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Sample records for bulbar palsy progressive

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

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    Bianca Helena Brum Batista

    2002-09-01

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

  2. Creutzfeldt-Jakob disease presenting as bulbar palsy.

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    Mittal, Manoj; Hammond, Nancy; Husmann, Kathrin; Lele, Abhijit; Pasnoor, Mamatha

    2010-11-01

    Creutzfeldt-Jakob disease (CJD) is a degenerative neurological disorder caused by a prion protein. The diagnosis may be challenging in unusual early presentations. A bulbar symptom as the initial complaint is a rare presentation for CJD, with only a few reports so far. These patients can be misdiagnosed with motor neuron disease or the Miller Fisher variant of Guillain-Barré syndrome. We describe a 69-year-old woman with CJD who presented with bulbar symptoms at the onset.

  3. Progressive Supranuclear Palsy

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    Progressive supranuclear palsy (PSP) is a rare brain disease. It affects brain cells that control the movement of your eyes. This leads to ... speech, vision and swallowing problems. Doctors sometimes confuse PSP with Parkinson's disease or Alzheimer's disease. PSP has ...

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

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    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.

  5. 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.

  6. Zolpidem in Progressive Supranuclear Palsy

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    Sandip K. Dash

    2013-01-01

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

  7. Progressive supranuclear palsy: new concepts

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    Orlando Graziani Povoas Barsottini

    2010-12-01

    Full Text Available Progressive supranuclear palsy (PSP is a distinctive form of neurodegenerative disease which affects the brainstem and basal ganglia. Patients present supranuclear ophthalmoplegia, postural instability and mild dementia. PSP is defined neuropathologically by the accumulation of neurofibrillary tangles in the subthalamic nucleus, pallidum, red nucleus, substantia nigra, striatum, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. Over the last decade many lines of investigations have helped refine PSP in many aspects and it is the purpose of this review to help neurologists identify PSP, to better understand its pathophysiology and to provide a more focused, symptom-based treatment approach.

  8. Genetics of Progressive Supranuclear Palsy

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    Sun Young Im

    2015-09-01

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

  9. PHYSIOTHERAPY MANAGEMENT FOR PROGRESSIVE SUPRA NUCLEAR PALSY

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    P. Keerthi Chandra Shekhar.; A. Anitha kumari,; Chennupati. Ashok Chakravarthi

    2013-01-01

    Background:An elderly patient with disturbances in gait, impaired balance, difficulty moving the eyes andhistory of frequent falls are not commonly seen in physiotherapy referral cases. Progressive supranuclear palsy(PSP) is relatively uncommon and is the most frequently occurring form of Atypical Parkinsonism withcardinalfeatures of vertical gaze palsy, gait instability with frequent falls. However, because the initialclinical featuresoften resemble Parkinson’s disease (PD) many patients are...

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

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    Neeraja J. Boddu

    2013-01-01

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

  11. PHYSIOTHERAPY MANAGEMENT FOR PROGRESSIVE SUPRA NUCLEAR PALSY

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    P. Keerthi Chandra Shekhar.

    2013-06-01

    Full Text Available Background:An elderly patient with disturbances in gait, impaired balance, difficulty moving the eyes andhistory of frequent falls are not commonly seen in physiotherapy referral cases. Progressive supranuclear palsy(PSP is relatively uncommon and is the most frequently occurring form of Atypical Parkinsonism withcardinalfeatures of vertical gaze palsy, gait instability with frequent falls. However, because the initialclinical featuresoften resemble Parkinson’s disease (PD many patients are referred for rehabilitation services withthe wrongdiagnosis as PD. The progression of the symptoms in PSP is much faster than in PD and there is no cure oreffective medication to manage PSP. We describe a case of 59 years old male, patient who was referred tophysiotherapy department for asymmetric limb apraxia, markedly impaired balance and frequent falls duringtransitional movements. Two years before the patient was diagnosis as PD and later the patient was re-diag-nosed as PSP based on the progression of the disease. The patient was rehabilitated using coordination exer-cises and reciprocal rhythmic movements to reduce rigidity, transfer training exercises for balance,gait trainingusing weights strapped to ankles in parallel bar and visual tracking exercises. The exercises wereprogrammedfor 1 ½ hours a day, 5 days a week, for 8 weeks. After 15 weeks there was improvement in gait and balance ofthe patient with decrease in fall incidence on a Progressive Supranuclear Palsy Rating Scale (PSPRS.

  12. Hummingbird sign in progressive supranuclear palsy disease

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    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.

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

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    ... palsy and progressive scoliosis with mutations in ROBO3. Neurology. 2005 Apr 12;64(7):1196-203. Citation ... with progressive scoliosis maps to chromosome 11q23-25. Neurology. 2002 Aug 13;59(3):432-5. Citation ...

  14. Regional Cerebral Perfusion in Progressive Supranuclear Palsy

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

    1996-03-15

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

  15. Regional Cerebral Perfusion in Progressive Supranuclear Palsy

    International Nuclear Information System (INIS)

    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 (p0.05). 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

  16. A case of atypical progressive supranuclear palsy

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    Spaccavento S

    2013-12-01

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

  17. Hypokinesia without decrement distinguishes progressive supranuclear palsy from Parkinson's disease

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    Ling, Helen; Massey, Luke A.; Lees, Andrew J.; Brown, Peter; Day, Brian L.

    2012-01-01

    Repetitive finger tapping is commonly used to assess bradykinesia in Parkinson's disease. The Queen Square Brain Bank diagnostic criterion of Parkinson's disease defines bradykinesia as ‘slowness of initiation with progressive reduction in speed and amplitude of repetitive action’. Although progressive supranuclear palsy is considered an atypical parkinsonian syndrome, it is not known whether patients with progressive supranuclear palsy have criteria-defined bradykinesia. This study objective...

  18. Hypokinesia without decrement distinguishes progressive supranuclear palsy from Parkinson's disease.

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    Ling, Helen; Massey, Luke A; Lees, Andrew J; Brown, Peter; Day, Brian L

    2012-04-01

    Repetitive finger tapping is commonly used to assess bradykinesia in Parkinson's disease. The Queen Square Brain Bank diagnostic criterion of Parkinson's disease defines bradykinesia as 'slowness of initiation with progressive reduction in speed and amplitude of repetitive action'. Although progressive supranuclear palsy is considered an atypical parkinsonian syndrome, it is not known whether patients with progressive supranuclear palsy have criteria-defined bradykinesia. This study objectively assessed repetitive finger tap performance and handwriting in patients with Parkinson's disease (n = 15), progressive supranuclear palsy (n = 9) and healthy age- and gender-matched controls (n = 16). The motion of the hand and digits was recorded in 3D during 15-s repetitive index finger-to-thumb tapping trials. The main finding was hypokinesia without decrement in patients with progressive supranuclear palsy, which differed from the finger tap pattern in Parkinson's disease. Average finger separation amplitude in progressive supranuclear palsy was less than half of that in controls and Parkinson's disease (P Parkinson's disease OFF levodopa (-0.20°/cycle, P = 0.002). 'Hypokinesia', defined as Parkinson's disease OFF levodopa group. In progressive supranuclear palsy, the mean amplitude was not correlated with disease duration or other clinimetric scores. In Parkinson's disease, finger tap pattern was compatible with criteria-defined bradykinesia, characterized by slowness with progressive reduction in amplitude and speed and increased variability in speed throughout the tap trial. In Parkinson's disease, smaller amplitude, slower speed and greater speed variability were all associated with a more severe Unified Parkinson's Disease Rating Scale motor score. Analyses of handwriting showed that micrographia, defined as smaller than 50% of the control group's mean script size, was present in 75% of patients with progressive supranuclear palsy and 15% of

  19. Behavioral abnormalities in progressive supranuclear palsy.

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    Gerstenecker, Adam; Duff, Kevin; Mast, Benjamin; Litvan, Irene

    2013-12-30

    Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder in which, classically, patients present with postural instability and falls, parkinsonism, and slowing of vertical saccades. PSP patients typically have deficits in cognitive functioning, difficulties with most daily activities, and present with notable behavioral disturbances-particularly apathy, impulsivity, and irritability. Using data from 154 patients meeting criteria for clinically probable PSP, domain and total scores of the Neuropsychiatric Inventory were examined and compared to demographics, disease severity, cognition, and motor features. Behavioral abnormalities were common in this cohort of PSP patients, with more than half experiencing apathy, depression, and sleeping problems, and approximately one third displaying agitation, irritability, disinhibition, and eating problems. Few clinical correlates of neuropsychiatric symptoms were observed in this cohort. Given the prevalence of neuropsychiatric symptoms in PSP, these patients are expected to be frequently seen by psychiatrists and other mental health professionals for symptom management and increased quality of life. Clinical trials are clearly needed to address the neuropsychiatric morbidity in these patients.

  20. A Review of Treatment Options for Progressive Supranuclear Palsy.

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    Stamelou, Maria; Höglinger, Günter

    2016-07-01

    Progressive supranuclear palsy (PSP) is an atypical parkinsonian condition characterized by a symmetric akinetic-rigid syndrome, early falls, supranuclear gaze palsy, and a frontotemporal behavioral syndrome. The typical phenotype is termed Richardson's syndrome, but numerous other phenotypes have been described. The pathophysiology of PSP is not fully understood, but dysfunction of the tau protein seems to play a central role. Despite exciting new knowledge on the pathophysiology of PSP, there is still no highly effective symptomatic or disease-modifying treatment. We review the evidence on pharmacotherapy and experimental therapies in PSP and provide levels of recommendation for the off-label use of commonly used drugs in this disorder. PMID:27222018

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

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

    2009-02-15

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

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

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    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

  3. CoQ10 in progressive supranuclear palsy

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    Scala, Stephanie A.; Hamill, Robert W.; Simon, David K.; Pathak, Subash; Ruthazer, Robin; Standaert, David G.; Yacoubian, Talene A.

    2016-01-01

    Objective: An investigator-initiated, multicenter, randomized, placebo-controlled, double-blind clinical trial to determine whether coenzyme Q10 (CoQ10) is safe, well tolerated, and effective in slowing functional decline in progressive supranuclear palsy (PSP). Methods: Sixty-one participants received CoQ10 (2,400 mg/d) or placebo for up to 12 months. Progressive Supranuclear Palsy Rating Scale (PSPRS), Unified Parkinson's Disease Rating Scale, activities of daily living, Mini-Mental State Examination, the 39-item Parkinson's Disease Questionnaire, and 36-item Short Form Health Survey were monitored at baseline and months 3, 6, 9, and 12. The safety profile of CoQ10 was determined by adverse events, vital signs, and clinical laboratory values. Primary outcome measures were changes in PSPRS and Unified Parkinson's Disease Rating Scale scores from baseline to month 12. Results: CoQ10 was well tolerated. No statistically significant differences were noted between CoQ10 and placebo groups in primary or secondary outcome measures. A nonsignificant difference toward slower clinical decline in the CoQ10 group was observed in total PSPRS among those participants who completed the trial. Before the final study visit at 12 months, 41% of participants withdrew because of travel distance, lack of perceived benefit, comorbidities, or caregiver issues. Conclusions: High doses of CoQ10 did not significantly improve PSP symptoms or disease progression. The high withdrawal rate emphasizes the difficulty of conducting clinical trials in patients with PSP. ClinicalTrials.gov identifier: NCT00382824. Classification of evidence: This study provides Class II evidence that CoQ10 does not significantly slow functional decline in PSP. The study lacks the precision to exclude a moderate benefit of CoQ10.

  4. Progressive Bilateral Facial Palsy as a Manifestation of Granulomatosis With Polyangiitis: A Case Report.

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    Jeong, Sang Mee; Park, Joo Hyun; Lee, Jong In; Nam, Kyung Eun; Lee, Jung Soo; Kim, Joo Hee

    2016-08-01

    Bilateral facial palsy, which is usually combined with other diseases, occurs infrequently. It may imply a life-threatening condition. Therefore, the differential diagnosis of bilateral facial palsy is important. However, the etiology is variable, which makes diagnosis challenging. We report a rare case of progressive bilateral facial palsy as a manifestation of granulomatosis with polyangiitis (GPA). A 40-year-old male with otitis media and right facial palsy was referred for electroneurography (ENoG), which showed a 7.7% ENoG. Left facial palsy occurred after 2 weeks, and multiple cavitary opacities were noted on chest images. GPA was diagnosed by lung biopsy. His symptoms deteriorated and mononeuropathy multiplex developed. The possibility of systemic disease, such as GPA, should be considered in patients presenting with bilateral facial palsy, the differential diagnosis of which is summarized in this report. PMID:27606281

  5. Apparent diffusion coefficient measurements in progressive supranuclear palsy

    International Nuclear Information System (INIS)

    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.)

  6. Apparent diffusion coefficient measurements in progressive supranuclear palsy

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

    2000-09-01

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

  7. Clinical features of progressive supranuclear palsy in 105 Chinese patients

    Institute of Scientific and Technical Information of China (English)

    Jing Hou; Ruibiao Guo; Tong Chen; Xiaohong Zhang; Weiping Wu; Zhenfu Wang

    2011-01-01

    OBJECTIVE: To thoroughly investigate clinical characteristics of progressive supranuclear palsy (PSP) in a Chinese population.METHODS: Computer-based online searches through China National Knowledge Infrastructure and Weipu Periodical Database were performed to collect case reports of PSP published between 1980 and 2009. Clinical characteristics were analyzed.RESULTS: A total of 58 studies comprising 105 patients (76 males and 29 females) were included. All cases were sporadic and free of family history. The mean age at onset was 60.6 ± 9.1 years, and the mean course from onset of symptoms to diagnosis was 3.4 ± 2.4 years. The male-to-female ratio was approximately 3: 1. Onset was characterized by akinetic-rigid features and accounted for 34.3% of all cases, followed by early postural instability (25.5%), pseudobulbar palsy (9.8%), cognitive impairment (9.8%), and vertical supranuclear ophthalmoplegia (7.8%). With disease progression, vertical supranuclear ophthalmoplegia was reported in 95.1% of cases, followed by akinetic-rigid features (83.3%), pseudobulbar palsy (82.4%), axial dystonia (75.5%), cognitive impairment (72.5%), and early postural instability (69.6%). A total of 70.5% of patients exhibited abnormal electroencephalograms, and 21.4% exhibited mild abnormalities in cerebrospinal fluid. Brain CT scanning results of 37 patients showed 37.8% with midbrain and concurrent cerebral hemisphere atrophy, and 5.4% and 24.3% with midbrain and cerebral hemisphere atrophy, respectively. Brain MRI scanning results of 55 patients revealed a total of 16.4% patients with midbrain atrophy, 23.6% with midbrain and concurrent cerebral hemisphere atrophy, 32.7% with cerebral hemisphere atrophy, and 11% with brainstem atrophy. The percentage of midbrain atrophy revealed by MRI was greater than by CT. All 11 patients subjected to Mini-Mental State Examination scored < 23. A total of 10 patients underwent brain electrophysiological examination, and 80% presented with

  8. Basal ganglia cholinergic and dopaminergic function in progressive supranuclear palsy.

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    Warren, Naomi M; Piggott, Margaret A; Greally, Elizabeth; Lake, Michelle; Lees, Andrew J; Burn, David J

    2007-08-15

    Progressive Supranuclear Palsy (PSP) is a progressive neurodegenerative disorder. In contrast to Parkinson's disease (PD) and dementia with Lewy bodies (DLB), replacement therapy with dopaminergic and cholinergic agents in PSP has been disappointing. The neurochemical basis for this is unclear. Our objective was to measure dopaminergic and cholinergic receptors in the basal ganglia of PSP and control brains. We measured, autoradiographically, dopaminergic (dopamine transporter, 125I PE2I and dopamine D2 receptors, 125I epidepride) and cholinergic (nicotinic alpha4beta2 receptors, 125I 5IA85380 and muscarinic M1 receptors, 3H pirenzepine) parameters in the striatum and pallidum of pathologically confirmed PSP cases (n=15) and controls (n=32). In PSP, there was a marked loss of dopamine transporter and nicotinic alpha4beta2 binding in the striatum and pallidum, consistent with loss of nigrostriatal neurones. Striatal D2 receptors were increased in the caudate and muscarinic M1 receptors were unchanged compared with controls. These results do not account for the poor response to dopaminergic and cholinergic replacement therapies in PSP, and suggest relative preservation of postsynaptic striatal projection neurones bearing D2/M1 receptors. PMID:17534953

  9. 针刺配合吞咽训练对假性延髓麻痹患者血浆TXB2、6-keto-PGF1α的影响%Clinical Study on Effects of Acupuncture with Swallowing Training on Changes of TXB2、6-Keto-PGF1α on Patients With Pseudo BuLbar Palsy

    Institute of Scientific and Technical Information of China (English)

    刘聪; 梅建强; 崔莉; 赵云玲

    2012-01-01

    Objective- To investigate the therapeutic effect and mechanism of acupuncture with swallowing training for treating pseudo bulbar palsy. Methods: Two hundred patients with pseudo bulbar palsy were divided into two groups at random. The 100 patients in control group were treated with swallowing training, while the 100 patients in observation group were treated with both acupuncture and swallowing training. In the mean time the two groups were all treated with conventional drug therapy. The changes of the swallowing function classification and TXB2,6-keto-PGF1 a were observed before and after treatment. Result: The improvement of swallowing function in observation group was much better than the control group. There was remarkable difference on the decrease of TXB2, the increase of 6-keto- PGF, a in the observation group. The difference is very significant. Conclusion: The therapeutic effect of acupuncture with swallowing training treatment for pseudo bulbar palsy was positive. It could improve swallowing function obviously, regulate the balance of TXB2,6-keto-PGFla , and inhibit activition of platelet.%目的:为了探讨针刺配合吞咽训练治疗假性延髓麻痹的临床疗效及作用机制.方法:将200例假性延髓麻痹患者随机分为两组,对照组100例采用吞咽训练治疗,观察组100例采用针刺配合吞咽训练治疗,两组均同时应用常规药物.比较治疗前后两组病人吞咽功能的分级,以及血浆TXB2、6-keto-PGF1α的变化.结果:观察组吞咽功能提高优于对照组;观察组的血浆TXB2显著降低、6-keto-PGF1α显著升高,与对照组比较有统计学意义.结论:针刺配合吞咽训练治疗假性延髓麻痹患者疗效肯定,可明显改善吞咽功能,可调节TXB2、6-keto-PGF1α的平衡,抑制血小板活化.

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

    International Nuclear Information System (INIS)

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

  11. 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.

  12. 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.

  13. 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.

  14. 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…

  15. 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.

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

    International Nuclear Information System (INIS)

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

  17. Tideglusib reduces progression of brain atrophy in progressive supranuclear palsy in a randomized trial.

    Science.gov (United States)

    Höglinger, Günter U; Huppertz, Hans-Jürgen; Wagenpfeil, Stefan; Andrés, María V; Belloch, Vincente; León, Teresa; Del Ser, Teodoro

    2014-04-01

    It is believed that glycogen synthase kinase-3 hyperphosphorylates tau protein in progressive supranuclear palsy (PSP). The Tau Restoration on PSP (TAUROS) trial assessed the glycogen synthase kinase-3 inhibitor tideglusib as potential treatment. For the magnetic resonance imaging (MRI) substudy reported here, we assessed the progression of brain atrophy. TAUROS was a multinational, phase 2, double-blind, placebo-controlled trial in patients with mild-to-moderate PSP who were treated with oral tideglusib (600 mg or 800 mg daily) or with placebo for 1 year. A subset of patients underwent baseline and 52-week MRI. Automated, observer-independent, atlas-based, and mask-based volumetry was done on high-resolution, T1-weighted, three-dimensional data. For primary outcomes, progression of atrophy was compared both globally (brain, cerebrum) and regionally (third ventricle, midbrain, pons) between the active and placebo groups (Bonferroni correction). For secondary outcomes, 15 additional brain structures were explored (Benjamini & Yekutieli correction). In total, MRIs from 37 patient were studied (placebo group, N = 9; tideglusib 600 mg group, N = 19; tideglusib 800 mg group, N = 9). The groups compared well in their demographic characteristics. Clinical results showed no effect of tideglusib over placebo. Progression of atrophy was significantly lower in the active group than in the placebo group for the brain (mean ± standard error of the mean: -1.3% ± 1.4% vs. -3.1% ± 2.3%, respectively), cerebrum (-1.3% ± 1.5% vs. -3.2% ± 2.1%, respectively), parietal lobe (-1.6% ± 1.9% vs. -4.1% ± 3.0%, respectively), and occipital lobe (-0.3% ± 1.8% vs. -2.7% ± 3.2%, respectively). A trend toward reduced atrophy also was observed in the frontal lobe, hippocampus, caudate nucleus, midbrain, and brainstem. In patients with PSP, tideglusib reduced the progression of atrophy in the whole brain, particularly in the parietal and occipital lobes.

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

    International Nuclear Information System (INIS)

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

  19. 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.

  20. Cerebral palsy.

    Science.gov (United States)

    Graham, H Kerr; Rosenbaum, Peter; Paneth, Nigel; Dan, Bernard; Lin, Jean-Pierre; Damiano, Diane L; Becher, Jules G; Gaebler-Spira, Deborah; Colver, Allan; Reddihough, Dinah S; Crompton, Kylie E; Lieber, Richard L

    2016-01-01

    Cerebral palsy is the most common cause of childhood-onset, lifelong physical disability in most countries, affecting about 1 in 500 neonates with an estimated prevalence of 17 million people worldwide. Cerebral palsy is not a disease entity in the traditional sense but a clinical description of children who share features of a non-progressive brain injury or lesion acquired during the antenatal, perinatal or early postnatal period. The clinical manifestations of cerebral palsy vary greatly in the type of movement disorder, the degree of functional ability and limitation and the affected parts of the body. There is currently no cure, but progress is being made in both the prevention and the amelioration of the brain injury. For example, administration of magnesium sulfate during premature labour and cooling of high-risk infants can reduce the rate and severity of cerebral palsy. Although the disorder affects individuals throughout their lifetime, most cerebral palsy research efforts and management strategies currently focus on the needs of children. Clinical management of children with cerebral palsy is directed towards maximizing function and participation in activities and minimizing the effects of the factors that can make the condition worse, such as epilepsy, feeding challenges, hip dislocation and scoliosis. These management strategies include enhancing neurological function during early development; managing medical co-morbidities, weakness and hypertonia; using rehabilitation technologies to enhance motor function; and preventing secondary musculoskeletal problems. Meeting the needs of people with cerebral palsy in resource-poor settings is particularly challenging. PMID:27188686

  1. 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.

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

    International Nuclear Information System (INIS)

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

  3. 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.

    2010-01-01

    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 audio-bio

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

    Otsuka, Makoto; Ichiya, Yuici; Kuwabara, Yasuo (Kyushu Univ., Fukuoka (Japan). Faculty of Medicine) (and others)

    1989-11-01

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

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

    International Nuclear Information System (INIS)

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

  8. Progression of Hip Displacement during Radiographic Surveillance in Patients with Cerebral Palsy.

    Science.gov (United States)

    Park, Jae Young; Choi, Young; Cho, Byung Chae; Moon, Sang Young; Chung, Chin Youb; Lee, Kyoung Min; Sung, Ki Hyuk; Kwon, Soon-Sun; Park, Moon Seok

    2016-07-01

    Progression of hip displacement is common in patients with cerebral palsy (CP). We aimed to investigate the rate of progression of hip displacement in patients with CP by assessing changes in radiographic indices according to Gross Motor Function Classification System (GMFCS) level during hip surveillance. We analyzed the medical records of patients with CP aged GMFCS level, annual changes in radiographic indices were analyzed and adjusted for affecting factors, such as sex, laterality, and type of CP. A total of 197 patients were included in this study, and 1,097 radiographs were evaluated. GMFCS classifications were as follows: 100 patients were level I-III, 48 were level IV, and 49 were level V. MP increased significantly over the duration of hip surveillance in patients with GMFCS levels I-III, IV, and V by 0.3%/year (P GMFCS level IV, NSA increased significantly by 3.4°/year (P GMFCS level IV or V. Furthermore, physicians can predict and inform parents or caregivers regarding the progression of hip displacement in patients with CP.

  9. PROSPERA: a randomized, controlled trial evaluating rasagiline in progressive supranuclear palsy.

    Science.gov (United States)

    Nuebling, Georg; Hensler, Mira; Paul, Sabine; Zwergal, Andreas; Crispin, Alexander; Lorenzl, Stefan

    2016-08-01

    To date, pharmacological treatment options for progressive supranuclear palsy (PSP), a neurodegenerative tauopathy, are limited. The MAO-B inhibitor rasagiline has shown neuroprotective effects in preclinical models of neurodegeneration. To evaluate the safety, tolerability and therapeutic effect of rasagiline on symptom progression in PSP. In this 1-year randomized, double-blind, placebo-controlled trial, 44 patients fulfilling the NINDS-PSP criteria were randomized to 1 mg/d rasagiline or placebo. The combined primary endpoint included symptom progression as measured by the PSP rating scale (PSP-RS) and the requirement of L-dopa rescue medication. Secondary endpoints included Schwab and England Activities of Daily Living (SEADL), Montgomery-Åsberg Depression Rating Scale, Mini Mental State Examination, Frontal Assessment Battery and posturographic measurements. Of the 44 patients randomized, 26 completed the trial per protocol. Rasagiline was well tolerated, with a slight increase of known side effects (hallucinations, ventricular extrasystoles). No effect on the primary endpoint (p = 0.496) was detected. Symptom progression averaged at 11.2 (rasagiline) and 10.8 (placebo) points per year (ΔPSP-RS). No difference was seen in SEADL, depression, cognitive function, frontal executive function and posturographic measurements. Post hoc analyses of PSP-RS subdomains indicate a potential beneficial effect in the "limb motor" subdomain, whereas performance appeared lower in the "mentation" and "history" subdomains in the treatment group. While rasagiline is well tolerated in PSP, a beneficial effect on overall symptom progression was not detected. Post hoc analyses suggest the implementation of more specific endpoints in future studies. PMID:27230855

  10. 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.

