WorldWideScience

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

    Directory of Open Access Journals (Sweden)

    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. Adrenomyeloneuropathy with bulbar palsy: A rare association

    Directory of Open Access Journals (Sweden)

    Vishal Annaji Chafale

    2014-01-01

    Full Text Available Adrenomyeloneuropathy (AMN is a variant of adrenoleukodystrophy (ALD, an X-linked recessive peroxisomal disorder associated with accumulation of very long chain fatty acids (VLCFA. Mutations of this gene lead to abnormal peroxisomal β-oxidation, which results in the harmful accumulation of VLCFAs in affected cells. Neurological symptoms occur due to progressive demyelination and destruction of cerebral white matter and primary adrenal insufficiency. Bulbar palsy in a case of AMN is very unusual. We report a case of a 22-year-old male with AMN who developed adrenal insufficiency at the age of 4 years successfully treated by gluco- and mineralocorticoids followed by features of myeloneuropathy with bulbar palsy. AMN with prominent bulbar symptoms emphasizes the diverse clinical manifestation of this disease.

  3. Acute bulbar palsy plus syndrome: A rare variant of Guillain-Barre syndrome.

    Science.gov (United States)

    Ray, Sanghamitra; Jain, Prakash Chand

    2016-01-01

    Guillain-Barre syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide both in adult and pediatric population. Although flaccid paralysis is the hallmark of this disease, there are some rare variants which may be easily missed unless suspected. Here, we present a very rare variant of GBS - acute bulbar palsy plus syndrome in a pediatric patient. A 13-year-old female child presented with right-sided lower motor neuron type of facial palsy and palsy of bilateral glossopharyngeal and vagus nerve of 2 weeks duration. On detailed neurological examination, motor and sensory system were normal, but the deep tendon reflexes were absent universally. Nerve conduction study showed demyelinating motor neuropathy. Based on typical clinical course and electrophysiological studies, the diagnosis was made. To the best of our knowledge, this is the first pediatric case of unilateral facial palsy with bulbar involvement without any motor abnormality.

  4. Acute bulbar palsy plus syndrome: A rare variant of Guillain–Barre syndrome

    OpenAIRE

    Sanghamitra Ray; Prakash Chand Jain

    2016-01-01

    Guillain?Barre syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide both in adult and pediatric population. Although flaccid paralysis is the hallmark of this disease, there are some rare variants which may be easily missed unless suspected. Here, we present a very rare variant of GBS - acute bulbar palsy plus syndrome in a pediatric patient. A 13-year-old female child presented with right-sided lower motor neuron type of facial palsy and palsy of bilateral glossophary...

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

    Directory of Open Access Journals (Sweden)

    Pinank R. Mer

    2015-12-01

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

  6. A case of bulbar type cerebral palsy: representative symptoms of dorsal brainstem syndrome.

    Science.gov (United States)

    Hiyane, Masato; Saito, Yoshiaki; Saito, Takashi; Komaki, Hirofumi; Nakagawa, Eiji; Sugai, Kenji; Sasaki, Masayuki; Sato, Noriko; Yamamoto, Toshiyuki; Imai, Yoko

    2012-10-01

    In this study, we present the case of a 2-year-old boy who exhibited facial and bulbar paralysis since birth, severe dysphagia, signs of oculomotor disturbance, jaw jerks, pyramidal signs on both toes, intellectual disability, and severe gastroesophageal reflux. His blink reflex and auditory/somatosensory evoked potentials suggested abnormalities in the lower brainstem, and magnetic resonance imaging showed a T2 hyperintense area in the pontine tegmentum. These findings combined with the patient's symptoms suggested "dorsal brainstem syndrome" and indicated a possibility of prenatal asphyxia in this patient. Nosologic issues regarding this subgroup of cerebral palsy are discussed here. Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  7. Mitochondrial respiratory chain disease presenting as progressive bulbar paralysis of childhood

    NARCIS (Netherlands)

    Roeleveld-Versteegh, ABC; Braun, KPJ; Smeitink, JAM; Dorland, L; de Koning, TJ

    2004-01-01

    We report two siblings with a mitochondrial respiratory chain defect who presented with progressive bulbar paralysis of childhood ( Fazio-Londe disease). Mitochondrial respiratory chain defects should be considered in the differential diagnosis of this rare clinical entity.

  8. Mitochondrial respiratory chain disease presenting as progressive bulbar paralysis of childhood.

    NARCIS (Netherlands)

    Roeleveld-Versteegh, A.B.; Braun, K.P.; Smeitink, J.A.M.; Dorland, L.; Koning, T.J.

    2004-01-01

    We report two siblings with a mitochondrial respiratory chain defect who presented with progressive bulbar paralysis of childhood (Fazio-Londe disease). Mitochondrial respiratory chain defects should be considered in differential diagnosis of this rare clinical entity.

  9. Clinical Features of Isolated Bulbar Palsy of Amyotrophic Lateral Sclerosis in Chinese Population

    Directory of Open Access Journals (Sweden)

    Hua-Gang Zhang

    2017-01-01

    Conclusions: IBP was evidently different from PBP, which was characterized with the predominance of female, pure LMN bulbar signs, an older onset age, a relative preservation of respiratory function, and a better prognosis.

  10. Genetics Home Reference: progressive supranuclear palsy

    Science.gov (United States)

    ... dysphagia). Most affected individuals also experience changes in personality and behavior, such as a general loss of ... Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol. 2009 Mar;8( ...

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

    Science.gov (United States)

    ... Health Conditions HGPPS Horizontal gaze palsy with progressive scoliosis Printable PDF Open All Close All Enable Javascript ... collapse boxes. Description Horizontal gaze palsy with progressive scoliosis ( HGPPS ) is a disorder that affects vision and ...

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

    African Journals Online (AJOL)

    in our hospital revealed a fully conscious elderly man with bradykinesia, decreased fine motor skills, and hypophonia. His gait/posture was characterized by flexion at the waist, not swinging while walking and retrocollis. His face was blank and. Progressive Supranuclear Palsy Misdiagnosed as. Parkinson's Disease: A Case ...

  13. Slow saccades in bulbar-onset motor neurone disease.

    Science.gov (United States)

    Donaghy, Colette; Pinnock, Ralph; Abrahams, Sharon; Cardwell, Chris; Hardiman, Orla; Patterson, Victor; McGivern, R Canice; Gibson, J Mark

    2010-07-01

    Historical studies of eye movements in motor neurone disease (MND) have been conflicting although current findings suggest that eye movement abnormalities relate to frontal lobe impairment. Numerous case reports, however, describe slow saccades and supranuclear gaze palsies in patients with MND often associated with bulbar-onset disease. We performed a study of saccades and smooth pursuit in a large group of patients with MND to examine for any differences between bulbar-onset and spinal-onset patients. Forty-four patients (14 bulbar-onset and 30 spinal-onset patients) and 45 controls were recruited. Reflexive saccades, antisaccades and smooth pursuit were examined using infra-red oculography and all subjects then underwent neuropsychological evaluation. Reflexive saccades were found to be slower in bulbar-onset compared to spinal-onset patients and controls (p = 0.03, p = 0.05). Antisaccade latency (p = 0.01) and antisaccade type 1 errors (p = 0.03, p = 0.04) were increased in patients compared to controls. 'Proportion of time spent in smooth pursuit' and smooth pursuit 'velocity gain' were reduced in patients compared to controls (p = 0.000, p = 0.001). Antisaccade errors and velocity gain correlated with neuropsychological measures sensitive to lesions of the frontal lobes. This is the first study to highlight the presence of slow saccades in bulbar-onset MND. These findings suggest that slow saccades may be due to increased brainstem pathology in bulbar-onset disease that involves burst cell neurons. Furthermore these observations highlight the potential for overlap between bulbar-onset MND and progressive supranuclear palsy (PSP) as both can have a bulbar palsy and slowed saccades.

  14. Hummingbird sign in progressive supranuclear palsy disease

    Directory of Open Access Journals (Sweden)

    Sanjay Pandey

    2012-01-01

    Full Text Available Progressive supranuclear palsy (PSP is characterized by slowness, rigidity, bradykinesia, repeated falls, downgaze limitation and dementia. Midbrain atrophy on magnetic resonance imaging is highly suggestive of PSP and is described as "hummingbird sign". This sign is very helpful in differentiating PSP patients from those with Parkinson′s disease. We hereby report a 72-year-old female case of PSP primarily diagnosed with Parkinson′s disease.

  15. A rapidly progressive defective spermatogenesis in a Mexican family affected by spino-bulbar muscular atrophy.

    Science.gov (United States)

    Piña-Aguilar, Raul Eduardo; Regalado-Hernández, Miguel Ángel; Moreno-García, Jesús Daniel; Buentello-Volante, Beatriz; Chacón-Camacho, Oscar Francisco; Gallegos-Rivas, Mayra Celina; Kazakova, Ekaterina; Santillán-Hernández, Yuritzi; Zenteno, Juan Carlos

    2016-01-01

    Spino-bulbar muscular atrophy (SBMA) is an X-linked recessive adult progressive disorder affecting motor neurons. It is caused by a poly-glutamine tract expansion in the androgen receptor (AR) which generates protein aggregates that cannot be processed by proteasomes. A secondary mild androgen resistance is developed by AR dysfunction and patients present endocrine abnormalities including gynecomastia and poor function of testosterone in tissues; however, normally they are fertile. In this report we describe a Mexican family with three affected brothers with primary infertility caused by a progressive impairment of spermatogenesis leading to azoospermia before 40 years of age. They presented common features associated to patients affected by SMBA, such as gynecomastia, high level of CPK, muscle cramps, fasciculations, muscle wastage, and impaired swallowing. Two intracytoplasmic sperm injection (ICSI) cycles were performed in one of the patients resulting in fertilization failure. Molecular analysis of AR gene exon 1 revealed 54 CAG repeats in DNA extracted from leukocytes in affected patients and 22 repeats in the fertile non-affected brother. Severe impaired spermatogenesis of rapid progression has not been associated before to SBMA. This is the first report of assisted reproduction techniques indicated by male infertility in patients with this rare disorder. Further studies are required to confirm the unusual result of intracytoplasmic sperm injection cycles. We discuss the implications and possible pathogenesis of these unique features of SBMA in this family.

  16. Regional Cerebral Perfusion in Progressive Supranuclear Palsy

    Energy Technology Data Exchange (ETDEWEB)

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

    1996-03-15

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

  17. A case of atypical progressive supranuclear palsy

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  19. Frontal deficits differentiate progressive supranuclear palsy from Parkinson's disease.

    Science.gov (United States)

    Lee, Young-Eun C; Williams, David R; Anderson, Jacqueline F I

    2016-03-01

    The clinical differentiation of progressive supranuclear palsy from Parkinson's disease can be challenging, due to overlapping clinical features and a lack of diagnostic markers. Abnormalities in cognitive function form part of the clinical spectrums of these diseases and distinctive cognitive profiles may be helpful in differentiating these diseases in the diagnostic period. A comprehensive neuropsychological test battery was administered to 12 patients with clinically diagnosed progressive supranuclear palsy and 12 patients with Parkinson's disease matched for age and disease duration. Effect size (Cohen's d) was calculated for cognitive tests that were significantly different between groups. Patients with progressive supranuclear palsy performed significantly worse than those with Parkinson's disease on measures of processing speed, verbal fluency, planning, verbal abstract reasoning, verbal memory, and made more perseverative responses on a set shifting task. Measures of executive function, manual dexterity and processing speed were most diagnostically useful (Cohen's d > 2.0) in differentiating between progressive supranuclear palsy and Parkinson's disease. These findings suggest that more severe and prominent 'frontal' cognitive deficits in patients with progressive parkinsonism would be helpful in predicting progressive supranuclear palsy rather than Parkinson's disease and these findings may contribute to the development of diagnostic criteria. © 2014 The British Psychological Society.

  20. ALS Regional Variants (Brachial Amyotrophic Diplpegia, Leg Amyotrophic Diplegia, and Isolated Bulbar ALS)

    Science.gov (United States)

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

    2015-01-01

    Synopsis Amyotrophic lateral sclerosis is a rapidly progressive, invariably fatal disease, comprised of mixed upper and lower motor neuron involvement in different spinal cord regions. Characteristic initial presentations have implications for prognosis. Bulbar onset patients progress more rapidly than limb-onset patients, or patients with a pure lower motor neuron presentation. Other regional variants have been described where disease is restricted to one spinal region at presentation, including a flail arm or flail leg, and restricted respiratory or bulbar disease. More recent descriptions of regional variants suggest some ALS patients have disease isolated to a single spinal region for many years, including: brachial amyotrophic diplegia; leg amyotrophic diplegia; and isolated bulbar palsy. More clearly defining regional variants will have implications for prognosis, but also for understanding the pathophysiology of ALS, for identifying genetic factors related to slower disease progression, and for future clinical trial planning. PMID:26515621

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

    Science.gov (United States)

    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.

  2. Disease Severity and Progression in Progressive Supranuclear Palsy and Multiple System Atrophy: Validation of the NNIPPS – PARKINSON PLUS SCALE

    Science.gov (United States)

    Payan, Christine A. M.; Viallet, François; Landwehrmeyer, Bernhard G.; Bonnet, Anne-Marie; Borg, Michel; Durif, Franck; Lacomblez, Lucette; Bloch, Frédéric; Verny, Marc; Fermanian, Jacques; Agid, Yves; Ludolph, Albert C.

    2011-01-01

    Background The Natural History and Neuroprotection in Parkinson Plus Syndromes (NNIPPS) study was a large phase III randomized placebo-controlled trial of riluzole in Progressive Supranuclear Palsy (PSP, n = 362) and Multiple System Atrophy (MSA, n = 398). To assess disease severity and progression, we constructed and validated a new clinical rating scale as an ancillary study. Methods and Findings Patients were assessed at entry and 6-montly for up to 3 years. Evaluation of the scale's psychometric properties included reliability (n = 116), validity (n = 760), and responsiveness (n = 642). Among the 85 items of the initial scale, factor analysis revealed 83 items contributing to 15 clinically relevant dimensions, including Activity of daily Living/Mobility, Axial bradykinesia, Limb bradykinesia, Rigidity, Oculomotor, Cerebellar, Bulbar/Pseudo-bulbar, Mental, Orthostatic, Urinary, Limb dystonia, Axial dystonia, Pyramidal, Myoclonus and Tremor. All but the Pyramidal dimension demonstrated good internal consistency (Cronbach α≥0.70). Inter-rater reliability was high for the total score (Intra-class coefficient = 0.94) and 9 dimensions (Intra-class coefficient = 0.80–0.93), and moderate (Intra-class coefficient = 0.54–0.77) for 6. Correlations of the total score with other clinical measures of severity were good (rho≥0.70). The total score was significantly and linearly related to survival (p<0.0001). Responsiveness expressed as the Standardized Response Mean was high for the total score slope of change (SRM = 1.10), though higher in PSP (SRM = 1.25) than in MSA (SRM = 1.0), indicating a more rapid progression of PSP. The slope of change was constant with increasing disease severity demonstrating good linearity of the scale throughout disease stages. Although MSA and PSP differed quantitatively on the total score at entry and on rate of progression, the relative contribution of clinical dimensions to overall

  3. Disease severity and progression in progressive supranuclear palsy and multiple system atrophy: validation of the NNIPPS--Parkinson Plus Scale.

    Directory of Open Access Journals (Sweden)

    Christine A M Payan

    Full Text Available The Natural History and Neuroprotection in Parkinson Plus Syndromes (NNIPPS study was a large phase III randomized placebo-controlled trial of riluzole in Progressive Supranuclear Palsy (PSP, n = 362 and Multiple System Atrophy (MSA, n = 398. To assess disease severity and progression, we constructed and validated a new clinical rating scale as an ancillary study.Patients were assessed at entry and 6-montly for up to 3 years. Evaluation of the scale's psychometric properties included reliability (n = 116, validity (n = 760, and responsiveness (n = 642. Among the 85 items of the initial scale, factor analysis revealed 83 items contributing to 15 clinically relevant dimensions, including Activity of daily Living/Mobility, Axial bradykinesia, Limb bradykinesia, Rigidity, Oculomotor, Cerebellar, Bulbar/Pseudo-bulbar, Mental, Orthostatic, Urinary, Limb dystonia, Axial dystonia, Pyramidal, Myoclonus and Tremor. All but the Pyramidal dimension demonstrated good internal consistency (Cronbach α ≥ 0.70. Inter-rater reliability was high for the total score (Intra-class coefficient = 0.94 and 9 dimensions (Intra-class coefficient = 0.80-0.93, and moderate (Intra-class coefficient = 0.54-0.77 for 6. Correlations of the total score with other clinical measures of severity were good (rho ≥ 0.70. The total score was significantly and linearly related to survival (p<0.0001. Responsiveness expressed as the Standardized Response Mean was high for the total score slope of change (SRM = 1.10, though higher in PSP (SRM = 1.25 than in MSA (SRM = 1.0, indicating a more rapid progression of PSP. The slope of change was constant with increasing disease severity demonstrating good linearity of the scale throughout disease stages. Although MSA and PSP differed quantitatively on the total score at entry and on rate of progression, the relative contribution of clinical dimensions to overall severity and progression was similar.The NNIPPS-PPS has suitable validity, is

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2009-02-15

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

  5. Clinical neurorestorative progresses in cerebral palsy

    Directory of Open Access Journals (Sweden)

    Sharma A

    2017-02-01

    Full Text Available Alok Sharma,1 Tongchao Geng,2 Hemangi Sane,3 Pooja Kulkarni3 1Department of Medical Services & Clinical Research, NeuroGen Brain and Spine Institute, Mumbai, India; 2Department of Neurology, Yuquan Hospital, Tsinghua University, Beijing, People’s Republic of China; 3Department of Research and Development, Neurogen Brain and Spine Institute, Mumbai, India Abstract: Cerebral palsy (CP, group of permanent nonprogressive clinical disorders in children, is caused by damage to the immature brain. Conventionally available treatments for CP are mainly targeted toward management of its symptoms. With the upcoming field of neurorestorative strategies, we are now able to repair the core brain damage in CP. There are various drugs, stem cells, etc, which have been implicated to have neurorestorative properties. Autologous bone marrow stem cells, umbilical cord stem cells, neural stem cells, and olfactory ensheathing cells have shown the safety and efficacy in preliminary studies. Here, we review the different medicines and cell types that have shown beneficial effects in clinical studies. We propose that combination strategies may be the future of neurorestoration. Keywords: stem cell therapy, bone marrow, umbilical cord, neural cells, regenerative medicine, brain

  6. Critical appraisal of the role of davunetide in the treatment of progressive supranuclear palsy

    Directory of Open Access Journals (Sweden)

    Gozes I

    2012-02-01

    Full Text Available Michael Gold1, Stefan Lorenzl2, Alistair J Stewart1, Bruce H Morimoto1, David R Williams3, Illana Gozes1,41Allon Therapeutics Inc, Vancouver, BC, Canada; 2Interdisciplinary Center for Palliative Medicine, Munich University Hospital Klinikum Grosshadern, Munich, Germany; 3Van Cleef/Roet Centre for Nervous Diseases, Monash University, Melbourne, Australia; 4The Dr Diana and Zelman Elton (Elbaum Laboratory for Molecular Neuroendocrinology, The Lily and Avraham Gildor Chair for the Investigation of Growth Factors, The Adams Super Center for Brain Studies, and the Department of Human Molecular Genetics and Biochemistry, School of Neuroscience, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelAbstract: Progressive supranuclear palsy (PSP is a rare neurodegenerative disease characterized by the accumulation of tau protein aggregates in the basal ganglia, brainstem and cerebral cortex leading to rapid disease progression and death. The neurofibrillary tangles that define the neuropathology of PSP are comprised of aggregated 4R tau and show a well-defined distribution. Classically, PSP is diagnosed by symptoms that include progressive gait disturbance, early falls, vertical ophthalmoparesis, akinetic-rigid features, prominent bulbar dysfunction and fronto-subcortical dementia. There are currently no effective therapies for the treatment of this rapidly degenerating and debilitating disease. Davunetide is a novel neuroprotective peptide that is thought to impact neuronal integrity and cell survival through the stabilization of microtubules. Preclinical activity in models of tauopathy has been translated to clinical studies, demonstrating pharmacologic activity that has supported further development. Davunetide's efficacy and tolerability are being tested in a placebo-controlled study in PSP patients, making it the most advanced drug candidate in this indication. This review examines the disease characteristics of PSP, the rationale for

  7. The "hummingbird" lady: A case report on progressive supranuclear palsy

    Directory of Open Access Journals (Sweden)

    Mosam V Phirke

    2015-01-01

    Full Text Available Progressive supranuclear palsy (PSP is a rare neurodegenerative disease, which tends to be progressive, causing weakness of the structures in the brain stem that controls eye movements. PSP falls under the rubric of Parkinsonism plus syndromes that are a group of heterogeneous degenerative neurological disorders that differ from the classical idiopathic Parkinson′s disease. PSP is often underreported, making it important for clinicians to be aware of this disorder. Here we report a case of PSP, which presented primarily with speech disturbances and recurrent falls due to postural instability. On investigating, the magnetic resonance imaging (MRI revealed a classical sign diagnostic of PSP.

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

    Science.gov (United States)

    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.

  9. Communication impairments in people with progressive supranuclear palsy: A tutorial.

    Science.gov (United States)

    Kim, Jae-Hyun; McCann, Clare M

    2015-01-01

    Progressive supranuclear palsy (PSP) is a progressive neurological condition, whose main features include supranuclear gaze palsy, frequent falls, bradykinesia, axial rigidity, cognitive decline and communication impairments. Even though communication impairments are early and prominent manifestations, there is a significant lack of research on the nature of these impairments in PSP and the role of speech-language pathologists (SLPs). This tutorial article aims to describe the communication impairments observed in people with PSP; provide clinical guidelines for SLPs when assessing motor speech, language and other communication impairments; and to present facilitation and compensation approaches to treatment for people with PSP. The predominant motor speech impairment is mixed dysarthria, but there is a lack of consensus about the classification of language impairments. The involvement of SLPs in the assessment and treatment of people with PSP should be early, on-going and in collaboration with other health professionals, with the primary focus of maintaining quality of life for these patients and their family members. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Visual-vestibular interaction in progressive supranuclear palsy.

    Science.gov (United States)

    Das, V E; Leigh, R J

    2000-01-01

    We measured the stability of gaze during horizontal head rotations at 1-3 Hz in four patients with progressive supranuclear palsy (PSP), while they viewed a stationary target. Median gain of compensatory eye movements was 0.94, similar to control subjects. During rotation in darkness, median gain of vestibulo-ocular reflex (VOR) was 0.88, similar to controls. Conversely, the median gain of smooth-pursuit eye movements at 1.0 Hz was 0.23, lower than controls. A simple superposition model of smooth pursuit and the VOR could not account for the observed gaze stability during fixation. Our results are further evidence that a visually mediated mechanism, independent of smooth pursuit, optimizes eye movements to compensate for head rotations.

  11. Apparent diffusion coefficient measurements in progressive supranuclear palsy

    Energy Technology Data Exchange (ETDEWEB)

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

    2000-09-01

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

  12. Deterioration of horizontal saccades in progressive supranuclear palsy.

    Science.gov (United States)

    Terao, Yasuo; Fukuda, Hideki; Shirota, Yuichiro; Yugeta, Akihiro; Yoshioka, Masayuki; Suzuki, Masahiko; Hanajima, Ritsuko; Nomura, Yoshiko; Segawa, Masaya; Tsuji, Shoji; Ugawa, Yoshikazu

    2013-02-01

    To investigate horizontal saccade changes according to disease stage in patients with progressive supranuclear palsy (PSP). We studied visually and memory guided saccades (VGS and MGS) in 36 PSP patients at various disease stages, and compared results with those in 66 Parkinson's disease (PD) patients and 58 age-matched normal controls. Both vertical and horizontal saccades were affected in PSP patients, usually manifesting as "slow saccades" but sometimes as a sequence of small amplitude saccades with relatively well preserved velocities. Disease progression caused saccade amplitude reduction in PSP but not PD patients. In contrast, VGS and MGS latencies were comparable between PSP and PD patients, as were the frequencies of saccades to cue, suggesting that voluntary initiation and inhibitory control of saccades are similar in both disorders. Hypermetria was rarely observed in PSP patients with cerebellar ataxia (PSPc patients). The progressively reduced accuracy of horizontal saccades in PSP suggests a brainstem oculomotor pathology that includes the superior colliculus and/or paramedian pontine reticular formation. In contrast, the functioning of the oculomotor system above the brainstem was similar between PSP and PD patients. These findings may reflect a brainstem oculomotor pathology. Copyright © 2012 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  13. 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

  14. Pulmonary embolism: ′the great masquerader′ of pneumonia in a patient with progressive supranuclear palsy

    Directory of Open Access Journals (Sweden)

    Robin G Manappallil

    2016-01-01

    Full Text Available Patients with Parkinson′s disease are at risk of developing aspiration pneumonia. Pulmonary embolism is a rare but life-threatening complication in such patients, but could the same be true in progressive supranuclear palsy, an atypical form of Parkinsonism? This case report aims at highlighting the development of unprovoked pulmonary embolism in a patient with progressive supranuclear palsy and also describes how pulmonary embolism can mimic pneumonia in such patients.

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

    Science.gov (United States)

    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 (pParkinson'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.

  16. Meaning in life in patients with progressive supranuclear palsy.

    Science.gov (United States)

    Fegg, Martin Johannes; Kögler, Monika; Abright, Carina; Hensler, Mira; Lorenzl, Stefan

    2014-08-01

    This exploratory study investigated meaning in life (MiL) in patients with progressive supranuclear palsy (PSP). In the "Schedule for Meaning in Life Evaluation" (SMiLE), respondents list individual MiL areas before rating their current satisfaction and importance with them (index of weighting [IoW], index of satisfaction [IoS], and index of weighted satisfaction [IoWS], range 0-100). A total of 38 patients with PSP completed the SMiLE (IoS: 68.6 ± 25.6, IoW: 79.6 ± 12.6, and IoWS: 69.2 ± 26.1). A representative sample of healthy participants (n = 977) scored significantly higher in the IoS (82.8 ± 14.7, P < .001), the IoW (85.6 ± 12.3, P = .006), and the IoWS (83.3 ± 14.8, P < .001). Compared to healthy individuals, patients with PSP were less likely to list health (P = .001) and more likely to list partner (P = .04), leisure (P = .01), home/garden (P = .01), and pleasure (P = .02). Patients with PSP seem to focus on supportive relationships and leisure, while the decreasing health status is becoming less important to them. © The Author(s) 2013.

  17. Tracking and predicting disease progression in progressive supranuclear palsy: CSF and blood biomarkers.

    Science.gov (United States)

    Jabbari, Edwin; Zetterberg, Henrik; Morris, Huw R

    2017-10-01

    Progressive supranuclear palsy (PSP) is a rare and progressive neurodegenerative condition characterised pathologically by neuronal cell loss due to abnormal tau deposits. Clinically, the condition manifests as parkinsonism with the addition of progressive balance, speech, swallowing, eye movement and cognitive impairment, ultimately leading to death. Measuring change over time in neurodegenerative conditions is central to defining the effects of therapeutic intervention and disease biology. The current gold standard for measuring clinical disease progression in PSP is the PSP Rating Scale score. However, such scales may be affected by intrarater and inter-rater variability. In addition, their use in clinical trials may be hindered by differences in the time interval between pathological disease progression/response to therapeutics and change in clinical state. Therefore, the need for reliable disease progression biomarkers to complement clinical rating scales is clear. Here we discuss the benefits of using biomarkers to predict and track disease progression in both clinical and research settings. Through reviewing the literature to date on the role of cerebrospinal fluid (CSF) and blood biomarkers, we highlight data that reveals the ability of CSF and plasma neurofilament light chain (NF-L) to predict and track clinical disease progression in PSP. We also discuss the need for large-scale longitudinal studies to identify novel biomarkers. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2008-11-15

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

  19. Characteristics of Nonmotor Symptoms in Progressive Supranuclear Palsy

    Directory of Open Access Journals (Sweden)

    Ruwei Ou

    2016-01-01

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

  20. Cerebellar ataxia in progressive supranuclear palsy: An autopsy study of PSP-C.

    Science.gov (United States)

    Koga, Shunsuke; Josephs, Keith A; Ogaki, Kotaro; Labbé, Catherine; Uitti, Ryan J; Graff-Radford, Neill; van Gerpen, Jay A; Cheshire, William P; Aoki, Naoya; Rademakers, Rosa; Wszolek, Zbigniew K; Ross, Owen A; Dickson, Dennis W

    2016-05-01

    Cerebellar ataxia is an exclusion criterion for the clinical diagnosis of progressive supranuclear palsy, but a variant with predominant cerebellar ataxia has been reported. The aims of this study were to estimate the frequency of progressive supranuclear palsy with predominant cerebellar ataxia in an autopsy series from the United States and to compare clinical, pathologic, and genetic differences between progressive supranuclear palsy with and without predominant cerebellar ataxia. We selected 100 consecutive patients with pathologically confirmed progressive supranuclear palsy who had been evaluated at the Mayo Clinic (referred to as the Mayo Clinic patient series) from our brain bank database (N = 1085). We next enriched in cases likely to have cerebellar ataxia by searching the remaining 985 cases for (1) an antemortem diagnosis of multiple system atrophy or (2) neuropathologic evidence of prominent degeneration of the cerebellum or cerebellar afferent nuclei. Subsequently, clinical, pathologic, and genetic features were compared between the two groups. One patient in the Mayo Clinic patient series (1%) met criteria for progressive supranuclear palsy with predominant cerebellar ataxia and had both cerebellar and mild midbrain atrophy on MRI. Four patients were identified with the targeted search. Four of the five patients were clinically misdiagnosed as multiple system atrophy. The severity of tau-related pathology and cerebellar degeneration were not different between the two groups. No differences were detected in tau genotypes. Although our data cannot provide definitive information about how to make an accurate clinical diagnosis, they should serve to raise awareness of progressive supranuclear palsy with predominant cerebellar ataxia in the differential diagnosis of multiple system atrophy. © 2016 Movement Disorder Society. © 2016 International Parkinson and Movement Disorder Society.

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

    Directory of Open Access Journals (Sweden)

    Stefano Zanigni

    2016-01-01

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

  2. Conventional magnetic resonance imaging in confirmed progressive supranuclear palsy and multiple system atrophy

    NARCIS (Netherlands)

    Massey, L.A.; Micallef, C.; Paviour, D.C.; O'Sullivan, S.S.; Ling, H.; Williams, D.R.; Kallis, C.; Holton, J.L.; Revesz, T.; Burn, D.J.; Yousry, T.; Lees, A.J.; Fox, N.C.; Jäger, H.R.

    2012-01-01

    Conventional magnetic resonance imaging (cMRI) is often used to aid the diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), but its ability to predict the histopathological diagnosis has not been systematically studied. cMRI from 48 neuropathologically confirmed

  3. Horizontal Gaze Palsy and Progressive Scoliosis With ROBO 3 Mutations in Patients From Cape Verde.

    Science.gov (United States)

    Mendes Marques, Nadine B P S; Barros, Sandra R; Miranda, Ana F; Nobre Cardoso, João; Parreira, Sónia; Fonseca, Teresa; Donaire, Nelvia M; Campos, Nuno

    2017-06-01

    Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare and autosomal recessive syndrome. We describe 2 cases of HGPPS which are the first documented in patients of African ancestry from an isolated population in Cape Verde. They demonstrated typical findings on neuro-ophthalmic examination and brain magnetic resonance imaging. One patient had novel heterozymous mutations of the ROB0 3 gene.

  4. Raven's Coloured Progressive Matrices as a Measure of Cognitive Functioning in Cerebral Palsy

    Science.gov (United States)

    Pueyo, R.; Junque, C.; Vendrell, P.; Narberhaus, A.; Segarra, D.

    2008-01-01

    Background: Cognitive dysfunction is frequent in Cerebral Palsy (CP). CP motor impairment and associated speech deficits often hinder cognitive assessment, with the result being that not all CP studies consider cognitive dysfunction. Raven's Coloured Progressive Matrices is a simple, rapid test which can be used in persons with severe motor…

  5. Familial bulbar urethral strictures

    Science.gov (United States)

    Jindal, Tarun; Pal, Partha; Sinha, Rajan Kumar; Karmakar, Dilip

    2014-01-01

    Strictures are commonly encountered in the urological practice. The most common aetiologies are infection, trauma and iatrogenic events. Familial occurrence of urethral stricture is exceptionally rare. We present a case in which bulbar urethral strictures developed in a father and his two sons. PMID:24591388

  6. Levodopa-Induced Facial Dystonia in a Case of Progressive Supranuclear Palsy

    Directory of Open Access Journals (Sweden)

    Eun Joo Chung

    2012-05-01

    Full Text Available Progressive supranuclear palsy (PSP is frequently misdiagnosed as other Parkinsonism because of clinical heterogeneity of PSP. We present here a case of a 67-year-old male patient with frontotemporal dementia-like cognitive impairment including language difficulties and abnormal behaviors. He showed severe facial dystonia after the levodopa treatment. Herein, we describe an unusual case of a patient presenting with PSP which, we believe could contribute to our knowledge about atypical leveodopa-induced facial dystonia in PSP.

  7. Exome sequencing establishes a gelsolin mutation as the cause of inherited bulbar-onset neuropathy.

    Science.gov (United States)

    Caress, James B; Johnson, Janel O; Abramzon, Yevgeniya A; Hawkins, Gregory A; Gibbs, J Raphael; Sullivan, Elizabeth A; Chahal, Chamanpreet S; Traynor, Bryan J

    2017-11-01

    Progressive bulbar motor neuropathy is primarily caused by bulbar-onset ALS. Hereditary amyloidosis type IV also presents with a bulbar neuropathy that mimics motor neuron disease. The disease is prevalent in Finland only and is not commonly included in the differential diagnosis of ALS. We studied 18 members of a family in which some had bulbar motor neuropathy, and we performed exome sequencing. Five affected family members were found to have a D187Y substitution in the GSN gene known to cause hereditary amyloidosis type IV. This American family presented with progressive bulbar neuropathy due to a gelsolin mutation not found in Finland. Hereditary amyloidosis type IV presents with bulbar motor neuropathy and not with peripheral neuropathy as occurs with common forms of amyloidosis. This report demonstrates the power of exome sequencing to determine the cause of rare hereditary diseases with incomplete or atypical phenotypes. Muscle Nerve 56: 1001-1005, 2017. © 2016 Wiley Periodicals, Inc.

  8. A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis (ALS)

    OpenAIRE

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

    2011-01-01

    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 instrumenta...

  9. [A study on the cerebral glucose metabolism in progressive supranuclear palsy].

    Science.gov (United States)

    Ma, Ai-jun; Guo, Xiao-jun; Li, Da-cheng; Zhang, Ben-shu; Pan, Xu-dong

    2012-11-01

    To study the regional cerebral glucose utilization with (18)F-fluorodeoxyglucose (FDG) PET and to investigate the correlation between cerebral glucose metabolism and the clinical characteristic of progressive supranuclear palsy (PSP). A total of 13 patients with PSP and 30 matched healthy controls were performed (18)F-FDG PET imaging at rest state. Visual inspection and statistical parametric mapping (SPM) were used to investigate regional cerebral metabolic rate of glucose (rCMRglc). Based on the visual inspection, PET imaging in the PSP patients showed that the focal hypometabolic areas mainly included the bilateral frontal cortex, midbrain and subcortical structures. Compared to the controls, voxel-based analysis showed that the regional glucose metabolism decreased in bilateral superior, middle frontal gyrus, cingulate gyrus, midbrain and subcortical structures including basal ganglion and thalamus, which were consisted with the clinical characteristics, such as vertical gaze palsy, pseudobulbar palsy, postural instability, axial rigidity, dementia and so on. (18)F-FDG PET imaging is helpful for the early diagnosis of PSP.

  10. 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.

  11. 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.

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

    Directory of Open Access Journals (Sweden)

    Jain Nitin

    2011-01-01

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

  13. Randomized placebo-controlled trial of sodium valproate in progressive supranuclear palsy.

    Science.gov (United States)

    Leclair-Visonneau, Laurène; Rouaud, Tiphaine; Debilly, Bérangère; Durif, Franck; Houeto, Jean-Luc; Kreisler, Alexandre; Defebvre, Luc; Lamy, Estelle; Volteau, Christelle; Nguyen, Jean-Michel; Dily, Séverine Le; Damier, Philippe; Boutoleau-Bretonnière, Claire; Lejeune, Pascal; Derkinderen, Pascal

    2016-07-01

    Results from preclinical studies suggest that inhibition of glycogen synthase kinase (GSK-3) is a therapeutic option for tauopathies. The aim of the present study was therefore to determine the effects of sodium valproate (VPA), a GSK-3 inhibitor, on disease progression in progressive supranuclear palsy (PSP). We performed a double-blind, randomized, placebo-controlled trial, in 28 PSP patients who received VPA (1500mg/day) or matching placebo for 24 months. The primary endpoint was the change from baseline in Progressive Supranuclear Palsy Rating Scale (PSPRS) at 12 and 24 months. Secondary endpoints evaluated the effects of VPA on cognitive and behavioral status (MMSE, Mattis Dementia Rating Scale, Wisconsin Card Sorting, Gröber and Buschke and Oral Denomination 80 tests), tolerability of treatment, and patient compliance. There were no baseline differences between active treatment and placebo groups in age and clinical rating scores. PSPRS score at 12 months was significantly higher in the VPA than in the placebo group (60.8±20 versus 46.9±18.6 respectively, p=0.01), but was similar between the two groups at 24 months. No significant differences were observed between VPA and placebo groups for the secondary endpoints. Our results suggest that VPA is not effective as a disease-modifying agent in PSP. Copyright © 2016 Elsevier B.V. All rights reserved.

  14. Allelic ROBO3 heterogeneity in Tunisian patients with horizontal gaze palsy with progressive scoliosis.

    Science.gov (United States)

    Amouri, Rim; Nehdi, Houda; Bouhlal, Yosr; Kefi, Mounir; Larnaout, Abdelmajid; Hentati, Fayçal

    2009-11-01

    Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare autosomal recessive disorder characterized by the congenital absence of horizontal gaze, progressive scoliosis, and failure of the corticospinal and somatosensory axon tracts to decussate in the medulla. HGPPS is caused by mutations of the ROBO3 gene, which encodes a protein that shares homology with the roundabout family of transmembrane receptors that are important in axon guidance and neuronal migration. To date, over 15 mutations have been found in consanguineous families of Greek, Italian, Turkish, Pakistani, Saudi Arabian, and Indian descent. To detail clinical, cerebral magnetic resonance imaging (MRI) and genetic findings of ten HGPPS patients from four unrelated Tunisian families. Four unrelated consanguineous Tunisian families with a total of ten patients suffering from horizontal gaze palsy with progressive scoliosis. Genetic linkage analysis and direct sequencing of the ROBO3 gene. All patients shared similar clinical gaze movement abnormalities and variable degrees of scoliosis. Four distinct homozygous mutations were identified. This study extends the molecular spectrum of the ROBO3 gene and the geographic origin of patients with ROBO3 gene mutations, and underlines the homogeneity of the motor ocular syndrome whatever type of mutation is encountered.

  15. Bulbar and speech motor assessment in ALS: challenges and future directions.

    Science.gov (United States)

    Green, Jordan R; Yunusova, Yana; Kuruvilla, Mili S; Wang, Jun; Pattee, Gary L; Synhorst, Lori; Zinman, Lorne; Berry, James D

    2013-12-01

    Bulbar motor deterioration due to amyotrophic lateral sclerosis (ALS) leads to the eventual impairment of speech and swallowing functions. Despite these devastating consequences, no standardized diagnostic procedure for assessing bulbar dysfunction in ALS exists and adequate objective markers of bulbar deterioration have not been identified. In this paper, we consider objective measures of speech motor function, which show promise for forming the basis of a comprehensive, quantitative bulbar motor assessment in ALS. These measures are based on the assessment of four speech subsystems: respiratory, phonatory, articulatory, and resonatory. The goal of this research is to design a non-invasive, comprehensive bulbar motor assessment instrument intended for early detection, monitoring of disease progression, and clinical trial application. Preliminary data from an ongoing study of bulbar motor decline are presented, which demonstrate the potential clinical efficacy of the speech subsystem approach.

  16. TRACHEOTOMY IN BULBAR POLIOMYELITIS

    Science.gov (United States)

    Miller, Alden H.; Buck, Leonard S.

    1950-01-01

    Tracheotomy was performed on 181 of 351 patients with bulbar poliomyelitis. The essential indication for tracheotomy was secretional obstruction of the respiratory tract that could not be relieved by postural drainage and aspiration. Comparison of mortality rates in this series with those of previous series in which tracheotomy was not done in the presence of similar indications, suggests that the procedure may be life-saving in a considerable percentage of cases. Outside the respirator the tracheotomy can be done with or without the aid of the bronchoscope or endotracheal anesthesia tube. When done inside the opened respirator the Bennett flow-sensitive positive pressure machine should be used to supply oxygen to the patient while the respirator is not operating. PMID:15398892

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1988-09-01

    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.

  18. [An 81-year-old woman with progressive motor disturbance, extrapyramidal features, dementia, and oculomotor palsy].

