and computerized tomography angiography (CTA)) in the evaluation of patients with a third nerve palsy. Clinical opinion-perspective and literature review. The patient with an isolated third nerve palsy might have a vasculopathic (and typically benign, self limited course) etiology or a life threatening intracranial posterior communicating artery aneurysm. Although it is clear that non-isolated third nerve palsies require imaging directed at the topographical localization of the clinical findings, the evaluation of the neurologically isolated third nerve palsy remains controversial. The completeness of the external (i.e. somatic) motor dysfunction and the presence or absence of internal (i.e. pupillary) dysfunction are generally used to guide the choice of initial neuroimaging. Advances in MRA and CTA technology have reduced but not eliminated our dependence upon catheter angiography in this setting. A properly performed and interpreted MRA or CTA probably will be able to detect greater than 95 % of aneurysms producing a third nerve palsy. The issues surrounding the use of catheter angiography in third nerve palsy are reviewed. If the clinician is highly confident in the capability, availability, and reliability of the neuroradiologist and of their institutional experience and quality of less invasive non-catheter MRA and CTA and if the risk of aneurysm is low or if the risk of angiography is high (e.g. elderly, renal failure, iodinated contrast allergy, risk of stroke) then MRI and MRA (or CTA) may be a reasonable alternative to catheter angiography. Patients with a moderate or uncertain risk of aneurysm and a lower risk for catheter angiography or if there is a low confidence in the quality or the interpretation of the institutional MRA (or CTA) may still require catheter

  1. HERPES SIMPLEX VIRUS IN SALIVA OF PATIENTS WITH BELL'S PALSY

    Directory of Open Access Journals (Sweden)

    M.H. Harirchian

    2008-04-01

    Full Text Available Acute idiopathic peripheral facial paralysis (Bell's palsy is the most common disorder of the facial nerve. Most patients recover completely, although some have permanent disfiguring facial weakness. Many studies have attempted to identify an infectious etiology for this disease. Although the cause of Bell's palsy remains unknown, recent studies suggest a possible association with Herpes Simplex Virus-1(HSV-1 infection. In this case-control study we investigated the presence of DNA of HSV in the saliva of 26 patients with Bells palsy in first and second weeks of disorder compared to normal population who were matched in sex, age, as well as history of diabetes mellitus, hypertension and labial herpes. In the case group 3 and 7 patients had positive polymerase chain reaction (PCR for HSV in first and second weeks of disease respectively compared to 4 in controls. It means that there was not any relationship between Bell's palsy and HSV in saliva either in first or in second week. Two and 6 of positive results from the sample of first and second weeks were from patients with severe (grade 4-6 Bell's palsy. Although the positive results were more in second week in patient group and more in severe palsies, but a significant relationship between Bell's palsy or its severity and positive PCR for HSV was not detected (P >0.05.

  2. Maternal Infections during Pregnancy and Cerebral Palsy

    DEFF Research Database (Denmark)

    Miller, Jessica; Pedersen, Lars Henning; Streja, Elani

    2013-01-01

    BACKGROUND: Cerebral palsy (CP) is a common motor disability in childhood. We examined the association between maternal infections during pregnancy and the risk of congenital CP in the child. METHODS: Liveborn singletons in Denmark between 1997 and 2003 were identified from the Danish National...... the Danish Cerebral Palsy Registry. Adjusted hazard ratio (HR) and 95% confidence interval (CI) were estimated by Cox proportional hazard models. RESULTS: Of the 440 564 singletons with follow-up data, 840 were diagnosed with congenital CP. Maternal genito-urinary tract infections (HR 2.1, 95% CI 1.4, 3...

  3. Pathophysiology of muscle contractures in cerebral palsy.

    Science.gov (United States)

    Mathewson, Margie A; Lieber, Richard L

    2015-02-01

    Patients with cerebral palsy present with a variety of adaptations to muscle structure and function. These pathophysiologic symptoms include functional deficits such as decreased force production and range of motion, in addition to changes in muscle structure such as decreased muscle belly size, increased sarcomere length, and altered extracellular matrix structure and composition. On a cellular level, patients with cerebral palsy have fewer muscle stem cells, termed satellite cells, and altered gene expression. Understanding the nature of these changes may present opportunities for the development of new muscle treatment therapies. Published by Elsevier Inc.

  4. Cerebral palsy and congenital malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Dolk, Helen; Krägeloh-Mann, Inge

    2007-01-01

    AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were...... classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP...... were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children...

  5. MR imaging in Bell's palsy and herpes zoster opticus: correlation with clinical findings

    International Nuclear Information System (INIS)

    Kwon, Jung Ho; Mo, Jong Hyun; Moon, Sung Hee; Lee, Sang Sun; Park, Yang Hee; Lee, Kyung Hee; Choi, Ik Joon

    1998-01-01

    To evaluate the MRI findings of acute facial nerve paralysis in Bell's palsy and herpes zoster opticus, and to correlate these with the clinical findings. We retrowspectively reviewed the MRI findings in six cases of BEll's palsy(BP) and two of herpes zoster oticus(HZO), and compared them with the findings for 30 normal facial nerves. This nerve was considered abnormal when its signal intensity was greater than that of brain parenchyma or the contralateral normal side on Gd-enhanced T1-weighted axial and coronal MR images. We analysed the location and degree of contrast enhancement, interval change, and clinical progression in correlation with House-Brackmann(HB) grade and electroneuronography (ENoG) findings. Fifteen of 30 normal facial nerves(50%) seen on Gd-enhanced MRI were mildly enhanced in the geniculate ganglion, the proximal tympanic, and the proximal mastoid segment of the facial nerve. No enhancement of the internal auditory canal(IAC) or labyrinthine segment of the facial nerve was noted, however. In BP and HZO, Gd-enhanced MR images revealed fair to marked enhancement for more than two segments from the internal auditory canal to the mastoid segment of the facial nerve. During follow-up MRI, enhancement of the facial nerve varied in location and signal intensity, though gradually decreased in intensity approximately eight weeks after the onset of facial nerve palsy. No correlation between clinical HB grade, ENoG, and follow up MRI findings was noted. Except in the internal auditory canal and labyrinthine segment, normal facial nevemay show mild and relatively symmetrical enhancement. In BP and HZO, the facial nerve showed diffuse enhancement from the IAC to the mastoid segment.=20

  6. Biomechanical bases of rehabilitation of children with cerebral palsy

    Science.gov (United States)

    Davlet'yarova, K. V.; Korshunov, S. D.; Kapilevich, L. V.

    2015-11-01

    Biomechanical analysis and the study results of children's with cerebral palsy (CP) muscles bioelectrical activity while walking on a flat surface are represented. Increased flexion in the hip and shoulder joints and extension in the elbow joint in children with cerebral palsy were observed, with the movement of the lower limbs had less smooth character in comparison with the control group. Herewith, the oscillation amplitude was significantly increased, and the frequency in the m. gastrocnemius and m. lateralis was decreased. It was shown, that the dynamic stereotype of walking in children with cerebral palsy was characterized by excessive involvement of m. gastrocnemius and m.latissimus dorsi in locomotion. Thus, resulting biomechanical and bioelectrical parameters of walking should be considered in the rehabilitation programs development.

  7. Learn More About Cerebral Palsy

    Centers for Disease Control (CDC) Podcasts

    2008-03-30

    This podcast describes the causes, preventions, types, and signs and symptoms of cerebral palsy.  Created: 3/30/2008 by National Center on Birth Defects and Developmental Disabilities.   Date Released: 3/21/2008.

  8. Surgical and Clinical Decision Making in Isolated Long Thoracic Nerve Palsy.

    Science.gov (United States)

    Noland, Shelley S; Krauss, Emily M; Felder, John M; Mackinnon, Susan E

    2017-10-01

    Isolated long thoracic nerve palsy results in scapular winging and destabilization. In this study, we review the surgical management of isolated long thoracic nerve palsy and suggest a surgical technique and treatment algorithm to simplify management. In total, 19 patients who required surgery for an isolated long thoracic nerve palsy were reviewed retrospectively. Preoperative demographics, electromyography (EMG), and physical examinations were reviewed. Intraoperative nerve stimulation, surgical decision making, and postoperative outcomes were reviewed. In total, 19 patients with an average age of 32 were included in the study. All patients had an isolated long thoracic nerve palsy caused by either an injury (58%), Parsonage-Turner syndrome (32%), or shoulder surgery (10%); 18 patients (95%) underwent preoperative EMG; 10 with evidence of denervation (56%); and 13 patients had motor unit potentials in the serratus anterior (72%). The preoperative EMG did not correlate with intraoperative nerve stimulation in 13 patients (72%) and did correlate in 5 patients (28%); 3 patients had a nerve transfer (3 thoracodorsal to long thoracic at lateral chest, 1 pec to long thoracic at supraclavicular incision). In the 3 patients who had a nerve transfer, there was return of full forward flexion of the shoulder at an average of 2.5 months. A treatment algorithm based on intraoperative nerve stimulation will help guide surgeons in their clinical decision making in patients with isolated long thoracic nerve palsy. Intraoperative nerve stimulation is the gold standard in the management of isolated long thoracic nerve palsy.

  9. [New developments in spastic unilateral cerebral palsy].

    Science.gov (United States)

    Chabrier, S; Roubertie, A; Allard, D; Bonhomme, C; Gautheron, V

    2010-01-01

    Hemiplegic (or spastic unilateral) cerebral palsy accounts for about 30% of all cases of cerebral palsy. With a population prevalence of 0.6 per 1000 live births, it is the most common type of cerebral palsy among term-born children and the second most common type after diplegia among preterm infants. Many types of prenatal and perinatal brain injury can lead to congenital hemiplegia and brain MRI is the most useful tool to classify them with accuracy and to provide early prognostic information. Perinatal arterial ischemic stroke thus appears as the leading cause in term infants, whereas encephalopathy of prematurity is the most common cause in premature babies. Other causes include brain malformations, neonatal sinovenous thrombosis, parenchymal hemorrhage (for example due to coagulopathy or alloimmune thrombocytopenia) and the more recently described familial forms of porencephaly associated with mutations in the COL4A1 gene. In adjunction with pharmacologic treatment (botulinium neurotoxin injection), new evidence-based rehabilitational interventions, such as constraint-induced movement therapy and mirror therapy, are increasingly being used.

  10. Correlation with neuropsychological assessment and SPM analysis of brain perfusion SPECT in patients with progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Jeong, Young Jin; Kang, Do Young; Park, Kyung Won; Kim, Jae Woo

    2004-01-01

    Progressive supranuclear palsy (PSP) is a degenerative condition of unknown aetiology that produces an akinetic-rigid form of parkinsonism characterised by early falls, dementia and abnormalities of extraocular movements. The patterns of decreased regional cerebral blood flow and cognitive impairment in PSP compared with normal control have been insufficiently investigated and a limited number of studies have been performed. We evaluated clinical symptoms, functional neuroimaging study using Tc-99m HMPAO SPECT and neuropsychological profiles in patients with PSP. Eleven patients with PSP diagnosed by the clinical criteria of National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) (mean age: 70.5±5.6 years, educational period: 4.5±4.7 years) and age-matched 10 healthy control subjects (mean age: 68.1±4.5 years, educational period: 6.5±4.1 years) participated in this study were participated. All patients were given a neurologic examination, brain MRI and cerebral perfusion SPECT using Tc-99m HMPAO. We concomittently evaluated several cognitive profiles using the Seoul Neuropsychological Screening Battery. SPM analysis of the SPECT image showed significant perfusion deficits in the left inferior frontal gyrus, left caudate nucleus, left middle frontal gyrus and cingulate gyrus in the patients with PSP compared with age-matched healthy control (uncorrected p<0.01). On neuropsychological assessment, cognitive deficits on verbal and visual memory, word fluency and frontal executive functions were prominent in most patients with PSP compared with healthy control subjects. Our findings suggest that measurement of regional cerebral blood flow by perfusion SPECT and voxel-based SPM analysis with neuropsychological assessment are useful to understanding the correlation between perfusion deficits and abnormal cognitive profiles in patients with PSP

  11. Correlation with neuropsychological assessment and SPM analysis of brain perfusion SPECT in patients with progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Young Jin; Kang, Do Young; Park, Kyung Won; Kim, Jae Woo [School of Medicine, Dong-A University, Busan (Korea, Republic of)

    2004-07-01

    Progressive supranuclear palsy (PSP) is a degenerative condition of unknown aetiology that produces an akinetic-rigid form of parkinsonism characterised by early falls, dementia and abnormalities of extraocular movements. The patterns of decreased regional cerebral blood flow and cognitive impairment in PSP compared with normal control have been insufficiently investigated and a limited number of studies have been performed. We evaluated clinical symptoms, functional neuroimaging study using Tc-99m HMPAO SPECT and neuropsychological profiles in patients with PSP. Eleven patients with PSP diagnosed by the clinical criteria of National Institute of Neurological Disorders and Stroke and the Society for PSP (NINDS-SPSP) (mean age: 70.5{+-}5.6 years, educational period: 4.5{+-}4.7 years) and age-matched 10 healthy control subjects (mean age: 68.1{+-}4.5 years, educational period: 6.5{+-}4.1 years) participated in this study were participated. All patients were given a neurologic examination, brain MRI and cerebral perfusion SPECT using Tc-99m HMPAO. We concomittently evaluated several cognitive profiles using the Seoul Neuropsychological Screening Battery. SPM analysis of the SPECT image showed significant perfusion deficits in the left inferior frontal gyrus, left caudate nucleus, left middle frontal gyrus and cingulate gyrus in the patients with PSP compared with age-matched healthy control (uncorrected p<0.01). On neuropsychological assessment, cognitive deficits on verbal and visual memory, word fluency and frontal executive functions were prominent in most patients with PSP compared with healthy control subjects. Our findings suggest that measurement of regional cerebral blood flow by perfusion SPECT and voxel-based SPM analysis with neuropsychological assessment are useful to understanding the correlation between perfusion deficits and abnormal cognitive profiles in patients with PSP.

  12. New Predictive Parameters of Bell"s Palsy: Neutrophil to Lymphocyte Ratio and Platelet to Lymphocyte Ratio

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    Doğan Atan

    2015-06-01

    Full Text Available Background: Bell’s palsy is the most frequent cause of unilateral facial paralysis. Inflammation is thought to play an important role in the pathogenesis of Bell’s palsy. Aims: Neutrophil to lymphocyte ratio (NLR and platelet to lymphocyte ratio (PLR are simple and inexpensive tests which are indicative of inflammation and can be calculated by all physicians. The aim of this study was to reveal correlations of Bell’s palsy and degree of paralysis with NLR and PLR. Study Design: Case-control study. Methods: The retrospective study was performed January 2010 and December 2013. Ninety-nine patients diagnosed as Bell’s palsy were included in the Bell’s palsy group and ninety-nine healthy individuals with the same demographic characteristics as the Bell’s palsy group were included in the control group. As a result of analyses, NLR and PLR were calculated. Results: The mean NLR was 4.37 in the Bell’s palsy group and 1.89 in the control group with a statistically significant difference (p<0.001. The mean PLR was 137.5 in the Bell’s palsy group and 113.75 in the control group with a statistically significant difference (p=0.008. No statistically significant relation was detected between the degree of facial paralysis and NLR and PLR. Conclusion: The NLR and the PLR were significantly higher in patients with Bell’s palsy. This is the first study to reveal a relation between Bell’s palsy and PLR. NLR and PLR can be used as auxiliary parameters in the diagnosis of Bell’s palsy.

  13. Caracterization of adults with cerebral palsy.

    Science.gov (United States)

    Margre, Anna L M; Reis, Maria G L; Morais, Rosane L S

    2010-01-01

    cerebral Palsy (CP) is a group of permanent disorders of the development of movement and posture that cause functional limitation and are attributed to non-progressive disorders which occur in the fetal or infant brain. In recent years, with the increase in life expectancy of individuals with CP, several studies have described the impact of musculoskeletal disabilities and functional limitations over the life cycle. to characterize adults with CP through sociodemographic information, classifications, general health, associated conditions, physical complications and locomotion. twenty-two adults with CP recruited from local rehabilitation centers in an inner town of Brazil participated in this study. A questionnaire was used to collect data on sociodemographic characteristics, comorbities, and physical complications. A brief physical therapy evaluation was carried out, and the Gross Motor Function Classification System (GMFCS) and the Manual Ability Classification System (MACS) were applied. Data were analyzed through descriptive statistics. the mean age was 28.7 (SD 10.6) years, 86.4% of participants lived with parents, and 4.5% were employed. Most of the sample consisted of spastic quadriplegic subjects, corresponding to levels IV and V of the GMFCS and MACS. Different comorbidities and important physical complications such as scoliosis and muscle contractures were present. More than half of the participants were unable to walk. Most participants demonstrated important restrictions in social participation and lower educational level. Adults with CP can be affected by several physical complications and progressive limitations in gait.

  14. Therapeutic outcome of nasopharyngeal carcinoma with cranial nerve palsy: a single institution experience of 104 patients

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    Huang CC

    2017-04-01

    Full Text Available Chun-Chieh Huang,1,2 Fu-Min Fang,1 Hui-Chun Chen,1 Hsuan-Chih Hsu,1 Tai-Lin Huang,3 Yu-Li Su,3 Ya-Chun Chang4 1Department of Radiation Oncology, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, 2Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, 3Department of Hematology and Oncology, 4Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan, Republic of China Purpose: Cranial nerve (CN palsy is the main symptom in patients with locally advanced nasopharyngeal carcinoma (NPC. This study aimed to evaluate the therapeutic outcome of NPC with CN palsy and to analyze the prognostic factors.Patients and methods: A total of 104 NPC patients with CN palsy curatively treated by conventional (n=44 or conformal (n=60 radiotherapy (RT were enrolled. Upper CN palsy was present in 81 patients, lower CN palsy in four patients, and both upper and lower CN palsy in 19 patients. Forty-one patients had CN palsy for >2 months before diagnosis.Results: Complete recovery of CN palsy was observed in 74 patients. The actuarial 5-year locoregional control (LRC, distant metastasis-free survival (DMFS, and overall survival (OS rates were 58.2%, 62.2%, and 38.4%, respectively. No significant difference was observed in CN recovery, LRC, DMFS, or OS for patients treated by conventional versus conformal technique. However, significant reduction of grade 3 or greater toxicities was found in those treated by the conformal technique (odds ratio =0.28.Conclusion: Patients with CN palsy presenting >2 months before diagnosis were hard to recover from palsy. The LRC, OS, and recovery from CN palsy did not significantly change with the treatment evolution. Patients with complete recovery from CN palsy had longer OS. Keywords: nasopharyngeal carcinoma, cranial nerve palsy, radiotherapy

  15. Update on managing Bell's palsy.

    Science.gov (United States)

    2008-07-01

    Each year in the UK, around 1 in 5,000 people develops Bell's palsy--idiopathic unilateral lower motor neurone facial weakness of rapid onset. Of those who are not treated, about 16% end up with permanent moderate to severe weakness, which can result in facial dysfunction and disfigurement, and psychological difficulties. There has been longstanding controversy about what, if any, treatment should be given, with potential alternatives including corticosteroids, antiviral drugs, acupuncture and physiotherapy. We last reviewed this condition in 2006, indicating that "published trials on the efficacy of drug treatments have been poor and no firm conclusions can be drawn about the benefit of any single drug", and "it is unclear what place, if any, acupuncture and physiotherapy have in the management of patients with Bell's palsy". Here we update our conclusions in the light of recently published evidence.