  11. Progression of Hip Displacement during Radiographic Surveillance in Patients with Cerebral Palsy

    Science.gov (United States)

    2016-01-01

    Progression of hip displacement is common in patients with cerebral palsy (CP). We aimed to investigate the rate of progression of hip displacement in patients with CP by assessing changes in radiographic indices according to Gross Motor Function Classification System (GMFCS) level during hip surveillance. We analyzed the medical records of patients with CP aged < 20 years who underwent at least 6 months interval of serial hip radiographs before any surgical hip intervention, including reconstructive surgery. After panel consensus and reliability testing, radiographic measurements of migration percentage (MP), neck-shaft angle (NSA), acetabular index (AI), and pelvic obliquity (PO) were obtained during hip surveillance. For each GMFCS level, annual changes in radiographic indices were analyzed and adjusted for affecting factors, such as sex, laterality, and type of CP. A total of 197 patients were included in this study, and 1,097 radiographs were evaluated. GMFCS classifications were as follows: 100 patients were level I-III, 48 were level IV, and 49 were level V. MP increased significantly over the duration of hip surveillance in patients with GMFCS levels I-III, IV, and V by 0.3%/year (P < 0.001), 1.9%/year (P < 0.001), and 6.2%/year (P < 0.001), respectively. In patients with GMFCS level IV, NSA increased significantly by 3.4°/year (P < 0.001). Our results suggest that periodic monitoring and radiographic hip surveillance is warranted for patients with CP, especially those with GMFCS level IV or V. Furthermore, physicians can predict and inform parents or caregivers regarding the progression of hip displacement in patients with CP. PMID:27366015

  12. Progression of Hip Displacement during Radiographic Surveillance in Patients with Cerebral Palsy.

    Science.gov (United States)

    Park, Jae Young; Choi, Young; Cho, Byung Chae; Moon, Sang Young; Chung, Chin Youb; Lee, Kyoung Min; Sung, Ki Hyuk; Kwon, Soon-Sun; Park, Moon Seok

    2016-07-01

    Progression of hip displacement is common in patients with cerebral palsy (CP). We aimed to investigate the rate of progression of hip displacement in patients with CP by assessing changes in radiographic indices according to Gross Motor Function Classification System (GMFCS) level during hip surveillance. We analyzed the medical records of patients with CP aged < 20 years who underwent at least 6 months interval of serial hip radiographs before any surgical hip intervention, including reconstructive surgery. After panel consensus and reliability testing, radiographic measurements of migration percentage (MP), neck-shaft angle (NSA), acetabular index (AI), and pelvic obliquity (PO) were obtained during hip surveillance. For each GMFCS level, annual changes in radiographic indices were analyzed and adjusted for affecting factors, such as sex, laterality, and type of CP. A total of 197 patients were included in this study, and 1,097 radiographs were evaluated. GMFCS classifications were as follows: 100 patients were level I-III, 48 were level IV, and 49 were level V. MP increased significantly over the duration of hip surveillance in patients with GMFCS levels I-III, IV, and V by 0.3%/year (P < 0.001), 1.9%/year (P < 0.001), and 6.2%/year (P < 0.001), respectively. In patients with GMFCS level IV, NSA increased significantly by 3.4°/year (P < 0.001). Our results suggest that periodic monitoring and radiographic hip surveillance is warranted for patients with CP, especially those with GMFCS level IV or V. Furthermore, physicians can predict and inform parents or caregivers regarding the progression of hip displacement in patients with CP. PMID:27366015

  13. Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy.

    Science.gov (United States)

    Kouri, Naomi; Ross, Owen A; Dombroski, Beth; Younkin, Curtis S; Serie, Daniel J; Soto-Ortolaza, Alexandra; Baker, Matthew; Finch, Ni Cole A; Yoon, Hyejin; Kim, Jungsu; Fujioka, Shinsuke; McLean, Catriona A; Ghetti, Bernardino; Spina, Salvatore; Cantwell, Laura B; Farlow, Martin R; Grafman, Jordan; Huey, Edward D; Ryung Han, Mi; Beecher, Sherry; Geller, Evan T; Kretzschmar, Hans A; Roeber, Sigrun; Gearing, Marla; Juncos, Jorge L; Vonsattel, Jean Paul G; Van Deerlin, Vivianna M; Grossman, Murray; Hurtig, Howard I; Gross, Rachel G; Arnold, Steven E; Trojanowski, John Q; Lee, Virginia M; Wenning, Gregor K; White, Charles L; Höglinger, Günter U; Müller, Ulrich; Devlin, Bernie; Golbe, Lawrence I; Crook, Julia; Parisi, Joseph E; Boeve, Bradley F; Josephs, Keith A; Wszolek, Zbigniew K; Uitti, Ryan J; Graff-Radford, Neill R; Litvan, Irene; Younkin, Steven G; Wang, Li-San; Ertekin-Taner, Nilüfer; Rademakers, Rosa; Hakonarsen, Hakon; Schellenberg, Gerard D; Dickson, Dennis W

    2015-01-01

    Corticobasal degeneration (CBD) is a neurodegenerative disorder affecting movement and cognition, definitively diagnosed only at autopsy. Here, we conduct a genome-wide association study (GWAS) in CBD cases (n=152) and 3,311 controls, and 67 CBD cases and 439 controls in a replication stage. Associations with meta-analysis were 17q21 at MAPT (P=1.42 × 10(-12)), 8p12 at lnc-KIF13B-1, a long non-coding RNA (rs643472; P=3.41 × 10(-8)), and 2p22 at SOS1 (rs963731; P=1.76 × 10(-7)). Testing for association of CBD with top progressive supranuclear palsy (PSP) GWAS single-nucleotide polymorphisms (SNPs) identified associations at MOBP (3p22; rs1768208; P=2.07 × 10(-7)) and MAPT H1c (17q21; rs242557; P=7.91 × 10(-6)). We previously reported SNP/transcript level associations with rs8070723/MAPT, rs242557/MAPT, and rs1768208/MOBP and herein identified association with rs963731/SOS1. We identify new CBD susceptibility loci and show that CBD and PSP share a genetic risk factor other than MAPT at 3p22 MOBP (myelin-associated oligodendrocyte basic protein). PMID:26077951

  14. Pathological and Clinical Spectrum of Progressive Supranuclear Palsy: With Special Reference to Astrocytic Tau Pathology.

    Science.gov (United States)

    Yokoyama, Yuichi; Toyoshima, Yasuko; Shiga, Atsushi; Tada, Mari; Kitamura, Hideaki; Hasegawa, Kazuko; Onodera, Osamu; Ikeuchi, Takeshi; Someya, Toshiyuki; Nishizawa, Masatoyo; Kakita, Akiyoshi; Takahashi, Hitoshi

    2016-03-01

    Progressive supranuclear palsy (PSP) is a four-repeat tauopathy with tau-positive, argyrophilic tuft-shaped astrocytes (TAs). We performed a pathological and clinical investigation in 40 consecutive autopsied Japanese patients with pathological diagnoses of PSP or PSP-like disease. Unequivocal TAs were present in 22 cases, all of which were confirmed to be PSP. Such TAs were hardly detected in the other 18 cases, which instead exhibited tau-positive, argyrophilic astrocytes, appearing as comparatively small clusters with central nuclei of irregularly shaped, coarse structures (equivocal TAs). Cluster analysis of the distribution pattern of tau-related pathology for these 18 cases identified two subgroups, pallido-nigro-luysian atrophy (PNLA) Type 1 (n = 9) and Type 2 (n = 9), the former being distinguished from the latter by the presence of tau-related lesions in the motor cortex, pontine nucleus and cerebellar dentate nucleus in addition to the severely affected PNL system. The duration from symptom onset until becoming wheelchair-bound was significantly longer in PNLA Type 1. Immunoblotting of samples from the three disease conditions revealed band patterns of low-molecular-mass tau fragments at ∼35 kDa. These findings shed further light on the wide pathological and clinical spectrum of four-repeat tauopathy, representing PSP in the broad sense rather than classical PSP. PMID:25974705

  15. 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.

  16. Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy

    Science.gov (United States)

    Kouri, Naomi; Ross, Owen A.; Dombroski, Beth; Younkin, Curtis S.; Serie, Daniel J.; Soto-Ortolaza, Alexandra; Baker, Matthew; Finch, Ni Cole A.; Yoon, Hyejin; Kim, Jungsu; Fujioka, Shinsuke; McLean, Catriona A.; Ghetti, Bernardino; Spina, Salvatore; Cantwell, Laura B.; Farlow, Martin R.; Grafman, Jordan; Huey, Edward D.; Ryung Han, Mi; Beecher, Sherry; Geller, Evan T.; Kretzschmar, Hans A.; Roeber, Sigrun; Gearing, Marla; Juncos, Jorge L.; Vonsattel, Jean Paul G.; Van Deerlin, Vivianna M.; Grossman, Murray; Hurtig, Howard I.; Gross, Rachel G.; Arnold, Steven E.; Trojanowski, John Q.; Lee, Virginia M.; Wenning, Gregor K.; White, Charles L.; Höglinger, Günter U.; Müller, Ulrich; Devlin, Bernie; Golbe, Lawrence I.; Crook, Julia; Parisi, Joseph E.; Boeve, Bradley F.; Josephs, Keith A.; Wszolek, Zbigniew K.; Uitti, Ryan J.; Graff-Radford, Neill R.; Litvan, Irene; Younkin, Steven G.; Wang, Li-San; Ertekin-Taner, Nilüfer; Rademakers, Rosa; Hakonarsen, Hakon; Schellenberg, Gerard D.; Dickson, Dennis W.

    2015-01-01

    Corticobasal degeneration (CBD) is a neurodegenerative disorder affecting movement and cognition, definitively diagnosed only at autopsy. Here, we conduct a genome-wide association study (GWAS) in CBD cases (n=152) and 3,311 controls, and 67 CBD cases and 439 controls in a replication stage. Associations with meta-analysis were 17q21 at MAPT (P=1.42 × 10−12), 8p12 at lnc-KIF13B-1, a long non-coding RNA (rs643472; P=3.41 × 10−8), and 2p22 at SOS1 (rs963731; P=1.76 × 10−7). Testing for association of CBD with top progressive supranuclear palsy (PSP) GWAS single-nucleotide polymorphisms (SNPs) identified associations at MOBP (3p22; rs1768208; P=2.07 × 10−7) and MAPT H1c (17q21; rs242557; P=7.91 × 10−6). We previously reported SNP/transcript level associations with rs8070723/MAPT, rs242557/MAPT, and rs1768208/MOBP and herein identified association with rs963731/SOS1. We identify new CBD susceptibility loci and show that CBD and PSP share a genetic risk factor other than MAPT at 3p22 MOBP (myelin-associated oligodendrocyte basic protein). PMID:26077951

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

    Directory of Open Access Journals (Sweden)

    Eero Rissanen

    2014-01-01

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

  18. Whole-Brain Atrophy Rate in Idiopathic Parkinson's Disease, Multiple System Atrophy, and Progressive Supranuclear Palsy.

    Science.gov (United States)

    Guevara, C; Bulatova, K; Barker, G J; Gonzalez, G; Crossley, N; Kempton, M J

    2016-01-01

    In multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), the absence of surrogate endpoints makes clinical trials long and expensive. We aim to determine annualized whole-brain atrophy rates (a-WBAR) in idiopathic Parkinson's disease (IPD), MSA, and PSP. Ten healthy controls, 20 IPD, 12 PSP, and 8 MSA patients were studied using a volumetric MRI technique (SIENA). In controls, the a-WBAR was 0.37% ± 0.28 (CI 95% 0.17-0.57), while in IPD a-WBAR was 0.54% ± 0.38 (CI 95% 0.32-0.68). The IPD patients did not differ from the controls. In PSP, the a-WBAR was 1.26% ± 0.51 (CI 95%: 0.95-1.58). In MSA, a-WBAR was 1.65% ± 1.12 (CI 95%: 0.71-2.59). MSA did not differ from PSP. The a-WBAR in PSP and MSA were significantly higher than in the IPD group (p = 0.004 and p < 0.001, resp.). In PSP, the use of a-WBAR required one-half of the patients needed for clinical scales to detect a 50% reduction in their progression. In MSA, one-quarter of the patients would be needed to detect the same effect. a-WBAR is a reasonable candidate to consider as a surrogate endpoint in short clinical trials using smaller sample sizes. The confidence intervals for a-WBAR may add a potential retrospective application for a-WBAR to improve the diagnostic accuracy of MSA and PSP versus IPD.

  19. 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.

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

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

    2015-11-15

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

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

    International Nuclear Information System (INIS)

    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.)

  2. 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.

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

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

    2008-05-15

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

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

    International Nuclear Information System (INIS)

    To determine whether h magnetic resonance spectroscopy (MRS) is useful in differentiating idiopathic Parkinson's disease (IPD) from progressive supranuclear palsy (PSP), based on metabolite ratios. Using a 1.5 T MR Unit, single voxel H MRS using STEAM with a TR of 2000 ms and a TE of 135ms was performed in seven PD and eight PSP patients. Five age-matched volunteers(mean age, 63 years) and another five younger healthy volunteers(mean age, 30 tears) were studied as normal controls. The regions of interest were the putamen and pallidum, with a size of 2X2X2 cm. After measuring the spectral intensities of each metabolite (N-acetylaspartate=NAA, choline=Cho, creatine=Cr and lactate), relative peak height ratios of NAA/Cr, Cho/Cr and NAA/ Cho, and lactate levels among four groups were compared. NAA/ Cho and NAA/ Cr ratios were statistically lower in the PSP group than the IPD group (1.21 ± 0.26 versus 1.45 ± 0.20, and 1.26 ± 0.23 versus 1.38 ± 0.19, respectively : p0.05). Cho/Cr ratios were not different among four groups. Lactate was not detected in any patients. NAA/Cho and NAA/Cr ratios in the corpus stratum were significantly lower in the PSP group than in the age-matched control and IPD groups. These results suggest that loss of neuron cells in the corpus stratum is more prominent in PSP than in IPD, and that NAA/Cho and NAA/Cr ratios may help in differential diagnosis of IPD and PSP

  5. Whole-Brain Atrophy Differences between Progressive Supranuclear Palsy and Idiopathic Parkinson’s Disease

    Science.gov (United States)

    Guevara, Carlos; Bulatova, Katherina; Barker, Gareth J.; Gonzalez, Guido; Crossley, Nicolas A.; Kempton, Matthew J.

    2016-01-01

    Background: The absence of markers for ante-mortem diagnosis of progressive supranuclear palsy (PSP), results in this disorder 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 the 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 (UPDRS III), Hoehn and Yahr (H&Y), the Clinical Global Impression for Disease Severity Scale (CGI-S) and the Frontal Assessment Battery (FAB). 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, the 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. PMID:27679572

  6. Whole-Brain Atrophy Differences between Progressive Supranuclear Palsy and Idiopathic Parkinson’s Disease

    Science.gov (United States)

    Guevara, Carlos; Bulatova, Katherina; Barker, Gareth J.; Gonzalez, Guido; Crossley, Nicolas A.; Kempton, Matthew J.

    2016-01-01

    Background: The absence of markers for ante-mortem diagnosis of progressive supranuclear palsy (PSP), results in this disorder 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 the 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 (UPDRS III), Hoehn and Yahr (H&Y), the Clinical Global Impression for Disease Severity Scale (CGI-S) and the Frontal Assessment Battery (FAB). 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, the 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.

  7. Neuropsychological functions in progressive supranuclear palsy, multiple system atrophy and Parkinson′s disease

    Directory of Open Access Journals (Sweden)

    Krishnan Syam

    2006-01-01

    Full Text Available Background: Few studies have compared cognitive functions in multiple system atrophy (MSA, progressive supranuclear palsy (PSP and Parkinson′s disease (PD. Aim: To compare the results of cognitive function tests in the three diseases and examine their relation with the severity of parkinsonism. Settings and Design: Clinic-based open prospective study. Materials and Methods: Global cognitive function tests and tests specific for frontal lobe functions were used in 25 cases of each disease. UPDRS III was used to measure the severity of parkinsonism. Statistical Analysis: ANOVA was done for group comparisons, followed by t-test for independent samples with Bonferroni correction. Pearson′s correlation test was done to assess the relation between severity of parkinsonism and cognitive functions. Results: The severity of parkinsonism was worst in PD followed by PSP and least in MSA. Patients with PSP exhibited the worst performance in both sets of cognitive tests. Even though patients with MSA did better than PD in global function tests, they performed worse than PD in some frontal function tests. There was a negative correlation between severity of parkinsonism and scores in cognitive tests in the MSA group but not in others. Conclusions: Global and frontal dysfunction was worst in PSP. The frontal dysfunction in MSA was more severe than PD, correlated with the severity of parkinsonism and was worse in clinically probable than possible cases of MSA. The severity of cognitive dysfunction in these diseases may be related to the distribution and extent of pathological changes affecting the striato-frontal circuits in them.

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

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    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.

  9. Meta-analysis of tau genetic polymorphism and sporadic progressive supranuclear palsy susceptibility

    Institute of Scientific and Technical Information of China (English)

    Hai Yuan; Xiuyan Yang; Hanlin Kang; Ying Cheng; Huiming Ren; Xiaotong Wang

    2011-01-01

    OBJECTIVE: To quantitatively evaluate the association between tau genetic polymorphism (H, and H2) and susceptibility to sporadic progressive supranuclear palsy (PSP).DATA SOURCES: Relevant Medical Subject Heading terms and text words were used to identify articles from MEDLINE (1966/2010 07), EMBASE (1984/2010-07), and Chinese National Knowledge Infrastructure (1979/2010), as well as references of the retrieved articles.STUDY SELECTION: The selected articles met the following criteria: sporadic PSP case group and healthy control group, as well as genotype frequency (H,/H, and H,/H2+H2/H2) in cases and controls.Genotype distribution in the control groups was tested using the Hardy-Weinberg Equilibrium (HWE).Articles irrelevant to HWE were excluded, and a forest plot was performed to combine all selected articles with Review Manager (Version 5.0).MAIN OUTCOME MEASURES: The summary odds ratios and corresponding 95% confidence intervals (95%Cl)for tau polymorphism (H,/H, and H,/H2+H2/H2) between sporadic PSP case and healthy control groups were estimated using the fixed effects model to assess whether tau genetic polymorphism is associated with sporadic PSP susceptibility.RESULTS: According to inclusion and exclusion criteria, a total of 16 articles, which included 1 337 sporadic PSP cases and 2 073 controls, were used in the study.Two articles were excluded because of deviation from HWE in the control groups.The combined result, based on all studies,showed a significant difference in genotype distribution between cases and controls: H,H, vs.H,H2 +H2H2 (odds ratio (OR)=4.98, 95%Cl: 3.97-6.23, P < 0.01).Stratifying for geographic distribution of PSP, sporadic PSP cases exhibited a significantly higher frequency of H,H, genotypes than controls in the United States (OR=4.07, 95%CF.3.16-5.25, P < 0.01) and Europe (OR=8.60,95%C1.5.05-14.64, P < 0.01).CONCLUSION: Tau genetic polymorphism is associated with sporadic PSP susceptibility, and geographic distribution might

  10. 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

  11. Gene expression, methylation and neuropathology correlations at progressive supranuclear palsy risk loci.

    Science.gov (United States)

    Allen, Mariet; Burgess, Jeremy D; Ballard, Travis; Serie, Daniel; Wang, Xue; Younkin, Curtis S; Sun, Zhifu; Kouri, Naomi; Baheti, Saurabh; Wang, Chen; Carrasquillo, Minerva M; Nguyen, Thuy; Lincoln, Sarah; Malphrus, Kimberly; Murray, Melissa; Golde, Todd E; Price, Nathan D; Younkin, Steven G; Schellenberg, Gerard D; Asmann, Yan; Ordog, Tamas; Crook, Julia; Dickson, Dennis; Ertekin-Taner, Nilüfer

    2016-08-01

    To determine the effects of single nucleotide polymorphisms (SNPs) identified in a genome-wide association study of progressive supranuclear palsy (PSP), we tested their association with brain gene expression, CpG methylation and neuropathology. In 175 autopsied PSP subjects, we performed associations between seven PSP risk variants and temporal cortex levels of 20 genes in-cis, within ±100 kb. Methylation measures were collected using reduced representation bisulfite sequencing in 43 PSP brains. To determine whether SNP/expression associations are due to epigenetic modifications, CpG methylation levels of associated genes were tested against relevant variants. Quantitative neuropathology endophenotypes were tested for SNP associations in 422 PSP subjects. Brain levels of LRRC37A4 and ARL17B were associated with rs8070723; MOBP with rs1768208 and both ARL17A and ARL17B with rs242557. Expression associations for LRRC37A4 and MOBP were available in an additional 100 PSP subjects. Meta-analysis revealed highly significant associations for PSP risk alleles of rs8070723 and rs1768208 with higher LRRC37A4 and MOBP brain levels, respectively. Methylation levels of one CpG in the 3' region of ARL17B associated with rs242557 and rs8070723. Additionally, methylation levels of an intronic ARL17A CpG associated with rs242557 and that of an intronic MOBP CpG with rs1768208. MAPT and MOBP region risk alleles also associated with higher levels of neuropathology. Strongest associations were observed for rs242557/coiled bodies and tufted astrocytes; and for rs1768208/coiled bodies and tau threads. These findings suggest that PSP variants at MAPT and MOBP loci may confer PSP risk via influencing gene expression and tau neuropathology. MOBP, LRRC37A4, ARL17A and ARL17B warrant further assessment as candidate PSP risk genes. Our findings have implications for the mechanism of action of variants at some of the top PSP risk loci. PMID:27115769

  12. 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.

  13. Midbrain-to-pons ratio in autopsy-confirmed progressive supranuclear palsy: replication in an independent cohort.

    Science.gov (United States)

    Kaasinen, Valtteri; Kangassalo, Noora; Gardberg, Maria; Isotalo, Juuso; Karhu, Jari; Parkkola, Riitta; Sonninen, Pirkko

    2015-07-01

    Recent neuropathologically confirmed clinical data suggest that the midbrain-to-pons ratio, as calculated from conventional brain MRI, has high specificity and sensitivity for the diagnosis of progressive supranuclear palsy (PSP). Here, we aimed to replicate these findings in an independent autopsy-confirmed cohort of 6 PSP patients and 23 non-PSP patients. Patients with confirmed PSP had clearly lower midbrain-to-pons ratios compared to non-PSP patients (p midbrain-to-pons ratios higher than 0.50, whereas all but one PSP patient had a ratio lower than 0.50. The positive predictive value (PPV) of the ratio (midbrain-to-brain ratios constitute reliable and clinically useful estimates of diagnostic midbrain atrophy in relation to PSP pathology.

  14. Conjugate Gaze Palsies

    Science.gov (United States)

    ... Nerve) Palsy Sixth Cranial Nerve (Abducens Nerve) Palsy Trigeminal Neuralgia Bell Palsy Hemifacial Spasm Glossopharyngeal Neuralgia Hypoglossal Nerve ... Nerve) Palsy Sixth Cranial Nerve (Abducens Nerve) Palsy Trigeminal Neuralgia Bell Palsy Hemifacial Spasm Glossopharyngeal Neuralgia Hypoglossal Nerve ...

  15. 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.

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

    International Nuclear Information System (INIS)

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

  17. 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 ...

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

    Directory of Open Access Journals (Sweden)

    Amanda Worker

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

  19. Progressive Brachial Plexus Palsy after Osteosynthesis of an Inveterate Clavicular Fracture

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    Marco Rosati

    2013-07-01

    Full Text Available Introduction: The thoracic outlet syndrome (TOS is a rare complication of clavicular fracture, occurring in 0.5-9% of cases . In the literature from 1965 – 2010, 425 cases of TOS complicating a claviclular fracture were described. However, only 5 were observed ​​after a surgical procedure of reduction and fixation. The causes of this complication were due to the presence of an exuberant callus, to technical surgery errors or to vascular lesions. In this paper we describe a case of brachial plexus plasy after osteosynthesis of clavicle fracture. Case Report: A 48 year old female, presented to us with inveterate middle third clavicle fracture of 2 months duration. She was an alcoholic, smoker with an history of opiate abuse and was HCV positive. At two month the fracture was displaced with no signs of union and open rigid fixation with plate was done. The immediate postoperative patient had signs of neurologic injury. Five days after surgery showed paralysis of the ulnar nerve, at 10 days paralysis of the median nerve, radial and ulnar paresthesias in the territory of the C5-C6-C7-C8 roots. She was treated with rest, steroids and neurotrophic drugs. One month after surgery the patient had signs of complete denervation around the brachial plexus. Implant removal was done and in a month ulnar and median nerve functions recovered. At three months post implant removal the neurological picture returned to normal. Conclusion: We can say that TOS can be seen as arising secondary to an “iatrogenic compartment syndrome” justified by the particular anatomy of the space cost joint. The appropriateness of the intervention for removal of fixation devices is demonstrated by the fact that the patient has returned to her daily activities in the absence of symptoms and good functional recovery in about three months, despite fracture nonunion. Keywords: Brachial plexus palsy, clavicle fractures, outlet thoracic syndrome.

  20. 干细胞移植治疗脑瘫的研究进展%The research progress of stem cells transplantation in treatment of cerebral palsy

    Institute of Scientific and Technical Information of China (English)

    梁宝仁; 逯军; 娄小丽

    2012-01-01

    脑瘫是一种严重危害儿童健康的疾病.虽然其病因、发病机制及临床表现已基本明确,但关于脑瘫的治疗目前还没有比较理想的方法.干细胞移植最近几年已逐渐成为脑瘫治疗的热点,现就干细胞移植治疗脑瘫研究进展作一综述.%Cerebral palsy is a serious disease that can be harm to children's health. Although its etiology, pathogenesis and clinical manifestations have been clarified, there still has no ideal way to its treatment. Stem cells transplantation has been the hot spot in the treatment of cerebral palsy in recent years. The literatures about the research progress of stem cells transplantation in the treatment of cerebral palsy were reviewed in this paper.

