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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. 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…

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. Genetics Home Reference: progressive supranuclear palsy

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    ... 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 ...

  9. 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

  10. 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.

  11. 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.

  12. Regional Cerebral Perfusion in Progressive Supranuclear Palsy

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    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

  13. 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

  14. 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

  15. 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.

  16. 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.)

  17. 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...

  18. 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...

  19. 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.

  20. Apparent diffusion coefficient measurements in progressive supranuclear palsy

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    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.)

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

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    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.

  2. 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.

  3. 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.

  4. 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

  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. 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.

  7. Advancing functional dysconnectivity and atrophy in progressive supranuclear palsy

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    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

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

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    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.

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

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    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.

  10. 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

  11. Characteristics of Nonmotor Symptoms in Progressive Supranuclear Palsy

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    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.

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

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    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

  13. 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

  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. Spinal and bulbar muscular atrophy.

    Science.gov (United States)

    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.

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

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    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.

  17. 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

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

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    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).

  19. 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.

  20. 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)

  1. 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.

  2. Parkinson disease and progressive supranuclear palsy: protein expression in skin.

    Science.gov (United States)

    Rodríguez-Leyva, Ildefonso; Chi-Ahumada, Erika G; Carrizales, Juan; Rodríguez-Violante, Mayela; Velázquez-Osuna, Salvador; Medina-Mier, Verónica; Martel-Gallegos, María G; Zarazúa, Sergio; Enríquez-Macías, Lourdes; Castro, Adriana; Calderón-Garcidueñas, Ana Laura; Jiménez-Capdeville, María E

    2016-03-01

    This study characterizes the expression of tau (p-tau) and α-synuclein (α-syn) by immunohistochemistry in the skin of three different populations: healthy control (HC), Parkinson disease (PD), and progressive supranuclear paralysis (PSP) subjects, with the purpose of finding a biomarker that could differentiate between subjects with PD and PSP. We evaluated the presence of p-tau and α-syn in a pilot study in the skin of three distinct groups of patients: 17 healthy subjects, 17 patients with PD, and 10 patients with PSP. Four millimeters punch biopsies were obtained from the occipital area and analyzed by immunohistochemistry using antibodies against α-syn and phosphorylated species of tau. PHF (paired helical filaments) antibody identifies p-tau in both normal and pathological conditions and AT8 recognizes p-tau characteristic of pathological conditions. Differences between the three groups were assessed by quantification of immunopositive areas in the epidermis. The immunopositivity pattern of p-tau and α-syn was significantly different among the three groups. Healthy subjects showed minimal staining using AT8 and α-syn. The PD group showed significantly higher α-syn and AT8 immunopositivity, while the PSP group only expressed higher AT8 immunopositivity than HCs. These data suggest that the skin reflects brain pathology. Therefore, immunohistochemical analysis of p-tau and α-syn in the skin can be useful for further characterization of PD and PSP.

  3. 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.

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

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    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.

  5. 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.

  6. 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

  7. 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

  8. 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=

  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. 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

  11. 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

  12. 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.

  13. 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)

  14. 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.

  15. 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

  16. 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

  17. 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.

  18. 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.

  19. 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’.

  20. 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.

  1. 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.

  2. 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.

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

    Science.gov (United States)

    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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

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

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    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.)

  9. 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.)

  10. 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.

  11. 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

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

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    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.)

  13. 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.)

  14. 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.

  15. 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.

  16. [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

  17. Frontal dynamic aphasia in progressive supranuclear palsy: Distinguishing between generation and fluent sequencing of novel thoughts.

    Science.gov (United States)

    Robinson, Gail A; Spooner, Donna; Harrison, William J

    2015-10-01

    Frontal dynamic aphasia is characterised by a profound reduction in spontaneous speech despite well-preserved naming, repetition and comprehension. Since Luria (1966, 1970) designated this term, two main forms of dynamic aphasia have been identified: one, a language-specific selection deficit at the level of word/sentence generation, associated with left inferior frontal lesions; and two, a domain-general impairment in generating multiple responses or connected speech, associated with more extensive bilateral frontal and/or frontostriatal damage. Both forms of dynamic aphasia have been interpreted as arising due to disturbances in early prelinguistic conceptual preparation mechanisms that are critical for language production. We investigate language-specific and domain-general accounts of dynamic aphasia and address two issues: one, whether deficits in multiple conceptual preparation mechanisms can co-occur; and two, the contribution of broader cognitive processes such as energization, the ability to initiate and sustain response generation over time, to language generation failure. Thus, we report patient WAL who presented with frontal dynamic aphasia in the context of progressive supranuclear palsy (PSP). WAL was given a series of experimental tests that showed that his dynamic aphasia was not underpinned by a language-specific deficit in selection or in microplanning. By contrast, WAL presented with a domain-general deficit in fluent sequencing of novel thoughts. The latter replicated the pattern documented in a previous PSP patient (Robinson, et al., 2006); however, unique to WAL, generating novel thoughts was impaired but there was no evidence of a sequencing deficit because perseveration was absent. Thus, WAL is the first unequivocal case to show a distinction between novel thought generation and subsequent fluent sequencing. Moreover, WAL's generation deficit encompassed verbal and non-verbal responses, showing a similar (but more profoundly reduced) pattern

  18. 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)

  19. 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

  20. 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.

  1. 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.

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

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    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.

  3. 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)

  4. Gross Motor Function Measure Evolution Ratio: Use as a Control for Natural Progression in Cerebral Palsy.

    Science.gov (United States)

    Marois, Pierre; Marois, Mikael; Pouliot-Laforte, Annie; Vanasse, Michel; Lambert, Jean; Ballaz, Laurent

    2016-05-01

    To develop a new way to interpret Gross Motor Function Measure (GMFM-66) score improvement in studies conducted without control groups in children with cerebral palsy (CP). The curves, which describe the pattern of motor development according to the children's Gross Motor Function Classification System level, were used as historical control to define the GMFM-66 expected natural evolution in children with CP. These curves have been modeled and generalized to fit the curve to particular children characteristics. Research center. Not applicable. Not applicable. Not applicable. Assuming that the GMFM-66 score evolution followed the shape of the Rosenbaum curves, by taking into account the age and GMFM-66 score of children, the expected natural evolution of the GMFM-66 score was predicted for any group of children with CP who were Ratio, was defined as follows: Gross Motor Function Measure Evolution Ratio=measured GMFM-66 score change/expected natural evolution. For practical or ethical reasons, it is almost impossible to use control groups in studies evaluating effectiveness of many therapeutic modalities. The Gross Motor Function Measure Evolution Ratio gives the opportunity to take into account the expected natural evolution of the gross motor function of children with CP, which is essential to accurately interpret the therapy effect on the GMFM-66. Copyright © 2016 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

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

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    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

  6. 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.

  7. 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.)

  8. 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.

  9. 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.)

  10. 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.

  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. 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.

  13. Progressive Supranuclear Palsy-like Syndrome After Aortic Aneurysm Repair: A Case Series

    Directory of Open Access Journals (Sweden)

    Sirisha Nandipati

    2013-12-01

    Full Text Available The syndrome of progressive supranuclear palsy‐like syndrome is a rare complication of ascending aortic aneurysm repair. We report two patients with videos and present a table of prior reported cases. To our knowledge there is no previously published video of this syndrome. The suspected mechanism is brainstem injury though neuroimaging is often negative for an associated infarct. We hope our report will increase recognition of this syndrome after aortic surgery, especially in patients with visual complaints.

  14. 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 ...

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

    Science.gov (United States)

    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

  16. 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

  17. 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 ...

  18. 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 ...

  19. 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

  20. 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.

  1. 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

  2. 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.

  3. 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.

  4. 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.

  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. 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.

  7. [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.

  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. 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.

  10. 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

  11. 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.

  12. Progressive Supranuclear Palsy

    Science.gov (United States)

    ... Disorders (NORD) 55 Kenosia Avenue Danbury, CT 06810 orphan@rarediseases.org https://rarediseases.org/ Tel: 203-744- ... Overview Loan Repayment Programs Administrative Supplements Peer Review Process Review Committees Application Support Library Clinical Research Next ...

  13. 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.

  14. [18F]FDDNP PET in Tauopathies: Correlation to post mortem Pathology in a Case of Progressive Supranuclear Palsy (PSP)

    Science.gov (United States)

    Villegas, Brendon Josef

    This investigation of [18F]FDDNP was conducted in an effort to confirm the presence of disease in a patient with Progressive Supranuclear Palsy (PSP) and to correlate the ante mortem PET scan results to the post mortem pathology. The immunohistochemical and immunofluorescent staining of Paired Helical Filamentous (PHF) tau (AT8) and Amyloid Beta (6F/3D) misfolded proteins demonstrated a widespread deposition in the cortical and subcortical nuclei, the white matter, cerebellar white matter and the medulla oblongata. The in vitro autoradiography demonstrated a neocortical signal comprised of well-delineated amyloid beta in the nucleated layers I/II and hyperphosphorylated tau in the deeper layers III through VI. The autoradiography was well correlated with the immunohistochemical staining in adjacent tissue slides. The binding of the parametric [ 18F]FDDNP distribution volume ratio (DVR) correlated well (Spearman's rho = 0.962, p = .004) with the deposition of tau but not with the presence of amyloid beta (Spearman's rho = -0.829, p = .041). The [ 18F]FDDNP DVR signal appears to be primarily due to the large amount of bound hyperphosphorylated tau (p-tau) and the amyloid beta negligibly contributes to the total signal. Unlabeled FDDNP was shown to bind to tau in the form of globose tangles in the rostral ventromedial medulla as confirmed with both Thioflavin S and PHF-tau Immunofluorescence. The binding of [18F]FDDNP to the human neuroanatomy was investigated in two cohorts of distinct tauopathies and compared to the binding in two tau-negative cohorts against control patients. A cohort of PSP patients (n = 12) with a mean age of 63.8 years and a cohort of Chronic Traumatic Encephalopathy (CTE) patients (n = 14) with a mean age of 58.1 years are both characterized by the presence of various degrees of tau pathology in their brains. The cohort of Parkinson's Disease (PD) patients (n = 16) with a mean age of 63.2 years is initially characterized by clinical symptoms

  15. [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.

  16. 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.)

  17. 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 ...

  18. 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) ...

  19. 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.

  20. [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.

  1. 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

  2. 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.

  3. 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.

  4. 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

  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. 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.

  7. 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 ...

  8. 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 ...

  9. 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.

  10. Femoral anteversion and tibial torsion only explain 25% of variance in regression analysis of foot progression angle in children with diplegic cerebral palsy

    Science.gov (United States)

    2013-01-01

    Background The relationship between torsional bony deformities and rotational gait parameters has not been sufficiently investigated. This study was to investigate the degree of contribution of torsional bony deformities to rotational gait parameters in patients with diplegic cerebral palsy (CP). Methods Thirty three legs from 33 consecutive ambulatory patients (average age 9.5 years, SD 6.9 years; 20 males and 13 females) with diplegic CP who underwent preoperative three dimensional gait analysis, foot radiographs, and computed tomography (CT) were included. Adjusted foot progression angle (FPA) was retrieved from gait analysis by correcting pelvic rotation from conventional FPA, which represented the rotational gait deviation of the lower extremity from the tip of the femoral head to the foot. Correlations between rotational gait parameters (FPA, adjusted FPA, average pelvic rotation, average hip rotation, and average knee rotation) and radiologic measurements (acetabular version, femoral anteversion, knee torsion, tibial torsion, and anteroposteriortalo-first metatarsal angle) were analyzed. Multiple regression analysis was performed to identify significant contributing radiographic measurements to adjusted FPA. Results Adjusted FPA was significantly correlated with FPA (r=0.837, pregression analysis, femoral anteversion (p=0.026) and tibial torsion (p=0.034) were found to be the significant contributing structural deformities to the adjusted FPA (R2=0.247). Conclusions Femoral anteversion and tibial torsion were found to be the significant structural deformities that could affect adjusted FPA in patients with diplegic CP. Femoral anteversion and tibial torsion could explain only 24.7% of adjusted FPA. PMID:23767833

  11. 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.

  12. 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 ...

  13. 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 ...

  14. 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 ...

  15. [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.

  16. 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.)

  17. Identification by [{sup 99m}Tc]ECD SPECT of anterior cingulate hypoperfusion in progressive supranuclear palsy, in comparison with Parkinson's disease

    Energy Technology Data Exchange (ETDEWEB)

    Varrone, Andrea [University Federico II, Biostructure and Bioimaging Institute, National Research Council/Department of Biomorphological and Functional Sciences, Napoli (Italy); Karolinska Hospital, Department of Clinical Neuroscience, Psychiatry Section, Stockholm (Sweden); Pagani, Marco; Salmaso, Dario [National Research Council, Institute of Cognitive Sciences and Technologies, Rome and Padua (Italy); Salvatore, Elena; Amboni, Marianna; De Michele, Giuseppe; Filla, Alessandro; Barone, Paolo [University Federico II, Department of Neurological Sciences, Napoli (Italy); Sansone, Valeria; Pappata, Sabina; Salvatore, Marco [University Federico II, Biostructure and Bioimaging Institute, National Research Council/Department of Biomorphological and Functional Sciences, Napoli (Italy); Nobili, Flavio [University of Genoa, Clinical Neurophysiology, Department of Endocrinological and Metabolic Sciences, Genoa (Italy)

    2007-07-15

    Progressive supranuclear palsy (PSP) is an akinetic-rigid syndrome that can be difficult to differentiate from Parkinson's disease (PD), particularly at an early stage. [{sup 99m}Tc]ethyl cysteinate dimer (ECD) SPECT could represent a widely available tool to assist in the differential diagnosis. In this study we used voxel-based analysis and Computerised Brain Atlas (CBA)-based principal component analysis (PCA) of [{sup 99m}Tc]ECD SPECT data to test whether: (1) specific patterns of rCBF abnormalities can differentiate PSP from controls and PD; (2) networks of dysfunctional brain regions can be found in PSP vs controls and PD. Nine PD patients, 16 PSP patients and ten controls were studied with [{sup 99m}Tc]ECD SPECT using a brain-dedicated device (Ceraspect). Voxel-based analysis was performed with statistical parametric mapping. PCA was applied to volume of interest data after spatial normalisation to CBA. The voxel-based analysis showed hypoperfusion of the anterior cingulate and medial frontal cortex in PSP compared with controls and PD. In PSP patients the rCBF impairment extended to the pre-supplementary motor area and prefrontal cortex, areas involved in executive function and motor networks. Compared with PSP patients, PD patients showed a mild rCBF decrease in associative visual areas which could be related to the known impairment of visuospatial function. The PCA identified three principal components differentiating PSP patients from controls and/or PD patients that included groups of cortical and subcortical brain regions with relatively decreased (cingulate cortex, prefrontal cortex and caudate) or increased (parietal cortex) rCBF, representing distinct functional networks in PSP. Anterior cingulate hypoperfusion seems to be an early, distinct brain abnormality in PSP as compared with PD. (orig.)

  18. Iodine 123-labeled meta-iodobenzylguanidine myocardial scintigraphy in the cases of idiopathic Parkinson`s disease, multiple system atrophy, and progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

    Yoshita, Mitsuhiro; Hayashi, Michiyuki; Hirai, Shunsaku [Tokyo Metropolitan Neurological Hospital (Japan)

    1997-06-01

    To investigate cardiac sympathetic function in Parkinson`s disease (PD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP), {sup 123}I-MIBG myocardial scintigraphy was performed in 25 patients with PD, 25 patients with MSA, 14 patients with PSP, and 20 control subjects. In planar imaging studies, the heart-to-mediastinum average count ratio (H/M) was calculated for both early and delayed images. The mean value of H/M in patients with PD was significantly lower than in those with MSA, PSP, or no disease. Regardless of disease severity or intensity of anti-parkinsonian pharmacotherapy, mean values for H/M were always low in patients with PD. The mean values of H/M in patients with MSA and PSP were significantly lower than in controls. There was no significant difference between the mean value of H/M in MSA with orthostatic hypotension (OH) and that in MSA without OH, and also there was no significant difference between the mean value of H/M in MSA with striatonigral degeneration and that in MSA with olivopontocerebellar atrophy. Although the mean value of H/M in PSP with amitriptyline treatment was significantly lower than that in PSP patients without amitriptyline treatment, there was no significant difference between the mean value of H/M in PSP patients without amitriptyline treatment and that in controls. There was no correlation between H/M and disease duration in those three akinetic-rigid disorders that we have studied here. Thus, PD may have an abnormality of cardiac sympathetic function which has not been detected by previous cardiovascular autonomic studies. Particularly in early stages, {sup 123}I-MIBG myocardial scintigraphy may help to differentiate PD from MSA and PSP. (K.H.)