    Science.gov (United States)

    Urabe, T; Mori, H; Sumino, S; Miwa, H; Wakiya, M; Shirai, T; Takubo, H; Mizuno, Y

    1998-03-01

    We report an 81-year-old woman who presented with motor disturbance in her right hand which was followed by parkinsonism, dementia, and supranuclear gaze palsy. She was well until her age of 73 (1989) when she had an onset of difficulty in using her right hand; she did not have weakness. She also developed small step gait. These symptoms had progressively become worse. She was admitted to our hospital in July of 1992 when she was 75 years old. On admission, she was alert and oriented, but she showed some difficulty in recent memory. She did not have aphasia or ideomotor apraxia, but she showed limb-kinetic apraxia in her right hand, ideational apraxia, dressing apraxia, constructional apraxia, tactile agnosia, and left-right disorientation. Alien-hand syndrome was observed in her right hand. Ocular movement was within normal limit for her age. She had oro-lingual dyskinesia. Otherwise, cranial nerves were intact. She walked in small-steps. She had rigidity and fine myoclonic movements in her right upper extremity. Deep reflexes were within normal limits and symmetric. Superficial and deep sensations were intact. Laboratory findings were unremarkable. She was discharged on August 15, 1992 for outpatient follow-up. Her motor and mental symptoms were progressive. By October of 1992, she developed supranuclear vertical gaze palsy, marked rigidity in the neck, and astereognosis. By June 1993, she became unable to walk without support. MRI taken in May of 1994 revealed atrophy of insular cortices, temporal lobe tips and parietal lobes more on the left side; the third ventricle was slightly dilated. She was admitted to another hospital on June 30, 1994. She had become a bed-ridden state with marked dementia and dysphagia. She developed fever on November 5, 1996 and expired on December 16 of the same year. She was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had corticobasal degeneration. Other diagnoses entertained

  19. 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

  20. 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

  1. Effectiveness of Functional Progressive Resistance Exercise Training on Walking Ability in Children with Cerebral Palsy: A Randomized Controlled Trial

    Science.gov (United States)

    Scholtes, Vanessa A.; Becher, Jules G.; Janssen-Potten, Yvonne J.; Dekkers, Hurnet; Smallenbroek, Linda; Dallmeijer, Annet 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=26) or control group (n=25, receiving usual care).…

  2. 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=

  3. ANCA-negative Wegener's granulomatosis with multiple lower cranial nerve palsies.

    Science.gov (United States)

    Kim, Sung-Hee; Park, Jin; Bae, Jung Ho; Cho, Min-Sun; Park, Kee Duk; Jeong, Jee Hyang

    2013-11-01

    Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.

  4. The Neuropsychology (Broadly Conceived) of Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration.

    Science.gov (United States)

    Gerstenecker, Adam

    2017-11-01

    To review the cognitive and behavioral features of the different atypical parkinsonian syndromes in which motor symptoms dominate early clinical symptomology: multiple systems atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The impact of cognitive and behavioral deficits on quality of life, associations between neuropsychological and neuropsychiatric findings and brain imaging, and cognitive and behavioral symptom management are also discussed. A review of the available MSA, PSP, and CBD literature was conducted, with emphasis given to studies investigating the cognitive and behavioral features of the syndromes. Although the three reviewed atypical parkinsonian syndromes share many similarities to each other and PD from a neuropsychological perspective, differences in prevalence and severity of cognitive impairment and patterns of performance on neuropsychological and neuropsychiatric measures exist in the research literature. Cognitive and behavioral features are early and pervasive aspects of MSA, PSP, and CBD.

  5. An autopsied case of progressive supranuclear palsy presenting with cerebellar ataxia and severe cerebellar involvement.

    Science.gov (United States)

    Iwasaki, Yasushi; Mori, Keiko; Ito, Masumi; Tatsumi, Shinsui; Mimuro, Maya; Yoshida, Mari

    2013-10-01

    A Japanese male patient presented with gait disturbance at the age of 69 years. His principal symptom was cerebellar ataxia for several years. He was initially diagnosed as having olivopontocerebellar atrophy because dysarthria and ataxia gradually developed, and head CT scan showed apparent atrophy of the cerebellum and brainstem and dilatation of the fourth ventricle. Later, he showed vertical gaze palsy, dysphagia, retrocollis, parkinsonism, axial dominant rigidity and grasp reflex, and therefore, the diagnosis was modified to progressive supranuclear palsy (PSP). Progressive atrophy of the frontotemporal lobe, cerebellum and brainstem, and dilatation of the lateral, third and fourth ventricles were evident on MRI. Gastrostomy and tracheotomy were performed 9 and 10 years after onset, respectively, and the patient died after 11 years disease duration. At autopsy the brain weighed 1000 g and showed atrophy of the frontotemporal lobe, cerebellum and brainstem. Neurofibrillary tangles, mainly globose-type revealed by Gallyas-Braak silver staining, were extensively observed in the cerebral cortex and subcortical grey matter. Numerous glial fibrillary tangles, including tuft-shaped astrocytes and coiled bodies, and extensive argyrophilic threads were also recognized, particularly in the frontal lobe, basal ganglia, cerebellar white matter, brainstem and spinal cord. The Purkinje cell layer showed severe neuron loss with Bergmann's gliosis, and the dentate nucleus showed severe neuron loss with grumose degeneration. Tau-positive/Gallyas-positive inclusions in the Purkinje cells and the glial cells of the Purkinje cell layer were observed. Pathological findings of the present patient were consistent with the diagnosis of PSP, but the olivopontocerebellar involvement, particularly in the cerebellum, was generally more severe, and the quantity of tau-positive/Gallyas-positive structures were more abundant than in typical PSP cases. The existence of a distinct, rare PSP

  6. The Prevalence, Rate of Progression, and Treatment of Elbow Flexion Contracture in Children with Brachial Plexus Birth Palsy

    Science.gov (United States)

    Sheffler, Lindsey C.; Lattanza, Lisa; Hagar, Yolanda; Bagley, Anita; James, Michelle A.

    2012-01-01

    Background: Elbow flexion contracture is a well-known complication of brachial plexus birth palsy that adversely affects upper-extremity function. The prevalence, risk factors, and rate of progression of elbow flexion contracture associated with brachial plexus birth palsy have not been established, and the effectiveness of nonoperative treatment involving nighttime splinting or serial casting has not been well studied. Methods: The medical records of 319 patients with brachial plexus birth palsy who had been seen at our institution between 1992 and 2009 were retrospectively reviewed to identify patients with an elbow flexion contracture (≥10°). The chi-square test for trend and the Kaplan-Meier estimator were used to evaluate risk factors for contracture, including age, sex, and the extent of brachial plexus involvement. Longitudinal models were used to estimate the rate of contracture progression and the effectiveness of nonoperative treatment. Results: An elbow flexion contracture was present in 48% (152) of the patients with brachial plexus birth palsy. The median age of onset was 5.1 years (range, 0.25 to 14.8 years). The contracture was ≥30° in 36% (fifty-four) of these 152 patients and was accompanied by a documented radial head dislocation in 6% (nine). The prevalence of contracture increased with increasing age (p contracture increased by 4.4% per year before treatment (p contracture decreased by 31% when casting was performed (p contracture did not improve when splinting was performed but the rate of increase thereafter decreased to contracture in children with brachial plexus birth palsy may be greater than clinicians perceive. The prevalence increased with patient age but was not significantly affected by sex or by the extent of brachial plexus involvement. Serial casting may initially improve severe contractures, whereas nighttime splinting may prevent further progression of milder contractures. Level of Evidence: Therapeutic Level IV. See

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

    Science.gov (United States)

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

    2011-01-01

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

  8. Trial of Zolpidem, Eszopiclone, and Other GABA Agonists in a Patient with Progressive Supranuclear Palsy

    Directory of Open Access Journals (Sweden)

    Andrew Young Chang

    2014-01-01

    Full Text Available Progressive supranuclear palsy (PSP is a progressive, debilitating neurodegenerative disease of the Parkinson-plus family of syndromes. Unfortunately, there are no pharmacologic treatments for this condition, as most sufferers of the classic variant respond poorly to Parkinson medications such as levodopa. Zolpidem, a gamma aminobutyric acid (GABA agonist specific to the α-1 receptor subtype, has been reported to show improvements in symptoms of PSP patients, including motor dysfunction, dysarthria, and ocular disturbances. We observed a 73-year-old woman with a six-year history of PSP, who, upon administration of a single 12.5 mg dose of sustained-release zolpidem, exhibited marked enhancements in speech, facial expressions, and fine motor skills for five hours. These results were reproduced upon subsequent clinic visits. In an effort to find a sustainable medication that maximized these beneficial effects while minimizing side effects and addressing some of her comorbid neuropsychological conditions, a trial of five other GABA receptor agonists was performed with the patient’s consent, while she and her caregivers were blinded to the specific medications. She and her caretakers subsequently reported improvements, especially visual, while on eszopiclone, and, to a lesser degree, temazepam and flurazepam.

  9. Functional disconnection of thalamic and cerebellar dentate nucleus networks in progressive supranuclear palsy and corticobasal syndrome.

    Science.gov (United States)

    Upadhyay, Neeraj; Suppa, Antonio; Piattella, Maria Cristina; Giannì, Costanza; Bologna, Matteo; Di Stasio, Flavio; Petsas, Nikolaos; Tona, Francesca; Fabbrini, Giovanni; Berardelli, Alfredo; Pantano, Patrizia

    2017-06-01

    To assess functional rearrangement following neurodegeneration in the thalamus and dentate nucleus in patients with progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). We recruited 19 patients with PSP, 11 with CBS and 14 healthy subjects. All the subjects underwent resting-state (rs) fMRI using a 3T system. Whole brain functional connectivity of the thalamus and dentate nucleus were calculated by means of a seed-based approach with FEAT script in FSL toolbox. Thalamic volume was calculated by means of FIRST, and the dentate area by means of Jim software. Both thalamic volume and dentate area were significantly smaller in PSP and CBS patients than in healthy subjects. No significant difference emerged in thalamic volume between PSP and CBS patients, whereas dentate area was significantly smaller in PSP than in CBS. Thalamic functional connectivity was significantly reduced in both patient groups in various cortical, subcortical and cerebellar areas. By contrast, changes in dentate nucleus functional connectivity differed in PSP and CBS: it decreased in subcortical and prefrontal cortical areas in PSP, but increased asymmetrically in the frontal cortex in CBS. Evaluating the dentate nucleus size and its functional connectivity may help to differentiate patients with PSP from those with CBS. Copyright © 2017 Elsevier Ltd. All rights reserved.

  10. 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’.

  11. 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.

  12. Diagnostic value of blink reflex in multisystem atrophy, progressive supranuclear palsy and Parkinson disease.

    Science.gov (United States)

    Szmidt-Salkowska, Elzbieta; Gawel, Malgorzata; Jamrozik, Zygmunt; Salkowska-Wanat, Joanna; Gawel, Damian; Kaminska, Anna

    Abnormal blink reflex (BR) is a result of reticular brainstem pathways dysfunction and seems to be one of the features of brain degenerative disorders. The aim of the study was to estimate the diagnostic value of blink reflex in neurodegenerative diseases such as: multisystem atrophy (MSA), progressive supranuclear palsy (PSP) and Parkinson disease (PD). Material consisted of 99 patients with clinically probable MSA (51), PSP (28) and PD (20). MSA patients were divided into two subgroups, with dominant cerebellar (MSA-C) and parkinsonian signs (MSA-P). The mean age of patients was 64.9 years (47-79 years); males - 55.3%. Blink reflex was obtained in a typical way. The significant differences in mean values of blink reflex latencies between PD and other subgroups (MSA-P, MSA-C, PSP) were found, but all of them were in normal range. In individual patients with PD and PSP (50% and 18%, respectively) delayed R2 latencies were recorded. The most frequently abnormal blink reflexes, comparing the MSA, PSP and PD groups, were present in PD patients. We postulate that this may be explained by pathological influence of nigrostriatal pathway on the circuit linking the basal ganglia, cerebellum and brainstem. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  13. MRI gray and white matter measures in progressive supranuclear palsy and corticobasal syndrome.

    Science.gov (United States)

    Upadhyay, Neeraj; Suppa, Antonio; Piattella, Maria Cristina; Bologna, Matteo; Di Stasio, Flavio; Formica, Alessandra; Tona, Francesca; Colosimo, Carlo; Berardelli, Alfredo; Pantano, Patrizia

    2016-10-01

    We evaluated MRI measures of gray and white matter damages in 19 patients with progressive supranuclear palsy (PSP), 11 with corticobasal syndrome (CBS), and 14 healthy subjects (HS) to differentiate patients with PSP from those with CBS. We calculated surface-based maps of the cortical volume, cortical thickness, surface area, and voxel level maps of sub-cortical volume, and diffusion tensor imaging parameters using automated scripts implemented in FreeSurfer and FSL toolboxes. No significant differences in cortical volume loss were observed between PSP and CBS. When cortical volume was divided into cortical thickness and surface area, cortical thickness in peri-rolandic brain regions was significantly smaller in CBS than in PSP patients, whereas surface area was significantly smaller in PSP than HS. We also found widespread volume loss in sub-cortical structures in patients with PSP and CBS in comparison to HS. Both patient groups displayed diffusion tensor imaging abnormalities: compared to HS, widespread fractional anisotropy and radial diffusivity changes were observed in PSP, whereas axial and radial diffusivity changes were prominent in CBS. Mini-mental state examination positively correlated with diffusion changes in patients with PSP. In conclusion, cortical thickness, surface area, and diffusion tensor imaging parameters may be sensitive enough to help differentiate patients with PSP from those with CBS.

  14. Clinical value of MRI and acute madopar responsiveness test in diagnosing progressive supranuclear palsy

    Directory of Open Access Journals (Sweden)

    LI Xiao-hong

    2013-07-01

    Full Text Available Objective To investigate the MRI abnormalities and acute madopar responsiveness test in diagnosing progressive supranuclear palsy (PSP and Parkinson's disease (PD. Methods Seventeen patients with PSP and 17 gender and age matched patients with PD were studied with cranial MRI examinations and results of acute madopar responsiveness test, and the clinical manifestations of PSP were summarized. Results The atrophy of the midbrain tegmentum and hummingbird sign was demonstrated in all of the PSP patients in our study, but was not observed in the PD patients. The areas of the midbrain on mid-sagittal MRI in PSP patients [(77.35 ± 15.30 mm2] were significantly smaller than that in those with PD [(142.35 ± 31.49 mm2]. The average ratio of the area of the midbrain to the area of pons in the patients with PSP [(14.31 ± 2.47%] was significantly smaller than that in those with PD [(24.08 ± 4.73%; P = 0.000, for all]. According to the result of acute madopar responsiveness test, the maximum Unified Parkinson's Disease Rating Scale (UPDRS Ⅲ improvement rate of 2 patients with PSP and 16 patients with PD was more than 30% (χ2 = 23.142, P = 0.000. Conclusion The assessment of the mid-sagittal MRI and acute madopar responsiveness test may be a useful method to differentiate PSP from PD.

  15. 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.

  16. Exclusion of progressive brain disorders of childhood for a cerebral palsy monitoring system: a public health perspective.

    Science.gov (United States)

    Olney, Richard S; Doernberg, Nancy S; Yeargin-Allsop, Marshalyn

    2014-01-01

    Cerebral palsy (CP) is defined by its nonprogressive features. Therefore, a standard definition and list of progressive disorders to exclude would be useful for CP monitoring and epidemiologic studies. We reviewed the literature on this topic to 1) develop selection criteria for progressive brain disorders of childhood for public health surveillance purposes, 2) identify categories of disorders likely to include individual conditions that are progressive, and 3) ascertain information about the relative frequency and natural history of candidate disorders. Based on 19 criteria that we developed, we ascertained a total of 104 progressive brain disorders of childhood, almost all of which were Mendelian disorders. Our list is meant for CP surveillance programs and does not represent a complete catalog of progressive genetic conditions, nor is the list meant to comprehensively characterize disorders that might be mistaken for cerebral palsy. The criteria for progressive disorders that we developed could be applied by public health investigators in the future, as more children with very rare conditions are followed and new candidate disorders are identified.

  17. Progressive Supranuclear Palsy Syndrome and Semantic Dementia in Neuropathologically Proven Lewy Body Disease: A Report of Two Cases.

    Science.gov (United States)

    Lagarde, Julien; Hamelin, Lorraine; Hahn, Valé; Habert, Marie-Odile; Seilhean, Danielle; Duyckaerts, Charles; Sarazin, Marie

    2015-01-01

    The impact of neuropathological lesions on the clinical symptoms and progression of Lewy body disease (LBD) remains unclear. To address this issue, we describe two illustrative cases of autopsy-proven LBD that presented atypical phenotypes of progressive supranuclear palsy syndrome and semantic dementia. Postmortem examination revealed diffuse LBD with massive brainstem involvement in case 1, whereas Lewy bodies predominated in the amygdala and neocortex in case 2. Alzheimer's disease pathology was present in both cases, and TDP-43 inclusions were noted in case 2. These cases illustrate two contrasted clinical presentations and highlight the heterogeneity within the underlying proteinopathies of neurodegenerative diseases.

  18. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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.

  1. Whole-brain atrophy differences between progressive supranuclear palsy and idiopathic Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Carlos Guevara

    2016-09-01

    Full Text Available Background: The absence of markers for ante-mortem diagnosis of progressive supranuclear palsy (PSP results in this disorder’s being commonly mistaken for other conditions, such as idiopathic Parkinson's disease (IPD. Such mistakes occur particularly in the initial stages, when ‘plus syndrome’ has not yet clinically emerged.Objective. To investigate global brain volume and tissue loss in patients with PSP relative to patients with IPD and healthy controls and correlations between clinical parameters and magnetic resonance imaging (MRI-derived brain volume estimates.Methods: T1-weighted images were obtained from three groups of Chilean Latin American adults: 21 patients with IPD, 18 patients with PSP and 14 healthy controls. We used Structural Imaging Evaluation with Normalization of Atrophy (SIENAX to assess white matter, gray matter and whole-brain volumes (normalized to cranial volume. Imaging data were used to analyze putative correlations with the clinical status of PSP and IPD patients using the Unified Parkinson’s Disease Rating Scale Part III, Hoehn and Yahr, the Clinical Global Impression for Disease Severity Scale and the Frontal Assessment Battery.Results: PSP patients had significantly lower whole brain volume than both IPD patients and controls. Whole brain volume reduction in PSP patients was primarily attributable to gray matter volume reduction. We found a significant correlation between brain volume reduction and clinical status in the PSP group.Conclusions: At the group level, whole brain and gray matter volumes differentiated patients with PSP from patients with IPD. There was also significant clinical-imaging correlations with motor disturbances in PSP.

  2. Obsessive compulsive personality disorder in Progressive Supranuclear Palsy, Multiple System Atrophy and Essential Tremor.

    Science.gov (United States)

    Nicoletti, A; Luca, A; Luca, M; Donzuso, G; Mostile, G; Raciti, L; Contrafatto, D; Dibilio, V; Sciacca, G; Cicero, C E; Vasta, R; Petralia, A; Zappia, M

    2016-09-01

    aim of the study was to evaluate the presence of the Obsessive Compulsive Personality Disorder (OCPeD) in Multiple System Atrophy (MSA), Progressive Supranuclear Palsy (PSP) and Essential Tremor (ET) and in a group of healthy subjects. patients affected by MSA, PSP and ET diagnosed according to currently accepted diagnostic criteria and a group of healthy controls were enrolled in the study. Patients with cognitive impairment were excluded from the study. The Structured Clinical Interview for Personality Disorders-II (SCID-II) has been performed to evaluate the presence of personality disorders (PeDs). The diagnosis of OCPeD was confirmed by a psychiatric interview. fifteen MSA patients (8 men and 7 women; aged 62.9 ± 7.6 years), 14 PSP patients (8 men and 6 women; aged 69.8 ± 4.4 years), 16 ET patients (10 men and 6 women; aged 70.4 ± 6.4 years) and 20 healthy subjects (10 men and 10 women; aged 65.5 ± 6.0 years) were enrolled. OCPeD was recorded in 5 (35.7%) PSP patients, 2 (13.3%) MSA patients, 2 (12.5%) ET patient and 2 (10%) controls. a low frequency of OCPeD, close to those recorded in healthy subjects, was recorded in both MSA and ET patients. Conversely an higher frequency of OCPeD, similar to PD was found among PSP patients, supporting the possibility of an impairment of common basal ganglia network possibly involving the orbito-frontal circuits. Copyright © 2016 Elsevier Ltd. All rights reserved.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2015-11-15

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2008-05-15

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

  5. 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.

  6. The Disturbance of Gaze in Progressive Supranuclear Palsy: Implications for Pathogenesis

    Science.gov (United States)

    Chen, Athena L.; Riley, David E.; King, Susan A.; Joshi, Anand C.; Serra, Alessandro; Liao, Ke; Cohen, Mark L.; Otero-Millan, Jorge; Martinez-Conde, Susana; Strupp, Michael; Leigh, R. John

    2010-01-01

    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 50 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. PMID:21188269

  7. Force control deficits in individuals with Parkinson's disease, multiple systems atrophy, and progressive supranuclear palsy.

    Directory of Open Access Journals (Sweden)

    Kristina A Neely

    Full Text Available This study examined grip force and cognition in Parkinson's disease (PD, Parkinsonian variant of multiple system atrophy (MSAp, progressive supranuclear palsy (PSP, and healthy controls. PD is characterized by a slower rate of force increase and decrease and the production of abnormally large grip forces. Early-stage PD has difficulty with the rapid contraction and relaxation of hand muscles required for precision gripping. The first goal was to determine which features of grip force are abnormal in MSAp and PSP. The second goal was to determine whether a single variable or a combination of motor and cognitive measures would distinguish patient groups. Since PSP is more cognitively impaired relative to PD and MSAp, we expected that combining motor and cognitive measures would further distinguish PSP from PD and MSAp.We studied 44 participants: 12 PD, 12 MSAp, 8 PSP, and 12 controls. Patients were diagnosed by a movement disorders neurologist and were tested off anti-Parkinsonian medication. Participants completed a visually guided grip force task wherein force pulses were produced for 2 s, followed by 1 s of rest. We also conducted four cognitive tests.PD, MSAp, and PSP were slower at contracting and relaxing force and produced longer pulse durations compared to controls. PSP produced additional force pulses during the task and were more cognitively impaired relative to other groups. A receiver operator characteristic analysis revealed that the combination of number of pulses and Brief Test of Attention (BTA discriminated PSP from PD, MSAp, and controls with a high degree of sensitivity and specificity.Slowness in contracting and relaxing force represent general features of PD, MSAp, and PSP, whereas producing additional force pulses was specific to PSP. Combining motor and cognitive measures provides a robust method for characterizing behavioral features of PSP compared to MSAp and PD.

  8. Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy.

    Science.gov (United States)

    Josephs, Keith A; Duffy, Joseph R

    2008-12-01

    To highlight the fact that patients with corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) can sometimes present with a progressive apraxia of speech, nonfluent aphasia, or a combination of the two disorders. Corticobasal degeneration and PSP are neurodegenerative diseases characterized by neuronal loss and gliosis in cardinal brain regions, as well as the abnormal deposition of the microtubule associated protein tau in cell bodies and cell processes. The typical presenting features of CBD and PSP are akinesia and rigidity that are levodopa unresponsive, although there has been evidence that both diseases, moreso CBD, can present with a dementia syndrome. Recent clinicopathological studies have now also demonstrated that a subset of patients with CBD and PSP present with a progressive apraxia of speech, nonfluent aphasia, or a combination of the two disorders. Presenting features of progressive apraxia of speech or nonfluent aphasia are strongly associated with a diagnosis of CBD, PSP, or both.

  9. Features of Patients With Nonfluent/Agrammatic Primary Progressive Aphasia With Underlying Progressive Supranuclear Palsy Pathology or Corticobasal Degeneration.

    Science.gov (United States)

    Santos-Santos, Miguel A; Mandelli, Maria Luisa; Binney, Richard J; Ogar, Jennifer; Wilson, Stephen M; Henry, Maya L; Hubbard, H Isabel; Meese, Minerva; Attygalle, Suneth; Rosenberg, Lynne; Pakvasa, Mikhail; Trojanowski, John Q; Grinberg, Lea T; Rosen, Howie; Boxer, Adam L; Miller, Bruce L; Seeley, William W; Gorno-Tempini, Maria Luisa

    2016-06-01

    We provide novel evidence of specific clinical and neuroimaging features that may help for the in vivo prediction of underlying pathology in patients with nonfluent/agrammatic primary progressive aphasia (nfvPPA) and progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD) proved by autopsy. To characterize the neurological, cognitive, and neuroimaging features of patients with nfvPPA-in whom either PSP or CBD was eventually confirmed at autopsy-at initial presentation and at 1-year follow-up. A prospective longitudinal clinical-pathological study was conducted in a tertiary research clinic that specialized in cognitive disorders. Fourteen patients were evaluated between January 2002 and December 2014. Inclusion criteria for the study were a clinical diagnosis of nfvPPA; the availability of speech, language, and cognitive testing for at least 1 evaluation; magnetic resonance imaging within 6 months of initial evaluation; and a postmortem pathological diagnosis of PSP or CBD. Ten matched healthy control participants were also included. Clinical, cognitive, and neuroimaging longitudinal data were analyzed to characterize the whole nfvPPA-4-repeat-tau group and identify differences between nfvPPA-PSP and nfvPPA-CBD both at presentation and longitudinally. Patient groups did not differ significantly in age, sex, or handedness (nfvPPA-PSP group: median [interquartile range (IQR)] age, 74 [67-76] years; 1 of 5 male [20%]; 1 of 5 left-handed [20%]; and nfvPPA-CBD group: mean [IQR] age, 65 [54-81] years; 3 of 9 male [33%]; 0 left-handed). Motor speech impairment and left frontal white matter atrophy were the most prominent common features. At presentation, dysarthria (Motor Speech Examination median [IQR] score: nfvPPA-PSP, 4 [2-7]; nfvPPA-CBD, 0 [0-4]; P = .02), depression (Geriatric Depression Scale median [IQR] score: nfvPPA-PSP, 19 [3-28]; nfvPPA-CBD, 4 [0-16]; P = .04), and relatively selective white matter atrophy were typical of the nfv

  10. The use of lisuride, a potent dopamine and serotonin agonist, in the treatment of progressive supranuclear palsy.

    OpenAIRE

    Neophytides, A; Lieberman, A N; Goldstein, M; Gopinathan, G; Leibowitz, M; Bock, J; Walker, R

    1982-01-01

    Seven patients with progressive supranuclear palsy were treated with lisuride. Mean age was 62 years (range, 52 to 68 years), and duration of disease was 4.4 years (range, 1 to 7 years). All seven had been treated with levodopa/carbidopa and three with bromocriptine; four had, at one time, shown a partial response to levodopa. One patient had also shown a partial response to bromocriptine. Lisuride was used alone in four patients, and combined with levodopa/carbidopa in three patients. Mean d...

  11. Apraxia in Parkinson's disease, progressive supranuclear palsy, multiple system atrophy and neuroleptic-induced parkinsonism.

    Science.gov (United States)

    Leiguarda, R C; Pramstaller, P P; Merello, M; Starkstein, S; Lees, A J; Marsden, C D

    1997-01-01

    We studied 45 non-demented patients with Parkinson's disease (PD), 12 with progressive supranuclear palsy (PSP), 10 with multiple system atrophy (MSA) and 12 with neuroleptic-induced parkinsonism (NIP) for the presence of apraxia. Our aim was to determine whether a standard comprehensive assessment of different praxic functions would demonstrate specific types of errors not attributable to bradykinesia, rigidity, tremor or any other abnormal elementary motor deficit. PD patients on chronic levodopa treatment were examined in the 'on' and 'off' (treatment) states. Based on apraxia assessment scores, bilateral ideomotor apraxia for transitive movements was found in eight (75%) and 12 (27%) of PSP and PD patients, respectively. Ideomotor apraxia was mainly characterized by spatial errors (i.e., external and internal configuration, body-part-as-object and trajectory). Four PSP but no PD patients exhibited ideomotor apraxia for intransitive movements. PSP as well as PD patients with ideomotor apraxia also had difficulties in imitating hand and finger postures, but none of them failed on pantomime comprehension and pantomime recognition/discrimination. Some PSP patients exhibited, in addition, a limbkinetic type of apraxia and a minority of them displayed deficits on tasks involving multiple steps. Neither MSA nor NIP patients showed any disturbance of praxic functions. There were no differences in age, disease duration, Mini Mental State Examination (MMSE), Unified Parkinson's disease Rating Scale and Hoehn-Yahr scores between apraxic and non-apraxic PD patients, and ideomotor apraxia scores were similar in the 'on' and 'off' states. A correlation was found between ideomotor apraxia scores in PD patients and deficits in frontal lobe-related neuropsychological tasks such as the Tower of Hanoi, verbal fluency and the Trail Making Test. Furthermore, PD patients with apraxia showed higher Hamilton depression scores than non-apraxic PD patients. In PSP patients, ideomotor

  12. 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

  13. The use of lisuride, a potent dopamine and serotonin agonist, in the treatment of progressive supranuclear palsy.

    Science.gov (United States)

    Neophytides, A; Lieberman, A N; Goldstein, M; Gopinathan, G; Leibowitz, M; Bock, J; Walker, R

    1982-03-01

    Seven patients with progressive supranuclear palsy were treated with lisuride. Mean age was 62 years (range, 52 to 68 years), and duration of disease was 4.4 years (range, 1 to 7 years). All seven had been treated with levodopa/carbidopa and three with bromocriptine; four had, at one time, shown a partial response to levodopa. One patient had also shown a partial response to bromocriptine. Lisuride was used alone in four patients, and combined with levodopa/carbidopa in three patients. Mean dose of lisuride was 2.5 mg (range, 1.5 to 5.0 mg). Mean duration of treatment was 4 months (range, 1 to 10 months). While two patients showed a reduction in rigidity, one in tremor and two in bradykinesia, in only one of them was there an overall improvement. It is postulated that the relative lack of response to lisuride may be due to a loss of both the dopaminergic and serotonergic receptors in progressive supranuclear palsy.

  14. Effects of a group circuit progressive resistance training program compared with a treadmill training program for adolescents with cerebral palsy.

    Science.gov (United States)

    Aviram, Ronit; Harries, Netta; Namourah, Ibtisam; Amro, Akram; Bar-Haim, Simona

    2017-08-01

    To determine whether goal-directed group circuit progressive resistance exercise training (GT) can improve motor function in adolescents with cerebral palsy (CP) and to compare outcomes with a treadmill training (TT) intervention. In a multi-centered matched pairs study, 95 adolescents with spastic CP (GMFCS II-III) were allocated to GT or TT interventions for 30 bi-weekly one hour training. Outcome measures of GMFM-66, GMFM-D%, GMFM-E%, TUG, 10 meter walk test (10 MWT), and 6 minute walk test (6 MWT) were made at baseline (T1), after interventions (T2) and 6 months post training (T3). Both training programs induced significant improvement in all outcome measures (T2-T1) that were mostly retained at T3. At the end of the intervention, the GT group showed an advantage in all measured changes compared to the TT group and in percentage changes. Differences were significant (p programs were effective in improving motor function in adolescents with cerebral palsy. The GT program had generally greater benefits based on the functional measures.

  15. Lower limb strength training in children with cerebral palsy--a randomized controlled trial protocol for functional strength training based on progressive resistance exercise principles

    NARCIS (Netherlands)

    Scholtes, V.A.B.; Dallmeijer, A.J.; Rameckers, E.A.; Verschuren, O.; Tempelaars, E.; Hensen, M.; Becher, J.G.

    2008-01-01

    Background: Until recently, strength training in children with cerebral palsy (CP) was considered to be inappropriate, because it could lead to increased spasticity or abnormal movement patterns. However, the results of recent studies suggest that progressive strength training can lead to increased

  16. 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.

  17. Disease: H00841 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00841 Infantile progressive bulbar palsy, including: Brown-Vialetto-Van Laere synd...rome (BVVLS) ; Fazio-Londe disease Infantile progressive bulbar palsy is a rare neurological disorder that o...ccurs in children. Infantile progressive bulbar palsy presents as following two forms. The Brown-Vialetto-Va...n Laere syndrome (BVVLS) is characterized by progressive pontobulbar palsy associ...ou A, Vagiakou EA Infantile progressive bulbar palsy with deafness. Brain Dev 24:732-5 (2002) PMID:21110228

  18. [A mother and her son with autosomal dominant bulbar spinal muscular atrophy].

    Science.gov (United States)

    Ibi, T; Suoh, H; Igarashi, S; Tsuji, S; Sahashi, K

    1992-09-01

    We reported a 49-year-old mother and her 28-year-old son with autosomal dominantly inherited bulbar spinal muscular atrophy (AD-BSMA). They showed progressive bulbar paresis, muscle wasting and weakness dominant in the proximal groups of limb muscles, and finger tremor. Onset of illness was in adult life. In laboratory examinations, elevated creatine kinase in serum and neurogenic changes either in EMG or muscle biopsy were noted. The son had neither gynecomastia nor abnormal sexual hormone levels which were observed in the sex-linked recessive bulbar spinal muscular atrophy (SR-BSMA). Elongation due to the CAG repeats at the androgen receptor gene of the X chromosome in SR-BSMA was not detected. In conclusion, it is clear that AD-BSMA is different from SR-BSMA on the basis of clinical and genetical aspects.

  19. 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

    Pathomechanisms of spinal and bulbar muscular atrophy (SBMA) have been extensively investigated and are partially understood, but no effective treatment is currently available for this disabling disorder. Its rarity, the slow disease progression, and lack of sensitive-to-change outcome measures...

  20. The economic costs of progressive supranuclear palsy and multiple system atrophy in France, Germany and the United Kingdom.

    Directory of Open Access Journals (Sweden)

    Paul McCrone

    Full Text Available Progressive supranuclear palsy (PSP and multiple system atrophy (MSA are progressive disabling neurological conditions usually fatal within 10 years of onset. Little is known about the economic costs of these conditions. This paper reports service use and costs from France, Germany and the UK and identifies patient characteristics that are associated with cost. 767 patients were recruited, and 760 included in the study, from 44 centres as part of the NNIPPS trial. Service use during the previous six months was measured at entry to the study and costs calculated. Mean six-month costs were calculated for 742 patients. Data on patient sociodemographic and clinical characteristics were recorded and used in regression models to identify predictors of service costs and unpaid care costs (i.e., care from family and friends. The mean six-month service costs of PSP were €24,491 in France, €30,643 in Germany and €25,655 in the UK. The costs for MSA were €28,924, €25,645 and €19,103 respectively. Unpaid care accounted for 68-76%. Formal and unpaid costs were significantly higher the more severe the illness, as indicated by the Parkinson's Plus Symptom scale. There was a significant inverse relationship between service and unpaid care costs.

  1. 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 Motor Function Measure Evolution 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.

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

    Directory of Open Access Journals (Sweden)

    van Meerkerk-Aanen PJ

    2017-08-01

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

  3. A Rare Case of Progressive Palsy of the Lower Leg Caused by a Huge Lumbar Posterior Endplate Lesion after Recurrent Disc Herniation

    Directory of Open Access Journals (Sweden)

    Masatoshi Morimoto

    2016-01-01

    Full Text Available A lesion of the lumbar posterior endplate is sometimes identified in the spinal canal of children and adolescents; it causes symptoms similar to those of a herniated disc. However, the pathology of the endplate lesion and the pathology of the herniated disc are different. We present a rare case of a 23-year-old woman who developed progressive palsy of the lower leg caused by huge lumbar posterior endplate lesion after recurrent disc herniation.

  4. Electrical Stimulation of Eye Blink in Individuals with Acute Facial Palsy: Progress toward a Bionic Blink.

    Science.gov (United States)

    Frigerio, Alice; Heaton, James T; Cavallari, Paolo; Knox, Chris; Hohman, Marc H; Hadlock, Tessa A

    2015-10-01

    Elicitation of eye closure and other movements via electrical stimulation may provide effective treatment for facial paralysis. The authors performed a human feasibility study to determine whether transcutaneous neural stimulation can elicit a blink in individuals with acute facial palsy and to obtain feedback from participants regarding the tolerability of surface electrical stimulation for daily blink restoration. Forty individuals with acute unilateral facial paralysis, HB grades 4 through 6, were prospectively studied between 6 and 60 days of onset. Unilateral stimulation of zygomatic facial nerve branches to elicit eye blink was achieved with brief bipolar, charge-balanced pulse trains, delivered transcutaneously by adhesive electrode placement; results were recorded on a high-speed video camera. The relationship between stimulation parameters and cutaneous sensation was analyzed using the Wong-Baker Faces Pain Rating Scale. Complete eye closure was achieved in 55 percent of participants using stimulation parameters reported as tolerable. In those individuals, initial eye twitch was observed at an average current of 4.6 mA (±1.7; average pulse width of 0.7 ms, 100 to 150 Hz), with complete closure requiring a mean of 7.2 mA (±2.6). Transcutaneous facial nerve stimulation may artificially elicit eye blink in a majority of patients with acute facial paralysis. Although individuals varied widely in their reported degrees of discomfort from blink-eliciting stimulation, most of them indicated that such stimulation would be tolerable if it could restore eye closure. These patients would therefore benefit from a biomimetic device to facilitate eye closure until the recovery process is complete. Therapeutic, IV.

  5. Buccal Mucosa Urethroplasty for Bulbar Urethral Strictures ...

    African Journals Online (AJOL)

    After the bulbar urethra is exposed, we perform a dorsal endoscopic cold knife urethrotomy until the urethra is fully opened. After measurement of the defect, the graft is harvested from the lower lip and sutured to the urethra and to the corpora cavernosa. A transurethral grooved catheter and suprapubic drainage are left for 7 ...

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

    Science.gov (United States)

    Marx, Svenja; Respondek, Gesine; Stamelou, Maria; Dowiasch, Stefan; Stoll, Josef; Bremmer, Frank; Oertel, Wolfgang H; Höglinger, Günter U; Einhäuser, Wolfgang

    2012-01-01

    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 (VOG) to a wearable head-mounted device, which can be readily applied in clinical practice. We investigated the eye movements in 10 possible or probable PSP patients, 11 Parkinson's disease (PD) patients, and 10 age-matched healthy controls (HCs) 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. 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. Combined analysis of saccade velocity and saccade amplitude during the fixation protocol with the EyeSeeCam provides a simple, rapid (<20 s), and reliable tool to differentiate clinically established PSP patients from PD and HCs. As such, our findings prepare the ground for using wearable eye-tracking in patients with uncertain diagnoses.

  7. 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.)

  8. 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.

  9. 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.

  10. Cortical control of vertical and horizontal saccades in progressive supranuclear palsy: An exploratory fMRI study.

    Science.gov (United States)

    Lemos, J; Pereira, D; Almendra, L; Rebelo, D; Patrício, M; Castelhano, J; Cunha, G; Januário, C; Cunha, L; Freire, A; Castelo-Branco, M

    2017-02-15

    Progressive supranuclear palsy (PSP) is a neurodegenerative disorder showing predominant brainstem involvement, characterized by marked slowing of rapid eye movements (saccades), particularly along the vertical plane. While the contribution of the brainstem damage for the saccadic disturbance in PSP has been extensively studied, much less is known about its cortical and subcortical pathomechanisms. We measured reflexive (prosaccades) and voluntary (antisaccades) saccades in the vertical and horizontal plane in PSP patients (n=8) and controls (n=10) in an eye tracking study, followed by the measurement of blood oxygenation-level dependent (BOLD) activation (PSP, n=6; controls, n=10) during similar saccade paradigms. Behaviorally, PSP patients evidenced slower and lower amplitude prosaccades (horizontal and vertical) and lower amplitude antisaccades (vertical) than controls. Functionally, patients showed decreased frontostriatal BOLD activation during prosaccades (horizontal and vertical) and antisaccades (vertical), relative to controls. Additionally, PSP patients showed less default mode network (DMN) deactivation than controls for all types of saccades. Within groups, controls showed no BOLD differences between horizontal and vertical prosaccades while PSP patients demonstrated greater DMN deactivation during vertical prosaccades. Both groups evidenced greater DMN deactivation during vertical antisaccades when compared to their horizontal counterpart and patients further showed relative frontostriatal BOLD hypoactivity during vertical antisaccades. We found fMRI evidence of frontostriatal hypoactivity in PSP patients relative to controls, especially during vertical saccades. These new findings highlight the impact of cortical impairment in saccadic disturbance of PSP. Copyright © 2016 Elsevier B.V. All rights reserved.

  11. Bulbar and spinal muscular atrophy (Kennedy's disease): a review.

    Science.gov (United States)

    Finsterer, J

    2009-05-01

    Bulbar and spinal muscular atrophy (BSMA) is an adult-onset, X-linked recessive trinucleotide, polyglutamine disorder, caused by expansion of a polymorphic CAG tandem-repeat in exon 1 of the androgen-receptor (AR) gene on chromosome Xq11-12. Pathogenetically, mutated AR accumulates in nuclei and cytoplasm of motor neurons, resulting in their degeneration and loss. Phenotypically, patients present with amyotrophic, proximal or distal weakness and wasting of the facial, bulbar and limb muscles, occasionally sensory disturbances, and endocrinologic disturbances, such as androgen resistance, gynecomastia, elevated testosterone or progesterone, and reduced fertility. There may be mild hyper-CK-emia, abnormal motor and sensory nerve conduction studies, and neuropathic and myopathic alterations on muscle biopsy. The golden standard for diagnosing BSMA is genetic analysis, demonstrating a CAG-repeat number >40. No causal therapy is available, but symptomatic therapy should be provided for tremor, endocrinologic abnormalities, sensory disturbances, or muscle cramps. The course is slowly progressive, the ability to walk lost only late in life, only few patients require ventilatory support, and life expectancy only slightly reduced.