  1. 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.

  2. Correlation with neuropsychological assessment and SPM analysis of brain perfusion SPECT in patients with progressive supranuclear palsy

    International Nuclear Information System (INIS)

    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. 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.

  5. Cerebral Palsy

    Science.gov (United States)

    ... 1 • 2 • 3 For Teens For Kids For Parents MORE ON THIS TOPIC Cerebral Palsy: Keith's Story Physical Therapy I Have Cerebral Palsy. Can I Babysit? Body Image and Self-Esteem Contact Us Print Resources Send to a friend ...

  6. Cerebral palsy - resources

    Science.gov (United States)

    Resources - cerebral palsy ... The following organizations are good resources for information on cerebral palsy : National Institute of Neurological Disorders and Stroke -- www.ninds.nih.gov/disorders/cerebral_palsy/cerebral_palsy. ...

  7. Cerebral Palsy (For Parents)

    Science.gov (United States)

    ... Story" 5 Things to Know About Zika & Pregnancy Cerebral Palsy KidsHealth > For Parents > Cerebral Palsy Print A A ... kids who are living with the condition. About Cerebral Palsy Cerebral palsy is one of the most common ...

  8. microRNA profiling: increased expression of miR-147a and miR-518e in progressive supranuclear palsy (PSP).

    Science.gov (United States)

    Tatura, Roman; Buchholz, Malte; Dickson, Dennis W; van Swieten, John; McLean, Catriona; Höglinger, Günter; Müller, Ulrich

    2016-07-01

    Progressive supranuclear palsy is a sporadic neurodegenerative disorder. Genetic, environmental, and possibly epigenetic factors contribute to disease. In order to better understand the potential role of epigenetic changes in progressive supranuclear palsy, we investigated whether some microRNAs and their target genes are dysregulated. We analyzed expression of 372 well-characterized microRNAs in forebrains of a total of 40 patients and of 40 controls using TaqMan arrays and SYBR Green quantitative real-time PCR. The exploratory cohort included forebrains from 20 patients and 20 controls provided by the Erasmus Medical Centre in Rotterdam, Netherlands. Confirmatory samples were from Jacksonville, Florida, and from Melbourne, Australia. Both microRNA profiling and SYBR Green quantitative real-time PCR revealed significant upregulation of miR-147 (miR-147a) and miR-518e in the exploratory cohort. Highly increased expression of these two microRNAs was validated in the confirmatory samples. Target genes of miR-147a (NF1, ACLY, ALG12) and of miR-518e (CPEB1, JAZF1, RAP1B) were repressed in patients' forebrains. The results suggest that dysregulation of specific microRNAs contributes to disease by repressing target genes involved in various cellular functions. PMID:27052995

  9. 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 management of NPH. PMID:27581311

  10. Progress in Treatment of Bell's Palsy%贝尔氏面瘫的治疗进展

    Institute of Scientific and Technical Information of China (English)

    任平治; 马亚琳

    2004-01-01

    贝尔氏面瘫(Bells palsy),又称特发性面瘫(idiopathic palsy),是临床上最常见的周围性面神经麻痹,其病因不明,诊断依赖于对其他疾病的排除,治疗上尚无统一认识。尽管耳神经外科、神经电生理学、影像学(CT、MRI)及各种听力和前庭功能检查的发展,为鉴别面神经麻痹的病因、确定病变部位及

  11. Progressive Supranuclear Palsy

    Science.gov (United States)

    ... Also, people with PSP show accumulation of the protein tau in affected brain cells, while people with Parkinson’s ... is the accumulation of abnormal deposits of the protein tau in nerve cells in the brain, so that ...

  12. Insulinlike growth factor (IGF)-1 administration ameliorates disease manifestations in a mouse model of spinal and bulbar muscular atrophy.

    Science.gov (United States)

    Rinaldi, Carlo; Bott, Laura C; Chen, Ke-lian; Harmison, George G; Katsuno, Masahisa; Sobue, Gen; Pennuto, Maria; Fischbeck, Kenneth H

    2012-12-06

    Spinal and bulbar muscular atrophy is an X-linked motor neuron disease caused by polyglutamine expansion in the androgen receptor. Patients develop slowly progressive proximal muscle weakness, muscle atrophy and fasciculations. Affected individuals often show gynecomastia, testicular atrophy and reduced fertility as a result of mild androgen insensitivity. No effective disease-modifying therapy is currently available for this disease. Our recent studies have demonstrated that insulinlike growth factor (IGF)-1 reduces the mutant androgen receptor toxicity through activation of Akt in vitro, and spinal and bulbar muscular atrophy transgenic mice that also overexpress a noncirculating muscle isoform of IGF-1 have a less severe phenotype. Here we sought to establish the efficacy of daily intraperitoneal injections of mecasermin rinfabate, recombinant human IGF-1 and IGF-1 binding protein 3, in a transgenic mouse model expressing the mutant androgen receptor with an expanded 97 glutamine tract. The study was done in a controlled, randomized, blinded fashion, and, to reflect the clinical settings, the injections were started after the onset of disease manifestations. The treatment resulted in increased Akt phosphorylation and reduced mutant androgen receptor aggregation in muscle. In comparison to vehicle-treated controls, IGF-1-treated transgenic mice showed improved motor performance, attenuated weight loss and increased survival. Our results suggest that peripheral tissue can be targeted to improve the spinal and bulbar muscular atrophy phenotype and indicate that IGF-1 warrants further investigation in clinical trials as a potential treatment for this disease.

  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. Nemo-like kinase is a novel regulator of spinal and bulbar muscular atrophy.

    Science.gov (United States)

    Todd, Tiffany W; Kokubu, Hiroshi; Miranda, Helen C; Cortes, Constanza J; La Spada, Albert R; Lim, Janghoo

    2015-01-01

    Spinal and bulbar muscular atrophy (SBMA) is a progressive neuromuscular disease caused by polyglutamine expansion in the androgen receptor (AR) protein. Despite extensive research, the exact pathogenic mechanisms underlying SBMA remain elusive. In this study, we present evidence that Nemo-like kinase (NLK) promotes disease pathogenesis across multiple SBMA model systems. Most remarkably, loss of one copy of Nlk rescues SBMA phenotypes in mice, including extending lifespan. We also investigated the molecular mechanisms by which NLK exerts its effects in SBMA. Specifically, we have found that NLK can phosphorylate the mutant polyglutamine-expanded AR, enhance its aggregation, and promote AR-dependent gene transcription by regulating AR-cofactor interactions. Furthermore, NLK modulates the toxicity of a mutant AR fragment via a mechanism that is independent of AR-mediated gene transcription. Our findings uncover a crucial role for NLK in controlling SBMA toxicity and reveal a novel avenue for therapy development in SBMA. PMID:26308581

  15. Spinal and bulbar muscular atrophy and Charcot-Marie-Tooth type 1A: Co-existence of two rare neuromuscular genetic diseases in the same patient.

    Science.gov (United States)

    Sagnelli, Anna; Scaioli, Vidmer; Piscosquito, Giuseppe; Salsano, Ettore; Dalla Bella, Eleonora; Gellera, Cinzia; Pareyson, Davide

    2015-10-01

    Spinal and bulbar muscular atrophy is an X-linked neuromuscular disease caused by a trinucleotide CAG repeat expansion in the androgen receptor gene; it is clinically characterized by adult-onset, slowly progressive weakness and atrophy mainly affecting proximal limb and bulbar muscles. Charcot-Marie-Tooth disease type 1A is an autosomal dominant polyneuropathy due to peripheral myelin protein 22 gene duplication and characterized by slowly progressive distal limb muscle weakness, atrophy and sensory loss with foot deformities. Here we report the co-occurrence of both neuromuscular genetic diseases in the same male patient. Difficulties in climbing stairs and jaw weakness were presenting symptoms consistent with SBMA. However, predominant distal weakness and bilateral pes cavus were rather suggestive of a hereditary polyneuropathy. The combination of two diseases, even if extremely rare, should be considered in the presence of atypical symptoms; in the case of genetic diseases this event may have important implications on family members' counseling.

  16. 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

  17. Cerebral palsy

    International Nuclear Information System (INIS)

    This paper reviews cranial MR findings in patients with cerebral palsy (CP) to clarify and categorize this disorder. The MR images of 40 patients with clinical CP were retrospectively reviewed. All patients suffered either varying spastic plegias, hypotonicity, or choreoathetosis. Concomitantly, the patients suffered from static encephalopathy, developmental delay, and/or microcephaly. Twenty-four patients were born at or near term, 10 were premature, and incomplete birth histories were available in six. The MR images revealed mild to severe degrees of white matter damage in 24 patients (12 term, nine premature, three unknown)

  18. 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...

  19. 脑性瘫痪的外科治疗进展%Progress in surgical treatment for cerebral palsy

    Institute of Scientific and Technical Information of China (English)

    于炎冰

    2005-01-01

    目的:探讨脑性瘫痪的外科治疗进展.资料来源:应用计算机检索Medline 1997-01/2004-09的与脑性瘫痪的外科治疗相关文章,检索词"cerebral palsy,surgicaltreatment",并限定语言种类为English,以及中文全文数据库CNKI1997-01/2004-09相关文章,检索词"脑性瘫痪,外科治疗",并手工检索近期的相关中文期刊和有关书籍.资料选择:对资料进行初审,选出包含研究目的的文献,筛除与目的无关的文献.纳入标准为:①脑性瘫痪的外科治疗.②临床试验研究.排除标准为脑性瘫痪的非外科治疗.资料提炼:共找出相关性强的文献57篇,并查找全文,其中37篇涉及脑瘫的神经外科手术治疗,15篇涉及脑瘫的矫形外科手术治疗,5篇涉及脑瘫的综合的外科手术治疗.资料综合:57篇文献对有关脑瘫的外科手术治疗进行综合分析.结论:手术治疗总的原则为:全面临床评估,严格掌握手术适应证,通过解除痉挛、纠正畸形为康复治疗提供条件或起辅助作用.选择性脊神经后根部分切断术和周围神经选择性部分切断术是治疗脑瘫性肢体痉挛状态安全有效的神经外科手术方法.及时的、长期、正规的康复训练是治疗脑瘫的最主要方法,手术治疗只是为康复创造条件或为补充的手段而不能替代康复.

  20. Aerobic training in children with cerebral palsy.

    Science.gov (United States)

    Nsenga, A L; Shephard, R J; Ahmaidi, S; Ahmadi, S

    2013-06-01

    Rehabilitation is a major goal for children with cerebral palsy, although the potential to enhance cardio-respiratory fitness in such individuals remains unclear. This study thus compared current cardio-respiratory status between children with cerebral palsy and able-bodied children, and examined the ability to enhance the cardio-respiratory fitness of children with cerebral palsy by cycle ergometer training. 10 children with cerebral palsy (Gross Motor Function Classification System levels I and II) participated in thrice-weekly 30 min cycle ergometer training sessions for 8 weeks (mean age: 14.2±1.9 yrs). 10 additional subjects with cerebral palsy (mean age: 14.2±1.8 yrs) and 10 able-bodied subjects (mean age: 14.1±2.1 yrs) served as controls, undertaking no training. All subjects undertook a progressive cycle ergometer test of cardio-respiratory fitness at the beginning and end of the 8-week period. Cardio-respiratory parameters [oxygen intake V˙O2), ventilation V ˙ E) and heart rate (HR)] during testing were measured by Cosmed K4 b gas analyzer. The children with cerebral palsy who engaged in aerobic training improved their peak oxygen consumption, heart rate and ventilation significantly (pchildren with cerebral palsy can benefit significantly from cardio-respiratory training, and such training should be included in rehabilitation programs.

  1. United Cerebral Palsy

    Science.gov (United States)

    ... be sure to follow us on Twitter . United Cerebral Palsy UCP educates, advocates and provides support services to ... Partners Merz Logo Sprint Relay Copyright © 2015 United Cerebral Palsy 1825 K Street NW Suite 600 Washington, DC ...

  2. 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.)

  3. 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.

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

    Science.gov (United States)

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

    2015-11-01

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

  5. Cerebral palsy and epilepsy

    OpenAIRE

    Knežević-Pogančev Marija

    2010-01-01

    Introduction. Cerebral palsy is the most common cause of physical disability in early childhood. Epilepsy is known to have a high association with cerebral palsy. All types of epileptic seizures can be seen in patients with cerebral palsy. Complex partial and secondary generalized ones are the most frequent seizure types. In persons with cerebral palsy and mental retardation, the diagnosis of epilepsy presents unique difficulties. Generally they are not able to describe the epileptic ev...

  6. Rehabilitation outcomes in children with cerebral palsy during a 2 year period

    OpenAIRE

    İçağasıoğlu, Afitap; Mesci, Erkan; Yumusakhuylu, Yasemin; Turgut, Selin Turan; Murat, Sadiye

    2015-01-01

    [Purpose] To observe motor and functional progress of children with cerebral palsy during 2 years. [Subjects and Methods] Pediatric cerebral palsy patients aged 3–15 years (n = 35/69) with 24-month follow-up at our outpatient cerebral palsy clinic were evaluated retrospectively. The distribution of cerebral palsy types was as follows: diplegia (n = 19), hemiplegia (n = 4), and quadriplegia (n = 12). Participants were divided into 3 groups according to their Gross Motor Functional Classificati...

  7. 脑性瘫痪的危险因素研究进展(综述)%Risk factors of cerebral palsy research progress

    Institute of Scientific and Technical Information of China (English)

    周陶成; 王训

    2014-01-01

    The incidence of cerebral palsy is currently on the rise. Etiology and pathology of cerebral palsy is quite complex.Time of brain injury, the risk factors often are divided into prenatal factors, intrapartum factors and postnatal factors . Previously widely believed, intrapartum and postnatal factors are the main reason for cerebral palsy, but in recent years the view that prenatal factors are the main causes of cerebral palsy. Intrapartum and postnatal risk factors are likely to be the result of prenatal quantitative factors, rather than direct cause of cerebral palsy.%脑性瘫痪目前发病率有上升趋势,病因相当复杂,其危险因素按脑损伤发生的时间常分为产前因素、产时因素和产后因素。以前普遍认为,产时和产后因素是脑瘫的主要原因,但近年来认为,产前因素是脑瘫的主要病因,产时和产后危险因素很可能是产前因素导致的结果变量,而不是脑瘫的直接病因。

  8. 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.

  9. 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

  10. Rapidly Worsening Bulbar Symptoms in a Patient with Spinobulbar Muscular Atrophy

    OpenAIRE

    Montserrat Diaz-Abad; Porter, Neil C

    2013-01-01

    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 refl...

  11. Contractile dysfunction in muscle may underlie androgen-dependent motor dysfunction in spinal bulbar muscular atrophy.

    Science.gov (United States)

    Oki, Kentaro; Halievski, Katherine; Vicente, Laura; Xu, Youfen; Zeolla, Donald; Poort, Jessica; Katsuno, Masahisa; Adachi, Hiroaki; Sobue, Gen; Wiseman, Robert W; Breedlove, S Marc; Jordan, Cynthia L

    2015-04-01

    Spinal and bulbar muscular atrophy (SBMA) is characterized by progressive muscle weakness linked to a polyglutamine expansion in the androgen receptor (AR). Current evidence indicates that mutant AR causes SBMA by acting in muscle to perturb its function. However, information about how muscle function is impaired is scant. One fundamental question is whether the intrinsic strength of muscles, an attribute of muscle independent of its mass, is affected. In the current study, we assess the contractile properties of hindlimb muscles in vitro from chronically diseased males of three different SBMA mouse models: a transgenic (Tg) model that broadly expresses a full-length human AR with 97 CAGs (97Q), a knock-in (KI) model that expresses a humanized AR containing a CAG expansion in the first exon, and a Tg myogenic model that overexpresses wild-type AR only in skeletal muscle fibers. We found that hindlimb muscles in the two Tg models (97Q and myogenic) showed marked losses in their intrinsic strength and resistance to fatigue, but were minimally affected in KI males. However, diseased muscles of all three models showed symptoms consistent with myotonic dystrophy type 1, namely, reduced resting membrane potential and deficits in chloride channel mRNA. These data indicate that muscle dysfunction is a core feature of SBMA caused by at least some of the same pathogenic mechanisms as myotonic dystrophy. Thus mechanisms controlling muscle function per se independent of mass are prime targets for SBMA therapeutics.

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

    Science.gov (United States)

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

    2016-01-01

    Skull-base metastasis (SBM) from hepatocellular carcinoma (HCC) is extremely rare, and multiple cranial nerve paralysis due to SBM from HCC is also rare. 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. After several failed attempts to treat the primary tumor and bone metastases, neurological examination revealed left VII, IX, X, and XI cranial nerve paralysis. Computed tomography of the skull base subsequently revealed a large mass that had destroyed the left occipital and temporal bones and invaded the adjacent structure. After radiotherapy (27 Gy, 9 fractions), the patient experienced relief from his pain, and the cranial nerve dysfunction regressed. However, the patient ultimately died, due to the tumor's progression. Radiotherapy is usually the best option to relieve pain and achieve regression of cranial nerve dysfunction in cases of SBM from HCC, although early treatment is needed to achieve optimal outcomes. The present case helps expand our understanding regarding this rare metastatic pathway and indicates that improved awareness of SBM in clinical practice can help facilitate timely and appropriate treatment.

  13. An Analysis of Trends for People with MR, Cerebral Palsy, and Epilepsy Receiving Services from State VR Agencies: Ten Years of Progress.

    Science.gov (United States)

    Gilmore, Dana Scott; Schuster, Jennifer L.; Timmons, Jaimie Ciulla; Butterworth, John

    2000-01-01

    Presents the results of a secondary analysis of the RSA- 911 database from the Rehabilitation Services Administration. All successful vocational rehabilitation (VR) closures for individuals with mental retardation, cerebral palsy, and epilepsy for five data points between 1985 and 1995 were investigated. Trends in the use of competitive employment…

  14. 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.

  15. 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.

  16. The treatment progress and prevention introduction of cerebral palsy%小儿脑性瘫痪的防治进展

    Institute of Scientific and Technical Information of China (English)

    卢晓妹; 杨李

    2010-01-01

    脑性瘫痪(cerebral palsy,CP,以下简称脑瘫)是以非进展性中枢运动障碍和姿势异常为主要表现的临床综合征,本文就脑性瘫痪的治疗进展和预防进行复习并综述.

  17. Progress in the animal model of cerebral palsy%脑性瘫痪动物模型的研究进展

    Institute of Scientific and Technical Information of China (English)

    黄会芝

    2011-01-01

    Cerebral palsy is a major cause of severe chronic disability in children. Experimental models aim to replicate one or more features of cerebral palsy in developing animals. These models are as heterogeneous as the disorder itself, such as timing.and mechanism of injury (stroke, infection, congenital malformation or asphyxia),target of injury (white matter or neurons) or function outcome. This review is to summarize different experimental models in cerebral palsy.%脑性瘫痪(脑瘫)是导致儿童严重慢性残疾的重要原因。应用动物模型的目的是在动物身上复制一种或更多的脑瘫特征来进行研究,而这些模型和疾患本身一样是具有异质性的,如损伤发作的时间和机制(中风、感染、先天畸形或窒息)、损伤的靶点(白质或神经元)或功能结果等。该文对不同的脑瘫动物模型作一综述。

  18. Cerebral Palsy (CP) Quiz

    Science.gov (United States)

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

  19. 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.

  20. 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 < 0.001) and episcleral vessels (p = 0.03). Average episcleral diameter increased from 78.9 ± 8.7 μm (mean ± standard deviation) at normoxia to 97.6 ± 14.3 μm at hypoxia (p = 0.02). Diameters of bulbar conjunctival vessels showed no significant change from 80.1 ± 7.6 μm at normoxia to 80.6 ± 7.0 μm at hypoxia (p = 0.89). When exposed to ambient air, hypoxic bulbar conjunctival vessels rapidly reoxygenated due to oxygen diffusion from ambient air. Reoxygenation occured in an exponential manner, and SO2 reached normoxia baseline levels. The average

  1. Progression of knee joint kinematics in children with cerebral palsy with and without rectus femoris transfers: a long-term follow up.

    Science.gov (United States)

    Moreau, Noelle; Tinsley, Suzanne; Li, Li

    2005-10-01

    The purpose of this study was to compare long-term outcomes of multi-level surgery with and without rectus femoris transfer (RFT) in a group of children with cerebral palsy. Forty-one subjects with a diagnosis of cerebral palsy were divided into a RFT group (28 subjects with 50 sides) and non-RFT group (13 subjects with 22 sides). The study protocol included pre-operative gait analysis, multi-level orthopedic surgical intervention, one year post-operative gait analysis, and three year or greater post-operative gait analysis. All participants received inpatient physical therapy for 2-12 weeks either following surgery or following a period of immobilization, depending on surgical procedures performed. Results showed improved peak knee flexion during swing phase (PKFS) for the RFT group one year after surgery. The deviation from normal in PKFS in the RFT group improved, on average, from seven to five degrees. The deviation from normal in PKFS in the non-RFT group increased approximately four degrees in the same period of time. The knee flexion swing range (KFSR) of the RFT group increased dramatically by 11 degrees after surgery, where no significant KFSR changes observed in the non-RFT group. Those parameters maintained relatively stable from one to three years post surgery for both groups. These observations support our hypothesis that improvements after RFT surgery persist over time, counteracting the negative effects of growth and time.

  2. Cerebral Palsy Litigation

    OpenAIRE

    Sartwelle, Thomas P.; Johnston, James C.

    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 mothe...

  3. Rehabilitation in cerebral palsy.

    OpenAIRE

    Molnar, G. E.

    1991-01-01

    Cerebral palsy is the most frequent physical disability of childhood onset. Over the past four decades, prevalence has remained remarkably constant at 2 to 3 per 1,000 live births in industrialized countries. In this article I concentrate on the rehabilitation and outcome of patients with cerebral palsy. The epidemiologic, pathogenetic, and diagnostic aspects are highlighted briefly as they pertain to the planning and implementation of the rehabilitation process.

  4. Cranial nerve palsies in childhood

    OpenAIRE

    Lyons, C J; Godoy, F; ALQahtani, E

    2015-01-01

    We review ocular motor cranial nerve palsies in childhood and highlight many of the features that differentiate these from their occurrence in adulthood. The clinical characteristics of cranial nerve palsies in childhood are affected by the child's impressive ability to repair and regenerate after injury. Thus, aberrant regeneration is very common after congenital III palsy; Duane syndrome, the result of early repair after congenital VI palsy, is invariably associated with retraction of the g...

  5. Nanomedicine in cerebral palsy

    Directory of Open Access Journals (Sweden)

    Balakrishnan B

    2013-11-01

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

  6. 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 h

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

    Science.gov (United States)

    Gordon, Shirley C.

    2008-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Mohammed Abd-Alla Elgammal

    2009-08-01

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

  9. Testosterone treatment fails to accelerate disease in a transgenic mouse model of spinal and bulbar muscular atrophy

    Directory of Open Access Journals (Sweden)

    Erica S. Chevalier-Larsen

    2012-01-01

    Evidence from multiple animal models demonstrates that testosterone plays a crucial role in the progression of symptoms in spinal and bulbar muscular atrophy (SBMA, a condition that results in neurodegeneration and muscle atrophy in affected men. Mice bearing a transgene encoding a human androgen receptor (AR that contains a stretch of 112 glutamines (expanded polyglutamine tract; AR112Q mice reproduce several aspects of the human disease. We treated transgenic male AR112Q mice with testosterone for 6 months. Surprisingly, testosterone treatment of AR112Q males did not exacerbate the disease. Although transgenic AR112Q males exhibited functional deficits when compared with non-transgenics, long-term testosterone treatment had no effect on motor function. Testosterone treatment also failed to affect cellular markers of disease, including inclusion formation (the accumulation of large nuclear aggregates of mutant AR protein and levels of unphosphorylated neurofilament heavy chain. These data suggest that the mechanism of disease in SBMA saturates at close to endogenous hormone levels and that individuals with SBMA who take, or have taken, testosterone for its putative therapeutic properties are unlikely to suffer adverse effects.

  10. Further diffusion tensor imaging contribution in horizontal gaze palsy and progressive scoliosis Contribuição adicional das imagens por tensores de difusão em paralisia do olhar conjugado horizontal associada a escoliose progressiva

    Directory of Open Access Journals (Sweden)

    Maria Conceptión García Otaduy

    2009-12-01

    Full Text Available In two siblings with clinical diagnosis of horizontal gaze palsy associated with progressive scoliosis (HGPPS we could demonstrate by diffusion tensor imaging: (1 An anterior displacement of the transverse pontine fibers; (2 Posterior clumping of the corticospinal, medial lemniscus and central tegmental tracts and of the medial and dorsal longitudinal fasciculi complex; (3 Absent decussation of superior cerebellar peduncle. Those findings can contribute as surrogate markers for the diagnosis.Em dois irmãos com diagnóstico clínico de paralisia do olhar conjugado horizontal associada a escoliose progressiva, foi possível determinar através de imagens por tensores de difusão: (1 Deslocamento anterior das fibras pontinas transversas; (2 Agrupamento posterior do trato córtico-espinhal, lemnisco medial e trato tegmentar central e complexos dos fascículos longitudinais medial e dorsal; (3 Ausência da decussação dos pedúnculos cerebelares superiores. Tais achados podem contribuir como marcadores para o diagnóstico.