  19. Significance of apparent diffusion coefficient measurement for the differential diagnosis of multiple system atrophy, progressive supranuclear palsy, and Parkinson's disease: evaluation by 3.0-T MR imaging

    International Nuclear Information System (INIS)

    Tsukamoto, Kazumichi; Kanasaki, Yoshiko; Kakite, Suguru; Fujii, Shinya; Kaminou, Toshio; Ogawa, Toshihide; Matsusue, Eiji

    2012-01-01

    The clinical differentiation of Parkinson's disease (PD) from multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) may be challenging, especially in their early stages. The aim of this study was to evaluate the utility of apparent diffusion coefficient (ADC) measurement to distinguish among these degenerative disorders. Twenty-five MSA, 20 PSP, and 17 PD patients and 18 healthy controls were retrospectively studied. Axial diffusion-weighted and T2-weighted images were obtained using a 3-T MR system. Regions of interest (ROIs) were precisely placed in the midbrain, pons, putamen, globus pallidus, caudate nucleus, thalamus, superior cerebellar peduncle, middle cerebellar peduncle, cerebellar white matter, and cerebellar dentate nucleus, and the regional ADC (rADC) value was calculated in each ROI. In MSA, rADC values in the pons, middle cerebellar peduncle, cerebellar white matter, and cerebellar dentate nucleus were significantly higher than in PSP, PD, and controls. Furthermore, rADC values in the posterior putamen were significantly higher in MSA than in PSP and controls. In PSP, rADC values were significantly higher in the globus pallidus and midbrain than in MSA, PD, and controls. Furthermore, rADC values in the caudate nucleus and superior cerebellar peduncle were significantly higher in PSP than in MSA and controls. In PD, there were no significant differences in the rADC values compared to in MSA, PSP, and controls in all regions. Evaluation of rADC values in characteristic lesions in MSA, PSP, and PD by placing ROIs using 3-T systems can provide useful additional information for differentiating these disorders. (orig.)

  20. Upper midbrain profile sign and cingulate sulcus sign. MRI findings on sagittal images in idiopathic normal-pressure hydrocephalus, Alzheimer's disease, and progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Adachi, Michito; Ohshima, Fumi; Kawanami, Toru; Kato, Takeo

    2006-01-01

    On magnetic resonance imaging (MRI) sagittal sections, we sometimes encounter abnormal aspects of the superior profile of the midbrain and the cingulate sulcus in patients with dementia. In this preliminary study, we refer to these findings as the ''upper midbrain profile sign'' and the cingulate sulcus sign.'' We prospectively evaluated the usefulness of these signs for the diagnosis of idiopathic normal-pressure hydrocephalus (iNPH), Alzheimer's disease (AD) and progressive supranuclear palsy (PSP). We evaluated the upper midbrain profile sign and the cingulate sulcus sign on MRI sagittal images obtained from 21 people with headaches but no neurological deficit (controls), 10 iNPH patients, 11 AD patients, and 5 PSP patients. The upper midbrain profile sign indicated a concave shape to the superior profile of the midbrain on mid-sagittal images, and the cingulate sulcus sign indicated a narrow, tight aspect of the posterior part of the cingulate sulcus on paramedian-sagittal images. These signs were never seen in any images from the controls. The upper midbrain profile sign was seen in 7 of 10 patients with iNPH, 5 of 11 with AD, and 3 of 5 with PSP. The cingulate sulcus sign was seen in all 10 patients with iNPH but was never seen in any patient with AD or PSP. The upper midbrain profile sign could support a diagnosis of PSP but cannot discriminate among iNPH, AD, and PSP. In contrast, the cingulate sulcus sign has a very high sensitivity for iNPH and should facilitate the distinction of iNPH from other dementias. In the clinical setting, it is momentous to evaluate these signs easily by one simple MRI sequence. (author)

  1. 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.

  2. 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 ...

  3. 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 ...

  4. 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 ...

  5. 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.

  6. 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)

  7. Montreal Cognitive Assessment (MoCA) and Mini-Mental State Examination (MMSE) performance in progressive supranuclear palsy and multiple system atrophy.

    Science.gov (United States)

    Fiorenzato, Eleonora; Weis, Luca; Falup-Pecurariu, Cristian; Diaconu, Stefania; Siri, Chiara; Reali, Elisa; Pezzoli, Gianni; Bisiacchi, Patrizia; Antonini, Angelo; Biundo, Roberta

    2016-12-01

    To determine if Montreal Cognitive Assessment (MoCA) is more sensitive than the commonly used Mini-Mental State Examination (MMSE) in detecting cognitive abnormalities in patients with probable progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) compared with Parkinson's disease (PD). In this multicenter observational study, MMSE and MoCA were administered in a random order to 130 patients: 35 MSA, 30 PSP and 65 age, and education and gender matched-PD. We assessed between-group differences for MMSE, MoCA, and their subitems. Receiver-operating characteristic (ROC) curves were calculated. The mean MMSE was higher than the mean MoCA score in each MSA (27.7 ± 2.4 vs. 22.9 ± 3.0, p < 0.0001), PSP (26.0 ± 2.9 vs. 18.2 ± 3.9, p < 0.0001), and PD (27.3 ± 2.0 vs. 22.3 ± 3.5, p < 0.0001). MoCA total score as well as its letter fluency subitem differentiated PSP from MSA and PD with high specificity and moderate sensitivity. More specifically, a cut-off score of 7 F-words or less per minute would support a diagnosis of PSP (PSP vs. PD: 86 % specificity, 70 % sensitivity; PSP vs. MSA: 71 % specificity, 70 % sensitivity). By contrast, MMSE presented an overall ceiling effect for most subitems, except for the pentagon scores, where PSP did less well than MSA or PD patients. These preliminary results suggest that PSP and MSA, similar to PD patients, may present normal MMSE and reduced MoCA performance. Overall, MoCA is more sensitive than MMSE in detecting cognitive impairment in atypical parkinsonism and together with verbal fluency would be a useful test to support PSP diagnosis.

  8. 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 ...

  9. 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.

  10. 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.

  11. 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.

  12. 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.

  13. 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

  14. 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.

  15. 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.

  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. 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

  18. 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

  19. Parametric imaging of the rate constant K[sub i] using 18Fluoro-L-dopa positron emission tomography in progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

    Cordes, M. (Neurodegenerative Disorders Centre, Univ. Hospital, Univ. of British Columbia, Vancouver, BC (Canada) Strahlenklinik und Poliklinik, Universitaetsklinikum Rudolf-Virchow, Berlin (Germany)); Snow, B.J. (Neurodegenerative Disorders Centre, Univ. Hospital, Univ. of British Columbia, Vancouver, BC (Canada)); Morrison, S. (TRIUMF, Univ. of British Columbia, Vancouver, BC (Canada)); Sossi, V. (TRIUMF, Univ. of British Columbia, Vancouver, BC (Canada)); Ruth, T.J. (TRIUMF, Univ. of British Columbia, Vancouver, BC (Canada)); Calne, D.B. (Neurodegenerative Disorders Centre, Univ. Hospital, Univ. of British Columbia, Vancouver, BC (Canada))

    1993-01-01

    Positron emission tomography (PET) studies using 18F-L-dopa were carried out in 9 patients with supranuclear palsy and 13 controls. For quantification of PET data a rate constant K[sub i] was calculated for the radiotracer using a graphical method. Corrections for nonspecific activity were performed in both arterial plasma and brain tissue. The purpose of this study was to test the hypothesis that parametric images of the rate constant K mapping can be obtained on a pixel-by-pixel basis using an appropriate mathematical algorithm. K[sub i] values from these parametric images and the graphical approach were compared. Both correlated closely, with y=0.013+0.947[sup *]x, r=0.992 and y=-0.052+1.048[sup *]x, r=0.965 in patients and controls, respectively. Contrast measurements were also performed and showed a striking increase in contrast on parametric images. K mapping offers several advantages over the graphical approach, since parametric images are time-independent, i.e. one image represents the quantitative result of the study. In addition, parmetric images of the rate constant are normalized to arterial plasma radioactivity and corrected for tissue metabolites. Thus, parametric images of K[sub i] in different individuals can be compared directly without further processing in order to assess the nigrostriatal integrity. (orig.)

  20. 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.

  1. 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.

  2. 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

  3. 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

  4. 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- ...

  5. Magnetic Resonance Parkinsonism Index: diagnostic accuracy of a fully automated algorithm in comparison with the manual measurement in a large Italian multicentre study in patients with progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Nigro, Salvatore; Arabia, Gennarina; Antonini, Angelo; Weis, Luca; Marcante, Andrea; Tessitore, Alessandro; Cirillo, Mario; Tedeschi, Gioacchino; Zanigni, Stefano; Tonon, Caterina; Calandra-Buonaura, Giovanna; Pezzoli, Gianni; Cilia, Roberto; Zappia, Mario; Nicoletti, Alessandra; Cicero, Calogero Edoardo; Tinazzi, Michele; Tocco, Pierluigi; Cardobi, Nicolo; Quattrone, Aldo

    2017-01-01

    To investigate the reliability of a new in-house automatic algorithm for calculating the Magnetic Resonance Parkinsonism Index (MRPI), in a large multicentre study population of patients affected by progressive supranuclear palsy (PSP) or Parkinson's disease (PD), and healthy controls (HC), and to compare the diagnostic accuracy of the automatic and manual MRPI values. The study included 88 PSP patients, 234 PD patients and 117 controls. MRI was performed using both 3T and 1.5T scanners. Automatic and manual MRPI values were evaluated, and accuracy of both methods in distinguishing PSP from PD and controls was calculated. No statistical differences were found between automated and manual MRPI values in all groups. The automatic MRPI values differentiated PSP from PD with an accuracy of 95 % (manual MRPI accuracy 96 %) and 97 % (manual MRPI accuracy 100 %) for 1.5T and 3T scanners, respectively. Our study showed that the new in-house automated method for MRPI calculation was highly accurate in distinguishing PSP from PD. Our automatic approach allows a widespread use of MRPI in clinical practice and in longitudinal research studies. (orig.)

  6. Magnetic Resonance Parkinsonism Index: diagnostic accuracy of a fully automated algorithm in comparison with the manual measurement in a large Italian multicentre study in patients with progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

    Nigro, Salvatore [National Research Council, Institute of Bioimaging and Molecular Physiology, Catanzaro (Italy); Arabia, Gennarina [University ' ' Magna Graecia' ' , Institute of Neurology, Department of Medical and Surgical Sciences, Catanzaro (Italy); Antonini, Angelo; Weis, Luca; Marcante, Andrea [' ' Fondazione Ospedale San Camillo' ' - I.R.C.C.S, Parkinson' s Disease and Movement Disorders Unit, Venice-Lido (Italy); Tessitore, Alessandro; Cirillo, Mario; Tedeschi, Gioacchino [Second University of Naples, Department of Medical, Surgical, Neurological, Metabolic and Aging Sciences, Naples (Italy); Second University of Naples, MRI Research Center SUN-FISM, Naples (Italy); Zanigni, Stefano; Tonon, Caterina [Policlinico S. Orsola - Malpighi, Functional MR Unit, Bologna (Italy); University of Bologna, Department of Biomedical and Neuromotor Sciences, Bologna (Italy); Calandra-Buonaura, Giovanna [University of Bologna, Department of Biomedical and Neuromotor Sciences, Bologna (Italy); IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna (Italy); Pezzoli, Gianni; Cilia, Roberto [ASST G.Pini - CTO, ex ICP, Parkinson Institute, Milano (Italy); Zappia, Mario; Nicoletti, Alessandra; Cicero, Calogero Edoardo [University of Catania, Department ' ' G.F. Ingrassia' ' , Section of Neurosciences, Catania (Italy); Tinazzi, Michele; Tocco, Pierluigi [University Hospital of Verona, Department of Neurological and Movement Sciences, Verona (Italy); Cardobi, Nicolo [University Hospital of Verona, Institute of Radiology, Verona (Italy); Quattrone, Aldo [National Research Council, Institute of Bioimaging and Molecular Physiology, Catanzaro (Italy); University ' ' Magna Graecia' ' , Institute of Neurology, Department of Medical and Surgical Sciences, Catanzaro (Italy)

    2017-06-15

    To investigate the reliability of a new in-house automatic algorithm for calculating the Magnetic Resonance Parkinsonism Index (MRPI), in a large multicentre study population of patients affected by progressive supranuclear palsy (PSP) or Parkinson's disease (PD), and healthy controls (HC), and to compare the diagnostic accuracy of the automatic and manual MRPI values. The study included 88 PSP patients, 234 PD patients and 117 controls. MRI was performed using both 3T and 1.5T scanners. Automatic and manual MRPI values were evaluated, and accuracy of both methods in distinguishing PSP from PD and controls was calculated. No statistical differences were found between automated and manual MRPI values in all groups. The automatic MRPI values differentiated PSP from PD with an accuracy of 95 % (manual MRPI accuracy 96 %) and 97 % (manual MRPI accuracy 100 %) for 1.5T and 3T scanners, respectively. Our study showed that the new in-house automated method for MRPI calculation was highly accurate in distinguishing PSP from PD. Our automatic approach allows a widespread use of MRPI in clinical practice and in longitudinal research studies. (orig.)

  7. 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…

  8. 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

  9. 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

  10. 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

  11. 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…

  12. 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...

  13. 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...

  14. 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.

  15. 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...

  16. 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.

  17. High affinity radiopharmaceuticals based upon lansoprazole for PET imaging of aggregated tau in Alzheimer's disease and progressive supranuclear palsy: synthesis, preclinical evaluation, and lead selection.

    Science.gov (United States)

    Fawaz, Maria V; Brooks, Allen F; Rodnick, Melissa E; Carpenter, Garrett M; Shao, Xia; Desmond, Timothy J; Sherman, Phillip; Quesada, Carole A; Hockley, Brian G; Kilbourn, Michael R; Albin, Roger L; Frey, Kirk A; Scott, Peter J H

    2014-08-20

    Abnormally aggregated tau is the hallmark pathology of tauopathy neurodegenerative disorders and is a target for development of both diagnostic tools and therapeutic strategies across the tauopathy disease spectrum. Development of carbon-11- or fluorine-18-labeled radiotracers with appropriate affinity and specificity for tau would allow noninvasive quantification of tau burden using positron emission tomography (PET) imaging. We have synthesized [(18)F]lansoprazole, [(11)C]N-methyl lansoprazole, and [(18)F]N-methyl lansoprazole and identified them as high affinity radiotracers for tau with low to subnanomolar binding affinities. Herein, we report radiosyntheses and extensive preclinical evaluation with the aim of selecting a lead radiotracer for translation into human PET imaging trials. We demonstrate that [(18)F]N-methyl lansoprazole, on account of the favorable half-life of fluorine-18 and its rapid brain entry in nonhuman primates, favorable kinetics, low white matter binding, and selectivity for binding to tau over amyloid, is the lead compound for progression into clinical trials.

  18. High Affinity Radiopharmaceuticals Based Upon Lansoprazole for PET Imaging of Aggregated Tau in Alzheimer’s Disease and Progressive Supranuclear Palsy: Synthesis, Preclinical Evaluation, and Lead Selection

    Science.gov (United States)

    2014-01-01

    Abnormally aggregated tau is the hallmark pathology of tauopathy neurodegenerative disorders and is a target for development of both diagnostic tools and therapeutic strategies across the tauopathy disease spectrum. Development of carbon-11- or fluorine-18-labeled radiotracers with appropriate affinity and specificity for tau would allow noninvasive quantification of tau burden using positron emission tomography (PET) imaging. We have synthesized [18F]lansoprazole, [11C]N-methyl lansoprazole, and [18F]N-methyl lansoprazole and identified them as high affinity radiotracers for tau with low to subnanomolar binding affinities. Herein, we report radiosyntheses and extensive preclinical evaluation with the aim of selecting a lead radiotracer for translation into human PET imaging trials. We demonstrate that [18F]N-methyl lansoprazole, on account of the favorable half-life of fluorine-18 and its rapid brain entry in nonhuman primates, favorable kinetics, low white matter binding, and selectivity for binding to tau over amyloid, is the lead compound for progression into clinical trials. PMID:24896980

  19. 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.