  12. Current Status of Treatment of Spinal and Bulbar Muscular Atrophy

    Directory of Open Access Journals (Sweden)

    Fumiaki Tanaka

    2012-01-01

    Full Text Available Spinal and bulbar muscular atrophy (SBMA is the first member identified among polyglutamine diseases characterized by slowly progressive muscle weakness and atrophy of the bulbar, facial, and limb muscles pathologically associated with motor neuron loss in the spinal cord and brainstem. Androgen receptor (AR, a disease-causing protein of SBMA, is a well-characterized ligand-activated transcription factor, and androgen binding induces nuclear translocation, conformational change and recruitment of coregulators for transactivation of AR target genes. Some therapeutic strategies for SBMA are based on these native functions of AR. Since ligand-induced nuclear translocation of mutant AR has been shown to be a critical step in motor neuron degeneration in SBMA, androgen deprivation therapies using leuprorelin and dutasteride have been developed and translated into clinical trials. Although the results of these trials are inconclusive, renewed clinical trials with more sophisticated design might prove the effectiveness of hormonal intervention in the near future. Furthermore, based on the normal function of AR, therapies targeted for conformational changes of AR including amino-terminal (N and carboxy-terminal (C (N/C interaction and transcriptional coregulators might be promising. Other treatments targeted for mitochondrial function, ubiquitin-proteasome system (UPS, and autophagy could be applicable for all types of polyglutamine diseases.

  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. 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

  15. Predicting Early Bulbar Decline in Amyotrophic Lateral Sclerosis: A Speech Subsystem Approach

    Science.gov (United States)

    2015-01-01

    Purpose. To develop a predictive model of speech loss in persons with amyotrophic lateral sclerosis (ALS) based on measures of respiratory, phonatory, articulatory, and resonatory functions that were selected using a data-mining approach. Method. Physiologic speech subsystem (respiratory, phonatory, articulatory, and resonatory) functions were evaluated longitudinally in 66 individuals with ALS using multiple instrumentation approaches including acoustic, aerodynamic, nasometeric, and kinematic. The instrumental measures of the subsystem functions were subjected to a principal component analysis and linear mixed effects models to derive a set of comprehensive predictors of bulbar dysfunction. These subsystem predictors were subjected to a Kaplan-Meier analysis to estimate the time until speech loss. Results. For a majority of participants, speech subsystem decline was detectible prior to declines in speech intelligibility and speaking rate. Among all subsystems, the articulatory and phonatory predictors were most responsive to early bulbar deterioration; and the resonatory and respiratory predictors were as responsive to bulbar decline as was speaking rate. Conclusions. The articulatory and phonatory predictors are sensitive indicators of early bulbar decline due to ALS, which has implications for predicting disease onset and progression and clinical management of ALS. PMID:26136624

  16. Predicting Early Bulbar Decline in Amyotrophic Lateral Sclerosis: A Speech Subsystem Approach

    Directory of Open Access Journals (Sweden)

    Panying Rong

    2015-01-01

    Full Text Available Purpose. To develop a predictive model of speech loss in persons with amyotrophic lateral sclerosis (ALS based on measures of respiratory, phonatory, articulatory, and resonatory functions that were selected using a data-mining approach. Method. Physiologic speech subsystem (respiratory, phonatory, articulatory, and resonatory functions were evaluated longitudinally in 66 individuals with ALS using multiple instrumentation approaches including acoustic, aerodynamic, nasometeric, and kinematic. The instrumental measures of the subsystem functions were subjected to a principal component analysis and linear mixed effects models to derive a set of comprehensive predictors of bulbar dysfunction. These subsystem predictors were subjected to a Kaplan-Meier analysis to estimate the time until speech loss. Results. For a majority of participants, speech subsystem decline was detectible prior to declines in speech intelligibility and speaking rate. Among all subsystems, the articulatory and phonatory predictors were most responsive to early bulbar deterioration; and the resonatory and respiratory predictors were as responsive to bulbar decline as was speaking rate. Conclusions. The articulatory and phonatory predictors are sensitive indicators of early bulbar decline due to ALS, which has implications for predicting disease onset and progression and clinical management of ALS.

  17. 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

  18. Selective frontal neurodegeneration of the inferior fronto-occipital fasciculus in progressive supranuclear palsy (PSP demonstrated by diffusion tensor tractography

    Directory of Open Access Journals (Sweden)

    Elfgren Christina

    2011-01-01

    Full Text Available Abstract Background The clinical presentation in progressive supranuclear palsy (PSP, an atypical parkinsonian disorder, includes varying degrees of frontal dysexecutive symptoms. Using diffusion tensor imaging (DTI and tractography (DTT, we investigated whether diffusion changes and atrophy of the inferior fronto-occipital fasciculus (IFO occurs in PSP and if these changes correlate with disease stage and clinical phenotype. The corticospinal tract (CST, which is often involved in PSP, was investigated for comparison. Methods DTI of the whole brain was performed with a 3 T MR scanner using a single shot-EPI sequence with diffusion encoding in 48 directions. Scans were obtained in patients with PSP (n = 13 and healthy age-matched controls (n = 12. DTT of the IFO and CST was performed with the PRIDE fibre tracking tool (Philips Medical System. Fractional anisotropy (FA and apparent diffusion coefficient (ADC were calculated and correlated with disease stage and clinical phenotype. Results In patients with PSP, significantly decreased FA and increased ADC was found in the frontal part of IFO compared with the medial and occipital parts of IFO, as well as compared to controls. Four of the thirteen patients with PSP showed a marked decrease in the number of tracked voxels in the frontal part of IFO. These findings were most pronounced in patients with severe frontal cognitive symptoms, such as dysexecutive problems, apathy and personality change. There was a strong correlation (r2 = -0.84; p Conclusions DTT for identification of neuronal tracts with subsequent measurement of FA and ADC is a useful diagnostic tool for demonstrating patterns of neuronal tract involvement in neurodegenerative disease. In selected tracts, FA and ADC values might act as surrogate markers for disease stage.

  19. 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

    2017-12-13

    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.

  20. 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.

  1. 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.

  2. 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.

  3. Spin-lattice distribution MRI maps nigral pathology in progressive supranuclear palsy (PSP during life: a pilot study.

    Directory of Open Access Journals (Sweden)

    Michael Hutchinson

    Full Text Available An MRI biomarker for Parkinsonism has long been sought, but almost all attempts at conventional field strengths have proved unsatisfactory, since patients and controls are not separated. The exception is Spin-Lattice Distribution MRI (SLD-MRI, a technique which detects changes in the substantia nigra (SN due to changes in the spin-lattice relaxation time, T1. This easily separates patients with Parkinson's disease (PD from control subjects at 1.5 Tesla, suggesting that it may be sensitive to presymptomatic disease. SLD-MRI demonstrates a topography of signal change within the SN which is the same as the known topography of pathological change, where the lateral portions of the nucleus are more affected than the medial. In a further step towards its validation, we apply SLD-MRI to a disease control, Progressive Supranuclear Palsy (PSP, the most common of the atypical forms of Parkinsonism. In PSP the topography of pathological change in the SN is reversed. We therefore hypothesized that PSP would show a topography of SLD-MRI signal change in the SN that is the reverse of PD (i.e. the medial portion is more affected than the lateral. All 7 patients showed such a topography of MR signal, and all patients were separated from control subjects. Although this is a step toward validation of SLD-MRI with respect to sensitivity and disease specificity, nevertheless we stress that this is a pilot project only. Validation will only be possible when comparing larger cohorts of PSP, PD and control subjects.

  4. Incipient progressive supranuclear palsy is more common than expected and may comprise clinicopathological subtypes: a forensic autopsy series.

    Science.gov (United States)

    Yoshida, Koji; Hata, Yukiko; Kinoshita, Koshi; Takashima, Shutaro; Tanaka, Kortaro; Nishida, Naoki

    2017-05-01

    We investigated 998 serial Japanese forensic autopsy cases (0-101 years old, mean age 61.7 ± 21.9), with no case selection, using immunohistochemistry to detect cases with progressive supranuclear palsy (PSP). Twenty-nine cases (mean age 82.3 ± 7.2 years, 11 males, 18 females) fulfilled the National Institute of Neuronal Disorders and Stroke (NINDS)-PSP pathological criteria (2.9% of all cases, 4.6% of cases over 60). All had neuronal and glial inclusions in the basal ganglia and brainstem. However, 13 cases had low tau pathology and were categorized as atypical PSP. In addition to PSP pathology, multiple types of astrocytic inclusions and comorbid proteinopathies, particularly a high prevalence of argyrophilic grain disease, were found. All cases had not been diagnosed with PSP and had preserved daily functioning prior to death. However, 14 (48.3%), 11 (37.9%), and 16 (55.2%) cases showed signs of dementia, depressive state, and gait disturbance, respectively. Sixteen accidental death cases (55.2%), including from falls and getting lost, and 11 suicide cases (37.9%) appear to have a relationship with incipient PSP pathology. Cluster analysis using the distribution and amount of 4-repeat-tau pathology classified the cases into three subgroups: Group 1 (10 cases) had typical PSP pathology and seven cases (70.0%) had dementia as the most frequent symptom; Group 2 (7 cases) had significantly higher frequency of gait disorder (6 cases, 85.7%), and less neocortical tau pathology than Group 1; Group 3 (12 cases) had relatively mild PSP pathology and high argyrophilic grain burdens. Granular-shaped astrocytes were the dominant astrocytic inclusion in all cases. We conclude that in forensic cases incipient PSP occurs with a higher prevalence than expected. If these findings can be extrapolated to other population-based cohorts, PSP may be more common than previously thought.

  5. 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 ...

  6. 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

    2017-11-21

    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 communicative need in ALS. To investigate how long natural speech will remain functional and to identify the changes in the speech of persons with ALS. Altogether 30 consecutive participants were studied and divided into two groups based on the initial type of ALS, bulbar or spinal. Their speech disorder was evaluated on severity, articulation rate and intelligibility during the 2-year follow-up. The ability to speak deteriorated to poor and necessitated augmentative and alternative communication (AAC) methods with 60% of the participants. Their speech remained adequate on average for 18 months from the first bulbar symptom. Severity, articulation rate and intelligibility declined with nearly all participants during the study. To begin with speech deteriorated more in the bulbar group than in the spinal group and the difference remained during the whole follow-up with some exceptions. The onset of bulbar symptoms indicated the time to loss of speech better than when assessed from ALS diagnosis or the first speech therapy evaluation. In clinical work, it is important to take the initial type of ALS into consideration when determining the urgency of AAC measures as people with bulbar-onset ALS are more susceptible to delayed evaluation and AAC intervention. © 2017 Royal College of Speech and Language Therapists.

  7. Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy.

    Science.gov (United States)

    Montague, Karli; Malik, Bilal; Gray, Anna L; La Spada, Albert R; Hanna, Michael G; Szabadkai, Gyorgy; Greensmith, Linda

    2014-07-01

    Spinal and bulbar muscular atrophy is an X-linked degenerative motor neuron disease caused by an abnormal expansion in the polyglutamine encoding CAG repeat of the androgen receptor gene. There is evidence implicating endoplasmic reticulum stress in the development and progression of neurodegenerative disease, including polyglutamine disorders such as Huntington's disease and in motor neuron disease, where cellular stress disrupts functioning of the endoplasmic reticulum, leading to induction of the unfolded protein response. We examined whether endoplasmic reticulum stress is also involved in the pathogenesis of spinal and bulbar muscular atrophy. Spinal and bulbar muscular atrophy mice that carry 100 pathogenic polyglutamine repeats in the androgen receptor, and develop a late-onset neuromuscular phenotype with motor neuron degeneration, were studied. We observed a disturbance in endoplasmic reticulum-associated calcium homeostasis in cultured embryonic motor neurons from spinal and bulbar muscular atrophy mice, which was accompanied by increased endoplasmic reticulum stress. Furthermore, pharmacological inhibition of endoplasmic reticulum stress reduced the endoplasmic reticulum-associated cell death pathway. Examination of spinal cord motor neurons of pathogenic mice at different disease stages revealed elevated expression of markers for endoplasmic reticulum stress, confirming an increase in this stress response in vivo. Importantly, the most significant increase was detected presymptomatically, suggesting that endoplasmic reticulum stress may play an early and possibly causal role in disease pathogenesis. Our results therefore indicate that the endoplasmic reticulum stress pathway could potentially be a therapeutic target for spinal and bulbar muscular atrophy and related polyglutamine diseases. © The Author (2014). Published by Oxford University Press on behalf of the Guarantors of Brain.

  8. Dorsal onlay graft bulbar urethroplasty using buccal mucosa ...

    African Journals Online (AJOL)

    It can be used for penile urethral stricture repair and different types of dorsal onlay graft urethroplasty for bulbar urethral stricture repair. The current paper describes, step by step, Barbagli technique of dorsal onlay graft bulbar urethroplasty using buccal mucosa. The preoperative patient evaluation and postoperative course ...

  9. 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

    2012-07-01

    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.

  10. [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.

  11. Progressive Supranuclear Palsy

    Science.gov (United States)

    ... and fractures caused by falls. The most common cause of death is pneumonia. With good attention to medical and nutritional needs, ... and fractures caused by falls. The most common cause of death is pneumonia. With good attention to medical and nutritional needs, ...

  12. Progressive Supranuclear Palsy

    Science.gov (United States)

    ... Barré Syndrome Information Page Headache Information Page Hemicrania Continua Information Page Hemifacial Spasm Information Page Hereditary Spastic ... the Spotlight Find NINDS Clinical Trials Patient & Caregiver Education Fact Sheets Hope Through Research Know Your Brain ...

  13. Progressive supranuclear palsy

    Science.gov (United States)

    ... difficulty moving the eyes ( supranuclear opthalmoplegia ), lack of control over the eyes, problems keeping the eyes open Difficulty swallowing Tremors, jaw or face jerks or spasms Mild-to-moderate dementia Personality changes ...

  14. Cross-sectional and longitudinal features of non-fluent/agrammatic primary progressive aphasia with underlying corticobasal degeneration or progressive supranuclear palsy pathology

    Science.gov (United States)

    Santos-Santos, Miguel A.; Mandelli, Maria Luisa; Binney, Richard J.; Ogar, Jennifer; Wilson, Stephen M.; Henry, Maya L.; Hubbard, H. Isabel; Meese, Minerva; Attygalle, Suneth; Rosenberg, Lynne; Pakvasa, Mikhail; Trojanowski, John Q.; Grinberg, Lea T.; Rosen, Howie; Boxer, Adam L.; Miller, Bruce L.; Seeley, William W; Gorno-Tempini, Maria Luisa

    2016-01-01

    Importance We provide novel evidence of specific clinical and neuroimaging features that may help for the in vivo prediction of underlying pathology in non-fluent/agrammatic primary progressive aphasia (nfvPPA) patients with progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD) proved by autopsy. Objective To characterize the neurological, cognitive and neuroimaging features of patients with nvfPPA- in whom either PSP or CBD was eventually confirmed at autopsy- at initial presentation and at 1-year follow-up. Design, Setting, and Participants Prospective longitudinal clinical-pathological study was conducted in a tertiary research clinic that specialized in cognitive disorders. Patients (n=14) were evaluated between January 2002 and December 2014. Inclusion criteria were: a clinical diagnosis of nfvPPA; the availability of speech, language, and cognitive testing for at least one evaluation; magnetic resonance imaging within 6 months of initial evaluation, and a postmortem pathological diagnosis of PSP or CBD. Main Outcomes and Measures Clinical, cognitive, and neuroimaging longitudinal data were analyzed to characterize the whole nfvPPA-4R tau group and identify differences between nfvPPA-PSP and nfvPPA-CBD at presentation and longitudinally. Results Patient groups did not differ significantly in age, gender or handedness (nfvPPA-PSP: median [interquartile range] age 74 [67–76] years, 1/5 male: 1/4 left-handed; nfvPPA-CBD 65 [54–81] years, 3/9 male, 0/9 left-handed). Motor speech impairment and left frontal white matter atrophy were the most prominent common features. At presentation, dysarthria (Motor Speech Examination score median [interquartile range] nfvPPA-PSP: 4 [2–7], nfvPPA-CBD 0 [0–4]; p=0.02), depression (Geriatric Depression Scale score median [interquartile range] nfvPPA-PSP 19 [3–28], nfvPPA-CBD 4 [0–16]; p=0.04) and relatively selective white matter atrophy were typical of nfvPPA-PSP, while greater grey matter atrophy

  15. Scoliosis in Spastic Cerebral Palsy

    OpenAIRE

    J Gordon Millichap

    1998-01-01

    The natural history and risk factors for progressive severe scoliosis were evaluated in 37 institutionalized spastic cerebral palsy patients followed at the Department of Orthopaedic Surgery, Shinshu University School of Medicine, Matsumoto, and at Centers in Nagano, Japan.

  16. Bell's Palsy.

    Science.gov (United States)

    Reich, Stephen G

    2017-04-01

    Bell's palsy is a common outpatient problem, and while the diagnosis is usually straightforward, a number of diagnostic pitfalls can occur, and a lengthy differential diagnosis exists. Recognition and management of Bell's palsy relies on knowledge of the anatomy and function of the various motor and nonmotor components of the facial nerve. Avoiding diagnostic pitfalls relies on recognizing red flags or features atypical for Bell's palsy, suggesting an alternative cause of peripheral facial palsy. The first American Academy of Neurology (AAN) evidence-based review on the treatment of Bell's palsy in 2001 concluded that corticosteroids were probably effective and that the antiviral acyclovir was possibly effective in increasing the likelihood of a complete recovery from Bell's palsy. Subsequent studies led to a revision of these recommendations in the 2012 evidence-based review, concluding that corticosteroids, when used shortly after the onset of Bell's palsy, were "highly likely" to increase the probability of recovery of facial weakness and should be offered; the addition of an antiviral to steroids may increase the likelihood of recovery but, if so, only by a very modest effect. Bell's palsy is characterized by the spontaneous acute onset of unilateral peripheral facial paresis or palsy in isolation, meaning that no features from the history, neurologic examination, or head and neck examination suggest a specific or alternative cause. In this setting, no further testing is necessary. Even without treatment, the outcome of Bell's palsy is favorable, but treatment with corticosteroids significantly increases the likelihood of improvement.

  17. Bell's palsy

    OpenAIRE

    Holland, Julian; Bernstein, Jonathan

    2011-01-01

    Bell's palsy is an idiopathic, unilateral, acute paresis or paralysis of facial movement caused by dysfunction of the lower motor neurone. Up to 30% of people with acute peripheral facial palsy have an alternative cause diagnosed at presentation or during the course of their facial palsy. Alternative causes are higher in children (>50%), warranting specialist evaluation at presentation. Severe pain, vesicles (ear or oral), and hearing loss or imbalance, suggest Ramsay Hunt syndrome caused by ...

  18. Bulbar dysfunction in normal pressure hydrocephalus: a prospective study.

    Science.gov (United States)

    Chankaew, Ekawut; Srirabheebhat, Prajak; Manochiopinig, Sriwimon; Witthiwej, Theerapol; Benjamin, Itsara

    2016-09-01

    OBJECTIVE Normal pressure hydrocephalus (NPH) is clinically characterized by gait disturbance, cognitive impairment, and urinary incontinence, as well as enlargement of the ventricles. To the best of the authors' knowledge, there have been no previous publications regarding the correlation between bulbar dysfunction and NPH. The primary objective of this study was to compare preoperative and postoperative prevalence of bulbar dysfunction in patients with NPH. Secondary objectives included assessing the results of surgery for swallowing, speech, gait, cognition, and urination, and evaluating the correlation between bulbar dysfunction and triad symptoms. METHODS Fifty-three patients with NPH who underwent shunt placement surgery at Siriraj Hospital were included in the study. Patients were evaluated for gait, cognition, urination, swallowing, and speech before and 6 months after shunt placement. Triad symptoms were assessed using standard methods. Bulbar dysfunctions were assessed using the Swallowing Problem Questionnaire, Thai Articulation Test, Resonation Screening Test (RST), and Thai Nasality Test. The Thai Speech Assessment Program and nasometer were used for objective speech measurement. RESULTS Preoperatively, 86% (43/50) of patients had swallowing problems and 75% (37/49) had speech problems, as measured by the RST. Postoperatively, there was significant improvement in swallowing (p nasometer test. All triad symptoms were improved. There were significant correlations between swallowing impairment and gait disturbance (r = 0.358, p = 0.009), and RST and cognitive impairment (r = -0.502, p < 0.001). CONCLUSIONS This is the first study of bulbar dysfunction in patients with NPH. The results showed that the prevalence of bulbar dysfunction is very high. The correlation between bulbar dysfunction and the classic NPH triad has been documented and published. These bulbar symptoms also significantly improved after surgery. As such, bulbar dysfunction should be

  19. Cerebral palsy-management.

    Science.gov (United States)

    Singhi, Pratibha D

    2004-07-01

    Cerebral Palsy encompasses a heterogeneous group of non-progressive motor disorders caused by injury to the developing brain. Management is best done in a multidisciplinary set up under one roof. Comprehensive assessment of the child to evaluate functional ability and associated problems is followed by an individualized plan of management with long term goals and short term objectives. Participation of the family is pivotal to ensure proper habilitation of the child. A home-based management plan is advocated. Considerable experience, sensitivity and understanding are needed both for breaking the news and counselling the parents of a child with cerebral palsy.

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

    Energy Technology Data Exchange (ETDEWEB)

    Hanyu, H.; Asano, T.; Sakurai, H.; Takasaki, M. [Dept. of Geriatric Medicine, Tokyo Medical University (Japan); Shindo, H.; Abe, K. [Dept. of Radiology, Tokyo Medical University (Japan)

    2001-07-01

    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.)

  1. Hypospadias as a novel feature in spinal bulbar muscle atrophy.

    Science.gov (United States)

    Nordenvall, Anna Skarin; Paucar, Martin; Almqvist, Catarina; Nordenström, Anna; Frisén, Louise; Nordenskjöld, Agneta

    2016-04-01

    Spinal and bulbar muscle atrophy (SBMA) is an X-linked neuromuscular disorder caused by CAG repeat expansions in the androgen receptor (AR) gene. The SBMA phenotype consists of slowly progressive neuromuscular symptoms and undermasculinization features as the result of malfunction of the AR. The latter mainly includes gynecomastia and infertility. Hypospadias is also a feature of undermasculinization with an underdeveloped urethra and penis; it has not been described as part of the SBMA phenotype but has been suggested to be associated with a prolonged CAG repeat in the AR gene. This study includes the first epidemiologic description of the co-occurrence of hypospadias and SBMA in subjects and their male relatives in Swedish population-based health registers, as well as an additional clinical case. One boy with severe hypospadias was screened for mutations in the AR gene and was found to have 42 CAG repeats in it, which is in the full range of mutations causing SBMA later in life. We also detected a maximum of four cases displaying the combination of SBMA and hypospadias in our national register databases. This is the third case report with hypospadias in association with CAG repeat expansions in the AR gene in the full range known to cause SBMA later in life. Our findings suggest that hypospadias may be an under diagnosed feature of the SBMA phenotype and we propose that neurologists working with SBMA further investigate and report the true prevalence of hypospadias among patients with SBMA.

  2. Cerebral Palsy (For Teens)

    Science.gov (United States)

    ... Situations Talking to Your Parents - or Other Adults Cerebral Palsy KidsHealth > For Teens > Cerebral Palsy Print A A ... do just what everyone else does. What Is Cerebral Palsy? Cerebral palsy (CP) is a disorder of the ...

  3. Cerebral palsy

    Science.gov (United States)

    ... people who are affected by cerebral palsy) Social stigma When to Contact a Medical Professional Call your ... chap 598. Nass R, Sidhu R, Ross G. Autism and other developmental disabilities. In: Daroff RB, Jankovic ...

  4. 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.

  5. A sphenoid sinus mucocele simulating as retro bulbar optic neuritis

    Directory of Open Access Journals (Sweden)

    Anoop Kishore Gupta

    2012-01-01

    Full Text Available A 25-year-old male presented with complaints of sudden diminution of vision with pain on eye movement in the left eye which was diagnosed clinically as retro bulbar optic neuritis. However, magnetic resonance imaging (MRI showed lesion consistent with sphenoid sinus mucocele. Early surgical removal of mucocele led to complete recovery of vision, contrast and visual field. A high index of suspicion is necessary for intracranial lesions in all cases of retro bulbar neuritis, especially those with atypical symptoms.

  6. 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

  7. Ventral onlay graft bulbar urethroplasty using buccal mucosa

    African Journals Online (AJOL)

    H. Wessells

    2016-01-21

    Jan 21, 2016 ... web page of the journal www.ees.elsevier.com/afju · www.sciencedirect.com. Review. Ventral onlay graft bulbar urethroplasty using ..... use and increase probability of long term success in patients with compromised urethras. Conflict of interest. No conflict of interest. References. [1] Santucci RA, Joyce GF, ...

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

    African Journals Online (AJOL)

    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 almost circumferential substitution with oral graft is necessary, with suboptimal results. If the obliterative segment within a longer ...

  9. Ventral onlay graft bulbar urethroplasty using buccal mucosa ...

    African Journals Online (AJOL)

    Objective: To assess the ongoing role of ventral onlay oral mucosa free graft in the treatment of bulbar urethral stricture. Methods: Detailed review of technical consideration and outcomes from the author's institution along with review of other peer reviewed literature. Results: Of 62 patients undergoing ventral onlay buccal ...

  10. Non-transecting bulbar urethroplasty using buccal mucosa

    African Journals Online (AJOL)

    S. Bugeja

    2015-12-02

    Dec 2, 2015 ... Augmentation urethroplasty using oral mucosal graft has become the standard surgical treatment of long bulbar strictures. .... complications [14]. The legs are supported in Allen® Yellofin® stir- rups. Anti-thromboembolic stockings and pneumatic intermittent calf compression devices are used routinely.

  11. Infantile Erb's palsy (Smellie's palsy).

    Science.gov (United States)

    Johnson, E W; Alexander, M A; Koenig, W C

    1977-04-01

    Our experience with 32 infants seen with Erb's palsy, of whom 11 were followed as long as 18 years, suggests two management axioms. First, it is probably harmful to the infant to use airplane splints. Seocnd, passive stretching of the tight scapulohumeral adductors and passive normal range of motion exercises to shoulder and elbow will usually give a successful outcome in this obstetrical complication. Finally, even though the shoulder muscles regain their strenght, the "Erb engram" may remain as a mark of Erb's palsy.

  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. 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.

  15. (18) F-flortaucipir tau positron emission tomography distinguishes established progressive supranuclear palsy from controls and Parkinson disease: A multicenter study.

    Science.gov (United States)

    Schonhaut, Daniel R; McMillan, Corey T; Spina, Salvatore; Dickerson, Bradford C; Siderowf, Andrew; Devous, Michael D; Tsai, Richard; Winer, Joseph; Russell, David S; Litvan, Irene; Roberson, Erik D; Seeley, William W; Grinberg, Lea T; Kramer, Joel H; Miller, Bruce L; Pressman, Peter; Nasrallah, Ilya; Baker, Suzanne L; Gomperts, Stephen N; Johnson, Keith A; Grossman, Murray; Jagust, William J; Boxer, Adam L; Rabinovici, Gil D

    2017-10-01

    (18) F-flortaucipir (formerly (18) F-AV1451 or (18) F-T807) binds to neurofibrillary tangles in Alzheimer disease, but tissue studies assessing binding to tau aggregates in progressive supranuclear palsy (PSP) have yielded mixed results. We compared in vivo (18) F-flortaucipir uptake in patients meeting clinical research criteria for PSP (n = 33) to normal controls (n = 46) and patients meeting criteria for Parkinson disease (PD; n = 26). Participants underwent magnetic resonance imaging and positron emission tomography for amyloid-β ((11) C-PiB or (18) F-florbetapir) and tau ((18) F-flortaucipir). (18) F-flortaucipir standardized uptake value ratios were calculated (t = 80-100 minutes, cerebellum gray matter reference). Voxelwise and region-of-interest group comparisons were performed in template space, with receiver operating characteristic curve analyses to assess single-subject discrimination. Qualitative comparisons with postmortem tau are reported in 1 patient who died 9 months after (18) F-flortaucipir. Clinical PSP patients showed bilaterally elevated (18) F-flortaucipir uptake in globus pallidus, putamen, subthalamic nucleus, midbrain, and dentate nucleus relative to controls and PD patients (voxelwise p F-flortaucipir in any region. A patient with clinical PSP and pathological diagnosis of corticobasal degeneration had severe tau pathology in PSP-related brain structures with good correspondence between in vivo (18) F-flortaucipir and postmortem tau neuropathology. (18) F-flortaucipir uptake was elevated in PSP versus controls and PD patients in a pattern consistent with the expected distribution of tau pathology. Ann Neurol 2017;82:622-634. © 2017 American Neurological Association.

  16. 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.)

  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. A Rare Case of Progressive Palsy of the Lower Leg during Percutaneous Endoscopic Lumbar Discectomy via a Transforaminal Approach

    Directory of Open Access Journals (Sweden)

    Hiroaki Manabe

    2018-01-01

    Full Text Available Percutaneous endoscopic discectomy (PED for lumbar disc herniation is gaining popularity with the transforaminal (TF approach preferred because it allows surgery under local anesthesia and preserves the spinal muscles. Although this procedure has some characteristic complications, it is rare for PED to be converted to conventional open surgery due to worsening of symptoms intraoperatively. Here, we report PED via the TF approach that required conversion to open surgery. A 20-year-old man with a large disc herniation at L3/4 developed severe progressive leg pain and muscle weakness of the left leg intraoperatively. Magnetic resonance imaging revealed that the size of the herniation was unchanged and the endoscope did not reach the herniated mass. We converted to open surgery, and the patient’s postoperative course was favorable. We discuss the reasons for failure of the approach and suggest planning for an appropriate foraminoplasty to avoid the potential need for conversion to open surgery.

  19. Lower limb strength training in children with cerebral palsy – a randomized controlled trial protocol for functional strength training based on progressive resistance exercise principles

    Directory of Open Access Journals (Sweden)

    Verschuren Olaf

    2008-10-01

    Full Text Available Abstract Background Until recently, strength training in children with cerebral palsy (CP was considered to be inappropriate, because it could lead to increased spasticity or abnormal movement patterns. However, the results of recent studies suggest that progressive strength training can lead to increased strength and improved function, but low methodological quality and incomplete reporting on the training protocols hampers adequate interpretation of the results. This paper describes the design and training protocol of a randomized controlled trial to assess the effects of a school-based progressive functional strength training program for children with CP. Methods/Results Fifty-one children with Gross Motor Function Classification Systems levels I to III, aged of 6 to 13 years, were recruited. Using stratified randomization, each child was assigned to an intervention group (strength training or a control group (usual care. The strength training was given in groups of 4–5 children, 3 times a week, for a period of 12 weeks. Each training session focussed on four exercises out of a 5-exercise circuit. The training load was gradually increased based on the child's maximum level of strength, as determined by the 8 Repetition Maximum (8 RM. To evaluate the effectiveness of the training, all children were evaluated before, during, directly after, and 6 weeks after the intervention period. Primary outcomes in this study were gross motor function (measured with the Gross Motor Function Measure and functional muscle strength tests and walking ability (measured with the 10-meter, the 1-minute and the timed stair test. Secondary outcomes were lower limb muscle strength (measured with a 6 RM test, isometric strength tests, and a sprint capacity test, mobility (measured with a mobility questionnaire, and sport activities (measured with the Children's Assessment of Participation and Enjoyment. Spasticity and range of motion were assessed to evaluate any

  20. 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 ...

  1. 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.

  2. Assessment of the hand in cerebral palsy

    OpenAIRE

    Praveen Bhardwaj; S Raja Sabapathy

    2011-01-01

    Cerebral palsy is the musculoskeletal manifestation of a nonprogressive central nervous system lesion that usually occurs due to a perinatal insult to the brain. Though the cerebral insult is static the musculoskeletal pathology is progressive. Some patients with cerebral palsy whose hands are affected can be made better by surgery. The surgical procedures as such are not very technically demanding but the assessment, decision-making, and selecting the procedures for the given patient make th...

  3. Ventral free oral mucous membrane graft for bulbar urethral stricture.

    Science.gov (United States)

    Haque, M E; Rahman, M A; Islam, M F; Siddique, F H; Uddin, M M; Khondoker, M I; Kaiser, I; Siddiqui, O; Karim, M M; Saha, P; Salam, M A

    2012-10-01

    The use of oral mucous membrane graft onlay urethroplasty represents the most widespread method of bulbar urethral stricture repair. We investigated the short term result of oral mucous membrane graft placed on the ventral surface for management of bulbar urethral stricture. Patients with Bulbar urethral stricture of any length, infection free urinary tract and informed consent for oral mucosa harvesting and urethroplasty were selected for study. We enrolled 108 cases of bulbar urethral stricture patients from January 2004 to July 2009. The mean ± SD preoperative maximum flow rate of 5.2 ± 2.6 ml/sec and mean ± SD PVR 87 ± 58.3 ml were treated by substitution urethroplasty with oral mucous membrane by a single surgical team in a private hospital. Causes of stricture were trauma 26(24.1%), infection 58(53.7%), catheter induced 8(7.4%), post TURP 11(10.2%) and unknown 5(4.6%). Oral mucous membrane was harvested from the cheek or from the inner side of lower lip. Defect of the urethra displayed by longitudinal ventral urethrotomy and the graft was sutured over the edges of the incised urethral mucosa over a 14 Fr latex Foley's catheter. Spongiosum tissue was closed over the graft. Pericatheter urethrogram was performed in all cases to check for the anastomotic leakage and the Catheter was removed after 2 weeks of the procedure. After removal of catheter uroflowmetry & ultrasound scan of bladder were performed to estimate the maximum flow rate and post voidal residue. The patient was followed-up every 3 months with uroflowmetry & ultrasonography. The median (range) age of the patients was 32(21-72) years. Mean follow up period was 36 months (range 12-54). Mean ± SD stricture length was 3.7 ± 2.6 cm. The overall success rate was 91.7%. Mean ± SD flow rate was 23 ± 4.2 ml/sec, mean ± SD post void residue was 25 ± 15.5 ml and patient quality of life (QOL) was excellent in almost all patients. Overall complications were seen in 9(8.3%) cases. Of which

  4. 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 ...

  5. Cerebral Palsy (For Kids)

    Science.gov (United States)

    ... Dieting OK for Kids? Your Teeth Heart Murmurs Cerebral Palsy KidsHealth > For Kids > Cerebral Palsy Print A A A What's in this article? ... the first word you spoke? For kids with cerebral palsy, called CP for short, taking a first step ...

  6. 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.

  7. Cerebral palsy.

    Science.gov (United States)

    Kent, Ruth M

    2013-01-01

    Cerebral palsy affects movement and posture causing activity limitation; it is a lifelong condition, with foreseeable complications. There are evidence-based interventions that will prevent participation restriction. Childhood interventions are generally delivered within multidisciplinary rehabilitation programs. Sadly young adults are often not transferred to an appropriate multidisciplinary adult neurodisability service. An unexplained neurological deterioration should warrant further investigation. Pain is an important underreported symptom and musculoskeletal complaints are prevalent. Disabled adults have less participation socially, in employment, marriage, and independent living related to health problems, discrimination, or lack of access to information, support, and equipment. Evidence-based interventions include a variety of modalities at all International Classification of Functioning, Disability, and Health levels to include support and adaptations. Rehabilitation interventions that have been shown to be effective include surgery in childhood, ankle-foot orthoses, strength training, and electrical stimulation. Management of spasticity is beneficial and has an evidence base. Orthotics and casting are also used. Systematic reviews of upper limb therapies also show the benefit of physical therapy exercise, strengthening, fitness training, and constraint therapy. Occupational therapy has a weaker evidence base than in other disabling conditions but many modalities are transferable. Speech therapy is effective although no specific intervention is better. Psychological wellbeing interventions, including improving self-efficacy, health knowledge, and coping skills, are beneficial. Management of continence, nutrition, and fatigue promote wellbeing. Copyright © 2013 Elsevier B.V. All rights reserved.

  8. Cerebral palsy dysphagia: a systematic review

    OpenAIRE

    Menezes, Edênia da Cunha; Santos, Flávia Aparecida Hora; Alves, Flávia Lôbo

    2017-01-01

    ABSTRACT Cerebral palsy (CP) encompasses a group of definitive and non-progressive conditions, with a non-progressive character and its installation in neonatal period. Motor disorders caused by CP may lead to changes in swallowing, resulting in limitations in the activities that are attributed to non-progressive disorders that do not reach the developing child's brain. This article reviews the clinical aspects of dysphagia in CP and the treatments in the area. Only papers published in the pe...

  9. Familial spastic paraplegia, axonal sensory-motor polyneuropathy and bulbar amyotrophy with facial dysmorphia: new cases of Troyer-like syndrome.

    Science.gov (United States)

    Bertini, E; Sabatelli, M; Di Capua, M; Cilio, M R; Mignogna, T; Federico, A; Tonali, P

    1998-01-01

    We studied two Libyan siblings, born to healthy consanguineous parents, who had suffered from a progressive neurological disorder, characterized by facial dysmorphia, ataxia, spastic paraplegia and an axonal sensory-motor polyneuropathy, since the age of 3 years. The clinical picture progressed slowly over a 6-year period to involve also bulbar and distal limb muscles. Interestingly, we found unusual tubulofilamentous inclusions in peripheral nerves and presynaptic buttons at the neuromuscular junctions. Describing the clinical picture of this presumably new disorder, we comment on the difference from similar conditions.

  10. 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.

  11. Bulbar Paralysis and Facial Paralysis due to Metastatic Hepatocellular Carcinoma: A Case Report and Literature Review

    National Research Council Canada - National Science Library

    Liu, Min; Liu, Shixin; Liu, Bailong; Liu, Bin; Guo, Liang; Wang, Xu; Wang, Qiang; Yang, Shuo; Dong, Lihua

    2016-01-01

    .... We report a case of bulbar and facial paralysis due to SBM from HCC. A 46-year-old Chinese man presented with a hepatic right lobe lesion that was detected during a routine physical examination...

  12. Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia

    OpenAIRE

    Hiroo Ichikawa; Nobuyoshi Takahashi; Soutaro Hieda; Hideki Ohno; Mitsuru Kawamura

    2008-01-01

    The clinical significance and characteristics of writing errors in bulbar-onset amyotrophic lateral sclerosis (ALS) are not clear. We retrospectively investigated writing samples in 19 patients with bulbar-onset ALS without preceding extra-motor symptoms. Co-development of dementia and/or aphasia was also explored and single photon emission computed tomography (SPECT) images of the brain were reviewed. As a result, a high prevalence of writing errors (15 of the 19 patients) was found. Of note...

  13. Cerebral Palsy (CP) Quiz

    Science.gov (United States)

    ... What's this? Submit Button Past Emails Pop Quiz: Cerebral Palsy Language: English (US) Español (Spanish) Recommend on Facebook ... Sandy is the parent of a child with cerebral palsy and the Board President of Gio’s Garden , a ...

  14. Nonalcoholic fatty liver disease in spinal and bulbar muscular atrophy.

    Science.gov (United States)

    Guber, Robert D; Takyar, Varun; Kokkinis, Angela; Fox, Derrick A; Alao, Hawwa; Kats, Ilona; Bakar, Dara; Remaley, Alan T; Hewitt, Stephen M; Kleiner, David E; Liu, Chia-Ying; Hadigan, Colleen; Fischbeck, Kenneth H; Rotman, Yaron; Grunseich, Christopher

    2017-12-12

    To determine the prevalence and features of fatty liver disease in spinal and bulbar muscular atrophy (SBMA). Two groups of participants with SBMA were evaluated. In the first group, 22 participants with SBMA underwent laboratory analysis and liver imaging. In the second group, 14 participants with SBMA were compared to 13 female carriers and 23 controls. Liver biopsies were done in 4 participants with SBMA. Evidence of fatty liver disease was detected by magnetic resonance spectroscopy in all participants with SBMA in the first group, with an average dome intrahepatic triacylglycerol of 27% (range 6%-66%, ref ≤5.5%). Liver dome magnetic resonance spectroscopy measurements were significantly increased in participants with SBMA in the second group relative to age- and sex-matched controls, with average disease and male control measurements of 17% and 3%, respectively. Liver biopsies were consistent with simple steatosis in 2 participants and nonalcoholic steatohepatitis in 2 others. We observed evidence of nonalcoholic liver disease in nearly all of the participants with SBMA evaluated. These observations expand the phenotypic spectrum of the disease and provide a potential biomarker that can be monitored in future studies. Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

  15. 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.