  11. A study on the cerebral glucose metabolism in progressive supranuclear palsy%进行性核上性麻痹的脑葡萄糖代谢研究

    Institute of Scientific and Technical Information of China (English)

    马爱军; 郭晓军; 李大成; 张本恕; 潘旭东

    2012-01-01

    Objective 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).Methods A total of 13 patients with PSP and 30 matched healthy controls were performed 18F-FDG PET imaging at rest state.Visual inspection and statistical parametric mapping (SPM) were used to investigate regional cerebral metabolic rate of glucose (rCMRglc).Results 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.Conclusion 18 F-FDG PET imaging is helpful for the early diagnosis of PSP.%目的 探讨进行性核上性麻痹(PSP)患者18F-脱氧葡萄糖正电子发射体层(18F-FDG PET)脑显像特点及与临床特征的相关性.方法 对13例PSP患者和30例相匹配的健康对照者进行脑18F-FDG PET检查,应用视觉分析法与统计参数图(SPM)分析法比较2组脑葡萄糖代谢的差异.结果 与对照组相比,PSP患者脑18F-FDG PET显像视觉分析法显示双侧额叶皮质、中脑、皮质下核团如基底节、丘脑示踪剂摄取减少,SPM分析显示双侧双侧额上、中回、额叶内侧部皮质、扣带回、中脑及皮质下结构,基底节、丘脑葡萄糖代谢减低,与患者眼球垂直运动障碍、姿势障碍、肌张力增高及认知功能障碍等临床症状相一致.结论 结合临床症状,应用18F-FDG PET脑显像有助于PSP的早期诊断.

  12. TANG's Scalp Acupuncture and Infantile Cerebral Palsy:Case Reports of 34 Cases

    Institute of Scientific and Technical Information of China (English)

    秦秀娣; 韩丑萍

    2008-01-01

    @@ Cerebral palsy (CP) refers to non-progressive motor disorder resulting from a group of cerebral lesions. It can occur as a result of motor center injuries before, during or after birth and subsequently non-progressive central motor dysfunction, followed by mental retardation and motor impairment of the four limbs. The author treated 34 cases of cerebral palsy with the scalp acupuncture of Doctor TANG Song-yan. The report is now as follows.

  13. Observation on 105 Cases of Duodenal Bulbar Ulcer Treated by Combined Therapy of Catgut Embedding and Chinese Drugs

    Institute of Scientific and Technical Information of China (English)

    范兆金

    2001-01-01

    @@Duodenal bulbar ulcer is a frequently encountered disease. Clinically, it manifests itself by regular epigastric pain accompanied by belching and acid regurgitation, corresponding to epigastralgia and stomach distending pain in traditional Chinese medicine (TCM). Based on the TCM type-differentiation, the author has treated 105 cases of duodenal bulbar ulcer by the combined therapy of catgut embedding at point Zusanli (ST 36) and Chinese drugs with satisfactory results. A summary is as follows. Clinical Data 1. Case selection: The cases selected all had the clinical symptoms and signs of duodenal bulbar ulcer, and had been diagnosed by GI examination at this or other hospitals with no obvious therapeutic effects obtained after relevant treatment, and still symptoms and signs and repeated attacks of pain.

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

    Energy Technology Data Exchange (ETDEWEB)

    Tsukamoto, Kazumichi; Kanasaki, Yoshiko; Kakite, Suguru; Fujii, Shinya; Kaminou, Toshio; Ogawa, Toshihide [Tottori University, Division of Radiology, Department of Pathophysiological and Therapeutic Science, Faculty of Medicine, Yonago, Tottori (Japan); Matsusue, Eiji [Tottori Prefectural Central Hospital, Department of Radiology, Tottori (Japan)

    2012-09-15

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

  15. Dysphagia in cerebral palsy

    OpenAIRE

    Salghetti, Annamaria; Martinuzzi, Andrea

    2013-01-01

    Abstract. Feeding problemsare often present in children with neuromotor impairment: dysphagia is usuallyseen in the most severe form of cerebral palsy and it’s defined as thedifficulty with any of the four phases of swallowing. Clinical consequences aremalnutrition and recurrent chest infections that reduce expected duration andquality of life. In order to prevent these consequences it’s important todetect with clinical and instrumental examinations dysphagia symptoms and totreat them. Clinic...

  16. Nanomedicine in cerebral palsy

    OpenAIRE

    Balakrishnan B; Nance E; Johnston MV; Kannan R; Kannan S

    2013-01-01

    Bindu Balakrishnan,1 Elizabeth Nance,1 Michael V Johnston,2 Rangaramanujam Kannan,3 Sujatha Kannan1 1Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University; Baltimore, MD, USA; 2Department of Neurology and Pediatrics, Kennedy Krieger Institute, Baltimore, MD, USA; 3Department of Ophthalmology, Center for Nanomedicine, Johns Hopkins University, Baltimore, MD, USA Abstract: Cerebral palsy is a chronic childhood disorder that can have diverse etiologies. Injury to the...

  17. Objective classification of gait patterns in children with cerebral palsy

    OpenAIRE

    Azupardo, Mervic

    2007-01-01

    “Cerebral” is defined as pertaining to the brain, cerebrum or intellect, and “Palsy” refers to paralysis of muscle or group of muscles. Jointly, Cerebral Palsy (CP) is the term used to describe a group of conditions with motor impairments resulting from brain damage during the early stages of development. Cerebral palsy is non progressive and is usually not diagnosed until a child is about 2 to 3 years of age. About 2 to 3 children in 1,000 over the age of three have cerebral p...

  18. 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.

  19. 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. PMID:16391455

  20. Ophthalmologic Findings in Children with Cerebral Palsy

    OpenAIRE

    Ali Kurt; Kemal Türkyılmaz; Adem Gül

    2012-01-01

    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 ...

  1. CEREBRAL PALSY : ANTENATAL RISK FACTORS

    Directory of Open Access Journals (Sweden)

    Srinivasa Rao

    2015-05-01

    Full Text Available 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 cerebral palsy in the study group. MATERIA LS AND METHODS: Retrospective study was done to assess possible associated antenatal risk factors for cerebral palsy. Mothers of 100 cerebral palsy children were selected who are treated in Rani Chandramani Devi Hospital, a Government hospital in Visakhapa tn am, Andhra Pradesh State, India , from 2012 to 2014 and 100 controls, mothers of normal children were studied. Detailed antenatal history was obtained from the mothers of the children in both affected and control group. RESULTS: From the data, we conclude that the association of maternal anaemia with cerebral palsy is 7.3 times higher; association of maternal hypertension with cerebral palsy is 6.6 time higher, association with Pre - eclampsia is 6 times higher; association with Eclampsia is 8.6 times higher ; with antepartum haemorrhage, the association is 8.6 times higher and association of multiple pregnancy with cerebral palsy is 4.8 times higher than with controls. CONCLUSION: From this study of the role of antenatal risk factors, in the occurrence of cer ebral palsy in children it is concluded that the most common risk factor associated with cerebral palsy is the maternal anaemia and the other important risk factors associated being hypertension, pre eclampsia, eclampsia, antepartum haemorrhage and multipl e births.

  2. Physical fitness in children and adolescents with cerebral palsy

    OpenAIRE

    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 have distinctly subnormal aerobic and anaerobic capacity in comparison with typically developing peers. Also, muscle strength is reduced and energy cost of locomotion is high. Low levels on these fi...

  3. 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

  4. Palsies of Cranial Nerves That Control Eye Movement

    Science.gov (United States)

    ... Medical News Palsies of Cranial Nerves That Control Eye Movement By Michael Rubin, MDCM NOTE: This is the ... Gaze Palsies Palsies of Cranial Nerves That Control Eye Movement Third Cranial Nerve (Oculomotor Nerve) Palsy Fourth Cranial ...

  5. Cranial nerve palsies in childhood.

    Science.gov (United States)

    Lyons, C J; Godoy, F; ALQahtani, E

    2015-02-01

    We review ocular motor cranial nerve palsies in childhood and highlight many of the features that differentiate these from their occurrence in adulthood. The clinical characteristics of cranial nerve palsies in childhood are affected by the child's impressive ability to repair and regenerate after injury. Thus, aberrant regeneration is very common after congenital III palsy; Duane syndrome, the result of early repair after congenital VI palsy, is invariably associated with retraction of the globe in adduction related to the innervation of the lateral rectus by the III nerve causing co-contraction in adduction. Clinical features that may be of concern in adulthood may not be relevant in childhood; whereas the presence of mydriasis in III palsy suggests a compressive aetiology in adults, this is not the case in children. However, the frequency of associated CNS abnormalities in III palsy and the risk of tumour in VI palsy can be indications for early neuroimaging depending on presenting features elicited through a careful history and clinical examination. The latter should include the neighbouring cranial nerves. We discuss the impact of our evolving knowledge of congenital cranial dysinnervation syndromes on this field. PMID:25572578

  6. CEREBRAL PALSY AND MUSIC ACHIEVEMENT

    Directory of Open Access Journals (Sweden)

    Miodrag L. STOSHLJEVIKJ

    2008-12-01

    Full Text Available Pupils with cerebral palsy attend elementary education accordind to a regular and special teaching plan and program. Regular school curriculum was reformed in 1992, while special plan and program has not been changed and adapted according to pupil’s needs and capacities. Music is one of the best means of expressing oneself and plays a very important role in the development of every child, the child with cerebral palsy in particular.In order to test the possibility of pupils with cerebral palsy, with and without mental retardation, to apprehend the actual program content, we have conducted research on musical achievement of children with cerebral palsy. During 2007 a research was carried out, on the sample of 27 pupils with cerebral palsy and mild mental retardation who attended classes in the school “Miodrag Matikj”, and a sample of16 students with cerebral palsy without mental retardation who attended the school “Dr. Dragan Hercog” in Belgrade.Results of the research, as well as analysis of music curriculum content, indicated that the capacities of students with cerebral palsy to carry out the curriculum tasks require special approach and methodology. Therefore, we introduced some proposals to overcome the difficulties in fulfilling music curriculum demands of those pupils. We made special emphasis on the use of computer based Assistive technology which facilitates the whole process to a large extent.

  7. 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.)

  8. Bell's Palsy (Beyond the Basics)

    Science.gov (United States)

    ... changes caused by Bell's palsy will affect the appearance of your face, including how you smile. These ... are comfortable with some medical jargon. Patient information: Genital herpes (Beyond the Basics) Patient information: Shingles (Beyond ...

  9. Neurocysticercosis presenting as pseudobulbar palsy

    Directory of Open Access Journals (Sweden)

    Arinaganahalli Subbanna Praveen Kumar

    2014-01-01

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

  10. 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.

  11. Cerebral palsy: classification and etiology

    OpenAIRE

    Bialik, Gad M.; Givon, Uri

    2004-01-01

    Cerebral palsy (CP), a common condition of abnormalities in the brain, arises early in life. Since the term was first introduced in 1843, many authors have tried to define and classify CP. The most recent definition was released by the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) in 2005. This article summarizes the latest and familiar classifications of, and etiologies associated with CP.

  12. 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...

  13. Bone age in cerebral palsy

    OpenAIRE

    Miranda, Eduardo Régis de Alencar Bona; Palmieri, Maurício D'arc; de Assumpção, Rodrigo Montezuma César; Yamada, Helder Henzo; Rancan, Daniela Regina; Fucs, Patrícia Maria de Moraes Barros

    2013-01-01

    Objective To compare the chronological age and bone age among cerebral palsy patients in the outpatient clinic and its correlation with the type of neurological involvement, gender and functional status. Methods 401 patients with spastic cerebral palsy, and ages ranging from three months to 20 years old, submitted to radiological examination for bone age and analyzed by two independent observers according Greulich & Pyle. Results In the topographic distribution, there was a significant delay (p

  14. Left vocal cord paralysis caused by coalworkers' pneumoconiosis and progressive massive fibrosis.

    OpenAIRE

    Haffar, M.; Banks, J.

    1988-01-01

    We report a case of recurrent laryngeal nerve palsy caused by coalworkers' pneumoconiosis with progressive massive fibrosis (PMF). This illustrates that PMF alone may be added to the list of lesions which can cause recurrent laryngeal nerve palsy. However, efforts should always be made to exclude more common causes, in particular bronchogenic carcinoma, before attributing the palsy to PMF.

  15. 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)

  16. MR imaging of cerebral palsy

    International Nuclear Information System (INIS)

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

  17. 中医推拿治疗小儿脑性瘫痪研究现状%Research Progress of Traditional Chinese Medicine Massage in Children with Cerebral Palsy

    Institute of Scientific and Technical Information of China (English)

    王丹蓉(综述); 吴云川(审校)

    2015-01-01

    Cerebral palsy is a common nervous system disease in clinical,and the morbidity is increasing these years,which is one of the major diseases causing disability of children. Traditional Chinese medicine ( TCM) massage has a very unique advantage on children with cerebral palsy because of low price,conven-ience,and safety. TCM massage has an obvious improving effect on the motor development,respiratory sys-tem,digestive system, five sense organs′ function of children with cerebral palsy. However currently most research on the effects of the TCM massage on children with cerebral palsy still focuses on the influence on the patients′motion system and its evaluating system is defective. Besides,study on the standardization of the differentiation therapy of the massage is also a development direction in the future.%小儿脑性瘫痪(脑瘫)是临床上常见的一种神经系统疾病,也是小儿致残的重要疾病之一,且发病率呈上升趋势。中医推拿在治疗小儿脑瘫方面独具优势,经济、简便、安全,对脑瘫患儿的运动发育、呼吸系统、消化系统、五官功能等可起到明显的改善作用。但目前中医推拿治疗小儿脑瘫的研究还是多集中在对脑瘫患儿运动系统的影响,且评定系统不够完善。另外,中医推拿对小儿脑瘫的辩证施术的规范化研究,也是今后的拓展方向。

  18. Monocular Elevation Deficiency - Double Elevator Palsy

    Science.gov (United States)

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

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

    Science.gov (United States)

    ... A clue to what is going wrong with tau protein is that most of the tau protein in the neurofibrillary tangles of PSP are of ... the gene on chromosome 17 that encodes the tau protein is more common in PSP than in the ...

  20. Genetics Home Reference: progressive supranuclear palsy

    Science.gov (United States)

    ... appear to be related to abnormalities in the tau protein. In people with MAPT gene mutations, genetic changes ... affected individuals without MAPT gene mutations. The defective tau protein assembles into abnormal clumps within neurons and other ...

  1. Korioamnionitis sebagai Faktor Risiko Terjadinya Palsi Serebral

    OpenAIRE

    Lubis, Siska Mayasari

    2010-01-01

    Cerebral palsy affects motion, muscle strength, balance, and coordination. Despite improvements in perinatal medicine, the prevalence of cerebral palsy has increased over the last 2 decades, and the etiology of cerebral palsy remains poorly understood. Evidence suggests that 70-80% of cases are due to prenatal factors, and that birth asphyxia plays a relatively minor role. A number of studies have assessed the relationship between chorioamnionitis and cerebral palsy in infants. Multicenter co...

  2. Isolated trochlear nerve palsy with midbrain hemorrhage

    Directory of Open Access Journals (Sweden)

    Raghavendra S

    2010-01-01

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

  3. 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…

  4. Clinically relevant copy number variations detected in cerebral palsy

    OpenAIRE

    Oskoui, Maryam; Gazzellone, Matthew J.; Thiruvahindrapuram, Bhooma; Zarrei, Mehdi; Andersen, John; Wei, John; Wang, Zhuozhi; Wintle, Richard F; Marshall, Christian R.; Cohn, Ronald D; Weksberg, Rosanna; Stavropoulos, Dimitri J; Fehlings, Darcy; Shevell, Michael I.; Stephen W Scherer

    2015-01-01

    Cerebral palsy (CP) represents a group of non-progressive clinically heterogeneous disorders that are characterized by motor impairment and early age of onset, frequently accompanied by co-morbidities. The cause of CP has historically been attributed to environmental stressors resulting in brain damage. While genetic risk factors are also implicated, guidelines for diagnostic assessment of CP do not recommend for routine genetic testing. Given numerous reports of aetiologic copy number variat...

  5. Disease: H00841 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available ated with sensorineural deafness. The same clinical presentation without deafness is also known as Fazio Lon...ou A, Vagiakou EA Infantile progressive bulbar palsy with deafness. Brain Dev 24:732-5 (2002) PMID:21110228 ...n Laere syndrome (BVVLS) is characterized by progressive pontobulbar palsy associ

  6. Peripheral androgen receptor gene suppression rescues disease in mouse models of spinal and bulbar muscular atrophy.

    Science.gov (United States)

    Lieberman, Andrew P; Yu, Zhigang; Murray, Sue; Peralta, Raechel; Low, Audrey; Guo, Shuling; Yu, Xing Xian; Cortes, Constanza J; Bennett, C Frank; Monia, Brett P; La Spada, Albert R; Hung, Gene

    2014-05-01

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

  7. MiR-298 Counteracts Mutant Androgen Receptor Toxicity in Spinal and Bulbar Muscular Atrophy.

    Science.gov (United States)

    Pourshafie, Naemeh; Lee, Philip R; Chen, Ke-Lian; Harmison, George G; Bott, Laura C; Katsuno, Masahisa; Sobue, Gen; Burnett, Barrington G; Fischbeck, Kenneth H; Rinaldi, Carlo

    2016-05-01

    Spinal and bulbar muscular atrophy (SBMA) is a currently untreatable adult-onset neuromuscular disease caused by expansion of a polyglutamine repeat in the androgen receptor (AR). In SBMA, as in other polyglutamine diseases, a toxic gain of function in the mutant protein is an important factor in the disease mechanism; therefore, reducing the mutant protein holds promise as an effective treatment strategy. In this work, we evaluated a microRNA (miRNA) to reduce AR expression. From a list of predicted miRNAs that target human AR, we selected microRNA-298 (miR-298) for its ability to downregulate AR mRNA and protein levels when transfected in cells overexpressing wild-type and mutant AR and in SBMA patient-derived fibroblasts. We showed that miR-298 directly binds to the 3'-untranslated region of the human AR transcript, and counteracts AR toxicity in vitro. Intravenous delivery of miR-298 with adeno-associated virus serotype 9 vector resulted in efficient transduction of muscle and spinal cord and amelioration of the disease phenotype in SBMA mice. Our findings support the development of miRNAs as a therapeutic strategy for SBMA and other neurodegenerative disorders caused by toxic proteins.

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

    Directory of Open Access Journals (Sweden)

    Andrew P. Lieberman

    2014-05-01

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

  9. Goblet cells of the normal human bulbar conjunctiva and their assessment by impression cytology sampling.

    Science.gov (United States)

    Doughty, Michael J

    2012-07-01

    Goblet cells of the conjunctiva are the main source of mucus for the ocular surface. The objectives of this review are to consider the goblet cells as assessed by various histological, cytological and electron microscopy methods, and to assess the consistency of published reports (over more than 25 years) of goblet cell density (GCD) from impression cytology specimens from nominally healthy human subjects. Reported GCD values have been notably variable, with a range from 24 to 2226 cells/mm² for average values. Data analysis suggests that a high density of goblet cells should be expected for the healthy human conjunctiva, with a tendency toward higher values in samples taken from normally covered locations (inferior and superior bulbar conjunctiva) of the open eye (at 973 +/- 789 cells/ mm²) than in samples taken from exposed (interpalpebral) locations (at 427 +/- 376 cells/mm²). No obvious change in GCD was found with respect to age, perhaps because the variability of the data did not allow detection of any age-related decline in GCD. Analyses of published data from 33 other sources indicated a trend for GCD to be lower than normal across a spectrum of ocular surface diseases.

  10. Neurophysiologic findings in children with spastic cerebral palsy

    Directory of Open Access Journals (Sweden)

    Ruchi Kothari

    2010-01-01

    Full Text Available Context : Cerebral palsy (CP is a heterogeneous group of permanent, non-progressive motor disorders of movement and posture caused by chronic brain injuries. It is the most common cause of physical disability in childhood; spastic cerebral palsy being the most prevalent of its various forms. There is scanty information about the neurophysiologic investigations in children diagnosed as having spastic CP. Aims : The aim of the study was to investigate the relationship between abnormal VEP and BAEP findings with different clinical parameters in children with spastic cerebral palsy. Materials and Methods : Fifteen children with spastic CP in the age range 4 months to 10 years participated in this study. Evaluation of VEPs, brainstem evoked potentials (BAEPs were performed in all study patients as well as 35 healthy children as controls. The study was conducted after obtaining ethics committee approval and informed consent of parents. Statistical Analysis Used : Significance of difference in the mean values of different parameters in different groups was assessed by Student′s "t" test and the P value <0.05 was considered to be significant. All the values were expressed as mean ± 1 Std. Deviation. Results : A significant difference was found in the VEP latencies and amplitude between the subjects with CP and controls. Striking BAEP abnormalities in CP patients include prolongation of absolute latency of wave V, interpeak latencies of III-V and lowered I-V ratio. Abnormal VEPs and BAEPs in children with bilateral spastic cerebral palsy demonstrated a correlation with the presence of moderate to severe developmental delay. Conclusions : The differences in VEPs and BAEPs were determined between CP children and healthy children. The abnormalities found are probably linked to the neurological deficits present in cases of cerebral palsy.

  11. 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...

  12. CEREBRAL PALSY AND MUSIC ACHIEVEMENT

    OpenAIRE

    Miodrag L. STOSHLJEVIKJ; EMINOVIKJ Fadilj N.; NIKIKJ Radmila M.; Gordana I. ACHIKJ; Sanela R. PACIKJ

    2015-01-01

    Pupils with cerebral palsy attend elementary education accordind to a regular and special teaching plan and program. Regular school curriculum was reformed in 1992, while special plan and program has not been changed and adapted according to pupil’s needs and capacities. Music is one of the best means of expressing oneself and plays a very important role in the development of every child, the child with cerebral palsy in particular.In order to test the possibility of pupils with cerebral pal...

  13. Contemporary management of Bell palsy.

    Science.gov (United States)

    Jowett, Nate; Hadlock, Tessa A

    2015-04-01

    Bell palsy (BP) is the most common diagnosis in acute and chronic facial palsy. Although most patients fully recover, more than one-quarter will have residual dysfunction. Of these, nearly half will demonstrate severe limitations in facial expression. Though significant attention has been paid to acute management and prognosis, a paucity of literature exists addressing management of the long-term sequelae of BP. This article describes contemporary use of physical therapy, injectables, and static and dynamic surgical procedures in facial reanimation of acute and chronic BP. PMID:25958893

  14. 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

  15. Embodying Investigations of Cerebral Palsy

    DEFF Research Database (Denmark)

    Martiny, Kristian Møller Moltke

    The main question of Kristian Martiny’s dissertation is: how do we help persons living with the brain damage, cerebral palsy (CP)? This question is as complex and difficult to answer as any healthcare question. Martiny argues that we need to ‘open up’ how we do ( cognitive ) science in order...

  16. 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.

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

    Science.gov (United States)

    Gilra, Aditya; Bhalla, Upinder S

    2015-01-01

    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.

  18. Clearance of the mutant androgen receptor in motoneuronal models of spinal and bulbar muscular atrophy.

    Science.gov (United States)

    Rusmini, Paola; Crippa, Valeria; Giorgetti, Elisa; Boncoraglio, Alessandra; Cristofani, Riccardo; Carra, Serena; Poletti, Angelo

    2013-11-01

    Spinal and bulbar muscular atrophy (SBMA) is an X-linked motoneuron disease caused by an abnormal expansion of a tandem CAG repeat in exon 1 of the androgen receptor (AR) gene that results in an abnormally long polyglutamine tract (polyQ) in the AR protein. As a result, the mutant AR (ARpolyQ) misfolds, forming cytoplasmic and nuclear aggregates in the affected neurons. Neurotoxicity only appears to be associated with the formation of nuclear aggregates. Thus, improved ARpolyQ cytoplasmic clearance, which indirectly decreases ARpolyQ nuclear accumulation, has beneficial effects on affected motoneurons. In addition, increased ARpolyQ clearance contributes to maintenance of motoneuron proteostasis and viability, preventing the blockage of the proteasome and autophagy pathways that might play a role in the neuropathy in SBMA. The expression of heat shock protein B8 (HspB8), a member of the small heat shock protein family, is highly induced in surviving motoneurons of patients affected by motoneuron diseases, where it seems to participate in the stress response aimed at cell protection. We report here that HspB8 facilitates the autophagic removal of misfolded aggregating species of ARpolyQ. In addition, though HspB8 does not influence p62 and LC3 (two key autophagic molecules) expression, it does prevent p62 bodies formation, and restores the normal autophagic flux in these cells. Interestingly, trehalose, a well-known autophagy stimulator, induces HspB8 expression, suggesting that HspB8 might act as one of the molecular mediators of the proautophagic activity of trehalose. Collectively, these data support the hypothesis that treatments aimed at restoring a normal autophagic flux that result in the more efficient clearance of mutant ARpolyQ might produce beneficial effects in SBMA patients.

  19. A型肉毒毒素治疗痉挛型脑瘫的研究进展%Research progress of botulinum toxin type A in treatment of spastic cerebral palsy

    Institute of Scientific and Technical Information of China (English)

    刘春明(综述); 刘芸(审校)

    2015-01-01

    Cerebral palsy refers to brain injury caused by various reasons from before birth to 1 month after birth or movement disorders and abnormal posture caused by brain developmental defects. Children cerebral palsy is divided into 5 kinds in the clinic:spastic type, dyskinetic type, ataxia type, muscle hypotonia type and mixed type. Among them, the spastic type accounts for 60% ~70% , and is the main type which causes children physical disabilities. Once diagnosed, the comprehensive treatment method is ap-plied, including functional training, physical therapy, acupuncture, massage, medicine treatment, and so on. In recent years, the lo-cal injection of botulinum toxin A is used for treating the children with spastic cerebral palsy. A large number of clinical studies show that the drug is safe and effective; however, in the course of treatment, it should strictly control the drug indications and contraindica-tions. Especially, the dosage choice and the accurate localization of target muscle is the key factor to influence the treatment effect.%脑瘫是指出生前到出生后1个月内各种原因所引起的脑损伤或发育缺陷所致的运动障碍及姿势异常。小儿脑瘫临床上分为痉挛型,不随意运动型,共济失调型,肌张力低下型及混合型5种,其中,痉挛型占60%~70%,是造成儿童肢体残疾的最主要脑瘫类型。脑瘫一旦确诊多采用综合的治疗方法包括功能训练,物理疗法,中医针刺,按摩,药物治疗等。近年来,临床上开始广泛使用毒杆菌毒素 A(botuli-num toxin A,BTX-A)局部注射治疗痉挛型脑瘫儿童,大量临床研究结果显示该药安全、有效,但在治疗过程中应该严格掌握使用该药的适应证,禁忌证,而剂量的选择和靶肌的准确定位是影响治疗效果的关键因素。

  20. Temperament of premature infants with cerebral palsy

    OpenAIRE

    Ryu, Hyo Jeong; Don Kim, Kyoung

    2015-01-01

    [Purpose] The purpose of this study was to examine the infant temperaments of children with cerebral palsy due to premature birth. [Subjects and Methods] Data were collected through questionnaires sent to 118 mothers of infants diagnosed with cerebral palsy due to premature birth. [Results] Different infant temperament scores were obtained according to the degrees of disability, type of palsy, birth weights, gestational age, and periods of hospitalization in an NICU; however, the differences ...