  20. 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 ...

  1. 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

  2. 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.

  3. 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.

  4. Progressive Supranuclear Palsy (PSP): Frequently Asked Questions

    Science.gov (United States)

    ... PSP in some cases. Sinemet. This is the brand name for a combination of “levodopa” and “carbidopa.” ... A major goal of CurePSP is to increase awareness of PSP, CBD, MSA and related brain diseases ...

  5. Progressive Supranuclear Palsy Misdiagnosed as Parkinson's ...

    African Journals Online (AJOL)

    cerebellar peduncle were 0.30 cm2 and 0.28 cm on the right and left sides, respectively [Figure 2], and the pons to mid‑brain ratio was 5.71 [Figure 3]. ..... Longoni G, Agosta F, Kostić VS, Stojković T, Pagani E,. Stošić‑Opinćal T, et al.

  6. 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.

  7. 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

  8. 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.

  9. 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. ...

  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. 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.

  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. 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.

  14. 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.

  15. 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

  16. 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

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. 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.

  2. 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.)

  3. 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.)

  4. 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)

  5. 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)

  6. [Bell and his palsy].

    Science.gov (United States)

    van Gijn, Jan; Gijselhart, Joost P

    2011-01-01

    Unlike his eponymous fame suggests, Sir Charles Bell (1774-1842) was an anatomist, draughtsman and surgeon rather than purely a physiologist. He was born and educated in Edinburgh but spent most of his working life in London (1804 to 1836). It was there he started a School of Anatomy, alongside a fledgling surgical practice, just as his elder brother John had done in Edinburgh. In 1814 he joined the surgical staff at the Middlesex Hospital. In 1810 he surmised from occasional animal experiments that the anterior and posterior spinal roots differed in function. Yet it was left to the Frenchman Magendie to identify that these functions were motor and sensory: a discovery that induced Bell into an ungentlemanly feud. Bell also slightly erred on the functions of the trigeminal and facial nerve, but his description of the features of idiopathic facial palsy is unrivalled.

  7. 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 ...

  8. 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 ...

  9. 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 ...

  10. 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.

  11. 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…

  12. Nelson′s syndrome presenting as bilateral oculomotor palsy

    Directory of Open Access Journals (Sweden)

    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.

  13. 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.

  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. 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.

  16. 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

  17. 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.

  18. 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.

  19. 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

  20. 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.

  1. 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.

  2. 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...

  3. 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)

  4. 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.

  5. 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.

  6. 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.

  7. 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

  8. 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.

  9. Peripheral Androgen Receptor Gene Suppression Rescues Disease in Mouse Models of Spinal and Bulbar Muscular Atrophy

    Directory of Open Access Journals (Sweden)

    Andrew P. Lieberman

    2014-05-01

    Full Text Available Spinal and bulbar muscular atrophy (SBMA is caused by the polyglutamine androgen receptor (polyQ-AR, a protein expressed by both lower motor neurons and skeletal muscle. Although viewed as a motor neuronopathy, data from patients and mouse models suggest that muscle contributes to disease pathogenesis. Here, we tested this hypothesis using AR113Q knockin and human bacterial artificial chromosome/clone (BAC transgenic mice that express the full-length polyQ-AR and display androgen-dependent weakness, muscle atrophy, and early death. We developed antisense oligonucleotides that suppressed AR gene expression in the periphery but not the CNS after subcutaneous administration. Suppression of polyQ-AR in the periphery rescued deficits in muscle weight, fiber size, and grip strength, reversed changes in muscle gene expression, and extended the lifespan of mutant males. We conclude that polyQ-AR expression in the periphery is an important contributor to pathology in SBMA mice and that peripheral administration of therapeutics should be explored for SBMA patients.

  10. Single stage circumferential lingual mucosal graft urethroplasty in near obliterative bulbar urethra stricture: A novel technique.

    Science.gov (United States)

    Sharma, Umesh; Yadav, Sher Singh; Tomar, Vinay; Garg, Amit

    2016-01-01

    This is a prospective study of the use and efficacy of a novel technique of circumferential tubularised lingual mucosal graft (LMG) in obliterative and near obliterative bulbar urethral stricture of >2 cm where excisional and augmented anastomotic urethroplasty are not feasible. The stenotic urethral segment was opened dorsally in midline and fibrosed urethra was excised taking care to preserve the healthy spongiosum tissue. LMG (av. Length 3 cm) was placed from one end of corporal body towards spongy tissue in a circumferential manner. Another LMG was placed in similar manner to deal with longer stricture. The urethra was tubularised over 14 Fr silicone catheter. A total of 12 men, of mean age 47 years underwent this procedure. The mean follow up period was 11 months starting from July 2014 till manuscript submission. Follow up included voiding cystourethrogram at 3 weeks, cystoscopy at 3 months (one patient didn't turned up) and subsequent follow up. Mean stricture length was 4.66 cm (range, 3-8.5 cm) and mean operative time was 195 min. (range, 160 to 200 min.). The technique was successful (normal voiding with no need for any post-operative procedure) in 11(91.6%) patients. One patient developed early recurrence at 4 month of surgery and had anastomotic stricture which was successfully managed by direct visual internal urethrotomy. Single stage circumferential tubularised graft urethroplasty is an excellent technique for strictures that include segments of obliterative and near obliterative diseased urethra. It provide a wider neourethra than patch graft urethroplasty.

  11. 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.

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. [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).

  17. 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)

  18. Treatment of Bulbar Urethral Strictures. A Review, with Personal Critical Remarks

    Directory of Open Access Journals (Sweden)

    Willem Oosterlinck

    2003-01-01

    Full Text Available This is a review article on treatment of bulbar urethral strictures with personal critical remarks on newer developments. As a treatment of first intention there exists 4 options : dilatation, urethrotomy, end to end anastomosis and free graft, open urethroplasty. Success rate of dilatation and visual urethrotomy after 4 years is only 20 en 40 % respectively. Laser urethrotomy could not fulfill expectations. End to end anastomosis obtains a very high success rate but is only applicable for short strictures. Free graft urethroplasty obtains success rates of ± 80 %. There is considerable debate on the best material for grafting. Buccal mucosa graft is the new wave, but this is not based on scientific data. Whether this graft should be used dorsally or ventrally is also a point of discussion. In view of the good results published with both techniques it is probably of no importance. Intraluminal stents are not indicated for complicated cases and give only good results in those cases which can easily be treated with other techniques. Metal self-retaining urethral stent , resorbable stents and endoscopic urethroplasty is briefly discussed. Redo’s and complicated urethral strictures need often other solutions. Here skin flap from the penile skin and scrotal flap can be used. Advantages and drawbracks of both are discussed. There is still a place for two-stage procedures in complicated redo�s. The two-stage mesh-graft urethroplasty offers advantage over the use of scrotal skin. Some other rare techniques like substitution with bowel and pudendal thigh flap, to cover deep defects, are also discussed.

  19. Single stage circumferential lingual mucosal graft urethroplasty in near obliterative bulbar urethra stricture: A novel technique

    Directory of Open Access Journals (Sweden)

    Umesh Sharma

    2016-01-01

    Full Text Available Aims: This is a prospective study of the use and efficacy of a novel technique of circumferential tubularised lingual mucosal graft (LMG in obliterative and near obliterative bulbar urethral stricture of >2 cm where excisional and augmented anastomotic urethroplasty are not feasible. Materials and Methods: The stenotic urethral segment was opened dorsally in midline and fibrosed urethra was excised taking care to preserve the healthy spongiosum tissue. LMG (av. Length 3 cm was placed from one end of corporal body towards spongy tissue in a circumferential manner. Another LMG was placed in similar manner to deal with longer stricture. The urethra was tubularised over 14 Fr silicone catheter. Results: A total of 12 men, of mean age 47 years underwent this procedure. The mean follow up period was 11 months starting from July 2014 till manuscript submission. Follow up included voiding cystourethrogram at 3 weeks, cystoscopy at 3 months (one patient didn't turned up and subsequent follow up. Mean stricture length was 4.66 cm (range, 3–8.5 cm and mean operative time was 195 min. (range, 160 to 200 min.. The technique was successful (normal voiding with no need for any post-operative procedure in 11(91.6% patients. One patient developed early recurrence at 4 month of surgery and had anastomotic stricture which was successfully managed by direct visual internal urethrotomy. Conclusion: Single stage circumferential tubularised graft urethroplasty is an excellent technique for strictures that include segments of obliterative and near obliterative diseased urethra. It provide a wider neourethra than patch graft urethroplasty.

  20. 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.

  1. 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.

  2. 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...

  3. 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.

  4. 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.

  5. 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...

  6. 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.

  7. 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.

  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. A systematic assessment of goblet cell sampling of the bulbar conjunctiva by impression cytology.

    Science.gov (United States)

    Doughty, Michael J

    2015-07-01

    The purpose of this study was to assess the apparent goblet cell density (GCD) from conjunctival impression cytology (CIC) samples in relation to the number of conjunctival cells collected onto the filters. CIC specimens were collected from the superior-temporal bulbar conjunctiva of 16 pigmented rabbits onto Biopore (Millicell-CM) membranes, fixed with buffered glutaraldehyde and stained with Giemsa. Different numbers of microscope fields of view in each of the specimens were imaged by light microscopy using a 20× magnification objective lens (200× final magnification), and the goblet cells marked and counted. The GCD values/sq. mm were calculated. The same conjunctival region of 3 other rabbits was also prepared for transmission electron microscopy (TEM) by fixation, in situ, with the same buffered glutaraldehyde. Mean values for GCD estimates were found to vary from 399 to 1576 cells/sq. mm, depending on the image sampling and analysis strategy chosen, with the lowest inter-sample variance of around 10% being found if a maximum goblet cell count was taken on substantially multilayered regions of the CIC specimens. Counts of the number of goblet cells per 1000 visible conjunctival epithelial cells yielded a value of close to 90 (range 36-151), with modest inter-sample variability of around 30%. A three or ten 200× microscope field and random sampling strategy yielded mean GCD values between 542 and 670 cells/sq. mm, but with very high intra- and inter-sample variance of at least 60% and sometimes higher than 100%. TEM confirmed the multilayered organization of the conjunctiva and the deeper lying goblet cells. The general use of a goblet cell count as an objective marker for conjunctival normality or health is likely to be highly variable unless a more specific strategy is adopted. Beyond providing details of exactly the counting strategy used, it would be very useful to provide full details of the actual microscope field size used as well as information on

  10. 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.

  11. 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…

  12. 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 ...

  13. 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 ...

  14. 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

  15. 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.

  16. 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

  17. 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.

  18. 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

  19. 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.

  20. 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)

  1. 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...

  2. MR findings of cerebral palsy

    International Nuclear Information System (INIS)

    Yoon, Sang Hum; Chang, Seung Kuk; Cho, Mee Young; Park, Dong Woo; Kim, Jong Deok; Eun, Choong Ki

    1994-01-01

    To evaluate the MR findings of brain damage in cerebral palised patients and to correlate it with gestational age and the time of damage. A retrospective analysis was performed in 40 patients who underwent MR scanning for evaluation of brain lesion in clinically diagnosed cerebral palsy. Authors classified the patients into two groups as premature and full-term and compared MR findings of the two groups. Abnormal MR findings were noted in 28 cases (70%). Five out of 6 patients who had been born prematurely showed isolate periventricular white matter lesions. Twenty-three out of 34 patients who had been born at full-term showed abnormal MR findings. Of these 23 patients, migration anomalies in 7 patients, isolate periventricular white matter lesions in 3 patients, and other combined periventricular subcortical white matter and deep gray matter lesions in 14 patients were seen. At least, 10 patients(43%) of full term group showed abnormal MRI findings reflecting intrauterine brain damage and all 5 patients of premature group showed isolate periventricular white matter lesions suggesting immaturity of brain. MRI is thought to be very useful in the assessment of brain damage for the patients with cerebral palsy by recognizing the location of the lesion and estimating the time of damage

  3. 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...

  4. MR findings of cerebral palsy

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Sang Hum; Chang, Seung Kuk; Cho, Mee Young; Park, Dong Woo; Kim, Jong Deok; Eun, Choong Ki [Pusan Paik Hospital, Pusan (Korea, Republic of)

    1994-11-15

    To evaluate the MR findings of brain damage in cerebral palised patients and to correlate it with gestational age and the time of damage. A retrospective analysis was performed in 40 patients who underwent MR scanning for evaluation of brain lesion in clinically diagnosed cerebral palsy. Authors classified the patients into two groups as premature and full-term and compared MR findings of the two groups. Abnormal MR findings were noted in 28 cases (70%). Five out of 6 patients who had been born prematurely showed isolate periventricular white matter lesions. Twenty-three out of 34 patients who had been born at full-term showed abnormal MR findings. Of these 23 patients, migration anomalies in 7 patients, isolate periventricular white matter lesions in 3 patients, and other combined periventricular subcortical white matter and deep gray matter lesions in 14 patients were seen. At least, 10 patients(43%) of full term group showed abnormal MRI findings reflecting intrauterine brain damage and all 5 patients of premature group showed isolate periventricular white matter lesions suggesting immaturity of brain. MRI is thought to be very useful in the assessment of brain damage for the patients with cerebral palsy by recognizing the location of the lesion and estimating the time of damage.

  5. Herpes Simplex Virus-1 and Bell's Palsy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-05-01

    Full Text Available The association between herpes simplex virus-1 (HSV-1 infection and Bell palsy was determined in 47 children studied at Children's Hospital at Montefiore, Bronx, NY. Swabs of saliva and conjunctiva were taken for PCR testing.

  6. 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.

  7. 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.

  8. [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.

  9. VII NERVE PALSY — EVALUATION AND MANAGEMENT

    African Journals Online (AJOL)

    Enrique

    stapedial nerve — stapedius muscle in middle ear. As it exits ... facial palsy at birth. ... ticularly in the early stages of HIV and ... associated symptoms (hearing loss, ... neuron (UMN) or lower motor neuron .... gold weight implant or upper eyelid.

  10. 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...

  11. 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...

  12. [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.

  13. 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.

  14. 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.

  15. 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

  16. Embodying Investigations of Cerebral Palsy

    DEFF Research Database (Denmark)

    Martiny, Kristian Møller Moltke

    to understand what it means for persons to live with CP and then figure out how we should help them . Based on his method of open - minded cognitive science, Martiny presents data on neuro - physiological, psychological and social aspects of living with CP. From this theoretical work, Martiny develops......The main question of Kristian Martiny’s dissertation is: how do we help persons living with the brain damage, cerebral palsy (CP)? This question is as complex and difficult to answer as any healthcare question. Martiny argues that we need to ‘open up’ how we do ( cognitive ) science in order...... an embodied - based model of intervention for CP, focusing on the experience of self control as a way to help people with CP. In addition, a theatre performance, Humane Liquidation , and a documentary film, Natural Disorder, are developed so as to both communicate what it means to live with CP and empower...

  17. 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

  18. Cerebral palsy in preterm infants

    Directory of Open Access Journals (Sweden)

    Demeši-Drljan Čila

    2016-01-01

    Full Text Available Background/Aim. Cerebral palsy (CP is one of the leading causes of neurological impairment in childhood. Preterm birth is a significant risk factor in the occurrence of CP. Clinical outcomes may include impairment of gross motor function and intellectual abilities, visual impairment and epilepsy. The aim of this study was to examine the relationships among gestational age, type of CP, functional ability and associated conditions. Methods. The sample size was 206 children with CP. The data were obtained from medical records and included gestational age at birth, clinical characteristics of CP and associated conditions. Clinical CP type was determined according to Surveillance of Cerebral Palsy in Europe (SCPE and topographically. Gross motor function abilities were evaluated according to the Gross Motor Function Classification System (GMFCS. Results. More than half of the children with CP were born prematurely (54.4%. Statistically significant difference was noted with respect to the distribution of various clinical types of CP in relation to gestational age (p < 0.001. In the group with spastic bilateral CP type, there is a greater proportion of children born preterm. Statistically significant difference was noted in the functional classification based on GMFCS in terms of gestational age (p = 0.049, children born at earlier gestational age are classified at a higher GMFCS level of functional limitation. The greatest percentage of children (70.0% affected by two or more associated conditions was found in the group that had extremely preterm birth, and that number declined with increasing maturity at birth. Epilepsy was more prevalent in children born at greater gestational age, and this difference in distribution was statistically significant (p = 0.032. Conclusion. The application of antenatal and postnatal protection of preterm children should be a significant component of the CP prevention strategy. [Projekat Ministarstva nauke Republike

  19. 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.