  16. Prednisolone and acupuncture in Bell's palsy: study protocol for a randomized, controlled trial

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    Wang Kangjun

    2011-06-01

    Full Text Available Abstract Background There are a variety of treatment options for Bell's palsy. Evidence from randomized controlled trials indicates corticosteroids can be used as a proven therapy for Bell's palsy. Acupuncture is one of the most commonly used methods to treat Bell's palsy in China. Recent studies suggest that staging treatment is more suitable for Bell's palsy, according to different path-stages of this disease. The aim of this study is to compare the effects of prednisolone and staging acupuncture in the recovery of the affected facial nerve, and to verify whether prednisolone in combination with staging acupuncture is more effective than prednisolone alone for Bell's palsy in a large number of patients. Methods/Design In this article, we report the design and protocol of a large sample multi-center randomized controlled trial to treat Bell's palsy with prednisolone and/or acupuncture. In total, 1200 patients aged 18 to 75 years within 72 h of onset of acute, unilateral, peripheral facial palsy will be assessed. There are six treatment groups, with four treated according to different path-stages and two not. These patients are randomly assigned to be in one of the following six treatment groups, i.e. 1 placebo prednisolone group, 2 prednisolone group, 3 placebo prednisolone plus acute stage acupuncture group, 4 prednisolone plus acute stage acupuncture group, 5 placebo prednisolone plus resting stage acupuncture group, 6 prednisolone plus resting stage acupuncture group. The primary outcome is the time to complete recovery of facial function, assessed by Sunnybrook system and House-Brackmann scale. The secondary outcomes include the incidence of ipsilateral pain in the early stage of palsy (and the duration of this pain, the proportion of patients with severe pain, the occurrence of synkinesis, facial spasm or contracture, and the severity of residual facial symptoms during the study period. Discussion The result of this trial will assess the

  17. Microcystic adnexal carcinoma (MAC)-like squamous cell carcinoma as a differential diagnosis to Bell´s palsy: review of guidelines for refractory facial nerve palsy.

    Science.gov (United States)

    Mueller, S K; Iro, H; Lell, M; Seifert, F; Bohr, C; Scherl, C; Agaimy, A; Traxdorf, M

    2017-01-05

    Bell´s palsy is the most common cause of facial paralysis worldwide and the most common disorder of the cranial nerves. It is a diagnosis of exclusion, accounting for 60-75% of all acquired peripheral facial nerve palsies. Our case shows the first case of a microcystic adnexal carcinoma-like squamous cell carcinoma as a cause of facial nerve palsy. The patient, a 70-year-old Caucasian male, experienced subsequent functional impairment of the trigeminal and the glossopharyngeal nerve about 1½ years after refractory facial nerve palsy. An extensive clinical work-up and tissue biopsy of the surrounding parotid gland tissue was not able to determine the cause of the paralysis. Primary infiltration of the facial nerve with subsequent spreading to the trigeminal and glossopharyngeal nerve via neuroanastomoses was suspected. After discussing options with the patient, the main stem of the facial nerve was resected to ascertain the diagnosis of MAC-like squamous cell carcinoma, and radiochemotherapy was subsequently started. This case report shows that even rare neoplastic etiologies should be considered as a cause of refractory facial nerve palsy and that it is necessary to perform an extended diagnostic work-up to ascertain the diagnosis. This includes high-resolution MRI imaging and, as perilesional parotid biopsies might be inadequate for rare cases like ours, consideration of a direct nerve biopsy to establish the right diagnosis.

  18. The pattern of verbal, visuospatial and procedural learning in Richardson variant of progressive supranuclear palsy in comparison to Parkinson's disease.

    Science.gov (United States)

    Sitek, Emilia J; Wieczorek, Dariusz; Konkel, Agnieszka; Dąbrowska, Magda; Sławek, Jarosław

    2017-08-29

    Progressive supranuclear palsy (PSP) is regarded either within spectrum of atypical parkinsonian syndromes or frontotemporal lobar degeneration. We compared the verbal, visuospatial and procedural learning profiles in patients with PSP and Parkinson's disease (PD). Furthermore, the relationship between executive factors (initiation and inhibition) and learning outcomes was analyzed. Thirty-three patients with the clinical diagnosis of PSP-Richardson's syndrome (PSP-RS), 39 patients with PD and 29 age -and education -matched controls were administered Mini-Mental State Examination (MMSE), phonemic and semantic fluency tasks, Auditory Verbal Learning Test (AVLT), Visual Learning and Memory Test for Neuropsychological Assessment by Lamberti and Weidlich (Diagnosticum für Cerebralschädigung, DCS), Tower of Toronto (ToT) and two motor sequencing tasks. Patients with PSP-RS and PD were matched in terms of MMSE scores and mood. Performance on DCS was lower in PSP-RS than in PD. AVLT delayed recall was better in PSP-RS than PD. Motor sequencing task did not differentiate between patients. Scores on AVLT correlated positively with phonemic fluency scores in both PSP-RS and PD. ToT rule violation scores were negatively associated with DCS performance in PSP-RS and PD as well as with AVLT performance in PD. Global memory performance is relatively similar in PSP-RS and PD. Executive factors (initiation and inhibition) are closely related to memory performance in PSP-RS and PD. Visuospatial learning impairment in PSP-RS is possibly linked to impulsivity and failure to inhibit automatic responses.

  19. Voxelwise meta-ananlysis of gray matter anomalies in progressive supranuclear palsy and Parkinson’s disease using anatomic likelihood estimation

    Directory of Open Access Journals (Sweden)

    Huifang eShang

    2014-02-01

    Full Text Available Numerous voxel-based morphometry (VBM studies on gray matter (GM of patients with progressive supranuclear palsy (PSP and Parkinson’s disease (PD have been conducted separately. Identifying the different neuroanatomical changes in GM resulting from PSP and PD through meta-analysis will aid the differential diagnosis of PSP and PD. In this study, a systematic review of VBM studies of patients with PSP and PD relative to healthy controls (HC in the Embase and PubMed databases from January 1995 to April 2013 was conducted. The anatomical distribution of the coordinates of GM differences was meta-analyzed using anatomical likelihood estimation. Separate maps of GM changes were constructed and subtraction meta-analysis was performed to explore the differences in GM abnormalities between PSP and PD. Nine PSP studies and 24 PD studies were included. GM reductions were present in the bilateral thalamus, basal ganglia, midbrain, insular cortex and inferior frontal gyrus, and left precentral gyrus and anterior cingulate gyrus in PSP. Atrophy of GM was concentrated in the bilateral middle and inferior frontal gyrus, precuneus, left precentral gyrus, middle temporal gyrus, right superior parietal lobule, and right cuneus in PD. Subtraction meta-analysis indicated that GM volume was lesser in the bilateral midbrain, thalamus, and insula in PSP compared with that in PD. Our meta-analysis indicated that PSP and PD shared a similar distribution of neuroanatomical changes in the frontal lobe, including inferior frontal gyrus and precentral gyrus, and that atrophy of the midbrain, thalamus, and insula are neuroanatomical markers for differentiating PSP from PD.

  20. T2 Relaxometry MRI Predicts Cerebral Palsy in Preterm Infants.

    Science.gov (United States)

    Chen, L-W; Wang, S-T; Huang, C-C; Tu, Y-F; Tsai, Y-S

    2018-01-18

    T2-relaxometry brain MR imaging enables objective measurement of brain maturation based on the water-macromolecule ratio in white matter, but the outcome correlation is not established in preterm infants. Our study aimed to predict neurodevelopment with T2-relaxation values of brain MR imaging among preterm infants. From January 1, 2012, to May 31, 2015, preterm infants who underwent both T2-relaxometry brain MR imaging and neurodevelopmental follow-up were retrospectively reviewed. T2-relaxation values were measured over the periventricular white matter, including sections through the frontal horns, midbody of the lateral ventricles, and centrum semiovale. Periventricular T2 relaxometry in relation to corrected age was analyzed with restricted cubic spline regression. Prediction of cerebral palsy was examined with the receiver operating characteristic curve. Thirty-eight preterm infants were enrolled for analysis. Twenty patients (52.6%) had neurodevelopmental abnormalities, including 8 (21%) with developmental delay without cerebral palsy and 12 (31.6%) with cerebral palsy. The periventricular T2-relaxation values in relation to age were curvilinear in preterm infants with normal development, linear in those with developmental delay without cerebral palsy, and flat in those with cerebral palsy. When MR imaging was performed at >1 month corrected age, cerebral palsy could be predicted with T2 relaxometry of the periventricular white matter on sections through the midbody of the lateral ventricles (area under the receiver operating characteristic curve = 0.738; cutoff value of >217.4 with 63.6% sensitivity and 100.0% specificity). T2-relaxometry brain MR imaging could provide prognostic prediction of neurodevelopmental outcomes in premature infants. Age-dependent and area-selective interpretation in preterm brains should be emphasized. © 2018 by American Journal of Neuroradiology.

  1. Quality of life as assessed by adults with cerebral palsy.

    Science.gov (United States)

    Maestro-Gonzalez, Alba; Bilbao-Leon, M Cruz; Zuazua-Rico, David; Fernandez-Carreira, Jose M; Baldonedo-Cernuda, Ricardo F; Mosteiro-Diaz, M Pilar

    2018-01-01

    We explored the quality of life of adults with cerebral palsy without an intellectual disability and the predictors of quality of life. Because cerebral palsy is a disease that manifests in childhood, much of the research into quality of life for those dealing with it focuses on children; there are few studies that evaluate the quality of life of adults with cerebral palsy. Therefore, it is important to consider their perceptions in order to improve their general wellbeing and self-determination. This was a descriptive, cross-sectional study. Quality of life was measured using the GENCAT Quality of Life Scale. Demographic and personal variables were also collected and examined. Participants comprised 75 adults (58.7 percent men, mean age = 40.84 years) with cerebral palsy who were members of the National Cerebral Palsy Association of Spain between 2014 and 2015. A linear multivariate model was examined as well. The overall mean score indicator of participants' quality of life was 103.29, which corresponds to the 56.6th percentile on the GENCAT scale. Examining the level of qualification, we found significant differences in the factors "personal development" and "self-determination," and those with a university education obtained higher scores than their less-educated counterparts. Having a partner was related to higher quality of life standard scores. After constructing a linear model, it was observed that maintaining sexual relationships was another factor that increased participants' quality of life. This study highlights the importance of social and romantic relationships to achieve a better quality of life in adults with cerebral palsy who do not have an intellectual disability. Social integration and sexuality education programs should be developed to improve their quality of life.

  2. Gd-DTPA enhanced MR imaging in Bell's palsy

    International Nuclear Information System (INIS)

    Matsumoto, Yasushi; Kawamura, Yuji; Yanagihara, Naoaki; Sadamoto, Masanori; Sadamoto, Kazuhiko.

    1991-01-01

    Magnetic resonance imaging (MRI) was carried out in 27 patients with Bell's palsy. T1-weighted spin-echo images (TR500/TE25-34) were taken before and after the intravenous administration of Gd-DTPA (0.1 mmol/kg) with 0.5T superconductive MRI. There was significant enhancement at the geniculate ganglion and the horizontal segment of the involved facial nerve in 22 patients (81.5%) and at the vertical segment in 20 (74.1%). Enhancement at the meatal fundus was seen in only 4 patients (14.9%) and at the labyrinthine segment in 8 (29.6%). These results corroborate other evidence that the geniculate ganglion is involved most frequently in Bell's palsy. In addition, the enhancement of the proximal portion, i.e. the meatal fundus and the labyrinthine segment of the nerve, although not common, noted in this study may be important in the diagnosis and treatment of Bell's palsy. (author)

  3. Sudden onset odontoid fracture caused by cervical instability in hypotonic cerebral palsy.

    Science.gov (United States)

    Shiohama, Tadashi; Fujii, Katsunori; Kitazawa, Katsuhiko; Takahashi, Akiko; Maemoto, Tatsuo; Honda, Akihito

    2013-11-01

    Fractures of the upper cervical spine rarely occur but carry a high rate of mortality and neurological disabilities in children. Although odontoid fractures are commonly caused by high-impact injuries, cerebral palsy children with cervical instability have a risk of developing spinal fractures even from mild trauma. We herein present the first case of an odontoid fracture in a 4-year-old boy with cerebral palsy. He exhibited prominent cervical instability due to hypotonic cerebral palsy from infancy. He suddenly developed acute respiratory failure, which subsequently required mechanical ventilation. Neuroimaging clearly revealed a type-III odontoid fracture accompanied by anterior displacement with compression of the cervical spinal cord. Bone mineral density was prominently decreased probably due to his long-term bedridden status and poor nutritional condition. We subsequently performed posterior internal fixation surgically using an onlay bone graft, resulting in a dramatic improvement in his respiratory failure. To our knowledge, this is the first report of an odontoid fracture caused by cervical instability in hypotonic cerebral palsy. Since cervical instability and decreased bone mineral density are frequently associated with cerebral palsy, odontoid fractures should be cautiously examined in cases of sudden onset respiratory failure and aggravated weakness, especially in hypotonic cerebral palsy patients. Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  4. Histological chorioamnionitis is associated with cerebral palsy in preterm neonates.

    Science.gov (United States)

    Horvath, Boldizsár; Grasselly, Magda; Bodecs, Tamas; Boncz, Imre; Bodis, József

    2012-08-01

    To determine the interaction between histological chorioamnionitis and unexplained neonatal cerebral palsy among low birth weight infants. We studied 141 preterm infants below 1500 g delivered between 2000 and 2010. Clinical data, neonatal neuroimaging, laboratory results, the histopathological features of the placenta and gastric smear within the first hour of delivery, were evaluated. Cerebral palsy was detected in 11 out of 141 preterm newborns (7.8%). The incidence of silent histological chorioamnionitis was 33.6% (43 of 128 cases). Chorioamniontis was significantly associated with the risk of unexplained cerebral palsy (p=0.024). There were also significant correlations between maternal genital infections and chorioamnionitis (p=0.005), and between maternal infections and a positive smear of neonatal gastric aspirates (p=0.000). The rate of cesarean section was 67.4% (95 out of 141 deliveries), and elective cesarean section was performed in 68 cases. Intrauterine exposure to maternal infection was associated with a marked increase in the risk of cerebral palsy in preterm infants. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  5. Ptosis as partial oculomotor nerve palsy due to compression by infundibular dilatation of posterior communicating artery, visualized with three-dimensional computer graphics: case report.

    Science.gov (United States)

    Fukushima, Yuta; Imai, Hideaki; Yoshino, Masanori; Kin, Taichi; Takasago, Megumi; Saito, Kuniaki; Nakatomi, Hirofumi; Saito, Nobuhito

    2014-01-01

    Oculomotor nerve palsy (ONP) due to internal carotid-posterior communicating artery (PcomA) aneurysm generally manifests as partial nerve palsy including pupillary dysfunction. In contrast, infundibular dilatation (ID) of the PcomA has no pathogenic significance, and mechanical compression of the cranial nerve is extremely rare. We describe a 60-year-old woman who presented with progressive ptosis due to mechanical compression of the oculomotor nerve by an ID of the PcomA. Three-dimensional computer graphics (3DCG) accurately visualized the mechanical compression by the ID, and her ptosis was improved after clipping of the ID. ID of the PcomA may cause ONP by mechanical compression and is treatable surgically. 3DCG are effective for the diagnosis and preoperative simulation.

  6. High-intensity facial nerve lesions on T2-weighted images in chronic persistent facial nerve palsy

    Energy Technology Data Exchange (ETDEWEB)

    Kinoshita, T. [Dept. of Radiology, Sendai City Hospital, Sendai (Japan); Dept. of Radiology, Tottori Univ. (Japan); Ishii, K. [Dept. of Radiology, Sendai City Hospital, Sendai (Japan); Okitsu, T. [Dept. of Otolaryngology, Sendai City Hospital (Japan); Ogawa, T. [Dept. of Radiology, Tottori Univ. (Japan); Okudera, T. [Dept. of Radiology, Research Inst. of Brain and Blood Vessels-Akita, Akita (Japan)

    2001-05-01

    Our aim was to estimate the value of MRI in detecting irreversibly paralysed facial nerves. We examined 95 consecutive patients with a facial nerve palsy (14 with a persistent palsy, and 81 with good recovery), using a 1.0 T unit, with T2-weighted and contrast-enhanced T1-weighted images. The geniculate ganglion and tympanic segment had gave high signal on T2-weighted images in the chronic stage of persistent palsy, but not in acute palsy. The enhancement pattern of the facial nerve in the chronic persistent facial nerve palsy is similar to that in the acute palsy with good recovery. These findings suggest that T2-weighted MRI can be used to show severely damaged facial nerves. (orig.)

  7. Impaired riboflavin transport due to missense mutations in SLC52A2 causes Brown-Vialetto-Van Laere syndrome

    OpenAIRE

    Haack, Tobias B.; Makowski, Christine; Yao, Yoshiaki; Graf, Elisabeth; Hempel, Maja; Wieland, Thomas; Tauer, Ulrike; Ahting, Uwe; Mayr, Johannes A.; Freisinger, Peter; Yoshimatsu, Hiroki; Inui, Ken; Strom, Tim M.; Meitinger, Thomas; Yonezawa, Atsushi

    2012-01-01

    Brown-Vialetto-Van Laere syndrome (BVVLS [MIM 211530]) is a rare neurological disorder characterized by infancy onset sensorineural deafness and ponto-bulbar palsy. Mutations in SLC52A3 (formerly C20orf54), coding for riboflavin transporter 2 (hRFT2), have been identified as the molecular genetic correlate in several individuals with BVVLS. Exome sequencing of just one single case revealed that compound heterozygosity for two pathogenic mutations in the SLC52A2 gene coding for riboflavin tran...

  8. Cerebral palsy

    International Nuclear Information System (INIS)

    Truwit, C.L.; Barkovich, A.J.; Koch, T.; Ferreiro, D.

    1990-01-01

    This paper reviews cranial MR findings in patients with cerebral palsy (CP) to clarify and categorize this disorder. The MR images of 40 patients with clinical CP were retrospectively reviewed. All patients suffered either varying spastic plegias, hypotonicity, or choreoathetosis. Concomitantly, the patients suffered from static encephalopathy, developmental delay, and/or microcephaly. Twenty-four patients were born at or near term, 10 were premature, and incomplete birth histories were available in six. The MR images revealed mild to severe degrees of white matter damage in 24 patients (12 term, nine premature, three unknown)

  9. Case study of physiotherapeutic treatment of patient with diagnosis facial nerve peripheral palsy

    OpenAIRE

    Zahrádková, Tereza

    2015-01-01

    Title of Bachelorʼs thesis: Case study of physiotherapeutic treatment of patient with diagnosis facial nerve peripheral palsy. Aim of thesis: Summary of theoretical findings of patientʼs diagnosis, study metodology of physiotherapeutic care, treatment design, monitoring of treatment, and evaluate the effect of patient with diagnosis facial nerve peripheral palsy. Summary: This thesis comprehensively summarizes the findings of of peripheral facial nerve palsy and it's treatment with physiotera...

  10. Multi cranial nerve palsies as the presenting features of prostate carcinoma

    International Nuclear Information System (INIS)

    Mitchell, D.M.; Wynne, C.J.; Cowan, I.

    2008-01-01

    Full text: Cranial nerve palsies have previously been reported in metastatic prostate carcinoma, usually occurring late in the course of the disease. We describe the case of a 55-year-old man whose diagnosis of prostate cancer was made following investigation of multiple cranial nerve palsies.

  11. Bell Palsy and Acupuncture Treatment

    Directory of Open Access Journals (Sweden)

    Betul Battaloglu Ižnanc

    2013-08-01

    A 22-year-old female patient, a midwifery student, had treatment with corticosteroid and antiviral agents as soon as Bell Palsy (BP was diagnosed (House-Breckman stage 6. Six weeks later, patient didn’t recover, while in House-Breckman stage 3, acupuncture was perfomed and local and distal acupoints were used with ears, body and face. Ear acupuncture point was used two times with detection. In the course of six sessions body and face points were stimulated by electroacupuncture. After ten acupuncture treatments, the subjective symptoms and the facial motion on the affected side improved. There was an spotting ecchymosis the ST2 points on. The symmetry of the face is a determinant of facial charm and influences interpersonal attraction for adults, children and pregnant women. Medical options for the sequelae of BP are limited. Acupuncture’s effectively in Bell palsy patients’ should be shown with more clinical and electrophysiological studies.