  1. Parental age, genetic mutation, and cerebral palsy.

    OpenAIRE

    Fletcher, N A; Foley, J

    1993-01-01

    Parental age and birth order were studied in 251 patients with cerebral palsy. No parental age or birth order effects were observed in spastic quadriplegia or diplegia, but a paternal age effect was detected in those with athetoid/dystonic cerebral palsy and congenital hemiplegia. These observations indicate that some cases of athetoid/dystonic or hemiplegic cerebral palsy might arise by fresh dominant genetic mutation.

  2. Cerebral palsy in very low birthweight infants.

    OpenAIRE

    Cooke, R W

    1990-01-01

    Eighty one very low birthweight survivors with cerebral palsy were matched with controls by sex, gestational age, and place of birth. Using discriminant analysis, the perinatal profiles for infants with cerebral palsy and their controls were shown to differ significantly. When infants with various types of cerebral palsy were analysed with their controls the discriminating variables differed. Diplegic infants could be differentiated from controls on antenatal variables alone, but significant ...

  3. Evaluation measures for children with cerebral palsy

    OpenAIRE

    Sršen, Katja Groleger

    2013-01-01

    Abstract. Cerebral palsy is a well-recognized neurodevelopmental condition. The most recentdefinition describes cerebral palsy as a group of disorders of movement andposture, causing activity limitation. An important step in the process of(re)habilitation is evaluation of functional abilities of an individual. To beas accurate as possible in the evaluation of functioning, proper measurementinstruments have to be used. There are many different measurement tools forchildren with cerebral palsy,...

  4. Epilepsy in children with cerebral palsy

    OpenAIRE

    Bruck Isac; Antoniuk Sérgio Antônio; Spessatto Adriane; Bem Ricardo Schmitt de; Hausberger Romeu; Pacheco Carlos Gustavo

    2001-01-01

    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 w...

  5. The clinical and electrophysiological features of patients with spinal and bulbar muscular atrophy%脊髓延髓肌萎缩症临床及电生理特点分析

    Institute of Scientific and Technical Information of China (English)

    陈海; 笪宇威; 李韵; 张新卿; 贾建平

    2011-01-01

    Objective To analyze the clinical features of 5 Chinese patients with spinal and bulbar muscular atrophy ( SBMA ), a sex-linked inheritance disorder. Methods We collected the clinical data of 5 SBMA patients whose diagnosis were confirmed by gene examination to analyze their clinical features, as well as their serum levels of sex hormones, CSF, biochemical indicators and electromyogram. Results Patients with spinal and bulbar and muscular atrophy tend to have an adult onset, exhibiting a slow progression of lower motor neuronal weakness and atrophy involving limbs and bulbar zones. There was mild motor functional lesion. Part of the invalids presented signs of androgen insensitivity such as gynecomastia, the level of testosterone were increased. CAG were 43-51 ( mean 47. 2 ±3. 6). The degrees of creatine kinase( 481. 8 ±264. 8 IU/L ) were increased mildly. CSF were normal. Electromyogram had generally changes derived from lower motor neuron. Conclusions Basically, the clinical features of Chinese SBMA patients presents adult onset, slow progression of lower motor neuronal weakness and atrophy involving limbs and bulbar zones.%目的 分析5例脊髓延髓肌萎缩症患者的临床特征,以便临床医生对该病的认识.方法 收集基因确诊的5例脊髓延髓肌萎缩症患者的临床资料,分析其临床特点及血清性激素、各生化指标水平、脑脊液及肌电图特点.结果 脊髓延髓肌萎缩症患者青年发病,病情进展缓慢.神经系统表现为以肢体近端和延髓部受累为主的瘫痪.舌肌受累较早,运动功能损害较轻.血清睾酮(969.3±234.9ng/dl)、雌二醇(57.1±5.3pg/ml)水平增高,男性乳腺发育出现在病史较长的患者.三核苷酸(CAG)重复序列数目43~51(平均47.2±3.6pg/ml).患者的肌酸激酶(CK,481.8±264.8 IU/L)均增高,脑脊液检查均正常.肌电图为广泛神经源性损害.结论 脊髓延髓肌萎缩症患者的早期症状不典型,易误诊,临床特征为青年起

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

    DEFF Research Database (Denmark)

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

    2012-01-01

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

  7. Treatment of Infantile Cerebral Palsy by Scalp Acupuncture Combined with Point Injection in 48 Cases

    Institute of Scientific and Technical Information of China (English)

    SU Li-min; XIANG Li-min

    2003-01-01

    The author treated forty-eight cases of infantile cerebral palsy with scalp acupuncture and point injection. After two courses of treatment the infants made progress in different degrees. Eight cases were cured,sixteen cases were obviously improved, twenty-two cases were improved and two cases were unchanged. The total effective rate was 95.8%.

  8. 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.

  9. 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...

  10. Microsurgical Urethroplasty for Complex Bulbar Urethral Strictures Using the Radial Forearm Free Flap Prelaminated with Buccal Mucosa.

    Science.gov (United States)

    Chauhan, Ajay; Sham, Eric; Chee, Justin

    2016-06-01

    Background Complex bulbar urethral strictures are a heterogeneous group, including those secondary to radiotherapy, failed previous open urethroplasty, and total bulbar necrosis following pelvic trauma. Traditional urethroplasty techniques in this group are unpredictable. We describe a novel technique of a buccal mucosa-prelaminated radial forearm free flap urethroplasty, which seeks to improve the quality of life for this group of patients. Methods Known, reliable techniques from two surgical specialties were combined to create a novel surgical solution, consisting of a radial forearm free flap prelaminated with buccal mucosa. Prospective data were collected on patient and stricture characteristics, complications, and results, including voiding flow rates, urethrography, and cystourethroscopy. Success was defined as the ability to void per urethra. The procedure was performed in four patients, previously considered unreconstructable and who were suprapubic catheter dependent. Results Microsurgical transfer was successful in all four cases. All patients were voiding per urethra and remained catheter free at a minimum of 12-month follow-up. There was no significant donor morbidity and all patients were able to return to their usual occupation. Mean voiding flow rates were 17.3 mL/s. Flexible cystoscopy revealed well-vascularized, patent neomucosa. Conclusions We demonstrate proof of concept for a novel technique of microsurgical urethroplasty. We believe this technique may have widespread application in the treatment of radiation-induced and other complex urethral strictures where traditional urethroplasty has limited success. PMID:26848566

  11. Cerebral Palsy: A Dental Update

    OpenAIRE

    Sehrawat, Nidhi; Marwaha, Mohita; Bansal, Kalpana; Chopra, Radhika

    2014-01-01

    ABSTRACT Special and medically compromised patients present a unique population that challenges the dentist’s skill and knowledge. Providing oral care to people with cerebral palsy (CP) requires adaptation of the skills we use everyday. In fact, most people with mild or moderate forms of CP can be treated successfully in the general practice setting. This article is to review various dental considerations and management of a CP patient. How to cite this article: Sehrawat N, Marwaha M, Bansal ...

  12. Baclofen in Spastic Cerebral Palsy

    OpenAIRE

    J Akhundian

    2002-01-01

    To evaluate the effect of oral baclofen in spastic cerebral palsy (cp), we studied 40 children with different clinical types of spastic cp. Half of these children served as control group and the others received oral baclofen. All of them were treated with physiotherapy under equal conditions for 6 weeks. We used two methods, modified Ashworth scale and range of motion for evaluation. At the end of therapy we found a significant improvement in the baclofen group compared to control group. As a...

  13. Cerebral palsy in preterm infants

    OpenAIRE

    Demeši-Drljan Čila; Mikov Aleksandra; Filipović Karmela; Tomašević-Todorović Snežana; Knežević Aleksandar; Krasnik Rastislava

    2016-01-01

    Background/Aim. Cerebral palsy (CP) is one of the leading causes of neurological impairment in childhood. Preterm birth is a significant risk factor in the occurrence of CP. Clinical outcomes may include impairment of gross motor function and intellectual abilities, visual impairment and epilepsy. The aim of this study was to examine the relationships among gestational age, type of CP, functional ability and associated conditions. Methods. The sample size w...

  14. Common questions about Bell palsy.

    Science.gov (United States)

    Albers, Janet R; Tamang, Stephen

    2014-02-01

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

  15. Multiple nerve palsies in beta thalassaemia major.

    OpenAIRE

    Lamabadusuriya, S. P.

    1989-01-01

    A patient with beta thalassaemia major is described who developed a lower motor neurone facial nerve palsy on the left side, together with a phrenic nerve palsy on the same side, during the course of the illness. This complication has not been reported before in haemoglobinopathies.

  16. Assistive technology for people with cerebral palsy

    OpenAIRE

    Zupan, Anton; Jenko, Mojca

    2013-01-01

    Abstract. Assistive technology includes equipment, devicesand software solutions that increase functional capabilities of people withdisabilities and improve the quality of their lives. The article presentsassistive technology for people with cerebral palsy. These are mobility aidsthat enable people with cerebral palsy independent walking. For those whocannot walk, proper seating is very important. People, who cannot propel manualwheelchair, can control electric wheelchair with various contro...

  17. 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.

  18. 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…

  19. Peroneal Nerve Palsy After Cryotherapy.

    Science.gov (United States)

    Collins, K; Storey, M; Peterson, K

    1986-05-01

    In brief: Cryotherapy, a common treatment method for sports injuries, could result in peroneal nerve palsy. In this case a 26-year-old basketball coach who sustained a hamstring strain applied ice circumferentially around his knee on two occasions for one hour each. He subsequently suffered a severe peroneal neuropathy with weakness of the ankle, ankle evertors, and toe dorsiflexors. Electromyographic studies showed axonotmesis three months after the injury. Four months after the injury the patient was still recovering. This case demonstrates the importance of using cryotherapy cautiously. PMID:27442936

  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...

  1. Trends in birth prevalence of cerebral palsy.

    OpenAIRE

    Pharoah, P O; Cooke, T.; Rosenbloom, I; Cooke, R W

    1987-01-01

    A register of children with cerebral palsy born in the period 1966-77 to mothers resident in the Mersey region was compiled from several different data sources. There were 685 cases, with a male:female ratio of 1.4:1. The birth prevalence of cerebral palsy ranged from 1.18 to 1.97 per 1000 live births each year, with a mean of 1.51 per 1000 live births. There was no discernible trend in overall prevalence, but there was a highly significant upward trend in the prevalence of cerebral palsy amo...

  2. 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.

  3. 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.

  4. MR findings of cerebral palsy

    International Nuclear Information System (INIS)

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

  5. 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

  6. 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.

  7. Joubert syndrome labeled as hypotonic cerebral palsy

    OpenAIRE

    Dekair, Lubna H.; Kamel, Hussein; El-Bashir, Haitham O.

    2014-01-01

    Joubert syndrome (JS) is a rare autosomal recessive disorder with cerebellar vermis hypoplasia and complex brainstem malformation. The diagnosis of cases can be difficult as the presentation can be similar to cases of cerebral palsy. We present a case of JS in an 18-month-old girl who presented to pediatric rehabilitation with a diagnosis of hypotonic cerebral palsy and abnormal eye movements. The brain MRI confirmed the typical brain malformations.

  8. Tongue mobility in patients with cerebral palsy

    OpenAIRE

    Živković Zorica; Golubović Slavica

    2012-01-01

    Background/Aim. In children with cerebral palsy speech is a big problem. Speech of these children is more or less understandable, depending on the degree of reduced mobility of articulatory organs. Reduced mobility is affected by inability to control facial grimacing and poor muscle strength when performing targeted movements. The aim of this study was to determine the mobility of tongue in patients with cerebral palsy. Methods. The study included a sample of 34 children - patients with...

  9. Ocular problems in children with cerebral palsy

    OpenAIRE

    Esra Ayhan Tuzcu; Fatmagül Başarslan; Cahide Yılmaz; Seçil Arıca; Nilgün Üstün; Özgür İlhan; Mesut Coşkun; Uğurcan Keskin

    2012-01-01

    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...

  10. PRETERM BIRTH ASSOCIATION WITH CEREBRAL PALSY

    OpenAIRE

    Srinivasa Rao; Vidyullatha; Subbalakshmi

    2015-01-01

    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 wa...

  11. Isolated cranial nerve palsies in multiple sclerosis

    OpenAIRE

    Zadro, Ivana; Barun, Barbara; Habek, Mario; Brinar, Vesna V.

    1997-01-01

    During a 10 year period 24 patients with definite multiple sclerosis with isolated cranial nerve palsies were studied (third and fourth nerve: one patient each, sixth nerve: 12 patients, seventh nerve: three patients, eighth nerve: seven patients), in whom cranial nerve palsies were the presenting sign in 14 and the only clinical sign of an exacerbation in 10 patients. MRI was carried out in 20 patients and substantiated corresponding brainstem lesions in seven patients (...

  12. Surgical management of third nerve palsy

    Directory of Open Access Journals (Sweden)

    Anupam Singh

    2016-01-01

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

  13. Surgical management of third nerve palsy.

    Science.gov (United States)

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

    2016-01-01

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

  14. Surgical management of third nerve palsy

    Science.gov (United States)

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

    2016-01-01

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

  15. 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.

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

    Science.gov (United States)

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

    2005-11-01

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

  17. Cerebral palsy in preterm infants

    Directory of Open Access Journals (Sweden)

    Demeši-Drljan Čila

    2016-01-01

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

  18. 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

  19. 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....

  20. 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…

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

    NARCIS (Netherlands)

    Van Vulpen, L. F.; de Groot, 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) o

  2. Chronic progressive external ophthalmoplegia with inflammatory myopathy.

    Science.gov (United States)

    Chen, Ting; Pu, Chuanqiang; Shi, Qiang; Wang, Qian; Cong, Lu; Liu, Jiexiao; Luo, Hongyu; Fei, Lingna; Tang, Wei; Yu, Shanshan

    2014-01-01

    Chronic progressive external ophthalmoplegia is one of mitochondrial disorders, characterized by ptosis, limitation of eye movement, variably severe bulbar muscle weakness and proximal limb weakness. Chronic progressive external ophthalmoplegia complicated with acquired disease is extremely rare. We report a 44 years old male patient with more than 20 years of chronic progressive bilateral ptosis and limitation of eye movements manifested dysarthria, dysphagia and neck muscle weakness for 3 years. The first muscle biopsy showed red-ragged fibers and cytochrome c oxidase negative fibers as well as inflammatory cells infiltration. Electron microscopy revealed paracrystalline inclusions. Mitochondrial genetic analysis demonstrated a large-scale mtDNA deletion of m.8470_13446del4977. The patient was treated with prednisone. In a three-year follow-up study, the second biopsy was performed. Before the treatment, except bilateral ptosis and external ophthalmopelgia, this patient presented bulbar muscle weakness and neck muscle weakness. After treated with prednisone, the symptoms of dysphagia, dysarthria and neck muscle weakness were significantly improved, and the second biopsy showed only mitochondrial myopathy pathology but the inflammations disappeared. Here, we report a patient with chronic progressive external ophthalmoplegia complicated with inflammatory myopathy and after treated with prednisone as myositis, he had a significant therapeutic effect. PMID:25674260

  3. 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.

  4. Measuring intellectual ability in cerebral palsy: The comparison of three tests and their neuroimaging correlates.

    Science.gov (United States)

    Ballester-Plané, Júlia; Laporta-Hoyos, Olga; Macaya, Alfons; Póo, Pilar; Meléndez-Plumed, Mar; Vázquez, Élida; Delgado, Ignacio; Zubiaurre-Elorza, Leire; Narberhaus, Ana; Toro-Tamargo, Esther; Russi, Maria Eugenia; Tenorio, Violeta; Segarra, Dolors; Pueyo, Roser

    2016-09-01

    Standard intelligence scales require both verbal and manipulative responses, making it difficult to use in cerebral palsy and leading to underestimate their actual performance. This study aims to compare three intelligence tests suitable for the heterogeneity of cerebral palsy in order to identify which one(s) could be more appropriate to use. Forty-four subjects with bilateral dyskinetic cerebral palsy (26 male, mean age 23 years) conducted the Raven's Coloured Progressive Matrices (RCPM), the Peabody Picture Vocabulary Test-3rd (PPVT-III) and the Wechsler Nonverbal Scale of Ability (WNV). Furthermore, a comprehensive neuropsychological battery and magnetic resonance imaging were assessed. The results show that PPVT-III gives limited information on cognitive performance and brain correlates, getting lower intelligence quotient scores. The WNV provides similar outcomes as RCPM, but cases with severe motor impairment were unable to perform it. Finally, the RCPM gives more comprehensive information on cognitive performance, comprising not only visual but also verbal functions. It is also sensitive to the structural state of the brain, being related to basal ganglia, thalamus and white matter areas such as superior longitudinal fasciculus. So, the RCPM may be considered a standardized easy-to-administer tool with great potential in both clinical and research fields of bilateral cerebral palsy. PMID:27262445

  5. 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.

  6. 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.

  7. Recurrent oculomotor palsy due to haemorrhage in pituitary adenoma.

    OpenAIRE

    Mohanty, S.

    1980-01-01

    Haemorrhage in pituitary adenoma is an unusual cause of recurrent oculomotor palsy. Three episodes of right oculomotor palsy are reported from a patient having haemorrhage in pituitary adenoma. Early operation and decompression of haemorrhagic pituitary adenoma should be performed so that vision may be preserved and recovery of oculomotor palsy be assured.

  8. Abducens Palsy Due to Cerebral Venous Sinus Thrombosis in a Patient with Heart Failure

    OpenAIRE

    Cem Özgönül; Osman Melih Ceylan; Fatih Mehmet Mutlu

    2015-01-01

    Cerebral venous sinus thrombosis has a wide spectrum of presentation. The clinical manifestation depends on the location of the thrombus, its rate of progression, and the extent of venous collateralization. In this case report, we present the findings of cerebral venous sinus thrombosis presenting with abducens palsy and papilloedema in a patient with heart failure, an unusual etiology for cerebral venous sinus thrombosis. (Turk J Ophthalmol 2015; 45: 179-181)

  9. CLIVUS METASTASIS PRESENTING AS ISOLATED ABDUCEN S NERVE PALSY – CASE REPORT

    Directory of Open Access Journals (Sweden)

    Chandrashekhar

    2013-10-01

    Full Text Available ABSTRACT: A 50 year old lady with past history of breast carcinoma surgery presented with progressive diplopia of 15 days duration. Examination revealed paresis of right abducens nerve. Though risk factor like Hypertension was present, patient was ordered MRI which showed Clivus and verte bral metastatic foci highly suggestive of metastasis from breast carcinoma. The patient was referred for radiation therapy. Hence, meticulous neuroophthalmic examination and management is necessary to rule out localised metastasis causing isolated abducens nerve palsy.

  10. Design and Validation of Automated Femoral Bone Morphology Measurements in Cerebral Palsy

    OpenAIRE

    Park, Noyeol; Lee, Jehee; Sung, Ki Hyuk; Park, Moon Seok; Koo, Seungbum

    2013-01-01

    Accurate quantification of bone morphology is important for monitoring the progress of bony deformation in patients with cerebral palsy. The purpose of the study was to develop an automatic bone morphology measurement method using one or two radiographs. The study focused on four morphologic measurements—neck-shaft angle, femoral anteversion, shaft bowing angle, and neck length. Fifty-four three-dimensional (3D) geometrical femur models were generated from the computed tomography (CT) of cere...

  11. Perspectives on glenohumeral joint contractures and shoulder dysfunction in children with perinatal brachial plexus palsy.

    Science.gov (United States)

    Gharbaoui, Idris S; Gogola, Gloria R; Aaron, Dorit H; Kozin, Scott H

    2015-01-01

    Shoulder joint deformities continue to be a challenging aspect of treating upper plexus lesions in children with perinatal brachial plexus palsy (PBPP). It is increasingly recognized that PBPP affects the glenohumeral joint specifically, and that abnormal scapulothoracic movements are a compensatory development. The pathophysiology and assessment of glenohumeral joint contractures, the progression of scapular dyskinesia and skeletal dysplasia, and current shoulder imaging techniques are reviewed. PMID:25835253

  12. Analysis of the conformation of the androgen receptor in spinal bulbar muscular atrophy by atomic force microscopy.

    Science.gov (United States)

    Jochum, Tobias; Cato, Andrew C B

    2014-01-01

    Spinal bulbar muscular atrophy (SBMA) (also known as Kennedy's disease) is a motor degenerative disease caused by an amplification of the polyglutamine stretch at the N-terminus of the human androgen receptor (AR). Amplifications larger than 40 glutamine residues are thought to lead to the disease. A characteristic feature of this disease is a ligand-dependent misfolding and aggregation of the mutant receptor that lead to the death of motor neurons. Initially, large cytoplasmic and nuclear aggregates reaching sizes of 6 μm were thought to be the pathogenic agents. Later studies have suggested that oligomeric species with sizes of less than 1 μm that occur prior to the formation of the larger aggregates are the toxic agents. However, there have been disagreements regarding the shape of these oligomers, as most studies have been carried out with peptide fragments of the androgen receptor containing different lengths of polyglutamine stretch. We have isolated the wild-type AR with a polyglutamine stretch of 22 (ARQ22) and a mutant receptor with a stretch of 65 (ARQ65) using a baculovirus system and have analyzed the oligomeric structures formed by these receptors with atomic force microscopy. This method has allowed us to determine the conformations of the full-length wild-type and mutant AR and revealed the conformation of the mutant AR that causes SBMA.

  13. Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy.

    Science.gov (United States)

    Cortes, Constanza J; Ling, Shuo-Chien; Guo, Ling T; Hung, Gene; Tsunemi, Taiji; Ly, Linda; Tokunaga, Seiya; Lopez, Edith; Sopher, Bryce L; Bennett, C Frank; Shelton, G Diane; Cleveland, Don W; La Spada, Albert R

    2014-04-16

    X-linked spinal and bulbar muscular atrophy (SBMA) is characterized by adult-onset muscle weakness and lower motor neuron degeneration. SBMA is caused by CAG-polyglutamine (polyQ) repeat expansions in the androgen receptor (AR) gene. Pathological findings include motor neuron loss, with polyQ-AR accumulation in intranuclear inclusions. SBMA patients exhibit myopathic features, suggesting a role for muscle in disease pathogenesis. To determine the contribution of muscle, we developed a BAC mouse model featuring a floxed first exon to permit cell-type-specific excision of human AR121Q. BAC fxAR121 mice develop systemic and neuromuscular phenotypes, including shortened survival. After validating termination of AR121 expression and full rescue with ubiquitous Cre, we crossed BAC fxAR121 mice with Human Skeletal Actin-Cre mice. Muscle-specific excision prevented weight loss, motor phenotypes, muscle pathology, and motor neuronopathy and dramatically extended survival. Our results reveal a crucial role for muscle expression of polyQ-AR in SBMA and suggest muscle-directed therapies as effective treatments.

  14. "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 con

  15. 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

  16. Using health games for rehabilitation of patients with infantile cerebral palsy

    Science.gov (United States)

    Lee, Wan-Chen; Reyes-Fernández, Miriam C.; Posada-Gómez, Rubén; Juárez-Martínez, Ulises; Martínez-Sibaja, Albino; Alor-Hernández, Giner

    2016-01-01

    [Purpose] The purposes of this study were to evaluate whether the therapeutic games developed by the study team are significantly effective for upper limb rehabilitation of patients with cerebral palsy and to assess the development of the games and the evolution of patients throughout the therapy sessions. [Subjects and Methods] This study demonstrates the results of using therapeutic games in patients with infantile cerebral palsy. The therapies were performed in 30-minute sessions for about 1 to 4 months. This study shows the progress of five children with cerebral palsy during the sessions. The time it took the children on each road and the times required to complete a task were measured. In addition, the level of difficulty of the games was gradually increased at each session. [Results] Results have shown good progress on the accuracy of the movements and an increase in concentration level during the execution of the games, showing an improvement in the patients’ performance by 40–55% faster. [Conclusions] Health games encourage children to comply with therapy. The advantage of the game is that the patient can perform the therapy at home, which could help achieve further progress in patients. PMID:27630417

  17. Using health games for rehabilitation of patients with infantile cerebral palsy.

    Science.gov (United States)

    Lee, Wan-Chen; Reyes-Fernández, Miriam C; Posada-Gómez, Rubén; Juárez-Martínez, Ulises; Martínez-Sibaja, Albino; Alor-Hernández, Giner

    2016-08-01

    [Purpose] The purposes of this study were to evaluate whether the therapeutic games developed by the study team are significantly effective for upper limb rehabilitation of patients with cerebral palsy and to assess the development of the games and the evolution of patients throughout the therapy sessions. [Subjects and Methods] This study demonstrates the results of using therapeutic games in patients with infantile cerebral palsy. The therapies were performed in 30-minute sessions for about 1 to 4 months. This study shows the progress of five children with cerebral palsy during the sessions. The time it took the children on each road and the times required to complete a task were measured. In addition, the level of difficulty of the games was gradually increased at each session. [Results] Results have shown good progress on the accuracy of the movements and an increase in concentration level during the execution of the games, showing an improvement in the patients' performance by 40-55% faster. [Conclusions] Health games encourage children to comply with therapy. The advantage of the game is that the patient can perform the therapy at home, which could help achieve further progress in patients. PMID:27630417

  18. 脑瘫儿童的康复护理探讨%Effect of Rehabilitation Nursing on Cerebral Palsy Children

    Institute of Scientific and Technical Information of China (English)

    石娜娜

    2015-01-01

    目的:探讨脑瘫儿童的康复护理方法。方法对在2013年6月~2014年12月份我院收治的100例住院脑瘫儿童进行康复护理和训练。结果通过对脑瘫儿童进行早期的康复护理,不仅可以提高脑瘫儿童的治疗效果,同时也能极大提高脑瘫儿童的生活自理能力、语言能力及社交能力等。结论科学有效地康复护理可以帮助脑瘫儿童的治疗结果取得的进步。%ObjectiveTo discuss the cerebral palsy children's rehabilitation nursing methods. Methods From June 2013 to December 2014 in our hospital 100 cases of hospitalized children with cerebral palsy implemented rehabilitation care and training.Results Through the study of the early rehabilitation care of cerebral palsy children, not only could improve the effect of the treatment of cerebral palsy children, at the same time also could greatly enhance self-care ability,language ability and social ability.Conclusion Scientific rehabilitation nursing can effectively help the treatment of cerebral palsy children and the results made great progress.