  20. 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)

  1. 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

  2. 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....

  3. 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 ...

  4. 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? ...

  5. 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.

  6. 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.

  7. Towards a European Registry and Biorepository for Patients with Spinal and Bulbar Muscular Atrophy

    DEFF Research Database (Denmark)

    Pareyson, Davide; Fratta, Pietro; Pradat, Pierre-François

    2016-01-01

    as possible can be included, with the following aims: facilitate planning of clinical trials and recruitment of patients, define natural history of the disease, characterize epidemiology, develop standards of care, and inform the community of patients about research progresses and ongoing trials. We also aim...

  8. Is There a Role for Exercise in the Management of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis?

    Science.gov (United States)

    Plowman, Emily K.

    2015-01-01

    Purpose: The role of exercise in the management of people with amyotrophic lateral sclerosis (PALS) is controversial and currently unclear. The purpose of this review article is to review literature examining the impact of limb, respiratory, and oral motor exercise on function, disease progression, and survival in PALS and the transgenic ALS…

  9. Speech Deterioration in Amyotrophic Lateral Sclerosis (ALS) after Manifestation of Bulbar Symptoms

    Science.gov (United States)

    Makkonen, Tanja; Ruottinen, Hanna; Puhto, Riitta; Helminen, Mika; Palmio, Johanna

    2018-01-01

    Background: The symptoms and their progression in amyotrophic lateral sclerosis (ALS) are typically studied after the diagnosis has been confirmed. However, many people with ALS already have severe dysarthria and loss of adequate speech at the time of diagnosis. Speech-and-language therapy interventions should be targeted timely based on…

  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. 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.

  12. 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: ...

  13. 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 ...

  14. 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 ...

  15. 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…

  16. 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.

  17. 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

  18. 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.

  19. 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.

  20. 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

  1. Genetics Home Reference: horizontal gaze palsy with progressive scoliosis

    Science.gov (United States)

    ... eyes to track moving objects. Up-and-down (vertical) eye movements are typically normal. In people with ... Shattuck DW, Hageman N, Rüb U, Salamon N, Drain AE, Demer JL, Engle EC, Alger JR, Baloh ...

  2. Dermatoglyphs and brachial plexus palsy.

    Science.gov (United States)

    Polovina, Svetislav; Cvjeticanin, Miljenko; Milicić, Jasna; Proloscić, Tajana Polovina

    2006-09-01

    Perinatal brachial plexus palsy (PBPP) is a handicap quite commonly encountered in daily routine. Although birth trauma is considered to be the major cause of the defect, it has been observed that PBPP occurs only in some infants born under identical or nearly identical conditions. The aim of this study was to test the hypothesis of genetic predisposition for PBPP. It is well known that digito-palmar dermatoglyphs can be used to determine hereditary roots of some diseases. Thus, we found it meaningful to do a study analysis of digito-palmar dermatoglyphs in this disease as well, conducting it on 140 subjects (70 males and 70 females) diagnosed with PBPP. The control group was composed of fingerprints obtained from 400 adult and phenotypically healthy subjects (200 males and 200 females) from the Zagreb area. The results of multivariate and univariate analysis of variance have shown statistically significant differences between the groups observed. In spite of lower percentage of accurately classified female subjects by discriminant analysis, the results of quantitative analysis of digito-palmar dermatoglyphs appeared to suggest a genetic predisposition for the occurrence of PBPP.

  3. 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

  4. Is there a way to predict failure after direct vision internal urethrotomy for single and short bulbar urethral strictures?

    Science.gov (United States)

    Harraz, Ahmed M; El-Assmy, Ahmed; Mahmoud, Osama; Elbakry, Amr A; Tharwat, Mohamed; Omar, Helmy; Farg, Hashim; Laymon, Mahmoud; Mosbah, Ahmed

    2015-12-01

    To identify patient and stricture characteristics predicting failure after direct vision internal urethrotomy (DVIU) for single and short (urethroplasty. Predictors of failure were analysed. In all, 430 adult patients with a mean (SD) age of 50 (15) years were included. The main causes of stricture were idiopathic followed by iatrogenic in 51.6% and 26.3% of patients, respectively. Most patients presented with obstructive lower urinary tract symptoms (68.9%) and strictures were proximal bulbar, i.e. just close to the external urethral sphincter, in 35.3%. The median (range) follow-up duration was 29 (3-132) months. In all, 250 (58.1%) patients did not require any further instrumentation, while RSD was maintained in 116 (27%) patients, including 28 (6.5%) who required a redo DVIU or urethroplasty. In 64 (6.5%) patients, a redo DVIU or urethroplasty was performed. On multivariate analysis, older age at presentation [odds ratio (OR) 1.017; P = 0.03], obesity (OR 1.664; P = 0.015), and idiopathic strictures (OR 3.107; P = 0.035) were independent predictors of failure after DVIU. The failure rate after DVIU accounted for 41.8% of our present cohort with older age at presentation, obesity, and idiopathic strictures independent predictors of failure after DVIU. This information is important in counselling patients before surgery.

  5. A new cholesterol biosynthesis and absorption disorder associated with epilepsy, hypogonadism, and cerebro-cerebello-bulbar degeneration.

    Science.gov (United States)

    Korematsu, Seigo; Uchiyama, Shin-ichi; Honda, Akira; Izumi, Tatsuro

    2014-06-01

    Cholesterol is one of the main components of human cell membranes and constitutes an essential substance in the central nervous system, endocrine system, and its hormones, including sex hormones. A 19-year-old male patient presented with failure to thrive, psychomotor deterioration, intractable epilepsy, hypogonadism, and cerebro-cerebello-bulbar degeneration. His serum level of cholesterol was low, ranging from 78.7 to 116.5 mg/dL. The serum concentrations of intermediates in the cholesterol biosynthesis pathway, such as 7-dehydrocholesterol, 8-dehydrocholesterol, desmosterol, lathosterol, and dihydrolanosterol, were not increased. In addition, the levels of the urinary cholesterol biosynthesis marker mevalonic acid, the serum cholesterol absorption markers, campesterol and sitosterol, and the serum cholesterol catabolism marker, 7α-hydroxycholesterol, were all low. A serum biomarker analysis indicated that the patient's basic abnormality differed from that of Smith-Lemli-Opitz syndrome and other known disorders of cholesterol metabolism. Therefore, this individual may have a new metabolic disorder with hypocholesterolemia because of decreased biosynthesis and absorption of cholesterol. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. [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.

  7. 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.

  8. 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.

  9. 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...

  10. 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...

  11. 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....

  12. 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.

  13. 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.

  14. 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.

  15. Cerebral Palsy. NICHCY Disability Fact Sheet #2

    Science.gov (United States)

    National Dissemination Center for Children with Disabilities, 2010

    2010-01-01

    Cerebral palsy--also known as CP--is a condition caused by injury to the parts of the brain that control the body's ability to use muscles effectively. Often the injury happens before birth, sometimes during delivery or soon after birth. The symptoms will differ from person to person and change as children and their nervous systems mature. This…

  16. Early identification and intervention in cerebral palsy

    DEFF Research Database (Denmark)

    Herskind, Anna; Greisen, Gorm; Nielsen, Jens Bo

    2015-01-01

    Infants with possible cerebral palsy (CP) are commonly assumed to benefit from early diagnosis and early intervention, but substantial evidence for this is lacking. There is no consensus in the literature on a definition of 'early', but this review focuses on interventions initiated within...

  17. Pretend Play of Children with Cerebral Palsy

    Science.gov (United States)

    Pfeifer, Luzia Iara; Pacciulio, Amanda Mota; dos Santos, Camila Abrao; dos Santos, Jair Licio; Stagnitti, Karen Ellen

    2011-01-01

    Background and Purpose: Evaluate self-initiated pretend play of children with cerebral palsy. Method: Twenty preschool children participated in the study. Pretend play ability was measured by using the child-initiated pretend play assessment culturally adapted to Brazil. Results: There were significant negative correlations between the children's…

  18. Gait Stability in Children with Cerebral Palsy

    Science.gov (United States)

    Bruijn, Sjoerd M.; Millard, Matthew; van Gestel, Leen; Meyns, Pieter; Jonkers, Ilse; Desloovere, Kaat

    2013-01-01

    Children with unilateral Cerebral Palsy (CP) have several gait impairments, amongst which impaired gait stability may be one. We tested whether a newly developed stability measure (the foot placement estimator, FPE) which does not require long data series, can be used to asses gait stability in typically developing (TD) children as well as…

  19. Social integration of adults with cerebral palsy

    DEFF Research Database (Denmark)

    Michelsen, Susan Ishøy; Uldall, Peter; Hansen, Thomas

    2006-01-01

    Social integration and independence is the ultimate goal of habilitation and social support for patients with cerebral palsy (CP). Having a partner and having children provide support for social integration of adults with or without a disability. We studied 416 participants with CP born between...

  20. Parental infertility and cerebral palsy in children

    DEFF Research Database (Denmark)

    Zhu, Jin Liang; Hvidtjørn, Dorte; Basso, Olga

    2010-01-01

    Children born after in vitro fertilization (IVF) or intracytoplasmic sperm injection (ICSI) have been reported to have a higher risk of cerebral palsy (CP), perhaps due to the higher frequency of preterm birth, multiple births or vanishing embryo in the pregnancies. However, it has been suggested...

  1. Educational Solutions for Children with Cerebral Palsy

    Science.gov (United States)

    Driver, Lynn; Omichinski, Donna Riccio; Miller, Nicole; Sandella, Danielle; Warschausky, Seth

    2010-01-01

    This paper characterizes educational strengths and needs of children with cerebral palsy (CP) and connects research findings from the University of Michigan's Adapted Cognitive Assessment Lab (ACAL) to current special educational requirements. It acknowledges the uniqueness of educating a child with significant motor and communication disabilities…

  2. 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.

  3. 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.

  4. 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.

  5. [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.

  6. 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.

  7. 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.

  8. 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.

  9. Bulbar impairment score predicts noninvasive volume-cycled ventilation failure during an acute lower respiratory tract infection in ALS.

    Science.gov (United States)

    Servera, Emilio; Sancho, Jesús; Bañuls, Pilar; Marín, Julio

    2015-11-15

    Amyotrophic lateral sclerosis (ALS) patients can suffer episodes of lower respiratory tract infections (LRTI) leading to an acute respiratory failure (ARF) requiring noninvasive ventilation (NIV). To determine whether clinical or functional parameters can predict noninvasive management failure during LRTI causing ARF in ALS. A prospective study involving all ALS patients with ARF requiring NIV in a Respiratory Care Unit. NIV was provided with volume-cycled ventilators. 63 ALS patients were included (APACHE II: 14.93±3.56, Norris bulbar subscore (NBS): 18.78±9.68, ALSFRS-R: 19.90±6.98, %FVC: 40.01±18.07%, MIC: 1.62±0.74L, PCF 2.51±1.15L/s, PImax -34.90±19.44cmH2O, PEmax 51.20±28.84cmH2O). In 73.0% of patients NIV was successful in averting death or endotracheal intubation. Differences were found between the success and failure in the NBS (22.08±6.15 vs 8.66±3.39, pNIV failure was the NBS (OR 0.53, 95% CI 0.31-0.92, p 0.002) with a cut-off point of 12 (S 0.93; E 0.97; PPV 0.76; NPV 0.97). NBS can predict noninvasive management failure during LRTI in ALS. Copyright © 2015 Elsevier B.V. All rights reserved.

  10. Prominent fatigue in spinal muscular atrophy and spinal and bulbar muscular atrophy: evidence of activity-dependent conduction block.

    Science.gov (United States)

    Noto, Yu-ichi; Misawa, Sonoko; Mori, Masahiro; Kawaguchi, Naoki; Kanai, Kazuaki; Shibuya, Kazumoto; Isose, Sagiri; Nasu, Saiko; Sekiguchi, Yukari; Beppu, Minako; Ohmori, Shigeki; Nakagawa, Masanori; Kuwabara, Satoshi

    2013-09-01

    To clarify whether patients with spinal muscular atrophy (SMA) or spinal and bulbar muscular atrophy (SBMA) suffer disabling muscle fatigue, and whether activity-dependent conduction block (ADCB) contributes to their fatigue. ADCB is usually caused by reduced safety factor for impulse transmission in demyelinating diseases, whereas markedly increased axonal branching associated with collateral sprouting may reduce the safety factor in chronic lower motor neuron disorders. We assessed the fatigue severity scale (FSS) in 22 patients with SMA/SBMA, and in 100 disease controls (multiple sclerosis, myasthenia gravis, chronic inflammatory demyelinating polyneuropathy (CIDP), and axonal neuropathy). We then performed stimulated-single fibre electromyography (s-SFEMG) in the extensor digitorum communis (EDC) muscle of 21 SMA/SBMA patients, 6 CIDP patients, and 10 normal subjects. The FSS score was the highest in SMA/SBMA patients [4.9 ± 1.1 (mean ± SD)], with 81% of them complaining of disabling fatigue, compared with normal controls (3.5 ± 1.0), whereas patients with multiple sclerosis (4.3 ± 1.6), myasthenia gravis (4.0 ± 1.6) or CIDP (4.3 ± 1.4) also showed higher FSS score. When 2000 stimuli were delivered at 20 Hz in s-SFEMG, conduction block of single motor axons developed in 46% of patients with SMA/SBMA, and 40% of CIDP patients, but in none of the normal controls. SMA/SBMA patients frequently suffer from disabling fatigue presumably caused by ADCB induced by voluntary activity. ADCB could be the mechanism for muscle fatigue in chronic lower motor neuron diseases. Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  11. Microarray analysis of gene expression by skeletal muscle of three mouse models of Kennedy disease/spinal bulbar muscular atrophy.

    Directory of Open Access Journals (Sweden)

    Kaiguo Mo

    2010-09-01

    Full Text Available Emerging evidence implicates altered gene expression within skeletal muscle in the pathogenesis of Kennedy disease/spinal bulbar muscular atrophy (KD/SBMA. We therefore broadly characterized gene expression in skeletal muscle of three independently generated mouse models of this disease. The mouse models included a polyglutamine expanded (polyQ AR knock-in model (AR113Q, a polyQ AR transgenic model (AR97Q, and a transgenic mouse that overexpresses wild type AR solely in skeletal muscle (HSA-AR. HSA-AR mice were included because they substantially reproduce the KD/SBMA phenotype despite the absence of polyQ AR.We performed microarray analysis of lower hindlimb muscles taken from these three models relative to wild type controls using high density oligonucleotide arrays. All microarray comparisons were made with at least 3 animals in each condition, and only those genes having at least 2-fold difference and whose coefficient of variance was less than 100% were considered to be differentially expressed. When considered globally, there was a similar overlap in gene changes between the 3 models: 19% between HSA-AR and AR97Q, 21% between AR97Q and AR113Q, and 17% between HSA-AR and AR113Q, with 8% shared by all models. Several patterns of gene expression relevant to the disease process were observed. Notably, patterns of gene expression typical of loss of AR function were observed in all three models, as were alterations in genes involved in cell adhesion, energy balance, muscle atrophy and myogenesis. We additionally measured changes similar to those observed in skeletal muscle of a mouse model of Huntington's Disease, and to those common to muscle atrophy from diverse causes.By comparing patterns of gene expression in three independent models of KD/SBMA, we have been able to identify candidate genes that might mediate the core myogenic features of KD/SBMA.