  12. Recurrent Isolated Oculomotor Nerve Palsy after Radiation of a Mesencephalic Metastasis. Case Report and Mini Review

    Science.gov (United States)

    Grabau, Olga; Leonhardi, Jochen; Reimers, Carl D.

    2014-01-01

    Introduction: Recurrent oculomotor nerve palsies are extremely rare clinical conditions. Case report: Here, we report on a unique case of a short-lasting recurrent unilateral incomplete external and complete internal oculomotor nerve palsy. The episodic palsies were probably caused by an ipsilateral mesencephalic metastasis of a breast carcinoma and occurred after successful brain radiation therapy. Discussion: While the pathogenic mechanism remains unclear, the recurrent sudden onset and disappearance of the palsies and their decreasing frequency after antiepileptic treatment suggest the occurrence of epilepsy-like brainstem seizures. A review of case reports of spontaneous reversible oculomotor nerve palsies is presented. PMID:25104947

  13. Recurrent isolated oculomotor nerve palsy after radiation of a mesencephalic metastasis. Case report and mini-review.

    Directory of Open Access Journals (Sweden)

    Olga eGrabau

    2014-07-01

    Full Text Available Introduction: Recurrent oculomotor nerve palsies are extremely rare clinical conditions. Case report: Here, we report on a unique case of a short-lasting recurrent unilateral incomplete external and complete internal oculomotor nerve palsy. The episodic palsies were probably caused by an ipsilateral mesencephalic metastasis of a breast carcinoma and occurred after successful brain radiation therapy. Discussion: While the pathogenic mechanism remains unclear, the recurrent sudden onset and disappearance of the palsies and their decreasing frequency after antiepileptic treatment suggest the occurrence of epilepsy-like brainstem seizures. A review of case reports of spontaneous reversible oculomotor nerve palsies is presented.

  14. Cerebral palsy in eastern Denmark: declining birth prevalence but increasing numbers of unilateral cerebral palsy in birth year period 1986-1998

    DEFF Research Database (Denmark)

    Ravn, Susanne Holst; Flachs, Esben Meulengracht; Uldall, Peter

    2010-01-01

    The Cerebral Palsy Registry in eastern Denmark has been collecting cases using a uniform data sampling procedure since birth year 1979. Children are included by two child neurologists and an obstetrician. Information on pregnancy, birth, neonatal period, impairments and demographic data...... are registered. The total cerebral palsy birth prevalence has been significantly decreasing since the birth period 1983-1986 with 3.0 per 1000 live births until the period 1995-1998 with 2.1 per 1000 live births. The overall decrease was seen in preterm infants (...

  15. Cerebral palsy in eastern Denmark: declining birth prevalence but increasing numbers of unilateral cerebral palsy in birth year period 1986-1998

    DEFF Research Database (Denmark)

    Ravn, Susanne Holst; Flachs, Esben Meulengracht; Uldall, Peter

    2010-01-01

    The Cerebral Palsy Registry in eastern Denmark has been collecting cases using a uniform data sampling procedure since birth year 1979. Children are included by two child neurologists and an obstetrician. Information on pregnancy, birth, neonatal period, impairments and demographic data are regis......The Cerebral Palsy Registry in eastern Denmark has been collecting cases using a uniform data sampling procedure since birth year 1979. Children are included by two child neurologists and an obstetrician. Information on pregnancy, birth, neonatal period, impairments and demographic data...

  16. Cerebral palsy characterization by estimating ocular motion

    Science.gov (United States)

    González, Jully; Atehortúa, Angélica; Moncayo, Ricardo; Romero, Eduardo

    2017-11-01

    Cerebral palsy (CP) is a large group of motion and posture disorders caused during the fetal or infant brain development. Sensorial impairment is commonly found in children with CP, i.e., between 40-75 percent presents some form of vision problems or disabilities. An automatic characterization of the cerebral palsy is herein presented by estimating the ocular motion during a gaze pursuing task. Specifically, After automatically detecting the eye location, an optical flow algorithm tracks the eye motion following a pre-established visual assignment. Subsequently, the optical flow trajectories are characterized in the velocity-acceleration phase plane. Differences are quantified in a small set of patients between four to ten years.

  17. Stability and Harmony of Gait in Children with Cerebral Palsy

    Science.gov (United States)

    Iosa, Marco; Marro, Tiziana; Paolucci, Stefano; Morelli, Daniela

    2012-01-01

    The aim of this study was to quantitatively assess the stability and harmony of gait in children with cerebral palsy. Seventeen children with spastic hemiplegia due to cerebral palsy (5.0 [plus or minus] 2.3 years old) who were able to walk autonomously and seventeen age-matched children with typical development (5.7 [plus or minus] 2.5 years old,…

  18. A case report of physiotherapy treatment of a patient with peripheral facial palsy

    OpenAIRE

    Příhonská, Jana

    2016-01-01

    Thesis title: A case report of physiotherapy treatment of a patient with peripheral facial palsy Thesis summary: The thesis focuses on physiotherapy treatment techniques for peripheral facial palsy. It consists of a theoretical part and a practical part. The theoretical part covers basic anatomy and neurology, the characteristics, clinical picture, diagnosis, and treatment. The practical part is a case report of a patient with peripheral facial palsy. The case report includes the clinical exa...

  19. The association of Varicella zoster virus reactivation with Bell's palsy in children.

    Science.gov (United States)

    Abdel-Aziz, Mosaad; Azab, Noha A; Khalifa, Badwy; Rashed, Mohammed; Naguib, Nader

    2015-03-01

    Bell's palsy is considered the most common cause of facial nerve paralysis in children. Although different theories have been postulated for its diagnosis, reactivation of the Varicella zoster virus (VZV) has been implicated as one of the causes of Bell's palsy. The aim of the study was to evaluate the association of Varicella-zoster virus infection with Bell's palsy and its outcome in children. A total of 30 children with Bell's palsy were recruited and were assayed for evidence of VZV infection. The severity of facial nerve dysfunction and the recovery rate were evaluated according to House-Brackmann Facial Nerve Grading Scale (HB FGS). Paired whole blood samples from all patients were obtained at their initial visit and 3 weeks later, and serum samples were analyzed for VZV IgG and IgM antibodies using ELISA. A significantly higher percentage of Bell's palsy patients were seropositive for VZV IgM antibodies than controls (36.6% of patients vs 10% of controls) while for VZV IgG antibodies the difference was statistically nonsignificant. HB FGS in Bell's palsy patients with serologic evidence of VZV recent infection or reactivation showed a statistiacally significant less cure rate than other patients. VZV reactivation may be an important cause of acute peripheral facial paralysis in children. The appropriate diagnosis of VZV reactivation should be done to improve the outcome and the cure rate by the early use of antiviral treatment. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  20. Pain in young people aged 13 to 17 years with cerebral palsy

    DEFF Research Database (Denmark)

    Parkinson, Kathryn N; Dickinson, Heather O; Arnaud, Catherine

    2013-01-01

    To determine the prevalence and associations of self- and parent-reported pain in young people with cerebral palsy (CP).......To determine the prevalence and associations of self- and parent-reported pain in young people with cerebral palsy (CP)....

  1. THE EFFECTIVENESS OF CONDUCTIVE EDUCATION ON MOTOR SKILLS IN CHILDREN WITH CEREBRAL PALSY

    Directory of Open Access Journals (Sweden)

    Negin Khoshvaght

    2017-12-01

    Full Text Available Background: Cerebral palsy is a nonprogressive neuro-developmental disorders that are caused by damage to the developing brain and affect movement and posture. Children with cerebral palsy suffer difficulty in motor function (coordination and control.The present inquiry investigated the impact of conductive education on motor skills in children having cerebral palsy. Methods: A quasi-experimental research was done using pretest-posttest and control group design. The study subjects consisted of all children with cerebral palsy in Shiraz. A sample of 30 subjects was randomly chosen to employ convenience sampling procedure and classified to two groups of treatment (15 subjects and control (15 subjects. The pretest was performed for both groups, and the experimental group received conductive education in 20 sessions. While the control subjects did not have this education, finally, the post-test was performed for both groups. The Lincoln-Oseretsky test was used to measure motor skills. The data were analyzed using ANCOVA and MANCOVA. Results: The results showed that conductive education had a significant effect on motor skills (P<0.001 and its subscales such as speed of movement (P<0.001, general static coordination (P<0.001, general dynamic coordination (P<0.001, dynamic manual coordination (P<0.001, synchronous-asymmetrical voluntary movements (P<0.001, and asynchronous-asymmetrical voluntary movements (P<0.001 in children with cerebral palsy. Conclusion: The findings indicated the effectiveness of conductive education on cerebral palsy children’s motor skills. Therefore, it is recommended to design and implement a conductive education program to improve motor skills of cerebral palsy children.

  2. Hippotherapy on postural balance in the sitting position of children with cerebral palsy - Longitudinal study.

    Science.gov (United States)

    Moraes, Andréa Gomes; Copetti, Fernando; Ângelo, Vera Regina; Chiavoloni, Luana; de David, Ana Cristina

    2018-06-11

    To verify the effects of 12, 24, 36 hippotherapy sessions over time on postural balance while sitting in children with cerebral palsy as well the effects of treatment after one interruption period of 45 days. Hippotherapy program with a twice-weekly treatment with a total of 13 children aged 5-10 years old. Measurements of postural balance during sitting were performed using the AMTI AccuSway Plus platform. There was a statistically significant reduction in mediolateral and anteroposterior sway after the first 12 hippotherapy sessions, and further significant sway reduction occurred as the treatment progressed. Changes in the center of pressure displacement velocity variable began to occur after 24 sessions. Seated postural balance improved in children with cerebral palsy, as evidenced by lower COP displacement, particularly after a greater number of sessions. After the last evaluations, when completing 36 sessions of hippotherapy, it was verified that the improvements to the postural balance continued to occur. Therefore, further studies with a longer treatment period may help to clarify if, at some point, there is stabilization in the improvement of postural balance. Furthermore, it is important to analyze the impact of hippotherapy on functional activities over time.

  3. Facial nerve palsy associated with a cystic lesion of the temporal bone.

    Science.gov (United States)

    Kim, Na Hyun; Shin, Seung-Ho

    2014-03-01

    Facial nerve palsy results in the loss of facial expression and is most commonly caused by a benign, self-limiting inflammatory condition known as Bell palsy. However, there are other conditions that may cause facial paralysis, such as neoplastic conditions of the facial nerve, traumatic nerve injury, and temporal bone lesions. We present a case of facial nerve palsy concurrent with a benign cystic lesion of the temporal bone, adjacent to the tympanic segment of the facial nerve. The patient's symptoms subsided after facial nerve decompression via a transmastoid approach.

  4. Theory of Mind and Irony Comprehension in Children with Cerebral Palsy

    Science.gov (United States)

    Caillies, Stephanie; Hody, Anais; Calmus, Arnaud

    2012-01-01

    The main goal of the present study was to characterise the pragmatic abilities of French children with cerebral palsy through their understanding of irony and other people's mental states. We predicted that children with cerebral palsy would have difficulty understanding false-belief and ironic remarks, due to the executive dysfunction that…

  5. Cranial nerve palsies in Nigerian children

    African Journals Online (AJOL)

    PROF. EZECHUKWU

    2014-01-08

    Jan 8, 2014 ... Introduction. Cranial nerve palsy is a common clinical problem ... Methodology ... The two cases with three-nerve involvement were re- lated to viral encephalitis and cerebral contusion from ... RTA = road traffic accident.

  6. Facial nerve palsy: analysis of cases reported in children in a suburban hospital in Nigeria.

    Science.gov (United States)

    Folayan, M O; Arobieke, R I; Eziyi, E; Oyetola, E O; Elusiyan, J

    2014-01-01

    The study describes the epidemiology, treatment, and treatment outcomes of the 10 cases of facial nerve palsy seen in children managed at the Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife over a 10 year period. It also compares findings with report from developed countries. This was a retrospective cohort review of pediatric cases of facial nerve palsy encountered in all the clinics run by specialists in the above named hospital. A diagnosis of facial palsy was based on International Classification of Diseases, Ninth Revision, Clinical Modification codes. Information retrieved from the case note included sex, age, number of days with lesion prior to presentation in the clinic, diagnosis, treatment, treatment outcome, and referral clinic. Only 10 cases of facial nerve palsy were diagnosed in the institution during the study period. Prevalence of facial nerve palsy in this hospital was 0.01%. The lesion more commonly affected males and the right side of the face. All cases were associated with infections: Mainly mumps (70% of cases). Case management include the use of steroids and eye pads for cases that presented within 7 days; and steroids, eye pad, and physical therapy for cases that presented later. All cases of facial nerve palsy associated with mumps and malaria infection fully recovered. The two cases of facial nerve palsy associated with otitis media only partially recovered. Facial nerve palsy in pediatric patients is more commonly associated with mumps in the study environment. Successes are recorded with steroid therapy.

  7. X-Ray Hip Examination in Patients with Cerebral Palsy

    OpenAIRE

    Holiuk, Ye.L.

    2017-01-01

    Background. X-ray indicators of the hip are important diagnostic factors of spastic hip dislocation in cerebral palsy. Correct X-ray examination has a decisive influence on the treatment strategy. Correct positioning parameters are well known, but their importance is often underestimated. This could be a trigger factor for further diagnostic and treatment errors. Materials and me-thods. The material was radiographs of the hip joints of 126 patients with cerebral palsy aged 2 to 18 years. Retr...

  8. Clinical significance of quantitative analysis of facial nerve enhancement on MRI in Bell's palsy.

    Science.gov (United States)

    Song, Mee Hyun; Kim, Jinna; Jeon, Ju Hyun; Cho, Chang Il; Yoo, Eun Hye; Lee, Won-Sang; Lee, Ho-Ki

    2008-11-01

    Quantitative analysis of the facial nerve on the lesion side as well as the normal side, which allowed for more accurate measurement of facial nerve enhancement in patients with facial palsy, showed statistically significant correlation with the initial severity of facial nerve inflammation, although little prognostic significance was shown. This study investigated the clinical significance of quantitative measurement of facial nerve enhancement in patients with Bell's palsy by analyzing the enhancement pattern and correlating MRI findings with initial severity of facial palsy and clinical outcome. Facial nerve enhancement was measured quantitatively by using the region of interest on pre- and postcontrast T1-weighted images in 44 patients diagnosed with Bell's palsy. The signal intensity increase on the lesion side was first compared with that of the contralateral side and then correlated with the initial degree of facial palsy and prognosis. The lesion side showed significantly higher signal intensity increase compared with the normal side in all of the segments except for the mastoid segment. Signal intensity increase at the internal auditory canal and labyrinthine segments showed correlation with the initial degree of facial palsy but no significant difference was found between different prognostic groups.

  9. Acupuncture for sequelae of Bell's palsy: a randomized controlled trial protocol.

    Science.gov (United States)

    Kwon, Hyo-Jung; Kim, Jong-In; Lee, Myeong Soo; Choi, Jun-Yong; Kang, Sungkeel; Chung, Jie-Yoon; Kim, Young-Jin; Lee, Seung-Hoon; Lee, Sanghoon; Nam, Dongwoo; Kim, Yong-Suk; Lee, Jae-Dong; Choi, Do-Young

    2011-03-09

    Incomplete recovery from facial palsy has a long-term impact on the quality of life, and medical options for the sequelae of Bell's palsy are limited. Invasive treatments and physiotherapy have been employed to relieve symptoms, but there is limited clinical evidence for their effectiveness. Acupuncture is widely used on Bell's palsy patients in East Asia, but there is insufficient evidence for its effectiveness on Bell's palsy sequelae. The objective is to evaluate the efficacy and safety of acupuncture in patients with sequelae of Bell's palsy. This study consists of a randomized controlled trial with two parallel arms: an acupuncture group and a waitlist group. The acupuncture group will receive acupuncture treatment three times per week for a total of 24 sessions over 8 weeks. Participants in the waitlist group will not receive any acupuncture treatments during this 8 week period, but they will participate in the evaluations of symptoms at the start of the study, at 5 weeks and at 8 weeks after randomization, at which point the same treatment as the acupuncture group will be provided. The primary outcome will be analyzed by the change in the Facial Disability Index (FDI) from baseline to week eight. The secondary outcome measures will include FDI from baseline to week five, House-Brackmann Grade, lip mobility, and stiffness scales.

  10. Motion Tracking of Infants in Risk of Cerebral Palsy

    DEFF Research Database (Denmark)

    Olsen, Mikkel Damgaard

    Every year 2-3 out of 1000 infants are born with cerebral cerebral palsy. Among others, the disorder often affects motor, cognitive and perceptual skills. The disorder is usually detected when the infants are old enough the crawl and walk, i.e. when the infant is 1-2 years old. However, studies...... show that the infant’s movements are affected already in the first year of life and methods exist for assessing the movements. The methods often require observation of the movements and qualitative evaluation of these. A more objective measure is desired in order to be able to diagnose cerebral palsy...... for automatic assessment of infant movement. This includes a preliminary study on automatic classification of movements related to cerebral palsy. The contributions included in this thesis can be divided into two groups. The first two contributions consider the analysis in order to estimate and track the body...

  11. A Stepwise Approach: Decreasing Infection in Deep Brain Stimulation for Childhood Dystonic Cerebral Palsy.

    Science.gov (United States)

    Johans, Stephen J; Swong, Kevin N; Hofler, Ryan C; Anderson, Douglas E

    2017-09-01

    Dystonia is a movement disorder characterized by involuntary muscle contractions, which cause twisting movements or abnormal postures. Deep brain stimulation has been used to improve the quality of life for secondary dystonia caused by cerebral palsy. Despite being a viable treatment option for childhood dystonic cerebral palsy, deep brain stimulation is associated with a high rate of infection in children. The authors present a small series of patients with dystonic cerebral palsy who underwent a stepwise approach for bilateral globus pallidus interna deep brain stimulation placement in order to decrease the rate of infection. Four children with dystonic cerebral palsy who underwent a total of 13 surgical procedures (electrode and battery placement) were identified via a retrospective review. There were zero postoperative infections. Using a multistaged surgical plan for pediatric patients with dystonic cerebral palsy undergoing deep brain stimulation may help to reduce the risk of infection.

  12. Speech therapy in peripheral facial palsy: an orofacial myofunctional approach

    Directory of Open Access Journals (Sweden)

    Hipólito Virgílio Magalhães Júnior

    2009-12-01

    Full Text Available Objective: To delineate the contributions of speech therapy in the rehabilitation of peripheral facial palsy, describing the role of orofacial myofunctional approach in this process. Methods: A literature review of published articles since 1995, held from March to December 2008, based on the characterization of peripheral facial palsy and its relation with speechlanguage disorders related to orofacial disorders in mobility, speech and chewing, among others. The review prioritized scientific journal articles and specific chapters from the studied period. As inclusion criteria, the literature should contain data on peripheral facial palsy, quotes on the changes in the stomatognathic system and on orofacial miofunctional approach. We excluded studies that addressed central paralysis, congenital palsy and those of non idiopathic causes. Results: The literature has addressed the contribution of speech therapy in the rehabilitation of facial symmetry, with improvement in the retention of liquids and soft foods during chewing and swallowing. The orofacial myofunctional approach contextualized the role of speech therapy in the improvement of the coordination of speech articulation and in the gain of oral control during chewing and swallowing Conclusion: Speech therapy in peripheral facial palsy contributed and was outlined by applying the orofacial myofunctional approach in the reestablishment of facial symmetry, from the work directed to the functions of the stomatognathic system, including oralfacial exercises and training of chewing in association with the training of the joint. There is a need for a greater number of publications in this specific area for speech therapy professional.