  19. A case of possible paraneoplastic neurological syndrome presenting as multiple cranial nerve palsies associated with gallbladder cancer.

    Science.gov (United States)

    Kaido, Misako; Yuasa, Yoshihito; Yamamoto, Tameyoshi; Munakata, Satoru; Tagawa, Naohiro; Tanaka, Keiko

    2016-09-29

    We report the case of a patient who had developed multiple cranial nerve palsies in the course of possible paraneoplastic neurological syndrome (PNS) associated with gallbladder cancer. Twelve days prior to visiting our hospital, a 69-year-old man began experiencing neurological symptoms, beginning with diplopia and progressing to ptosis of the left palpebra and subsequent complete closure of the eye within 8 days. Results of the initial medical examination indicated paresis of left oculomotor (III) and abducens (VI) nerves. MRI of the brain revealed no focal lesion that could have resulted in compression of the affected nerves, while further examination ruled out diabetes mellitus, infection, vasculitis, and other systemic autoimmune diseases as potential causes. Gadolinium-enhanced MRI revealed high intensity located in the oculomotor nerves, and steroid pulse therapy was performed based on the assumption of inflammatory diseases. Although slight improvement was observed with respect to the left extraocular paresis, subsequent emergence of bilateral facial nerve (VII) palsy, right abducens nerve palsy, and right oculomotor nerve palsy occurred in succession. PET/CT performed under suspicion of PNS, confirmed the presence of gallbladder cancer. Surgical extirpation of the cancer occurred 3 months following the appearance of left oculomotor paralysis, after which the patient underwent postoperative chemotherapy. All cranial nerve palsies resolved within 2 months after the operation, and both cancer and PNS have shown no recurrence for over 5 years. Pathological examination of the resected tumor revealed well-differentiated tubular adenocarcinoma showing some signs of epithelial-mesenchymal transition, typically an indicator of a poor prognosis. Nevertheless, lymph node metastasis did not progress beyond N2, and the cancer was completely removed by lymph node dissection. Therefore, the presence of multiple cranial palsies in this patient led to early detection of

  20. Guillain-Barre Syndrome Presenting With Bilateral Facial Nerve Palsy

    Directory of Open Access Journals (Sweden)

    Soroor INALOO

    2014-01-01

    Full Text Available How to Cite This Article: Inaloo S, Katibeh P. Guillain-Barre Syndrome Presenting With Bilateral Facial Nerve Palsy. Iran J Child Neurol. 2014 Winter;8(1:69-71.ObjectiveThis case study is about an 11-year-old girl with bilateral facial weakness, abnormal taste sensation, and deep tendon reflexes of both knees and ankles were absent. However, the muscle power of the lower and upper extremities across all muscle groups was normal. After 2 days, she developed paresthesia and numbness in the lower extremities. Other neurologic examinations, such as fundoscopic evaluation of the retina were normal with the muscle power of both upper- and lower-extremities intact. A lumbar puncture revealed albumincytological dissociation. EMG and NCV were in favor of Guillain-Barre syndrome, for which IVIG was prescribed and the abnormal sensations in the lower limbs rapidly improved. Bilateral facial diplegia without weakness and paresthesia is a variant of Guillain-Barre syndrome that mostly presents withacute onset, rapid progression with or without limb weakness, paresthesia, and decreased or absent DTR and albumin-cytological dissociation.References:Barbi F, Ariatti A, Funakoshi K, Meacci M, Odaka M, Galassi G. Parvovirus B19 infection antedating Guillain-Barre’ syndrome variant with prominent facial diplegia. J Neurol 2011 Aug; 258(8:1551-2. doi: 10.1007/s00415-011-5949-5. Epub 2011 Feb 15.Yardimci N, Avci AY, Kayhan E, Benli S. Bilateral facial nerve enhancement demonstrated by magnetic resonance imaging in Guillain-Barré syndrome. Neurol Sci 2009 Oct; 30(5:431-3. doi:10.1007/s10072-009-0120-0.Lim TC, Yeo WS, Loke KY, Quek SC. Bilateral facial nerve palsy in Kawasaki disease. Ann Acad Med Singapore 2009; 38(8:737-8.Quintas E, Silva A, Sarmento A. Bilateral facial palsy in a young patient after meningococcal meningitis, associated to herpetic infection. Arq Neuro-Psiquiatr 2009; 67(3a: 712-14.Jain V, Deshmukh A, Gollomp S. Bilateral facial

  1. The hip in cerebral palsy.

    Science.gov (United States)

    Bleck, E E

    1980-01-01

    Orthopedic surgery can alleviate the hip flexion, adduction, and medial rotation deformities of the hip and improve the function and appearance of gait. To accomplish this, however, careful examination and prudence in the operative procedure to avoid overdoing and overcorrecting are important. Orthopedic surgery can prevent subluxation and dislocation of the hip before the age of seven years, and consequently repetitive radiographic examinations of the hip in children who have spastic paralysis of the hip musculature should be a routine procedure. Subluxation and dislocation of the hip, when established, can be successfully treated with orthopedic surgical procedures. Physicians must keep in mind that the spastic paralysis of cerebral palsy originates in the brain, and therefore the spasticity cannot be eliminated. The best that can be done is to weaken or remove some muscles as deforming forces and to achieve compromises for continued function. The goal should be optimal independence for the child and adolescent during development, and freedom from pain with deteriorating function due to degenerative arthritis in the adult. PMID:7360505

  2. Maternal Infections during Pregnancy and Cerebral Palsy

    DEFF Research Database (Denmark)

    Miller, Jessica; Pedersen, Lars Henning; Streja, Elani;

    2013-01-01

    BACKGROUND: Cerebral palsy (CP) is a common motor disability in childhood. We examined the association between maternal infections during pregnancy and the risk of congenital CP in the child. METHODS: Liveborn singletons in Denmark between 1997 and 2003 were identified from the Danish National...... the Danish Cerebral Palsy Registry. Adjusted hazard ratio (HR) and 95% confidence interval (CI) were estimated by Cox proportional hazard models. RESULTS: Of the 440 564 singletons with follow-up data, 840 were diagnosed with congenital CP. Maternal genito-urinary tract infections (HR 2.1, 95% CI 1.4, 3...

  3. Education and employment prospects in cerebral palsy

    DEFF Research Database (Denmark)

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

    2005-01-01

    Parents and paediatric neurologists need information on the long-term social prognosis of children with cerebral palsy (CP). No large population-based study has been performed on this topic. On 31 December 1999, to find predictors in childhood of subsequent education and employment, 819...... participants with CP born between 1965 and 1978 (471 males; mean age 28y 10 mo, SD 4y, range 21 to 35y) in the Danish Cerebral Palsy Registry were compared with 4406 controls without CP born between 1965 and 1978 (2546 males; mean age 28y 10 mo, SD 4y, range 21 to 35y). Diagnostic subtypes of the 819...

  4. 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....

  5. What is cerebral palsy - new advances in treatment of cerebral palsy?

    OpenAIRE

    Perat, Milivoj Veličković

    2013-01-01

    Abstract. Cerebral palsy is the most frequent motorimpairment in childhood. In many countries, its prevalence is predicted toincrease. There are an estimated 15 million people with cerebral palsy aroundthe world and more than half of them are mentally retarded and one third haveepilepsy. It should be seen that it is more than merely a motor disorder. Therecan be problems of development, perceptual-cognitive impairment, social andfunctional problems of daily living, emotional and behavioural p...

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

    Science.gov (United States)

    Giorgetti, Elisa; 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 (or dihydrotestosterone), and the polyQ triggers ARpolyQ misfolding and aggregation in spinal cord motoneurons and muscle cells. In motoneurons, testosterone triggers nuclear toxicity by inducing AR nuclear translocation. Thus, (i) prevention of ARpolyQ nuclear localization, combined with (ii) an increased ARpolyQ cytoplasmic clearance, should reduce its detrimental activity. Using the antiandrogen Bicalutamide (Casodex(®)), which slows down AR activation and nuclear translocation, and the disaccharide trehalose, an autophagy activator, we found that, in motoneurons, the two compounds together reduced ARpolyQ insoluble forms with higher efficiency than that obtained with single treatments. The ARpolyQ clearance was mediated by trehalose-induced autophagy combined with the longer cytoplasmic retention of ARpolyQ bound to Bicalutamide. This allows an increased recognition of misfolded species by the autophagic system prior to their migration into the nucleus. Interestingly, the combinatory use of trehalose and Bicalutamide was also efficient in the removal of insoluble species of AR with a very long polyQ (Q112) tract, which typically aggregates into the cell nuclei. Collectively, these data suggest that the combinatory use of Bicalutamide and trehalose is a novel approach to facilitate ARpolyQ clearance that has to be tested in other cell types target of SBMA (i.e. muscle cells) and in vivo in animal models of SBMA.

  7. Enhanced aggregation of androgen receptor in induced pluripotent stem cell-derived neurons from spinal and bulbar muscular atrophy.

    Science.gov (United States)

    Nihei, Yoshihiro; Ito, Daisuke; Okada, Yohei; Akamatsu, Wado; Yagi, Takuya; Yoshizaki, Takahito; Okano, Hideyuki; Suzuki, Norihiro

    2013-03-22

    Spinal and bulbar muscular atrophy (SBMA) is an X-linked motor neuron disease caused by a CAG repeat expansion in the androgen receptor (AR) gene. Ligand-dependent nuclear accumulation of mutant AR protein is a critical characteristic of the pathogenesis of SBMA. SBMA has been modeled in AR-overexpressing animals, but precisely how the polyglutamine (polyQ) expansion leads to neurodegeneration is unclear. Induced pluripotent stem cells (iPSCs) are a new technology that can be used to model human diseases, study pathogenic mechanisms, and develop novel drugs. We established SBMA patient-derived iPSCs, investigated their cellular biochemical characteristics, and found that SBMA-iPSCs can differentiate into motor neurons. The CAG repeat numbers in the AR gene of SBMA-iPSCs and also in the atrophin-1 gene of iPSCs derived from another polyQ disease, dentato-rubro-pallido-luysian atrophy (DRPLA), remain unchanged during reprogramming, long term passage, and differentiation, indicating that polyQ disease-associated CAG repeats are stable during maintenance of iPSCs. The level of AR expression is up-regulated by neuronal differentiation and treatment with the AR ligand dihydrotestosterone. Filter retardation assays indicated that aggregation of ARs following dihydrotestosterone treatment in neurons derived from SBMA-iPSCs increases significantly compared with neurological control iPSCs, easily recapitulating the pathological feature of mutant ARs in SBMA-iPSCs. This phenomenon was not observed in iPSCs and fibroblasts, thereby showing the neuron-dominant phenotype of this disease. Furthermore, the HSP90 inhibitor 17-allylaminogeldanamycin sharply decreased the level of aggregated AR in neurons derived from SBMA-iPSCs, indicating a potential for discovery and validation of candidate drugs. We found that SBMA-iPSCs possess disease-specific biochemical features and could thus open new avenues of research into not only SBMA, but also other polyglutamine diseases.

  8. 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.

  9. Progress in molecular diagnosis of Charcot-Marie-Tooth-disease type 1 (CMT 1, HMSN I) and hereditary neuropathy with liability to pressure palsies (HNPP) by fluorescence in situ hybridization (FISH)-detection of a potential genetic mosaicism

    Energy Technology Data Exchange (ETDEWEB)

    Bathke, K.; Liehr. T.; Ekici, A. [Institute for Human Genetics, Erlange (Germany)] [and others

    1994-09-01

    We tested 20 CMT 1 patients characterized according to the criteria of the European CMT consortium by Southern hybridization of MspI restricted genomic DNA with probes pVAW409R1, pVAW412Hec and pEW401HE. In 11 of the 20 CMT 1 cases (55%), we observed a duplication in 17q11.2; one patient had a dinucleotide insertion in exon 6 of the PO-gene (5%). One HNPP case had a typical 17p11.2 deletion. Analysis of CA-repeats was performed with primers RM11GT and Mfd41; SSCP-analysis of the PO, PMP22 and Cx32-genes is in progress. FISH was carried out with probe pVAW409R1. 125 interphase nuclei were analyzed for each proband by counting the signals per nucleus. Normal cells show a characteristic distribution of signals: 1 signal in 5.9% of nuclei, 2 in 86.3% and 3 in 7.8%. A duplication is indicated by a shift to 3 signals in more than approximately 60% and 2 in less than 25% of the nuclei. In contrast, the 17p11.2 deletion of the HNPP patient shifts to 82.4% of nuclei with a single hybridization signal versus 14.4% with 2 signals. We detected one case with significantly abnormal distribution of interphase nuclei hybridization signals compared to cultures of normal cells and to those with 17p11.2 duplication or deletion: 3.2% nuclei revealed 1 signal, 48.0% two signals and 48.8% 3 signals, indicating a pathogenic but moderate dosis increase compared to the throughout duplicated cases. FISH with probe pVAW409R1 is a versatile tool to detect the HNPP deletion both in interphase nuclei and in metaphase chromosomes. In CMT 1 disease interphase nuclei are required for FISH analysis due to the small duplication of 1.5 Mbp. In contrast to Southern techniques, FISH is able to detect genetic mosaicism.

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

    Directory of Open Access Journals (Sweden)

    A. R Karimi-Yazdi

    2008-08-01

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

  11. The therapy with the large dosage of methylprednisolone for the Bell palsy%大剂量甲基强的松龙冲击治疗面神经炎

    Institute of Scientific and Technical Information of China (English)

    魏新敏; 陆正齐; 蓝瑞琼

    2002-01-01

    @@ Background: The main pathological impairments include edema of facial nerve and different level degeneration of myelin sheath or axis cylinder in Bell's palsy. The prognosis of the disease results from severe degree of the disease and whether treatments are timely or not. The therapy with large dosages of Methylprednisolone (MPS) in vein for Bell's palsy, can relieve local edema,improve nerve conduction,avoid progressive impairments of myeline sheath and axis cylinder and promote repairs of myeline sheath.

  12. Prolactinoma presenting with intermittent third nerve palsy.

    OpenAIRE

    Wykes, W N

    1986-01-01

    A patient presented with a painful third nerve palsy. This resolved spontaneously, but recurred several months later. At his second presentation carotid angiography gave normal results, but a high resolution CT scan showed a tumour in the right parasellar region. The serum prolactin was raised at over 22,000 millimicrons/, showing this to be a prolactinoma.

  13. 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...

  14. 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...

  15. Social integration of adults with cerebral palsy

    DEFF Research Database (Denmark)

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

    2006-01-01

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

  16. 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...

  17. Thyroxine Level of Children with Cerebral Palsy

    Institute of Scientific and Technical Information of China (English)

    Zhang Jie

    2000-01-01

    Objective:To investigate the thyroxine level of Children with cerebral palsy so as to understand thd changes of their nevous endocrine. Methods:Radioimmunoassay was applied to 57 Children with cerebral palsy and 108 normal children.The serum level of tridothyronine(T3), thyroxine(T4)free tridothyronine(FT3),free thyroxin(FT4),and thyroid stimulating hormone(TSH) were measured for those children in the moming and and in condition without any food Rsults: (1)Chiidren with cerebral palsy all showed low T3 values.The difference of T3 value between CP children and norrmal children was significant (P<0.001). (2)Results from groups with difference ages:the CP toddler′s age group also showed low T4 and FT4 values The difference of T4 and FT4 values between the toddler′s age CP childrengroup and the toddler′s age normal children group tegted was significant (CP<0.01 for T4, P <0. 05 for FT4): Conclusion:The tlyroxine level of children with cerebral palsy showed lower values compared to normal children, especisly, the low T3 values were significant.

  18. 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.

  19. Rare copy number variation in cerebral palsy

    OpenAIRE

    McMichael, Gai; Girirajan, Santhosh; Moreno-De-Luca, Andres; Gecz, Jozef; Shard, Chloe; Nguyen, Lam Son; Nicholl, Jillian; Gibson, Catherine; Haan, Eric; Eichler, Evan; Martin, Christa Lese; MacLennan, Alastair

    2013-01-01

    Recent studies have established the role of rare copy number variants (CNVs) in several neurological disorders but the contribution of rare CNVs to cerebral palsy (CP) is not known. Fifty Caucasian families having children with CP were studied using two microarray designs. Potentially pathogenic, rare (

  20. Pharmacological treatment in Dyskinetic Cerebral Palsy

    OpenAIRE

    Allori P; Pasquinelli A; Varrella A

    2012-01-01

    Evaluation of the effect of Levo-Sulpiride, Trazodone and combined treatment in 46 subjects affected by pure and mixed forms of Dyskinetic Cerebral Palsy. The results were assessed according to "Neuromotor Disorders Assessment Scale" (Papini et al, 1995, 1998; Allori et al 2006).

  1. 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…

  2. 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…

  3. 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 pop

  4. Cerebral palsy: the first three years.

    Science.gov (United States)

    Hoffer, M M; Koffman, M

    1980-09-01

    The orthopedic surgeion should be an integral part of a medical team for evaluation and treatment of young children with cerebral palsy. Surgical procedures in this first three years of life are usually limited to the adductor releases about the hip. Stretching and plastic splints about the ankle and knee followed by ankle-foot orthoses are frequently effective in correction of deformity.

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

    Directory of Open Access Journals (Sweden)

    Laís Soares Rodrigues

    2014-12-01

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

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

    Science.gov (United States)

    Rodrigues, Lais S; Targa, Adriano D S; Noseda, Ana Carolina D; Aurich, Mariana F; Da Cunha, Cláudio; Lima, Marcelo M S

    2014-01-01

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

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

    Science.gov (United States)

    Rodrigues, Lais S.; Targa, Adriano D. S.; Noseda, Ana Carolina D.; Aurich, Mariana F.; Da Cunha, Cláudio; Lima, Marcelo M. S.

    2014-01-01

    Olfactory and rapid eye movement (REM) sleep deficits are commonly found in untreated subjects with a recent diagnosis of Parkinson’s disease (PD). Additionally, different studies report declines in olfactory performance during a short period of sleep deprivation. Mechanisms underlying these clinical manifestations are poorly understood, and impairment of dopamine (DA) neurotransmission in the olfactory bulb and the nigrostriatal pathway may have important roles in olfaction and REM sleep disturbances. Therefore, we hypothesized that modulation of the dopaminergic D2 receptors in the olfactory bulb could provide a more comprehensive understanding of the olfactory deficits in PD and REM sleep deprivation (REMSD). We decided to investigate the olfactory, neurochemical, and histological alterations generated through the administration of piribedil (a selective D2 agonist) or raclopride (a selective D2 antagonist) within the glomerular layer of the olfactory bulb, in rats subjected to intranigral rotenone and REMSD. Our findings provide evidence of the occurrence of a negative correlation (r = −0.52, P = 0.04) between the number of periglomerular TH-ir neurons and the bulbar levels of DA in the rotenone, but not sham, groups. A significant positive correlation (r = 0.34, P = 0.03) was observed between nigrostriatal DA levels and olfactory discrimination index (DI) for the sham groups, indicating that increased DA levels in the substantia nigra pars compacta (SNpc) are associated with enhanced olfactory discrimination performance. Also, increased levels in bulbar and striatal DA were induced by piribedil in the rotenone control and rotenone REMSD groups, consistent with reductions in the DI. The present evidence reinforce the idea that DA produced by periglomerular neurons, particularly the bulbar dopaminergic D2 receptors, is an essential participant in olfactory discrimination processes, as the SNpc, and the striatum. PMID:25520618

  8. Buccal mucosal graft urethroplasty for proximal bulbar urethral stricture: A revisit of the surgical technique and analysis of eleven consecutive cases

    Directory of Open Access Journals (Sweden)

    Irekpita Eshiobo

    2016-01-01

    Full Text Available Background: Urethral stricture disease is prevalent, and many surgical techniques have been developed to treat it. Currently, urethroplasty for bulbar strictures implies ventral or dorsal stricturotomy and a buccal mucosa graft (BMG patch. Objective: To describe the surgical approach of the ventral patch BMG urethroplasty for proximal bulbar urethral stricture and to analyze 11 consecutive cases for whom the technique was used. Patients and Methods: The diagnosis of urethral stricture was confirmed with a combined retrograde urethrography and micturating cystourethrography. A single team exposed the urethra, harvested, and planted the BMG in the lithotomy position under general anesthesia. The oral preoperative preparation was done with oraldene (hexetidine mouth wash three times daily beginning from the 2nd preoperative day. The buccal mucosa was harvested from the left inner cheek in all the patients. The donor site was left unclosed but packed with wet gauze. Data related to age, preoperative adverse conditions, stricture length, urine culture result, perineal/oral wound complications, postoperative residual urine volume, and duration of hospital stay were recorded. Results: Eleven patients with proximal bulbar urethral stricture had BMG urethroplasty from August 2013 to October 2015. Stricture length ranged from 2 to 5 cm. In six (54% of the men, the stricture resulted from urethritis thereby constituting the most common etiology of urethral stricture in this study. The preoperative adverse conditions were age above 70 in three, diabetes mellitus in two, severe dental caries in one, and recurrent stricture in two. All of them were able to resume reasonable oral intake 72 h postoperatively. One (9.2% had perineal wound infection, while two (18.2% still had mild pain at donor site 4 weeks postoperatively. Ten (90.9% of the 11 patients had <30 ml residual urine volume at 2 months of follow-up. Conclusion: Urethritis is still a common cause of

  9. 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.

  10. 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

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

    OpenAIRE

    A. R Karimi-Yazdi; A Vasheghani-Farahani; Sadeghi, M.; S. M Sadr-Hoseini; A. A Sazgar; M. A Harirchian; N. S Alirezaie

    2008-01-01

    "nDuring last decades many researchers have focused on the conditions associated with Bell's palsy including diabetes mellitus, hypertension, and viral infections. This study was performed to evaluate correlation of diabetes mellitus and Bell's palsy and some relevant features not discussed in the literature in an Iranian population. The presence of diabetes mellitus was evaluated in a total number of 275 subjects (75 patients with Bell's palsy and 200 control subjects). D...

  12. Familial Recurrence of Cerebral Palsy with Multiple Risk Factors

    OpenAIRE

    Lawrence P. Richer; Dower, Nancy A.; Norma Leonard; Chan, Alicia K. J.; Robertson, Charlene M. T.

    2012-01-01

    The recurrence of cerebral palsy in the same family is uncommon. We, however, report on two families with two or more affected siblings. In both families, numerous potential risk factors were identified including environmental, obstetric, and possible maternal effects. We hypothesize that multiple risk factors may lead to the increased risk of recurrence of cerebral palsy in families. Intrinsic and maternal risk factors should be investigated in all cases of cerebral palsy to properly counsel...

  13. Accessibility of mental health care for adults with cerebral palsy

    OpenAIRE

    Pihlaja, Kimmo; Päivärinta, Paula

    2014-01-01

    The purpose of this thesis is to describe the accessibility in mental health care from the point of view of an adult with cerebral palsy. The theoretical framework of this thesis is constructed from the related literature and previous studies closely linked to the topic. Research was done to clarify the concepts of disability, cerebral palsy, and mental health. The research showed cerebral palsy as a multidimensional physical disability which may include different types of accompanying im...

  14. 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.

  15. 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

  16. Proteasome-mediated proteolysis of the polyglutamine-expanded androgen receptor is a late event in spinal and bulbar muscular atrophy (SBMA) pathogenesis.

    Science.gov (United States)

    Heine, Erin M; Berger, Tamar R; Pluciennik, Anna; Orr, Christopher R; Zboray, Lori; Merry, Diane E

    2015-05-15

    Proteolysis of polyglutamine-expanded proteins is thought to be a required step in the pathogenesis of several neurodegenerative diseases. The accepted view for many polyglutamine proteins is that proteolysis of the mutant protein produces a "toxic fragment" that induces neuronal dysfunction and death in a soluble form; toxicity of the fragment is buffered by its incorporation into amyloid-like inclusions. In contrast to this view, we show that, in the polyglutamine disease spinal and bulbar muscular atrophy, proteolysis of the mutant androgen receptor (AR) is a late event. Immunocytochemical and biochemical analyses revealed that the mutant AR aggregates as a full-length protein, becoming proteolyzed to a smaller fragment through a process requiring the proteasome after it is incorporated into intranuclear inclusions. Moreover, the toxicity-predicting conformational antibody 3B5H10 bound to soluble full-length AR species but not to fragment-containing nuclear inclusions. These data suggest that the AR is toxic as a full-length protein, challenging the notion of polyglutamine protein fragment-associated toxicity by redefining the role of AR proteolysis in spinal and bulbar muscular atrophy pathogenesis.

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

    NARCIS (Netherlands)

    Geerdink, Y.A.; Aarts, P.; Geurts, A.C.H.