  12. Non-neural phenotype of spinal and bulbar muscular atrophy: results from a large cohort of Italian patients.

    Science.gov (United States)

    Querin, Giorgia; Bertolin, Cinzia; Da Re, Elisa; Volpe, Marco; Zara, Gabriella; Pegoraro, Elena; Caretta, Nicola; Foresta, Carlo; Silvano, Maria; Corrado, Domenico; Iafrate, Massimo; Angelini, Lorenzo; Sartori, Leonardo; Pennuto, Maria; Gaiani, Alessandra; Bello, Luca; Semplicini, Claudio; Pareyson, Davide; Silani, Vincenzo; Ermani, Mario; Ferlin, Alberto; Sorarù, Gianni

    2016-08-01

    To carry out a deep characterisation of the main androgen-responsive tissues involved in spinal and bulbar muscular atrophy (SBMA). 73 consecutive Italian patients underwent a full clinical protocol including biochemical and hormonal analyses, genitourinary examination, bone metabolism and densitometry, cardiological evaluation and muscle pathology. Creatine kinase levels were slightly to markedly elevated in almost all cases (68 of the 73; 94%). 30 (41%) patients had fasting glucose above the reference limit, and many patients had total cholesterol (40; 54.7%), low-density lipoproteins cholesterol (29; 39.7%) and triglyceride (35; 48%) levels above the recommended values. Although testosterone, luteinising hormone and follicle-stimulating hormone values were generally normal, in one-third of cases we calculated an increased Androgen Sensitivity Index reflecting the presence of androgen resistance in these patients. According to the International Prostate Symptom Score (IPSS), 7/70 (10%) patients reported severe lower urinal tract symptoms (IPSS score >19), and 21/73 (30%) patients were moderately symptomatic (IPSS score from 8 to 19). In addition, 3 patients were carriers of an indwelling bladder catheter. Videourodynamic evaluation indicated that 4 of the 7 patients reporting severe urinary symptoms had an overt prostate-unrelated bladder outlet obstruction. Dual-energy X-ray absorptiometry scan data were consistent with low bone mass in 25/61 (41%) patients. Low bone mass was more frequent at the femoral than at the lumbar level. Skeletal muscle biopsy was carried out in 20 patients and myogenic changes in addition to the neurogenic atrophy were mostly observed. Our study provides evidence of a wide non-neural clinical phenotype in SBMA, suggesting the need for comprehensive multidisciplinary protocols for these patients. 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/

  13. Human adipose-derived mesenchymal stem cells as a new model of spinal and bulbar muscular atrophy.

    Directory of Open Access Journals (Sweden)

    Marta Dossena

    Full Text Available Spinal and bulbar muscular atrophy (SBMA or Kennedy's disease is an X-linked CAG/polyglutamine expansion motoneuron disease, in which an elongated polyglutamine tract (polyQ in the N-terminal androgen receptor (ARpolyQ confers toxicity to this protein. Typical markers of SBMA disease are ARpolyQ intranuclear inclusions. These are generated after the ARpolyQ binds to its endogenous ligands, which promotes AR release from chaperones, activation and nuclear translocation, but also cell toxicity. The SBMA mouse models developed so far, and used in preclinical studies, all contain an expanded CAG repeat significantly longer than that of SBMA patients. Here, we propose the use of SBMA patients adipose-derived mesenchymal stem cells (MSCs as a new human in vitro model to study ARpolyQ toxicity. These cells have the advantage to express only ARpolyQ, and not the wild type AR allele. Therefore, we isolated and characterized adipose-derived MSCs from three SBMA patients (ADSC from Kennedy's patients, ADSCK and three control volunteers (ADSCs. We found that both ADSCs and ADSCKs express mesenchymal antigens, even if only ADSCs can differentiate into the three typical cell lineages (adipocytes, chondrocytes and osteocytes, whereas ADSCKs, from SBMA patients, showed a lower growth potential and differentiated only into adipocyte. Moreover, analysing AR expression on our mesenchymal cultures we found lower levels in all ADSCKs than ADSCs, possibly related to negative pressures exerted by toxic ARpolyQ in ADSCKs. In addition, with proteasome inhibition the ARpolyQ levels increased specifically in ADSCKs, inducing the formation of HSP70 and ubiquitin positive nuclear ARpolyQ inclusions. Considering all of this evidence, SBMA patients adipose-derived MSCs cultures should be considered an innovative in vitro human model to understand the molecular mechanisms of ARpolyQ toxicity and to test novel therapeutic approaches in SBMA.

  14. 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

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

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    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.

  16. [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.

  17. 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

  18. 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

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

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    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.

  20. 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.

  1. 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...

  2. 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

  3. 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.)

  4. 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.

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

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    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.

  6. Bell palsy: Clinical examination and management.

    Science.gov (United States)

    Patel, Donika K; Levin, Kerry H

    2015-07-01

    Bell palsy is a common neurologic disorder characterized by acute facial mononeuropathy of unclear cause presenting with unilateral facial weakness. Careful examination and a detailed history are important in making an accurate diagnosis. Early recognition is essential, as treatment with corticosteroids within 72 hours of onset has been shown to hasten recovery. Fortunately, most patients recover spontaneously within 3 weeks, even if untreated. Copyright © 2015 Cleveland Clinic.

  7. Hypoglossal Nerve Palsy After Cervical Spine Surgery

    OpenAIRE

    Ames, Christopher P.; Clark, Aaron J.; Kanter, Adam S.; Arnold, Paul M.; Fehlings, Michael G.; Mroz, Thomas E.; Riew, K. Daniel

    2017-01-01

    Study Design: Multi-institutional retrospective study. Objective: The goal of the current study is to quantify the incidence of 2 extremely rare complications of cervical spine surgery; hypoglossal and glossopharyngeal nerve palsies. Methods: A total of 8887 patients who underwent cervical spine surgery from 2005 to 2011 were included in the study from 21 institutions. Results: No glossopharyngeal nerve injuries were reported. One hypoglossal nerve injury was reported after a C3-7 laminectomy...

  8. NEONATAL NERVE PALSIES: A CONTEMPORARY OBSTETRIC PERSPECTIVE

    Directory of Open Access Journals (Sweden)

    Daren J. Roberts

    2014-05-01

    Full Text Available Background:Birth trauma and its often incorrect inference of iatrogenic causation has led to unfortunate implications for the affected child, the parents, the obstetrician and the midwife due to unwarranted medico-legal attention in our current litigious society.A more discerning evaluation of neonatal nerve palsies following labour and delivery has led to a better understanding of their aetiology with potentially more appropriate outcomes for all parties involved.

  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. 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

  11. 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.

  12. 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/.

  13. Does site of buccal mucosa graft for bulbar urethra stricture affect outcome? A comparative analysis of ventral, dorso-lateral and dorsal buccal mucosa graft augmentation urethroplasty.

    Science.gov (United States)

    Pathak, Hemant R; Jain, Tarunkumar Prakash; Bhujbal, Sachin A; Meshram, Kunal R; Gadekar, Chetan; Parab, Sandesh

    2017-09-01

    To compare long- term outcomes of buccal mucosa graft (BMG) augmentation urethroplasty for long segment bulbar urethral strictures done by placing the graft ventrally, dorso-laterally and dorsally. We conducted a single institution retrospective study on 112 who underwent BMG augmentation urethroplasty for non-traumatic bulbar urethral strictures between January 2005 to December 2014. The cases were divided into three groups based on the site of placement of BMG graft i.e. (a) Ventral (n=44), (b) Dorso-lateral (n=48) and (c) Dorsal (n=20). Follow-up period was from one year to five years. Patients with failed outcomes underwent urethroscopy or retrograde urethrogram to note the site of recurrence of stricture. Out of 112 cases 91 (81%) were successful and 21 (19%) failed. The success rates for ventral, dorso-lateral and dorsal BMG augmentation procedures were 89%, 79% and 70%, respectively (p=0.18). Among 21 failed cases, 12 cases (57%) had stricture at proximal anastomotic site, 4 cases (19%) at graft and 5 cases (24%) at distal anastomotic site (p=0.01). The overall success rate for BMG augmentation urethroplasty is equal for all techniques. Ventral onlay urethroplasty provides better exposure of proximal anastomotic site thus it is associated with minimum proximal anastomotic site recurrence rates. Patients with extensive spongiofibrosis and long segment strictures had higher rates of failure.

  14. 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…

  15. 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 ...

  16. 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

  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. 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.

  19. 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...

  20. 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

  1. Late-onset spastic paraplegia type 10 (SPG10) family presenting with bulbar symptoms and fasciculations mimicking amyotrophic lateral sclerosis.

    Science.gov (United States)

    Kaji, Seiji; Kawarai, Toshitaka; Miyamoto, Ryosuke; Nodera, Hiroyuki; Pedace, Lucia; Orlacchio, Antonio; Izumi, Yuishin; Takahashi, Ryosuke; Kaji, Ryuji

    2016-05-15

    Pathogenic mutations in the KIF5A-SPG10 gene, encoding the kinesin HC5A, can be associated with autosomal dominant hereditary spastic paraplegia (ADHSP). It accounts for about 10% of the complicated forms of ADHSP. Peripheral neuropathy, distal upper limb amyotrophy, and cognitive decline are the most common additional clinical features. We examined a 66-year-old Japanese woman manifesting gait disturbance and spastic dysarthria for 6years with positive family history. She showed evidence of upper and lower motor neuron involvement and fasciculations, thus mimicking amyotrophic lateral sclerosis (ALS). Genetic analysis revealed a heterozygous variant in KIF5A (c.484C>T, p.Arg162Trp) in 2 symptomatic members. The mutation was also identified in 4 asymptomatic members, including 2 elderly members aged over 78years. Electromyography in the 2 symptomatic members revealed evidence of lower motor neuron involvement and fasciculation potentials in distal muscles. This report describes the first known Asian family with a KIF5A mutation and broadens the clinical and electrophysiological spectrum associated with KIF5A-SPG10 mutations. Given that our cases showed pseudobulbar palsy, fasciculation and altered penetrance, KIF5A-SPG10 might well be considered as a differential diagnosis of sporadic ALS. Copyright © 2016 Elsevier B.V. All rights reserved.

  2. 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

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  5. Facial nerve problems and Bell's palsy

    OpenAIRE

    Sala, DV; Venter, C; Valenas, O

    2015-01-01

    Bell's palsy is paralysis or weakness of muscle at the hemifacial level, a form of temporary facial paralysis, probable a virus infection or trauma, to one or two facial nerves. Damage to the facial nerve innervating the muscles on one side of the face result in a flabby appearance, fell the respective hemiface. Nerve damage can also affect the sense of taste and salivary and lacrimal secretion. This condition begins suddenly, often overnight, and usually gets better on its own within a few w...

  6. REHABILITATION OF PERSONS WITH CEREBRAL PALSY

    Directory of Open Access Journals (Sweden)

    Natasa CICEVSKA-JOVANOVA

    2000-06-01

    Full Text Available The persons with cerebral palsy with motoric impairments as a primary demmages, they have other following disables: visual impairments, hearing impairments, speech disables and very often they have intellectual difficulties.This persons in school have problems with writing, they couldn’t oriented in the books, they have difficulties with manipulation with school’s supplies and didactic materials, they couldn’t follow the order of the words in the line during the reading and the writing and etc.Using the exercises of psycho-motor reeducation, all before mentioned difficulties and problems can be mitigate or disappear.

  7. Education and employment prospects in cerebral palsy

    DEFF Research Database (Denmark)

    Michelsen, Susan Ishøy; Uldall, Peter; Kejs, Anne Mette T

    2005-01-01

    information was obtained from Denmark's unique registries. Of the participants with CP, 33% vs 77% of controls, had education beyond lower secondary school (i.e. after age 15-16y), 29% were competitively employed (vs 82% of controls), 5% were studying, and 5% had specially created jobs. Excluding participants......Parents and paediatric neurologists need information on the long-term social prognosis of children with cerebral palsy (CP). No large population-based study has been performed on this topic. On 31 December 1999, to find predictors in childhood of subsequent education and employment, 819...

  8. 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.

  9. 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.

  10. 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.

  11. 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

  12. [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.

  13. 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...

  14. Herpes Zoster ophthalmicus with occulomotor nerve palsy

    Directory of Open Access Journals (Sweden)

    Hayati Kandiş

    2010-09-01

    Full Text Available Dear Editor;A 79-year-old male patient was admitted to our emergency department with a complaining of eruption over his face for 10 days and inability to open his eyes for a few days. The patient had hypertension and diabetes mellitus. He had no history of smoking, alcohol. On examination, there was vesicular cutaneous eruption, erosions and crusts, as well as ptosis, in some areas in the ophthalmic division of the trigeminal nerve on the left side of his face (Figure 1. The patient did not have extraocular muscle palsy. Patient was cachectic and dehydrated appearance. Other systemic examinations were unremarkable. Laboratory investigations showed total white cell count of 16500 (neutrophil: 15000, N: 5200–12400, and CRP: 15 mg/dL (N: 0.1–0.5. A clinical diagnosis of ophthalmic zoster with occulomotor nerve palsy was made and the valasiclovir 3g/d was given to patient, wet dressing with an aluminum acetate solution 0,5%. The patient’s lesions had markedly improved within 10 days.

  15. A case misdiagnosed as bilateral abducens palsy

    International Nuclear Information System (INIS)

    Takeda, Naoya; Kuwamura, Keiichi; Shirataki, Kunio; Tamaki, Norihiko; Matsumoto, Satoshi

    1984-01-01

    A 66-year-old male was admitted to our neurosurgical floor because of double vision and gait disturbance. Neurological examinations revealed bilateral 6th nerve palsy with both eyes pointing toward the midline. Initially, using a tentative diagnosis of intracranial mass lesions, especially localized at the base of the skull, computerized tomography of the head, cerebral angiography, orbital venography, and metrizamide CT cisternography were performed; the findings were normal. An orbital CT scan showed an enlargement of the bilateral medial rectus muscles, and the thyroid functions of T 3 and T 4 and the T 3 uptake were all elevated, which was compatible with the diagnosis of Graves' disease. The limitations of both eyeballs were considered to be due not to the 6th nerve palsy, but to the hypertrophy of the bilateral medial rectus muscles. We neurosurgeons should recall Graves' disease as well as intracranial lesions, cerebrovascular disease, and post-traumatic sequelae when examining a patient who presents limitations of external ocular movement. (author)

  16. 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.

  17. 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.)

  18. Early intervention to improve hand function in hemiplegic cerebral palsy

    Directory of Open Access Journals (Sweden)

    Anna Purna Basu

    2015-01-01

    Full Text Available Children with hemiplegic cerebral palsy often have marked hand involvement with excessive thumb adduction and flexion and limited active wrist extension from infancy. Post-lesional aberrant plasticity can lead to progressive abnormalities of the developing motor system. Disturbances of somatosensory and visual function and developmental disregard contribute to difficulties with hand use. Progressive soft tissue and bony changes may occur, leading to contractures which further limit function in a vicious cycle. Early intervention might help to break this cycle: however, the precise nature and appropriateness of the intervention must be carefully considered. Traditional approaches to the hemiplegic upper limb include medications and botulinum toxin injections to manage abnormalities of tone, and surgical interventions. Therapist input, including provision of orthoses, remains a mainstay although many therapies have not been well evaluated. There has been a recent increase in interventions for the hemiplegic upper limb, mostly aimed outside the period of infancy. These include trials of constraint-induced movement therapy and bimanual therapy as well as the use of virtual reality and robot-assisted therapy. In future, non-invasive brain stimulation may be combined with therapy. Interventions under investigation in the infant age group include modified constraint-induced movement therapy and action observation therapy. A further approach which may be suited to the infant with thumb-in-palm deformity, but which requires evaluation, is the use of elastic taping. Enhanced cutaneous feedback through mechanical stimulation to the skin provided by the tape during movement has been postulated to modulate ongoing muscle activity. If effective, this would represent a low-cost, safe, widely applicable early intervention.

  19. Crossing boundaries : improving communication in cerebral palsy care

    NARCIS (Netherlands)

    Gulmans, J.; Gulmans, J.

    2012-01-01

    In the Netherlands, children with cerebral palsy are the largest diagnostic group treated in paediatric rehabilitation, requiring specialized health-, education- and social services of multiple professionals from diverse organizations. To provide ‘integrated care’ in these settings, effective care

  20. 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.

  1. 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

  2. Gait Trainer for Children with Spastic Cerebral Palsy

    National Research Council Canada - National Science Library

    Urhan, Oguzhan

    2001-01-01

    A device is developed to improve the walking ability of children with Spastic Cerebral Palsy, who have damages to the area of their brain which controls the muscle tone and that causes trouble walking...