  13. [A young woman with central facial nerve palsy].

    Science.gov (United States)

    Broere, Christiaan M; de Witte, B R René; Claes, J F H M Franka

    2014-01-01

    The distinction between central and peripheral facial nerve palsy can be difficult but is very important for the workup and treatment. A tumefactive demyelinating lesion (TDL) is a rare condition that can sometimes cause diagnostic difficulties due to its similarity to a brain tumour. We present a 20-year-old female patient who visited her GP with a discrete right-sided drooping corner of her mouth. The GP started treatment with oral glucorticoids because of presumed Bell's palsy and referred her to the neurology outpatient clinic. Repeated neurological examination showed central facial palsy on the right side of the face. An MRI study of the brain revealed a single large contrast-enhanced abnormality in the left hemisphere that was diagnosed as TDL after exclusion of other causes. In view of the limited number of clinical symptoms, an expectative policy was conducted. The patient recovered spontaneously and repeated MRI studies showed partial regression of TDL. TDL is often considered to be a first presentation of multiple sclerosis. Accurate analysis with MRI can help in making a diagnosis without the need for a biopsy.

  14. Handling the Cerebral Palsied Child: Multi-Level Skills Transfer in Pakistan.

    Science.gov (United States)

    Miles, M.; Frizzell, Yvonne

    1990-01-01

    The majority of children with cerebral palsy in developing countries have no access to trained therapists; for example, in Pakistan, there is less than one trained general physiotherapist per million population. In Pakistan, cerebral palsy handling skills were taught to a group of parents, teachers, and paraprofessionals in a series of practical…

  15. Attentional and executive impairments in children with spastic cerebral palsy

    DEFF Research Database (Denmark)

    Bottcher, Louise; Flachs, Esben Meulengracht; Uldall, Peter

    2010-01-01

    Children with cerebral palsy (CP) are reported to have learning and social problems. The aim of the present study was to examine whether children with CP have impairments in attention or executive function.......Children with cerebral palsy (CP) are reported to have learning and social problems. The aim of the present study was to examine whether children with CP have impairments in attention or executive function....

  16. Executive function in relation to arithmetic development in children with cerebral palsy

    NARCIS (Netherlands)

    Jenks, K.M.; de Moor, J.; van Lieshout, E.C.D.M.

    2009-01-01

    Background: Although it is believed that children with cerebral palsy are at high risk for learning difficulties and arithmetic difficulties in particular, few studies have investigated this issue. Methods: Arithmetic ability was longitudinally assessed in children with cerebral palsy in special (n

  17. Carcinoma de glándulas sebáceas limitado a conjuntiva bulbar

    Directory of Open Access Journals (Sweden)

    Manuel Fernando Buitrago-Torrado

    Full Text Available El carcinoma de glándulas sebáceas es un tumor infrecuente que puede desarrollarse a partir de cualquier glándula sebácea en la piel. El 75 % de las veces es de origen ocular y afecta principalmente las glándulas de Zeiss, Meibomio y de la carúncula. Se caracteriza por un comportamiento agresivo, con alta probabilidad de invasión a piel, conjuntiva y córnea. Sin embargo, el compromiso de la conjuntiva como localización primaria es raro. Se presenta un caso con diagnóstico de carcinoma sebáceo de patrón nodular primario de la conjuntiva bulbar, atendido en el Hospital Universitario de Santander durante los años 2014-2016. El propósito del presente estudio es dar a conocer el caso de una patología infrecuente con pocos casos reportados en la literatura, enfatizar su importancia dentro de los diagnósticos diferenciales de masas en la conjuntiva y del estudio histopatológico como método para obtener un diagnóstico definitivo y realizar un abordaje temprano.

  18. Rehabilitation of Bells' palsy from a multi-team perspective.

    Science.gov (United States)

    Hultcrantz, Malou

    2016-01-01

    Conclusions Defectively healed facial paralysis causes difficulties to talk and eat, involuntary spasms (synkinesis), and cosmetic deformities which can give rise both to severe psychological and physical trauma. A team consisting of Ear-Nose-Throat specialists, Plastic surgeons and Physiotherapists can offer better care, treatment and outcome for patients suffering from Bells' palsy. Objectives Patients suffering from Bells' palsy from all ENT hospitals in Sweden and the University Hospital in Helsinki has been included. Methods Results have been drawn and statistically processed for different outcomes from a prospective, double blind cross over study. Results from a pilot surgical study and therapeutic results from physiotherapy studies have been included. Ideas concerning different kinds of surgery will be reviewed and the role of physiotherapy discussed. Results According to common results, treatment with Prednisolone enhances the recovery rate and should, if possible, be used early in the course. Sunnybrook grading at 1 month after onset most accurately predicts non-recovery at 12 months in Bells' palsy and a risk factor curve will be presented in order to predict outcome and selection of patients for undergoing facial surgery. This report is focusing on how to handle patients with Bells' palsy from a multi-rehabilitation team point of view, and what will be recommended to provide these patients with the best clinical and surgical help.

  19. Combined measurement of plasma cystatin C and low-density lipoprotein cholesterol: A valuable tool for evaluating progressive supranuclear palsy.

    Science.gov (United States)

    Weng, Ruihui; Wei, Xiaobo; Yu, Bin; Zhu, Shuzhen; Yang, Xiaohua; Xie, Fen; Zhang, Mahui; Jiang, Ying; Feng, Zhong-Ping; Sun, Hong-Shuo; Xia, Ying; Jin, Kunlin; Chan, Piu; Wang, Qing; Gao, Xiaoya

    2018-07-01

    Progressive supranuclear palsy (PSP) was previously thought as a cause of atypical Parkinsonism. Although Cystatin C (Cys C) and low-density cholesterol lipoprotein-C (LDL-C) are known to play critical roles in Parkinsonism, it is unknown whether they can be used as markers to distinguish PSP patients from healthy subjects and to determine disease severity. We conducted a cross-sectional study to determine plasma Cys C/HDL/LDL-C levels of 40 patients with PSP and 40 healthy age-matched controls. An extended battery of motor and neuropsychological tests, including the PSP-Rating Scale (PSPRS), the Non-Motor Symptoms Scale (NMSS), Geriatric Depression Scale (GDS) and Mini-Mental State Examination (MMSE), was used to evaluate the disease severity. Receiver operating characteristic (ROC) curves were adopted to assess the prognostic accuracy of Cys C/LDL-C levels in distinguishing PSP from healthy subjects. Patients with PSP exhibited significantly higher plasma levels of Cys C and lower LDL-C. The levels of plasma Cys C were positively and inversely correlated with the PSPRS/NMSS and MMSE scores, respectively. The LDL-C/HDL-C ratio was positively associated with PSPRS/NMSS and GDS scores. The ROC curve for the combination of Cys C and LDL-C yielded a better accuracy for distinguishing PSP from healthy subjects than the separate curves for each parameter. Plasma Cys C and LDL-C may be valuable screening tools for differentiating PSP from healthy subjects; while they could be useful for the PSP intensifies and severity evaluation. A better understanding of Cys C and LDL-C may yield insights into the pathogenesis of PSP. Copyright © 2018 Elsevier Ltd. All rights reserved.

  20. MR imaging in Bell's palsy and herpes zoster opticus: correlation with clinical findings

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Jung Ho; Mo, Jong Hyun; Moon, Sung Hee; Lee, Sang Sun; Park, Yang Hee; Lee, Kyung Hee [National Police Hospital, Seoul (Korea, Republic of); Choi, Ik Joon [Sejong General Hospital, Seoul (Korea, Republic of)

    1998-09-01

    To evaluate the MRI findings of acute facial nerve paralysis in Bell's palsy and herpes zoster opticus, and to correlate these with the clinical findings. We retrowspectively reviewed the MRI findings in six cases of BEll's palsy(BP) and two of herpes zoster oticus(HZO), and compared them with the findings for 30 normal facial nerves. This nerve was considered abnormal when its signal intensity was greater than that of brain parenchyma or the contralateral normal side on Gd-enhanced T1-weighted axial and coronal MR images. We analysed the location and degree of contrast enhancement, interval change, and clinical progression in correlation with House-Brackmann(HB) grade and electroneuronography (ENoG) findings. Fifteen of 30 normal facial nerves(50%) seen on Gd-enhanced MRI were mildly enhanced in the geniculate ganglion, the proximal tympanic, and the proximal mastoid segment of the facial nerve. No enhancement of the internal auditory canal(IAC) or labyrinthine segment of the facial nerve was noted, however. In BP and HZO, Gd-enhanced MR images revealed fair to marked enhancement for more than two segments from the internal auditory canal to the mastoid segment of the facial nerve. During follow-up MRI, enhancement of the facial nerve varied in location and signal intensity, though gradually decreased in intensity approximately eight weeks after the onset of facial nerve palsy. No correlation between clinical HB grade, ENoG, and follow up MRI findings was noted. Except in the internal auditory canal and labyrinthine segment, normal facial nevemay show mild and relatively symmetrical enhancement. In BP and HZO, the facial nerve showed diffuse enhancement from the IAC to the mastoid segment.=20.

  1. Self-reported quality of life of adolescents with cerebral palsy

    DEFF Research Database (Denmark)

    Colver, Allan; Rapp, Marion; Eisemann, Nora

    2015-01-01

    compared QoL against matched controls in the general population. We used multivariable regression to relate QoL of adolescents with cerebral palsy to impairments (cross-sectional analysis) and to childhood QoL, pain, psychological problems, and parenting stress (longitudinal analysis). FINDINGS: Severity...... QoL. Child psychological problems and parenting stress in childhood or their worsening between childhood and adolescence predicted only small reductions in adolescent QoL. INTERPRETATION: Individual and societal attitudes should be affected by the similarity of the QoL of adolescents with and without...... cerebral palsy. Adolescents with cerebral palsy need particular help to maintain and develop peer relationships. Interventions in childhood to alleviate psychological difficulties, parenting stress, and especially pain, are justified for their intrinsic value and for their longer term effect on adolescent...

  2. Traumatic facial nerve neuroma with facial palsy presenting in infancy.

    Science.gov (United States)

    Clark, James H; Burger, Peter C; Boahene, Derek Kofi; Niparko, John K

    2010-07-01

    To describe the management of traumatic neuroma of the facial nerve in a child and literature review. Sixteen-month-old male subject. Radiological imaging and surgery. Facial nerve function. The patient presented at 16 months with a right facial palsy and was found to have a right facial nerve traumatic neuroma. A transmastoid, middle fossa resection of the right facial nerve lesion was undertaken with a successful facial nerve-to-hypoglossal nerve anastomosis. The facial palsy improved postoperatively. A traumatic neuroma should be considered in an infant who presents with facial palsy, even in the absence of an obvious history of trauma. The treatment of such lesion is complex in any age group but especially in young children. Symptoms, age, lesion size, growth rate, and facial nerve function determine the appropriate management.

  3. Acoustic Reflex and House-Brackmann Rating Scale as Prognostic Indicators of Peripheral Facial Palsy in Neuroborreliosis.

    Science.gov (United States)

    Sekelj, Alen; Đanić, Davorin

    2017-09-01

    Lyme borreliosis is a vector-borne infectious disease characterized by three disease stages. In the areas endemic for borreliosis, every acute facial palsy indicates serologic testing and implies specific approach to the disease. Th e aim of the study was to identify and confirm the value of acoustic refl ex and House-Brackman (HB) grading scale as prognostic indicators of facial palsy in neuroborreliosis. Th e study included 176 patients with acute facial palsy divided into three groups based on serologic testing: borreliosis, Bell's palsy, and facial palsy caused by herpes simplex virus type 1 (HSV-1). Study patients underwent baseline audiometry with tympanometry and acoustic reflex, whereas current state of facial palsy was assessed by the HB scale. Subsequently, the same tests were obtained on three occasions, i.e. in week 3, 6 and 12 of presentation. Th e patients diagnosed with borreliosis, Bell's palsy and HSV-1 differed according to the time to acoustic refl ex recovery, which took longest time in patients with borreliosis. Th ese patients had the highest percentage of suprastapedial lesions at all time points and recovery was achieved later as compared with the other two diagnoses. Th e mean score on the HB scale declined with time, also at a slower rate in borreliosis patients. Th e prognosis of acoustic refl ex and facial palsy recovery according to HB scale was not associated with the length of elapsed time. The results obtained in the present study strongly confirmed the role of acoustic reflex and HB grading scale as prognostic indicators of facial palsy in neuroborreliosis.

  4. Case report of physiotherapy care for a patient with facial nervus peripheral palsy

    OpenAIRE

    Kubartová, Veronika

    2016-01-01

    The title of the thesis: Case report of physiotherapy care for a patient with facial nervus peripheral palsy The aim of the thesis: The aim is to get acquainted with theoretical knowledge on the issues and the development of case studies, including design and the course of therapy for a patient with a diagnosis of facial nervus peripheral palsy. Methodology and evaluation: The content of this thesis is to introduce theoretical knowledge of facial nervus peripheral palsy, its treatment and reh...

  5. A prospective study of 228 cases of Bell's palsy

    Directory of Open Access Journals (Sweden)

    Soltanzadeh A

    1993-05-01

    Full Text Available Two hundred and twenty-eight patients who had developed Bell's palsy were investigated in a prospective study. Among them, 55% were male and 45% were female. The most common age of the disease onset was the third and beginning of the fourth decade of life. We found definite correlation between Bell's palsy, hypertension, and diabetes; its recurrence rate was 9%. The most important complication was facial asymmetry that remained in 15% of the patients. The most common symptom prior to the development of facial palsy was the existence of pain in the back of ears and neck. Our patients responded well to 8 mg dexamethasone administered for a period of one week

  6. Clinical study on the efficacy of Rajayapana Basti and Baladi Yoga in motor disabilities of cerebral palsy in children.

    Science.gov (United States)

    Shailaja, U; Rao, Prasanna N; Girish, K J; Arun Raj, G R

    2014-01-01

    Cerebral palsy is a static encephalopathy that may be defined as a non-progressive disorder of posture and movement often associated with epilepsy and abnormalities in speech, vision and intellect resulting from a defect or lesion of the developing brain. There are 25 lakhs cerebral palsy affected children in India. To assess the efficacy of Rajayapana Basti (RB) and Baladi Yoga in motor disabilities of cerebral palsy in children. Total 98 children satisfying diagnostic criteria and between the age group of 2-10 years were included and randomly divided into two groups. In RB with Baladi group (n = 40) patients were treated with Mustadi Rajayapana Basti for 8 days, followed by oral administration of Baladi Yoga with honey and ghee for 60 days. Before administering Basti, patients were subjected to Sarvanga Abhyanga and Sastikashali Pinda Sveda. In the control group (n = 40), patients were given tablets of Godhuma Choorna for 60 days. Before administering the placebo tablet, the patients of the control group were given Sarvanga Abhyanga and Sastikashali Pinda Sveda for 8 days. The patients of the control group were given Basti with lukewarm water for 8 days. RB group has shown improvements in understanding ability (13.43%), speech (10%) and performance skill (11.11%), in fine motor functions such as putting small object in to a container (14.3%), throws the ball in all direction (21.8%), use of thumb and index finger (10.93%), retaining 2 inch cube in fist (19.04%), folds paper and inserts into envelope (10.30%), in gross motor functions such as in crawling (26.7%), sitting (31.7%), standing (13.75%), walking (9.5%) and claps hands (13.9%) respectively. Mustadi RB along with Baladi Yoga provided a significant improvement in all the parameters and has promising result in managing motor disabilities of cerebral palsy in children.

  7. Changes in Cardiorespiratory Responses and Kinematics with Hippotherapy in Youth with and without Cerebral Palsy

    Science.gov (United States)

    Rigby, Brandon Rhett; Gloeckner, Adam Robert; Sessums, Suzanne; Lanning, Beth Anne; Grandjean, Peter Walter

    2017-01-01

    Purpose: The purpose of this study was to characterize pelvic displacement and cardiorespiratory responses to simulated horseback riding and walking in youth with cerebral palsy and to compare responses to youth without cerebral palsy before and after 8 weeks of hippotherapy. Method: Eight youth with cerebral palsy (M[subscript age] = 10 ± 4…

  8. A three-generation family with idiopathic facial palsy suggesting an autosomal dominant inheritance with high penetrance

    DEFF Research Database (Denmark)

    Larsen, Christian Grønhøj; Gyldenløve, Mette; Jønch, Aia Elise

    2015-01-01

    Idiopathic facial palsy (IFP), also known as Bell's palsy, is a common neurologic disorder, but recurrent and familial forms are rare. This case series presents a three-generation family with idiopathic facial palsy. The mode of inheritance of IFP has previously been suggested as autosomal dominant...

  9. Acupuncture for sequelae of Bell's palsy: a randomized controlled trial protocol

    Directory of Open Access Journals (Sweden)

    Kim Yong-Suk

    2011-03-01

    Full Text Available Abstract Objective Incomplete recovery from facial palsy has a long-term impact on the quality of life, and medical options for the sequelae of Bell's palsy are limited. Invasive treatments and physiotherapy have been employed to relieve symptoms, but there is limited clinical evidence for their effectiveness. Acupuncture is widely used on Bell's palsy patients in East Asia, but there is insufficient evidence for its effectiveness on Bell's palsy sequelae. The objective is to evaluate the efficacy and safety of acupuncture in patients with sequelae of Bell's palsy. Method/Design This study consists of a randomized controlled trial with two parallel arms: an acupuncture group and a waitlist group. The acupuncture group will receive acupuncture treatment three times per week for a total of 24 sessions over 8 weeks. Participants in the waitlist group will not receive any acupuncture treatments during this 8 week period, but they will participate in the evaluations of symptoms at the start of the study, at 5 weeks and at 8 weeks after randomization, at which point the same treatment as the acupuncture group will be provided. The primary outcome will be analyzed by the change in the Facial Disability Index (FDI from baseline to week eight. The secondary outcome measures will include FDI from baseline to week five, House-Brackmann Grade, lip mobility, and stiffness scales. Trial registration Current Controlled-Trials ISRCTN43104115; registration date: 06 July 2010; the date of the first patient's randomization: 04 August 2010

  10. Acupuncture for sequelae of Bell's palsy: a randomized controlled trial protocol

    Science.gov (United States)

    2011-01-01

    Objective Incomplete recovery from facial palsy has a long-term impact on the quality of life, and medical options for the sequelae of Bell's palsy are limited. Invasive treatments and physiotherapy have been employed to relieve symptoms, but there is limited clinical evidence for their effectiveness. Acupuncture is widely used on Bell's palsy patients in East Asia, but there is insufficient evidence for its effectiveness on Bell's palsy sequelae. The objective is to evaluate the efficacy and safety of acupuncture in patients with sequelae of Bell's palsy. Method/Design This study consists of a randomized controlled trial with two parallel arms: an acupuncture group and a waitlist group. The acupuncture group will receive acupuncture treatment three times per week for a total of 24 sessions over 8 weeks. Participants in the waitlist group will not receive any acupuncture treatments during this 8 week period, but they will participate in the evaluations of symptoms at the start of the study, at 5 weeks and at 8 weeks after randomization, at which point the same treatment as the acupuncture group will be provided. The primary outcome will be analyzed by the change in the Facial Disability Index (FDI) from baseline to week eight. The secondary outcome measures will include FDI from baseline to week five, House-Brackmann Grade, lip mobility, and stiffness scales. Trial registration Current Controlled-Trials ISRCTN43104115; registration date: 06 July 2010; the date of the first patient's randomization: 04 August 2010 PMID:21388554

  11. Treatment of os odontoideum in a patient with spastic quadriplegic cerebral palsy.

    Science.gov (United States)

    Akpolat, Yusuf T; Fegale, Ben; Cheng, Wayne K

    2015-08-01

    Severe atlantoaxial instability due to os odontoideum in a patient with spastic cerebral palsy has not been well described. There is no consensus on treatment, particularly with regard to conservative or surgical options. Our patient was a 9-year-old girl with spastic cerebral palsy and unstable os odontoideum as an incidental finding. During the waiting period for elective surgical treatment, the patient developed respiratory compromise. Surgery was performed to reduce the subluxation and for C1-C2 arthrodesis and the girl regained baseline respiratory function. A CT scan was obtained 1 year after the initial surgery and revealed adequate maintenance of reduction and patency of the spinal canal. This patient highlights the fact that unstable os odontoideum can cause mortality due to respiratory distress in patients with spastic cerebral palsy. This is an important factor in deciding treatment options for cerebral palsy patients with low functional demand. We review the relevant literature. Copyright © 2015 Elsevier Ltd. All rights reserved.