    2013-01-01

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

  18. Motor Learning Curve and Long-Term Effectiveness of Modified Constraint-Induced Movement Therapy in Children with Unilateral Cerebral Palsy: A Randomized Controlled Trial

    Science.gov (United States)

    Geerdink, Yvonne; Aarts, Pauline; Geurts, Alexander C.

    2013-01-01

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

  19. 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…

  20. 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.

  1. CT findings in patients with cerebral palsy

    International Nuclear Information System (INIS)

    Clinical findings and CT findings in 73 cases of cerebral palsy were studied. The causes of cerebral palsy were presumed to be as follows: abnormal cerebral development (36%), asphyxial delivery (34%), and immature delivery (19%), etc. CT findings were abnormal in 58% of the 73 cases, 83% of the spastic tetraplegia patients and all of the spastic hemiplegia patients showed abnormal CT findings. All the patients with spastic monoplegia presented normal CT findings. In 75% of the spastic hemiplegia cases, the CT abnormalities were due to cerebral parenchymal abnormality such as porencephaly and regional low absorption. In cases of spastic tetraplegia, cerebral parenchymal abnormality was found only in 10%. Cortical atrophy was found only in 15 of the 73 cases, whereas central atrophy was found in 36 cases. (Ueda, J.)

  2. Education and employment prospects in cerebral palsy

    DEFF Research Database (Denmark)

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

    2005-01-01

    Parents and paediatric neurologists need information on the long-term social prognosis of children with cerebral palsy (CP). No large population-based study has been performed on this topic. On 31 December 1999, to find predictors in childhood of subsequent education and employment, 819...... participants with CP born between 1965 and 1978 (471 males; mean age 28y 10 mo, SD 4y, range 21 to 35y) in the Danish Cerebral Palsy Registry were compared with 4406 controls without CP born between 1965 and 1978 (2546 males; mean age 28y 10 mo, SD 4y, range 21 to 35y). Diagnostic subtypes of the 819...... participants with CP were: 31% hemiplegia, 43% diplegia, 18% tetraplegia, and 8% other types. Level of motor impairment with respect to walking ability was: 62% able to walk without assistance, 21% with assistance, and 16% not able to walk (for 1% of study children walking ability was not known). Relevant...

  3. Sociopragmatic skills in children with cerebral palsy

    OpenAIRE

    Kenda, Nataša

    2013-01-01

    Communication is one of the most important means that enables us life in society. My thesis is therefore dedicated to the use of language among people or pragmatics. I am mainly interested in special features in communication and pragmatics in children with cerebral palsy. In the theoretical introduction, I firstly presented the characteristics of communication and the process of language development of children in the early years of age. Special attention is paid to pragmatics, its deve...

  4. 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.

  5. EVALUATION OF NEUROIMAGING IN CEREBRAL PALSY

    OpenAIRE

    S.H. Hasanpour avanji

    2008-01-01

    ObjectiveCerebral palsy (CP), a common static motor neurological disorder of childhood with wide spectrum of underlying etiologies, can be demonstrated with different neuro imaging techniques. We undertook this study to investigate the diagnosis of intracranial lesions in children with CP and its correlation between clinical deficits and neuroradiological findings.Materials and methodsIn this prospective hospital-based study, the data of 120 patients with CP, aged below 18 years, referring to...

  6. Maternal Risk Factors for Congenital Cerebral Palsy

    OpenAIRE

    Streja, Elani

    2012-01-01

    Congenital Cerebral Palsy (CP) is the most common physical disability in children. In spite of major advances in medical technology, the etiology of CP is still not well understood. There is growing evidence that brain damage leading to CP development occurs during pregnancy and that maternal phenotype contributes to this intrauterine environment. We hypothesized that maternal factors such as infections, smoking, comorbidities and genetics can increase the risk of CP in children. Additionally...

  7. Cancer mortality in cerebral palsy in California

    OpenAIRE

    Day, Steven,; Brooks, Jordan; Strauss, David; Shumway, Sharon; Shavelle, Robert; Kush, Scott; Sasco, Annie

    2008-01-01

    Exposure to lifestyle, occupational, and environmental risk factors for cancer are undoubtedly different in cerebral palsy (CP) than in the general population, and these differences and others may result in a specific pattern of cancer mortality in CP. Objective: To study the cancer mortality of CP in California. Study group: 40,482 CP cases (contributing 357,928 person-years) among 210,155 persons having received annual evaluations from the California Department of Developmental Services ove...

  8. Lever arm dysfunction in cerebral palsy gait

    OpenAIRE

    Theologis, Tim

    2013-01-01

    Skeletal structures act as lever arms during walking. Muscle activity and the ground reaction against gravity exert forces on the skeleton, which generate torque (moments) around joints. These lead to the sequence of movements which form normal human gait. Skeletal deformities in cerebral palsy (CP) affect the function of bones as lever arms and compromise gait. Lever arm dysfunction should be carefully considered when contemplating treatment to improve gait in children with CP.

  9. Hand Functioning in Children with Cerebral Palsy

    OpenAIRE

    Arnould, Carlyne; Bleyenheuft, Yannick; Thonnard, Jean-Louis

    2014-01-01

    Brain lesions may disturb hand functioning in children with cerebral palsy (CP), making it difficult or even impossible for them to perform several manual activities. Most conventional treatments for hand dysfunction in CP assume that reducing the hand dysfunctions will improve the capacity to manage activities (i.e., manual ability, MA). The aim of this study was to investigate the directional relationships (direct and indirect pathways) through which hand skills influence MA in children wit...

  10. Epidemiology of Cerebral Palsy in the Netherlands

    OpenAIRE

    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 population prevalence of CP in the Netherlands. A representative Dutch area with 1.2 million inhabitants of which 172,000 were born between 1977 and 1988 was studied. To ascertain the children with CP...

  11. Visual Impairments in Children with Cerebral Palsy

    OpenAIRE

    Alimović, Sonja

    2012-01-01

    Cerebral palsy (CP) is the neurological developmental disorder mainly affecting motor abilities. Considering the high rate of associated impairments even the definition of CP is revised and changed. Visual impairment is one of the most common associated impairment. Unfortunately, it is often unrecognized and considered to be a normal consequence of motor problems. Sense of sight is most important for early child development, motivation, learning through imitation. It is, therefore, indispensa...

  12. Rehabilitation Outcomes of Children with Cerebral Palsy

    OpenAIRE

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

    2014-01-01

    [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,...

  13. Chronic conditions in adults with cerebral palsy

    OpenAIRE

    Peterson, MD; Ryan, JM; Hurvitz, EA; E Mahmoudi

    2015-01-01

    Adults with cerebral palsy (CP) represent a growing population whose health status and healthcare needs are poorly understood.1 Mortality records reveal that death due to ischemic heart disease and cancer is higher among adults with CP;2 however, there have been no national surveillance efforts to track disease risk in this population. We examined estimates of chronic conditions in a population-representative sample of adults with CP.

  14. Kinematic Deviations In Children With Cerebral Palsy

    OpenAIRE

    Sangeux, Morgan; ARMAND, Stéphane

    2015-01-01

    In gait analysis, a large portion of the work consists in finding the underlying causes of the abnormal movement observed during walking. The patient’s kinematics of walking is compared to that of typically developed children and the deviations are further analysed. Over the years, clinicians have observed multiple-joints kinematics deviations that were frequent in children with cerebral palsy and devised gait patterns in order to group patients and support management algorithms. However, the...

  15. Hip and Spine in Cerebral Palsy

    OpenAIRE

    Persson-Bunke, Måns

    2015-01-01

    Abstract Background: Children with cerebral palsy (CP) have an increased risk of scoliosis, contractures including windswept hip deformity (WS), and hip dislocation. In 1994, a follow-up program and registry for children and adolescents with CP (CPUP) was initiated in Sweden to allow the early detection and prevention of hip dislocations and other musculoskeletal deformities. Purpose: To analyze the prevalence of scoliosis and WS in children with CP and to study the effect of CPUP. To e...

  16. Trends of Cerebral Palsy in Rajasthan, India

    OpenAIRE

    Sumeet Goel; Nisha Ojha

    2015-01-01

    The aim of this study was to determine the incidence of etiological factor and clinical features of children with cerebral palsy (CP) in Rajasthan. Five dissertations done in the Post Graduate Department of Pediatrics Ay., National Institute of Ayurveda, Jaipur with diagnosed case of spastic CP, from year 2010 to 2014 were included in the study. Age, sex, etiological factors, clinical classifications, and epidemiological characteristics as well as the problems associated with CP were analysed...

  17. Hand functioning in children with cerebral palsy

    OpenAIRE

    CarlyneArnould; YannickBleyenheuft; Jean-LouisThonnard

    2014-01-01

    Brain lesions may disturb hand functioning in children with cerebral palsy (CP), making it difficult or even impossible for them to perform several manual activities. Most conventional treatments for hand dysfunction in CP assume that reducing the hand dysfunctions will improve the capacity to manage activities (i.e., manual ability, MA). The aim of this study was to investigate the directional relationships (direct and indirect pathways) through which hand skills influence MA in children wit...

  18. 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.

  19. Foot Deformities in Patients with Cerebral Palsy

    OpenAIRE

    E Ameri; A. Yeganeh

    2007-01-01

    Introduction & Objective: In patients with cerebral palsy (CP) the most common presentation is lower extremity deformity specially foot deformity. Inability to ambulation is the one of the most important disabilities, that dependent to the variety of factors such as severity of disease, kind of CP, etc. This study was aimed to assess prevalence of kinds of foot deformity in CP and communication between kind of CP and foot deformity and another hand inability to ambulation.Materials & Methods...

  20. Occupational therapy for children with cerebral palsy.

    OpenAIRE

    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 will be entered in the review. 1) Randomised controlled clinical trial (RCT): An experiment in which investigators randomly allocate eligible people into treatment and control groups. Cross-over tri...

  1. Narrative ability in children with cerebral palsy.

    OpenAIRE

    Holck, Pernille; Dahlgren Sandberg, Annika; 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) children on a narrative task, in order to search for possible underlying causes to the problems in the CP group. The results of the BST for 10 childre...

  2. Birthweight specific trends in cerebral palsy.

    OpenAIRE

    Pharoah, P O; Cooke, T.; Cooke, R W; Rosenbloom, L

    1990-01-01

    A register of infants with cerebral palsy born to mothers resident in the Mersey region from 1967-84 has been maintained using various sources of information. A total of 1056 patients are registered of whom 331 (31%) have hemiplegia or mixed hemiplegia, 236 (22%) have diplegias or mixed diplegia, and 369 (35%) have quadriplegia or mixed quadriplegia. The remainder have dyskinetic or dystonic forms except for seven, who are unclassified. There has been no significant change in the prevalence o...

  3. Dietary Practices in Saudi Cerebral Palsy Children

    OpenAIRE

    Al-Hammad, Nouf S.

    2015-01-01

    Objectives: To determine the dietary practices of Saudi cerebral palsy (CP) children. Methods: A self-administered questionnaire was used to collect the following information from parents of CP children: demographics, main source of dietary information, frequency of main meals, foods/drinks used for main meals and in-between-meals. Results: Parents of 157 CP children participated. Parents were divided into three, while children were divided into two age groups. The main sources of dietary inf...

  4. Cause of long thoracic nerve palsy: a possible dynamic fascial sling cause.

    Science.gov (United States)

    Hester, P; Caborn, D N; Nyland, J

    2000-01-01

    Long thoracic nerve palsy can result from sudden or repetitive external biomechanical forces. This investigation describes a possible dynamic cause from internal forces. Six fresh cadaveric shoulders (3 female, 3 male, 4 left, 2 right) with full range of motion were systematically dissected to evaluate the anatomic course of the long thoracic nerve. In all specimens a tight fascial band of tissue arose from the inferior aspect of the brachial plexus, extended just superior to the middle scalene muscle insertion on the first rib, and presented a digitation that extended to the proximal aspect of the serratus anterior muscle. With progressive manual abduction and external rotation, the long thoracic nerve was found to "bow-string" across the fascial band. Medial and upward migration of the superior most aspect of the scapula was found to further compress the long thoracic nerve. Previous investigations have reported that nerves tolerate a 10% increase in their resting length before a stretch-induced neuropraxia develops. Previous studies postulated that long thoracic nerve palsy resulted from the tethering effect of the scalenus medius muscle as it actively or passively compressed the nerve; however, similar neuromuscular relationships occur in many other anatomic sites without ill effect. We propose that the cause of long thoracic nerve palsy may be this "bow-stringing" phenomenon of the nerve across this tight fascial band. This condition may be further exacerbated with medial and upward migration of the superior aspect of the scapula as is commonly seen with scapulothoracic dyskinesia and fatigue of the scapular stabilizers. Rehabilitation for long thoracic nerve palsy may therefore benefit from special attention to scapulothoracic muscle stabilization. PMID:10717860

  5. Clinical Observation of Combined Acupuncture and Medications in Treating 90 Cases of Facial Palsy

    Institute of Scientific and Technical Information of China (English)

    WU Chun-huan; XIAO Yuan-chun

    2008-01-01

    @@ Peripheral facial palsy, namely Bell palsy, is one ofthe common diseases in the department ofacupuncture. Facial palsy, popularly called wry mouthand eye, is primarily manifested by the motormalfunction of the muscles of the expression on theaffected side. The author adopted combinedacupuncture and medications to treat 90 cases offacial palsy from June 2005 to June 2007, andattained good effects. It is now reported as follows.

  6. 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.

  7. Clinical Profile of Extraocular Muscle Palsy: A Retrospective Study

    Directory of Open Access Journals (Sweden)

    Suman Adhikari, BOptom

    2013-12-01

    Full Text Available Background: The sixth cranial nerve has been found to be the most commonly affected in previous studies of cranial nerve palsies. This study was carried out to determine the most common nerve involved in extraocular muscle (EOM palsies and the most common cause of EOM palsy in Nepal.Methods: The diagnosed cases of third, fourth, or sixth nerve palsy for 10 years (2000-2010 at the B.P. Koirala Lions Center for Ophthalmic Studies outpatient department were included in the study. A retrospective review of patients’ records was performed, and the causes of EOM palsy were grouped as: vascular, trauma, tumor,aneurysm, undetermined, and others. Recovery of the palsy was evaluated by reviewing the records of the patients who were followed up one month after the initial visit.Results: A total of 838 patients was included in the study. The average patient age was 37 years. The sixth nerve was most commonly affected (n=458, 54.65%, and the most common etiology was undetermined (n=408, 48.68%. Among the cases where the cause of palsy was known, the largest number of patients had trauma (n=188, 16.46%.Conclusion: It was concluded that in Nepal, the most commonly affected cranial nerve is the sixth nerve, in accordance with the other studies done in the past in different parts of the world. Most of the cases of cranial nerve palsy were found to have no specific cause and were not associated with diagnosed systemic disease.

  8. Lateral rectus palsy following coronary angiography and percutaneous coronary intervention

    Science.gov (United States)

    Nicholson, Luke; Jones, Ruth; Hughes, David S

    2014-01-01

    We present a rare case of unilateral lateral rectus palsy following an elective coronary angiography and percutaneous coronary intervention in a 78-year-oldwoman. Ophthalmoplegia following coronary angiography is extremely rare and this is the first case of a unilateral lateral rectus palsy following the procedure. PMID:24536054

  9. Chemo-port insertion: A cause of vocal cord palsy.

    Science.gov (United States)

    Alazzawi, Sarmad; Hindi, Khalid; Malik, Ausama; Wee, Chong Aun; Prepageran, Narayanan

    2015-11-01

    We describe extremely rare cases of vocal cord palsy following surgical insertion of a chemo port. Our cohort consisted of patients with cancer who developed hoarseness immediately after central venous line placement for the administration of chemotherapy, with vocal cord palsy confirmed with flexible laryngoscopy. Given the timing, central venous line placement appears to be the most likely cause. PMID:26108861

  10. 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…

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

    Science.gov (United States)

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

    2016-05-15

    Pathogenic mutations in the KIF5A-SPG10 gene, encoding the kinesin HC5A, can be associated with autosomal dominant hereditary spastic paraplegia (ADHSP). It accounts for about 10% of the complicated forms of ADHSP. Peripheral neuropathy, distal upper limb amyotrophy, and cognitive decline are the most common additional clinical features. We examined a 66-year-old Japanese woman manifesting gait disturbance and spastic dysarthria for 6years with positive family history. She showed evidence of upper and lower motor neuron involvement and fasciculations, thus mimicking amyotrophic lateral sclerosis (ALS). Genetic analysis revealed a heterozygous variant in KIF5A (c.484C>T, p.Arg162Trp) in 2 symptomatic members. The mutation was also identified in 4 asymptomatic members, including 2 elderly members aged over 78years. Electromyography in the 2 symptomatic members revealed evidence of lower motor neuron involvement and fasciculation potentials in distal muscles. This report describes the first known Asian family with a KIF5A mutation and broadens the clinical and electrophysiological spectrum associated with KIF5A-SPG10 mutations. Given that our cases showed pseudobulbar palsy, fasciculation and altered penetrance, KIF5A-SPG10 might well be considered as a differential diagnosis of sporadic ALS. PMID:27084214

  12. Dynamic touch is affected in children with cerebral palsy.

    Science.gov (United States)

    Ocarino, Juliana M; Fonseca, Sergio T; Silva, Paula L P; Gonçalves, Gabriela G P; Souza, Thales R; Mancini, Marisa C

    2014-02-01

    Children with developmental disorders such as cerebral palsy have limited opportunities for effortful interactions with objects and tools. The goal of the study was to investigate whether children with cerebral palsy have deficits in their ability to perceive object length by dynamic touch when compared to typically developing children. Fourteen children with typical development and 12 children with cerebral palsy were asked to report the length of hand-held rods after wielding them out of sight. Multilevel regression models indicated that I1 (maximum principal moment of inertia) was a significant predictor of perceived length - LP (pcerebral palsy (group factor) partially explained such variance (p=.002). In addition, accuracy and reliability of the length judgments made by children with cerebral palsy were significantly lower than the typically developing children (p<.05). Theoretical and clinical implications of these results were identified and discussed.

  13. Cerebral Palsy in 1-12 Year Old Children in Southern Iran

    OpenAIRE

    Inaloo, Soroor; Pegah KATIBEH; GHASEMOF, Masroor

    2016-01-01

    Objective Cerebral palsy (CP) is a non-progressive CNS disorder due to an insult to the growing brain, usually occurring in the first two years of life. During the recent years, its etiology has been changed; perinatal and postnatal insults are not considered as its main causes in developed countries any more. The aim of this study was to evaluate the causes of CP in children in southern Iran. Materials & Methods Overall, 200 children with CP aged 1-12 yr old referring to Pediatric Neurology ...

  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 centu

  15. Diagnosis, Treatment, and Prevention of Cerebral Palsy in Near-Term/Term Infants

    OpenAIRE

    O’Shea, T. Michael

    2008-01-01

    Cerebral palsy is the most prevalent cause of persisting motor function impairment. In a majority of cases, the predominant motor abnormality is spasticity; other forms of cerebral palsy include dyskinetic (dystonia or choreoathetosis) and ataxic cerebral palsy. The care of individuals with cerebral palsy should include the provision of a primary care medical home for care coordination and suppor and diagnostic evaluations. Current strategies to decrease the risk of cerebral palsy include int...

  16. Intelligence level in children with cerebral palsy (CP) at YPAC Medan

    OpenAIRE

    Abdul M. Rambe; Amir Syarifuddin; Bistok Saing

    2002-01-01

    Children with cerebral palsy has been investigated at YPAC (Yayasan Pembinaan Anak Cacat / Institute for Crippled Children) Medan to obtain the detailed description of patient’s intelligence level referring to cerebral palsy types and to determine the relationship between palsy types and mental retardation level as well as to relate cerebral palsy types and sex. The study is cross-sectionally conducted involving all cerebral palsy patients listed in registration book of YPAC Policlinic Medan ...

  17. 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.

  18. Gait stability in children with cerebral palsy

    OpenAIRE

    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 children with CP. In doing so, we tested the FPE’s sensitivity to the assumptions needed to calculate this measure, as well as the ability of the FPE to de...

  19. 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.

  20. Etiopathogenesis of lower motor neuron facial palsy: Our experience

    Directory of Open Access Journals (Sweden)

    M Venugopal

    2011-01-01

    Full Text Available Introduction : Facial nerve is the seventh cranial nerve having important functions, and hence its paralysis can lead to a great deal of mechanical impairment and emotional embarrassment. Etiopathogenisis of lower motor neuron facial palsy is still a diagnostic challenge and the literature has shown varying results pertaining to the same. This study was designed to sketch out the prevalence of disease causation and the profile of peripheral facial palsy patients presenting to the ENT department at Government Medical College, Kozhikode. Materials and Methods : A prospective study involving 60 patients with facial nerve palsy, presented during the period November 2006 to October 2008, was undertaken. Detailed analysis of etiopathogenesis, age and sex distribution, severity of palsy, anatomical levels and follow up for 1 year was done. Results : Trauma, both iatrogenic and non-iatrogenic, was the most widespread etiology in our study, followed by Bell′s palsy which is described as the commonest cause in world literature. Majority of the patients belonged to the age group of 31-40 years and there was slight male preponderance Non-iatrogenic facial palsy following road traffic accident was common in young males, while females dominated in infectious palsies. Majority of cases reported with grade III palsy, followed by grade IV. High-resolution computed tomography of temporal bone is exceedingly sensitive in delineating facial canal. Conclusions : Data analysis shows similarity with the existing literature except a novel trend towards amplified incidence of trauma surpassing Bell′s palsy. The need for comprehensive history taking, meticulous clinical examination, judicious investigations and appropriate intervention is substantiated by the study.

  1. 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. PMID:26294586

  2. Novel transcriptional profile in wrist muscles from cerebral palsy patients

    Directory of Open Access Journals (Sweden)

    Subramaniam Shankar

    2009-07-01

    Full Text Available Abstract Background Cerebral palsy (CP is an upper motor neuron disease that results in a progressive movement disorder. Secondary to the neurological insult, muscles from CP patients often become spastic. Spastic muscle is characterized by an increased resistance to stretch, but often develops the further complication of contracture which represents a prominent disability in children with CP. This study's purpose is to characterize alterations of spastic muscle on the transcriptional level. Increased knowledge of spastic muscle may lead to novel therapies to improve the quality of life for children with CP. Method The transcriptional profile of spastic muscles were defined in children with cerebral palsy and compared to control patients using Affymetrix U133A chips. Expression data were verified using quantitative-PCR (QPCR and validated with SDS-PAGE for select genes. Significant genes were determined using a 2 × 2 ANOVA and results required congruence between 3 preprocessing algorithms. Results CP patients clustered independently and 205 genes were significantly altered, covering a range of cellular processes. Placing gene expression in the context of physiological pathways, the results demonstrated that spastic muscle in CP adapts transcriptionally by altering extracellular matrix, fiber type, and myogenic potential. Extracellular matrix adaptations occur primarily in the basal lamina although there is increase in fibrillar collagen components. Fiber type is predominately fast compared to normal muscle as evidenced by contractile gene isoforms and decrease in oxidative metabolic gene transcription, despite a paradoxical increased transcription of slow fiber pathway genes. We also found competing pathways of fiber hypertrophy with an increase in the anabolic IGF1 gene in parallel with a paradoxical increase in myostatin, a gene responsible for stopping muscle growth. We found evidence that excitation-contraction coupling genes are altered in

  3. 小儿脑瘫康复治疗浅析%An analysis of the recovery of infantile cerebral palsy

    Institute of Scientific and Technical Information of China (English)

    吴丽影; 张园园

    2015-01-01

    小儿脑瘫是脑实质的损害,出现非进行性、中枢性运动功能障碍,进而影响小儿生长发育,其康复评定、中医和西医治疗手段丰富,故探讨治疗方法的适用性,为小儿脑瘫康复提供治疗思路。%Children with cerebral palsy is substantial brain damage, appears non-progressive, central motor dysfunction, affecting the growth and development of children. Rehabilitation assessment, TCM and western medicine can provide a method of treating cerebral palsy in children.

  4. 小儿脑瘫康复治疗浅析%An analysis of the recovery of infantile cerebral palsy

    Institute of Scientific and Technical Information of China (English)

    吴丽影; 张园园

    2015-01-01

    Children with cerebral palsy is substantial brain damage, appears non-progressive, central motor dysfunction, affecting the growth and development of children. Rehabilitation assessment, TCM and western medicine can provide a method of treating cerebral palsy in children.%小儿脑瘫是脑实质的损害,出现非进行性、中枢性运动功能障碍,进而影响小儿生长发育,其康复评定、中医和西医治疗手段丰富,故探讨治疗方法的适用性,为小儿脑瘫康复提供治疗思路。

  5. Evaluation of facial palsy by moire topography

    Science.gov (United States)

    Inokuchi, Ikuo; Kawakami, Shinichiro; Maeta, Manabu; Masuda, Yu

    1991-08-01

    Society of Facial Research is used frequently. It is of great value clinically, but the method has several weak points concerning objective and quantitative assessment. This study uses moire topography to solve these problems. mA moire camera, FM3013, of the lattice irradiation type was used for measurement of the face. Five moire photographs were taken: at rest, wrinkling the forehead, closing the eyes lightly, blowing out the cheeks and grinning. The degree of facial palsy was determined by the Asymmetry Index (AI) as a measure of the degree of facial deviation. Total AI was expressed as the average AI based on calculations of the measurement in 5 photos. Severe paralysis is represented by an AI of more than 20%. Partial paralysis has a range of 20-8%. Nearly normal is judged to be less than 8%. Ten normal individuals are measured as control and show an AI of 3% or less. Moire topography is useful in assessing the recovery process because it has the benefit of making the site and grade of palsy easily achieved by the AI and the deviation in its patterns. The authors propose that the moire method is better for an objective and quantitative evaluation than the society's method.