  3. 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

  4. Combination of Citicoline and Physiotherapy in Children with Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Jafar Nasiri

    2014-01-01

    Conclusions: Results demonstrated that citicoline in combination to physiotherapy appears to be a promising agent to improve gross motor function in patients with cerebral palsy versus physiotherapy alone. Although, further studies are need to be done.

  5. 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.

  6. 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.)

  7. 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.

  8. 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...

  9. 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)

  10. 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)

  11. 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...

  12. 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

  13. 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,…

  14. 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....

  15. 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.

  16. 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

  17. 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.

  18. 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.

  19. [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.

  20. 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.

  1. Hypoglossal Nerve Palsy After Cervical Spine Surgery.

    Science.gov (United States)

    Ames, Christopher P; Clark, Aaron J; Kanter, Adam S; Arnold, Paul M; Fehlings, Michael G; Mroz, Thomas E; Riew, K Daniel

    2017-04-01

    Multi-institutional retrospective study. The goal of the current study is to quantify the incidence of 2 extremely rare complications of cervical spine surgery; hypoglossal and glossopharyngeal nerve palsies. A total of 8887 patients who underwent cervical spine surgery from 2005 to 2011 were included in the study from 21 institutions. No glossopharyngeal nerve injuries were reported. One hypoglossal nerve injury was reported after a C3-7 laminectomy (0.01%). This deficit resolved with conservative management. The rate by institution ranged from 0% to 1.28%. Although not directly injured by the surgical procedure, the transient nerve injury might have been related to patient positioning as has been described previously in the literature. Hypoglossal nerve injury during cervical spine surgery is an extremely rare complication. Institutional rates may vary. Care should be taken during posterior cervical surgery to avoid hyperflexion of the neck and endotracheal tube malposition.

  2. Education and employment prospects in cerebral palsy

    DEFF Research Database (Denmark)

    Michelsen, Susan Ishøy; Uldall, Peter; Kejs, Anne Mette T

    2005-01-01

    information was obtained from Denmark's unique registries. Of the participants with CP, 33% vs 77% of controls, had education beyond lower secondary school (i.e. after age 15-16y), 29% were competitively employed (vs 82% of controls), 5% were studying, and 5% had specially created jobs. Excluding participants......Parents and paediatric neurologists need information on the long-term social prognosis of children with cerebral palsy (CP). No large population-based study has been performed on this topic. On 31 December 1999, to find predictors in childhood of subsequent education and employment, 819...... with epilepsy versus those without epilepsy. The severity of motor impairment among participants with CP able to walk had just a minor influence. Only half the participants with CP who had attended mainstream schooling were employed. In conclusion several childhood characteristics seemed to predict long...

  3. The Child with Cerebral Palsy and Anaesthesia

    Directory of Open Access Journals (Sweden)

    A Rudra

    2008-01-01

    Full Text Available Cerebral palsy (CP is the result of an injury to the developing brain during the antenatal, perinatal or postnatal period. Clinical manifestation relate to the areas affected. Patients with CP often present for elective surgical proce-dures to correct various deformities. Anaesthetic concerns of anaesthesia are intraoperative hypothermia , and slow emergence. Suxamethonium does not cause hyperkalaemia in these patients, and a rapid sequence induction may be indicated. Temperature should be monitored and an effort made to keep the patient warm. Cerebral abnormalities may lead to slow awakening; the patient should remain intubated until fully awake and airway reflexes have returned. Pulmonary infection can complicate the postoperative course. Postoperative pain management and the prevention of muscle spasms are important and drugs as baclofen and botulinum toxin are discussed. Epidural analgesia is particu-larly valuable when major orthopaedic procedures are performed.

  4. Family adaptation to cerebral palsy in adolescents

    DEFF Research Database (Denmark)

    Guyard, Audrey; Michelsen, Susan I; Arnaud, Catherine

    2017-01-01

    modelled with structural equations. RESULTS: 31.8% of parents living with an adolescent with CP showed clinically significant high stress requiring professional assistance. The main stressors were the level of motor impairment and behavioural disorders in adolescent. A good family functioning was the best......BACKGROUND AND AIM: Factors promoting family adaptation to child's disability are poorly studied together. The aim of the study was to describe the family adaptation to disability and to identify determinants associated with using a global theoretical model. MATERIALS AND METHODS: 286 families...... of teenagers [13-17 years] with cerebral palsy (CP) from 4 European disability registers were included and visited at home. Face to face interviews were performed in order to measure parental distress, perceived impact in various dimensions of family life, family resources and stressors. Relationships were...

  5. 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.

  6. 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

  7. Survey on Types and Associated disorders of Cerebral Palsy in Eastern and Northern Districts of Tehran

    Directory of Open Access Journals (Sweden)

    Farin Soleimani

    2011-10-01

    Conclusion: In this study, unilateral - spastic cerebral palsy was found as the most common type. Therefore, more evaluation to determine the about etiology of this type of cerebral palsy in our population is necessary.

  8. 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.

  9. Treatment with Creatine Monohydrate in Spinal and Bulbar Muscular Atrophy: Protocol for a Randomized, Double-Blind, Placebo-Controlled Trial.

    Science.gov (United States)

    Hijikata, Yasuhiro; Katsuno, Masahisa; Suzuki, Keisuke; Hashizume, Atsushi; Araki, Amane; Yamada, Shinichiro; Inagaki, Tomonori; Ito, Daisuke; Hirakawa, Akihiro; Kinoshita, Fumie; Gosho, Masahiko; Sobue, Gen

    2018-03-05

    Although spinal and bulbar muscular atrophy (SBMA) has been classified as a motor neuron disease, several reports have indicated the primary involvement of skeletal muscle in the pathogenesis of this devastating disease. Recent studies reported decreased intramuscular creatine levels in skeletal muscles in both patients with SBMA and transgenic mouse models of SBMA, which appears to contribute to muscle weakness. The present study aimed to examine the efficacy and safety of oral creatine supplementation to improve motor function in patients with SBMA. A randomized, double-blind, placebo-controlled, three-armed clinical trial was conducted to assess the safety and efficacy of creatine therapy in patients with SBMA. Patients with SBMA eligible for this study were assigned randomly in a 1:1:1 ratio to each group of placebo, 10 g, or 15 g daily dose of creatine monohydrate in a double-blind fashion. Participants took creatine or placebo orally 3 times a day for 8 weeks. Outcome measurements were results of neurological assessments, examinations, and questionnaires collected at baseline and at weeks 4, 8, and 16 after a washout period. The primary endpoint was the change in handgrip strength values from baseline to week 8. The secondary endpoints included the following: results of maximum voluntary isometric contraction tests of extremities; tongue pressure; results of the 15-foot timed walk test and the rise from bed test; modified quantitative myasthenia gravis score; respiratory function test results; activities of daily living assessed with the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale and the Spinal and Bulbar Muscular Atrophy Functional Rating Scale; skeletal muscle mass measured with dual-energy X-ray absorptiometry; urinary 8-hydroxydeoxyguanosine levels; and questionnaires examining the quality of life, swallowing function, and fatigue. Participant enrollment in the trial started from June 2014 and follow-up was completed in July 2015. The

  10. 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.

  11. 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...

  12. Immature spinal locomotor output in children with Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Germana Cappellini

    2016-10-01

    Full Text Available Detailed descriptions of gait impairments have been reported in cerebral palsy (CP, but it is still unclear how maturation of the spinal motoneuron output is affected. Spatiotemporal alpha-motoneuron activation during walking can be assessed by mapping the electromyographic activity profiles from several, simultaneously recorded muscles onto the anatomical rostrocaudal location of the motoneuron pools in the spinal cord, and by means of factor analysis of the muscle activity profiles. Here, we analysed gait kinematics and EMG activity of 11 pairs of bilateral muscles with lumbosacral innervation in 35 children with CP (19 diplegic, 16 hemiplegic, 2-12 years and 33 typically developing (TD children (1-12 years. TD children showed a progressive reduction of EMG burst durations and a gradual reorganization of the spatiotemporal motoneuron output with increasing age. By contrast, children with CP showed very limited age-related changes of EMG durations and motoneuron output, as well as of limb intersegmental coordination and foot trajectory control (on both sides for diplegic children and the affected side for hemiplegic children. Factorization of the EMG signals revealed a comparable structure of the motor output in children with CP and TD children, but significantly wider temporal activation patterns in children with CP, resembling the patterns of much younger TD infants. A similar picture emerged when considering the spatiotemporal maps of alpha-motoneuron activation. Overall, the results are consistent with the idea that early injuries to developing motor regions of the brain substantially affect the maturation of the spinal locomotor output and consequently the future locomotor behaviour.

  13. 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.

  14. 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.

  15. 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.

  16. 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.

  17. 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.

  18. 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.

  19. Virtual rehabilitation in a school setting: is it feasible for children with cerebral palsy?

    Science.gov (United States)

    Rosie, Juliet A; Ruhen, Shelley; Hing, Wayne A; Lewis, Gwyn N

    2015-01-01

    To determine the feasibility of a school-based virtual rehabilitation intervention for children with cerebral palsy. A feasibility study was conducted using a mixed method approach. Participants were five children with cerebral palsy who were currently attending a rural school. Each child received an 8-week rehabilitation programme involving an Interactive Virtual Reality Exercise (IREX) system. The IREX was placed in the child's school for the duration of the intervention. Each child's programme was designed by a physiotherapist but supervised by a teacher aide at the school. Feasibility of the intervention was assessed through a questionnaire completed by the child and an interview conducted with the teacher supervisor. The children all rated the IREX intervention as fun, easy to use, and beneficial for their arm. Categories from the supervisor interviews centred on resolving technical issues, the enjoyment of taking part due to the child's progress, and the central role of interacting with the child. Input from the research physiotherapist was critical to the success of the intervention. The IREX is feasible to implement in a school-based setting supervised by teachers. This provides an option for delivering physiotherapy to children in isolated areas who do not receive on-going therapy. Implication for Rehabilitation Virtual rehabilitation programmes using the IREX are feasible in a school-based setting. The negative impact of technical difficulties is likely to be overcome by the user's enjoyment and rehabilitation benefits gained. Input from a therapist in designing and monitoring the programme is critical.

  20. 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.

  1. 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…

  2. [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

  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. Corticosteroids for Bell's palsy (idiopathic facial paralysis).

    Science.gov (United States)

    Madhok, Vishnu B; Gagyor, Ildiko; Daly, Fergus; Somasundara, Dhruvashree; Sullivan, Michael; Gammie, Fiona; Sullivan, Frank

    2016-07-18

    Inflammation and oedema of the facial nerve are implicated in causing Bell's palsy. Corticosteroids have a potent anti-inflammatory action that should minimise nerve damage. This is an update of a review first published in 2002 and last updated in 2010. To determine the effectiveness and safety of corticosteroid therapy in people with Bell's palsy. On 4 March 2016, we searched the Cochrane Neuromuscular Specialised Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE and LILACS. We reviewed the bibliographies of the randomised trials and contacted known experts in the field to identify additional published or unpublished trials. We also searched clinical trials registries for ongoing trials. Randomised trials and quasi-randomised trials comparing different routes of administration and dosage schemes of corticosteroid or adrenocorticotrophic hormone therapy versus a control group receiving no therapy considered effective for this condition, unless the same therapy was given in a similar way to the experimental group. We used standard Cochrane methodology. The main outcome of interest was incomplete recovery of facial motor function (i.e. residual facial weakness). Secondary outcomes were cosmetically disabling persistent sequelae, development of motor synkinesis or autonomic dysfunction (i.e. hemifacial spasm, crocodile tears) and adverse effects of corticosteroid therapy manifested during follow-up. We identified seven trials, with 895 evaluable participants for this review. All provided data suitable for the primary outcome meta-analysis. One of the trials was new since the last version of this Cochrane systematic review. Risk of bias in the older, smaller studies included some unclear- or high-risk assessments, whereas we deemed the larger studies at low risk of bias. Overall, 79/452 (17%) participants allocated to corticosteroids had incomplete recovery of facial motor function six months or more after randomisation

  5. Theory of mind, empathy and neuropsychological functioning in X-linked spinal and bulbar muscular atrophy: a controlled study of 20 patients.

    Science.gov (United States)

    Di Rosa, Elisa; Sorarù, Gianni; Kleinbub, Johann Roland; Calvo, Vincenzo; Vallesi, Antonino; Querin, Giorgia; Marcato, Sonia; Grasso, Irene; Palmieri, Arianna

    2015-02-01

    Recent studies have described brain involvement, mainly at frontal level, in patients with spinal and bulbar muscular atrophy (SBMA), a rare adult-onset motor neuron disease caused by a CAG repeat in the androgen receptor (AR) gene. The aim of our research was to investigate the poorly characterized neuropsychological and psychological profile of these patients, on the basis of previous literature. We administered a neuropsychological screening and tests relating to cognitive and affective empathy, attributed to the theory of mind (ToM) framework, to 20 males with SBMA, and to age- and education-matched controls. Although patients' neuropsychological performance was unimpaired, a clear dissociation emerged between their cognitive and affective empathy. Patients had distinctive deficits in mentalizing, as assessed with the Faux Pas Test, whilst affective empathy (i.e., sharing experience), assessed with the Reading the Mind in the Eyes test, appeared to be preserved. The likely implications of subtle frontal lobe impairments on the one hand, and a protective influence of androgen insensitivity in these patients on the other, are discussed in the light of our results.

  6. 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.

  7. 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

  8. 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/

  9. 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.

  10. 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)

  11. 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)

  12. 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)

  13. 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.

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

    Directory of Open Access Journals (Sweden)

    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. Large subgaleal hematoma producing turban head in 10 year boy with cerebral palsy: rare case report with review of literature

    Directory of Open Access Journals (Sweden)

    Tripathi Praveen Kumar

    2016-12-01

    Full Text Available Subgaleal hematomas (SGHs are not uncommon. Because the subgaleal space has no anatomical boundaries, SGHs usually involve a large space and are typically limited to the parietal region. Cases of SGHs involving whole of head are relatively rare. In this study we report a rare case of massive enlargement of head after SGH causing severe pain and giving an appearance of turban. A 10 year old, male patient with cerebral palsy presented with progressive enlargement of head attaining a size of turban due to habitual head banging and self-punching overhead. SGH drainage and hematoma aspiration were performed and the patient’s head size was restored.

  16. 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

  17. Neuropsychological profiles of children with cerebral palsy.

    Science.gov (United States)

    Stadskleiv, Kristine; Jahnsen, Reidun; Andersen, Guro L; von Tetzchner, Stephen

    2018-02-01

    To explore factors contributing to variability in cognitive functioning in children with cerebral palsy (CP). A geographical cohort of 70 children with CP was assessed with tests of language comprehension, visual-spatial reasoning, attention, working memory, memory, and executive functioning. Mean age was 9;9 years (range 5;1-17;7), 54.3% were girls, and 50.0% had hemiplegic, 25.7% diplegic, 12.9% quadriplegic, and 11.4% dyskinetic CP. For the participants with severe motor impairments, assessments were adapted for gaze pointing. A cognitive quotient (CQ) was computed. Mean CQ was 78.5 (range 19-123). Gross motor functioning, epilepsy, and type of brain injury explained 35.5% of the variance in CQ (F = 10.643, p = .000). Twenty-four percent had an intellectual disability, most of them were children with quadriplegic CP. Verbal comprehension and perceptual reasoning scores did only differ for the 21% with an uneven profile, of whom two-thirds had challenges with perceptual reasoning.

  18. Cerebral Palsy: Comprehensive Review and Update

    International Nuclear Information System (INIS)

    Jan, Mohammed M.S.