  12. What constitutes cerebral palsy in the twenty-first century?

    DEFF Research Database (Denmark)

    Smithers-Sheedy, Hayley; Badawi, Nadia; Blair, Eve

    2014-01-01

    AIM: Determining inclusion/exclusion criteria for cerebral palsy (CP) surveillance is challenging. The aims of this paper were to (1) define inclusion/exclusion criteria that have been adopted uniformly by surveillance programmes and identify where consensus is still elusive, and (2) provide...... (SCPE; 1976-1998). An expert panel used a consensus building technique, which utilized the SCPE 'decision tree' and the original 'What constitutes cerebral palsy?' paper as frameworks. RESULTS: CP surveillance programmes agree on key clinical criteria pertaining to the type, severity, and origin...

  13. [Treatment of idiopathic peripheral facial nerve paralysis (Bell's palsy)].

    Science.gov (United States)

    Meyer, Martin Willy; Hahn, Christoffer Holst

    2013-01-28

    Bell's palsy is defined as an idiopathic peripheral facial nerve paralysis of sudden onset. It affects 11-40 persons per 100,000 per annum. Many patients recover without intervention; however, up to 30% have poor recovery of facial muscle control and experience facial disfigurement. The aim of this study was to make an overview of which pharmacological treatments have been used to improve outcomes. The available evidence from randomized controlled trials shows significant benefit from treating Bell's palsy with corticosteroids but shows no benefit from antivirals.

  14. [Functional electric stimulation (FES) in cerebral palsy].

    Science.gov (United States)

    Miyazaki, M H; Lourenção, M I; Ribeiro Sobrinho, J B; Battistella, L R

    1992-01-01

    Our study concerns a patient with cerebral palsy, submitted to conventional occupational therapy and functional electrical stimulation. The results as to manual ability, spasticity, sensibility and synkinesis were satisfactory.

  15. Lyme disease and Bell's palsy: an epidemiological study of diagnosis and risk in England.

    Science.gov (United States)

    Cooper, Lilli; Branagan-Harris, Michael; Tuson, Richard; Nduka, Charles

    2017-05-01

    Lyme disease is caused by a tick-borne spirochaete of the Borrelia species. It is associated with facial palsy, is increasingly common in England, and may be misdiagnosed as Bell's palsy. To produce an accurate map of Lyme disease diagnosis in England and to identify patients at risk of developing associated facial nerve palsy, to enable prevention, early diagnosis, and effective treatment. Hospital episode statistics (HES) data in England from the Health and Social Care Information Centre were interrogated from April 2011 to March 2015 for International Classification of Diseases 10th revision (ICD-10) codes A69.2 (Lyme disease) and G51.0 (Bell's palsy) in isolation, and as a combination. Patients' age, sex, postcode, month of diagnosis, and socioeconomic groups as defined according to the English Indices of Deprivation (2004) were also collected. Lyme disease hospital diagnosis increased by 42% per year from 2011 to 2015 in England. Higher incidence areas, largely rural, were mapped. A trend towards socioeconomic privilege and the months of July to September was observed. Facial palsy in combination with Lyme disease is also increasing, particularly in younger patients, with a mean age of 41.7 years, compared with 59.6 years for Bell's palsy and 45.9 years for Lyme disease ( P = 0.05, analysis of variance [ANOVA]). Healthcare practitioners should have a high index of suspicion for Lyme disease following travel in the areas shown, particularly in the summer months. The authors suggest that patients presenting with facial palsy should be tested for Lyme disease. © British Journal of General Practice 2017.

  16. Effects of electrical stimulation in early Bells palsy on facial disability index scores

    Directory of Open Access Journals (Sweden)

    P. Alakram

    2011-01-01

    Full Text Available Recovery following facial nerve palsy is variable. Physiotherapists try  to restore  function  in  patients  with  Bell’s  palsy.  The  choice  of treatment modality  depends  on  the  stage  of  the  condition.  Although limited  evidence  exists  for  the  use  of  electrical  stimulation  in  the acute  stage  of  Bell’s  palsy, some physiotherapists in South Africa have been applying this modality. This study examined the effects of electrical stimulation on functional recovery from  Bell’s palsy using the Facial Disability Index, a tool that documents recovery from the patients’ perspective. A two group pre-test post-test experimental design comprising of 16 patients with Bell’s Palsy of less than 30 days duration was utilized. Patients with a clinical diagnosis of Bell’s Palsy were systematically allocated to the control and experimental groups. Patients (n=16 were pre-tested and post-tested using the Facial Disability Index. Both groups were treated with heat, massage, exercises and given a home program. The experimental group also received electrical stimulation. The FDI of the control group improved between 17, 8% and 95, 4% with a mean of 52, 8%. The improvement in the experimental group ranged between 14, 8% and 126% with a mean of 49, 8%. Certain clinical residuals persisted in a mild form in both groups on discharge from the study.  The effects of electrical stimulation as used in this study during the acute phase of Bell’s palsy, quantified as the FDI was clinically but not statistically significant. A larger sample size, longer stimulation time or both should be investigated.

  17. Bell's palsy before Bell : Cornelis Stalpart van der Wiel's observation of Bell's palsy in 1683

    NARCIS (Netherlands)

    van de Graaf, RC; Nicolai, JPA

    Bell's palsy is named after Sir Charles Bell (1774-1842), who has long been considered to be the first to describe idiopathic facial paralysis in the early 19th century. However, it was discovered that Nicolaus Anton Friedreich (1761-1836) and James Douglas (1675-1742) preceded him in the 18th

  18. Parental Stress and Related Factors in Parents of Children with Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Hui-Yi Wang

    2004-07-01

    Full Text Available Children with cerebral palsy display prominent motor dysfunction associated with other developmental disorders. Parenting a child with cerebral palsy presents a number of challenges and stresses. The first purpose of this study was to compare parental stress in parents of children with cerebral palsy to that in parents of children with typical development. The second purpose was to analyze the correlations between parental stress and parents' characteristics, the child's characteristics, the child's earliest age when rehabilitation was first commenced, and weekly frequency of rehabilitation for the child. A convenience sample of 63 parents of children with cerebral palsy (mean age of children, 4.3 ± 1.8 years was recruited. Forty parents of children with typical development were recruited as a comparison group. All parents filled out the Chinese version of the Parenting Stress Index (PSI, which consists of child domain and parent domain scales. The scores reported by parents of children with cerebral palsy in the child domain, parent domain, and PSI total scale were significantly higher than those for parents in the comparison group. The child domain score was significantly correlated to the child's age and severity of motor disability. A significant correlation was also found between the parent domain score and the child's earliest age of commencing rehabilitation. The PSI total scale score was significantly associated with both the child's severity of motor disability and age of commencing rehabilitation. Clinical professionals should be concerned about parental stress in parents of children with cerebral palsy and provide resources to support such parents. We suggest some strategies to reduce parental stress by strengthening parents' child-care skills.

  19. The natural history and management of brachial plexus birth palsy

    OpenAIRE

    Buterbaugh, Kristin L.; Shah, Apurva S.

    2016-01-01

    Brachial plexus birth palsy (BPBP) is an upper extremity paralysis that occurs due to traction injury of the brachial plexus during childbirth. Approximately 20 % of children with brachial plexus birth palsy will have residual neurologic deficits. These permanent and significant impacts on upper limb function continue to spur interest in optimizing the management of a problem with a highly variable natural history. BPBP is generally diagnosed on clinical examination and does not typically req...

  20. Antiviral treatment for Bell's palsy (idiopathic facial paralysis).

    Science.gov (United States)

    Gagyor, Ildiko; Madhok, Vishnu B; Daly, Fergus; Somasundara, Dhruvashree; Sullivan, Michael; Gammie, Fiona; Sullivan, Frank

    2015-11-09

    Corticosteroids are widely used in the treatment of idiopathic facial paralysis (Bell's palsy), but the effectiveness of additional treatment with an antiviral agent is uncertain. Significant morbidity can be associated with severe cases of Bell's palsy. This review was first published in 2001 and revised several times, most recently in 2009. This version replaces an update of the review in Issue 7 of the Cochrane Library subsequently withdrawn because of an ongoing investigation into the reliability of data from an included study. To assess the effects of antiviral treatments alone or in combination with any other therapy for Bell's palsy. On 7 October 2014 we searched the Cochrane Neuromuscular Disease Group Specialized Register, CENTRAL, MEDLINE, EMBASE, LILACS, DARE, NHS EED, and HTA. We also reviewed the bibliographies of the identified trials and contacted trial authors and known experts in the field and relevant drug companies to identify additional published or unpublished data. We searched clinical trials registries for ongoing studies. We considered randomised controlled trials or quasi-randomised controlled trials of antivirals with and without corticosteroids versus control therapies for the treatment of Bell's palsy. We excluded trials that had a high risk of bias in several domains. Pairs of authors independently assessed trials for relevance, eligibility, and risk of bias, using standard Cochrane procedures. Ten trials, including 2280 participants, met the inclusion criteria and are included in the final analysis. Some of the trials were small, and a number were at high or unclear risk of bias. Other trials did not meet current best standards in allocation concealment and blinding. Incomplete recoveryWe found a significant benefit from adding antivirals to corticosteroids in comparison with corticosteroids alone for people with Bell's palsy (risk ratio (RR) 0.61, 95% confidence interval (CI) 0.39 to 0.97, n = 1315). For people with severe Bell

  1. Variant Anterior Digastric Muscle Transfer for Marginal Mandibular Branch of Facial Nerve Palsy

    Directory of Open Access Journals (Sweden)

    Matthew J. Zdilla, DC

    2014-02-01

    Full Text Available Summary: Marginal mandibular branch of facial nerve (MMBFN palsy is a common consequence of head and neck surgeries. MMBFN palsy results in paralysis of muscles which depress the inferior lip. Current management of MMBFN palsy involves ruination of normal neuromuscular anatomy and physiology to restore symmetry to the mouth. The article outlines the possibility to transfer variant anterior digastric musculature to accomplish reanimation of the mouth without adversely affecting normal nonvariant anatomy. The procedure may have the additional cosmetic benefit of correcting asymmetrical muscular bulk in the submental region.

  2. Congenital Double Elevator Palsy with Sensory Exotropia: A Unique Surgical Management.

    Science.gov (United States)

    Nagpal, R C; Raj, Anuradha; Maitreya, Amit

    2017-01-01

    To report a unique surgical approach for congenital double elevator palsy with sensory exotropia. A 7-year-old boy with congenital double elevator palsy and sensory exotropia was managed surgically by Callahan's procedure with recession and resection of the horizontal recti for exotropia without inferior rectus recession, followed by frontalis sling surgery for congenital ptosis. Favourable surgical outcome was achieved without any complication.

  3. Effectiveness of the hydrotherapy in children with chronic encephalopathy no progressive of the childhood: a systematic review

    OpenAIRE

    Jacques, Karoline de Carvalho; Drumond, Nayana Rocha; Andrade, Silmara Aparecida Figueiredo; Chaves Júnior, Israel Penaforte; Toffol, Walesca Carla de

    2010-01-01

    INTRODUCTION: Now Cerebral Palsy is considered like Chronic Encephalopathy no Progressive of the Childhood and defined as any disorder characterized by alteration in the structure and function of the body, activity and participation due to a lesion no progressive of the brain in development. Several techniques physiotherapy exist for individuals rehabilitation with that pathology, however, no technique stands out as more effective in the literature. The use of the approach hydrotherapy become...

  4. Mielinólise pontina central e extra-pontina em paciente alcoolista sem distúrbios hidro-eletrolíticos: relato de caso Central pontine and extra-pontine myelinolysis in an alcoholic patient without electrolyte disturbances: case report

    Directory of Open Access Journals (Sweden)

    Francisco Manoel B. Germiniani

    2002-12-01

    Full Text Available A mielinólise pontina central (MPC e extra-pontina (MEP é enfermidade desmielinizante do encéfalo associada principalmente com a correção rápida da hiponatremia, que se manifesta principalmente por tetraparesia espástica e paralisia pseudo-bulbar. Existem na literatura poucos relatos de MPC/MEP em pacientes sem evidências de distúrbios hidro-eletrolíticos. Relatamos o caso de um paciente de 39 anos de idade, com história de alcoolismo intenso, com quadro de rápida evolução com tetraparesia espástica associada a paresia de diversos nervos cranianos, com exame de ressonância magnética demonstrando lesões compatíveis com MPC/MEP. Houve boa evolução clínica após tratamento com costicosteróide.Central pontine myelinolysis (CPM and extra-pontine myelinolysis (EPM are different presentations of a demyelinating disorder of the brain more commonly associated with rapid correction of hyponatremia, spastic tetraparesia and pseudo-bulbar palsy. There are in the literature a few cases of CPM/EPM in patients without electrolyte disturbances. We report the case of a 39 year-old man with severe alcoholism, who presented with spastic tetraparesis and palsy of several cranial nerves, associated with lesions in the magnetic resonance compatible with CPM/EPM. The patient had a good follow-up after pulse therapy with corticosteroids.

  5. [Osseous and soft tissue operations for treatment of joint malpositioning in infantile cerebral palsy].

    Science.gov (United States)

    Senst, S

    2013-12-01

    If untreated infantile cerebral palsy (ICP) leads to a progressive shortening of muscles and to progressive joint malpositioning. In addition a number of other disorders also result, in particular sensory disorders, intelligence deficits and epilepsy. In order to optimally treat as many as possible of the problems of multi-handicapped children with ICP it is of immanent importance that physiotherapists, occupational therapists, speech therapists, orthopedic technicians, pediatric orthopedists as well as neuropediatricians and social pediatricians work as a team. Surgical measures to correct joint malpositioning serve to improve the function and also to avoid or alleviate pain. Functional improvement measures are ideally undertaken during or shortly before elementary school age, thus enabling a further verticalization/straightening and mobilization. To improve symmetry and therefore the sitting posture, surgery is also indicated for profoundly disabled patients, sometimes making foot operations necessary. It is only in this way that maximum participation can be guaranteed and if necessary utilizing additional aids, such as Zimmer frames or E-wheelchairs.

  6. Ocular cranial nerve palsies secondary to sphenoid sinusitis

    Institute of Scientific and Technical Information of China (English)

    Aiman El Mograbi; Ethan Soudry

    2017-01-01

    Objective:The clinical presentation of sphenoid sinusitis can be highly variable.Rarely,sphenoid sinusitis may present with cranial nerve complications due to the proximity of these structures to the sphenoid sinus.Method:A case series from Rabin Medical Center and all cases of cranial nerves palsies secondary to sphenoid sinusitis that have been reported in the literature were reviewed.Results:Seventeen patients were identified.The abducent nerve was the most common cranial nerve affected (76%),followed by the oculomotor nerve (18%).One patient had combined oculomotor,trochlear and abducent palsies.The most common pathology was isolated purulent sphenoid sinusitis in 64% followed by allergic fungal sinusitis (AFS) in 18%,and fungal infection in 18%.94% had an acute presentation.The majority (85%) received a combined intravenous antibiotics and surgical treatment.The remainder received conservative treatment alone.Complete recovery of cranial nerve palsy was noted in 82% during follow up.Conclusion:Sphenoid sinusitis presenting as diplopia and headaches is rare.A neoplastic process must be ruled out and early surgical intervention with intravenous antimicrobial therapy carry an excellent outcome with complete resolution of symptoms.

  7. Epilepsy and cerebral palsy: characteristics and trends in children born in 1976-1998

    DEFF Research Database (Denmark)

    Sellier, Elodie; Uldall, Peter; Calado, Eulalia

    2012-01-01

    Although epilepsy is common in children with cerebral palsy (CP), no data exists on prevalence rates of CP and epilepsy.......Although epilepsy is common in children with cerebral palsy (CP), no data exists on prevalence rates of CP and epilepsy....

  8. Multiple Cranial Nerve Palsy Due to Cerebral Venous Thrombosis

    Directory of Open Access Journals (Sweden)

    Esra Eruyar

    2017-04-01

    Full Text Available Cerebral venous thrombosis (CVT is a rare clinical condition between cerebrovasculer diases. The most common findings are headache, seizure and focal neurological deficit. Multiple cranial nerve palsy due to CVT is rarely seen and it is not clear pathology. A pathology that could explain the lack of cranial nerve imaging is carrying suspected diagnosis but the disease is known to provide early diagnosis and treatment. We want to emphasize with this case multipl cranial nerve palsy due to CVT is seen rarely and good response to treatment.

  9. Impact of the surgical strategy on the incidence of C5 nerve root palsy in decompressive cervical surgery.

    Directory of Open Access Journals (Sweden)

    Theresa Krätzig

    Full Text Available Our aim was to identify the impact of different surgical strategies on the incidence of C5 palsy.Degenerative cervical spinal stenosis is a steadily increasing morbidity in the ageing population. Postoperative C5 nerve root palsy is a common complication with severe impact on the patients´ quality of life.We identified 1708 consecutive patients who underwent cervical decompression surgery due to degenerative changes. The incidence of C5 palsy and surgical parameters including type and level of surgery were recorded to identify predictors for C5 nerve palsy.The overall C5 palsy rate was 4.8%, with 18.3% of cases being bilateral. For ACDF alone the palsy rate was low (1.13%, compared to 14.0% of C5 palsy rate after corpectomy. The risk increased with extension of the procedures. Hybrid constructs with corpectomy plus ACDF at C3-6 showed significantly lower rates of C5 palsy (10.7% than corpectomy of two vertebrae (p = 0.005. Multiple regression analysis identified corpectomy of C4 or C5 as a significant predictor. We observed a lower overall incidence for ventral (4.3% compared to dorsal (10.9% approaches (p<0.001. When imaging detected a postoperative shift of the spinal cord at index segment C4/5, palsy rate increased significantly (33.3% vs. 12.5%, p = 0.034.Extended surgical strategies, such as dorsal laminectomies, multilevel corpectomies and procedures with extensive spinal cord shift were shown to display a high risk of C5 palsy. The use of extended procedures should therefore be employed cautiously. Switching to combined surgical methods like ACDF plus corpectomy can reduce the rate of C5 palsy.