  16. Microcirculation changes of bulbar conjunctiva at primary pterygium

    Directory of Open Access Journals (Sweden)

    A. V. Petrayevsky

    2014-07-01

    Full Text Available Purpose. To reveal microcirculation changes in nasal part of bulbar conjunctiva at various stages of primary pterygium formation using fluorescent angiography.Methods. 10 eyes (10 patients with pinguecula, 15 eyes (14 patients with primary pterygium were investigated. As a control group 10 eyes (10 patients without pathological changes in nasal part of conjunctiva were investigated. Biomicroscopy and anterior segment fluorescent angiography were performed for all patients.Results. Severe changes of microcirculation in nasal part of conjunctiva in patients with pinguecula and pterygium were revealed in comparison with a control group. The defect of terminal limbal arcades confirmed by fluorescent angiography can be a sign of transformation of pinguecula into pterygium. Primary pterygium gets blood supply from posterior conjunctival arteries, capillary network of a semilunar fold and also from returnable branches of anterior ciliary arteries. Neovascularization going from a semilunar fold to a body of pterygium is one of possible components in pathogenesis of pterygium.Conclusion. The obtained data testify about sign of transformation of pinguecula into pterygium, blood supply sources of pterygium and a role of neogenic vessels from a capillary network of a semilunar fold in pathogenesis of this disease.

  17. 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.

  18. In vivo oximetry of human bulbar conjunctival and episcleral microvasculature using snapshot multispectral imaging.

    Science.gov (United States)

    MacKenzie, L E; Choudhary, T R; McNaught, A I; Harvey, A R

    2016-08-01

    Multispectral imaging (MSI) is a well-established technique for non-invasive oximetry of retinal blood vessels, which has contributed to the understanding of a variety of retinal conditions, including glaucoma, diabetes, vessel occlusion, and retinal auto-regulation. We report the first study to use snapshot multi-spectral imaging (SMSI) for oximetry of the bulbar conjunctival and episcleral microvasculature in the anterior segment of the eye. We report the oxygen dynamics of the bulbar conjunctival and episcleral microvasculature at normoxia and at acute mild hypoxia conditions. A retinal-fundus camera fitted with a custom Image-Replicating Imaging Spectrometer was used to image the bulbar conjunctival and episcleral microvasculature in ten healthy human subjects at normoxia (21% Fraction of Inspired Oxygen [FiO2]) and acute mild hypoxia (15% FiO2) conditions. Eyelid closure was used to control oxygen diffusion between ambient air and the sclera surface. Four subjects were imaged for 30 seconds immediately following eyelid opening. Vessel diameter and Optical Density Ratio (ODR: a direct proxy for oxygen saturation) of vessels was computed automatically. Oximetry capability was validated using a simple phantom that mimicked the scleral vasculature. Acute mild hypoxia resulted in a decrease in blood oxygen saturation (SO2) (i.e. an increase in ODR) when compared with normoxia in both bulbar conjunctival (p microvasculature, and consequently, the first study to directly observe the rapid reoxygenation of hypoxic bulbar conjunctival vessels when exposed to ambient air. Oximetry of bulbar conjunctival vessels could potentially provide insight into conditions where oxygen dynamics of the microvasculature are not fully understood, such as diabetes, sickle-cell diseases, and dry-eye syndrome. Oximetry in the bulbar conjunctival and episcleral microvasculature could be complimentary or alternative to retinal oximetry. Copyright © 2016 Elsevier Ltd. All rights reserved.

  19. A randomised, placebo-controlled trial of dutasteride in spinal and bulbar muscular atrophy

    Science.gov (United States)

    Fernández-Rhodes, Lindsay E; Kokkinis, Angela D; White, Michelle J; Watts, Charlotte A; Auh, Sungyoung; Jeffries, Neal O; Shrader, Joseph A; Lehky, Tanya J; Li, Li; Ryder, Jennifer E; Levy, Ellen W; Solomon, Beth I; Harris-Love, Michael O; La Pean, Alison; Schindler, Alice B; Chen, CheunJu; Di Prospero, Nicholas A; Fischbeck, Kenneth H

    2011-01-01

    Summary Background Spinal and bulbar muscular atrophy (SBMA) is caused by polyglutamine expansion in the androgen receptor, which results in ligand-dependent toxicity. Animal models have a neuromuscular deficit that is mitigated by androgen-reducing treatment. Methods We explored the efficacy and safety of the 5-alpha-reductase inhibitor, dutasteride, in a single-site, two-year, double-blind, placebo-controlled clinical trial. Physical, neurophysiological, quality of life, and biochemical outcomes were assessed in 50 ambulatory, symptomatic, genetically confirmed, male SBMA subjects randomised to receive dutasteride or placebo (25 in each group). Findings At 24 months, the placebo group showed a decrease of 5% (−0.30 kg/kg) in the primary outcome measure, change in weight-scaled muscle strength as indicated by quantitative muscle assessment (QMA), and the dutasteride group showed an increase in strength of 1% (+0.14 kg/kg); the difference between the groups (6%; CI 18%, −6%) was not significant. Secondary measures of creatine kinase, muscle strength and function, motor and sensory nerve conduction, activities of daily living, and erectile function did not show a significant difference between the study groups in change from baseline. However, quality of life as measured by the SF-36v2 physical component summary favored dutasteride, while the mental component summary favored placebo. The dutasteride group had fewer falls; there were no other significant differences in reported adverse events. Interpretation This study did not show a significant effect of dutasteride on the progression of muscle weakness in SBMA, although there were secondary indications of benefit. A longer trial duration or larger number of subjects may be needed to show an effect on the disease progression. Performance testing, QMA, and quality of life measures were identified as potentially useful endpoints for future therapeutic trials. Funding National Institutes of Health PMID:21216197

  20. 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.)

  1. 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 ... this condition. Some factors that can cause birth trauma (injury) include: Large baby size (may be seen ...

  2. Trends in cerebral palsy.

    Science.gov (United States)

    Blair, E

    2001-05-01

    The terms trend and cerebral palsy are defined emphazing the non-diagnostic nature of the cerebral palsy label. Criteria necessary for valid estimation of trends include constant methods of estimating population based numerators and denominators over a number of years, which render them hard to obtain, particularly in developing countries. Trends in cerebral palsy are an important source of aetiological hypotheses for congenital cerebral palsy, provide corroborative evidence for existing hypotheses and may direct strategies to prevent post neonatally acquired cerebral palsy. In developed countries the overall frequency of congenital cerebral palsy has changed little during the last decades. However this masks a dramatic increase in the frequency in the infants born most preterm, a decline in those born moderately preterm and little change in those born at term, but the severity of impairments of those born very preterm is decreasing while for those born at term severity in increasing. These changes may be the result of the increasing ability of perinatal care to rescue very vulnerable infants. There is less agreement in trends of post neonatally acquired cerebral palsy which are more sensitive to social well being.

  3. Isolated dysphagia unmasking bulbar neurosarcoidosis and pulmonary sarcoidosis.

    Science.gov (United States)

    Abdallah, Tarek; Abdallah, Mokhtar; Elsayegh, Dany; Chalhoub, Michel; Khoueiry, Georges; Glatman, Alex; Maniatis, Theodore

    2014-06-01

    Dysphagia is a rare manifestation of sarcoidosis. It is more commonly the result of esophageal compression by enlarged mediastinal lymph nodes rather than direct esophageal involvement and rarely secondary to neurosarcoidosis and oropharyngeal dysphagia. We report a 54 year old female presenting with a six month history of worsening dysphagia. She denied respiratory symptoms. Physical exam was normal. ESR was 61 mm/hr. Serum ACE level was 65 mcg/L. Chest X-ray was normal. Esophagram revealed a large amount of contrast pooling in pharyngeal recesses with intermittent laryngeal aspiration. Swallow videofluorography showed a decreased retraction of the base of the tongue, limited laryngeal elevation, and a large amount of contrast pooling in pharyngeal recesses with intermittent laryngeal aspiration. EGD showed a normal opening of the upper esophageal sphincter and the cricopharyngeus appeared normal. Proximal esophageal biopsies were normal. Brain MRI with gadolinium was normal. Lumbar puncture was performed. CSF showed a moderate pleocytosis, a WBC count of 19 with 97% lymphocytes, an elevated total protein level of 85 mg/dl (15-60). Neck CT scan showed no oropharyngeal tissue thickening or infiltration, no masses or enlarged lymph nodes. Chest CT scan showed enlarged intrathoracic lymph nodes and no esophageal compression. Bronchoscopy showed the vocal cords to be intact, and the CD4/CD8 ratio in BAL was 5.3. Subcarinal lymph node EBUS biopsy revealed non caseating granulomas. The patient was started on IV methylprednisolone. Three days later, the swallow videofluorography showed a near complete response to steroids. The patient tolerated regular consistency diet with thin liquids, and she was discharged on a slow taper of prednisone over a period of three months. A unique case of isolated dysphagia unmasking bulbar neurosarcoidosis and pulmonary sarcoidosis is herein reported. Copyright © 2014 Arab Journal of Gastroenterology. Published by Elsevier Ltd. All

  4. Reconstruction of the bulbar urethra using dorsal onlay buccal mucosal grafts: New concepts and surgical tricks

    Directory of Open Access Journals (Sweden)

    Guido Barbagli

    2006-01-01

    Full Text Available Buccal mucosa onlay graft urethroplasty represents one of the most widespread methods for the repair of strictures in the bulbar urethra, because of its thick and highly vascular spongiosum tissue. Recently the location of the patch has become a contentious issue, since we described our original techniques of dorsal onlay graft urethroplasty. The design rationale for this approach was based on the concept that the corporeal body remains a healthy host for receiving a free transplanted tissues. Moreover, graft fixation onto a defined surface may decrease graft shrinkage and sacculation. The success rate using buccal mucosa grafts for the repair of bulbar urethral strictures has generally been high with dorsal or ventral onlay grafts or using an augmented roof-strip anastomotic urethroplasty. We describe here the fundamental concepts of the bulbar urethra reconstruction using buccal mucosal grafts, presenting a new surgical technique of dorsal onlay buccal mucosa graft urethroplasty using fibrin glue.

  5. Bell's Palsy Symptoms

    Science.gov (United States)

    ... may remain, such as a small amount of facial paralysis or reduced movement on one side of your ... I fix my focusing problems resulting from my facial paralysis? Aug 12, 2017 Bell's Palsy and Sensitivity to ...

  6. Dysphagia in cerebral palsy

    National Research Council Canada - National Science Library

    Annamaria Salghetti; Andrea Martinuzzi

    2012-01-01

      Feeding problems are often present in children with neuromotor impairment: dysphagia is usually seen in the most severe form of cerebral palsy and it's defined as the difficulty with any of the four phases of swallowing...

  7. Bell's Palsy (For Kids)

    Science.gov (United States)

    ... person look different because that one side is frozen or droopy. The condition might cause pain and ... is unknown, but most of the time a virus causes Bell's palsy. Another cause, especially in kids ...

  8. Cerebral Palsy in Cinema

    Directory of Open Access Journals (Sweden)

    María Lucila Merino Marcos

    2008-10-01

    Full Text Available In the last twenty-five years, a number of important films have been made in which the protagonist or supporting actor has had, or simulated having, cerebral palsy.  On occasion, these characters have been played by people with disabilities.  In the course of this field’s history, films have portrayed many diverse aspects of cerebral palsy such as healthcare and social and familial obligations.

  9. 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.

  10. Late results of bulbar trigeminal tractotomy. Some remarks on recovery of sensibility.

    Science.gov (United States)

    Moffie, D

    1971-06-01

    Re-examination of eight patients in whom bulbar trigeminal tractotomy had been performed 13 to 15 years previously showed that four had no complaints, and the other four had only very slight complaints about pain. In two patients a Spiller-Frazier operation had been performed after tractotomy, in two patients exairesis of the infraorbital or supraorbital nerve had been done. As bulbar trigeminal tractotomy is a major operation and the risk of recurrence is substantial, the indications for this type of operation have to remain very restricted. Theories to explain the recovery of sensation are discussed. It is possible that regeneration of transected fibres is responsible for the loss of analgesia.

  11. 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.

  12. 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

  13. Bulbar muscle MRI changes in patients with SMA with reduced mouth opening and dysphagia.

    NARCIS (Netherlands)

    Wadman, R.I.; Bruggen, H.W. van; Witkamp, T.D.; Kalaykova, S.I.; Stam, M.; Berg, L.H. van den; Steenks, M.H.; Pol, W.L. van der

    2014-01-01

    OBJECTIVE: We performed a study in patients with proximal spinal muscular atrophy (SMA) to determine the prevalence of reduced maximal mouth opening (MMO) and its association with dysphagia as a reflection of bulbar dysfunction and visualized the underlying mechanisms using MRI. METHODS: We

  14. 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…

  15. Anti-GQ1b-negative Miller Fisher syndrome presented with one-sided horizontal gaze palsy.

    Science.gov (United States)

    Akinci, Gülçin; Oztura, Ibrahim; Hiz-Kurul, Semra

    2010-01-01

    Miller Fisher syndrome classically presents with ophthalmoplegia, ataxia and areflexia. The syndrome may present rarely with atypical clinical features. Whether the central or peripheral nervous system is primarily involved remains controversial. Miller Fisher syndrome usually follows an infection, the most likely being Campylobacter jejuni. Mycoplasma pneumoniae has been reported rarely as the antecedent infectious agent in some patients. Herein, we report a 13-year-old girl with positive mycoplasma immunoglobulin (Ig)M and IgG serology who presented with one-sided horizontal gaze palsy, ataxia, areflexia, and bulbar palsy. Her cranial magnetic resonance imaging was normal and blood serum was negative for anti-GQ1b IgG antibodies.

  16. Ocular, bulbar, limb, and cardiopulmonary involvement in oculopharyngeal muscular dystrophy

    DEFF Research Database (Denmark)

    Witting, N; Mensah, A; Køber, L

    2014-01-01

    OBJECTIVES: To assess skeletal muscle weakness and progression as well as the cardiopulmonary involvement in oculopharyngeal muscular dystrophy (OPMD). MATERIALS AND METHODS: Cross-sectional study including symptomatic patients with genetically confirmed OPMD. Patients were assessed by medical...

  17. Assessment of the hand in cerebral palsy.

    Science.gov (United States)

    Bhardwaj, Praveen; Sabapathy, S Raja

    2011-05-01

    Cerebral palsy is the musculoskeletal manifestation of a nonprogressive central nervous system lesion that usually occurs due to a perinatal insult to the brain. Though the cerebral insult is static the musculoskeletal pathology is progressive. Some patients with cerebral palsy whose hands are affected can be made better by surgery. The surgical procedures as such are not very technically demanding but the assessment, decision-making, and selecting the procedures for the given patient make this field challenging. When done well, the results are rewarding not only in terms of improvement in hand function but also in appearance and personal hygiene, which leads to better self-image and permits better acceptance in the society. This article focuses on the clinical examination, patient selection, and decision-making while managing these patients.

  18. Assessment of the hand in cerebral palsy

    Directory of Open Access Journals (Sweden)

    Praveen Bhardwaj

    2011-01-01

    Full Text Available Cerebral palsy is the musculoskeletal manifestation of a nonprogressive central nervous system lesion that usually occurs due to a perinatal insult to the brain. Though the cerebral insult is static the musculoskeletal pathology is progressive. Some patients with cerebral palsy whose hands are affected can be made better by surgery. The surgical procedures as such are not very technically demanding but the assessment, decision-making, and selecting the procedures for the given patient make this field challenging. When done well, the results are rewarding not only in terms of improvement in hand function but also in appearance and personal hygiene, which leads to better self-image and permits better acceptance in the society. This article focuses on the clinical examination, patient selection, and decision-making while managing these patients.

  19. Cerebral Palsy Litigation

    Science.gov (United States)

    Sartwelle, Thomas P.

    2015-01-01

    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. PMID:25183322

  20. Oculomotor palsy in diabetics.

    Science.gov (United States)

    Lajmi, H; Hmaied, W; Ben Jalel, W; Chelly, Z; Ben Yakhlef, A; Ben Zineb, F; El Fekih, L

    2017-12-28

    Oculomotor palsy is one of the most frequent neuro-ophthalmologic complications of diabetic patients. It generates less interest in the literature than the other ocular manifestations. Our goal was to study the clinical, epidemiological, therapeutic and prognostic characteristics of oculomotor palsy in the diabetic. This is a retrospective study of 24 diabetic patients with oculomotor palsy. The ophthalmological examination emphasized ocular motility. We performed an orthoptic assessment and a Hess-Lancaster test. Neuro-imaging was ordered in case of IIIrd and IVth nerve involvement, bilateral involvement, multiple ocular cranial nerve palsy or associated optic neuropathy. Treatment consisted of glucose management and alternating monocular occlusion or prisms for the diplopia. Data were entered and analyzed on SPSS 11.5 software. The mean age of the patients was 58.5±11.9 years. Binocular diplopia was the main symptom. The oculomotor palsy involved the VIth nerve in 50% of cases and was bilateral in two cases. Three patients also had an optic neuropathy. The mean duration of diabetes was 11.7±11 years; poorly controlled diabetes was found in 75% of cases and an association with diabetic retinopathy was noted in 56% of cases. Long-standing uncontrolled type 2 diabetes, hypertension, coronary artery disease, left ventricular hypertrophy, and elevated hematocrit are the most common risk factors. The VIth nerve is commonly involved. Certain characteristics of the pupillary light reflex can help to differentiate an ischemic insult from an aneurysmal injury to the IIIrd nerve. Copyright © 2017. Published by Elsevier Masson SAS.

  1. 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

  2. Acute deterioration of bulbar function after botulinum toxin treatment for sialorrhoea in amyotrophic lateral sclerosis.

    Science.gov (United States)

    Meijer, Jan-Willem G; van Kuijk, Annet A; Geurts, Alexander C H; Schelhaas, H Jurgen; Zwarts, Machiel J

    2008-04-01

    Transcutaneous botulinum toxin injection in the salivary glands was introduced in 2000 as a new treatment for sialorrhoea in amyotrophic lateral sclerosis (ALS). We describe an ALS patient who developed serious complications of botulinum toxin treatment for sialorrhoea, and we review the relevant literature. A 64-yr-old woman with bulbar ALS for 6 mos was treated for disabling sialorrhoea. She had moderate dysphagia, but she was able to swallow. The submandibular and parotid glands were injected transcutaneously, under ultrasound guidance, with botulinum toxin (Dysport), 80 U on each side. Four days later, her bulbar function rapidly deteriorated, resulting in complete aphagia and anarthria on the fifth day. A PEG catheter was placed. Although according to the literature this treatment can be made safer by cautiously increasing the dosage and injecting the parotid glands first, BTX should not be the first-line treatment of sialorrhoea in ALS; comparative studies of BTX, amitryptiline, scopolamine, and radiation should be performed first.

  3. 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

    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...... in SBMA also occurs independently of motor neuron damage. METHODS: Eight patients with SBMA engaged in regular cycling exercise for 12 weeks. Maximum oxygen uptake (Vo(2max)), maximal work capacity (W(max)), muscle morphology, citrate synthase (CS) activity, body composition, EMG, static strength......) 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...

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

    African Journals Online (AJOL)

    diagnosis, such as Alzheimer's or Parkinson's disease[3] and because of its increasingly recognized clinical diversity, it may be difficult, particularly in resource‑poor settings, to diagnose. PSP. Consequently, the underdiagnosis or misdiagnosis of PSP neurodegenerative disorder is not uncommon.[4]. Recent studies have ...

  5. 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 ...

  6. Genetics Home Reference: spinal and bulbar muscular atrophy

    Science.gov (United States)

    ... affected, which causes progressive problems with swallowing and speech. Additionally, muscle twitches (fasciculations) are common. Some males with the disorder experience unusual breast development (gynecomastia) and may be unable to father a child (infertile). Related Information What does it mean if ...

  7. Decreased Peak Expiratory Flow Associated with Muscle Fiber-Type Switching in Spinal and Bulbar Muscular Atrophy.

    Directory of Open Access Journals (Sweden)

    Shinichiro Yamada

    Full Text Available The aim of this study was to characterize the respiratory function profile of subjects with spinal and bulbar muscular atrophy (SBMA, and to explore the underlying pathological mechanism by comparing the clinical and biochemical indices of this disease with those of amyotrophic lateral sclerosis (ALS. We enrolled male subjects with SBMA (n = 40 and ALS (n = 25 along with 15 healthy control subjects, and assessed their respiratory function, motor function, and muscle strength. Predicted values of peak expiratory flow (%PEF and forced vital capacity were decreased in subjects with SBMA compared with controls. In SBMA, both values were strongly correlated with the trunk subscores of the motor function tests and showed deterioration relative to disease duration. Compared with activities of daily living (ADL-matched ALS subjects, %PEF, tongue pressure, and grip power were substantially decreased in subjects with SBMA. Both immunofluorescence and RT-PCR demonstrated a selective decrease in the expression levels of the genes encoding the myosin heavy chains specific to fast-twitch fibers in SBMA subjects. The mRNA levels of peroxisome proliferator-activated receptor gamma coactivator 1-alpha and peroxisome proliferator-activated receptor delta were up-regulated in SBMA compared with ALS and controls. In conclusion, %PEF is a disease-specific respiratory marker for the severity and progression of SBMA. Explosive muscle strength, including %PEF, was selectively affected in subjects with SBMA and was associated with activation of the mitochondrial biogenesis-related molecular pathway in skeletal muscles.

  8. 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 ...

  9. Agraphia in Bulbar-Onset Amyotrophic Lateral Sclerosis: Not Merely a Consequence of Dementia or Aphasia

    Directory of Open Access Journals (Sweden)

    Hiroo Ichikawa

    2008-01-01

    Full Text Available The clinical significance and characteristics of writing errors in bulbar-onset amyotrophic lateral sclerosis (ALS are not clear. We retrospectively investigated writing samples in 19 patients with bulbar-onset ALS without preceding extra-motor symptoms. Co-development of dementia and/or aphasia was also explored and single photon emission computed tomography (SPECT images of the brain were reviewed. As a result, a high prevalence of writing errors (15 of the 19 patients was found. Of note were isolated writing errors with neither dementia nor aphasia verified in 2 patients whose dysarthria was mild enough to evaluate spoken language. The remaining 13 patients also showed agraphia, but either dysarthria was too severe to evaluate aphasia or frontotemporal dementia (FTD-like features co-existed. Of these patients, one who initially lacked dementia subsequently developed FTD-like features. The frequent writing errors were omission or substitution of kana letters and syntactic errors. SPECT images showed bilateral or left-side dominant hypoperfusion in the frontotemporal lobes as a consistent feature. These results show that patients with bulbar-onset ALS frequently exhibit agraphic writing errors and that these are not merely consequences of dementia or aphasia. However, these writing errors may indicate the involvement of frontotemporal language-related areas beyond the primary motor cortex.

  10. Agraphia in bulbar-onset amyotrophic lateral sclerosis: not merely a consequence of dementia or aphasia.

    Science.gov (United States)

    Ichikawa, Hiroo; Takahashi, Nobuyoshi; Hieda, Soutaro; Ohno, Hideki; Kawamura, Mitsuru

    2008-01-01

    The clinical significance and characteristics of writing errors in bulbar-onset amyotrophic lateral sclerosis (ALS) are not clear. We retrospectively investigated writing samples in 19 patients with bulbar-onset ALS without preceding extra-motor symptoms. Co-development of dementia and/or aphasia was also explored and single photon emission computed tomography (SPECT) images of the brain were reviewed. As a result, a high prevalence of writing errors (15 of the 19 patients) was found. Of note were isolated writing errors with neither dementia nor aphasia verified in 2 patients whose dysarthria was mild enough to evaluate spoken language. The remaining 13 patients also showed agraphia, but either dysarthria was too severe to evaluate aphasia or frontotemporal dementia (FTD)-like features co-existed. Of these patients, one who initially lacked dementia subsequently developed FTD-like features. The frequent writing errors were omission or substitution of kana letters and syntactic errors. SPECT images showed bilateral or left-side dominant hypoperfusion in the frontotemporal lobes as a consistent feature. These results show that patients with bulbar-onset ALS frequently exhibit agraphic writing errors and that these are not merely consequences of dementia or aphasia. However, these writing errors may indicate the involvement of frontotemporal language-related areas beyond the primary motor cortex.

  11. Growth hormone deficiency and cerebral palsy

    Directory of Open Access Journals (Sweden)

    Jesús Devesa

    2010-09-01

    Full Text Available Jesús Devesa1,2, Nerea Casteleiro2, Cristina Rodicio2, Natalia López2, Pedro Reimunde1,21Department of Physiology, School of Medicine of Santiago de Compostela, Spain; 2Medical Center Proyecto Foltra, 15886 Teo, SpainAbstract: Cerebral palsy (CP is a catastrophic acquired disease, occurring during development of the fetal or infant brain. It mainly affects the motor control centres of the developing brain, but can also affect cognitive functions, and is usually accompanied by a cohort of symptoms including lack of communication, epilepsy, and alterations in behavior. Most children with cerebral palsy exhibit a short stature, progressively declining from birth to puberty. We tested here whether this lack of normal growth might be due to an impaired or deficient growth hormone (GH secretion. Our study sample comprised 46 CP children, of which 28 were male and 18 were female, aged between 3 and 11. Data obtained show that 70% of these children lack normal GH secretion. We conclude that GH replacement therapy should be implemented early for CP children, not only to allow them to achieve a normal height, but also because of the known neurotrophic effects of the hormone, perhaps allowing for the correction of some of the common disabilities experienced by CP children.Keywords: growth hormone, IGF-I, cerebral palsy, short stature

  12. Cerebral palsy and aging

    OpenAIRE

    Haak, Peterson; Lenski, Madeleine; Hidecker, Mary Jo Cooley; Li, Min; Paneth, Nigel

    2009-01-01

    Cerebral palsy (CP), the most common major disabling motor disorder of childhood, is frequently thought of as a condition that affects only children. Deaths in children with CP, never common, have in recent years become very rare, unless the child is very severely and multiply disabled. Thus, virtually all children assigned the diagnosis of CP will survive into adulthood. Attention to the adult with CP has been sparse, and the evolution of the motor disorder as the individual moves through ad...

  13. Pseudobulbar palsy associated with trismus.

    Science.gov (United States)

    Lai, M. M.; Howard, R. S.

    1994-01-01

    A 60 year old patient presented with an acute pseudobulbar palsy associated with trismus. A computed tomography scan revealed low attenuation areas consistent with infarction affecting the genu of the internal capsules bilaterally. Trismus has not previously been described as the presenting feature of a pseudobulbar palsy. Images Figure 1 PMID:7824418

  14. Pseudobulbar palsy associated with trismus.

    OpenAIRE

    Lai, M M; Howard, R. S.

    1994-01-01

    A 60 year old patient presented with an acute pseudobulbar palsy associated with trismus. A computed tomography scan revealed low attenuation areas consistent with infarction affecting the genu of the internal capsules bilaterally. Trismus has not previously been described as the presenting feature of a pseudobulbar palsy.

  15. 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.

  16. Reflex postural control of patients with cerebral palsy for odontological assistance

    OpenAIRE

    Aguiar, Sandra Maria Herondina Coelho Ávila de [UNESP; Rezende, Maria Cristina Rosifini Alves [UNESP

    2013-01-01

    Cerebral palsy (CP) describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.A child with cerebral palsy may have impairments in motor control, which contributes to loss of functional abilities in posture and mobility. The severity of the impairment on the neuromuscular system determines the variations of functional mobility in chil...

  17. Eagle's syndrome with facial palsy

    OpenAIRE

    Al-Hashim, Mohammed; Al-Jazan, Nasser; Abdulqader, Abdulrahman; Al-Ghamdi, Mohammed

    2017-01-01

    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 sc...

  18. 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.

  19. 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.

  20. Conjunctival Impression Cytology and Bulbar Surface Epithelium Changes in Patients with Psoriasis

    Directory of Open Access Journals (Sweden)

    Sevda Söker

    2007-01-01

    Full Text Available In this study, we evaluated bulbar surface epithelium changes with conjunctival impression cytology (IC in patients with psoriasis. Our study group consisted of 32 psoriatic patients (64 eyes, who were followed up at Dermatology Department of Dicle University Hospital. Control group comprised 32 healthy volunteers (64 eyes who had no abnormality on routine ophthalmological examination and were in the same age and sex distribution. Specimens for conjunctival IC were obtained with a cellulose acetate filter paper from the upper bulbar conjunctiva and fixed with 70 % ethyl alcohol, 37 % formaldehyde and 20:1:1 glicial asetic acid solution. Specimens were stained with periodic acid Schiff’s and Hematoxylin-eosin. The grades of Nelson system were evaluated with light microscopy. Of the patients with psoriasis, 39 % had grade 0, 36 % grade I, and 25 % grade II conjunctival IC differentiation compared with 78, 22, and 0 %, respectively in the control group (p< 0.001. Snake-like appearance of nuclear chromatin in conjunctival epithelial cells was demonstrated in 3 % of eyes in group I but in no eyes in group II. In conclusion, we showed that there could be early conjunctival changes and squamose metaplasia as well as increased goblet cell density in patients with psoriasis when compared with control group.

  1. [Two cases of familial amyotrophic lateral sclerosis with a SOD1L126S mutation showing high age at onset and slow progression].

    Science.gov (United States)

    Iwashima, Tomo; Tateishi, Takahisa; Yamasaki, Ryo; Motomura, Kyoko; Ohyagi, Yasumasa; Kira, Jun-Ichi

    2010-03-01

    An 80-year-old man (patient 1) was admitted to our hospital with numbness of the right leg and weakness of the lower extremities, predominantly in the right leg, for 1 year previously. Neurological examination revealed moderate weakness without atrophy, and fasciculation in the bilateral lower extremities. No hyperreflexia was noted, and the plantar response was flexor. Neither bulbar palsy nor sensory disturbance was observed. Electromyography (EMG) showed a chronic neurogenic pattern, including giant motor unit potentials and reduced interference in all four limb muscles. MRI images of the cervical and lumbar spines showed severe age-related spondylosis. The clinical and laboratory findings led to a diagnosis of spinal progressive muscular atrophy. Motor paralysis progressed slowly for the following four years, culminating in respiratory failure. A 79-year-man, the younger brother of patient 1 (patient 2), suffered from gait disturbance for 3 years before the admission to our hospital. During the following 3 years, bilateral leg weakness developed, causing difficulty walking. Neurological examination revealed a diffuse mild weakness with atrophy and fasciculation in the bilateral lower extremities; the right deltoid muscle was also mildly weak. Mild hyperreflexia was also noted on the left side, and the plantar response was extensor on the left. EMG showed acute and chronic neurogenic patterns in the four limb muscles. Because the missense mutation c.377 T > C; p.L126S was found on exon 5 of the superoxide dismutase (SOD) 1 gene in this patient, a diagnosis of familial ALS was made. Eight patients reported as familial ALS with the SOD1L126S mutation, including the present cases, all developed an onset of weakness in the lower extremities, but showed few upper motor neuron signs. It is important to consider the possibility of this type of familial ALS in a case of spinal progressive muscular atrophy with late-onset and mild progression, if family history is

  2. CEREBRAL PALSY : ANTENATAL RISK FACTORS

    OpenAIRE

    Srinivasa Rao; Vidyullatha; Subbalakshmi

    2015-01-01

    INTRODUCTION: Cerebral palsy (CP) is a group of permanent movement disorders that appear in early childhood. Cerebral palsy is caused by abnormal development or damage to the parts of the brain that control movement, balance, and posture. Most often the problems occur during pregnancy; however, they may also occur during childbirth, or shortly after birth. Often the cause is unknown. AIM: To study the different antenatal maternal risk factors associated with cere...

  3. Convergence palsy secondary to amoxicillin.

    Science.gov (United States)

    Pérez-Roca, F; Alfaro Juárez, A; Sánchez Merino, C; Navarro Mingorance, A

    2016-12-01

    We present the case of an 11-year-old boy with acute diplopia in near vision secondary to transient convergence palsy, possibly in relation to amoxicillin. Convergence palsy is an uncommon eye disorder. The causes are reviewed, and amoxicilin is identified as presumptive etiologic agent. This is the first case reported. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. 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)

  5. Cerebral Palsy: An Overview.

    Science.gov (United States)

    Gulati, Sheffali; Sondhi, Vishal

    2017-11-20

    Cerebral palsy (CP) is a neurodevelopmental disorder characterized by abnormalities of muscle tone, movement and motor skills, and is attributed to injury to the developing brain. The clinical features of this entity evolve over time and the specific CP syndrome may be recognizable only after 3-5 y of age; although suggestive signs and symptoms may be present at an earlier age. The management involves neurological rehabilitation (addressing muscle tonal abnormalities, and devising physical and occupational therapies) and diagnosis and management of co-morbidities (including epilepsy, impairment of cognition, vision, hearing, and disturbances of growth and gastrointestinal function). The management, therefore, is multidisciplinary involving the treating physician working with a team of rehabilitation-, orthopedic-, psychologic-, and social care- providers.

  6. 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 ...

  7. 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, P<0.05). This was the first study to determine the sampling 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.

  8. 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

  9. [Motor replacement surgery via tendon transfer in radial nerve palsy].

    Science.gov (United States)

    Koulaxouzidis, G; Stark, G B; Lampert, F M

    2015-02-01

    Restoration of active extension of wrist, thumb and digits by muscle-tendon transposition. Radial nerve palsy due to peripheral nerve injury. Peripheral nerve disease. Muscle or tendon injury. Restoration of wrist extension in high radial nerve palsy. Reversible distal radial nerve palsy, absence of suitable donor muscles, spasticity, limited range of motion of affected joints, extensive scarring and inappropriate soft tissue conditions, unjustifiable loss of function at donor site. Reinnervated donor muscles, progressive muscle disease, insufficient patient compliance. Dissection of the flexor carpi ulnaris, palmaris longus and pronator teres tendon insertion. Transposition of the tendons. Interweaving of tendons of the pronator teres and extensor carpi radialis brevis muscles, the extensor digitorum communis and flexor carpi ulnaris muscles, as well as the extensor pollicis longus and palmaris longus muscles using the Pulvertaft technique. 3 Weeks immobilization in forearm splint. Additional immobilization for 2 weeks at night. Subsequently, intensive physical and occupational therapy for another 4-6 weeks is required, starting 3 weeks postoperatively. The procedure was carried out in 12 patients over the past 14 years. We treated proximal radial nerve palsy in nine cases. In accordance with the current medical literature, we consider the described motor replacement surgery a reliable procedure.

  10. Risk factors for cerebral palsy.

    Science.gov (United States)

    Suvanand, S; Kapoor, S K; Reddaiah, V P; Singh, U; Sundaram, K R

    1997-01-01

    Cerebral palsy is a major cause of crippling in children, but it's etiology is poorly understood. This case control study was done to assess some of the identified risk factors for cerebral palsy, 125 cerebral palsy cases selected from hospital clinic and 125 age and sex matched neighbourhood controls, all aged less than 5 years and residing in Delhi (India) were studied. Information regarding antenatal, natal and postnatal period was collected by mother's interview, and wherever available, from hospital records with the study subjects. Most common type of cerebral palsy was spastic (88%). Quadriplegia was the commonest topographical subtype (86.4%). Birth asphyxia was found to be present in only 25.6% of cases. The commonest risk factor amongst cases was low birth weight (28.8%). The multivariate odds ratios (confidence limits) for the risk factors found to be significantly associated with cerebral palsy were 36.1 (7.76-160) for birth asphyxia, 13.8 (4.95-38.3) for low birth weight, 37.4 (4.47-313) for neonatal convulsion, 23 (4.7-112) for neonatal jaundice, 14.4 (3.69-56.4) for neonatal infection, 24.9 (2.78-223) for instrument assisted delivery and 15.4 (1.57-152) for antepartum hemorrhage. Precipitate labour, caesarean section, twins, toxemia, breech delivery and head injury were not found to be significantly associated with cerebral palsy. Thus birth asphyxia, low birth weight, neonatal convulsions, neonatal jaundice, neonatal infection, instrument assisted delivery and antepartum hemorrhage are significant risk factors for cerebral palsy.

  11. 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.)

  12. 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…

  13. The difficulty identifying intoeing gait in cerebral palsy.

    Science.gov (United States)

    O'Sullivan, R; Kiernan, D; Walsh, M; Brien, T O

    2013-05-01

    In-toeing in children with cerebral palsy can lead to functional difficulties during gait. This may require surgical management to restore a normal foot progression angle. For this reason it is important to indentify the presence of internal rotation and to establish where the abnormal rotation is occurring. This can be done relatively easily in otherwise healthy subjects by examining foot progression angle as the subject walks towards the assessor. In cerebral palsy the often severely affected gait pattern and potential asymmetry at the pelvis means that in-toeing may be more difficult to identify. Gait laboratory data of 245 subjects with cerebral palsy were studied retrospectively. Of these 102 (41.63%) demonstrated in-toeing relative to the pelvis of one or both limbs. Eleven diplegic subjects (16.18%) in-toed bilaterally giving a total of 113 in-toeing limbs for analysis. Of those, 17 (50%) hemiplegic limbs and 20 (25.32%) diplegic limbs demonstrated a foot progression angle within normal limits due to compensations at the pelvis.

  14. Epidemiologic associations with cerebral palsy.

    Science.gov (United States)

    O'Callaghan, Michael E; MacLennan, Alastair H; Gibson, Catherine S; McMichael, Gai L; Haan, Eric A; Broadbent, Jessica L; Goldwater, Paul N; Dekker, Gustaaf A

    2011-09-01

    To estimate epidemiologic risk factors for cerebral palsy. Data were collected by linkage to state-based perinatal repositories and cerebral palsy registers and using a maternal questionnaire. The cohort included 587 individuals with cerebral palsy and 1,154 non-cerebral palsy controls. The following factors were associated with cerebral palsy: recorded maternal infection during pregnancy (41.4% patients compared with 31.3% controls; odds ratio [OR] 1.55, 95% confidence interval 1.26-1.91), small for gestational age ([birth weight less than third customized centile] 43.9% patients compared with 6.3% controls; OR 11.75, 6.25-22.08), gestational age less than 32 weeks (29.3% patients compared with 0.7% controls; OR 59.20, 28.87-121.38), multiple birth (OR 6.62, 4.00-10.95), a relative with cerebral palsy (OR 1.61, 1.12-2.32), breech position (13.7% patients compared with 6.0% controls; OR 2.48, 1.76-3.49), bleeding at any time in pregnancy (29.3% patients compared with 16.9% controls; OR 2.04, 1.61-2.58), male sex (58.8% patients compared with 45.8% controls; OR 1.68, 1.38-2.06), multiple miscarriage (7.7% patients compared with 3.5% controls; OR 2.30, 1.38-3.82), smoking (14.0% patients compared with 10.6% controls; OR 1.37, 1.02-1.85), and illicit drug use (3.3% patients compared with 1.5% controls; OR 2.22, 1.14-4.30). Factors not associated with cerebral palsy were "disappearing twin," diabetes, maternal body mass index, hypertension, alcohol consumption, anemia, maternal hypothyroidism, forceps or vacuum delivery, and maternal age. Preterm birth, intrauterine growth restriction, perinatal infection, and multiple birth present the largest risks for a cerebral palsy outcome. Reassuringly, upper respiratory tract and gastrointestinal infections during pregnancy were not associated with cerebral palsy. II.

  15. [Emotional impact of facial palsy].

    Science.gov (United States)

    Dobel, C; Miltner, W H R; Witte, O W; Volk, G F; Guntinas-Lichius, O

    2013-01-01

    Facial palsy is not only a movement disorder but leads also to an emotional and communicative disorder in chronic stage but also in some patients already during the acute phase of the disease. The present review describes the current knowledge of the neurobiological and psychological fundamentals on the relation of facial movement and its emotional context. So far there is not much knowledge on the impact of a facial palsy on the interaction between facial movement, emotional processing and communicative skills of the patient. The emotional contagion seems to be reduced in patients with facial palsy. The ability to express emotions seems also to be reduced. Moreover, the patients feel to be perceived negatively. In fact, most of the expressions of patients with facial palsy are allocated with a negative affect even when the patients are smiling. Patients with facial palsy react with negative stress, anxiety and depression. The patients avoid social contacts. In turn, this reinforces the communicative disorder. The otorhinolaryngologist can use the Facial Disability Index as a simple questionnaire to detect such dysfunctions. Diagnostics that are necessary to develop a therapy program are presented in this review. Standardized therapy concepts that are not only treat the movement disorder but also the emotional context is missing so far. Finally, the review will give an outlook on potential therapy strategies. © Georg Thieme Verlag KG Stuttgart · New York.

  16. Short term general anesthesia for retro-bulbar block in ophthalmic surgery generates no significant hypercapnia.

    Science.gov (United States)

    Baulig, Werner; Weber, Monica; Beck-Schimmer, Beatrice; Theusinger, Oliver M; Biro, Peter

    2017-03-11

    To assess the impact of short time hypnosis for retro-bulbar anesthesia on ventilation in patients undergoing ophthalmic surgery of the anterior eye chamber. In all patients, a combined continuous transcutaneous carbon dioxide tension (PtcCO2) and partial oxygen saturation (SpO2) measurement was applied in addition to routine monitoring. To enable unconscious application of retro-bulbar anesthesia, intravenous thiopental was given in one to multiple bolus doses. Transient breathing support included chin lift, Esmarch maneuver and manual hand-bag ventilation via face mask. Main endpoints were apnea time, recovery time according to the Richmond Agitation Sedation Scale, as well as SpO2 and PtcCO2 readings at predefined time points. Fifty-two patients with a mean age of 68 ± 13 years were included. Average thiopental dose was 2.7 ± 0.6 mg/kg. In seven (13.5%) patients repeated doses of thiopental were necessary to a total of 3.3 ± 1.1 mg/kg. Except one patient, no severe, significant or clinical relevant hypercapnia or desaturation periods were observed, and the occurring elevation of PtcCO2 values did not correlate with the application of repeated doses of thiopental or the need for the Esmarch maneuver. Higher PtcCO2 values were associated with the presence of hypertension and smoking. Apnea (p < 0.001) and recovery (p = 0.003) time were significantly prolonged in the patients needing the Esmarch maneuver. Short term anesthesia with thiopental in ophthalmic surgery is associated with a mild but not clinically relevant hypercapnia.