    2006-01-01

    Cerebral palsy (CP) is a common pediatric disorder occurring in about 2 to 2.5 per 1000 live births. It is a chronic motor disorder resulting from a nonprogressive (static) insult to the developing brain. CP is the clinical presentation of a wide variety of cerebral cortical or sub-cortical insults occurring during the first year of life. The commonest cause of CP remains unknown in 50% of the cases; prematurity remains the commonest risk factor. Children with CP suffer multiple problems and potential disabilities such as mental retardation, epilepsy, feeding difficulties, and ophthalmologic and hearing impairments. Screening for those conditions should be part of the initial assessment. The child with CP is best cared for with an individualized treatment plan that provides a combination of interventions. This requires the provision of a number of family-centered services that make a difference in the lives of these children and their families. Management of spasticity can be challenging with a wide variety of possible therapeutic interventions. The treatment must be goal oriented, such as to assist with mobility, reduce or prevent contractures, improve positioning and hygiene, and provide comfort. Each member of the child's multidisciplinary team, including the child and both parents, should participate in the serial evaluations and treatment planning. (author)

  19. Computerized tomographic studies in cerebral palsy

    International Nuclear Information System (INIS)

    Sugie, Yoko

    1981-01-01

    Computed tomographic (CT) findings in 200 children with cerebral palsy (CP) were analysed from the viewpoint of clinical manifestations, disease complications and etiological factors. CT scans of 135 cases (67.5%) were found to be abnormal and there were 14 (7%) borderline cases. The major abnormality found on CT scans was cerebral atrophy. Other important changes included focal or diffuse low density area in the brain tissue, congenital malformation, and cerebellar atrophy. From the clinical point of view, a large number of patients with spastic tetraplegia and spastic diplegia showed highly abnormal CT scans. On the other hand, in patients with spastic monoplegia, spastic paraplegia, and athetotic type, CT findings were normal or revealed only minor cerebral atrophy. Most children showing asymmetric clinical symptoms had corresponding asymmetric CT abnormalities which included ventricular enlargement, low density area in the brain tissue, and hemispherical volume. There was a significant correlation between the severity of physical impairment and the extent of CT abnormalities. Severely affected children had grossly abnormal CT scans such as hydranencephaly, polycystic change, and extensive cerebral atrophy. In the patients complicated with epilepsy, the incidence and severity of abnormal CT were higher than those of non-epileptic patients. Mentally retarded patients had variable enlargement of the subarachnoidal space depending on the severity of their mental retardation. Patients with suspected postnatal etiology also had high incidence of severe CT abnormality. CT scan is a valuable tool for evaluating patients with CP and in some cases, possible etiology of the disease may be discovered. (author)

  20. Mastery motivation in adolescents with cerebral palsy.

    Science.gov (United States)

    Majnemer, Annette; Shikako-Thomas, Keiko; Lach, Lucy; Shevell, Michael; Law, Mary; Schmitz, Norbert

    2013-10-01

    The aim of this study is to describe motivation in adolescents with cerebral palsy (CP) and factors associated with motivation level. The Dimensions of Mastery Questionnaire (DMQ) measures motivation in mastering challenging tasks and expressive elements. It was completed by 153 parents and 112 adolescents with CP. Adolescents (GMFCS in n=146 - I:50, II:43, III:13, IV:15, V:25) were assessed using the Leiter IQ and Gross Motor Function Measure. Parents completed the Vineland Adaptive Behavior Scale and the Strengths and Difficulties Questionnaire. Motivation scores were highest for mastery pleasure and social persistence with adults and lowest for gross motor and object-oriented persistence. Socio-demographic factors were not strongly correlated with DMQ. Higher gross motor ability (r=0.24-0.52) and fewer activity limitations (r=0.30-0.64, pProsocial behaviors correlated with high motivation (r=0.39-0.53, pmotivation scores were higher than parents' scores. Adolescents with CP express high mastery pleasure, not related to abilities. High motivation was associated with fewer activity limitations and prosocial behaviors and aspects of family environment. Findings elucidate those at-risk for low motivation, which can influence treatment adherence and participation in challenging but meaningful activities. Copyright © 2013 Elsevier Ltd. All rights reserved.

  1. Rehabilitation outcomes of children with cerebral palsy.

    Science.gov (United States)

    Yalcinkaya, Ebru Yilmaz; Caglar, Nil Sayıner; Tugcu, Betul; Tonbaklar, Aysegul

    2014-02-01

    [Purpose] To evaluate the results of Bobath-based rehabilitation performed at a pediatric cerebral palsy (CP) inpatient clinic. [Subjects and Methods] The study subjects were 28 children with CP who were inpatients at a pediatric service. Inclusion criteria were: being an inpatient of our hospital aged 2-12 with a diagnosis of CP; having one permanent primary caregiver; and the caregiver having no medical or psychotic problems. All of the patients received Bobath treatment for 1 hour per day, 5 days a week. The locomotor system, neurologic and orthopedic examination, Gross Motor Function Measure (GMFM) of the patients, and Short Form-36 (SF-36) of permanent caregivers were evaluated at the time of admission to hospital, discharge from hospital, and at 1 and 3 months after discharge. [Results] Post-admission scores of GMFM at discharge, and 1 and 3 months later showed significant increase. Social function and emotional role subscores of SF-36 had increased significantly at discharge. [Conclusion] Bobath treatment is promising and randomized controlled further studies are needed for rehabilitation technics.

  2. 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.

  3. 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.

  4. 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.

  5. 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...

  6. 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.)

  7. 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.

  8. 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.

  9. Narcolepsy, REM sleep behavior disorder, and supranuclear gaze palsy associated with Ma1 and Ma2 antibodies and tonsillar carcinoma.

    Science.gov (United States)

    Adams, Chris; McKeon, Andrew; Silber, Michael H; Kumar, Rajeev

    2011-04-01

    To describe a patient with diencephalic and mesencephalic presentation of a Ma1 and Ma2 antibody-associated paraneoplastic neurological disorder. Case report. The Colorado Neurological Institute Movement Disorders Center in Englewood, Colorado, and the Mayo Clinic in Rochester, Minnesota. A 55-year-old man with a paraneoplastic neurological disorder characterized by rapid eye movement sleep behavior disorder, narcolepsy, and a progressive supranuclear palsy-like syndrome in the setting of tonsillar carcinoma. Immunotherapy for paraneoplastic neurological disorder, surgery and radiotherapy for cancer, and symptomatic treatment for parkinsonism and sleep disorders. Polysomnography, multiple sleep latency test, and neurological examination. The cancer was detected at a limited stage and treatable. After oncological therapy and immunotherapy, symptoms stabilized. Treatment with modafinil improved daytime somnolence. Rapid onset and progression of multifocal deficits may be a clue to paraneoplastic etiology. Early treatment of a limited stage cancer (with or without immunotherapy) may possibly slow progression of neurological symptoms. Symptomatic treatment may be beneficial.

  10. 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.

  11. 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.

  12. 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.

  13. Treatment of Cerebral Palsy with Stem Cells: A Report of 17 Cases.

    Science.gov (United States)

    Abi Chahine, Nassim H; Wehbe, Tarek W; Hilal, Ramzi A; Zoghbi, Victoria V; Melki, Alia E; Habib, Emil B Bou

    2016-05-30

    Cerebral Palsy (CP) is a disabling condition that affects a child's life and his/her family irreversibly. It is usually a non-progressive condition but improvement over time is rarely seen. The condition can be due to prenatal hypoxia, metabolic, genetic, infectious, traumatic or other causes. It is therefore a heterogeneous group that results in functional motor disability associated with different degrees of cognitive abnormalities. There are no treatments that can cure or even improve CP and the best available approach aims at functional, social and nutritional supportive care and counseling. In this paper, we report 17 sequential patients with CP treated with intrathecal administration of Bone Marrow Mononuclear Cells (BMMC). All patients had an uneventful post-injection course with 73% of the evaluable patients treated having a good response using the Gross Motor Function Classification System (GMFCS). The average improvement was 1.3 levels on the GMFCS with cognitive improvements as well.

  14. Electrical stimulation treatment for facial palsy after revision pleomorphic adenoma surgery.

    Science.gov (United States)

    Goldie, Simon; Sandeman, Jack; Cole, Richard; Dennis, Simon; Swain, Ian

    2016-04-22

    Surgery for pleomorphic adenoma recurrence presents a significant risk of facial nerve damage that can result in facial weakness effecting patients' ability to communicate, mental health and self-image. We report two case studies that had marked facial weakness after resection of recurrent pleomorphic adenoma and their progress with electrical stimulation. Subjects received electrical stimulation twice daily for 24 weeks during which photographs of expressions, facial measurements and Sunnybrook scores were recorded. Both subjects recovered good facial function demonstrating Sunnybrook scores of 54 and 64 that improved to 88 and 96, respectively. Neither subjects demonstrated adverse effects of treatment. We conclude that electrical stimulation is a safe treatment and may improve facial palsy in patients after resection of recurrent pleomorphic adenoma. Larger studies would be difficult to pursue due to the low incidence of cases. Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2016.

  15. 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.

  16. 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

  17. [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.

  18. 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...

  19. 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.

  20. Congenital Cytomegalovirus among Children with Cerebral Palsy.

    Science.gov (United States)

    Smithers-Sheedy, Hayley; Raynes-Greenow, Camille; Badawi, Nadia; Fernandez, Marian A; Kesson, Alison; McIntyre, Sarah; Leung, Kin-Chuen; Jones, Cheryl A

    2017-02-01

    To determine the proportion of children with cerebral palsy (CP) and cytomegalovirus (CMV) DNA detected retrospectively in their newborn screening cards (NBSC), to compare the proportion of children with CMV DNA in their NBSC across spastic subtypes of CP, and to compare the sex and other characteristics of children with CP and CMV detected on their NSBC with those in whom CMV DNA was not detected. Retrospective observational study. Data were extracted from patient records on children with CP (birth years 1996-2014) from 2 Australian state CP registers and state-wide paediatric rehabilitation services with consent. NBSCs were retrospectively analyzed for CMV DNA by nested polymerase chain reaction (PCR) using primers against gB. Positive samples were validated using real time PCR for CMV UL83. Of 401 children recruited, 323 (80.5%) had an available NBSC. Of these, 31 (9.6%; 95% CI, 6.8-13.3) tested positive for CMV DNA by nested PCR for CMV gB, of whom 28 (8.7%; 95% CI, 6.1-12.2) also had CMV DNA detected by real-time PCR for CMV UL83. Detection of CMV DNA was significantly associated with epilepsy, but not with clinical or epidemiologic characteristics, including sex and pattern of spasticity. CMV viremia in the newborn period, indicating congenital CMV infection, is highly prevalent among children with CP. Further research is needed to investigate the mechanisms and contribution of congenital CMV to the causal pathways to CP. Copyright © 2016 Elsevier Inc. All rights reserved.

  1. Mobile applications in children with cerebral palsy.

    Science.gov (United States)

    Rodríguez Mariblanca, M; Cano de la Cuerda, R

    2017-12-21

    Cerebral palsy (CP) is one of the most common developmental disorders. Technological development has enabled a transformation of the healthcare sector, which can offer more individualised, participatory, and preventive services. Within this context of new technology applied to the healthcare sector, mobile applications, or apps, constitute a very promising tool for the management of children with CP. The purpose of this article is to perform a systematic review of the information published about various mobile applications either directly related to CP or with potential to be useful in the context of the disease, and to describe, analyse, and classify these applications. A literature search was carried out to gather articles published in English or Spanish between 2011 and 2017 which presented, analysed, or validated applications either specifically designed or potentially useful for CP. Furthermore, a search for mobile applications was conducted in the main mobile application markets. A total of 63 applications were found in biomedical databases and mobile application markets, of which 40 were potentially useful for CP and 23 were specifically designed for the condition (11 for information, 3 for evaluation, and 9 for treatment). There are numerous mobile applications either specifically designed for or with potential to be useful in the field of CP. However, despite the existing scientific evidence, the low methodological quality of scientific articles makes it impossible to generalise the use of these tools. Copyright © 2017 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  2. The cranial MRI in severe cerebral palsy

    International Nuclear Information System (INIS)

    Yamada, Kazutaka; Itoh, Masahiro; Fueki, Noboru; Hirasawa, Kyoko; Suzuki, Noriko; Kurata, Kiyoko; Sato, Junichi; Morimatsu, Yoshio; Yagishita, Akira.

    1993-01-01

    The magnetic resonance examination was performed in 38 patients with severe cerebral palsy (CP; 15 males and 23 females) who had both motor delay (unable to move anywhere) and mental retardation (I.Q. or D.Q. below 30). Neuroimaging findings were compared with the CP type, etiology, and grade of understanding of language. Cranial magnetic resonance imagings (MRI) in CP were divided into five types. In type 1, nine predominantly showed cyst-liked ventricles and periventricular hyperintensity on T 2 -weighted imaging (PVH) and only scarred basal ganglia and thalamus were visible. All suffered from neonatal asphyxia and the clinical type was rigospastic tetraplegia (RST). In type 2, eleven predominantly showed PVH and hyperintensity on T 2 -weighted (HT2) in basal ganglia and thalamus. All suffered from neonatal asphyxia and the clinical type was RST or rigospastic diplegia. In type 3, five showed PVH and three had cortical atrophy. All suffered from neonatal asphyxia and the clinical type was spastic diplegia. In type 4, four predominantly showed HT2 in putamen and thalamus. Three had cortical atrophy. All suffered from neonatal asphyxia. The clinical type was athetotic CP (ATH). In type 5, nine predominantly showed HT2 in globus pallidus. Four had cortical atrophy and two had hippocampal atrophy. All suffered from neonatal jaundice and the clinical type was ATH. All patients who suffered from neonatal asphyxia and spastic CP had MRI in PVH. All patients who suffered from neonatal asphyxia and ATH showed HT2 in putamen and thalamus. Almost patients who suffered from neonatal jaundice and ATH showed HT2 in globus pallidus. With athetotic CP, cases with atrophy of the cerebral cortex and/or hippocampus were lower grade of understanding of language than no atrophy of both. The results of studies of MRI are in agreement with neuropathological findings. (author)

  3. Cranial nerve palsies in childhood parameningeal rhabdomyosarcoma.

    Science.gov (United States)

    Zorzi, Alexandra P; Grant, Ronald; Gupta, Abha A; Hodgson, David C; Nathan, Paul C

    2012-12-15

    Children with parameningeal rhabdomyosarcoma (PM RMS) and cranial nerve palsy (CNP) are at risk for permanent neurologic dysfunction. Clinicians often consider the use of emergent therapies such as expedited radiation and/or corticosteroids; however, there is a paucity of information describing the natural history of CNP in PM RMS. We sought to describe the clinical features of patients with PM RMS plus associated CNP and to evaluate the patient, disease, and treatment-related factors that impacted neurologic recovery. We conducted a retrospective review of PM RMS cases treated at the Hospital for Sick Children between 1985 and 2010. Thirty-five children were treated for PM RMS, 19 (54%) of whom presented with CNP. Children with CNP were nine times more likely to have other high-risk features (cranial base bony erosion and/or intracranial extension) at the time of presentation than children without CNP (OR 9.6, 95% CI 1.69, 54.79, P = 0.013). In addition to commencing chemotherapy, 13 patients (68%) received expedited RT and corticosteroids, four (21%) corticosteroids alone, and two (11%) received only standard chemotherapy and RT. At last follow up of the 11 survivors, neurologic recovery was complete in five (45%), partial in five (45%), and absent in one (9%). In our cohort, recovery of PM RMS associated CNP was often incomplete despite multi-modal therapy. A larger cohort of patients is required to determine the utility of emergent initiation of radiation or corticosteroids. This study will facilitate the counseling of future families on the long-term neurologic recovery CNP in PM RMS. Copyright © 2012 Wiley Periodicals, Inc.

  4. Hand functioning in children with cerebral palsy

    Directory of Open Access Journals (Sweden)

    Carlyne eArnould

    2014-04-01

    Full Text Available Brain lesions may disturb hand functioning in children with cerebral palsy (CP, making it difficult or even impossible for them to perform several manual activities. Most conventional treatments for hand dysfunction in CP assume that reducing the hand dysfunctions will improve the capacity to manage activities (i.e., manual ability, MA. The aim of this study was to investigate the directional relationships (direct and indirect pathways through which hand skills influence MA in children with CP. A total of 136 children with CP (mean age: 10 years; range: 6–16 years; 35 quadriplegics, 24 diplegics, 77 hemiplegics were assessed. Six hand skills were measured on both hands: touch-pressure detection (Semmes-Weinstein aesthesiometer, stereognosis (Manual Form Perception Test, proprioception (passive mobilization of the metacarpophalangeal joints, grip strength (Jamar dynamometer, gross manual dexterity (Box and Block Test, and fine finger dexterity (Purdue Pegboard Test. MA was measured with the ABILHAND-Kids questionnaire. Correlation coefficients were used to determine the linear associations between observed variables. A path analysis of structural equation modeling was applied to test different models of causal relationships among the observed variables. Purely sensory impairments did seem not to play a significant role in the capacity to perform manual activities. According to path analysis, gross manual dexterity in both hands and stereognosis in the dominant hand were directly related to MA, whereas grip strength was indirectly related to MA through its relationship with gross manual dexterity. However, one-third of the variance in MA measures could not be explained by hand skills. It can be concluded that MA is not simply the integration of hand skills in daily activities and should be treated per se, supporting activity-based interventions.