  10. Restricted Arm Swing Affects Gait Stability and Increased Walking Speed Alters Trunk Movements in Children with Cerebral Palsy

    Science.gov (United States)

    Delabastita, Tijs; Desloovere, Kaat; Meyns, Pieter

    2016-01-01

    Observational research suggests that in children with cerebral palsy, the altered arm swing is linked to instability during walking. Therefore, the current study investigates whether children with cerebral palsy use their arms more than typically developing children, to enhance gait stability. Evidence also suggests an influence of walking speed on gait stability. Moreover, previous research highlighted a link between walking speed and arm swing. Hence, the experiment aimed to explore differences between typically developing children and children with cerebral palsy taking into account the combined influence of restricting arm swing and increasing walking speed on gait stability. Spatiotemporal gait characteristics, trunk movement parameters and margins of stability were obtained using three dimensional gait analysis to assess gait stability of 26 children with cerebral palsy and 24 typically developing children. Four walking conditions were evaluated: (i) free arm swing and preferred walking speed; (ii) restricted arm swing and preferred walking speed; (iii) free arm swing and high walking speed; and (iv) restricted arm swing and high walking speed. Double support time and trunk acceleration variability increased more when arm swing was restricted in children with bilateral cerebral palsy compared to typically developing children and children with unilateral cerebral palsy. Trunk sway velocity increased more when walking speed was increased in children with unilateral cerebral palsy compared to children with bilateral cerebral palsy and typically developing children and in children with bilateral cerebral palsy compared to typically developing children. Trunk sway velocity increased more when both arm swing was restricted and walking speed was increased in children with bilateral cerebral palsy compared to typically developing children. It is proposed that facilitating arm swing during gait rehabilitation can improve gait stability and decrease trunk movements in

  11. Isolated oculomotor nerve palsy resulting from acute traumatic tentorial subdural hematoma

    Directory of Open Access Journals (Sweden)

    Cui V

    2016-10-01

    Full Text Available Victoria Cui,1 Timur Kouliev2 1Washington University School of Medicine, St Louis, MO, USA; 2Emergency Department, Beijing United Family Hospital, Beijing, China Abstract: Acute subdural hematoma (SDH resulting from head trauma is a potentially life-threatening condition that requires expedient diagnosis and intervention to ensure optimal patient outcomes. Rapidly expanding or large hematomas, elevated intracranial pressure, and associated complications of brain herniation are associated with high mortality rates and poor recovery of neurological function. However, smaller bleeds (clot thickness <10 mm or hematomas occurring in infrequent locations, such as the tentorium cerebelli, may be difficult to recognize and patients may present with unusual or subtle signs and symptoms, including isolated cranial nerve palsies. Knowledge of neuroanatomy supported by modern neuroimaging can greatly aid in recognition and diagnosis of such lesions. In this report, we present a case of isolated oculomotor nerve palsy resulting from compressive tentorial SDH following blunt head trauma, review the literature concerning similar cases, and make recommendations regarding the diagnosis of SDH in patients presenting with isolated cranial nerve palsies. Keywords: head injury, oculomotor, palsy, subdural hematoma, trauma, tentorium, cerebral herniation, intracranial hemorrhage

  12. Bangladesh Cerebral Palsy Register (BCPR): a pilot study to develop a national cerebral palsy (CP) register with surveillance of children for CP.

    Science.gov (United States)

    Khandaker, Gulam; Smithers-Sheedy, Hayley; Islam, Johurul; Alam, Monzurul; Jung, Jenny; Novak, Iona; Booy, Robert; Jones, Cheryl; Badawi, Nadia; Muhit, Mohammad

    2015-09-25

    The causes and pathogenesis of cerebral palsy (CP) are all poorly understood, particularly in low- and middle-income countries (LMIC). There are gaps in knowledge about CP in Bangladesh, especially in the spheres of epidemiological research, intervention and service utilization. In high-income countries CP registers have made substantial contributions to our understanding of CP. In this paper, we describe a pilot study protocol to develop, implement, and evaluate a CP population register in Bangladesh (i.e., Bangladesh Cerebral Palsy Register - BCPR) to facilitate studies on prevalence, severity, aetiology, associated impairments and risk factors for CP. The BCPR will utilise a modified version of the Australian Cerebral Palsy Register (ACPR) on a secured web-based platform hosted by the Cerebral Palsy Alliance Research Institute, Australia. A standard BCPR record form (i.e., data collection form) has been developed in consultation with local and international experts. Using this form, the BPCR will capture information about maternal health, birth history and the nature of disability in all children with CP aged CP will be identified by using the community based Key Informants Method (KIM). Data from the completed BPCR record together with details of assessment by a research physician will be entered into an online data repository. Once implemented, BCPR will be, to the best of our knowledge, the first formalised CP register from a LMIC. Establishment of the BCPR will enable estimates of prevalence; facilitate clinical surveillance and promote research to improve the care of individuals with CP in Bangladesh.

  13. Ocular defects in cerebral palsy

    Directory of Open Access Journals (Sweden)

    Katoch Sabita

    2007-01-01

    Full Text Available There is a high prevalence of ocular defects in children with developmental disabilities. This study evaluated visual disability in a group of 200 cerebral palsy (CP patients and found that 68% of the children had significant visual morbidity. These findings emphasize the need for an early ocular examination in patients with CP.

  14. MANAGEMENT OF CHILDREN WITH CEREBRAL PALSY

    African Journals Online (AJOL)

    Enrique

    ing children with cerbral palsy spans more than 4 decades, and has inspired so many of us to work in .... toy. Prone lying over a longitudinal pillow with weight bearing on the forearms should alternate .... ing being blind and deaf. Assessing a.

  15. Arithmetic difficulties in children with cerebral palsy are related to executive function and working memory.

    Science.gov (United States)

    Jenks, Kathleen M; de Moor, Jan; van Lieshout, Ernest C D M

    2009-07-01

    Although it is believed that children with cerebral palsy are at high risk for learning difficulties and arithmetic difficulties in particular, few studies have investigated this issue. Arithmetic ability was longitudinally assessed in children with cerebral palsy in special (n = 41) and mainstream education (n = 16) and controls in mainstream education (n = 16). Second grade executive function and working memory scores were used to predict third grade arithmetic accuracy and response time. Children with cerebral palsy in special education were less accurate and slower than their peers on all arithmetic tests, even after controlling for IQ, whereas children with cerebral palsy in mainstream education performed as well as controls. Although the performance gap became smaller over time, it did not disappear. Children with cerebral palsy in special education showed evidence of executive function and working memory deficits in shifting, updating, visuospatial sketchpad and phonological loop (for digits, not words) whereas children with cerebral palsy in mainstream education only had a deficit in visuospatial sketchpad. Hierarchical regression revealed that, after controlling for intelligence, components of executive function and working memory explained large proportions of unique variance in arithmetic accuracy and response time and these variables were sufficient to explain group differences in simple, but not complex, arithmetic. Children with cerebral palsy are at risk for specific executive function and working memory deficits that, when present, increase the risk for arithmetic difficulties in these children.

  16. Lifetime costs of cerebral palsy

    DEFF Research Database (Denmark)

    Kruse, Marie; Michelsen, Susan Ishøy; Flachs, Esben Meulengracht

    2009-01-01

    This study quantified the lifetime costs of cerebral palsy (CP) in a register-based setting. It was the first study outside the US to assess the lifetime costs of CP. The lifetime costs attributable to CP were divided into three categories: health care costs, productivity costs, and social costs....... social care costs and productivity costs associated with CP point to a potential gain from labour market interventions that benefit individuals with CP.......This study quantified the lifetime costs of cerebral palsy (CP) in a register-based setting. It was the first study outside the US to assess the lifetime costs of CP. The lifetime costs attributable to CP were divided into three categories: health care costs, productivity costs, and social costs...... in 2000. The prevalence of CP in eastern Denmark was approximately 1.7 per 1000. Information on productivity and the use of health care was retrieved from registers. The lifetime cost of CP was about euro860 000 for men and about euro800 000 for women. The largest component was social care costs...

  17. Neuroimpairment, activity limitation, and participation restriction among children with cerebral palsy in Hong Kong.

    Science.gov (United States)

    Chan, H S S; Lau, P H B; Fong, K H; Poon, D; Lam, C C C

    2005-10-01

    To study children with cerebral palsy in Hong Kong, their neuroimpairment, activity limitation, and participation restriction in society. Parents' opinion on current medical and rehabilitation services was also sought. Systematic survey using questionnaires. Four associations in Hong Kong: Child Assessment Service, Hong Kong Association for Parents of Children with Physical Disabilities, Association of Parents of the Severely Mentally Handicapped, and Hong Kong Physically Handicapped and Able-Bodied Association. Parents of children with cerebral palsy. Neuroimpairment, activity limitation, and participation restriction. Information from 181 children with cerebral palsy was analysed. Among them, 56% were boys. The mean age was 7 years 6 months (standard deviation, 3 years 11 months). The most common diagnostic type was spastic cerebral palsy. Co-morbidities in children with cerebral palsy were common. Limitation in daily activities including mobility and self-care tasks was considerable and this posed great stress to parents when taking care of their children. Children's participation in both social and leisure activities was regarded as a low priority. A high percentage (70%) of parents reported difficulty in travelling. The reasons involved problems in transportation, building access (entry and exit), and attitudes of the general public. These environmental factors restricted the social participation of the children and their families. Over 75% of parents were satisfied with the current medical and rehabilitation services. Children with cerebral palsy have multiple and complex needs. The findings of this study may serve as a reference for parents, service providers, and policy makers to work in partnership to achieve a more comprehensive health-care service for children with cerebral palsy and to facilitate better integration into the community.

  18. Primary oculomotor nerve palsy due to mild head injury. Report of two cases

    International Nuclear Information System (INIS)

    Katsuno, Makoto; Kobayashi, Shiro; Yokota, Hiroyuki; Teramoto, Akira

    2008-01-01

    Two patients with primary oculomotor nerve palsy due to direct mild head injury are reported. They presented with internal ophthalmoplegia, dilated nonreactive pupils, and very mild disturbance in consciousness. Except for the persistent oculomotor nerve palsy, both the patients recovered fully within one week. Neither demonstrated a history that was suggestive of a cause for their oculomotor nerve palsy. Initial CT scans demonstrated localized subarachnoid hemorrhage around the brain stem. One of the patients had sustained a fracture of the anterior clinoid process. As the underlying pathophysiologic mechanism underlying the oculomotor nerve palsy we suspected mild injury to the pupillomotor fibers at the anterior petroclinoidal ligament and that of the pupillary fibers at the posterior petroclinoidal ligament. We speculate that these perforating fibers at the anterior petroclinoidal ligament acted as a fulcrum due to downward displacement of the brainstem at the time of impact. (author)

  19. Recurrent unilateral facial nerve palsy in a child with dehiscent facial nerve canal

    Directory of Open Access Journals (Sweden)

    Christopher Liu

    2016-12-01

    Full Text Available Objective: The dehiscent facial nerve canal has been well documented in histopathological studies of temporal bones as well as in clinical setting. We describe clinical and radiologic features of a child with recurrent facial nerve palsy and dehiscent facial nerve canal. Methods: Retrospective chart review. Results: A 5-year-old male was referred to the otolaryngology clinic for evaluation of recurrent acute otitis media and hearing loss. He also developed recurrent left peripheral FN palsy associated with episodes of bilateral acute otitis media. High resolution computed tomography of the temporal bones revealed incomplete bony coverage of the tympanic segment of the left facial nerve. Conclusions: Recurrent peripheral FN palsy may occur in children with recurrent acute otitis media in the presence of a dehiscent facial nerve canal. Facial nerve canal dehiscence should be considered in the differential diagnosis of children with recurrent peripheral FN palsy.

  20. Evaluation of a physiotherapeutic treatment intervention in "Bell's" facial palsy.

    Science.gov (United States)

    Cederwall, Elisabet; Olsén, Monika Fagevik; Hanner, Per; Fogdestam, Ingemar

    2006-01-01

    The aim of this study was to evaluate a physiotherapeutic treatment intervention in Bell's palsy. A consecutive series of nine patients with Bell's palsy participated in the study. The subjects were enrolled 4-21 weeks after the onset of facial paralysis. The study had a single subject experimental design with a baseline period of 2-6 weeks and a treatment period of 26-42 weeks. The patients were evaluated using a facial grading score, a paresis index and a written questionnaire created for this study. Every patient was taught to perform an exercise program twice daily, including movements of the muscles surrounding the mouth, nose, eyes and forehead. All the patients improved in terms of symmetry at rest, movement and function. In conclusion, patients with remaining symptoms of Bell's palsy appear to experience positive effects from a specific training program. A larger study, however, is needed to fully evaluate the treatment.

  1. Phrenic Nerve Palsy as Initial Presentation of Large Retrosternal Goitre.

    Science.gov (United States)

    Hakeem, Arsheed Hussain; Hakeem, Imtiyaz Hussain; Wani, Fozia Jeelani

    2016-12-01

    Unilateral phrenic nerve palsy as initial presentation of the retrosternal goitre is extremely rare event. This is a case report of a 57-year-old woman with history of cough and breathlessness of 3 months duration, unaware of the thyroid mass. She had large cervico-mediastinal goiter and chest radiograph revealed raised left sided hemidiaphragm. Chest CT scan did not reveal any lung parenchymal or mediastinal pathology. The patient underwent a total thyroidectomy through a cervical approach. The final pathology was in favor of multinodular goitre. Even after 1 year of follow up, phrenic nerve palsy did not improve indicating permanent damage. Phrenic nerve palsy as initial presentation of the retrosternal goitre is unusual event. This case is reported not only because of the rare nature of presentation, but also to make clinicians aware of the entity so that early intervention may prevent attendant morbidity.

  2. Hereditary Neuropathy with Liability to Pressure Palsy: A Recurrent and Bilateral Foot Drop Case Report

    Directory of Open Access Journals (Sweden)

    Filipa Flor-de-Lima

    2013-01-01

    Full Text Available Hereditary neuropathy with liability to pressure palsy is characterized by acute, painless, recurrent mononeuropathies secondary to minor trauma or compression. A 16-year-old boy had the first episode of right foot drop after minor motorcycle accident. Electromyography revealed conduction block and slowing velocity conduction of the right deep peroneal nerve at the fibular head. After motor rehabilitation, he fully recovered. Six months later he had the second episode of foot drop in the opposite site after prolonged squatting position. Electromyography revealed sensorimotor polyneuropathy of left peroneal, sural, posterior tibial, and deep peroneal nerves and also of ulnar, radial, and median nerves of both upper limbs. Histological examination revealed sensory nerve demyelination and focal thickenings of myelin fibers. The diagnosis of hereditary neuropathy with liability to pressure palsy was confirmed by PMP22 deletion of chromosome 17p11.2. He started motor rehabilitation and avoidance of stressing factors with progressive recovery. After one-year followup, he was completely asymptomatic. Recurrent bilateral foot drop history, “sausage-like” swellings of myelin in histological examination, and the results of electromyography led the authors to consider the diagnosis despite negative family history. The authors highlight this rare disease in pediatric population and the importance of high index of clinical suspicion for its diagnosis.

  3. Diffusion tensor imaging of Parkinson's disease, multiple system atrophy and progressive supranuclear palsy: a tract-based spatial statistics study.

    Directory of Open Access Journals (Sweden)

    Amanda Worker

    Full Text Available Although often clinically indistinguishable in the early stages, Parkinson's disease (PD, Multiple System Atrophy (MSA and Progressive Supranuclear Palsy (PSP have distinct neuropathological changes. The aim of the current study was to identify white matter tract neurodegeneration characteristic of each of the three syndromes. Tract-based spatial statistics (TBSS was used to perform a whole-brain automated analysis of diffusion tensor imaging (DTI data to compare differences in fractional anisotropy (FA and mean diffusivity (MD between the three clinical groups and healthy control subjects. Further analyses were conducted to assess the relationship between these putative indices of white matter microstructure and clinical measures of disease severity and symptoms. In PSP, relative to controls, changes in DTI indices consistent with white matter tract degeneration were identified in the corpus callosum, corona radiata, corticospinal tract, superior longitudinal fasciculus, anterior thalamic radiation, superior cerebellar peduncle, medial lemniscus, retrolenticular and anterior limb of the internal capsule, cerebral peduncle and external capsule bilaterally, as well as the left posterior limb of the internal capsule and the right posterior thalamic radiation. MSA patients also displayed differences in the body of the corpus callosum corticospinal tract, cerebellar peduncle, medial lemniscus, anterior and superior corona radiata, posterior limb of the internal capsule external capsule and cerebral peduncle bilaterally, as well as the left anterior limb of the internal capsule and the left anterior thalamic radiation. No significant white matter abnormalities were observed in the PD group. Across groups, MD correlated positively with disease severity in all major white matter tracts. These results show widespread changes in white matter tracts in both PSP and MSA patients, even at a mid-point in the disease process, which are not found in patients

  4. Rhinoscintigraphic analysis of nasal mucociliary function in patients with Bell's palsy.

    Science.gov (United States)

    Boynuegri, S; Ozer, S; Peksoy, I; Acikalin, A; Tuna, E Ü; Dursun, E; Eryilmaz, A

    2016-01-01

    Mucociliary transport (MCT) is an important defense mechanism of the respiratory tract. One of the major factors determining MCT is the ciliary activity of the respiratory epithelium. Rhinoscintigraphy is the most commonly used method for the analysis of mucociliary activity. The aim of this study was to investigate the effect of facial paralysis on the nasal mucociliary clearance. This study included 38 Bell's palsy patients as the study group and 10 subjects without any history of paranasal sinus disease or facial paralysis as the control group. A drop of technetium 99m-labeled macroaggregated albumin (Tc-99m MAA) was placed posterior to the head of the inferior turbinate and followed with a gamma camera. MCT rate was measured as the velocity of Tc-99m MAA drop. The mean MCT rate was 4.27 ± 0.76 millimeters per minute (mm/min) on 20 sides of 10 healthy controls, 4.11 ± 2.91 mm/min on the affected sides of the patients with Bell's palsy, and 6.03 ± 3.13 mm/min on the nonparalyzed sides of the patients. MCT rate was statistically significantly faster in the nonparalyzed side when compared to the paralyzed side in Bell's palsy patients (P = 0.001). MCT rates were not significantly different in the control group and paralyzed sides of the Bell's palsy patients (P = 0.810). The MCT rate was statistically significantly faster in the nonparalyzed sides of Bell's palsy patients when compared to the controls (P = 0.017). This study showed a faster MCT rate on the nonparalyzed side in Bell's palsy patients when compared to the paralyzed side and the control subjects. A compensatory mechanism could be the underlying reason for faster MCT on the nonparalyzed side. Further studies on larger patient groups are needed to investigate the effect of facial paralysis on the MCT and changes of facial nerve function on the opposite, nonparalyzed side of the face.

  5. Neonatal risk factors for cerebral palsy in very preterm babies: case-control study.

    Science.gov (United States)

    Murphy, D. J.; Hope, P. L.; Johnson, A.

    1997-01-01

    OBJECTIVE: To identify neonatal risk factors for cerebral palsy among very preterm babies and in particular the associations independent of the coexistence of antenatal and intrapartum factors. DESIGN: Case-control study. SETTING: Oxford health region. SUBJECTS: Singleton babies born between 1984 and 1990 at less than 32 weeks' gestation who survived to discharge from hospital: 59 with cerebral palsy and 234 randomly selected controls without cerebral palsy. MAIN OUTCOME MEASURES: Adverse neonatal factors expressed as odds ratios and 95% confidence intervals. RESULTS: Factors associated with an increased risk of cerebral palsy after adjustment for gestational age and the presence of previously identified antenatal and intrapartum risk factors were patent ductus arteriosus (odds ratio 2.3; 95% confidence interval 1.2 to 4.5), hypotension (2.3; 1.3 to 4.7), blood transfusion (4.8; 2.5 to 9.3), prolonged ventilation (4.8; 2.5 to 9.0), pneumothorax (3.5; 1.6 to 7.6), sepsis (3.6; 1.8 to 7.4), hyponatraemia (7.9; 2.1 to 29.6) and total parenteral nutrition (5.5; 2.8 to 10.5). Seizures were associated with an increased risk of cerebral palsy (10.0; 4.1 to 24.7), as were parenchymal damage (32; 12.4 to 84.4) and appreciable ventricular dilatation (5.4; 3.0 to 9.8) detected by cerebral ultrasound. CONCLUSION: A reduction in the rate of cerebral palsy in very preterm babies requires an integrated approach to management throughout the antenatal, intrapartum, and neonatal periods. PMID:9040385

  6. Correlation between transverse plan kinematics and foot progression angle in children with spastic diplegia.

    Science.gov (United States)

    Presedo, Ana; Simon, Anne-Laure; Mallet, Cindy; Ilharreborde, Brice; Mazda, Keyvan; Pennecot, Georges-François

    2017-05-01

    In diplegic patients, the orientation of foot progression depends on multiple factors. We investigated the relationship between foot progression alignment, hip and pelvic rotations during gait, femoral anteversion, and tibial torsion. Kinematic and clinical parameters were evaluated for 114 children who walked independently and had not undergone previous surgery. Causes of intoeing presented combined in 72% of cases. Internal foot progression correlated with internal hip rotation and showed an inverse correlation with tibial torsion. Our results indicate that data from clinical examination and gait analysis should be evaluated carefully before making treatment recommendations, especially in terms of the correction of torsional problems, in patients with cerebral palsy.