  17. Delayed facial palsy in Miller Fisher syndrome.

    Science.gov (United States)

    Tan, Cheng-Yin; Yuki, Nobuhiro; Shahrizaila, Nortina

    2015-11-15

    Miller Fisher syndrome is characterised by the triad of ophthalmoplegia, ataxia and areflexia. However, facial palsy can occur during the course of the illness although development of facial palsy when other cardinal signs of Miller Fisher syndrome have reached nadir or improving, is unusual. This delayed appearance of facial palsy can be easily overlooked by the treating clinician. Here, we report four patients with Miller Fisher syndrome and delayed-onset facial palsy. We discuss the possible underlying reasons behind the delay in facial palsy. Copyright © 2015 Elsevier B.V. All rights reserved.

  18. The origin of "Saturday night palsy"?

    Science.gov (United States)

    Spinner, Robert J; Poliakoff, Michael B; Tiel, Robert L

    2002-09-01

    THE TERM Saturday night palsy has become synonymous with radial nerve compression in the arm resulting from direct pressure against a firm object. It typically follows deep sleep on the arm, often after alcohol intoxication. The commonly accepted origin of the phrase is the association of Saturday night with carousing. We offer an alternate explanation: we think that the term Saturday night palsy was introduced mistakenly as a simplification of saturnine palsy (much like the way the word palsy was shortened from paralysis). Saturnine palsy, which is a relatively common complication of lead poisoning, has the same clinical presentation of radial nerve compression, and Saturday night palsy even sounds like saturnine palsy. Moreover, Saturday, lead, carousing, and alcohol are associated with each other through their connection to Saturn, the Roman god of agriculture, which encourages the association of the two syndromes with one another.

  19. 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....... The population analyzed was retrieved from the Danish Cerebral Palsy Register, which covers the eastern part of the country and has registered about half of the Danish population of individuals with CP since 1950. For this study we analyzed 2367 individuals with CP, who were born in 1930 to 2000 and were alive...

  20. [Palsy of the upper limb: Obstetrical brachial plexus palsy, arthrogryposis, cerebral palsy].

    Science.gov (United States)

    Salazard, B; Philandrianos, C; Tekpa, B

    2016-10-01

    "Palsy of the upper limb" in children includes various diseases which leads to hypomobility of the member: cerebral palsy, arthrogryposis and obstetrical brachial plexus palsy. These pathologies which differ on brain damage or not, have the same consequences due to the early achievement: negligence, stiffness and deformities. Regular entire clinical examination of the member, an assessment of needs in daily life, knowledge of the social and family environment, are key points for management. In these pathologies, the rehabilitation is an emergency, which began at birth and intensively. Splints and physiotherapy are part of the treatment. Surgery may have a functional goal, hygienic or aesthetic in different situations. The main goals of surgery are to treat: joints stiffness, bones deformities, muscles contractures and spasticity, paresis, ligamentous laxity. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  1. Cerebral Palsy: A Lifelong Challenge Asks for Early Intervention

    Science.gov (United States)

    Panteliadis, Christos P; Hagel, Christian; Karch, Dieter; Heinemann, Karl

    2015-01-01

    One of the oldest and probably well-known examples of cerebral palsy is the mummy of the Pharaoh Siptah about 1196–1190 B.C., and a letter from Hippocrates (460–390 B.C.). Cerebral palsy (CP) is one of the most common congenital or acquired neurological impairments in paediatric patients, and refers to a group of children with motor disability and related functional defects. The visible core of CP is characterized by abnormal coordination of movements and/or muscle tone which manifest very early in the development. Resulting from pre- or perinatal brain damage CP is not a progressive condition per se. However, without systematic medical and physiotherapeutic support the dystonia leads to muscle contractions and to deterioration of the handicap. Here we review the three general spastic manifestations of CP hemiplegia, diplegia and tetraplegia, describe the diagnostic procedures and delineate a time schedule for an early intervention. PMID:26191093

  2. 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.

  3. Molybdenum cofactor deficiency mimics cerebral palsy: differentiating factors for diagnosis.

    Science.gov (United States)

    Kikuchi, Kenjiro; Hamano, Shin-ichiro; Mochizuki, Hiroshi; Ichida, Kimiyoshi; Ida, Hiroyuki

    2012-08-01

    We describe an infant with molybdenum cofactor deficiency, initially diagnosed as cerebral palsy. Clinical features of molybdenum cofactor deficiency, e.g., neonatal seizures, hypertonus/hypotonus, and feeding and respiratory difficulties, resemble those of neonatal hypoxic-ischemic encephalopathy. Our patient, a 2-year-old boy, presented with spastic quadriplegia and mental retardation. He manifested intractable neonatal seizures and diffuse cerebral atrophy. When admitted with bronchitis at age 18 months, his uric acid levels in blood and urine were undetectable. A urinary sulfite test revealed positive results. Further tests revealed elevated urinary levels of xanthine, hypoxanthine, and S-sulfocystein. Sequencing of the MOCS2A gene revealed heterozygosity for c.[265T>C] + [266A>G], diagnosed as molybdenum cofactor deficiency type B. Neonatal seizures, progressive cerebral atrophy, and low serum levels of uric acid may provide diagnostic clues in patients with cerebral palsy of undetermined cause. Copyright © 2012 Elsevier Inc. All rights reserved.

  4. MRI in Congenital Facial Palsy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-03-01

    Full Text Available Magnetic resonance (MR findings in a 12-month-old boy with congenital unilateral facial palsy and a 9-month-old girl with atypical Moebius syndrome are reported from the National Center of Neurology and Psychiatry, Kodaira, Japan.

  5. Ocular defects in cerebral palsy

    OpenAIRE

    Katoch Sabita; Devi Anjana; Kulkarni Prajakta

    2007-01-01

    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.

  6. 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.

  7. Ocular defects in cerebral palsy.

    Science.gov (United States)

    Katoch, Sabita; Devi, Anjana; Kulkarni, Prajakta

    2007-01-01

    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.

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

    Science.gov (United States)

    ... Health Care Decisions Cerebral Palsy Checklist: Teens & Young Adults KidsHealth > For Parents > Cerebral Palsy Checklist: Teens & Young ... plan healthy meals. continue Step 3: Explore Young-Adult Education Young adults with cerebral palsy are entitled ...

  9. Association of Lead Levels and Cerebral Palsy

    OpenAIRE

    Neha Bansal MD; Anju Aggarwal MD, FIAP, MNAMS; M. M. A. Faridi MD, FIAP, MNAMS; Tusha Sharma PhD; B. D. Baneerjee PhD

    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 leve...

  10. Altered arm posture in children with cerebral palsy is related to instability during walking

    NARCIS (Netherlands)

    Meyns, P.; Desloovere, K.; Gestel, L. Van; Massaad, F.; Smits-Engelsman, B.C.M.; Duysens, J.E.J.

    2012-01-01

    BACKGROUND: Toddlers learning to walk adopt specific 'guard' arm postures to maintain their balance during forward progression. In Cerebral Palsy (CP), the cause of the altered arm postures during walking has not been studied. AIM: To investigate whether the altered arm posture in children with CP

  11. Mandibular dysfunction as a reflection of bulbar involvement in SMA type 2 and 3.

    Science.gov (United States)

    van Bruggen, H Willemijn; Wadman, Renske I; Bronkhorst, Ewald M; Leeuw, Maureen; Creugers, Nico; Kalaykova, Stanimira I; van der Pol, W Ludo; Steenks, Michel H

    2016-02-09

    In a cross-sectional study, we aimed to determine (1) the effect of spinal muscular atrophy (SMA) type 2 and 3 on mandibular function reflected as masticatory performance, mandibular range of motion, and bite force and (2) the predictors of mandibular dysfunction. Sixty patients with SMA type 2 and 3 (mean age 32.3 years, SD 17.4 years) and 60 age-matched controls filled out questionnaires about impairments of mandibular function. All participants underwent detailed clinical examination to document the mandibular range of motion including maximal mouth opening, bite force, and masticatory function. All mandibular movements, including mouth opening, lateral range of motion, and protrusion of the mandible, were reduced in patients with SMA type 2 and 3 compared to healthy controls (p mandibular range of motion (R(2) = 0.82) and weakness of neck muscles for bite force (R(2) = 0.47). Reduced mandibular mobility and bite force are common complications in SMA. SMA type and neck muscle strength are important correlates of these complications. We provide further evidence for clinically relevant bulbar involvement in patients with SMA. © 2016 American Academy of Neurology.

  12. The neuropathological signature of bulbar-onset ALS: A systematic review.

    Science.gov (United States)

    Shellikeri, S; Karthikeyan, V; Martino, R; Black, S E; Zinman, L; Keith, J; Yunusova, Y

    2017-04-01

    ALS is a multisystem disorder affecting motor and cognitive functions. Bulbar-onset ALS (bALS) may be preferentially associated with cognitive and language impairments, compared with spinal-onset ALS (sALS), stemming from a potentially unique neuropathology. The objective of this systematic review was to compare neuropathology findings reported for bALS and sALS subtypes in studies of cadaveric brains. Using Cochrane guidelines, we reviewed articles in MEDLINE, Embase, and PsycINFO databases using standardized search terms for ALS and neuropathology, from inception until July 16th 2016. 17 studies were accepted for analysis. The analysis revealed that both subtypes presented with involvement in motor and frontotemporal cortices, deep cortical structures, and cerebellum and were characterized by neuronal loss, spongiosis, myelin pallor, and ubiquitin+ and TDP43+ inclusion bodies. Changes in Broca and Wernicke areas - regions associated with speech and language processing - were noted exclusively in bALS. Further, some bALS cases presented with atypical pathology such as neurofibrillary tangles and basophilic inclusions, which were not found in sALS cases. Given the limited number of studies, all with methodological biases, further work is required to better understand neuropathology of ALS subtypes. Copyright © 2017 Elsevier Ltd. All rights reserved.

  13. Dysfunction of bulbar central pattern generator in ALS patients with dysphagia during sequential deglutition.

    Science.gov (United States)

    Aydogdu, Ibrahim; Tanriverdi, Zeynep; Ertekin, Cumhur

    2011-06-01

    The aim of this study is to investigate a probable dysfunction of the central pattern generator (CPG) in dysphagic patients with ALS. We investigated 58 patients with ALS, 23 patients with PD, and 33 normal subjects. The laryngeal movements and EMG of the submental muscles were recorded during sequential water swallowing (SWS) of 100ml of water. The coordination of SWS and respiration was also studied in some normal cases and ALS patients. Normal subjects could complete the SWS optimally within 10s using 7 swallows, while in dysphagic ALS patients, the total duration and the number of swallows were significantly increased. The novel finding was that the regularity and rhythmicity of the swallowing pattern during SWS was disorganized to irregular and arhythmic pattern in 43% of the ALS patients. The duration and speed of swallowing were the most sensitive parameters for the disturbed oropharyngeal motility during SWS. The corticobulbar control of swallowing is insufficient in ALS, and the swallowing CPG cannot work very well to produce segmental muscle activation and sequential swallowing. CPG dysfunction can result in irregular and arhythmical sequential swallowing in ALS patients with bulbar plus pseudobulbar types. The arhythmical SWS pattern can be considered as a kind of dysfunction of CPG in human ALS cases with dysphagia. Copyright © 2010 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  14. [Ambulatory intensive therapy in the bulbar form of amyotrophic lateral sclerosis].

    Science.gov (United States)

    Kirisits, H; Reisecker, F

    1996-01-01

    Due to the instability of their respiratory functions, patients suffering from ALS are potentially patients for permanent intensive care. The desire to provide care in the familiar environment at home on the one hand, and qualified professional support on the other hand, gives rise to the concept of ambulatory intensive care. This concept might be successfully implemented if one proceeds according to the motto: it is not the patient who is committed to technical facilities, but rather that the technical facilities ought to be committed to the patient. Home care, gastrostomy, tracheostomy, mobile suction drainage and the feasibility of home ventilation provide the groundwork for competent palliative medical care even in the bulbar form of ALS. Home care of such patients would be enhanced in terms of both security and quality if it were possible to have regular ambulatory check of the vital functions at intensive care units. In emergency cases, once their respiratory functions are stabilized patients could be discharged into home nursing after a short-term stay at the intensive care unit. Ambulatory intensive therapy would both serve to ease the burden of intensive care units in terms of costs and personnel, and to improve the life quality of patients.

  15. 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.

  16. 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...

  17. Accessory nerve palsy following thoracotomy.

    Science.gov (United States)

    Morrell, S; Roberson, J R; Rooks, M D

    1989-01-01

    A previously unreported cause of 11th cranial nerve palsy is described in a 53-year-old man. Dysfunction of the trapezius branch of the spinal accessory nerve occurred following median sternotomy and was documented by electromyography. This injury resulted in dysfunction of the trapezius muscle with loss of support of the shoulder girdle and pain. The injury may have been due to stretching from sternal retraction or injury secondary to internal jugular venous cannulation.

  18. Novel GFAP Variant in Adult-onset Alexander Disease With Progressive Ataxia and Palatal Tremor.

    Science.gov (United States)

    Gass, Jennifer M; Cheema, Anvir; Jackson, Jessica; Blackburn, Patrick R; Van Gerpen, Jay; Atwal, Paldeep S

    2017-11-01

    Alexander disease is a rare neurodegenerative disease caused by variants in the glial fibrillary acidic protein gene (GFAP). This disorder can develop as an infantile, juvenile or adult-onset form and is characterized by several clinical features, including macrocephaly, seizures, ataxia, and bulbar/pseudobulbar signs. While the majority of these patients have the more progressive infantile form which causes severe leukodystrophy and early death; the less common adult form is more variable (ie, onset age, symptoms), with bulbar dysfunction as the primary feature. In our investigation, we describe a patient with progressive neuromuscular issues including dyspnea, dysphagia, dysarthria and progressive ataxia with palatal tremor. Through genetic testing, we determined that our patient has a novel variant in GFAP typical of Alexander disease.

  19. The child with cerebral palsy--clinical considerations and management.

    Science.gov (United States)

    Singhi, P

    2001-06-01

    Cerebral Palsy (CP) is a non-progressive disorder of the motor system secondary to brain injury during early development. Associated non-motor problems are frequent and contribute significantly to the disability. While spastic CP is the commonest, dyskinetic CP continues to be a problem in our contribute. Acquired CP mostly secondary to CNS infections constitutes a significant proportion of cases in developing countries. Early diagnosis, proper assessment of the functional capacity and needs of the child and providing early intervention are important. A multidisciplinary approach to management with active parental involvement helps the child achieve his potential.

  20. 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

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

    African Journals Online (AJOL)

    diplegia and spastic tetraplegia types. Beckung,. Hagberg and Uldall (2008: e187-92) classified them as Spastic unilateral cerebral palsy, Spastic bilateral cerebral palsy and dyskinetic cerebral palsy and ataxic cerebral palsy. In the spastic type there is stiffness and movement difficulties, in ataxic form there is a disturbance ...

  2. Ataxic cerebral palsy and genetic predisposition.

    OpenAIRE

    Miller, G.

    1988-01-01

    It was calculated that in the 962 family members of 36 patients with ataxic cerebral palsy there were 75 (8%) with a history of neurodevelopmental disorder and 31 (3%) with a major congenital malformation. This was not significantly greater than expected, and does not support the hypothesis of a genetic non-Mendelian role in the aetiology of ataxic cerebral palsy.

  3. VII NERVE PALSY — EVALUATION AND MANAGEMENT

    African Journals Online (AJOL)

    Enrique

    gold weight implant or upper eyelid spring is indicated. Bell's palsy. The treatment of Bell's remains contro- versial. The effectiveness of any med- ical regimen in altering the natural his- tory of the palsy (85 - 90% complete recovery in 1 month) remains unproven. Despite this, experts believe that early oral prednisone (1 ...

  4. 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.

  5. 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 ...

  6. 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…

  7. Bulbar paralysis associated with Miller-Fisher syndrome and its overlaps in Chinese patients.

    Science.gov (United States)

    Hu, Qiaoxia; Li, Hongfu; Tian, Jun; Zhang, Baorong

    2017-11-09

    The study aimed to determine the incidence and the onset time of bulbar paralysis (BP) associated with Miller-Fisher syndrome (MFS) and its overlaps, to better understand the clinical characteristics among patients with MFS and its overlaps. Medical records from 48 patients with MFS and its overlaps were divided into two groups based on the presence (MFS-BP+) or absence (MFS-BP-) of BP. Their clinical features, laboratory and electrophysiological findings, neuroimaging data, and treatment plan were analyzed and compared between two groups. The incidence of BP associated with MFS and its overlaps was 48%. Eighty-two percent of the patients developed BP within 1 week after the onset of MFS and its overlaps. The cerebrospinal fluid (CSF) protein level in patients was higher in MFS-BP+ than in MFS-BP- group (67.69 ± 26.59 vs. 50.15 ± 20.44 mg/dl; P < 0.05). Frequencies of severe limb weakness, hypoglossal paralysis, disturbance of consciousness, and tracheal intubation required were also significantly higher in MFS-BP+ than in MFS-BP- group. Positive results of anti-GQ1b and anti-GT1b antibodies were all found in MFS-BP+ group. The prevalence of BP in MFS and its overlap was higher, the majority of BP occurred within 7 days after the onset of the disease, and early diagnosis of BP concurrence is helpful to decide the treatment plan.

  8. Bulbar microcircuit model predicts connectivity and roles of interneurons in odor coding.

    Directory of Open Access Journals (Sweden)

    Aditya Gilra

    Full Text Available Stimulus encoding by primary sensory brain areas provides a data-rich context for understanding their circuit mechanisms. The vertebrate olfactory bulb is an input area having unusual two-layer dendro-dendritic connections whose roles in odor coding are unclear. To clarify these roles, we built a detailed compartmental model of the rat olfactory bulb that synthesizes a much wider range of experimental observations on bulbar physiology and response dynamics than has hitherto been modeled. We predict that superficial-layer inhibitory interneurons (periglomerular cells linearize the input-output transformation of the principal neurons (mitral cells, unlike previous models of contrast enhancement. The linearization is required to replicate observed linear summation of mitral odor responses. Further, in our model, action-potentials back-propagate along lateral dendrites of mitral cells and activate deep-layer inhibitory interneurons (granule cells. Using this, we propose sparse, long-range inhibition between mitral cells, mediated by granule cells, to explain how the respiratory phases of odor responses of sister mitral cells can be sometimes decorrelated as observed, despite receiving similar receptor input. We also rule out some alternative mechanisms. In our mechanism, we predict that a few distant mitral cells receiving input from different receptors, inhibit sister mitral cells differentially, by activating disjoint subsets of granule cells. This differential inhibition is strong enough to decorrelate their firing rate phases, and not merely modulate their spike timing. Thus our well-constrained model suggests novel computational roles for the two most numerous classes of interneurons in the bulb.

  9. The impact of intrathecal baclofen on the natural history of scoliosis in cerebral palsy.

    Science.gov (United States)

    Shilt, Jeffrey S; Lai, Lawrence P; Cabrera, Michael N; Frino, John; Smith, Beth P

    2008-09-01

    Intrathecal baclofen (ITB) is an effective treatment of spasticity in patients with cerebral palsy. However, several recent reports have raised concerns that the treatment may be associated with a rapid progression of scoliosis. The objective of this study was to further examine the effect of ITB treatment on the progression of scoliosis in patients with cerebral palsy. Spastic cerebral palsy patients who were ITB candidates were followed radiographically. Baseline Cobb angles of the primary curve were measured during the period of ITB pump insertion and at the most recent follow-up visit. Each patient was matched with a control patient by the diagnosis of cerebral palsy, age, sex, topographic involvement, and initial Cobb angle. The mean rate of change in Cobb angle was compared between ITB and control patients using paired t test. A multiple linear regression model was used to examine the difference, controlling for age, sex, topographic involvement, and initial Cobb angle. Fifty ITB patients and 50 controls were included in the analysis. There was no statistically significant difference between the mean change in Cobb angle in ITB patients (6.6 degrees per year) compared with the matched control patients (5.0 degrees per year, P = 0.39). The results from the multiple regression analysis also failed to show a statistically significant difference (0.92 degrees per year difference between ITB patients and controls, P = 0.56). The progression of scoliosis in cerebral palsy patients with ITB treatment is not significantly different from those without ITB treatment. The findings suggest that patients receiving ITB experience a natural progression of scoliosis similar to the natural history reported in the literature. Level III.

  10. Generation of sibling-matched induced pluripotent stem cell lines from spinal and bulbar muscular atrophy patients

    Directory of Open Access Journals (Sweden)

    Gunaseelan Narayanan

    2017-04-01

    Full Text Available Spinal and bulbar muscular atrophy (SBMA is a neurodegenerative disease caused by the expansion of CAG repeats in the Androgen Receptor gene (AR. We report the generation of induced pluripotent stem cell (iPSC lines from two SBMA patients and their healthy siblings. The SBMA and healthy iPSC lines retain the number of AR CAG repeats, express pluripotency markers and are able to differentiate into the three germ layers. The iPSC lines are also free of Sendai virus transgenes and have normal karyotypes. The SBMA iPSC lines with their sibling-matched controls would serve as useful tools to study SBMA disease mechanism.

  11. Ophthalmologic Findings in Children with Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Ali Kurt

    2012-05-01

    Full Text Available Cerebral palsy (CP is a chronic disease which is seen in early childhood, i.e. in the first two years of life. It is a non-progressive disorder resulting from a defect or lesion in the immature brain and thus leading to posture and movement disorders. The reason for facing high rates of ophthalmologic problems in CP cases is that visual functions are covered in a large area in the brain. While vision defect in the normal population ranges from 4 to 5%, this rate in children with CP is nearly ten times more . Given that patients with CP are not followed up, the ophthalmologic examination is performed in advanced stages and that there are difficulties in spectacle use, amblyopia is detected more often in those patients than in healthy people. The treatment of CP patients needs multi-disciplinary cooperation. So, CP patients must be referred to ophthalmologists for a detailed examination in the early stages. Furthermore, other accompanying disorders (audiologic, orthopedic and psychiatric should be evaluated and rehabilitation programmes should be carefully applied in early stages to help CP patients achieve healthy lifestyle. (Turk J Oph thal mol 2012; 42: 219-26

  12. Visual disorders in cerebral palsy

    Directory of Open Access Journals (Sweden)

    Govind Amita

    1988-01-01

    Full Text Available Seventy children with cere-bral palsy were examined for aetiological factors responsible, type of disorder and ocular abnormalities. The overall inci-dence of ocular abnormalities was 68.69%, the highest frequency being of squint (35.7%. Other anomalies detected inclu-ded refractive errors (28.5%, optic atrophy (10% and coloboma (2.9%. Most children were spastic quadriplegics with asphyxia as the major aetiological factor. The study created an awareness amongst parents of these children. The visual status of those affected could be improved for imparting education and training in a better way.

  13. I can't move my face! a case of bilateral facial palsy.

    Science.gov (United States)

    Greenberg, Marna Rayl; Urquhart, Megan C; Eygnor, Jessica K; Worrilow, Charles C; Gesell, Nicole Ceccacci; Porter, Bernadette Glenn; Miller, Andrew C

    2013-10-01

    The authors present a case of bilateral facial palsy in a 52-year-old man. The patient presented to an emergency department in Pennsylvania, describing left-sided neck pain and headache from "sleeping wrong," symptoms which eventually progressed to facial diplegia by his fourth visit in 2 weeks. His admitting diagnosis was Bell palsy; he was ultimately tested for and found to have Lyme disease. Delay in treatment of patients with Lyme disease may lead to bilateral facial paralysis and disease progression. Thorough history taking, physical examination, and scrutiny of prior records are important elements of identifying and treating patients such as these (ie, whose vague symptoms progress to facial diplegia) appropriately.

  14. 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.

  15. Pontine stroke presenting as isolated facial nerve palsy mimicking Bell's palsy: a case report

    Directory of Open Access Journals (Sweden)

    Saluja Paramveer

    2011-07-01

    Full Text Available Abstract Introduction Isolated facial nerve palsy usually manifests as Bell's palsy. Lacunar infarct involving the lower pons is a rare cause of solitary infranuclear facial paralysis. The present unusual case is one in which the patient appeared to have Bell's palsy but turned out to have a pontine infarct. Case presentation A 47-year-old Asian Indian man with a medical history of hypertension presented to our institution with nausea, vomiting, generalized weakness, facial droop, and slurred speech of 14 hours' duration. His physical examination revealed that he was conscious, lethargic, and had mildly slurred speech. His blood pressure was 216/142 mmHg. His neurologic examination showed that he had loss of left-sided forehead creases, inability to close his left eye, left facial muscle weakness, rightward deviation of the angle of the mouth on smiling, and loss of the left nasolabial fold. Afferent corneal reflexes were present bilaterally. MRI of the head was initially read as negative for acute stroke. Bell's palsy appeared less likely because of the acuity of his presentation, encephalopathy-like imaging, and hypertension. The MRI was re-evaluated with a neurologist's assistance, which revealed a tiny 4 mm infarct involving the left dorsal aspect of the pons. The final diagnosis was isolated facial nerve palsy due to lacunar infarct of dorsal pons and hypertensive encephalopathy. Conclusion The facial nerve has a predominant motor component which supplies all muscles concerned with unilateral facial expression. Anatomic knowledge is crucial for clinical localization. Bell's palsy accounts for around 72% of facial palsies. Other causes such as tumors and pontine infarcts can also present as facial palsy. Isolated dorsal infarct presenting as isolated facial palsy is very rare. Our case emphasizes that isolated facial palsy should not always be attributed to Bell's palsy. It can be a presentation of a rare dorsal pontine infarct as observed

  16. INTEGRATED REHABILITATION APPROACH IN CEREBRAL PALSY PATIENTS

    National Research Council Canada - National Science Library

    Bellomo, R.G; Barassi, G; Verì, N; Giannuzzo, G; Di Felice, P; Pecoraro, I; D’Ettole, S; Saggini, R

    2017-01-01

    Introduction: cerebral palsy (CP) is a condition determined by a damage occurred in the developing brain of children during the first year of life, resulting in cognitive alterations and motor disorders like spasticity...

  17. 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.

  18. Botulinum A toxin utilizations in obstetric palsy

    Directory of Open Access Journals (Sweden)

    Atakan Aydin

    2012-12-01

    Conclusion: We conclude that with the help of botulinum A toxin and physyotherapy, obstetrical palsy patient with cocontractions can significantly improve movements and may have less surgery. [Hand Microsurg 2012; 1(3.000: 89-94

  19. Bell's Palsy: MedlinePlus Health Topic

    Science.gov (United States)

    ... with Steroids and Antiviral Drugs (American Academy of Neurology) - PDF Clinical Trials ClinicalTrials.gov: Bell Palsy (National Institutes of Health) Journal Articles References and abstracts from MEDLINE/PubMed (National Library ...

  20. 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

  1. 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...

  2. Treatment of Spasticity in Cerebral Palsy

    OpenAIRE

    Gül Mete Civelek; Ayçe Atalay

    2016-01-01

    The cerebral palsy diagnosis is a clinical one and it includes motor development delay, abnormal muscle tone and hyperreflexia. Muscle weakness, spasticity, loss of coordination, the continuation of the primitive reflexes and non-developed normal motor control are often seen in children with cerebral palsy. Spasticity is the velocity dependent increase in the resistance against passive muscle stretching. Spasticity treatment program should be established after detailed evaluation of degree an...

  3. Management of peripheral facial nerve palsy.

    Science.gov (United States)

    Finsterer, Josef

    2008-07-01

    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 scull and mastoid, cerebral MRI, or nerve conduction studies. Bell's palsy may be diagnosed after exclusion of all secondary causes, but causes of secondary FNP and Bell's palsy may coexist. Treatment of secondary FNP is based on the therapy of the underlying disorder. Treatment of Bell's palsy is controversial due to the lack of large, randomized, controlled, prospective studies. There are indications that steroids or antiviral agents are beneficial but also studies, which show no beneficial effect. Additional measures include eye protection, physiotherapy, acupuncture, botulinum toxin, or possibly surgery. Prognosis of Bell's palsy is fair with complete recovery in about 80% of the cases, 15% experience some kind of permanent nerve damage and 5% remain with severe sequelae.

  4. Association of cerebral palsy with epilepsy.

    Science.gov (United States)

    Kaushik, A; Agarwal, R P; Sadhna

    1997-10-01

    Fifty patients of various types of cerebral palsy were studied to find out an association between cerebral palsy, EEG abnormalities and development quotient. Seventy-six per cent patients had spastic cerebral palsy. Hypotonic cerebral palsy was the next common type (14%). Athetosis and ataxic forms were found to be rare (2% each). Epilepsy was associated with 56% patients. Clinical types of seizures observed were: Generalised tonic-clonic (43%), myoclonic (17.9%), generalised tonic (10.7%), partial simple (10.7%) and partial complex (17.9%). The incidence of seizures was highest in hypotonic type in which 85.7% had epilepsy. Mean developmental quotient of cerebral palsy patients was 34.9% with maximum retardation in hypotonic cerebral palsy (25.14%). Sixty per cent of patients had abnormal EEG, out of these hypotonic patients had maximum (70%) chances of EEG abnormality followed by spastic patients (55%). Developmental retardation was more severe statistically in the patients with abnormal EEG than normal EEG.

  5. Intermediate Type of Obstetric Brachial Plexus Palsy.

    Science.gov (United States)

    El-Sayed, Amel A F

    2016-12-01

    Data of 829 infants with obstetric brachial plexus palsy were reviewed to identify any cases that could not be fitted into the any of the well-known types of palsy. These unusual cases were studied in detail with regard to clinical presentation and electrophysiological findings as well as management and spontaneous motor recovery. Erb's, extended Erb's, and total palsies were seen in 42.8%, 28.8%, and 28.0% of cases, respectively. Three cases (0.4%) did not fit into any of the classic types. One case had bilateral palsy, and the remaining 2 cases had unilateral palsy. All affected limbs presented with "abducted arms," "flexed forearms," and electrophysiological evidence of denervation of shoulder adductors and triceps. All cases had excellent spontaneous recovery within 6-12 months. It was concluded that these cases represent mild "intermediate" types of palsy in which the C7 root was the predominant site of injury. Good spontaneous recovery is expected. © The Author(s) 2016.

  6. Tumors Presenting as Multiple Cranial Nerve Palsies

    Directory of Open Access Journals (Sweden)

    Kishore Kumar

    2017-04-01

    Full Text Available Cranial nerve palsy could be one of the presenting features of underlying benign or malignant tumors of the head and neck. The tumor can involve the cranial nerves by local compression, direct infiltration or by paraneoplastic process. Cranial nerve involvement depends on the anatomical course of the cranial nerve and the site of the tumor. Patients may present with single or multiple cranial nerve palsies. Multiple cranial nerve involvement could be sequential or discrete, unilateral or bilateral, painless or painful. The presentation could be acute, subacute or recurrent. Anatomic localization is the first step in the evaluation of these patients. The lesion could be in the brain stem, meninges, base of skull, extracranial or systemic disease itself. We present 3 cases of underlying neoplasms presenting as cranial nerve palsies: a case of glomus tumor presenting as cochlear, glossopharyngeal, vagus and hypoglossal nerve palsies, clivus tumor presenting as abducens nerve palsy, and diffuse large B-cell lymphoma presenting as oculomotor, trochlear, trigeminal and abducens nerve palsies due to paraneoplastic involvement. History and physical examination, imaging, autoantibodies and biopsy if feasible are useful for the diagnosis. Management outcomes depend on the treatment of the underlying tumor.

  7. Ocular problems in children with cerebral palsy

    Directory of Open Access Journals (Sweden)

    Esra Ayhan Tuzcu

    2012-09-01

    Full Text Available The aim of this study is to evaluate eye problemsin children with cerebral palsy in our region.Materials and Methods: 90 patients which was diagnosedas cerebral palsy, treated and followed up in PediatricNeurology Department of Mustafa Kemal University,were included to this study. The history was taken, anda physical examination was performed to determine theetiology of the disease and type of SP. All of the patientswere underwent a detailed ophthalmological examinationincluding visual acuity, refractive error, amblyopia, strabismus,nystagmus and fundus examination.Results: Totally 90 patients, 51 male and 39 female,were included to the study. When the etiologic factorswere evaluated, the asphyxia was seen in 33.3% of thepatients. The most common type of cerebral palsy wasspastic quadriplegia at the rate of 43.3%. Eye problemswere detected in 60% of our cases. Of this, 54.4% wererefractive errors, 35.6% were strabismus, and 22.2%were optic nerve pathologies. Amblyopia was found in11.1% of cases. Although strabismus is more common inspastic diplegia type of cerebral palsy, there was no statisticallysignificant differenceConclusions: In conclusion, eye problems are commonin children with cerebral palsy. Therefore, we recommendroutine eye examination in these patients due to be beneficialin reducing the detection and communication difficulties.Key words: Cerebral palsy, refractive error, strabismus,optic atrophy

  8. 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)

  9. 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

  10. Functional movement, strength, and intervention for an adolescent with cerebral palsy.

    Science.gov (United States)

    Hedgecock, James B; Rapport, Mary Jane; Sutphin, Andrew R

    2015-01-01

    This case report describes the examination, intervention, and outcome of a 3-month episode of physical therapy (PT) using combined functional training and progressive resistance exercise for an adolescent with cerebral palsy. The patient presented with a long history of PT intervention and strength impairments that limited functional and transitional movement, agility, and peer-level participation in school and the community. Functional strength, aerobic conditioning, and anaerobic power were examined and addressed during intervention. Lower extremity strength was improved. Improvements in functional strength, agility, and anaerobic power generation were clinically insignificant. Most importantly, patient defined participation improved and was accompanied by achievement of new transitional and functional movements. Outcomes described support the use of progressive resistance exercise and functional training to improve strength and functional mobility in an adolescent with cerebral palsy.

  11. 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....

  12. 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.

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

    Directory of Open Access Journals (Sweden)

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

    2017-01-01

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

  14. 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.

  15. Childhood cerebral palsy and the use of positioning systems to control body posture: Current practices.

    Science.gov (United States)

    Pérez-de la Cruz, S

    One of the consequences of poor postural control in children with cerebral palsy is hip dislocation. This is due to the lack of weight-bearing in the sitting and standing positions. Orthotic aids can be used to prevent onset and/or progression. The aim of this study is to analyse the effectiveness of positioning systems in achieving postural control in patients with cerebral palsy, and discuss these findings with an emphasis on what may be of interest in the field of neurology. We selected a total of 18 articles on interventions in cerebral palsy addressing posture and maintenance of ideal postures to prevent deformities and related problems. The main therapeutic approaches employed combinations of botulinum toxin and orthoses, which reduced the incidence of hip dislocation although these results were not significant. On the other hand, using positioning systems in 3 different positions decreases use of botulinum toxin and surgery in children under 5 years old. The drawback is that these systems are very uncomfortable. Postural control systems helps control hip deformities in children with cerebral palsy. However, these systems must be used for prolonged periods of time before their effects can be observed. Copyright © 2015 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Botulinum toxin type A management of spasticity in the context of orthopaedic surgery for children with spastic cerebral palsy.

    Science.gov (United States)

    Graham, H K

    2001-11-01

    Cerebral palsy is the most common cause of physical disability affecting children in developed countries. Although cerebral palsy is, by definition, a 'static encephalopathy' the associated musculoskeletal pathology is progressive and current definitions are therefore somewhat inadequate. Understanding the stages of the musculoskeletal pathology is fundamental to understanding current management strategies, including spasticity management, strengthening programmes and deformity correction by orthopaedic surgery. In this review, a number of new management strategies are described, in which spasticity management by intramuscular injections of botulinum toxin type A and deformity correction, by orthopaedic surgery, are combined.

  17. Peripheral magnetic stimulation to decrease spasticity in cerebral palsy.

    Science.gov (United States)

    Flamand, Véronique H; Beaulieu, Louis-David; Nadeau, Line; Schneider, Cyril

    2012-11-01

    Muscle spasticity in pediatric cerebral palsy limits movement and disrupts motor performance, thus its reduction is important in rehabilitation to optimize functional motor development. Our pilot study used repetitive peripheral magnetic stimulation, because this emerging technology influences spinal and cerebral synaptic transmission, and its antispastic effects were reported in adult neurologic populations. We tested whether five sessions of tibial and common peroneal nerve stimulation exerted acute and long-term effects on spasticity of the ankle plantar flexor muscles in five spastic diparetic children (mean age, 8 years and 3 months; standard deviation, 1 year and 10 months). Muscle resistance to fast stretching was measured with a manual dynamometer as a spasticity indicator. A progressive decrease was observed for the more impaired leg, reaching significance at the third session. This sustained reduction of spasticity may reflect that the peripheral stimulation improved the controls over the spinal circuitry. It thus suggests that a massive stimulation-induced recruitment of sensory afferents may be able to influence central nervous system plasticity in pediatric cerebral palsy. Copyright © 2012 Elsevier Inc. All rights reserved.

  18. Clinical Practice Guideline of Acupuncture for Bell's Palsy

    Directory of Open Access Journals (Sweden)

    Xi Wu

    2016-10-01

    Full Text Available Backgroud: Acupuncture is common used for Bell's palsy in clinic, however, recent systematic reviews all shows that there is no sufficient evidence to support the effectiveness of acupuncture for Bell's palsy because ofthe poor quality and heterogeneity. It's urgently necessary to develop a guideline of acupuncture for Bell's palsy based on principles of evidence-based medicine to optimize acupuncture treating, standardize outcomes evaluating and to improve the quality of acupuncture for patients with Bell's palsy under general circumstances.

  19. Assessment of Consistency in Assignment of Severe (Grade 3) Squamous Metaplasia to Human Bulbar Conjunctiva Impression Cytology Cell Samples.

    Science.gov (United States)

    Doughty, Michael J

    2015-10-01

    Nucleus-to-cytoplasm (N:C) characteristics of published images of human bulbar conjunctival cells designated as showing severe (grade 3) squamous metaplasia were retrospectively assessed. From publications over a 40-year period, measurements were made from images of cell and nucleus size (based on areas and dimensions) and four different calculations were made for nucleo-cytoplasmic (N:C) ratios. From 54 published images, the mean nucleus-to-cytoplasm area ratio (NU/CYT AREA ratio) was 0.145 +/- 0.077 (range 0.052 to 0.346), compared to two different reference set values of 0.069 +/- 0.017 and 0.080 +/- 0.021. Similarly, a nucleus-to-cytoplasm length ratio (as LNLONG) was 0.308 +/- 0.080 (range 0.191 to 0.475) compared to reference values of 0.226 +/- 0.032 and 0.236 +/- 0.034. Similar differences in reference values were obtained using two other N:C ratio calculations. A wide range of values was found for morphometric N:C indices from published images, especially those without a scale bar, indicating a high incidence of inconsistent grading assignments. Overall, only about 30% of the published images showed morphological features consistent with severe squamous metaplasia (i.e., with the rest neither enlarged nor showing substantially changed N:C ratios) with no substantial pictorial evidence indicating that cells from the human bulbar conjunctiva have pyknotic nuclei. Current evidence indicates that grade 3 squamous metaplasia cells should be substantially enlarged. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. PRETERM BIRTH ASSOCIATION WITH CEREBRAL PALSY

    Directory of Open Access Journals (Sweden)

    Srinivasa Rao

    2015-04-01

    Full Text Available INTRODUCTION: Cerebral palsy ( CP is a group of permanent movement disorders that appear in early childhood. Preterm birth is the birth of baby before 37 completed weeks, a full term birth is birth at 37 to 42 weeks of gestation . AIM: To show the extent of association of preterm deliveries as a risk factor in development of cerebral palsy. MATERIALS AND METHODS: This r etrospective cohort study was conducted by eliciting history from the mothers of 99 cerebral palsy children who w ere treated in Rani Chandra Mani Devi Hospital, Visakhapatnam, Andhra Pradesh, India. De tailed history was taken from the mothers of 99 cerebral palsy children who were treated in this hospital. History regarding the period of gestation at which the child was born (preterm or full term, any previous history of pre - term delivery or abortions, was obtained from the mothers and the data analyzed . RESULTS: From this study it was observed the proportional association of pre - term births to cerebral palsy is 33 out 99 i.e., about 33.33%, Of these 33 cerebral palsy children highest association being with birth at 28 wks gestation (51 %. This study also shows th at the mothers with a previous history of preterm delivery have 14.4 times higher risk of subsequent pre term delivery; those with previous history of abortions have 5.7 times risk of pre - term delivery than mothers without such history. CONCLUSION: From th is study it was concluded that the pre - term birth plays a major role as a risk factor in the development of cerebral palsy with mothers having previous pre term delivery and previous abortions adding further to this risk.

  1. 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 cerebral palsy cases and 2.89 ± 3.04 µg/dL in their controls (P 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.