  5. Treating cerebral palsy with aculaser therapy

    Science.gov (United States)

    Anwar, Shahzad; Nazir Khan, Malik M.; Nadeem Khan, Malik M.; Qazi, Faiza M.; Awan, Abid H.; Dar, Irfan

    2008-03-01

    A single, open and non comparative study was conducted at Anwar Shah Trust for C.P. & Paralysis in collaboration with the Departments of Neurology and Neurosurgery, Children Hospital Lahore, Pakistan to evaluate the effects of ACULASER THERAPY in childern suffering from Cerebral Palsy (C.P.) and associated Neurological Disorders like epilepsy, cortical blindness, spasticity, hemiplegia, paraplegia, diplegia, quadriplegia, monoplegia, sensory-neural deafness and speech disorders. In all 250 childern were treated and the data was gathered during a period of 3 years from December 2003 till December 2006. These children were further classified according to the type of C.P. (spastic, athetoid, mixed) they suffered from and associated Neurological Disorders. This article shows results in C.P. childern who were treated with ACULASER THERAPY for minimum 6 weeks and more or had minimum of 15 treatment sessions and more. This article also shows that those childern who were given a break in the treatment for 1 month to 1 year did not show any reversal of the signs and symptoms. Analysis of the data showed that out of 171 children with Spasticity and Stiffness 147 showed marked improvement showing 87% success rate, out of 126 children with Epileptic fits, there was a significant reduction in the intensity, frequency and duration of Epileptic fits in 91 children showing 72% success rate, out of 48 children with Cortical Blindness 30 children showed improvement accounting for 63% efficacy rate, out of 105 children with Hearing Difficulties, 63 showed marked improvement accounting for 60% improvement rate, out of 190 children with Speech Disorders 122 showed improvement reflecting 64% improvement rate, out of 96 children with Hemiplegia 71 showed improvement in movement, tone and power accounting for 74% improvement rate, out of 76 children with Quadriplegia 52 showed improvement in gross and fine motor functions showing 69% success rate and out of 58 children with Paraplegia of

  6. Crossing boundaries : improving communication in cerebral palsy care

    NARCIS (Netherlands)

    Gulmans-Weitenberg, Jitske

    2012-01-01

    The aim of the present thesis was to contribute to the improvement of patient care communication across the integrated care setting of children with cerebral palsy. Hereto, we followed two subsequent phases: 1) obtaining a better understanding of the experienced quality of patient care communication

  7. 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.

  8. 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.

  9. A case of Todd's Palsy following unilateral electroconvulsive therapy

    Science.gov (United States)

    Bell, Christine; Lepping, Peter; Clifford, John; Gardner-Thorpe, Catherine

    2012-01-01

    This case describes a woman undergoing unilateral electroconvulsive therapy (ECT) who developed a Todd's Palsy following the treatment, and which resolved when converted to bilateral ECT. We go on to hypothesize that this rare side effect may be an indication of the need to switch laterality during a course of ECT. PMID:22988330

  10. 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.

  11. 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.

  12. Transient Femoral Nerve Palsy Following Ilioinguinal Nerve Block ...

    African Journals Online (AJOL)

    2018-04-20

    Apr 20, 2018 ... a 3‑year period under ilioinguinal nerve block only were assessed for evidence of TFNP. All patients ... loss over the anterior aspect of the thigh, weakness of extension at the knee joint, .... and may result in falls with fractures which carry severe ... recovery of the palsy and subsequently discharged same.

  13. Contralateral reinnervation of midline muscles in nonidiopathic facial palsy.

    NARCIS (Netherlands)

    Gilhuis, H.J.; Beurskens, C.H.G.; Vries, J. de; Marres, H.A.M.; Hartman, E.H.M.; Zwarts, M.J.

    2003-01-01

    The purpose of this study was to analyze contralateral reinnervation of the facial nerve in eight patients with complete facial palsy after surgery or trauma and seven healthy volunteers. All patients had contralateral reinnervation of facial muscles as demonstrated by electrical nerve stimulation

  14. 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.

  15. Acoustic Predictors of Pediatric Dysarthria in Cerebral Palsy

    Science.gov (United States)

    Allison, Kristen M.; Hustad, Katherine C.

    2018-01-01

    Purpose: The objectives of this study were to identify acoustic characteristics of connected speech that differentiate children with dysarthria secondary to cerebral palsy (CP) from typically developing children and to identify acoustic measures that best detect dysarthria in children with CP. Method: Twenty 5-year-old children with dysarthria…

  16. Understanding Participation of Preschool-Age Children with Cerebral Palsy

    Science.gov (United States)

    Chiarello, Lisa Ann; Palisano, Robert J.; Orlin, Margo N.; Chang, Hui-Ju; Begnoche, Denise; An, Mihee

    2012-01-01

    Participation in home, school, and community activities is a primary outcome of early intervention services for children with disabilities and their families. The objectives of this study were to (a) describe participation of preschool-age children with cerebral palsy (CP); (b) determine effects of sex, age, and gross motor function on intensity…

  17. Altered sense of agency in children with spastic cerebral palsy

    DEFF Research Database (Denmark)

    Ritterband-Rosenbaum, Anina; Christensen, Mark S; Kliim-Due, Mette

    2011-01-01

    ABSTRACT: Background Children diagnosed with spastic Cerebral Palsy (CP) often show perceptual and cognitive problems, which may contribute to their functional deficit. Here we investigated if altered ability to determine whether an observed movement is performed by themselves (sense of agency...

  18. Functional Electrical Stimulation in Children and Adolescents with Cerebral Palsy

    Science.gov (United States)

    van der Linden, Marietta

    2012-01-01

    In this article, the author talks about functional electrical stimulation in children and adolescents with cerebral palsy. Functional electrical stimulation (FES) is defined as the electrical stimulation of muscles that have impaired motor control, in order to produce a contraction to obtain functionally useful movement. It was first proposed in…

  19. Predictors of Verbal Working Memory in Children with Cerebral Palsy

    Science.gov (United States)

    Peeters, Marieke; Verhoeven, Ludo; de Moor, Jan

    2009-01-01

    The goal of the present study was to examine the precursors of verbal working memory in 52 children with cerebral palsy with varying degrees of speech impairments in the first grade of special education. Following Baddeley's model of working memory, children's verbal working memory was measured by means of a forced-recognition task. As precursors…

  20. Robot-Assisted Task-Specific Training in Cerebral Palsy

    Science.gov (United States)

    Krebs, Hermano I.; Ladenheim, Barbara; Hippolyte, Christopher; Monterroso, Linda; Mast, Joelle

    2009-01-01

    Our goal was to examine the feasibility of applying therapeutic robotics to children and adults with severe to moderate impairment due to cerebral palsy (CP). Pilot results demonstrated significant gains for both groups. These results suggest that robot-mediated therapy may be an effective tool to ameliorate the debilitating effects of CP and…

  1. Incidence of inguinal hernia in children with congenital cerebral palsy

    DEFF Research Database (Denmark)

    Reimers, J I; Latocha, J E

    1990-01-01

    The incidence of inguinal hernia among 247 children with cerebral palsy was ascertained. During the first year of life, 20 of the 153 boys developed hernia, as did one of the 94 girls. Among boys with birthweights of 1000 to 2000g the incidence was 31 per cent, which is twice the rate for normal...

  2. Maternal Prepregnancy BMI and Risk of Cerebral Palsy in Offspring

    DEFF Research Database (Denmark)

    Forthun, Ingeborg; Wilcox, Allen J; Strandberg-Larsen, Katrine

    2016-01-01

    OBJECTIVES: To investigate the association between maternal pre-pregnancy BMI and risk of cerebral palsy (CP) in offspring. METHODS: The study population consisted of 188 788 children in the Mothers and Babies in Norway and Denmark CP study, using data from 2 population-based, prospective birth...

  3. Home Literacy Environment: Characteristics of Children with Cerebral Palsy

    Science.gov (United States)

    Peeters, Marieke; Verhoeven, Ludo; van Balkom, Hans; de Moor, Jan

    2009-01-01

    Background: Various aspects of the home literacy environment are considered to stimulate the emergent literacy development in children without disabilities. It is important to gain insight into the home literacy environment of children with cerebral palsy given that they have been shown to have difficulty acquiring literacy skills. Aims: The aims…

  4. Executive Functions in Youth With Spastic Cerebral Palsy

    NARCIS (Netherlands)

    Pirila, Silja; van der Meere, Jaap J.; Rantanen, Kati; Jokiluoma, Maria; Eriksson, Kai

    Dependent on criteria used, between 35% and 53% of the participants with cerebral palsy fulfilled the criteria of clinically relevant executive function problems as defined by Conners' (1994) Continuous Performance Test. Executive function problems were noticed mainly in participants with bilateral

  5. Predictors of participation of adolescents with cerebral palsy

    DEFF Research Database (Denmark)

    Dang, Van Mô; Colver, Allan; Dickinson, Heather O

    2014-01-01

    We investigated whether childhood factors that are amenable to intervention (parenting stress, child psychological problems and pain) predicted participation in daily activities and social roles of adolescents with cerebral palsy (CP). We randomly selected 1174 children aged 8-12 years from eight...

  6. Reproducibility of Tactile Assessments for Children with Unilateral Cerebral Palsy

    Science.gov (United States)

    Auld, Megan Louise; Ware, Robert S.; Boyd, Roslyn Nancy; Moseley, G. Lorimer; Johnston, Leanne Marie

    2012-01-01

    A systematic review identified tactile assessments used in children with cerebral palsy (CP), but their reproducibility is unknown. Sixteen children with unilateral CP and 31 typically developing children (TDC) were assessed 2-4 weeks apart. Test-retest percent agreements within one point for children with unilateral CP (and TDC) were…

  7. Predictors of verbal working memory in children with cerebral palsy.

    NARCIS (Netherlands)

    Peeters, M.; Verhoeven, L.; Moor, J.M.H. de

    2009-01-01

    The goal of the present study was to examine the precursors of verbal working memory in 52 children with cerebral palsy with varying degrees of speech impairments in the first grade of special education. Following Baddeley's model of working memory, children's verbal working memory was measured by

  8. Predictors of verbal working memory in children with cerebral palsy

    NARCIS (Netherlands)

    Peeters, M.H.J.; Verhoeven, L.T.W.; Moor, J.M.H. de

    2009-01-01

    The goal of the present study was to examine the precursors of verbal working memory in 52 children with cerebral palsy with varying degrees of speech impairments in the first grade of special education. Following Baddeley's model of working memory, children's verbal working memory was measured by

  9. Physical activity in young children with cerebral palsy

    NARCIS (Netherlands)

    Zwier, J.N.; van Schie, P.E.M.; Becher, J.G.S.J.S.; Smits, D.W.; Gorter, J.W.; Dallmeijer, A.J.

    2010-01-01

    Purpose. The aim of this study was to describe the physical activity levels of 5- and 7-year-old children with cerebral palsy (CP, n=97), to compare their physical activity levels with those of typically developing peers (TD, n=57) and the Dutch recommendation for physical activity, and to

  10. Language and motor speech skills in children with cerebral palsy

    NARCIS (Netherlands)

    Pirila, Sija; van der Meere, Jaap; Pentikainen, Taina; Ruusu-Niemi, Pirjo; Korpela, Raija; Kilpinen, Jenni; Nieminen, Pirkko; Ruusu-Niemin, P; Kilpinen, R

    2007-01-01

    The aim of the study was to investigate associations between the severity of motor limitations, cognitive difficulties, language and motor speech problems in children with cerebral palsy. Also, the predictive power of neonatal cranial ultrasound findings on later outcome was investigated. For this

  11. Sciatic nerve palsy associated with intramuscular quinine injections ...

    African Journals Online (AJOL)

    Sct?ior ikfeclical O[ficcr. Department of Orthopaeclics, Mulago Hospital, Makerere University, Kampala, Uganda. Key Words: Sciatic nerve palsy, intramuscular injections, children, quinine dil~ydrochloride. The purpose of this paper is to show that, in children, gluteal injection of quinine dihydrochloride (QDH) may result in ...

  12. Intermittent versus Continuous Physiotherapy in Children with Cerebral Palsy

    Science.gov (United States)

    Christiansen, Annette Sandahl; Lange, Christa

    2008-01-01

    The aim of this study was to compare the effect of the delivery of the same amount of intermittent versus continuous physiotherapy given to children with cerebral palsy (CP). This was organized either in an intermittent regime four times a week for 4 weeks alternating with a 6-week treatment pause, or a continuous once or twice a week regime, both…

  13. Facial nerve palsy as a primary presentation of advanced carcinoma ...

    African Journals Online (AJOL)

    Introduction: Cranial nerve neuropathy is a rare presentation of advanced cancer of the prostate. Observation: We report a case of 65-year-old man who presented with right lower motor neuron (LMN) facial nerve palsy. The prostate had malignant features on digital rectal examination (DRE) and the prostate specific antigen ...

  14. Aerobic Capacity in Children and Adolescents with Cerebral Palsy

    Science.gov (United States)

    Verschuren, Olaf; Takken, Tim

    2010-01-01

    This study described the aerobic capacity [VO[subscript 2peak] (ml/kg/min)] in contemporary children and adolescents with cerebral palsy (CP) using a maximal exercise test protocol. Twenty-four children and adolescents with CP classified at Gross Motor Functional Classification Scale (GMFCS) level I or level II and 336 typically developing…

  15. Aerobic capacity in children and adolescents with cerebral palsy

    NARCIS (Netherlands)

    Verschuren, Olaf; Takken, Tim

    2010-01-01

    This study described the aerobic capacity [VO(2peak) (ml/kg/min)] in contemporary children and adolescents with cerebral palsy (CP) using a maximal exercise test protocol. Twenty-four children and adolescents with CP classified at Gross Motor Functional Classification Scale (GMFCS) level I or level

  16. surgery of the hand in infants with cerebral palsy* 655

    African Journals Online (AJOL)

    1971-06-19

    Jun 19, 1971 ... cerebral palsy is by re-education-physical, occupational, speech and drug therapy."· Orthopaedic surgery is usually considered as an adjunct to therapy'" and is indicated only for its value ... than long-continued postural training and stretching.",13 ... In each child in this series, the hand on the affected side.

  17. Parental adaptation in families of young children with cerebral palsy

    NARCIS (Netherlands)

    Rentinck, I.C.M.

    2009-01-01

    Background For most parents, the birth of their child is a unique and touching moment. However, in some families a child is born with a physical disability. Among the large variety of childhood developmental disabilities, cerebral palsy (CP) is considered to be the major physical disability

  18. Cerebral Palsy Symptoms in Children Decreased Following Massage Therapy

    Science.gov (United States)

    Hernandez-Reif, Maria; Field, Tiffany; Largie, Shay; Diego, Miguel; Manigat, Natasha; Seoanes, Jacqueline; Bornstein, Joan

    2005-01-01

    Twenty young children (mean age = 32 months) with cerebral palsy (CP) recruited from early intervention programs received 30 minutes of massage or reading twice weekly for 12 weeks. The children receiving massage therapy showed fewer physical symptoms including reduced spasticity, less rigid muscle tone overall and in the arms, and improved fine…

  19. Socio-clinical issues in cerebral palsy in Sagamu, Nigeria

    African Journals Online (AJOL)

    Nigeria, provides specialist paediatric neurological care to at least three states of the ... parental social indices, clinical diagnoses and the frequencies of clinic .... palsy and oral motor dysfunction.16 Special feeding devices may therefore be .... Knowledge, attitude and practice of community health workers in. Nigeria.

  20. Home literacy environment: characteristics of children with cerebral palsy

    NARCIS (Netherlands)

    Peeters, M.H.J.; Verhoeven, L.T.W.; Balkom, L.J.M. van; Moor, J.M.H. de

    2009-01-01

    Background: Various aspects of the home literacy environment are considered to stimulate the emergent literacy development in children without disabilities. It is important to gain insight into the home literacy environment of children with cerebral palsy given that they have been shown to have