  7. Quality of life of mothers of children with cerebral palsy and their ...

    African Journals Online (AJOL)

    Quality of life of mothers of children with cerebral palsy and their agematched controls. BOA Adegoke, OO Adenuga, OA Olaleye, CO Akosile. Abstract. Background Caring for a child with cerebral palsy (CP) could negatively impact the quality of life (QoL) of the mothers who are usually the primary caregivers. Studies on the ...

  8. Pittsburgh Compound B and AV-1451 positron emission tomography assessment of molecular pathologies of Alzheimer's disease in progressive supranuclear palsy.

    Science.gov (United States)

    Whitwell, Jennifer L; Ahlskog, J Eric; Tosakulwong, Nirubol; Senjem, Matthew L; Spychalla, Anthony J; Petersen, Ronald C; Jack, Clifford R; Lowe, Val J; Josephs, Keith A

    2018-03-01

    Little is known about Alzheimer's disease molecular proteins, beta-amyloid and paired helical filament (PHF) tau, in progressive supranuclear palsy (PSP). Recent techniques have been developed to allow for investigations of these proteins in PSP. We determined the frequency of beta-amyloid deposition in PSP, and whether beta-amyloid deposition in PSP is associated with PHF-tau deposition pattern, or clinical features. Thirty probable PSP participants underwent MRI, [ 18 F]AV-1451 PET and Pittsburgh compound B (PiB) PET. Apolipoprotein (APOE) genotyping was also performed. A global PiB standard-uptake value ratio (SUVR) was calculated. AV-1451 SUVRs were calculated for a set of Alzheimer's disease (AD)-related regions and a set of PSP-related regions. Voxel-level analyses were conducted to assess for differences in AV-1451 uptake patterns and MRI atrophy between PiB(+) and PiB(-) cases compared to 60 normal PiB(-) controls. Statistical testing for correlations and associations between variables of interest were also performed. Twelve subjects (40%) showed beta-amyloid deposition. Higher PiB SUVR correlated with older age but not with AV-1451 SUVR in the AD- or PSP-related regions. Higher AV-1451 SUVR in AD-related regions was associated with higher AV-1451 SUVR in PSP-related regions. We found little evidence for beta-amyloid related differences in clinical metrics, proportion of APOE e4 carriers, pattern of AV-1451 uptake, or pattern of atrophy. Beta-amyloid deposition occurs in a relatively high proportion of PSP subjects. Unlike in Alzheimer's disease, however, there is little evidence that beta-amyloid, and PHF-tau, play a significant role in neurodegeneration in PSP. Copyright © 2017 Elsevier Ltd. All rights reserved.

  9. Physical activity as a prescription for the children with cerebral palsy

    Directory of Open Access Journals (Sweden)

    Arghavan Shariat

    2014-03-01

    Full Text Available After so many years devoted to practicing medicine as children physiotherapists, the therapists finally found the importance of balance training exercises in children who suffer from cerebral palsy. It is only through controlling balance that we can achieve improvement in body movement and position that will culminate into performance independence in a child. Formerly, working over controlling balance in cerebral palsy children has been very difficult, because contracture and spasticity did not let us to have an effective balance training exercise. In this respect, we have summarized the results of previous authors that specify the level of effectiveness of exercise therapy. The results of different studies showed that level of effectiveness of exercise therapy on alleviating the symptoms of spastic cerebral palsy is average in accordance with Cohen's effect size Interpretation table.

  10. Role of magnetic resonance planimetry and magnetic resonance parkinsonism index in discriminating Parkinson’s disease and progressive supranuclear palsy: a retrospective study based on 1.5 and 3 T MRI

    Directory of Open Access Journals (Sweden)

    Nizamani WM

    2017-10-01

    Full Text Available Waseem Mehmood Nizamani,1 Fatima Mubarak,1 Muhammad Danish Barakzai,1 Muhammad Saad Ahmed2 1Department of Radiology, Aga Khan University Hospital, Karachi, Pakistan, 2Department of Radiology, Ziauddin University Hospital, Karachi, Pakistan Objective: The objective of the study was to assess magnetic resonance (MR planimetric measurements and MR parkinsonism index (MRPI in differentiating progressive supranuclear palsy (PSP from Parkinson’s disease (PD using 1.5 and 3 T MRI scanner. Subjects and methods: After ethical approval was obtained, analysis of 34 consecutive patients with PSP, 34 patients with PD and 34 healthy controls (HCs was performed. HCs were age-matched adults without any history of neurodegenerative disease or movement disorders. Retrospective data from the past 10 years (from January 2006 to December 2015 were obtained from the Hospital Information Management System, and informed consent was obtained from all participants. The measurements of pons area–midbrain area ratio (P/M and MCP width–superior cerebellar peduncle (SCP width ratio (MCP/SCP were used, and MRPI was calculated by the formula ([P/M]×[MCP/SCP]. Results: Midbrain area and SCP width in patients with PSP (19 males, 15 females; mean age =66.7 years were significantly (P<0.001 smaller than in patients with PD (20 males, 14 females; mean age =66.7 years and control participants (17 males, 17 females; mean age =66.1 years. P/M and MCP/SCP were significantly higher in patients with PSP than in patients with PD and control participants. All measurements showed some overlap of values between patients with PSP and patients from PD group and control participants. MRPI value was significantly higher in patients with PSP (mean 21.00 than in patients with PD (mean 9.50; P<0.001 and control participants (mean 9.6; P<0.001, without any overlap of values among groups. No correlation was found between the duration of disease, PSP rating scale, PSP staging system and MRPI in

  11. Children's perceptions of their cerebral palsy and their impact on life satisfaction.

    Science.gov (United States)

    Chong, Jimmy; Mackey, Anna H; Broadbent, Elizabeth; Stott, N Susan

    2012-01-01

    To assess an individual child's cognitive and emotional perceptions of their cerebral palsy (CP) and how these are associated with their reported life satisfaction and their functional walking ability. Convenience sample of 48 children with cerebral palsy, GMFCS (Gross Motor Function Classification System) I-IV, mean age of 12.2 ± 2.5 years was recruited from tertiary level out-patient clinics. All children completed the Brief Illness Perception Questionnaire-Cerebral Palsy version (BIPQ-CP), Students' Life Satisfaction Scale (SLSS) and 1- and 6-min walk tests. Children with CP reported levels of global life satisfaction (mean score 31.4/42) equivalent to previous studies of typically developing children. Higher total SLSS scores were associated with lower concern about CP (rho = -0.61, p life satisfaction. Life satisfaction in this group of children was strongly associated with a child's perceptions of their CP but was not associated with functional walking ability. Although the cross-sectional nature of the study precludes assumptions of causality, understanding children's cognitive and emotional beliefs about their cerebral palsy would seem to be an important adjunct to clinical management.

  12. Unilateral abducens and bilateral facial nerve palsies associated with posterior fossa exploration surgery.

    Science.gov (United States)

    Khalil, Ayman; Clerkin, James; Mandiwanza, Tafadzwa; Green, Sandra; Javadpour, Mohsen

    2016-03-06

    Multiple cranial nerves palsies following a posterior fossa exploration confined to an extradural compartment is a rare clinical presentation. This case report describes a young man who developed a unilateral abducens and bilateral facial nerve palsies following a posterior fossa exploration confined to an extradural compartment. There are different theories to explain this presentation, but the exact mechanism remains unclear. We propose that this patient cranial nerve palsies developed following cerebrospinal fluid (CSF) leak, potentially as a consequence of rapid change in CSF dynamics. Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2016.

  13. The Effect of Hominis Placenta Herbal Acupuncture on Bell's palsy

    Directory of Open Access Journals (Sweden)

    Yun Jeong-hun

    2000-07-01

    Full Text Available This report was done to observe the effect of Hominis placenta herbal acupuncture on Bell's palsy. The study group comprised 16 patients who arrived at Woo-suk university oriental hospital from January, 1999 till January, 2000 for Bell's palsy. All patients were divided into two group. One was herbal acupunture group, and the other was control group. Acupunture group was done herbal acupuncture therapy on the facial acupuncture points. Followings are achievement and a term of each group. In herbal acupuncture group, 100% motor recovery was 7 case, 75% was 1 case, and 25% motor recovery term was 7.38±5.21 days, 50% was 11.00±6.16 days, 75% was 15.13±9.55 days, 100% was 23.14±7.97 days. In control group, 100% motor recovery was 4 case, 75% was 2 case, 25% below was 2 case and 25% motor recovery term was 11.17±4.96days, 50% was 18.17±6.82 days, 75% was 29.50±6.95 days, 100% was 44.00±11.49 days. The above results indicate that Hominis placenta herbal acupuncture is a useful effect on Bell's palsy. thus, continuous herbal acupunture study will be needed for more clinical application on Bell' palsy.

  14. Prognostic factors for recovery in Portuguese patients with Bell's palsy.

    Science.gov (United States)

    Ferreira, Margarida; Firmino-Machado, João; Marques, Elisa A; Santos, Paula C; Simões, Ana Daniela; Duarte, José A

    2016-10-01

    The main aim of this study was to identify the prognostic factors that contribute to complete recovery at 6 weeks and 6 months in patients with Bell's palsy. This is a prospective, longitudinal, and descriptive study that included 123 patients diagnosed with facial nerve palsy (FNP) at a hospital in Guimarães, Portugal. However, only 73 patients with Bell's palsy (BP) were included in the assessment of recovery at 6 weeks and 6 months. We analyzed the demographic and clinical characteristics of the patients, including sex, age, paralyzed side, occupation, previous and associated symptoms, seasonal occurrence, familial facial palsy, patient perception, intervention options, and baseline grade according to the House-Brackmann facial grading system (HB-FGS). Of the 123 cases with FNP, 79 (64.2%) patients had BP. Age, sex, and baseline HB-FGS grades were significant predictors of complete recovery at 6 weeks. Patients with HB-FGS grade III or lower (6 weeks baseline) had significant recovery of function at 6 months. Baseline severity of BP, elderly patients, and male sex were early predictors of poor prognosis. Patients with mild and moderate dysfunction according to the HB-FGS achieved significant normal facial function at 6 months. Further prospective studies with longer observation periods and larger samples are needed to verify the results.

  15. Isolated unilateral sixth cranial nerve palsy: A rare presentation of dengue fever

    Directory of Open Access Journals (Sweden)

    Yang Liang Boo

    2016-11-01

    Full Text Available Dengue fever is a common mosquito-borne viral infection endemic in tropical and subtropical countries. Neurological manifestations in dengue infection are relatively uncommon, and include encephalitis, encephalopathy, neuromuscular disorders and neuro-ocular disorders. Cranial mononeuropathy is a rare manifestation of dengue infection. A 40-year-old man was diagnosed with isolated, unilateral sixth cranial nerve palsy complicating dengue infection. The patient was managed accordingly, and full ocular recovery was observed. This was the first reported case of isolated sixth cranial nerve palsy associated with dengue fever in Malaysia. It is important for clinicians to consider dengue as a differential diagnosis in patients presenting with fever and sixth cranial nerve palsy.

  16. CT findings in patients with cerebral palsy

    International Nuclear Information System (INIS)

    Konno, Kimiichi

    1982-01-01

    Clinical findings and CT findings in 73 cases of cerebral palsy were studied. The causes of cerebral palsy were presumed to be as follows: abnormal cerebral development (36%), asphyxial delivery (34%), and immature delivery (19%), etc. CT findings were abnormal in 58% of the 73 cases, 83% of the spastic tetraplegia patients and all of the spastic hemiplegia patients showed abnormal CT findings. All the patients with spastic monoplegia presented normal CT findings. In 75% of the spastic hemiplegia cases, the CT abnormalities were due to cerebral parenchymal abnormality such as porencephaly and regional low absorption. In cases of spastic tetraplegia, cerebral parenchymal abnormality was found only in 10%. Cortical atrophy was found only in 15 of the 73 cases, whereas central atrophy was found in 36 cases. (Ueda, J.)

  17. Association of cerebral palsy with Apgar score in low and normal birthweight infants: population based cohort study

    Science.gov (United States)

    Grøholt, Else-Karin; Eskild, Anne

    2010-01-01

    Objectives To assess the association of Apgar score 5 minutes after birth with cerebral palsy in both normal weight and low birthweight children, and also the association with the cerebral palsy subdiagnoses of quadriplegia, diplegia, and hemiplegia. Design Population based cohort study. Setting The Medical Birth Registry of Norway was used to identify all babies born between 1986 and 1995. These data were linked to the Norwegian Registry of Cerebral Palsy in Children born 1986-95, which was established on the basis of discharge diagnoses at all paediatric departments in Norway. Population All singletons without malformations born in Norway during 1986-95 and who survived the first year of life (n=543 064). Main outcome measure Cerebral palsy diagnosed before the age of 5 years. Results 988 children (1.8 in 1000) were diagnosed with cerebral palsy before the age of 5 years. In total, 11% (39/369) of the children with Apgar score of less than 3 at birth were diagnosed with cerebral palsy, compared with only 0.1% (162/179 515) of the children with Apgar score of 10 (odds ratio (OR) 53, 95% CI 35 to 80 after adjustment for birth weight). In children with a birth weight of 2500 g or more, those with an Apgar score of less than 4 were much more likely to have cerebral palsy than those who had an Apgar score of more than 8 (OR 125, 95% confidence interval 91 to 170). The corresponding OR in children weighing less than 1500 g was 5 (95% CI 2 to 9). Among children with Apgar score of less than 4, 10-17% in all birthweight groups developed cerebral palsy. Low Apgar score was strongly associated with each of the three subgroups of spastic cerebral palsy, although the association was strongest for quadriplegia (adjusted OR 137 for Apgar score 8, 95% CI 77 to 244). Conclusions Low Apgar score was strongly associated with cerebral palsy. This association was high in children with normal birth weight and modest in children with low birth weight. The strength of the

  18. Health issues in young adults with cerebral palsy: towards a life-span perspective.

    NARCIS (Netherlands)

    Hilberink, S.R.; Roebroeck, M.E.; Nieuwstraten, W.; Jalink, L.; Verheijden, J.M.; Stam, H.J.

    2007-01-01

    OBJECTIVE: To obtain better insight into the health issues of young adults with cerebral palsy. DESIGN: Cross-sectional. SUBJECTS: Two data sources were used: 54 adults with cerebral palsy (age range 25-36 years) and 48 physicians (members of the Netherlands Society of Physical and Rehabilitation

  19. Teeth grinding, oral motor performance and maximal bite force in cerebral palsy children.

    Science.gov (United States)

    Botti Rodrigues Santos, Maria Teresa; Duarte Ferreira, Maria Cristina; de Oliveira Guaré, Renata; Guimarães, Antonio Sergio; Lira Ortega, Adriana

    2015-01-01

    Identify whether the degree of oral motor performance is related to the presence of teeth grinding and maximal bite force values in children with spastic cerebral palsy. Ninety-five spastic cerebral palsy children with and without teeth grinding, according to caregivers' reports, were submitted to a comprehensive oral motor performance evaluation during the feeding process using the Oral Motor Assessment Scale. Maximal bite force was measured using an electronic gnathodynamometer. The teeth grinding group (n = 42) was younger, used anticonvulsant drugs, and was more frequently classified within the subfunctional oral motor performance category. Teeth grinding subfunctional spastic cerebral palsy children presented lower values of maximal bite force. The functional groups showing the presence or absence of teeth grinding presented higher values of maximal bite force compared with the subfunctional groups. In spastic cerebral palsy children, teeth grinding is associated with the worse oral motor performance. © 2015 Special Care Dentistry Association and Wiley Periodicals, Inc.

  20. Cosmetic appreciation of lateralization of peripheral facial palsy: 'preference for left or right, true or mirror image?'.

    Science.gov (United States)

    Pouwels, Sjaak; Ingels, Koen; van Heerbeek, Niels; Beurskens, Carien

    2014-09-01

    There have been several studies in the past depicting asymmetry in 'normal' human faces. Evidence supports the fact that the right hemisphere is superior in the recognition of emotions expressed by the human face and indicates a right hemispheric specialization for processing emotional information. The primary aim of this study is to determine whether there is a difference in cosmetic appreciation of a left peripheral facial palsy compared to a right peripheral facial palsy? Pictures of patients with a facial palsy with House-Brackmann II-VI were reversed as a mirror image and offered as a pair of pictures, together with the true image. Forty-two patients and 24 medical professionals familiar with facial palsy were asked to choose the most attractive photograph. The primary 'end' point was the most attractive side in the pictures chosen by medical professionals and patients. The secondary 'end' points consisted of the preferences for the mirror or true image, and influences of the House-Brackmann score and age. Medical professionals preferred the photographs from patients with a right and left peripheral facial palsy (PFP) in, respectively, a mean of 44 % (41-48 %) and 56 % (52-59 %) of the pictures (p = 0.02). When comparing mirror and true image, patients with a left-sided facial palsy chose their mirror and true image as most attractive in 90 and 10 %, respectively (p 0.05). Subanalysis of patients with a PFP House-Brackmann score V and VI showed that medical professionals did not have a significant preference for a left nor right-sided facial palsy. Patients with a left-sided facial palsy chose their mirror image in all cases and patients with a right-sided palsy chose their mirror and true image in resp. 33 and 67 %. The House-Brackmann score (p = 0.52) and age (p = 0.73) of the patients did not influence preferences. This study, demonstrating that medical professionals find a right-sided facial palsy cosmetically less attractive than a left-sided, has

  1. Middle ear osteoma causing progressive facial nerve weakness: a case report

    OpenAIRE

    Curtis, Kate; Bance, Manohar; Carter, Michael; Hong, Paul

    2014-01-01

    Introduction Facial nerve weakness is most commonly due to Bell’s palsy or cerebrovascular accidents. Rarely, middle ear tumor presents with facial nerve dysfunction. Case presentation We report a very unusual case of middle ear osteoma in a 49-year-old Caucasian woman causing progressive facial nerve deficit. A subtle middle ear lesion was observed on otoscopy and computed tomographic images demonstrated an osseous middle ear tumor. Complete surgical excision resulted in the partial recovery...