  2. 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.

  3. Sixth Nerve Palsy in Paediatric Intracranial Hypertension.

    Science.gov (United States)

    Reid, Julia E; Reem, Rachel E; Aylward, Shawn C; Rogers, David L

    2016-02-01

    The purpose of this study was to report the incidence and describe the characteristics of sixth cranial nerve (CN VI) palsy in paediatric patients with intracranial hypertension (IH). A retrospective chart review of central Ohio children diagnosed with IH over the 3-year period from 2010 to 2013 was conducted. IH without identifiable cause was defined as idiopathic intracranial hypertension (IIH), whereas IH with identifiable pathologic aetiology was deemed secondary intracranial hypertension (SIH). A subset of patients with CN VI palsy was identified. Data collected included patient age, gender, past medical history, aetiology of SIH, ophthalmic examination, lumbar puncture results, neuroimaging results, and response to treatment. Seventy-eight children with intracranial hypertension were included in the study. Nine (11.5%) children (four males, five females; median age 14, range: 3-18) were found to have a unilateral (n = 2) or bilateral (n = 7) CN VI palsy. Five children had IIH; the remaining four had SIH from cerebral venous sinus thrombosis (n = 2) and infection (n = 2). The mean lumbar puncture opening pressure for the nine patients with CN VI palsy was 40 cm H2O (range: 21-65 cm H2O). Papilloedema was present in 8/9 (89%) patients. One patient required a lumboperitoneal shunt, and two others required optic nerve sheath fenestrations in addition to medical management. All cases of CN VI palsy resolved with treatment. In our primary service area, the incidence of CN VI palsy is approximately 12% among paediatric IH patients. The majority of cases with CN VI palsy presented with papilloedema and all cases resolved with treatment of intracranial hypertension.

  4. Aetiological profile of facial nerve palsy in north central Nigeria ...

    African Journals Online (AJOL)

    Bell's palsy 13(52%), road traffic injury 5(20%) and chronic suppurative otitis media 3(12%) are the commonest cause. The others are stroke 2(8%), measles infection 1(4%) and middle ear tumor 1(4%). Spontaneous recovery was observed in 40%. Conclusion: Bell's palsy was the commonest cause of facial nerve palsy, ...

  5. 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…

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

    African Journals Online (AJOL)

    Pattern of facial palsy in a typical Nigerian specialist hospital. S Lamina, S Hanif. Abstract. Background: Data on incidence of facial palsy is generally lacking in Nigeria. Objective: To assess six years' incidence of facial palsy in Murtala Muhammed Specialist Hospital (MMSH), Kano, Nigeria. Method: The records of patients ...

  7. 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 ...

  8. Profile of Children with Cerebral Palsy Attending Out- patient ...

    African Journals Online (AJOL)

    Olusola Ayanniyi

    Cerebral palsy (CP) is a major cause of childhood disability. The objective of this study was to investigate the causes ... KEY WORDS: cerebral palsy, jaundice, asphyxia, infections, childhood disabilities. INTRODUCTION. Cerebral palsy (CP) is an ...... The effects of multiple pre- and perinatal risk factors on the occurrence of ...

  9. Therapeutic results in sixth nerve palsy

    Directory of Open Access Journals (Sweden)

    Pruna Violeta-Ioana

    2015-03-01

    Full Text Available Authors aim to assess through a retrospective study the efficiency of different therapeutic methods used in VIth nerve palsy. 60 patients with VIth nerve palsy, admitted and treated in Oftapro Clinic, were divided into two groups: a group with partial dysfunction (paresis of sixth nerve and a group with the complete abolition of neuromuscular function (VIth nerve palsy. Initial examination included assessment of neuromuscular function, binocular vision and existence of medial rectus muscle contracture (ipsi- and contralateral and contralateral lateral rectus inhibitory palsy. Neuromuscular dysfunction was graded from - 8 (paralysis to 0 (normal abduction. Therapeutic modalities ranged from conservative treatment (occlusion, prism correction, botulinum toxin chemodenervation and surgical treatment: medial rectus recession + lateral rectus resection, in cases of paresis, and transposition procedures (Hummelscheim and full tendon transfer in cases of sixth nerve palsy. Functional therapeutic success was defined as absence of diplopia in primary position, with or without prism correction, and surgical success was considered obtaining orthoptic alignment in primary position or a small residual deviation (under 10 PD. 51 patients had unilateral dysfunction, and 9 patients had bilateral VI-th nerve dysfunction. 8 patients had associated fourth or seventh cranial nerves palsy. The most common etiology was traumatic, followed by tumor and vascular causes. There were 18 cases of spontaneous remission, partial or complete (4-8 months after the onset, and 6 cases enhanced by botulinum toxin chemodenervation. 17 paretic eyes underwent surgery, showing a very good outcome, with restoration of binocular single vision. The procedure of choice was recession of medial rectus muscle, combined with resection of lateral rectus muscle. All patients with sixth nerve palsy underwent surgery, except one old female patient, who refused surgery. Hummelscheim procedure was

  10. "All that palsies is not Bell's" - The need to define Bell's palsy as an adverse event following immunization

    NARCIS (Netherlands)

    Rath, Barbara; Linder, Thomas; Cornblath, David; Hudson, Michael; Fernandopulle, Rohini; Hartmann, Katharina; Heininger, Ulrich; Izurieta, Hector; Killion, Leslie; Kokotis, Pangiotis; Oleske, James; Vajdy, Michael; Wong, Virginia

    2007-01-01

    Bell's palsy has been reported as an adverse event following immunization (AEFI). Review of the published literature reveals that several characteristics have been used to describe Bell's palsy, which differ significantly from author to author. Evidently, the definition of "Bell's palsy" remains

  11. Epilepsy in children with cerebral palsy

    Directory of Open Access Journals (Sweden)

    Bruck Isac

    2001-01-01

    Full Text Available OBJECTIVE: To describe the prevalence and characteristics of epilepsy in patients with cerebral palsy in a tertiary center. METHODS: a total of 100 consecutive patients with cerebral palsy were retrospectively studied. Criteria for inclusion were follow-up period for at least 2 years. Types and incidence of epilepsy were correlated with the different forms of cerebral palsy. Other factors associated with epilepsy such as age of first seizure, neonatal seizures and family history of epilepsy were also analysed. RESULTS: follow-up ranged between 24 and 151 months (mean 57 months. The overall prevalence of epilepsy was 62%. Incidence of epilepsy was predominant in patients with hemiplegic and tetraplegic palsies: 70.6% and 66.1%, respectively. First seizure occurred during the first year of life in 74.2% of patients with epilepsy. Generalized and partial were the predominant types of epilepsy (61.3% and 27.4%, respectively. Thirty-three (53.2% of 62 patients were seizure free for at least 1 year. Neonatal seizures and family history of epilepsy were associated with a higher incidence of epilepsy. CONCLUSIONS: epilepsy in cerebral palsy can be predicted if seizures occur in the first year of life, in neonatal period and if there is family history of epilepsy.

  12. Candidate genes and cerebral palsy: a population-based study.

    Science.gov (United States)

    Gibson, Catherine S; Maclennan, Alastair H; Dekker, Gustaaf A; Goldwater, Paul N; Sullivan, Thomas R; Munroe, David J; Tsang, Shirley; Stewart, Claudia; Nelson, Karin B

    2008-11-01

    The objective of this study was to examine whether selected genetic polymorphisms in the infant are associated with later-diagnosed cerebral palsy. A population-based case-control study was conducted of 28 single-nucleotide polymorphisms measured in newborn screening blood spots. A total of 413 children with later-diagnosed cerebral palsy were born to white women in South Australia in 1986-1999, and there were 856 control children. Distributions of genotypic frequencies were examined in total cerebral palsy, in gestational age groups, and by types of cerebral palsy and gender. Genotyping was performed by using a TaqMan assay. For inducible nitric-oxide synthase, possession of the T allele was more common in all children with cerebral palsy and for heterozygotes who were born at term. For lymphotoxin alpha, homozygous variant status was associated with risk for cerebral palsy and with spastic hemiplegic or quadriplegic cerebral palsy. Among term infants, heterozygosity for the endothelial protein C receptor single-nucleotide polymorphism was more frequent in children with cerebral palsy. In preterm infants, the variant A allele of interleukin 8 and heterozygosity for the beta-2 adrenergic receptor were associated with cerebral palsy risk. Interleukin 8 heterozygote status was associated with spastic diplegia. Variants of several genes were associated with cerebral palsy in girls but not in boys. Two of the 28 single-nucleotide polymorphisms examined were associated with all types of spastic cerebral palsy in both gestational age groups and others with cerebral palsy in gestational age or cerebral palsy subgroups. Some of these associations support previous findings. There may be a genetic contribution to cerebral palsy risk, and additional investigation is warranted of genes and gene-environment interactions in cerebral palsy.

  13. [Retrospect of the history of cerebral palsy cognition].

    Science.gov (United States)

    Chen, Shao-jun; Wang, Xiu-li

    2008-07-01

    Throughout the cognition history of cerebral palsy, though these literature and art works related to the description of cerebral palsy, yet it only shows that it was an "old disease". The real initiative medical study of cerebral palsy started at mid 19th century. In the mid to late 19th century, Little, Osler, and Freud, the three scholars, perfected the denomination of cerebral palsy, the brain-derived concept, pathogenic classification, diverse clinical characteristics of dyskinesia--oriented manifestations, thus established the basic framework for the study of cerebral palsy.

  14. Diagnosis and management of idiopathic facial palsy in children.

    Science.gov (United States)

    Mardassi, Ali; Sayeh, Aicha; Turki, Senda; Hachicha, Amani; Benzarti, Sonia

    2017-01-01

    Idiopathic or Bell's palsy is an acute peripheral-nerve palsy involving the facial nerve. The disorder is quite infrequent under the age of 10 years. The proposed etiologies of Bell's palsy include ischemic neuropathy and vascular diseases. This case series presents five children with Bell's palsy. The epidemiologic, diagnostic and therapeutic measures were summarized. The evolution regarding especially the facial motricity was detailed. The results about the role of some thrombophilic polymorphisms suggest a probable involvement of factor V haplotype, MTHFR and factor XIII in the etiology of Bell's palsy in five Tunisian children.

  15. Brain Disorders (Cerebral Palsy And The Ability Of Academic

    Directory of Open Access Journals (Sweden)

    Faisal Rachmat

    2017-08-01

    Full Text Available The purpose of this empirically is to get a clear picture of low intervensi given mild mental retardation (cerebral palsy in children to improve their academic abilities. Research that started from Maret 2017 through may 2017, on a boy named AMR student in the Homeschooling Seto Brothers (HKS Bintaro, Tangerang Selatan. This study used a qualitative approach with case study method. The collection of data through observation, interview and document research. The data were analyzed qualitatively. The result sducahowedan increase in academic knowledge after being given an educational intervention for 4 months. This is evidenced by the significant increase in work in class AMR, The LKS (Student Worksheet. Suggestions keep motivate students to remain willing to learn, the cooperation between teachers and parents should pay more attention to giving the effect is more significant in the progress of interventions undertaken in the school, pursued no alternations teacher.

  16. Impact of an educational program on parental knowledge of cerebral palsy.

    Science.gov (United States)

    Karande, Sunil; Patil, Shailesh; Kulkarni, Madhuri

    2008-09-01

    To investigate parental knowledge of cerebral palsy, and to evaluate the impact of an educational intervention on it. From May 2003 to April 2004, 26 parents of newly diagnosed children with cerebral palsy were interviewed. After the interview, each parent was administered a structured educational program and re-interviewed after three months. The pre and post intervention responses were compared using Chi-square test. After the intervention, there was a significant improvement in parental knowledge: (i) of the cause of the disorder (5/26 vs 20/26, P = 0.0001), (ii) that it is non-progressive (16/26 vs 24/26, P = 0.021), (iii) that it is not curable (10/26 vs 23/26, P = 0.0005), (iv) that it is treatable (12/26 vs 24/26, P=0.0009), (v) of the frequency and duration of therapy necessary to improve functional abilities (7/26 vs 17/26, P = 0.005), and, (vi) of the importance of following up regularly with a pediatrician (17/26 vs 26/26, P = 0.003). However, there was no significant improvement in parental knowledge: (i) of the meaning of the term 'cerebral palsy' (0/26 vs 5/26, P = 0.060), (ii) that 'early intervention therapy' given by a team of therapists is its recommended therapy (18/26 v 23/26, P = 0.174), (iii) of the meaning of the term 'early intervention therapy' (12/26 vs 17/26, P = 0.163), and (iv) that it is preventable with good medical care (8/26 vs 10/26, P = 0.560). Parental knowledge of cerebral palsy is inadequate. A single-session educational program can significantly improve parental knowledge about many 'core basic issues' regarding cerebral palsy.

  17. 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.

  18. [Muscle weakness in cerebral palsy].

    Science.gov (United States)

    Givon, Uri

    2009-01-01

    Over the last two decades, muscle weakness has been shown to be a major component of cerebral palsy (CP) pathology. Caused by multiple etiologies including variations in the muscle fiber type, pathologic motor unit function, co-contraction of agonists and antagonists, and muscle size and rigidity, weakness interferes with function and leads to limited function and participation. Muscle strength was found to be associated with walking ability and with functional scales. Children with CP were found to be weaker than typically developing children, and differences were found with respect to muscle groups in children with CP. Muscle weakness should be evaluated as objectively as possible to improve the quality of diagnosis and treatment. Manual muscle testing is not sufficient for evaluation, and instrumented muscle testing is validated in CP. Muscle strengthening is an important part of treatment of CP. Several methods of strengthening have been described. Muscle lengthening and other spasticity-modifying therapies have been shown to have a positive effect on muscle strength. Children who participated in muscle strengthening programs had a better quality of life and improved function.

  19. 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

  20. Validation of a cerebral palsy register

    DEFF Research Database (Denmark)

    Topp, Monica Wedell; Langhoff-Roos, J; 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...... 12 variables in the CPR and obstetrical medical records has been analysed using kappa-statistics. RESULTS: Of 468 children in the DNPR, only 237 fulfilled the inclusion criteria of the CPR; and 35 (15%) of these cases had not been reported to the CPR. Data agreement was generally good...

  1. Ocular manifestations in children with cerebral palsy.

    Science.gov (United States)

    Sasmal, Nirmal Kumar; Maiti, Prasenjit; Mandal, Radhakrishna; Das, Debabrata; Sarkar, Subhra; Sarkar, Piyali; Biswas, Mukul Chandra; Dey, Asim Kumar; Chatterjee, Sanjay

    2011-05-01

    To evaluate the prevalence of ocular abnormalities among children with cerebral palsy, 140 patients with age between 6 months and 16 years were selected and the overall incidence of ocular abnormalities in this study was 42.1%. Two major ocular abnormalities identified in these cases were strabismus in 36.4%. Myopia was detected in 12.9% children while hypermetropia in 8.6% and astigmatism in 3.6% cases. Non-glaucomatous optic atrophy was present in 10.7% cases and nystagmus in 9.3% cases. Raised intra-ocular pressure was detected in 2.1% cases. Cortical visual impairment was seen in 20.7% children. Ocular abnormalities are frequent manifestations in cerebral palsy patients. Therefore, evaluation of all cerebral palsy cases emphasises the need for a full ophthalmological examination in order to detect ocular problems and to institute necessary therapy of the abnormalities for better livelihood of these physically challenged patients.

  2. Progression in ALS is not linear but is curvilinear.

    Science.gov (United States)

    Gordon, Paul H; Cheng, Bin; Salachas, Francois; Pradat, Pierre-Francois; Bruneteau, Gaelle; Corcia, Philippe; Lacomblez, Lucette; Meininger, Vincent

    2010-10-01

    The aim of the study is to determine the shape of the progression curve in ALS, assess the impact of clinical variables on the rate of progression, and evaluate the association between functional decline and survival. Data were prospectively collected and entered into a clinical database from all patients seen in 2002-2008 at the Centre SLA, Hôpital de la Salpêtrière, Paris. Variables analyzed were demographic and baseline information, the ALS functional rating scale (ALSFRS-R), strength testing (MMT), and survival. Generalized additive mixed models characterized changes in ALSFRS-R and MMT scores over time. Linear mixed effects assessed the impact of demographic and clinical measures on rate of progression and Cox models examined their effect on survival. Of 2,452 patients with ALS identified, 1,884 had adequate data for analysis. The ALSFRS-R and MMT declined in a curvilinear way; a quadratic fit described the trends but a linear fit did not. The total ALSFRS-R score was negatively associated with age-of-onset (p age-at-onset and bulbar-onset predicted shorter survival. Deterioration in ALS is non-linear. The early and late phases of the illness show the most rapid rates of decline. Older age and bulbar signs are associated with a steeper decline, and along with more rapid initial rate of decline, but not current functional status, also predict survival.

  3. Body image in adolescents with cerebral palsy.

    Science.gov (United States)

    Hammar, Görel Råsmark; Ozolins, Andrejs; Idvall, Ewa; Rudebeck, Carl Edward

    2009-03-01

    The study aims to describe important features of body image in adolescents with motor disabilities and compare them against similar features in able-bodied peers. Relational aspects of body image were given preference in a questionnaire distributed to 35 adolescents with cerebral palsy and 98 adolescents with no known disabilities. Similarities were shown, but also significant differences, indicating a less favourable body image in adolescents with cerebral palsy. It is paramount for young people who are constantly reminded of physical restrictions to experience body vitality. Professionals need to consider the importance of how they interact with young people when seeking to promote a positive body image.

  4. 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

  5. Long-term follow-up of spinal and bulbar muscular atrophy in Taiwan

    Directory of Open Access Journals (Sweden)

    Ser-Chen Fu

    2013-06-01

    Conclusion: Patients with SBMA may present with a myriad of symptoms, including limbs weakness, tremor, muscle atrophy, and perioral fasciculations. Elevated serum CK and decreased CMAP and SNAP amplitudes were supportive laboratory findings of SBMA. Disease progression was gradual, and most patients remained functionally independent many years after the onset of weakness.

  6. 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…

  7. Chronic Inflammatory Demyelinating Polyneuropathy Manifesting as Neuropathy With Liability to Pressure Palsies: A Case Report.

    Science.gov (United States)

    Shah, Akshay; Rison, Richard A; Beydoun, Said R

    2015-12-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is a progressive demyelinating neuropathy, which typically presents with proximal and distal neuropathic symptoms and is typically responsive to immunomodulatory therapies. Many variants have been subsequently described in the literature and have similarly shown to be responsive to immunotherapy. We present a case of a 43-year-old Middle Eastern/Arabic man presenting with symptoms of mixed sensorimotor neuropathy most evident at entrapment sites mimicking hereditary neuropathy with liability to pressure palsies. His electrodiagnostic study revealed features of acquired demyelinating neuropathy and a negative genetic workup. Alternative diagnosis of CIDP was considered in the context of symptomatic disease progression, negative genetic workup, and electrodiagnosis leading to initiation of immunotherapy with intravenous immunoglobulins. His neuropathy responded confirming our diagnosis of an inflammatory demyelinating polyneuropathy. We describe a previously unknown variant of CIDP with phenotypic characteristics of hereditary neuropathy with liability to pressure palsies and its potential for successful treatment with intravenous immunoglobulins. This case illustrates an unusual presentation of CIDP mimicking hereditary neuropathy with liability to pressure palsies.

  8. Muscle strengthening is not effective in children and adolescents with cerebral palsy: a systematic review.

    Science.gov (United States)

    Scianni, Aline; Butler, Jane M; Ada, Louise; Teixeira-Salmela, Luci F

    2009-01-01

    Do strengthening interventions increase strength without increasing spasticity and improve activity, and is there any carryover after cessation in children and adolescents with cerebral palsy? Systematic review with meta-analysis of randomised trials. Children with spastic cerebral palsy between school age and 20 years. Strengthening interventions that involved repetitive, strong, or effortful muscle contractions and progressed as ability changed, such as biofeedback, electrical stimulation, and progressive resistance exercise. Strength was measured as continuous measures of maximum voluntary force or torque production. Spasticity was measured as velocity-dependent resistance to passive stretch. Activity was measured as continuous measures, eg, 10-m Walk Test, or using scales eg, the Gross Motor Function Measure. Six studies were identified and five had data that could be included in a metaanalysis. Strengthening interventions had no effect on strength (SMD 0.20, 95% CI -0.17 to 0.56), no effect on walking speed (MD 0.02 m/s, 95% CI -0.13 to 0.16), and had a small statistically-significant but not clinically-worthwhile effect on Gross Motor Function Measure (MD 2%, 95% CI 0 to 4). Only one study measured spasticity but did not report the between-group analysis. In children and adolescents with cerebral palsy who are walking, the current evidence suggests that strengthening interventions are neither effective nor worthwhile.

  9. 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.

  10. 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.

  11. Therapeutic effects of kinesio taping in children with cerebral palsy: a systematic review.

    Science.gov (United States)

    Ortiz Ramírez, Joaquín; Pérez de la Cruz, Sagrario

    2017-12-01

    Pediatric cerebral palsy is a non-progressive neurological disorder. It is one of the most common causes of disability among children. Numerous physical therapy techniques are currently used for treatment, and kinesio taping is one of them. The main objective of this study was to review the outcomes of using kinesio taping in published scientific studies conducted in pediatric patients with cerebral palsy and determine their methodological quality. The main scientific databases and the studies published in the official site of the Asociación Española de Vendaje Neuromuscular (Spanish Association for Neuromuscular Taping) were reviewed. Nine studies were included, which provided important outcomes. These studies show the effectiveness of recovering upper limb and motor function and solving dysphagia, which could be present in these patients, although scientific evidence may expand due to improvements in methodology. Sociedad Argentina de Pediatría.

  12. Facial nerve palsy and hemifacial spasm.

    Science.gov (United States)

    Valls-Solé, Josep

    2013-01-01

    Facial nerve lesions are usually benign conditions even though patients may present with emotional distress. Facial palsy usually resolves in 3-6 weeks, but if axonal degeneration takes place, it is likely that the patient will end up with a postparalytic facial syndrome featuring synkinesis, myokymic discharges, and hemifacial mass contractions after abnormal reinnervation. Essential hemifacial spasm is one form of facial hyperactivity that must be distinguished from synkinesis after facial palsy and also from other forms of facial dyskinesias. In this condition, there can be ectopic discharges, ephaptic transmission, and lateral spread of excitation among nerve fibers, giving rise to involuntary muscle twitching and spasms. Electrodiagnostic assessment is of relevance for the diagnosis and prognosis of peripheral facial palsy and hemifacial spasm. In this chapter the most relevant clinical and electrodiagnostic aspects of the two disorders are reviewed, with emphasis on the various stages of facial palsy after axonal degeneration, the pathophysiological mechanisms underlying the various features of hemifacial spasm, and the cues for differential diagnosis between the two entities. Copyright © 2013 Elsevier B.V. All rights reserved.

  13. Occupational therapy for children with cerebral palsy.

    NARCIS (Netherlands)

    Steultjens, E.M.J.; Dekker, J.; Bouter, L.M.; Nes, J.C.M. van de; Lambregts, B.L.M.; Ende, C.H.M. van den

    2003-01-01

    Objectives: The object of our systematic review, therefore, was to determine whether OT interventions improve functional abilities and social participation in children with cerebral palsy. Criteria for considering Studies for this Review: Types of studies: Studies with one of the following designs

  14. Epidemiology of Cerebral Palsy in the Netherlands

    NARCIS (Netherlands)

    M.J. Wichers (Marc Jan)

    2011-01-01

    textabstractChildren with cerebral palsy (CP) and their families often make strong demands on diagnostic, therapeutic, technical and social facilities. Prevalence estimates are needed to improve treatment and services. As recent Dutch data are not available, the present study aimed to assess the

  15. 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…

  16. 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...

  17. 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…

  18. 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…

  19. Narrative Ability in Children with Cerebral Palsy

    Science.gov (United States)

    Holck, Pernille; Sandberg, Annika Dahlgren; Nettelbladt, Ulrika

    2011-01-01

    In a previous study a group of children with cerebral palsy (CP) were found to have considerable difficulties with narratives, performing several standard deviations below the criteria for the Information score of the Bus Story Test (BST). To examine in depth the performance of children with CP and a control group with typically developing (TD)…

  20. 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.

  1. 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.

  2. MANAGEMENT OF CHILDREN WITH CEREBRAL PALSY

    African Journals Online (AJOL)

    Enrique

    The neurological lesion causing cerebral palsy (CP) is static, but the clinical con- dition changes over time. CP has a wide clinical spectrum with a number of aeti- ologies. It is not sufficient just to make the diagnosis of CP; the term needs further qualification by a more detailed description of the clinical condition. There are.

  3. Treatment of paediatric cerebral palsy with Dysport.

    Science.gov (United States)

    Ubhi, T

    2000-10-01

    Dysport (Clostridium botulinum type A toxin-haemagglutin complex) has had its licence extended to include treatment of children aged 2 years and over with dynamic equinus foot deformity, caused by spasticity associated with cerebral palsy. Dysport reduces muscle tone, thus improving function, relieving pain, and facilitating physiotherapy, application and tolerability of splints.

  4. 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…

  5. Facial nerve palsy associated with underwater barotrauma.

    Science.gov (United States)

    Whelan, T. R.

    1990-01-01

    This report describes a case of facial nerve palsy following barotitis media sustained at shallow depth. The neuropraxia is likely to have been due to the direct effect of pressure, facilitated by a congenital hiatus in the bony canal protecting the facial nerve in the middle ear. PMID:2216996

  6. C5 nerve root palsy after posterior cervical spine surgery.

    Science.gov (United States)

    Pan, Fu-Min; Wang, Shan-Jin; Ma, Bin; Wu, De-Sheng

    2017-01-01

    C5 palsy is a serious complication after cervical decompression surgery in which the patient shows a deterioration in power of the deltoid or biceps brachii by at least one grade in the manual muscle test without aggravation of lower extremity function. Although there are several hypotheses regarding the etiology of C5 palsy, the pathogenesis and preventive measures remain unidentified and many other controversies remain. To systematically review the clinical features, risk factors, mechanism, and preventive measures of C5 palsy after posterior cervical decompression surgery. PubMed was searched to identify eligible studies that contained more than 10 cases and focused on C5 palsy. Microsoft Excel was used to analyze the data. Statistical comparisons were made when appropriate. Out of 718 papers involving C5 palsy, 28 met the inclusion criteria. The average incidence rate was 7.8% (range, 1.4-23.0%). Risk factors for C5 palsy included age, male gender, ossification of the posterior longitudinal ligament, and stenosis of the C4-C5 intervertebral foramen. C5 palsy occurred from immediately to 2 months after surgery, and recovery time ranged from 48 h to 41 months. Hypotheses for the mechanism of C5 palsy included root involvement and spinal cord impairment. Foraminotomy and intraoperative neuromonitoring were the two main methods used to prevent C5 palsy. C5 palsy is a serious complication occurring at the early stage after cervical decompression surgery. Foraminotomy and intraoperative neuromonitoring were the two main methods to prevent C5 palsy. The incidence of C5 palsy is low, but it can place a serious burden on the patients' quality of life and finances. The risk factors and mechanism of C5 palsy are still controversial. However, under conservative therapy, the prognosis is usually good. Higher quality studies are necessary for drawing more reliable and convincing conclusions about this disease.

  7. Etiological risk factors for brachial plexus palsy.

    Science.gov (United States)

    Hudić, Igor; Fatusić, Zlatan; Sinanović, Osman; Skokić, Fahrija

    2006-10-01

    To investigate risk factors for brachial plexus palsy in newborns. We analyzed 45 544 live-born children, born over a nine-year period from January 1, 1996 to December 31, 2004. The analysis was retrospective and based on the medical documentation of the Clinic for Gynecology and Obstetrics, Clinic for Neurology, and Clinic for Physical Medicine and Rehabilitation of the University Clinical Center Tuzla. We compared study and control groups of newborns. Rates among groups were compared using Chi-square, with significance at p labor pattern itself, it was found that the highest factors of risk for brachial plexus injury were birth weight of over 4000 g, a precipitous second stage of labor (labor. Brachial plexus palsy was more frequent when the mothers were overweight, with a body mass index >or=29 kg/m2. None of the parturient women, whose newborns were diagnosed with brachial plexus palsy, had external conjugate diameter <18 cm. Newborns delivered vaginally were not diagnosed with a higher frequency of brachial plexus palsy when compared to newborns who were delivered by cesarean section, but newborns who were vaginal breech-delivered were diagnosed to have a higher incidence of brachial plexus palsy. Newborns whose mothers were older than 35 years were diagnosed to have brachial plexus palsy more frequently, but a statistically significant difference between primiparas and multiparas was not found. A total of 39 newborns (45.2%) were diagnosed with a fracture of the clavicle, which was the most frequently combined damage with brachial plexus injury. Forty-two newborns (48.8%) had an Apgar score of

  8. 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

  9. 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.

  10. Sialorrhea in children with cerebral palsy.

    Science.gov (United States)

    Dias, Bruno Leonardo Scofano; Fernandes, Alexandre Ribeiro; Maia Filho, Heber de Souza

    To review the literature on sialorrhea in children with cerebral palsy. Non-systematic review using the keywords "sialorrhea" and "child" carried out in the PubMed ® , LILACS ® , and SciELO ® databases during July 2015. A total of 458 articles were obtained, of which 158 were analyzed as they were associated with sialorrhea in children; 70 had content related to sialorrhea in cerebral palsy or the assessment and treatment of sialorrhea in other neurological disorders, which were also assessed. The prevalence of sialorrhea is between 10% and 58% in cerebral palsy and has clinical and social consequences. It is caused by oral motor dysfunction, dysphagia, and intraoral sensitivity disorder. The severity and impact of sialorrhea are assessed through objective or subjective methods. Several types of therapeutic management are described: training of sensory awareness and oral motor skills, drug therapy, botulinum toxin injection, and surgical treatment. The most effective treatment that addresses the cause of sialorrhea in children with cerebral palsy is training of sensory awareness and oral motor skills, performed by a speech therapist. Botulinum toxin injection and the use of anticholinergics have a transient effect and are adjuvant to speech therapy; they should be considered in cases of moderate to severe sialorrhea or respiratory complications. Atropine sulfate is inexpensive and appears to have good clinical response combined with good safety profile. The use of trihexyphenidyl for the treatment of sialorrhea can be considered in dyskinetic forms of cerebral palsy or in selected cases. Copyright © 2016 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    Akshay Gopinathan Nair

    2014-01-01

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

  12. 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...... palsy, severity of handicap. RESULTS: Breech presentation at term was associated with a borderline significantly higher risk of cerebral palsy than vertex presentation (OR 1.56; 95% CI 0.9-2.4). Breech presentation infants more often had a lower Apgar score (

  13. Faktor Risiko Terjadinya Epilepsi Pada Anak Palsi Serebral

    OpenAIRE

    Syafendra, Mega Oktariena

    2016-01-01

    Background. Epilepsy is one of the comorbidities that occur in children with cerebral palsy. Initial recognition of risk factors in epilepsy will improve the quality of life in cerebral palsy. Objective. To determine risk factors of epilepsy in children with cerebral palsy. Methods. a case control study was done in child neurology out patient clinic of Haji Adam Malik Hospital, Medical Rehabilitation Installation of Pirngadi Hospital and Clinic 77 in Medan between January to Mei 2015. ...

  14. Rule Based Expert System for Cerebral Palsy Diagnosis

    OpenAIRE

    Borgohain, Rajdeep; Sanyal, Sugata

    2012-01-01

    The use of Artificial Intelligence is finding prominence not only in core computer areas, but also in cross disciplinary areas including medical diagnosis. In this paper, we present a rule based Expert System used in diagnosis of Cerebral Palsy. The expert system takes user input and depending on the symptoms of the patient, diagnoses if the patient is suffering from Cerebral Palsy. The Expert System also classifies the Cerebral Palsy as mild, moderate or severe based on the presented symptoms.

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

    OpenAIRE

    Lee Hyun; Lee Byung-ryul; Ann Taek-won

    2003-01-01

    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...

  16. Bell's palsy: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Tashika Kushraj

    2014-06-01

    Full Text Available Bell's palsy is considered as a disease of exclusion. It is a form of lower motor neuron paralysis affecting the facial muscles. Rapid onset of paralysis causes panic to the patients. For speedy recovery, correct diagnosis and early treatment are crucial. Here a case of Bell's palsy is reported and the literature on Bell's palsy is reviewed. [Cukurova Med J 2014; 39(3.000: 581-588

  17. Non-traumatic peroneal nerve palsy: MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, J.Y. [Departments of Radiology St Vincent' s Hospital, Catholic University of Korea, Paldal-gu, Suwon city, Gyeonggi-do (Korea, Republic of); Ihn, Y.K. [Departments of Radiology St Vincent' s Hospital, The Catholic University of Korea, Paldal-gu, Suwon city, Gyeonggi-do (Korea, Republic of)]. E-mail: Ihn@catholic.ac.kr; Kim, J.S. [Department of Rehabilitation medicine, St Vincent' s Hospital, The Catholic University of Korea, Paldal-gu, Suwon city, Gyeonggi-do (Korea, Republic of); Chun, K.A. [Department of Radiology, Uijeongbu St. Mary' s Hospital, The Catholic University of Korea, Geumo-dong, Uijeongbu city, Gyeonggi-do (Korea, Republic of); Sung, M.S. [Department of Radiology, Holy Family Hospital, The Catholic University of Korea, Sosa-dong, Wonmi-gu, Bucheon city, Gyeonggi-do (Korea, Republic of); Cho, K.H. [Department of Radiology, College of Medicine, Yeungnam Univ. Medical Center, Nam-gu, Daegu (Korea, Republic of)

    2007-01-15

    Aim: To present magnetic resonance imaging (MRI) findings of non-traumatic peroneal nerve palsy and to evaluate the usefulness of MRI in patients with non-traumatic peroneal nerve palsy. Materials and methods: In a retrospective study, 11 consecutive patients presenting with peroneal nerve palsy were included. MR images of the lower leg and electrophysiological examinations were also reviewed. The cause of peroneal nerve palsy was determined on the basis of MRI findings and was evaluated using electrophysiological data. Nine patients with causative lesions detected on MRI, underwent surgery. Results: Clinical examination and electromyography (EMG) disclosed 11 peroneal lesions. MRI and EMG revealed three types of signal intensity change, i.e. deep peroneal nerve palsy type, common peroneal nerve palsy type, and superficial peroneal nerve palsy type. The MRI and EMG findings were in agreement in seven (65%) of the 11 study patients. In nine patients the causative lesions were identified using MRI, including ganglion cyst (n = 6), osteochondroma (n = 1), synovial cyst (n = 1), and aneurysm (n = 1). Conclusion: Ganglion cyst is the most common cause of non-traumatic peroneal nerve palsy. MRI offers a noninvasive method for obtaining useful information to assess, localize, and monitor peripheral peroneal nerve palsy.

  18. Cerebral palsy-definition, classification, etiology and early diagnosis.

    Science.gov (United States)

    Sankar, Chitra; Mundkur, Nandini

    2005-10-01

    Cerebral palsy is a common neurodevelopmental condition encountered by pediatricians. The condition may present itself in many different clinical spectra. The etiological and risk factors are many and an awareness of the interplay of multiple factors in the causation of CP is crucial. In many cases, the cause of Cerebral palsy may not be apparent. Cerebral palsy is invariably associated with many deficits such as mental retardation, speech and language and oromotor problems. A thorough neurodevelopmental assessment of the child with Cerebral Palsy should include evaluation of associated deficits so that a comprehensive early intervention program an be planned and executed.

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

    Directory of Open Access Journals (Sweden)

    Virender Sachdeva

    2013-01-01

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

  20. Clinical characteristics and cerebrospinal fluid parameters in patients with peripheral facial palsy caused by Lyme neuroborreliosis compared with facial palsy of unknown origin (Bell's palsy

    Directory of Open Access Journals (Sweden)

    Hagberg Lars

    2011-08-01

    Full Text Available Abstract Background Bell's palsy and Lyme neuroborreliosis are the two most common diagnoses in patients with peripheral facial palsy in areas endemic for Borrelia burgdorferi. Bell's palsy is treated with corticosteroids, while Lyme neuroborreliosis is treated with antibiotics. The diagnosis of Lyme neuroborreliosis relies on the detection of Borrelia antibodies in blood and/or cerebrospinal fluid, which is time consuming. In this study, we retrospectively analysed clinical and cerebrospinal fluid parameters in well-characterised patient material with peripheral facial palsy caused by Lyme neuroborreliosis or Bell's palsy, in order to obtain a working diagnosis and basis for treatment decisions in the acute stage. Methods Hospital records from the Department of Infectious Diseases, Sahlgrenska University Hospital, for patients with peripheral facial palsy that had undergone lumbar puncture, were reviewed. Patients were classified as Bell's palsy, definite Lyme neuroborreliosis, or possible Lyme neuroborreliosis, on the basis of the presence of Borrelia antibodies in serum and cerebrospinal fluid and preceding erythema migrans. Results One hundred and two patients were analysed; 51 were classified as Bell's palsy, 34 as definite Lyme neuroborreliosis and 17 as possible Lyme neuroborreliosis. Patients with definite Lyme neuroborreliosis fell ill during the second half of the year, with a peak in August, whereas patients with Bell's palsy fell ill in a more evenly distributed manner over the year. Patients with definite Lyme neuroborreliosis had significantly more neurological symptoms outside the paretic area of the face and significantly higher levels of mononuclear cells and albumin in their cerebrospinal fluid. A reported history of tick bite was uncommon in both groups. Conclusions We found that the time of the year, associated neurological symptoms and mononuclear pleocytosis were strong predictive factors for Lyme neuroborreliosis as a

  1. [Cerebral palsy in children--motor function and new treatment strategies].

    Science.gov (United States)

    Lofterød, Bjørn; Jahnsen, Reidun; Terjesen, Terje

    2006-10-19

    Substantial changes have taken place in the management of motor function in children with cerebral palsy during the last decade. The objective of this article is to describe some of these changes. The article is based on search of databases and own clinical experiences. New treatment methods have been introduced and the management is more specialised and individualised. No single treatment method has proven to be sufficient alone and the challenge is to find the best combination of methods. This requires the ability to cooperate across disciplines. Medical treatment of spasticity (botulinum toxin A, intrathecal baclofen) may be an alternative or a supplement to orthopaedic surgery. For children who can walk, preoperative gait analysis has made it easier to find the right time for an operation and to choice the right type of intervention. Physiotherapy has become task oriented and specifically goal directed, based on documented principles of motor learning, strength and fitness training. Correct choose and use of orthosis is an essential part of the treatment. The different types of cerebral palsy have different natural progressions, risk factors and needs of follow-up. A multidisciplinary clinical evaluation is therefore needed as a basis for choosing the right management strategy. Given the complexity of this disorder, priority between various focus areas for different age groups is a challenge. A close collaboration with the parents is therefore essential. Children with cerebral palsy are a relatively small group, and the increased specialisation may indicate that parts of the treatment should be centralised.

  2. 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.......,580 live births from 2003 to 2008. RESULTS: The study included 43 children diagnosed with CP. The overall prevalence of CP was 2.4 per 1,000 live births (95% confidence interval (CI): 1.8-3.2). The gestational age (GA)-specific prevalence ranged from 63.5 per 1,000 live births for GA

  3. CT findings in patients with cerebral palsy

    Energy Technology Data Exchange (ETDEWEB)

    Konno, K. (Akita Univ. (Japan))

    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.

  4. Botulinum toxin in children with cerebral palsy.

    Science.gov (United States)

    Singhi, Pratibha; Ray, Munni

    2004-12-01

    Botulinum toxin is a neurotoxin that blocks the synaptic release of acetylcholine from cholinergic nerve terminals mainly at the neuromuscular junction, resulting in irreversible loss of motor end plates. It is being widely tried as a targeted antispasticity treatment in children with cerebral palsy. A number of studies have shown that it reduces spasticity and increases the range of motion and is particularly useful in cases with dynamic contractures. However improvement in function has not been convincingly demonstrated. It is an expensive mode of therapy and the injections need to be repeated after 3-6 months. Whereas Botulinum toxin can be a valuable adjunct in select cases, it should not be projected as a panacea for children with spastic cerebral palsy.

  5. 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.

  6. 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.

  7. Lower urinary tract dysfunction in cerebral palsy.

    OpenAIRE

    Reid, C. J.; Borzyskowski, M

    1993-01-01

    The clinical features and management of 27 children with cerebral palsy referred with symptoms of lower urinary tract dysfunction were reviewed. The mean age at referral was 9.9 years. Daytime urinary incontinence was the commonest presenting symptom. Videourodynamic studies were abnormal in 23 patients (85%). Only two children had evidence of upper renal tract damage. Treatment was determined by urodynamic findings, and led to improvement in symptoms in all patients for whom there was follow...

  8. Peripheral Facial Nerve Palsy after Therapeutic Endoscopy

    OpenAIRE

    Kim, Eun Jeong; Lee, Jun; Lee, Ji Woon; Lee, Jun Hyung; Park, Chol Jin; Kim, Young Dae; Lee, Hyun Jin

    2015-01-01

    Peripheral facial nerve palsy (FNP) is a mononeuropathy that affects the peripheral part of the facial nerve. Primary causes of peripheral FNP remain largely unknown, but detectable causes include systemic infections (viral and others), trauma, ischemia, tumor, and extrinsic compression. Peripheral FNP in relation to extrinsic compression has rarely been described in case reports. Here, we report a case of a 71-year-old man who was diagnosed with peripheral FNP following endoscopic submucosal...

  9. 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.

  10. Neonatal brachial plexus palsy: a permanent challenge

    Directory of Open Access Journals (Sweden)

    Carlos Otto Heise

    2015-09-01

    Full Text Available Neonatal brachial plexus palsy (NBPP has an incidence of 1.5 cases per 1000 live births and it has not declined despite recent advances in obstetrics. Most patients will recover spontaneously, but some will remain severely handicapped. Rehabilitation is important in most cases and brachial plexus surgery can improve the functional outcome of selected patients. This review highlights the current management of infants with NBPP, including conservative and operative approaches.

  11. Literacy development in children with cerebral palsy

    OpenAIRE

    Critten, Valerie

    2013-01-01

    This thesis concerns the literacy difficulties of fifteen children with cerebral palsy (CP). The children were an opportunistic sample from two schools, and were initially selected on the basis that they had typical abilities in other school subjects. A review of the literature pertaining to the development of literacy and related aspects of cognitive development in typically-developing children and children with CP informed the development of the research strategy. The children's literacy, t...

  12. Worster-Drought Syndrome: Poorly Recognized despite Severe and Persistent Difficulties with Feeding and Speech

    Science.gov (United States)

    Clark, Maria; Harris, Rebecca; Jolleff, Nicola; Price, Katie; Neville, Brian G. R.

    2010-01-01

    Aim: Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is a permanent movement disorder of the bulbar muscles causing persistent difficulties with swallowing, feeding, speech, and saliva control owing to a non-progressive disturbance in early brain development. As such, it falls within the cerebral palsies. The aim of this study…

  13. 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.

  14. Untitled

    African Journals Online (AJOL)

    sclerosis (ALS), Primary lateral sclerosis, progressive bulbar palsy, and spinal muscular atrophy (1,2). Although several putative agents(3), which include neurotoxins, glutamate toxicity (4), oxidative stress, and trauma (5.6,) have been implicated in the causation of motor neurone disease, mutations ot'superoxide dismutase ...

  15. [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.

  16. Epilepsy in children with cerebral palsy.

    Science.gov (United States)

    Aneja, S; Ahuja, B; Taluja, V; Bhatia, V K

    2001-02-01

    This article deals with the clinical profile of children with cerebral palsy and epilepsy, and to study the clinical predictors of response to anti-epileptic drugs. It is a prospective hospital based follow-up study. All the children who presented with cerebral palsy and history of seizure (other than neonatal seizures) over a period of one year were included. Seizures were classified according to ILAE classification. An EEG was obtained in all cases. Neuroimaging was done in all patients. Eighty-five patients were studied and followed for minimum of 12 months. Perinatal factors accounted for 62 (72.3%) cases. The motor deficits seen were quadriparesis (n = 64), hemiplegia (n = 12) and diplegia (n = 9). Associated mental retardation was seen in 80.9% patients with quadriparesis. A predominance of generalised epilepsy was seen with generalised tonic clonic seizures (32.9%) followed by mycolonic seizures (30.6%) and localisation related epilepsy (24.7%). The patients with quadriparesis were more likely to have generalised epilepsy and 52.4% of them required two or more anti-epileptic drugs for control of seizures. Patients with hemiplegia had localisation related epilepsy in 83.3% of cases. On multivariate analysis presence of quadriparesis, microcephaly, mental retardation and myoclonic epilepsy were found to predict the poor response to AED. Epilepsy in patients with cerebral palsy is of severe nature and difficult to control. Presence of quadriparesis, mental retardation and myoclonic seizures was predictive of poor response to anti- epileptic drugs.

  17. Acute Myeloid Leukaemia Presenting as Gaze Palsy

    Directory of Open Access Journals (Sweden)

    Evripidis Sykakis

    2011-11-01

    Full Text Available The most frequent initial ocular manifestation of acute myeloid leukaemia (AML is retinal involvement. Here, we report an unusual case of AML associated with a pontine chloroma presenting with gaze palsy as the initial symptom. A 77-year-old Caucasian man presented to the Eye Casualty complaining of a one-day history of blurred vision. On examination, his face was turned to the left, both eyes were fixed in dextroversion and the patient demonstrated left gaze palsy associated with left motor neurone VII palsy. Baseline blood investigations revealed leucocytosis with 60% circulating myeloblasts. A bone marrow biopsy confirmed the diagnosis of myelomonocytic leukaemia. A CT scan showed a well-circumscribed lesion in the dorsal pons, most likely representing a chloroma. Chloromas or myeloblastomas related to AML are localised extramedullary tumours composed of leukaemic myeloid cells. Chloromas may be present at the time of the initial diagnosis of leukaemia or may precede the diagnosis by 1 month to 2 years; however, their occurrence in the central nervous system is rare, comprising 1–6% of all chloromas. This case illustrates the many different ways that AML can manifest itself in the eyes, and ophthalmologists should be aware of the great variety of presenting symptoms in undiagnosed AML.

  18. [Influence of early stimulation in cerebral palsy].

    Science.gov (United States)

    García-Navarro, M E; Tacoronte, M; Sarduy, I; Abdo, A; Galvizú, R; Torres, A; Leal, E

    Early stimulation is known to be useful, necessary treatment aimed at developing as much as possible the social psychophysical potential of any child at high environmental and/or biological risk. This group includes children with cerebral palsy, a disorder of the nervous system which may cause retardation in the processes of maturation of the central nervous system and be expressed from the earliest months of the child's life as retardation of psychomotor development. To show the efficiency of early stimulation in children diagnosed as having cerebral palsy and retardation of psychomotor development. A retrospective study was made of 20 children aged between 9 and 41 months with this diagnosis, in the hospital of CIREN (Cuba). They were treated for a period of 1 to 3 months by a multi-disciplinary team and participated in the programme for Early Stimulation. Assessment was made by the Neuropsychology Department at the start and end of the treatment period, using the first part of the Brunet-Lezine scale for the measurement of psychomotor development in early childhood. In all patients there was a favorable course and new abilities were acquired. There was better performance than before the treatment was started and accelerated rate of development during the period of treatment. Patients with cerebral palsy and psychomotor retardation benefit from application of a programme of Early Stimulation.

  19. Artificial urinary sphincter in male patients with spina bifida: Comparison of perioperative and functional outcomes between bulbar urethra and bladder neck cuff placement.

    Science.gov (United States)

    Khene, Zine-Eddine; Paret, Fanny; Perrouin-Verbe, Marie-Aime E; Prudhomme, Thomas; Hascoet, Juliette; Nedelec, Mathilde; Kerdraon, Jacques; Menard, Helene; Jezequel, Magali; Le Normand, Loïc; Manunta, Andrea; Game, Xavier; Peyronnet, Benoit

    2017-10-13

    To evaluate the perioperative and long-term functional outcomes of bladder neck and peribulbar cuff placement of an artificial urinary sphincter (AUS) in a population of adult male patients with spinal dysraphism. A retrospective analysis of the French spina bifida (SB) network database was performed. Patients who underwent implantation of an AUS from January 1985 to November 2015 were selected and stratified into two groups according to cuff location: bladder neck vs. bulbar urethra. Explantation-free and revision-free device survivals were estimated using the Kaplan-Meier method and compared using the log rank test. Cox regression models were used to assess prognostic factors of AUS device failure. Sixty-five patients were included. Most patients were not wheelchair-bound. The cuff was implanted around the bulbar urethra in 46 procedures (59%) and around the bladder neck in 32 procedures (41%). Median revision-free device survivals were 11.7 and 14.3 years (p=0.73) and median explantation-free device survivals were 18.5 and 24.5 years (p=0.08) in peribulbar and bladder neck groups respectively. In multivariate analysis, clean-intermittent catheterization was the only predictor of AUS device failure (no influence of cuff location). At the last follow-up, satisfactory continence was similar in both groups (83% vs. 75%, p=0.75). In SB male patients, the morbidity and functional outcomes were similar between bladder neck and bulbar urethra cuff placement but with a trend towards a longer survival without explantation in the bladder neck group. Clean-intermittent catheterization was the only predictor of shorter device survival in multivariate analysis. Copyright © 2017 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  20. Manual muscle test at C5 palsy onset predicts the likelihood of and time to C5 palsy resolution.

    Science.gov (United States)

    Macki, Mohamed; Alam, Ridwan; Kerezoudis, Panagiotis; Gokaslan, Ziya; Bydon, Ali; Bydon, Mohamad

    2016-02-01

    The primary objective of this study was to identify time to and prognostic factors of C5 palsy resolution. All patients over a 7 year period who experienced C5 palsy following a posterior decompression and instrumented fusion surgery were retrospectively reviewed. C5 palsy resolution was defined as a recovery of deltoid muscle function equal to or greater than the preoperative condition as defined by the manual muscle test (MMT). Of the 511 patients who met the selection criteria, 8.6% (n=44) experienced C5 palsy. MMT information was available for 43 patients; 81.4% (n=35) had full resolution from their condition. Of the 35 patients who resolved, the median MMT score at onset was 3-. Following a discrete-time proportional hazards model, the hazards of C5 palsy resolution increased by 19% for every one-grade increase in MMT score at symptom onset (hazard ratio [HR]=1.19, p=0.005). Moreover, males displayed a 71% lower hazard of resolution than females (HR=0.29, p=0.003). Following an adjusted Kaplan-Meier analysis, the median time to C5 palsy resolution was between 6 months and 1 year. In a multiple linear regression, a lower MMT score at the onset of C5 palsy predicted a longer time to C5 palsy resolution (coefficient=-0.19, p=0.003). Time to C5 palsy onset was not statistically associated with hazards of palsy resolution (p=0.381) or time to resolution (p=0.121). A higher MMT score at the onset of C5 palsy statistically significantly predicted a higher chance of resolution and a shorter recovery time. Female sex was also associated with a higher hazard of resolution. Copyright © 2015 Elsevier Ltd. All rights reserved.

  1. Palliative hip surgery in severe cerebral palsy: a systematic review

    NARCIS (Netherlands)

    Boldingh, E.J.K.; Bouwhuis, C.B.; van der Heijden-Maessen, H.; Bos, C.F.; Lankhorst, G.J.

    2014-01-01

    We performed a systematic review of the results of palliative hip surgery in severe cerebral palsy. Individuals with severe cerebral palsy frequently suffer from pain and other impairments because of dislocation or malformation of the hips. When preventive or reconstructive surgery fails, palliative

  2. 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…

  3. Bilateral lower motor neuron facial nerve palsy due to HIV ...

    African Journals Online (AJOL)

    A 34-year-old-woman presented with acute onset of headache and bilateral facial nerve paralysis. On examination bilateral lower motor neuron 7th cranial nerve palsy in keeping with bilateral Bell's palsy was apparent. Investigations showed aseptic meningitis, with a low CD4 count of 352 cells/μl and an elevated viral load ...

  4. Risk factors for recurrent nerve palsy after thyroid surgery

    DEFF Research Database (Denmark)

    Godballe, Christian; Madsen, Anders Rørbæk; Sørensen, Christian Hjort

    2014-01-01

    with thyroid surgery from 1 January 2001 to the 31 December 2008 at the Danish departments of ENT-HNS were analyzed. Unilateral RLN palsy was found in 2.1 % and bilateral in 0.1 %. In benign histology, RLN palsies were registered in 1.3 %. Malignant histology and accordingly neck dissection were the most...

  5. Facial Nerve palsy: the Experience at a Nigerian Teaching Hospital ...

    African Journals Online (AJOL)

    Background: We observed at our center that facial nerve palsy (FNP) presented in crops at a particular season of the year and was usually associated with emotional stress on the patient. Objective: The aims of this study were to evaluate the aetiology, and the management options for patient with facial nerve palsy in our ...

  6. Cranial nerve palsies in Nigerian children | Eyong | Nigerian Journal ...

    African Journals Online (AJOL)

    Background: Cranial nerve palsies are common clinical problem routinely encountered in neurological practice; the dysfunction can occur at any point in the course of the nerve and may point to serious pathology. The aim of this study was to determine the pattern and underlying aetiology of cranial nerve palsies in Nigerian ...

  7. Risk Factors for Cerebral Palsy in Children in Botswana.

    Science.gov (United States)

    Monokwane, Baphaleng; Johnson, Allison; Gambrah-Sampaney, Claudia; Khurana, Esha; Baier, James; Baranov, Esther; Westmoreland, Kate D; Mazhani, Loeto; Steenhoff, Andrew P; Bearden, David R

    2017-12-01

    Although cerebral palsy is reported to have a higher prevalence in low-resource settings, there are few studies describing risk factors for cerebral palsy in these settings. A better understanding of the unique risk factors affecting children with cerebral palsy in low-resource settings could optimize both resource allocation and preventative strategies. A case-control study comparing children with cerebral palsy at ages two to 18 years with age-matched healthy control subjects was conducted between 2013 and 2014 at a referral center in Gaborone, Botswana. Study participants were enrolled from inpatient and outpatient settings, and data were collected through caregiver interviews, review of medical records, and physical examination of subjects. Risk factors were evaluated using conditional logistic regression models. We studied 56 subjects with cerebral palsy and 56 age-matched control subjects. Significant risk factors for cerebral palsy included a history of serious neonatal infection (odds ratio 15.0, P = 0.009), complications during delivery (odds ratio 13.5, P cerebral palsy in Botswana differ from those described in high-resource settings. Modifiable risk factors such as maternal HIV infection should be targeted as a potential strategy to reduce the incidence of cerebral palsy in Botswana. Further studies are necessary to determine optimal preventative and treatment strategies in this population. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. 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.)

  9. Mirror movements in progressive hemifacial atrophy

    Directory of Open Access Journals (Sweden)

    Rajesh Verma

    2015-01-01

    Full Text Available Mirror movements are simultaneous, involuntary, identical movements occurring during contralateral voluntary movements. These movements are considered as soft neurologic signs seen uncommonly in clinical practice. The mirror movements are described in various neurological disorders which include parkinsonism, cranio veretebral junction anamolies, and hemiplegic cerebral palsy. These movements are intriguing and can pose significant disability. However, no such observation regarding mirror movements in progressive hemifacial atrophy have been reported previously. We are reporting a teenage girl suffering from progressive hemifacial atrophy and epilepsy with demonstrable mirror movements in hand.

  10. Amyotrophic lateral sclerosis progression: Iran-ALS clinical registry, a multicentre study.

    Science.gov (United States)

    Shamshiri, Hosein; Fatehi, Farzad; Davoudi, Farnoush; Mir, Elham; Pourmirza, Behin; Abolfazli, Roya; Etemadifar, Masoud; Harirchian, Mohammad Hossein; Gharagozli, Koroush; Ayromlou, Hormoz; Basiri, Keivan; Zamani, Babak; Rohani, Mohammad; Sedighi, Behnaz; Roudbari, Ali; Delavar Kasmaei, Hossein; Nikkhah, Karim; Ranjbar Naeini, Alireza; Nafissi, Shahriar

    2015-01-01

    This study was designed to evaluate ALS progression among different subgroups of Iranian patients. Three hundred and fifty-eight patients from centres around the country were registered and their progression rate was evaluated using several scores including Manual Muscle Test scoring (MMT) and the revised ALS Functional Rating Scale (ALSFRS-R). Progression rate was analysed separately in subgroups regarding gender, onset site, stage of disease and riluzole consumption. A significant difference in MMT deterioration rate (p = 0.01) was noted between those who used riluzole and those who did not. No significant difference was observed in progression rates between male/female and bulbar-onset/limb-onset groups using riluzole. In conclusion, riluzole has a significant effect on muscle force deterioration rate but not functional scale. Progression rate was not influenced by site of onset or gender.

  11. Intrathecal baclofen for treating spasticity in children with cerebral palsy.

    Science.gov (United States)

    Hasnat, Monika J; Rice, James E

    2015-11-13

    Cerebral palsy is a disorder of movement and posture arising from a non-progressive lesion in the developing brain. Spasticity, a disorder of increased muscle tone, is the most common motor difficulty and is associated with activity limitation to varying degrees in mobility and self care.Oral baclofen, a gamma-aminobutyric acid (GABA) agonist, has been used in oral form to treat spasticity for some time, but it has a variable effect on spasticity and the dose is limited by the unwanted effect of excessive sedation. Intrathecal baclofen produces higher local concentrations in cerebrospinal fluid at a fraction of the equivalent oral dose and avoids this excessive sedation. To determine whether intrathecal baclofen is an effective treatment for spasticity in children with cerebral palsy. We searched the CENTRAL, MEDLINE, EMBASE and CINAHL databases, handsearched recent conference proceedings, and communicated with researchers in the field and pharmaceutical and drug delivery system companies. We included studies which compared the effect of intrathecal baclofen treatment on spasticity, gross motor function or other areas of function with controls. Two authors selected studies, two authors extracted data and two authors assessed the methodological quality of included studies. Six studies met the inclusion criteria. The data obtained were unsuitable for the conduct of a meta-analysis; we have completed a qualitative summary.All studies were found to have high or unclear risk of bias in some aspects of their methodology.Five of the six studies reported data collected in the randomised controlled phase of the study. A sixth study did not report sufficient results to determine the effect of intrathecal baclofen versus placebo. Of these five studies, four were conducted using lumbar puncture or other short-term means of delivering intrathecal baclofen. One study assessed the effectiveness of implantable intrathecal baclofen pumps over six months.The four short-term studies

  12. Ophthalmic profile and systemic features of pediatric facial nerve palsy.

    Science.gov (United States)

    Patil-Chhablani, Preeti; Murthy, Sowmya; Swaminathan, Meenakshi

    2015-12-01

    Facial nerve palsy (FNP) occurs less frequently in children as compared to adults but most cases are secondary to an identifiable cause. These children may have a variety of ocular and systemic features associated with the palsy and need detailed ophthalmic and systemic evaluation. This was a retrospective chart review of all the cases of FNP below the age of 16 years, presenting to a tertiary ophthalmic hospital over the period of 9 years, from January 2000 to December 2008. A total of 22 patients were included in the study. The average age at presentation was 6.08 years (range, 4 months to 16 years). Only one patient (4.54%) had bilateral FNP and 21 cases (95.45%) had unilateral FNP. Seventeen patients (77.27%) had congenital palsy and of these, five patients had a syndromic association, three had birth trauma and nine patients had idiopathic palsy. Five patients (22.72%) had an acquired palsy, of these, two had a traumatic cause and one patient each had neoplastic origin of the palsy, iatrogenic palsy after surgery for hemangioma and idiopathic palsy. Three patients had ipsilateral sixth nerve palsy, two children were diagnosed to have Moebius syndrome, one child had an ipsilateral Duane's syndrome with ipsilateral hearing loss. Corneal involvement was seen in eight patients (36.36%). Amblyopia was seen in ten patients (45.45%). Neuroimaging studies showed evidence of trauma, posterior fossa cysts, pontine gliosis and neoplasms such as a chloroma. Systemic associations included hemifacial macrosomia, oculovertebral malformations, Dandy Walker syndrome, Moebius syndrome and cerebral palsy FNP in children can have a number of underlying causes, some of which may be life threatening. It can also result in serious ocular complications including corneal perforation and severe amblyopia. These children require a multifaceted approach to their care.

  13. Spontaneous carotid dissection presenting lower cranial nerve palsies.

    Science.gov (United States)

    Guidetti, D; Pisanello, A; Giovanardi, F; Morandi, C; Zuccoli, G; Troiso, A

    2001-03-01

    Cranial nerve palsy in internal carotid artery (ICA) dissection occurs in 3--12% of all patients, but in 3% of these a syndrome of hemicranias and ipsilateral cranial nerve palsy is the sole manifestation of ICA dissection, and in 0.5% of cases there is only cranial nerve palsy without headache. We present two cases of lower cranial nerve palsy. The first patient, a 49-year-old woman, developed left eleventh and twelfth cranial nerve palsies and ipsilateral neck pain. The angio-RM showed an ICA dissection with stenosis of 50%, beginning about 2 cm before the carotid channel. The patient was treated with oral anticoagulant therapy and gradually improved, until complete clinical recovery. The second patient, a 38-year-old woman, presented right hemiparesis and neck pain. The left ICA dissection, beginning 2 cm distal to the bulb, was shown by ultrasound scanning of the carotid and confirmed by MR angiogram and angiography with lumen stenosis of 90%. Following hospitalisation, 20 days from the onset of symptoms, paresis of the left trapezius and sternocleidomastoideus muscles became evident. The patient was treated with oral anticoagulant therapy and only a slight right arm paresis was present at 10 months follow-up. Cranial nerve palsy is not rare in ICA dissection, and the lower cranial nerve palsies in various combinations constitute the main syndrome, but in most cases these are present with the motor or sensory deficit due to cerebral ischemia, along with headache or Horner's syndrome. In the diagnosis of the first case, there was further difficulty because the cranial nerve palsy was isolated without hemiparesis, and the second case presented a rare association of hemiparesis and palsy of the eleventh cranial nerve alone. Compression or stretching of the nerve by the expanded artery may explain the palsies, but an alternative cause is also possible, namely the interruption of the nutrient vessels supplying the nerve, which in our patients is more likely.

  14. 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

    2005-01-01

    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

  15. 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.

  16. 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.

  17. Effect of serial casting in spastic cerebral palsy.

    Science.gov (United States)

    Jain, Shweta; Mathur, Navnendra; Joshi, Mrinal; Jindal, Rajeshwari; Goenka, Sunil

    2008-10-01

    Cerebral palsy (CP) is a range of non progressive syndromes of posture and motor impairment due to an insult to developing brain. Spasticity and incoordination are major causes of disability in these children which can be managed by different modalities like casting, botulinum toxin, surgery etc. Cast application in spastic equinus is a well established procedure in CP but cast application in patients of CP with bilateral involvement of hip, knee and ankle is not document. A study was conducted on 22 children of spastic CP in age range of 3-8 years with bilateral involvement of hip, knee and ankle in 20 cases, hip and ankle in one case and only ankle in one case. Sixty eight % children were spastic diplegics. Serial weekly cast with (11 cases) or without abductor bar (11 cases) was applied for four weeks. They were followed up variably with an average period of 7 months. Significant improvement was noticed in range of motion around hip, knee and ankle which as maintained over hip and knee after average follow up. Spasticity was also reduced as measured by Modified Ashworth Scale (MAS). This ultimately improved the ambulatory status and functional ability of these children. Thus serial casting is a very simple, safe and cost effective procedure which can be applied even in children with mental sub normality having all three major joints involved bilaterally.

  18. On the Use of Dance as a Rehabilitation Approach for Children with Cerebral Palsy: A Single Case Study.

    Science.gov (United States)

    Morán Pascual, Patricia; Mortes Roselló, Esther; Domingo Jacinto, Amparo; Belda Lois, Juan Manuel; Bermejo, Ignacio; Medina, Enric; Barberà Guillem, Ricard

    2015-01-01

    Cerebral Palsy (CP) is the most common motor disability in childhood. It is a group of permanent disorders that affect child development causing disorders of movement and posture and activity limitations. The impairment of psychomotor skills of children with Cerebral Palsy is attributed to a permanent alteration occurred in non-progressive brain development of the fetus or nursing infant. Some motor related symptoms can be treated using proper physical therapy. However, one of the biggest problems of the usual physical therapy is adherence to therapy. Ballet can be an alternative or a complement to physiotherapy, with the added attraction of not being part of a to therapy, but a fun activity with the extra reward associated with the realization of an artistic activity. For some years the ballet is used as therapeutically valuable for both children with cerebral palsy: Intensive ballet training can generate changes in the sensorimotor cortex. Ballet is characterized by a complex process of movements that have to be in a musical rhythm (hence have to be precise), in which there is an overall coordination of the muscles. It is also a highly motivating and rewarding activity that allows many children with CP sharing the activities of their peers without special needs. Objective measurements of the Full Port de Bras movement has been chosen as an index of improvement. The results shows progressive improvements of the execution in a single case.

  19. 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...

  20. Peripheral facial nerve palsy after therapeutic endoscopy.

    Science.gov (United States)

    Kim, Eun Jeong; Lee, Jun; Lee, Ji Woon; Lee, Jun Hyung; Park, Chol Jin; Kim, Young Dae; Lee, Hyun Jin

    2015-03-01

    Peripheral facial nerve palsy (FNP) is a mononeuropathy that affects the peripheral part of the facial nerve. Primary causes of peripheral FNP remain largely unknown, but detectable causes include systemic infections (viral and others), trauma, ischemia, tumor, and extrinsic compression. Peripheral FNP in relation to extrinsic compression has rarely been described in case reports. Here, we report a case of a 71-year-old man who was diagnosed with peripheral FNP following endoscopic submucosal dissection. This case is the first report of the development of peripheral FNP in a patient undergoing therapeutic endoscopy. We emphasize the fact that physicians should be attentive to the development of peripheral FNP following therapeutic endoscopy.

  1. Recent advances in management of cerebral palsy.

    Science.gov (United States)

    Sharan, Deepak

    2005-11-01

    Recent advances in clinical research have increased our understanding of causal pathways, opportunities for primary prevention, and the value of specific intervention strategies in the management of Cerebral Palsy (CP). Despite the increasing popularity of newer treatment alternatives, e.g., Botulinum Toxin and Intrathecal Baclofen, single event multilevel Orthopaedic bony and soft tissue surgery, in the context of a multi-disciplinary rehabilitation team, remains a vital component in the overall management plan. A meticulous clinical examination, augmented in some cases by instrumented gait analysis, allows for a comprehensive treatment plan addressing the entire extremity. This paper provides a critical review of the currently available treatment modalities.

  2. 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.

  3. 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.

  4. 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.

  5. Treatment Of Scoliosis In Children With Cerebral Palsy Using The Vertical Expandable Prosthetic Titanium Rib (veptr) [tratamento Da Escoliose Em Crianças Com Paralisia Cerebral Utilizando A Prótese Vertical Expansível De Titânio Para Costela (veptr)

    OpenAIRE

    De Quental Tyba K.; Maiacavali P.T.; Santos M.A.M.; Junqueirarossato A.; Lehoczki M.A.; Risso-Neto M.I.; Veiga I.G.; Landim E.

    2011-01-01

    Objective: To evaluate the use of vertical expandable prosthetic titanium rib (VEPTR) as an option for initial treatment of scoliosis in younger children with cerebral palsy. Methods: We evaluated 10 patients with cerebral palsy (CP) treated with VEPTR by the group of scoliosis of the AACD in Sao Paulo. The characteristics of the subjects were progressive scoliosis and skeletal immaturity without severe deformity in the sagittal plane. We evaluated the curve by the Cobb method pre-and postope...

  6. Incidence of C5 nerve root palsy after cervical surgery

    Science.gov (United States)

    Wang, Tao; Wang, Hui; Liu, Sen; Ding, Wen-Yuan

    2017-01-01

    Abstract Purpose: We aim to perform a meta-analysis on incidence of C5 nerve root palsy (C5 palsy) for patients after cervical surgery. Methods: An extensive search of the literature was performed in PubMed/MEDLINE, Embase, the Cochrane library, CNKI, and WANFANG databases on incidence of C5 palsy from January 2007 to January 2017. Prevalence of C5 palsy related to different surgery methods was calculated and data analysis was conducted with STATA 12.0. Results: A total of 61 studies containing 721 patients with C5 palsy in total 11,481 patients (6.3%) were included in our study. The incidences after anterior cervical discectomy and fusion (ACDF), anterior cervical corpectomy and fusion (ACCF), anterior corpectomy combined with discectomy (ACCDF), laminoplasty (LP) and laminectomy and fusion (LF) were 5.5%, 7.5%, 6%, 4.4%, and 12.2%, respectively. Compared with anterior approaches (5%), female patients (4%) and patients with cervical spondylotic myelopathy (CSM) (4.8%), posterior approaches (6.2%), male patients (5.7%) and patients with ossification of posterior longitudinal ligament (OPLL) (8.1%) have a higher prevalence. In ACDF and LP, patients with OPLL (5.5%, 8.1%, respectively) have a higher incidence than those in patients with CSM (4.7%, 3.1%, respectively); however, in LF, patients with CSM and OPLL have similar incidence of C5 palsy (13% vs 13.1%). In most cases, C5 palsy was unilateral (74.5%). Conclusions: Based on our meta-analysis, posterior approaches, male patients and patients with OPLL have a higher incidence of C5 palsy. In ACDF and LP, patients with OPLL have a higher incidence of C5 palsy, but in LF, patients with CSM and OPLL have similar result. PMID:29137073

  7. Unilateral isolated hypoglossal nerve palsy due to pathologically adherent PICA fusiform aneurysm – A case report

    Science.gov (United States)

    Ekuma, Mike E.; Goto, Tetsuya; Hanaoka, Yoshiki; Kanaya, Kohei; Horiuchi, Tetsuyoshi; Hongo, Kazuhiro; Ohaegbulam, Samuel C.

    2017-01-01

    Background: Isolated hypoglossal nerve palsy due to mechanical compression by a vascular lesion is rare. Case Description: We report the case of a 72-year-old man who presented with a 4-year history of swallowing disturbance and subsequently progressively worsening left-sided tongue atrophy. He was referred to our department by a neurologist due a magnetic resonance imaging detected left vertebral artery compression of the medulla. Neurological examination was unremarkable except for left hypoglossal nerve dysfunction, which presented as left-sided atrophy and impaired movement of the tongue. Three-dimensional computed tomography angiography showed proximal left posterior inferior cerebellar artery (PICA) origin fusiform aneurysm. Microvascular decompression was done through a left transcondylar fossa approach. Intraoperative findings were thickened arachnoid around the lower cranial nerves, fusiform aneurysm of the left PICA at its origin from the left vertebral artery which was severely adherent to and compressing the left hypoglossal nerve rootlets. Conclusion: The PICA has a very close relationship to the hypoglossal nerve, and its fusiform dilatation could cause isolated hypoglossal nerve dysfunction. Pathological adhesions between hypoglossal rootlets and the PICA aneurysm wall could be a possible contributor in the development and progression of hypoglossal nerve palsy. PMID:28680733

  8. Mitrofanoff urinary diversion in a patient with cerebral palsy

    Directory of Open Access Journals (Sweden)

    Sayalee S Narwade

    2017-05-01

    Full Text Available Cerebral palsy is the most common motor disability in childhood which result in huge socioeconomic costs. This children have a significant incidence of lower urinary tract symptoms. Clean intermittent self-catheterization is needed to avoid deterioration of renal function. But significant spasticity and resulting contractures of the adductors can interfere with the caretakers’ ability to provide perineal hygiene. Surgery in cerebral palsy affected child is challenging due to multiple associated comorbidities. The aim of this report is to describe quality of life and renal function for a mentally retarded child with cerebral palsy before and after Mitrofanoff diversion without bladder augmentation

  9. The history of facial palsy and spasm: Hippocrates to Razi.

    Science.gov (United States)

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

    2011-07-12

    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.

  10. Contralateral diaphragmatic palsy in acute stroke: an interesting observation.

    Science.gov (United States)

    Kumar, Sudhir; Reddy, Rajesh; Prabhakar, Subhashini

    2009-01-01

    Diaphragmatic palsy in hemiparetic stroke is not well recognized. Further, its implications on stroke outcome have not been studied. Here, we report a patient with left-sided diaphragmatic palsy due to an acute right middle cerebral artery territory infarction. The diagnosis was suspected on finding an elevated dome of the diaphragm on the left side in a routine chest radiograph and was confirmed by finding decreased movements of the left hemidiaphragm on fluoroscopic examination. We hypothesize that this condition is probably under-recognized in clinical practice and its clinical importance not well known. The pathophysiological basis of diaphragmatic palsy in acute stroke and its possible clinical implications are discussed.

  11. [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.

  12. 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...

  13. Natural history of scoliosis in nonambulatory spastic tetraplegic cerebral palsy.

    Science.gov (United States)

    Gu, Yaoming; Shelton, Jean E; Ketchum, Jessica M; Cifu, David X; Palmer, Dorothy; Sparkman, Ann; Jermer-Gu, Melinda K; Mendigorin, Marianne

    2011-01-01

    To analyze the development and progression of scoliosis in children and adolescents with nonambulatory spastic tetraplegic cerebral palsy. Retrospective longitudinal review. Pediatric nursing home. A total of 110 children and adolescents history of hip dislocation, tracheostomy. The Cobb angle increased with age, weight, and height in a nonlinear fashion. A square root transformation of the Cobb angle was chosen to model the nonlinear relationship between the Cobb angle and predictors. Age, height, and weight were significant univariate predictors of the square root of the Cobb angle (slopes = 0.377, 0.067, and 0.06, respectively). In the multivariate mixed model, age remained a significant predictor of the Cobb angle (slope = 0.456), but height and weight did not. If the Cobb angle was > 40° by age 12 years, scoliosis was more likely to progress than if the Cobb angle was ≤ 40°. The effect of age was stronger for those with history of tracheostomy (age slope = 0.631 vs 0.281) than those without. The relationship of age and Cobb angle did not differ significantly between hip dislocated and non-hip-dislocated groups. Age was found to be the most significant predictor of Cobb angle, and the effect of age was greater in the tracheostomy group than in the nontracheostomy group. After adjustment for age, the weight and height were not significant predictors of Cobb angle. Cobb angles of > 40° by the age of 12 years were associated with greater increases in Cobb angle with age. Copyright © 2011 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.

  14. 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.

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

    Science.gov (United States)

    Basu, Anna Purna; Pearse, Janice; Kelly, Susan; Wisher, Vicki; Kisler, Jill

    2014-01-01

    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 (CIMT) 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 CIMT 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.

  16. 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.......2) were associated with CP in all births, in term births (HR 1.9, 95% CI 1.1, 3.2), in children with spastic CP (HR 2.1, 95% CI 1.4, 3.3), and among first-born children (HR 1.9, 95% CI 1.4, 3.3). Overall, we found associations between redeemed nitrofurantoin (HR 1.7, 95% CI 1.1, 2.8) and CP. Among...

  17. Gait analysis in children with cerebral palsy

    Science.gov (United States)

    Armand, Stéphane; Decoulon, Geraldo; Bonnefoy-Mazure, Alice

    2016-01-01

    Cerebral palsy (CP) children present complex and heterogeneous motor disorders that cause gait deviations. Clinical gait analysis (CGA) is needed to identify, understand and support the management of gait deviations in CP. CGA assesses a large amount of quantitative data concerning patients’ gait characteristics, such as video, kinematics, kinetics, electromyography and plantar pressure data. Common gait deviations in CP can be grouped into the gait patterns of spastic hemiplegia (drop foot, equinus with different knee positions) and spastic diplegia (true equinus, jump, apparent equinus and crouch) to facilitate communication. However, gait deviations in CP tend to be a continuum of deviations rather than well delineated groups. To interpret CGA, it is necessary to link gait deviations to clinical impairments and to distinguish primary gait deviations from compensatory strategies. CGA does not tell us how to treat a CP patient, but can provide objective identification of gait deviations and further the understanding of gait deviations. Numerous treatment options are available to manage gait deviations in CP. Generally, treatments strive to limit secondary deformations, re-establish the lever arm function and preserve muscle strength. Additional roles of CGA are to better understand the effects of treatments on gait deviations. Cite this article: Armand S, Decoulon G, Bonnefoy-Mazure A. Gait analysis in children with cerebral palsy. EFORT Open Rev 2016;1:448-460. DOI: 10.1302/2058-5241.1.000052. PMID:28698802

  18. [A 65-year-old man with Parkinsonism, gaze palsy, and dementia].

    Science.gov (United States)

    Mizuno, Y; Yokochi, F; Ohta, S; Mori, H; Takubo, H

    1996-04-01

    We report a 65-year-old man with parkinsonism, supranuclear gaze palsy, and dementia. The patient was well until 58 years of the age (1984) when he noted an onset of tremor in his right hand. He visited our neurology service two years after the onset; neurologic examination at that time revealed moderate restriction in down ward gaze, horizontal gaze nystagmus in left and right gaze, stooped posture with loss of arm swing when he walked, slight rigidity in the neck and the right upper and lower extremities, and resting tremor in his right hand and foot; mentation was intact. He was treated with 600 mg of levodopa with carbidopa; his tremor partially improved. He received left Vim thalamotomy on March 14 of 1987. His tremor disappeared after the thalamotomy. Post-operative course was complicated by transient clouding of consciousness due to subdural hematoma which developed after the surgery. Six months after the surgery, he noted increase in the unsteadiness of gait; he also experienced urinary incontinence once in a while, and he became mentally dull. In November of 1988, he had episodes of stiffening of his body. Although his spontaneous speech was very much reduced, he repeatedly hummed a same tune; no one could make him stop humming. In June of 1989, he was totally unable to move his eyes in the vertical direction. He was hospitalized to another hospital in May of 1990 where he died six month after admission because of pneumonia. The clinical course of this patient was characterized by the onset with parkinsonian resting tremor, and supranuclear gaze palsy and dementia in the later course. The patient was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had an overlap syndrome of progressive supranuclear palsy (PSP) and diffuse Lewy body disease. Majority of the participants thought that the patient had progressive supranuclear palsy, but many of them had an impression that parkinsonian tremor responding to

  19. Multiple cranial nerve palsy revealing hypertrophic pachymeningitis with positive myeloperoxidase-antineutrophil cytoplasmic antibody

    Directory of Open Access Journals (Sweden)

    S. El Aoud

    2013-12-01

    Full Text Available Pachymeningitis is a progressive disease resulting in a diffuse thickening of dura mater due to inflammation, tumor or autoimmune diseases, but most cases are idiopathic. Here, we report the case of a 60-year old man who had a progressive sensorineural hearing loss, visual disturbance and others cranial nerve involvement with an accompanying headache over several months. Brain magnetic resonance imaging showed diffusely thickened dura mater, highly enhanced after gadolinium administration, which was consistent with pachymeningitis. It was assumed to be related to autoimmune pathogenesis on the basis of elevated serum myeloperoxidase-antineutrophil cytoplasmic antibody titers. After empirical steroid and cyclophosphamide therapy, the neurological problems were partially improved. Therefore, in the case of atypical sensorineural hearing loss accompanied by cranial nerve palsy or headache, pachymeningitis should be considered in the differential diagnosis.

  20. 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.

  1. Pseudoradial Nerve Palsy Caused by Acute Ischemic Stroke

    Directory of Open Access Journals (Sweden)

    Hassan Tahir MD

    2016-07-01

    Full Text Available Pseudoperipheral palsy has been used to characterize isolated monoparesis secondary to stroke. Isolated hand nerve palsy is a rare presentation for acute cerebral stroke. Our patient presented with clinical features of typical peripheral radial nerve palsy and a normal computed tomography scan of the head, which, without a detailed history and neurological examination, could have been easily misdiagnosed as a peripheral nerve lesion deferring further investigation for a stroke. We stress the importance of including cerebral infarction as a critical differential diagnosis in patients presenting with sensory-motor deficit in an isolated peripheral nerve pattern. A good history and physical exam can differentiate stroke from peripheral neuropathy as the cause of radial nerve palsy.

  2. Factors affecting epilepsy development and epilepsy prognosis in cerebral palsy.

    Science.gov (United States)

    Mert, Gulen Gul; Incecik, Faruk; Altunbasak, Sakir; Herguner, Ozlem; Mert, Mustafa Kurthan; Kiris, Nurcihan; Unal, Ilker

    2011-08-01

    A study was conducted between November 2006 and October 2009 to determine the factors predicting the presence and prognosis of epilepsy in patients with cerebral palsy. We enrolled 2 groups of patients: 42 with cerebral palsy in group 1 and 56 patients with cerebral palsy and epilepsy in group 2. The subjects in group 2 were considered to have good epilepsy prognosis if they were free of seizures for the previous year; otherwise they were considered to have poor epilepsy prognosis. In group 2, neonatal epilepsy, family history of epilepsy, and moderate to severe mental retardation were significantly higher than in group 1 (P history of epilepsy, and mental retardation were found to be important and independent predictors of development of epilepsy in patients with cerebral palsy. Copyright © 2011 Elsevier Inc. All rights reserved.

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

    DEFF Research Database (Denmark)

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

    2008-01-01

    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......%, and no walking ability was reported for 30%. The proportion of children who were unable to walk was rather stable over time in all of the centers, with a mean proportion of 28%. Walking ability related significantly to cerebral palsy types, that is, spastic unilateral, spastic bilateral, dyskinetic, and ataxic...

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

    Science.gov (United States)

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

    2014-01-01

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

  5. 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...

  6. A specialized walking frame for children with cerebral palsy.

    Science.gov (United States)

    Myles, J W

    1983-11-01

    To help severely physically handicapped children with cerebral palsy gain some independent mobility, a special walking frame of great stability has been evolved. It incorporates a variable-resistance roller and automatic reversing brake, and can be folded.

  7. Postural control in sitting children with cerebral palsy

    NARCIS (Netherlands)

    Brogren, E; Hadders-Algra, M; Forssberg, H

    Children with cerebral palsy (CP) display postural problems, largely interfering with daily life activities. Clarification of neural mechanisms controlling posture in these children could serve as a base for more successful intervention. Studies on postural adjustments following horizontal forward

  8. 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.