  2. Effects of electrical stimulation on House-Brackmann scores in early Bell's palsy.

    Science.gov (United States)

    Alakram, Prisha; Puckree, Threethambal

    2010-04-22

    ABSTRACT Limited evidence may support the application of electrical stimulation in the subacute and chronic stages of facial palsy, yet some physiotherapists in South Africa have been applying this modality in the acute stage in the absence of published evidence of clinical efficacy. This preliminary study's aim was to determine the safety and potential efficacy of applying electrical stimulation to the facial muscles during the early phase of Bells palsy. A pretest posttest control vs. experimental groups design composed of 16 patients with Bell's palsy of less than 30 days' duration. Adult patients with clinical diagnosis of Bell's palsy were systematically (every second patient) allocated to the control and experimental groups. Each group (n = 8) was pretested and posttested using the House-Brackmann index. Both groups were treated with heat, massage, exercises, and a home program. The experimental group also received electrical stimulation. The House-Brackmann Scale of the control group improved between 17% and 50% with a mean of 30%. The scores of the experimental group ranged between 17% and 75% with a mean of 37%. The difference between the groups was not statistically significant (two-tailed p = 0.36). Electrical stimulation as used in this study during the acute phase of Bell's palsy is safe but may not have added value over spontaneous recovery and multimodal physiotherapy. A larger sample size or longer stimulation time or both should be investigated.

  3. Traumatic facial nerve palsy: CT patterns of facial nerve canal fracture and correlation with clinical severity

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Jae Cheol; Kim, Sang Joon; Park, Hyun Min; Lee, Young Suk; Lee, Jee Young [College of Medicine, Dankook Univ., Chonan (Korea, Republic of)

    2002-07-01

    To analyse the patterns of facial nerve canal injury seen at temporal bone computed tomography (CT) in patients with traumatic facial nerve palsy and to correlate these with clinical manifestations and outcome. Thirty cases of temporal bone CT in 29 patients with traumatic facial nerve palsy were analyzed with regard to the patterns of facial nerve canal involvement. The patterns were correlated with clinical grade, the electroneurographic (ENoG) findings, and clinical outcome. For clinical grading, the House-Brackmann scale was used, as follows:grade I-IV, partial palsy group; grade V-VI, complete palsy group. The electroneuronographic findings were categorized as mild to moderate (below 90%) or severe (90% and over) degeneration. In 25 cases, the bony wall of the facial nerve canals was involved directly (direct finding): discontinuity of the bony wall was onted in 22 cases, bony spicules in ten, and bony wall displacement in five. Indirect findings were canal widening in nine cases and adjacent bone fracture in two. In one case, there were no direct or indirect findings. All cases in which there was complete palsy (n=8) showed one or more direct findings including spicules in six, while in the incomplete palsy group (n=22), 17 cases showed direct findings. In the severe degeneration group (n=13), on ENog, 12 cases demonstrated direct findings, including spicules in nine cases. In 24 patients, symptoms of facial palsy showed improvement at follow up evaluation. Four of the five patients in whom symptoms did not improve had spicules. Among ten patients with spicules, five underwent surgery and symptoms improved in four of these; among the five patients not operated on , symptoms did not improve in three. In most patients with facial palsy after temporal bone injury, temporal bone CT revealed direct or indirect facial nerve canal involvement, and in complete palsy or severe degeneration groups, there were direct findings in most cases. We believe that meticulous

  4. Gd-DTPA enhanced MR imaging in Bell's palsy

    Energy Technology Data Exchange (ETDEWEB)

    Matsumoto, Yasushi; Kawamura, Yuji (Takanoko Hospital, Matsuyama, Ehime (Japan)); Yanagihara, Naoaki; Sadamoto, Masanori; Sadamoto, Kazuhiko

    1991-09-01

    Magnetic resonance imaging (MRI) was carried out in 27 patients with Bell's palsy. T1-weighted spin-echo images (TR500/TE25-34) were taken before and after the intravenous administration of Gd-DTPA (0.1 mmol/kg) with 0.5T superconductive MRI. There was significant enhancement at the geniculate ganglion and the horizontal segment of the involved facial nerve in 22 patients (81.5%) and at the vertical segment in 20 (74.1%). Enhancement at the meatal fundus was seen in only 4 patients (14.9%) and at the labyrinthine segment in 8 (29.6%). These results corroborate other evidence that the geniculate ganglion is involved most frequently in Bell's palsy. In addition, the enhancement of the proximal portion, i.e. the meatal fundus and the labyrinthine segment of the nerve, although not common, noted in this study may be important in the diagnosis and treatment of Bell's palsy. (author).

  5. Gadolinium-enhanced MR imaging in evaluation of Bell palsy

    International Nuclear Information System (INIS)

    Wang, A.M.; Wesolowski, D.P.; Bojrab, D.I.; Ernstoff, R.M.; Farah, J.

    1989-01-01

    Eight patients with Bell palsy were evaluated with Gd-DTPA (Magnevist)-enhanced MR imaging in a 1.0-T Siemen's Magnetom unit. Axial pre-and postcontrast and coronal postcontrast T1-weighted MR images of facial nerves were studied. Significant unilateral enhancement of the facial nerve within the internal auditory canal, with or without involvement of the geniculate ganglia, was found in six patients. Three of these patients without satisfactory response to medical treatment underwent surgical decompression, with excellent recovery of facial nerve function. The authors believe that gadolinium-enhanced MR imaging is valuable in the evaluation and management of Bell palsy

  6. Motor palsies of cranial nerves (excluding VII) after vaccination: reports to the US Vaccine Adverse Event Reporting System.

    Science.gov (United States)

    Woo, Emily Jane; Winiecki, Scott K; Ou, Alan C

    2014-01-01

    We reviewed cranial nerve palsies, other than VII, that have been reported to the US Vaccine Adverse Event Reporting System (VAERS). We examined patterns for differences in vaccine types, seriousness, age, and clinical characteristics. We identified 68 reports of cranial nerve palsies, most commonly involving the oculomotor (III), trochlear (IV), and abducens (VI) nerves. Isolated cranial nerve palsies, as well as palsies occurring as part of a broader clinical entity, were reported. Forty reports (59%) were classified as serious, suggesting that a cranial nerve palsy may sometimes be the harbinger of a broader and more ominous clinical entity, such as a stroke or encephalomyelitis. There was no conspicuous clustering of live vs. inactivated vaccines. The patient age range spanned the spectrum from infants to the elderly. Independent data may help to clarify whether, when, and to what extent the rates of cranial nerve palsies following particular vaccines may exceed background levels.

  7. PERIPHERAL FACIAL PALSY IN CHILDHOOD - LYME BORRELIOSIS TO BE SUSPECTED UNLESS PROVEN OTHERWISE

    NARCIS (Netherlands)

    CHRISTEN, HJ; BARTLAU, N; HANEFELD, F; EIFFERT, H; THOMSSEN, R

    1990-01-01

    27 consecutive cases with acute peripheral facial palsy were studied for Lyme borreliosis. In 16 out of 27 children Lyme borreliosis could be diagnosed by detection of specific IgM antibodies in CSF. CSF findings allow a clear distinction according to etiology. All children with facial palsy due to

  8. Maternal thyroid disorder in pregnancy and risk of cerebral palsy in the child: a population-based cohort study.

    Science.gov (United States)

    Petersen, Tanja Gram; Andersen, Anne-Marie Nybo; Uldall, Peter; Paneth, Nigel; Feldt-Rasmussen, Ulla; Tollånes, Mette Christophersen; Strandberg-Larsen, Katrine

    2018-05-31

    Cerebral palsy is the most frequent motor disability in childhood, but little is known about its etiology. It has been suggested that cerebral palsy risk may be increased by prenatal thyroid hormone disturbances. The objective of this study was to investigate whether maternal thyroid disorder is associated with increased risk of cerebral palsy. A population-based cohort study using two study populations. 1) 1,270,079 children born in Denmark 1979-2007 identified in nationwide registers, and 2) 192,918 children born 1996-2009 recruited into the Danish National Birth Cohort and The Norwegian Mother and Child Cohort study, combined in the MOthers and BAbies in Norway and Denmark (MOBAND) collaboration cohort. Register-based and self-reported information on maternal thyroid disorder was studied in relation to risk of cerebral palsy and its unilateral and bilateral spastic subtypes using multiple logistic regression. Children were followed from the age of 1 year to the age of 6 years, and cerebral palsy was identified in nationwide registers with verified diagnoses. In register data, hypothyroidism was recognized in 12,929 (1.0%), hyperthyroidism in 9943 (0.8%), and unclassifiable thyroid disorder in 753 (cerebral palsy was 1.0 (95% CI: 0.7-1.5). Maternal thyroid disorder identified during pregnancy was associated with elevated risk of unilateral spastic cerebral palsy (odds ratio 3.1 (95% CI: 1.2-8.4)). In MOBAND, 3042 (1.6%) of the mothers reported a thyroid disorder in pregnancy, which was not associated with cerebral palsy overall (odds ratio 1.2 (95% CI: 0.6-2.4)). Maternal thyroid disorder overall was not related to bilateral spastic cerebral palsy, but maternal thyroid disorder identified in pregnancy was associated with increased risk of unilateral spastic cerebral palsy. These findings should be replicated in studies making use of maternal blood samples.

  9. Biofeedback interventions for people with cerebral palsy: a systematic review protocol.

    Science.gov (United States)

    MacIntosh, Alexander; Vignais, Nicolas; Biddiss, Elaine

    2017-01-13

    Cerebral palsy is a life-long disability that affects motor control and activities of daily living. Depending on the type of cerebral palsy, some individuals may have trouble performing tasks with one or both of their arms and/or legs. Different strategies exist to help develop motor capacity. Biofeedback therapy is a commonly applied rehabilitation strategy. In biofeedback therapy, information about the motor behavior while completing a task is given back to the individual to help improve their performance. This can provide valuable information that would otherwise be unknown to the individual. Biofeedback may also have a unique method of operation in clinical populations, such as people with cerebral palsy. Therefore, it is important to identify the most effective mechanisms for specific populations. This review aims to evaluate the effects of biofeedback interventions that have been used towards improving motor performance and motor learning in people with cerebral palsy. Using a customized strategy, MEDLINE, CINAHL, Embase, PsycINFO, Cochrane Central Register of Controlled Trials, SCOPUS, SPORTDiscus, and PEDro databases will be searched. Two independent reviewers will screen titles and abstracts, review full texts for inclusion criteria, and extract data from relevant articles using a standardized template. Quality of evidence and risk of bias will be assessed through the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology. Several studies have investigated biofeedback-based interventions for people with cerebral palsy. However, there is a great variety and limited consensus regarding how to implement biofeedback mechanisms. This systematic review will consolidate the current evidence to direct future study and develop effective biofeedback rehabilitation strategies. PROSPERO ID: CRD42016047612.

  10. Physical therapy with drug treatment in Bell palsy: a focused review.

    Science.gov (United States)

    Ferreira, Margarida; Marques, Elisa E; Duarte, José A; Santos, Paula C

    2015-04-01

    The physical therapy (PT) associated with standard drug treatment (SDT) in Bell palsy has never been investigated. Randomized controlled trials or quasirandomized controlled trials have compared facial PT (except treatments such as acupuncture and osteopathic) combined with SDT against a control group with SDT alone. Participants included those older than 15 yrs with a clinical diagnosis of Bell palsy, and the primary outcome measure was motor function recovery by the House-Brackmann scale. The methodologic quality of each study was also independently assessed by two reviewers using the PEDro scale. Four studies met the inclusion criteria. Three trials indicate that PT in association with SDT supports higher motor function recovery than SDT alone between 15 days and 1 yr of follow-up. On the other hand, one trial showed that electrical stimulation added to conventional PT with SDT did not influence treatment outcomes. The present review suggests that the current practice of Bell palsy treatment by PT associated with SDT seems to have a positive effect on grade and time recovery compared with SDT alone. However, there is very little quality evidence from randomized controlled trials, and such evidence is insufficient to decide whether combined treatment is beneficial in the management of Bell palsy.

  11. Sensory Feedback Training for Improvement of Finger Perception in Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Tobias Blumenstein

    2015-01-01

    Full Text Available Purpose. To develop and to test a feedback training system for improvement of tactile perception and coordination of fingers in children and youth with cerebral palsy. Methods. The fingers of 7 probands with cerebral palsy of different types and severity were stimulated using small vibration motors integrated in the fingers of a hand glove. The vibration motors were connected through a microcontroller to a computer and to a response 5-button keyboard. By pressing an appropriate keyboard button, the proband must indicate in which finger the vibration was felt. The number of incorrect responses and the reaction time were measured for every finger. The perception and coordination of fingers were estimated before and after two-week training using both clinical tests and the measurements. Results. Proper functioning of the developed system in persons with cerebral palsy was confirmed. The tactile sensation of fingers was improved in five of seven subjects after two weeks of training. There was no clear tendency towards improvement of selective use of fingers. Conclusion. The designed feedback system could be used to train tactile perception of fingers in children and youth with cerebral palsy. An extensive study is required to confirm these findings.

  12. Parental stress in mothers of children and adolescents with cerebral palsy

    Directory of Open Access Journals (Sweden)

    Maysa Ferreira Martins Ribeiro

    2014-06-01

    Full Text Available OBJECTIVES: to evaluate parental stress of mothers of children and adolescents with cerebral palsy; to verify whether parental stress undergoes variations according to the level of motor compromise, the child's phase of life, and sociodemographic variables.METHOD: a cross-sectional, descriptive study, with 223 mothers of children and adolescents with cerebral palsy.RESULTS: 45.3% of the mothers presented high levels of stress; there were differences in stress between mothers of children with mild and severe motor impairment; mothers of older children were more stressed than mothers of younger children and of adolescents; paid work and leisure activities reduced the stress.CONCLUSION: mothers of children and adolescents with cerebral palsy, whose children present mild to severe motor impairment are vulnerable to parental stress. Paid work and leisure activities were the factors that contributed most to reducing the stress.

  13. When is facial paralysis Bell palsy? Current diagnosis and treatment.

    Science.gov (United States)

    Ahmed, Anwar

    2005-05-01

    Bell palsy is largely a diagnosis of exclusion, but certain features in the history and physical examination help distinguish it from facial paralysis due to other conditions: eg, abrupt onset with complete, unilateral facial weakness at 24 to 72 hours, and, on the affected side, numbness or pain around the ear, a reduction in taste, and hypersensitivity to sounds. Corticosteroids and antivirals given within 10 days of onset have been shown to help. But Bell palsy resolves spontaneously without treatment in most patients within 6 months.

  14. Cranial nerve palsy in Wegener's granulomatosis--lessons from clinical cases

    DEFF Research Database (Denmark)

    Nowack, Rainer; Wachtler, Paul; Kunz, Jürgen

    2009-01-01

    The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener's granulomatosis. One patient developed de novo 6(th) nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging...... by the overall clinical presentations. Cranial neuropathy may be the first obvious vasculitic manifestation preceding other organ disease, and since single reliable tests for its diagnosis are lacking, a multidisciplinary approach is advocated here to detect vasculitic manifestations in other organs....

  15. Genetic Variation in the Dopamine System Influences Intervention Outcome in Children with Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Rochellys Diaz Heijtz

    2018-02-01

    Interpretation: Naturally occurring genetic variation in the dopamine system can influence treatment outcomes in children with cerebral palsy. A polygenic dopamine score might be valid for treatment outcome prediction and for designing individually tailored interventions for children with cerebral palsy.

  16. Position as a Cause of Deformity in Children with Cerebral Palsy (1976)

    Science.gov (United States)

    Scrutton, David

    2008-01-01

    Deformities in the child with cerebral palsy have been ascribed to muscle imbalance (Sharrard 1961) and increased tone (Pollock 1959) or to the type of cerebral palsy (Bobath and Bobath 1975). As far as we know, the position in which the child is nursed, especially during the first year of life, has not been considered as a cause of deformity. It…

  17. Effects of electrical stimulation in early Bells palsy on facial disability index scores

    OpenAIRE

    P. Alakram; T. Puckree

    2011-01-01

    Recovery following facial nerve palsy is variable. Physiotherapists try  to restore  function  in  patients  with  Bell’s  palsy.  The  choice  of treatment modality  depends  on  the  stage  of  the  condition.  Although limited  evidence  exists  for  the  use  of  electrical  stimulation  in  the acute  stage  of  Bell’s  palsy, some physiotherapists in South Africa have been applying this modality. This study examined the effects of electrical stimulation on functional recovery from  Bell...

  18. Epidemiological survey of amyotrophic lateral sclerosis in the province of Reggio Emilia, Italy: influence of environmental exposure to lead.

    Science.gov (United States)

    Guidetti, D; Bondavalli, M; Sabadini, R; Marcello, N; Vinceti, M; Cavalletti, S; Marbini, A; Gemignani, F; Colombo, A; Ferrari, A; Vivoli, G; Solimè, F

    1996-01-01

    We carried out a retrospective incidence, prevalence and mortality survey of amyotrophic lateral sclerosis (ALS) in the province of Reggio Emilia, northern Italy. Based on 79 patients, the mean incidence per year for the period 1980 through 1992 was 1.5 cases per 100,000. On December 31st, 1992, the prevalence rate was 5.4 per 100,000. In the 10-year period of 1983-1992 the average mortality rate was 1.3 per 100,000 per year. The average age at onset was 61.3 +/- 10.2, the average survival period thereafter was 26.3 months +/- 17.7; 27.3 +/- 17.6 for classic ALS, 19.5 +/- 8.4 for progressive bulbar palsy and 36.3 +/- 41.4 for pseudopolyneuritic ALS. The incidence rate, recorded in public health district No.12, an area with documented lead pollution since the 1970s, was standardized to the sex and age of the population of the province. Its incidence and prevalence rate were comparable to the rates found in the remaining area of the province.

  19. X-linked spinal and bulbar muscular atrophy (Kennedy's disease with long-term electrophysiological evaluation: case report Atrofia muscular bulbo-espinal ligada ao cromossomo X (doença de Kennedy com seguimento eletrofisiológico de longo prazo: relato de caso

    Directory of Open Access Journals (Sweden)

    João Aris Kouyoumdjian

    2005-03-01

    Full Text Available X-linked spinal and bulbar muscular atrophy or Kennedy's disease is an adult-onset motor neuronopathy caused by a CAG repeat expansion within the first exon of an androgen receptor gene. We report the case of a 66-year-old man, previously diagnosed with motor neuron disease (MND, who presented acute and reversible left vocal fold (dysphonia and pharyngeal paresis, followed by a slowly progressive weakness and also bouts of weakness, wasting and fasciculation on tongue, masseter, face, pharyngeal, and some proximal more than distal upper limb muscles, associated to bilateral hand tremor and mild gynecomastia. There were 5 electroneuromyography exams between 1989 and 2003 that revealed chronic reinnervation, some fasciculations (less than clinically observed and rare fibrillation potentials, and slowly progressive sensory nerve action potentials (SNAP abnormality, leading to absent/low amplitude potentials. PCR techniques of DNA analysis showed an abnormal number of CAG repeats, found to be 44 (normal 11-34. Our case revealed an acute and asymmetric clinical presentation related to bulbar motoneurons; low amplitude/absent SNAP with mild asymmetry; a sub-clinical or subtle involvement of proximal/distal muscles of both upper and lower limbs; and a probable evolution with bouts of acute dennervation, followed by an efficient reinnervation.Atrofia muscular bulbo-espinal ligada ao cromossomo X (doença de Kennedy é uma neuronopatia motora em adultos causada por expansões na repetição CAG no gene do receptor andrógeno. Neste relato, descreve-se o caso de homem de 66 anos, com diagnóstico prévio de doença do neurônio motor (DNM que apresentou quadro agudo e reversível de paresia de prega vocal (disfonia e de músculos faríngeos à esquerda; posteriormente seguiram-se surtos de fraqueza lentamente progressiva, atrofia e fasciculações em língua, masseter, face, faringe e membros superiores predominantemente proximal, associada a tremor

  20. Bilateral sixth cranial nerve palsy in infectious mononucleosis.

    Science.gov (United States)

    Neuberger, J.; Bone, I.

    1979-01-01

    A 15-year-old girl who presented with a bilateral sixth nerve palsy caused by infectious mononucleosis is described. The neurological presentation of infectious mononucleosis is discussed. PMID:225738