  9. Assessment of traumatic dental injuries in patients with cerebral palsy

    Directory of Open Access Journals (Sweden)

    A Dubey

    2015-01-01

    Full Text Available Background: Cerebral palsy is an umbrella term for a group of conditions characterized essentially by motor dysfunctions that may be associated with sensory or cognitive impairment. Such children tend to have a higher incidence of traumatic dental injuries than the general population. This increased incidence is often attributed to poor muscular co-ordination that predisposes individuals with Cerebral palsy to trauma Aim: The study was conducted to assess different dental injuries and the risk factors for dental trauma to occur in patients with cerebral palsy. Materials and Methods: The study comprised 70 children and adolescents with cerebral palsy attending special school in Durg and Bhilai city between 7 and 18 years of age. Results: Dentinal fracture was seen in 40% of cases. Few cases had tooth displacement, discoloration, and pulpal involvement. Conclusion: Dentists should be well aware of the possible dental injuries in such patients. Preventive measure measures should be taken by health care provider to reduce traumatic exposure.

  10. Assessment of traumatic dental injuries in patients with cerebral palsy.

    Science.gov (United States)

    Dubey, A; Ghafoor, P A; Rafeeq, M

    2015-01-01

    Cerebral palsy is an umbrella term for a group of conditions characterized essentially by motor dysfunctions that may be associated with sensory or cognitive impairment. Such children tend to have a higher incidence of traumatic dental injuries than the general population. This increased incidence is often attributed to poor muscular co-ordination that predisposes individuals with Cerebral palsy to trauma Aim: The study was conducted to assess different dental injuries and the risk factors for dental trauma to occur in patients with cerebral palsy. The study comprised 70 children and adolescents with cerebral palsy attending special school in Durg and Bhilai city between 7 and 18 years of age. Dentinal fracture was seen in 40% of cases. Few cases had tooth displacement, discoloration, and pulpal involvement. Dentists should be well aware of the possible dental injuries in such patients. Preventive measure measures should be taken by health care provider to reduce traumatic exposure.

  11. Obstetrical brachial plexus palsy (OBPP) outcome with conservative management

    NARCIS (Netherlands)

    Eng, GD; Binder, H; Getson, P; ODonnell, R

    Resurgence of neurosurgical intervention oi obstetrical brachial plexus palsy prompted our review of 186 patients evaluated between 1981 and 1993, correlating clinical examination, electrodiagnosis, and functional outcome with conservative management. Eighty-eight percent had upper brachial plexus

  12. 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

  13. Effects of exercise in people with cerebral palsy. A review

    National Research Council Canada - National Science Library

    Priego J I Quesada; A G Lucas-Cuevas; S Llana-Belloch; P Pérez-Soriano

    2014-01-01

    .... Literature aiming to improve the quality of life of patients suffering from cerebral pathologies, such as cerebral palsy has observed that both strength and aerobic training lead to significant...

  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....

  15. Mitrofanoff urinary diversion in a patient with cerebral palsy

    OpenAIRE

    Narwade, Sayalee S; Sawant, Ajit S.; Tamhankar, Ashwin S; Patil, Sunil R.; Patil, Kaushik R

    2016-01-01

    Cerebral palsy is the most common motor disability in childhood which result in huge socioeconomic costs. This children have a significant incidence of lower urinary tract symptoms. Clean intermittent self-catheterization is needed to avoid deterioration of renal function. But significant spasticity and resulting contractures of the adductors can interfere with the caretakers’ ability to provide perineal hygiene. Surgery in cerebral palsy affected child is challenging due to multiple associat...

  16. 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,…

  17. 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....

  18. 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.

  19. Assessment of traumatic dental injuries in patients with cerebral palsy

    OpenAIRE

    A Dubey; P A Ghafoor; M Rafeeq

    2015-01-01

    Background: Cerebral palsy is an umbrella term for a group of conditions characterized essentially by motor dysfunctions that may be associated with sensory or cognitive impairment. Such children tend to have a higher incidence of traumatic dental injuries than the general population. This increased incidence is often attributed to poor muscular co-ordination that predisposes individuals with Cerebral palsy to trauma Aim: The study was conducted to assess different dental injuries and the ris...

  20. The Effect of Hominis Placenta Herbal Acupuncture on Bell's palsy

    OpenAIRE

    Yun Jeong-hun; Yook Tea-han; Song Beom-yong

    2000-01-01

    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. I...

  1. Cardiovascular disease risk in adults with spastic bilateral cerebral palsy

    OpenAIRE

    Slot, Wilma; Roebroeck, Marij; Nieuwenhuijsen, Channah; Bergen, Michael; Stam, Henk; Burdorf, Alex; Berg-Emons, Rita

    2013-01-01

    textabstractObjective: To explore: (i) cardiovascular disease risk factors and the 10-year clustered risk of a fatal cardiovascular event in adults with spastic bilateral cerebral palsy; and (ii) relationships between the 10-year risk and body fat, aerobic fitness and physical activity. Design: Cross-sectional study. Subjects: Forty-three adults with spastic bilateral cerebral palsy without severe cognitive impairment (mean age 36.6 years (standard deviation 6); 27 men). Methods: Biological a...

  2. 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.

  3. 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.

  4. Abducens nerve palsy after schwannoma resection.

    Science.gov (United States)

    Bobbio, Antonio; Hamelin-Canny, Emelyne; Roche, Nicolas; Taillia, Herve; Alifano, Marco

    2015-02-01

    Tumors of the posterior mediastinum are mostly neurogenic and could involve the intervertebral foramen and the medullary canal. We describe the case of a patient who underwent surgery for a nerve sheet tumor originating at the level of the right second neural root. Resection was associated with an incidental dural tear and cerebrospinal fluid leak that was promptly repaired. One week after surgery, horizontal diplopia occurred. A palsy of the left abducens nerve secondary to intracranial hypotension was diagnosed. We present the pathogenic cascade leading to this ocular complication after posterior mediastinal surgery. The surgical techniques to prevent this complication are discussed. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  5. Computed tomography in spastic cerebral palsy

    Energy Technology Data Exchange (ETDEWEB)

    Pedersen, H.; Taudorf, K.; Melchior, J.C.

    1982-09-01

    Eighty-three children with spastic cerebral palsy (CP) were examined with cranial CT. In 56 cases the CT findings were abnormal. The most frequent abnormality was atrophy, present in 44 patients. The frequency of pathologic CT increased with severity of the CP. Patients with CP of postnatal aetiology more often had abnormal CT than patients with other known causes. Pathologic CT findings were seen more often in patients with seizures than in patients without. Infarctions and hemiatrophy were much more frequent in patients with hemiplegia than in patients with other types of spastic CP. A special kind of central atrophy, called isolated atrophy around the cella media, is described. This condition was seen in 20% of cases, most often in hemi- and paraplegic patients. Early infarctions in the border areas between the vascular territories of the internal carotid and the posterior cerebral artery may be the reason for this kind of atrophy.

  6. 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.

  7. Evaluation of a child with cerebral palsy.

    Science.gov (United States)

    Aneja, S

    2004-07-01

    Evaluation of a child with cerebral palsy (CP) requires a multidisciplinary approach with a team of professionals comprising of a pediatrician or pediatric neurologist, occupational therapist, a physiotherapist, child psychologist, and a social worker. The assessment is necessary to confirm the diagnosis, determine the cause, assess the motor function and associated problems. The diagnosis of CP is clinical but selected investigations may be required for ascertaining the cause. Evaluation includes assessment for common medical problems of childhood particularly nutritional disorders and assessment of family functioning. Additional disabilities are common. Routine assessment of vision and hearing is required in children with CP. Since CP is a changing disorder, some limitations may not be evident early in life but manifest in the school age or later. The evaluation of a child with CP is an ongoing process and should be a part of continuing care as the child grows from infancy to adolescence.

  8. 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...... the first 6 months after term age. We cover basic neuroscience, arguing for a beneficial effect of early intervention, and discuss why clinical research to support this convincingly is lacking. We argue that infants offered early intervention in future clinical studies must be identified carefully......, and that the intervention should be focused on infants showing early signs of CP to determine an effect of treatment. Such signs may be efficiently detected by a combination of neuroimaging and the General Movements Assessment. We propose a research agenda directed at large-scale identification of infants showing early...

  9. Spasticity in Children with Cerebral Palsy?

    DEFF Research Database (Denmark)

    Willerslev-Olsen, Maria

    ) and passive stiffness (changes in the elastic properties of muscles) of ankle joint muscles at rest, during gait and in relation to gait training in children with cerebral palsy (CP). In study I we found that reflex mediated stiffness is difficult to distinguish clinically from changes in passive muscle...... stiffness and notably that passive muscle stiffness rather than increased reflex stiffness (spasticity) is the most frequent explanation of ankle muscle hyper tonicity in children with CP. These findings emphasises the importance of properly distinguishing different contributions to muscle stiffness...... in order to avoid unnecessary antispastic treatment. Findings from study II suggested that spasticity is not the main functional limitation for movement of the ankle joint during gait in children with CP. Despite relative exaggeration of reflexes during gait, unloading of the active plantar flexors...

  10. 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.

  11. The natural history of hip development in cerebral palsy.

    Science.gov (United States)

    Terjesen, Terje

    2012-10-01

    The purpose of this study was to evaluate a population-based radiographic hip surveillance programme for children with cerebral palsy (CP) and to assess the natural history of hip displacement. The study comprised 335 children (188 males, 147 females), born during 2002 to 2006 in the 10 south-eastern counties in Norway. Their mean age at the first radiograph was 3 years (range 6mo-7y 11mo) and the mean age at the most recent follow-up was 5 years 5 months. Distribution according to CP type was spastic hemiplegia in 38%, diplegia in 27%, quadriplegia in 21%, dyskinesia in 10%, and ataxia in 3%; Gross Motor Function Classification System (GMFCS) levels I to V were, 44%, 14%, 8%, 11%, and 23% respectively. Migration percentage (MP), acetabular index, and pelvic obliquity were measured on the radiographs. Hip displacement (MP>33%) occurred in 26% of all children (subluxation in 22% and dislocation in 4%) and in 63% of those in GMFCS levels IV or V. Dislocation occurred in 14 children at a mean age of 4 years 5 months (range 1y 10mo-9y 7mo). The mean migration percentage was 20.4% at the initial radiographs and 34.0% at the last follow-up. Mean progression in migration percentage increased markedly with decreasing functional level, from 0.2% per year at GMFCS level I to 9.5% at level V. There is a pronounced trend towards hip displacement in nonambulant children. Close surveillance from age 1 to 2 years is needed to find the appropriate time for preventive surgery. Since 12% of the nonambulant children developed dislocation, our routines for hip surveillance need improvement. © The Authors. Developmental Medicine & Child Neurology © 2012 Mac Keith Press.

  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. Self-regulation of spasm and spasticity in cerebral palsy.

    Science.gov (United States)

    Neilson, P D; McCaughey, J

    1982-04-01

    Four young adult cerebral palsied subjects with a mixture of spasticity and athetosis attended an experimental reflex training program for three one-hour sessions each week over an 18 month period. During each session on-line measures of contraction level and tonic stretch reflex sensitivity from the biceps brachii muscle were shown to the subject on meter displays. Subjects were asked to attempt to control the displays. They were given goals such as: (1) reduce both contraction level and reflex sensitivity displays to zero and (2) increase the contraction level display to 10% of maximum while keeping the reflex sensitivity display at a minimum. Achievement of goals was automatically sensed and used to activate a cassette tape of the subject's favourite music. Contraction level and reflex sensitivity scores were averaged over one-minute intervals to provide a record of long term progress. Elbow-angle and IEMG data were recorded on FM tape for off-line analysis. All four subjects learned to suppress involuntary muscle activity and resting tonic stretch reflex responses. They also learned to produce a two or three-fold variation in action tonic stretch reflex sensitivity while sustaining 10% maximum voluntary contraction. In other words, subjects learned to self-regulate spasm and spasticity at the elbow and to regulate tonic stretch reflex sensitivity independently of contraction level. A visual tracking task requiring voluntary movement about the elbow was employed to assess improvement in functional control of elbow movement. One athetotic subject improved tracking accuracy as a consequence of reducing the amount of involuntary arm movement while the other three subjects showed negligible improvement in functional control.

  14. Burkitt's non-Hodgkins lymphoma presenting as facial nerve palsy in HIV-positive patients.

    Science.gov (United States)

    Woodcock, H; Nelson, M

    2011-02-01

    An isolated facial nerve palsy is rare as the presentation of a central nervous system lymphoma. In this case series, we present the clinical features of three HIV-positive patients presenting with facial nerve palsies due to HIV-associated Burkitt's lymphoma. These patients had a non-resolving facial paralysis, which occurred during a late stage of HIV. Magnetic resonance imaging (MRI) did not show leptomeningeal enhancement. Cerebrospinal fluid revealed a lymphocytosis with elevated protein and low glucose levels. The diagnosis of Burkitt's lymphoma was made on histology which showed the characteristic 'starry sky' appearance due to scattered tangible body-laden macrophages. The patients were commenced on the intensive chemotherapy regimen of CODOX-M/IVAC. Two patients died of disease progression and the third patient died of chemotherapy toxicity. This case series highlights the need for a high index of suspicion for underlying malignancy when a patient presents with a persistent facial paralysis in the later stages of HIV infection.

  15. 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.

  16. 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.

  17. [Alteration in bulbar conjunctiva microcirculation and interventional effect of Pentoxifylline after high-voltage electrical burn in rabbits].

    Science.gov (United States)

    Zhang, Qing-fu; Yan, Bao-yong; Zhou, Hui-min; Wang, Che-jiang

    2010-06-01

    To study the changes in bulbar conjunctiva microcirculation (BCM) and the therapeutic effect of Pentoxifylline on BCM disturbance after high-voltage electrical burn (HEB) in rabbits. Forty-five rabbits were divided into control group (C), electrical burn group (EB), and Pentoxifylline treatment group (PT) according to random number table, with 15 rabbits in each group. Model of HEB was reproduced in rabbits from EB and PT groups with voltage regulator and experimental transformer. Rabbits in C group were sham injured with the same devices without electrification. Changes in BCM were observed with microcirculation microscope at 15 minutes before HEB and 5 minutes, 1, 2, 4, 8 hour(s) post HEB (PHM or PHH), including: (1) morphology of microvessels, such as the discernible, diameters of arterioles, venules, and capillaries, the unevenness in caliber, and ischemic area; (2) dynamic changes in microvascular blood flow, such as blood flow speed in arterioles, venules, and capillaries, erythrocyte aggregation, and microthrombi formation; (3) condition of tissues surrounding microvessel, such as bleeding and exudation. Measurement data were processed with t test; enumeration data were processed with Fisher's exact test. (1) Morphology of microvessel: discernible of microvessels in EB and PT groups was decreased, but that of PT group was better than that of EB group. At PHM 5, diameter of arterioles, venules and capillaries was respectively (7.3+/-2.5), (12.3+/-2.4), (3.5+/-0.7) microm in EB group, all narrower than those of the control group [(14.6+/-3.1), (27.2+/-3.5), (9.0+/-1.4) microm, with t value respectively 5.23, 13.66, 14.04, P values all below 0.05]. Diameters of the microvessels in PT group [(10.2+/-3.8), (21.5+/-3.1), (7.1+/-1.2) microm] were larger than those in EB group (with t value respectively 2.21, 8.99, 10.18, P values all below 0.05). Diameters of arterioles, venules and capillaries in EB and PT groups recovered to the before HEB size at PHH 1. From PHH

  18. 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.

  19. Predicting Postoperative C5 Palsy Using Preoperative Spinal Cord Rotation.

    Science.gov (United States)

    Chugh, A Jessey; Gebhart, Jeremy J; Eubanks, Jason D

    2015-09-01

    The development of C5 nerve palsy after cervical decompression surgery has been well documented. The goal of this study was to determine whether preoperative spinal cord rotation could be used as a predictor of C5 palsy in patients who underwent posterior cervical decompression at C4-C6. The authors reviewed the records of 72 patients who had posterior decompression and 77 patients who had anterior decompression. With the patients undergoing anterior decompression used as a control group, magnetic resonance imaging scans were analyzed for area of the spinal cord, anterior-posterior diameter, and cord rotation relative to the vertebral body. The rate of C5 palsy was 7.3%. Average degrees of rotation were 3.83°±2.47° and 3.45°±2.23° in the anterior and posterior groups, respectively. A statistically significant association was detected between degree of rotation and C5 palsy. Point-biserial correlations were 0.58 (PC5 palsy in the posterior group were 0.67 (95% confidence interval, 0.24-0.94) and 0.95 (95% confidence interval, 0.86-0.98), respectively. The results suggested that preoperative spinal cord rotation may be a valid predictor of C5 nerve palsy after posterior cervical decompression. With mild rotation defined as less than 6°, moderate rotation as 6° to 10°, and severe rotation as greater than 10°, the prevalence of C5 palsy in the posterior group was 2 of 65 for mild rotation, 3 of 6 for moderate rotation, and 1 of 1 for severe rotation. Copyright 2015, SLACK Incorporated.

  20. [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

  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. 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:…

  3. Facial Dystonia with Facial Grimacing and Vertical Gaze Palsy with "Round the Houses" Sign in a 29-Year-Old Woman.

    Science.gov (United States)

    Crespi, J; Bråthen, G; Quist-Paulsen, P; Pagonabarraga, J; Roig-Arnall, C

    2016-02-01

    A 29-year-old woman developed progressive dysarthria and coordination problems from the age of 15. Examination showed dysarthria, facial dystonia, bibrachial dystonia, hyperreflexia, ataxia, and emotional incontinence. Downward supranuclear gaze palsy was prominent with a "Round the Houses" sign. Magnetic resonance imaging of the brain and medulla, electroneurography, and cerebrospinal fluid were normal. A computed tomography scan showed hepatosplenomegaly. This combination of progressive neurological symptoms together with hepatosplenomegaly was suggestive of inborn error of metabolism. A bone marrow biopsy showed an increased number of macrophages with foamy content, highly suggestive of lysosomal disease. Plasmatic chitotriosidase activity and CCL18 were increased. Genetic testing showed heterozygosis for the variation c.1070C→T (p.Ser357Leu) and c.1843→T (Arg615Cys), confirming the diagnosis of Niemann-Pick type C (NPC). The "Round the Houses" sign has only been described in patients with progressive supranuclear palsy (PSP). This sign is described as an inability to produce pure vertical saccades along the midline and instead moving the eyes in a lateral arc to accomplish the movement. The observation of this sign in a patient with NPC indicates that this bedside finding is not specific for PSP, but a sign of medial longitudinal fasciculus dysfunction. The presence of facial dystonia with facial grimacing together with supranuclear gaze palsy is highly characteristic and useful for the diagnosis of NPC. NPC is an important underdiagnosed condition, given the availability of treatment and a mean diagnostic delay of 6 years.

  4. Explosive resistance training increases rate of force development in ankle dorsiflexors and gait function in adults with cerebral palsy

    DEFF Research Database (Denmark)

    Kirk, Henrik; Geertsen, Svend Sparre; Lorentzen, Jakob

    2016-01-01

    development of ankle dorsiflexors (RFDdf), improves gait function and affects passive ankle joint stiffness in adults with CP.Thirty-five adults (age 36.5; range: 18-59 years) with CP were non-randomly assigned to a PRT or non-training control (CON) group in this explorative trial. The PRT group trained ankle......Alterations in passive elastic properties of muscles and reduced ability to quickly generate muscle force contribute to impaired gait function in adults with cerebral palsy (CP). Here, we investigated if 12 weeks of progressive and explosive resistance training (PRT) increases rate of force...... for adults with CP....

  5. SURGICAL TREATMENT OF STIFFNESS AND MOTION DISORDERS OF UPPER LIMB IN CHILDREN WITH SPASTIC CEREBRAL PALSY (REVIEW

    Directory of Open Access Journals (Sweden)

    A. A. Andreev

    2016-01-01

    Full Text Available Specific upper limb disorders accompanying spastic cerebral palsy substantially restrict self-care, writing abilities, hinder movements with external support aids and limit work capability. Spastic upper limb represents a complex multilevel abnormality progressing with age and leading to increased suffering of the patient. Little interest towards surgical correction of spastic upper limb along with insufficient awareness on surgical rehabilitation options leaves considerable number of patients without relevant treatment.The present paper describes advances and complexities of orthopaedic correction for spastic arm in ICP, key classifications used and treatment outcomes based on scientific literature analysis.

  6. 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

    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. The aims of the present study were to investigate whether the home literacy environment of children with cerebral palsy was comparable with that of peers without disabilities and to investigate to what extent speech, fine motor, and intellectual impairments limit their home literacy experiences. Questionnaires addressed to the parents of the children were devised to investigate differences in the home literacy environment in 40 children with cerebral palsy and in 62 peers without disabilities who were comparable on chronological age, i.e., 6 years old, socio-economic status (SES) and sex. The relative influence of speech intelligibility, fine motor skills, and intelligence skills for the home literacy environment factors of children with cerebral palsy was investigated. Only a few group differences were significant: children with cerebral palsy were less interested in participating in writing activities, and less involved in word-orientation activities during shared storybook reading. On the other hand, parents of children with cerebral palsy were doing more leisure activities with their child. The speech intelligibility scores of the children with cerebral palsy predicted the amount of emergent literacy activities they were doing with their parents, as wells as their active participation in word-related activities during storybook reading. In addition, the active participation of the child in story-related activities could be predicted by his or her fine motor skills. Furthermore, parents of the children in the comparison group often had high expectations, while parents of the children with cerebral palsy often did not know what expectations to

  7. 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

  8. 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.

  9. 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.

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

    Directory of Open Access Journals (Sweden)

    Ichikawa Jiro

    2012-07-01

    Full Text Available Abstract Background 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. Case presentation 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. Conclusions 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.

  11. 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.

  12. CT findings of cerebral palsy and behaviour development

    Energy Technology Data Exchange (ETDEWEB)

    Sakamoto, Zenji

    1987-06-01

    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.

  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. Magnetic resonance imaging in 122 children with spastic cerebral palsy.

    Science.gov (United States)

    Kwong, Karen L; Wong, Yiu C; Fong, Chek M; Wong, Sik N; So, Kwan T

    2004-09-01

    The interrelationship between magnetic resonance imaging findings, types of cerebral palsy, and gestation was studied. We analyzed the magnetic resonance imaging of brain in 122 children with spastic cerebral palsy. Forty-three patients had spastic hemiplegia, 61 had spastic diplegia, and 18 had spastic tetraplegia. Magnetic resonance imaging abnormalities were observed in 75% of patients. Periventricular leukomalacia accounted for 66% of abnormalities observed in patients with spastic diplegia; other types of brain lesions were uncommon. In patients with spastic tetraplegia, two types of magnetic resonance imaging abnormalities predominated: congenital brain anomalies and term-type brain injuries, 42% and 33% respectively. Types of magnetic resonance imaging abnormalities were more heterogeneous in patients with spastic hemiplegia. Preterm brain injuries (periventricular leukomalacia and posthemorrhagic porencephaly) were observed often in patients born at preterm but were also observed in patients born at term. Term-type brain injuries (term-type border-zone infarct, basal ganglia-thalamic lesion, subcortical leukomalacia, and multicystic encephalomalacia) were observed only in patients born at or near term. We conclude that magnetic resonance imaging findings for patients with spastic cerebral palsy were closely related to types of cerebral palsy and gestation at birth. Magnetic resonance imaging in patients with perinatal brain injury may reflect pathologic changes and is useful in understanding and evaluating cerebral palsy.

  15. Nutritional status of children with cerebral palsy in Turkey.

    Science.gov (United States)

    Tüzün, Emine Handan; Güven, Duygu Korkem; Eker, Levent; Elbasan, Bülent; Bülbül, Selda Fatma

    2013-03-01

    The aim of this study was to assess the nutritional status, and provide information regarding anthropometric measurements of cerebral-palsied children living in the city of Ankara, Turkey. A total of 447 children with cerebral palsy (CP) were participated in this cross-sectional study. Participants were assessed for functional motor impairment by the gross motor function classification system (GMFCS). Assesment of nutritional status was based on the triceps skinfold thickness (TSF), arm fat area (AFA) estimates derived from TSF and mid-upper arm circumference measurements. TSF and AFA Z-scores were computed using reference data. Cerebral-palsied children had lower TSF and AFA Z-scores compared to reference data from healthy children. The prevalence of underweight and overweight among boys was 8.3 and 9.5%, respectively, whereas it was 19.0 and 0.5% for girls. Underweight was more prevalent in the low functioning children than in moderate functioning children. The findings of this study indicate that cerebral-palsied children face nutritional challenges. Underweight is more prevalent than overweight among cerebral-palsied children. To optimize the outcomes of rehabilitation and prevention efforts, an understanding of the heterogeneity of nutritional status among children with CP is required.

  16. Scoliosis in Patients with Severe Cerebral Palsy: Three Different Courses in Adolescents.

    Science.gov (United States)

    Oda, Yoshiaki; Takigawa, Tomoyuki; Sugimoto, Yoshihisa; Tanaka, Masato; Akazawa, Hirofumi; Ozaki, Toshifumi

    2017-04-01

    Patients with cerebral palsy (CP) frequently present with scoliosis; however, the pattern of curve progression is difficult to predict. We aimed to clarify the natural course of the progression of scoliosis and to identify scoliosis predictors. This was a retrospective, single-center, observational study. Total of 92 CP patients from Asahikawasou Ryouiku Iryou Center in Okayama, Japan were retrospectively analyzed. Cobb angle, presence of hip dislocation and pelvic obliquity, and Gross Motor Function Classification System (GMFCS) were investigated. Severe CP was defined as GMFCS level IV or V. The mean observation period was 10.7 years. Thirtyfour severe CP patients presented with scoliosis and were divided into 3 groups based on their clinical courses: severe, moderate and mild. The mean Cobb angles at the final follow-up were 129°, 53°, and 13° in the severe, moderate, and mild groups, respectively. The average progressions from 18 to 25 years were 2.7°/year, 0.7°/year, and 0.1°/year in the severe, moderate, and mild curve groups, respectively. We observed the natural course of scoliosis and identified 3 courses based on the Cobb angle at 15 and 18 years of age. This method of classification may help clinicians predict the patients' disease progression.

  17. [Malignant lymphoma presented as recurrent multiple cranial nerve palsy after spontaneous regression of oculomotor nerve palsy: A case report].

    Science.gov (United States)

    Hirose, Takahiko; Nakajima, Hideto; Shigekiyo, Tarou; Yokote, Taiji; Ishida, Shimon; Kimura, Fumiharu

    2016-01-01

    We report the case of a 62-year-old man who presented with malignant lymphoma as recurrent multiple cranial nerve palsy after spontaneous regression of oculomotor nerve palsy. He developed ptosis and diplopia due to right oculomotor nerve palsy. Brain MRI/MRA showed no abnormality, and he recovered with conservative medical management. Three months later, he showed diplopia due to right abducens nerve palsy and facial pain and trigeminal sensory loss. Neurological examination revealed multiple cranial nerve palsy involved cranial nerve III, V, IX, and X of the right side. Serum soluble interleukin-2 receptor levels were normal, and cerebrospinal fluid examination was unremarkable. Steroid and subsequent intravenous immunoglobulin therapy didn't improve his symptoms. Six weeks after his admission, he showed rapid enlargement of the cervical lymph node and the right tonsil, and post-contrast T1-weighted MRI showed enlargement and enhancement of the left infraorbital nerve, the bilateral cavernous sinus, the bilateral facial nerves, and the left trigeminal nerve. The histopathologic examination of the tonsil biopsy revealed diffuse large B cell lymphoma. The cause of these symptoms was thought to be infiltrating the cavernous sinus, and adjacent nerves. Spontaneous regression of malignant lymphoma is an exceptional event, but this possibility should be considered so as to the correct diagnosis and proper treatment.

  18. 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.

  19. Musculoskeletal Evaluation of Children with Cerebral Palsy.

    Science.gov (United States)

    Johari, Ratna; Maheshwari, Shalin; Thomason, Pam; Khot, Abhay

    2016-11-01

    Cerebral Palsy (CP) is the most common chronic disability of childhood. The problems involved are complex; not only do these children have problems of mobility, but a plethora of associated problems [1]. A recent definition of CP includes secondary musculoskeletal problems [2]. The inclusion of this in the definition recognises the significance of musculoskeletal problems and the impact these problems have on the lives of children with CP and their families. Orthopedic management of the child with CP aims to reduce the impact of these musculoskeletal problems to help the child with CP to reach his maximum potential [3]. To accurately assess children and prepare management plans, a combination of medical history, physical examination, functional assessment, medical imaging, observational and instrumented gait analysis, and assessment of patient and family goals must be interpreted [4]. A detailed annual orthopedic assessment for all children with CP is recommended [5]. For an ambulant child, more frequent assessments are required during periods of rapid growth, observed deterioration in physical examination measures, and after interventions, including gait correction surgery. For a non-ambulant child, more frequent assessments are indicated according to hip surveillance guidelines [6, 7], during periods of observed deterioration, and following interventions such as hip or spine surgery. A systematic and practiced routine is conducive to efficiency and accuracy [5]. This paper discusses the Physical Examination Protocol used by the Hugh Williamson Gait Analysis Laboratory, in Melbourne, Australia.

  20. Isolated oculomotor nerve palsy in interventional neuroradiology

    Energy Technology Data Exchange (ETDEWEB)

    Lv Xianli; Jiang Chuhan; Li Youxiang; Yang Xinjian [Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, 6, Tiantan Xili, Chongwen, Beijing, 100050 (China); Wu Zhongxue, E-mail: ttyyzjb@sina.co [Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, 6, Tiantan Xili, Chongwen, Beijing, 100050 (China)

    2010-06-15

    Background: Isolated oculomotor nerve palsy (IOP) can be annoying for a patient and can also be the only clue to a potentially devastating and life-threatening disease. In order to understand its clinical spectrum and management better we analyzed the files of 13 patients seen at our institution over a 3-year period. Methods: Thirteen consecutive patients with IOP between January 2005 and August 2008 presented to our hospital. Magnetic resonance imaging and angiography were employed as investigations tailored to the individual patient. A vascular disorder [i.e. arteriovenous fistula, aneurysm of the posterior communicating artery (Pcoma)] was found in 7 patients (63%), most commonly a dural carotid-cavernous sinus fistula (DCCF) or a Pcoma aneurysm. Results: In 7 patients with a vascular abnormality (4 DCCFs; 3 Pcoma aneurysms), IOP was the presenting symptom. Pcoma aneurysms were coiled endovascularly and DCCFs were managed transvenously. Of the patients with more than 1 year (6 months to 2 years) of follow-up, all 7 endovascular patients recovered completely. Time to complete resolution of ONP was 6 months. Conclusion: Patients with IOP should be investigated with noninvasive techniques (MRI and MRA). If these are negative or to clarify abnormal findings of noninvasive techniques, selective angiography is needed for diagnosis and to guide treatment.

  1. Symptom burden in individuals with cerebral palsy

    Science.gov (United States)

    Hirsh, Adam T.; Gallegos, Juan C.; Gertz, Kevin J.; Engel, Joyce M.; Jensen, Mark P.

    2011-01-01

    The current study sought to (1) determine the relative frequency and severity of eight symptoms in adults with cerebral palsy (CP), (2) examine the perceived course of these eight symptoms over time, and (3) determine the associations between the severity of these symptoms and psychosocial functioning. Eighty-three adults with CP completed a measure assessing the frequency, severity, and perceived course of eight symptoms (pain, weakness, fatigue, imbalance, numbness, memory loss, vision loss, and shortness of breath). Respondents also completed measures of community integration and psychological functioning. The results indicated that pain, fatigue, imbalance, and weakness were the most common and severe symptoms reported. All symptoms were reported to have either stayed the same or worsened, rather than resolved, over time. The symptoms were more closely related to social integration than to home integration, productive activity, or psychological functioning. Memory loss was a unique predictor of social integration in the multivariate context. This study highlighted several common and problematic symptoms experienced by adults with CP. Additional research is needed to identify the most effective treatments for those symptoms that affect community integration and psychological functioning as a way to improve the quality of life of individuals with CP. PMID:21174251

  2. 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.

  3. 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......-15 years and children with symptoms of CP aged 0-5 years. MAIN VARIABLES: In the follow-up program, the children are offered examinations throughout their childhood by orthopedic surgeons, physiotherapists, occupational therapists, and pediatricians. Examinations of gross and fine motor function, manual...... 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...

  4. Correlates of degree of nerve involvement in early Bell's palsy

    Directory of Open Access Journals (Sweden)

    Hsieh Ru-Lan

    2009-06-01

    Full Text Available Abstract Background This study aimed to evaluate the still unknown factors correlating with the degree of nerve involvement in early Bell's palsy. Methods This retrospective chart review study of newly diagnosed cases of Bell's palsy was conducted over a three-year period. Information on age, sex, day of onset, comorbidities, corticosteroid use, and electroneurographic test results were collected. The electroneurographic quotient (amplitude of compound muscle action potential on the affected side divided by that on the healthy side and expressed in percent was used as an index of nerve involvement, with lower quotient indicating more severe disease. Results Data were collected on 563 patients. The mean electroneurographic quotient varied inversely with age (p vs. 40.3%; p = 0.002. There was no correlation between the degree of nerve involvement and sex, season of onset, hypertension, or diabetes. Conclusion The degree of nerve involvement in early Bell's palsy correlates positively with age and negatively with corticosteroid use.

  5. Computed tomographic (CT) scans in cerebral palsy (CP)

    Energy Technology Data Exchange (ETDEWEB)

    Kolawole, T.M.; Patel, P.J. (King Saud Univ., Riyadh (Saudi Arabia). Dept. of Radiology); Mahdi, A.H. (King Saud Univ., Riyadh (Saudi Arabia). Dept. of Paediatrics)

    1989-11-01

    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.).

  6. Biomechanical bases of rehabilitation of children with cerebral palsy

    Science.gov (United States)

    Davlet'yarova, K. V.; Korshunov, S. D.; Kapilevich, L. V.

    2015-11-01

    Biomechanical analysis and the study results of children's with cerebral palsy (CP) muscles bioelectrical activity while walking on a flat surface are represented. Increased flexion in the hip and shoulder joints and extension in the elbow joint in children with cerebral palsy were observed, with the movement of the lower limbs had less smooth character in comparison with the control group. Herewith, the oscillation amplitude was significantly increased, and the frequency in the m. gastrocnemius and m. lateralis was decreased. It was shown, that the dynamic stereotype of walking in children with cerebral palsy was characterized by excessive involvement of m. gastrocnemius and m.latissimus dorsi in locomotion. Thus, resulting biomechanical and bioelectrical parameters of walking should be considered in the rehabilitation programs development.

  7. 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......-15 years and children with symptoms of CP aged 0-5 years. MAIN VARIABLES: In the follow-up program, the children are offered examinations throughout their childhood by orthopedic surgeons, physiotherapists, occupational therapists, and pediatricians. Examinations of gross and fine motor function, manual...

  8. An Infant with Benign Isolated Abducens Palsy After Vaccination

    Directory of Open Access Journals (Sweden)

    Celebi Kocaoglu

    2014-02-01

    Full Text Available Benign isolated abducens palsy is a self-improving clinical entity characterized by esotropia and diplopia led by the deficiency of abduction, and accompanied by no other neurological findings. The entity may occur after experiencing minor fever episodes, viral infection. The pathophysiological mechanism of cellular injury remains unclear. Hypotheses involve damage arising from autoimmune mediation or direct viral invasion causing demyelination, localized arteritis or genetic predisposition, which could increase susceptibility to such nerve palsies. Diagnosed with benign isolated abducens palsy, a 19-month-old girl infant admitted to our outpatient clinic with an acute onset of esotropia in the right eye developing two weeks after the vaccination of diphtheria, acellular pertussis, tetanus, inactivated polio and Haemophilus influenzae type b (DTPa-IP-Hib was presented in this report.

  9. Pharmacologic interventions for reducing spasticity in cerebral palsy.

    Science.gov (United States)

    Patel, Dilip R; Soyode, Olufemi

    2005-10-01

    Motor function abnormalities are a key feature of cerebral palsy. Spasticity is one of the main motor abnormalities seen in children with cerebral palsy. Spasticity is a velocity dependent increased resistance to movement. While in some children, spasticity may adversely impact the motor abilities, in others, it may help maintain posture and ability to ambulate. Thus, treatment to reduce spasticity requires careful consideration of various factors. Non-pharmacologic interventions used to reduce spasticity include physiotherapy, occupational therapy, use of adaptive equipment, various orthopedic surgical procedures and neurosurgical procedures. Pharmacologic interventions used for reducing spasticity in children with cerebral palsy reviewed in this article include oral administration of baclofen, diazepam, dantrolene and tizanidine, intrathecal baclofen, and local injections of botulinum toxin, phenol, and alcohol.

  10. Esophageal eosinophilia in pediatric patients with cerebral palsy.

    Science.gov (United States)

    Nápolis, Ana Carolina Ramos de; Alves, Flavia Araujo; Rezende, Erica Rodrigues Mariano de Almeida; Segundo, Gesmar Rodrigues Silva

    2015-01-01

    To describe the clinical picture, test results, and clinical evolution of patients with cerebral palsy associated with diagnosis of eosinophilic esophagitis, monitored at tertiary centre. Cross-sectional, retrospective and descriptive study that evaluated the medical records data of pediatric patients with diagnosis of cerebral palsy and eosinophilic esophagitis in a tertiary center of pediatric gastroenterology between August 2005 and August 2013. Seven out of 131 patients with cerebral palsy had the diagnosis of eosinophilic esophagitis. The mean age at diagnosis of eosinophilic esophagitis was 52.3 months and the mean number of eosinophils in esophagus was 35 per high-power field. Symptoms more frequent were recurrent vomiting and disphagia. Endoscopic alterations found were mucosal thickening, vertical lines, mucosal opacificacion and white plaques. The frequency of eosinophilic esophagitis found was higher than in general pediatric population. The investigation of eosinophilic esophagitis should be done regularly in those patients, once this entity could overlap other gastrointestinal diseases.

  11. 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...

  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. 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.

  14. Progress Report

    DEFF Research Database (Denmark)

    Duer, Karsten

    1999-01-01

    Progress report describing the work carried out by the Danish participant in the ALTSET project in the period January 1999 to July 1999.......Progress report describing the work carried out by the Danish participant in the ALTSET project in the period January 1999 to July 1999....

  15. Three Cases of Radial Nerve Palsy with Bee Venom Therapy

    Directory of Open Access Journals (Sweden)

    Kim Hyo-Soo

    2004-06-01

    Full Text Available Objectives : The purpose of this study is to report the efficiency of Bee Venom Therapy by managering of radial nerve palsy patients. Methods : Three patients were treatmented by Bee Venom therapy and acupucture therapy. And We took pictures of someone's wrist and checked the power of muscles by the improving phase. Results : Almost cases shows the improvement in the movement of wrist and the numbness of hand. By using acuputure and Bee Venom therapy, the symptoms of radial nerv palsy was more fastly recovered.

  16. Literature review : Review of Clinical Anatomy and Management 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...

  17. 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.

  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. Infantile-onset ascending hereditary spastic paraplegia with bulbar involvement due to the novel ALS2 mutation c.2761C>T.

    Science.gov (United States)

    Wakil, Salma M; Ramzan, Khushnooda; Abuthuraya, Rula; Hagos, Samya; Al-Dossari, Haya; Al-Omar, Rana; Murad, Hatem; Chedrawi, Aziza; Al-Hassnan, Zuhair N; Finsterer, Josef; Bohlega, Saeed

    2014-02-15

    Recessive mutations in the alsin gene cause three clinically distinct motor neuron diseases: juvenile amyotrophic lateral sclerosis (ALS2), juvenile primary lateral sclerosis (JPLS) and infantile-onset ascending hereditary spastic paraplegia (IAHSP). A total of 23 different ALS2 mutations have been described for the three disorders so far. Most of these mutations result in a frameshift leading to a premature truncation of the alsin protein. We report the novel ALS2 truncating mutation c.2761C>T; p.R921X detected by homozygosity mapping and sequencing in two infants affected by IAHSP with bulbar involvement. The mutation c.2761C>T resides in the pleckstrin domain, a characteristic segment of guanine nucleotide exchange factors of the Rho GTPase family, which is involved in the overall neuronal development or maintenance. This study highlights the importance of using homozygosity mapping combined with candidate gene analysis to identify the underlying genetic defect as in this Saudi consanguineous family. Copyright © 2013 Elsevier B.V. All rights reserved.

  20. Ptosis as partial oculomotor nerve palsy due to compression by infundibular dilatation of posterior communicating artery, visualized with three-dimensional computer graphics: case report.

    Science.gov (United States)

    Fukushima, Yuta; Imai, Hideaki; Yoshino, Masanori; Kin, Taichi; Takasago, Megumi; Saito, Kuniaki; Nakatomi, Hirofumi; Saito, Nobuhito

    2014-01-01

    Oculomotor nerve palsy (ONP) due to internal carotid-posterior communicating artery (PcomA) aneurysm generally manifests as partial nerve palsy including pupillary dysfunction. In contrast, infundibular dilatation (ID) of the PcomA has no pathogenic significance, and mechanical compression of the cranial nerve is extremely rare. We describe a 60-year-old woman who presented with progressive ptosis due to mechanical compression of the oculomotor nerve by an ID of the PcomA. Three-dimensional computer graphics (3DCG) accurately visualized the mechanical compression by the ID, and her ptosis was improved after clipping of the ID. ID of the PcomA may cause ONP by mechanical compression and is treatable surgically. 3DCG are effective for the diagnosis and preoperative simulation.