  6. A case misdiagnosed as bilateral abducens palsy

    International Nuclear Information System (INIS)

    A 66-year-old male was admitted to our neurosurgical floor because of double vision and gait disturbance. Neurological examinations revealed bilateral 6th nerve palsy with both eyes pointing toward the midline. Initially, using a tentative diagnosis of intracranial mass lesions, especially localized at the base of the skull, computerized tomography of the head, cerebral angiography, orbital venography, and metrizamide CT cisternography were performed; the findings were normal. An orbital CT scan showed an enlargement of the bilateral medial rectus muscles, and the thyroid functions of T3 and T4 and the T3 uptake were all elevated, which was compatible with the diagnosis of Graves' disease. The limitations of both eyeballs were considered to be due not to the 6th nerve palsy, but to the hypertrophy of the bilateral medial rectus muscles. We neurosurgeons should recall Graves' disease as well as intracranial lesions, cerebrovascular disease, and post-traumatic sequelae when examining a patient who presents limitations of external ocular movement. (author)

  7. X-linked spinal and bulbar muscular atrophy (Kennedy's disease with long-term electrophysiological evaluation: case report Atrofia muscular bulbo-espinal ligada ao cromossomo X (doença de Kennedy com seguimento eletrofisiológico de longo prazo: relato de caso

    Directory of Open Access Journals (Sweden)

    João Aris Kouyoumdjian

    2005-03-01

    Full Text Available X-linked spinal and bulbar muscular atrophy or Kennedy's disease is an adult-onset motor neuronopathy caused by a CAG repeat expansion within the first exon of an androgen receptor gene. We report the case of a 66-year-old man, previously diagnosed with motor neuron disease (MND, who presented acute and reversible left vocal fold (dysphonia and pharyngeal paresis, followed by a slowly progressive weakness and also bouts of weakness, wasting and fasciculation on tongue, masseter, face, pharyngeal, and some proximal more than distal upper limb muscles, associated to bilateral hand tremor and mild gynecomastia. There were 5 electroneuromyography exams between 1989 and 2003 that revealed chronic reinnervation, some fasciculations (less than clinically observed and rare fibrillation potentials, and slowly progressive sensory nerve action potentials (SNAP abnormality, leading to absent/low amplitude potentials. PCR techniques of DNA analysis showed an abnormal number of CAG repeats, found to be 44 (normal 11-34. Our case revealed an acute and asymmetric clinical presentation related to bulbar motoneurons; low amplitude/absent SNAP with mild asymmetry; a sub-clinical or subtle involvement of proximal/distal muscles of both upper and lower limbs; and a probable evolution with bouts of acute dennervation, followed by an efficient reinnervation.Atrofia muscular bulbo-espinal ligada ao cromossomo X (doença de Kennedy é uma neuronopatia motora em adultos causada por expansões na repetição CAG no gene do receptor andrógeno. Neste relato, descreve-se o caso de homem de 66 anos, com diagnóstico prévio de doença do neurônio motor (DNM que apresentou quadro agudo e reversível de paresia de prega vocal (disfonia e de músculos faríngeos à esquerda; posteriormente seguiram-se surtos de fraqueza lentamente progressiva, atrofia e fasciculações em língua, masseter, face, faringe e membros superiores predominantemente proximal, associada a tremor

  8. ALS伴延髓麻痹24例瞬目反射检测结果分析%Study of application and clinical importance of blink reflex detection in the 24 ALS patients with bulbar palsy

    Institute of Scientific and Technical Information of China (English)

    陈丽华; 叶民; 王蔚; 王杰; 姚朝亚; 丁新生

    2016-01-01

    目的 应用神经电生理检测瞬目反射研究肌萎缩侧索硬化脑干损害情况.方法 临床诊断ALS患者24例,均伴有延髓麻痹表现;健康对照组24例.瞬目反射检测在室温22~28℃时进行.记录电极采用表面皮肤电极,两个记录电极分别置于下眼轮匝肌外侧,参考电极置于颞部.刺激电极用手柄式电极,正负极间距2.5 cm,频率为1 Hz,电流为11~13 mA.分析记录:滤波带通2~10 kHz,分析时间100 ms.分别刺激左右两侧后记录两侧R1、R2、R2'.记录波形以清晰稳定为准,取连续五次的平均波潜伏期和波的峰值为波幅进行统计.组间样本t检验.结果 检测运动神经68条,末端潜伏期(DML)异常26条,占38.2%,运动波幅(dCMAPA)异常22条,占32.3%;检查F波44条,传导速度异常12条,占27.3%;F波出现率异常21条,占47.7%.常规EMG共检查肌肉110块.研究组BR检测48例次,R2潜伏期延长40例,异常率83.3%;R2'潜伏期延长40例,异常率83.3%;R2波幅降低1例,异常率2.1%;R2'波幅降低4例,异常率8.3%.结论 BR检测判断脑干损害的是一项方便、无创的电生理检测项目,可判断ALS患者伴有延髓性麻痹时的脑干损害.

  9. 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.

  10. Lame from birth: early concepts of cerebral palsy.

    Science.gov (United States)

    Obladen, Michael

    2011-02-01

    Deformations have been attributed to supernatural causes since antiquity. Cerebral palsy was associated with God's wrath, witchcraft, the evil eye, or maternal imagination. Greek scholars recommended prevention by tight swaddling, a custom that persisted into modern times. In the Middle Ages, the midwife's negligence was held responsible as was difficult teething. Morgagni described in 1769 that the neonatal brain can liquefy, and Bednar described leukomalacia in 1850 as a distinct disorder of the newborn. In 1861, Little associated cerebral palsies with difficult or protracted labor and neonatal asphyxia, but he was challenged by Freud, who in 1897 declared that most cases are prenatal in origin. In 1868, Virchow demonstrated inflammatory changes, a view recently confirmed by Leviton and Nelson. Although a causal relationship of cerebral palsy to the birth never has been established, the habit to put the blame for cerebral palsy on someone remained a frequent attitude.

  11. 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.

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

    Science.gov (United States)

    ... THIS TOPIC A Parent's Guide to Surviving the Teen Years Understanding Puberty Cerebral Palsy Finding Respite Care for Your Child With Special Needs Marriage Advice for Parents of Children With Special Needs ...

  13. 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...

  14. 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

    2014-08-01

    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, on the basis of 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 nonambulatory children with cerebral palsy and foster long-term changes in physical activity behavior in all children with this condition.

  15. Postural control in sitting children with cerebral palsy

    NARCIS (Netherlands)

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

    1998-01-01

    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 a

  16. 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.

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

    NARCIS (Netherlands)

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

    1996-01-01

    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 p

  18. Acute Peripheral Facial Palsy after Chickenpox: A Rare Association

    Directory of Open Access Journals (Sweden)

    Helena Ferreira

    2014-01-01

    Full Text Available Chickenpox, resulting from primary infection by the varicella-zoster virus, is an exanthematous disease very common during childhood and with good prognosis. However, serious complications, namely, neurological syndromes, may develop during its course, especially in risk groups, including adolescents. Peripheral facial palsy is a rare neurologic complication that has been previously described. Conclusion. We report the case of a teenager with peripheral facial palsy as a complication of chickenpox, aiming to increase the awareness of this rare association.

  19. 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.

  20. 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....

  1. BIOMECHANICAL PRINCIPLES PHYSICAL REHABILITATION OF CHILDREN WITH CEREBRAL PALSY

    OpenAIRE

    S. D. Korshunov; K. V. Davletyarova; L. V. Kapilevich

    2016-01-01

    Aim. We studied the basic biomechanical principles of physical rehabilitation of children with cerebral palsy.Materials and methods. Methods of Motion Tracking and electromyography investigated the biomechanical characteristics of gait in children with cerebral palsy. It is shown that the main differences between dynamic stereotype walk pediatric patients is to delay moving forward center of gravity and the disorganization of the lower limb movements (especially knee) in the vertical plane. P...

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

    OpenAIRE

    Dubey, A.(Variable Energy Cyclotron Centre, Kolkata, India); P A Ghafoor; Rafeeq, M.

    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...

  3. 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...

  4. Management of Spasticity in Children with Cerebral Palsy

    OpenAIRE

    Shamsoddini, Alireza; AMIRSALARI, Susan; Hollisaz, Mohammad-Taghi; Rahimnia, Alireza; Khatibi-Aghda, Amideddin

    2014-01-01

    Cerebral palsy is the most common cause of spasticity and physical disability in children and spasticity is one of the commonest problems in those with neurological disease. The management of spasticity in children with cerebral palsy requires a multidisciplinary effort and should be started as early as possible. There are a number of treatments available for the management of spasticity. This article reviews the variety of options available for the clinical management of spasticity.

  5. Upper Extremity Muscle Endurance in Children with Cerebral Palsy

    OpenAIRE

    Gulliksen, Anette

    2011-01-01

    Aim: The aim of this study was to evaluate whether submaximal muscle endurance at 20% of maximal voluntary isometric contraction (MVIC) is as reduced as muscle strength in elbow flexion in children with cerebral palsy (CP), and whether motor unit recruitment to compensate muscle fatigue is hampered in this group.Methods: Twelve subjects with cerebral palsy and seventeen control subjects performed three MVICs of elbow extension and flexion, and an endurance task holding a load of approximately...

  6. Clinical significance of the corpus callosum in cerebral palsy

    International Nuclear Information System (INIS)

    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

  7. 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,…

  8. Dyslipidaemias and Physical Activity in Children with Cerebral Palsy

    OpenAIRE

    Maciste Habacuc Macías-Cervantes; Martha Susana Arriola-Nuñez; Francisco J. Díaz-Cisneros; Antonio E. Rivera-Cisneros; José María de la Roca-Chiapas; Victoriano Pérez-Vázquez

    2014-01-01

    Cerebral palsy people present movement difficulty and are liable to develop disorders associated with sedentary lifestyles such as dyslipidaemias and cardiovascular diseases. This study aimed to assess physical activity and the prevalence of lipid abnormalities in 29 children with cerebral palsy who were being treated in two care centers in the state of Guanajuato, Mexico. Physical activity was calculated using a survey. Blood glucose, total cholesterol, HDL-C, LDL-C, and triglycerides were d...

  9. 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.

  10. Gait analysis of children with spastic hemiplegic cerebral palsy

    Institute of Scientific and Technical Information of China (English)

    Xin Wang; Yuexi Wang

    2012-01-01

    An experiment was carried out in the key laboratory for Technique Diagnosis and Function Assessment of Winter Sports of China to investigate the differences in gait characteristics between healthy children and children with spastic hemiplegic cerebral palsy. With permission of their parents, 200 healthy children aged 3 to 6 years in the kindergarten of Northeastern University were enrolled in this experiment. Twenty children aged 3 to 6 years with spastic hemiplegic cerebral palsy from Shengjing Hospital, China were also enrolled in this experiment. Standard data were collected by simultaneously recording gait information from two digital cameras.DVracker was used to analyze the standard data. The children with hemiplegic cerebral palsy had a longer gait cycle, slower walking speed, and longer support phase than did the healthy children.The support phase was longer than the swing phase in the children with hemiplegic cerebral palsy. There were significant differences in the angles of the hip, knee, and ankle joint between children with cerebral palsy and healthy children at the moment of touching the ground and buff -ering, and during pedal extension. Children with hemiplegic cerebral palsy had poor motor coordination during walking, which basically resulted in a short stride, high stride frequency to maintain speed, more obvious swing, and poor stability.

  11. Evaluation of postural stability in children with hemiplegic cerebral palsy

    Science.gov (United States)

    Kenis-Coskun, Ozge; Giray, Esra; Eren, Beyhan; Ozkok, Ozlem; Karadag-Saygi, Evrim

    2016-01-01

    [Purpose] Postural stability is the ability of to maintain the position of the body within the support area. This function is affected in cerebral palsy. The aim of the present study was to compare static and dynamic postural stability between children with hemiplegic cerebral palsy and healthy controls. [Subjects and Methods] Thirty-seven children between the ages of 5 and 14 diagnosed with hemiplegic cerebral palsy (19 right, 18 left) and 23 healthy gender- and age-matched controls were included in the study. Postural stability was evaluated in both of the groups using a Neurocom Balance. Sway velocity was measured both with the eyes open and closed. Sit to stand and turning abilities were also assessed. [Results] The sway velocities with the eyes open and closed were significantly different between the groups. The weight transfer time in the Sit to Stand test was also significantly slower in children with cerebral palsy. Children with cerebral palsy also showed slower turning times and greater sway velocities during the Step and Quick Turn test on a force plate compared with their healthy counterparts. [Conclusion] Both static and dynamic postural stability parameters are affected in hemiplegic cerebral palsy. Further research is needed to define rehabilitation interventions to improve these parameters in patients. PMID:27313338

  12. Can global positioning systems quantify participation in cerebral palsy?

    Science.gov (United States)

    Ben-Pazi, Hilla; Barzilay, Yair; Shoval, Noam

    2014-06-01

    This study examined whether motor-related participation could be assessed by global positioning systems in individuals with cerebral palsy. Global positioning systems monitoring devices were given to 2 adolescent girls (14-year-old with diplegic cerebral palsy and her 15-year-old healthy sister). Outcome measures were traveling distances, time spent outdoors, and Children's Assessment of Participation and Enjoyment questionnaires. Global positioning systems documented that the girl with cerebral palsy did not visit nearby friends, spent less time outdoors and traveled shorter distances than her sister (P = .02). Participation questionnaire corroborated that the girl with cerebral palsy performed most activities at home alone. Lower outdoor activity of the girl with cerebral palsy measured by a global positioning system was 29% to 53% of that of her sibling similar to participation questionnaires (44%). Global positioning devices objectively documented low outdoor activity in an adolescent with cerebral palsy compared to her sibling reflecting participation reported by validated questionnaires. Global positioning systems can potentially quantify certain aspects of participation.

  13. 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.

  14. MR imaging in Bell's palsy and herpes zoster opticus: correlation with clinical findings

    International Nuclear Information System (INIS)

    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

  15. Reduced accommodation in children with cerebral palsy.

    Science.gov (United States)

    Leat, S J

    1996-09-01

    Accommodation in 43 subjects with cerebral palsy was measured objectively using a dynamic retinoscopy technique, which has already been shown to be reliable and repeatable. The subject's ages ranged from 3 to 35 years. Of these, 42% were found to have an accommodative response pattern which was different from the normal control group for his/her age. Nearly 29% had an estimated amplitude of accommodation of 4 D or less. The presence of reduced accommodation was found to be associated with reduced visual acuity, but was not associated with cognitive or communication ability, refractive error or age. The prevalence of other ocular disorders in this group is also high. These findings have developmental and educational implications.

  16. 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.

  17. 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.

  18. Acute sciatic neuropathy: "Saturday night palsy"

    Directory of Open Access Journals (Sweden)

    Manigoda Miodrag

    2005-01-01

    Full Text Available This is a case report of 25-year old, unemployed male, admitted to hospital due to acute onset of the left foot drop, subsequent walking difficulty and numbness of the left calf and foot. Symptoms began after prolonged sleep with previous heroin abuse by sniffing. During neurological examination, mild weakness of knee flexors, moderate weakness of plantar flexors and paralysis of foot dorsiflexors, together with hypesthesia of the left calf, foot and fingers, predominantly in the innervation area of common peroneal nerve on the same side, were observed. The electrophysiologic examination revealed predominant involvement of peroneal division within the sciatic nerve, together with recorded conduction block indicating the compression as possible mechanism of nerve injury. The patient was administered corticosteroid therapy during two months, what resulted in almost complete recovery. The peculiarity of this case report is in the presence of the sciatic nerve "Saturday night palsy" with possible effect of former heroin abuse.

  19. A case of Klumpke's obstetric brachial plexus palsy following a Cesarean section.

    Science.gov (United States)

    Al-Qattan, Mohammad M; El-Sayed, Amel A F

    2016-09-01

    It is generally thought that Klumpke's palsy is not seen as obstetric injury. The authors present a case of Klumpke's palsy with Horner syndrome following delivery by emergency Cesarean section. Neurolysis and nerve grafting partially corrected the paralysis. PMID:27648266

  20. Excess Pre-Pregnancy Weight May Slightly Raise Baby's Cerebral Palsy Risk

    Science.gov (United States)

    ... Excess Pre-Pregnancy Weight May Slightly Raise Baby's Cerebral Palsy Risk But, study found overall odds remain quite ... slight increased risk of having a baby with cerebral palsy, a new study suggests. After reviewing data from ...

  1. External anal sphincter electromyography and related clinical aspects in patients with multiple system atrophy, Parkinson's disease and progressive supranuclear palsy%帕金森病、P型多系统萎缩及进行性核上性麻痹的肛门括约肌肌电图及相关自主神经损害特点

    Institute of Scientific and Technical Information of China (English)

    王含; 崔丽英; 杜华; 李本红; 吴双; 管宇宙

    2011-01-01

    Objectives To assess the value of external anal sphincter electromyography (EASEMG) in evaluating the related autonomic dysfunction in Parkinson's disease ( PD), parkinsonism dominant multiple system atrophy (MSA-P) and progressive supranuclear palsy (PSP). Methods From the records of EAS-EMG collected in our lab (total 562 cases), 60 PD (male 41, female 19), 68 MSA-P (male 35,female 33) and 13 PSP (male 10, female 3) were included in the analysis in this study. Mean duration,polyphasic ratio and satellite potential occurrence rate were comparable among the groups. Mean duration prolongation were graded as normal ( < 10.0 ms), mild ( 10.0-11.9 ms), moderate ( 12.0-13.9 ms)and severe ( ≥ 14.0 ms). Results Among all related autonomic symptoms, the occurrence rate of constipation, urinary incontinence, urgency and frequency in patients with MSA-P(95.8% (23/24) ,94.6% (53/56) ,87.7% ( 50/57 ), 85.7% (42/49), 76.5% ( 39/51 ) ) were higher than that of PD ( 61.5%(16/26), 62.3% (33/53), 30.6% (15/49), 46.2% (24/52), 45.7% (21/46)) and PSP (75.0%(3/4) , 62.5% (5/8), 50.0% (4/8), 42.9% (3/7), 42.9% (3/7)). The abnormal rate of EAS-EMG in PD, MSA-P and PSP were 60.0%, 94.2% and 84.6%, accordingly. Mean duration ( PD ( 12.0 ± 1.6)ms, MSA-P (15.4±3.0) ms, PSP (13.8±1.8) ms), polyphasic ratio (PD 46.2% ±19.2%, MSA-P 63.9% ± 15.8%, PSP 51.5% ± 12.1% ) and satellite potential occurrence rate ( PD 9.5% ± 8.3%,MSA-P 26.5% ± 15.9%, PSP 19.2% ± 12.5% ) varied significantly different among the groups ( F =31.724, F = 17.412, x2 =45. 335, all P <0.01 ). Severe mean duration prolongation was overwhelming in MSA-P (66.2% ) , compared with mild 10.3% and moderate 23.5%. The predominant prolongation degree was moderate in PSP (61.5%, mild 7.7%, severe 30.8% ), and mild in PD (36.7%, moderate 36.7% ,severe 11.7%, normal 15.0% ). Conclusions EAS-EMG could play a role in evaluating the related autonomic dysfunctions in PD, MSA-P and PSP. The EAS-EMG impairment was severe and

  2. The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex.

    Science.gov (United States)

    Bott, Laura C; Salomons, Florian A; Maric, Dragan; Liu, Yuhong; Merry, Diane; Fischbeck, Kenneth H; Dantuma, Nico P

    2016-06-17

    Polyglutamine expansion in the androgen receptor (AR) causes spinal and bulbar muscular atrophy (SBMA), an X-linked neuromuscular disease that is fully manifest only in males. It has been suggested that proteins with expanded polyglutamine tracts impair ubiquitin-dependent proteolysis due to their propensity to aggregate, but recent studies indicate that the overall activity of the ubiquitin-proteasome system is preserved in SBMA models. Here we report that AR selectively interferes with the function of the ubiquitin ligase anaphase-promoting complex/cyclosome (APC/C), which, together with its substrate adaptor Cdh1, is critical for cell cycle arrest and neuronal architecture. We show that both wild-type and mutant AR physically interact with the APC/C(Cdh1) complex in a ligand-dependent fashion without being targeted for proteasomal degradation. Inhibition of APC/C(Cdh1) by mutant but not wild-type AR in PC12 cells results in enhanced neurite outgrowth which is typically followed by rapid neurite retraction and mitotic entry. Our data indicate a role of AR in neuronal differentiation through regulation of APC/C(Cdh1) and suggest abnormal cell cycle reactivation as a pathogenic mechanism in SBMA.

  3. p62/SQSTM1 differentially removes the toxic mutant androgen receptor via autophagy and inclusion formation in a spinal and bulbar muscular atrophy mouse model.

    Science.gov (United States)

    Doi, Hideki; Adachi, Hiroaki; Katsuno, Masahisa; Minamiyama, Makoto; Matsumoto, Shinjiro; Kondo, Naohide; Miyazaki, Yu; Iida, Madoka; Tohnai, Genki; Qiang, Qiang; Tanaka, Fumiaki; Yanagawa, Toru; Warabi, Eiji; Ishii, Tetsuro; Sobue, Gen

    2013-05-01

    Polyglutamine (polyQ) diseases are inherited neurodegenerative disorders that are caused by the expansion of trinucleotide CAG repeats in the causative genes. Spinal and bulbar muscular atrophy (SBMA) is an inherited motor neuron disease that is caused by the expansion of a polyQ tract within the androgen receptor (AR). p62 is a ubiquitin- and light-chain 3-binding protein that is known to regulate the degradation of targeted proteins via autophagy and inclusion formation. In this study, we examined the effects of p62 depletion and overexpression on cultured cells and in a transgenic mouse model that overexpressed the mutant AR. Here, we demonstrate that depletion of p62 significantly exacerbated motor phenotypes and the neuropathological outcome, whereas overexpression of p62 protected against mutant AR toxicity in SBMA mice. Depletion of p62 significantly increased the levels of monomeric mutant AR and mutant AR protein complexes in an SBMA mouse model via the impairment of autophagic degradation. In addition, p62 overexpression improved SBMA mouse phenotypes by inducing cytoprotective inclusion formation. Our results demonstrate that p62 provides two different therapeutic targets in SBMA pathogenesis: (1) autophagy-dependent degradation and (2) benevolent inclusion formation of the mutant AR.

  4. Clinical study on the efficacy of Rajayapana Basti and Baladi Yoga in motor disabilities of cerebral palsy in children

    Science.gov (United States)

    Shailaja, U.; Rao, Prasanna N.; Girish, K. J.; Arun Raj, G. R.

    2014-01-01

    Background: Cerebral palsy is a static encephalopathy that may be defined as a non-progressive disorder of posture and movement often associated with epilepsy and abnormalities in speech, vision and intellect resulting from a defect or lesion of the developing brain. There are 25 lakhs cerebral palsy affected children in India. Aim: To assess the efficacy of Rajayapana Basti (RB) and Baladi Yoga in motor disabilities of cerebral palsy in children. Materials and Methods: Total 98 children satisfying diagnostic criteria and between the age group of 2-10 years were included and randomly divided into two groups. In RB with Baladi group (n = 40) patients were treated with Mustadi Rajayapana Basti for 8 days, followed by oral administration of Baladi Yoga with honey and ghee for 60 days. Before administering Basti, patients were subjected to Sarvanga Abhyanga and Sastikashali Pinda Sveda. In the control group (n = 40), patients were given tablets of Godhuma Choorna for 60 days. Before administering the placebo tablet, the patients of the control group were given Sarvanga Abhyanga and Sastikashali Pinda Sveda for 8 days. The patients of the control group were given Basti with lukewarm water for 8 days. Results: RB group has shown improvements in understanding ability (13.43%), speech (10%) and performance skill (11.11%), in fine motor functions such as putting small object in to a container (14.3%), throws the ball in all direction (21.8%), use of thumb and index finger (10.93%), retaining 2 inch cube in fist (19.04%), folds paper and inserts into envelope (10.30%), in gross motor functions such as in crawling (26.7%), sitting (31.7%), standing (13.75%), walking (9.5%) and claps hands (13.9%) respectively. Conclusion: Mustadi RB along with Baladi Yoga provided a significant improvement in all the parameters and has promising result in managing motor disabilities of cerebral palsy in children. PMID:26664239

  5. 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:…

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

    Directory of Open Access Journals (Sweden)

    Rossella Spataro

    2015-01-01

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

  7. 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.

  8. A guide to the evaluation of fourth cranial nerve palsies.

    Science.gov (United States)

    Lee; Hayman; Beaver; Prager; Kelder; Scasta; Avilla; von Noorden GK; Tang

    1998-12-01

    PURPOSE To devise a cost-effective guide for the evaluation of fourth nerve palsies (FNP). METHODS A review of the pertinent English language literature was performed to devise a guide for the evaluation (including neuroimaging) of FNP. The authors report a retrospective review of imaging studies performed on 206 patients with FNP. RESULTS The literature was used to develop the imaging guide. In the retrospective chart review of 206 patients from two tertiary care centers, 28 patients (13.6%) underwent a computed tomography scan and/or a magnetic resonance scan. Of these patients, five had associated neurological symptoms (non-isolated), one was traumatic, five were congenital, four were vasculopathic, eleven were non-vasculopathic, and two were progressive. Following the recommendations of the imaging guide, the five isolated congenital FNP and the four isolated vasculopathic FNP would not have undergone neuroimaging studies. The total costs of these neuroimaging studies in these nine patients were 19,000 dollars. Four patients in the retrospective review with associated neurological deficits (non-isolated) should have undergone neuroimaging according to the guide, but did not. CONCLUSIONS Although the evaluation of FNP can be difficult, the decision to order neuroimaging can be improved by using an imaging guide. An imaging guide for the evaluation of FNP may allow more appropriate and cost-effective imaging of these patients. Isolated congenital, old traumatic, or vasculopathic FNP do not require neuroimaging studies. Patients with non-isolated FNP should have directed neuroimaging studies based upon the results of clinical examination.

  9. Positron Emission Tomography—Computer Tomography Scan Used as a Monitoring Tool Following Cellular Therapy in Cerebral Palsy and Mental Retardation—A Case Report

    OpenAIRE

    Sharma, Alok; Sane, Hemangi; Paranjape, Amruta; Gokulchandran, Nandini; Kulkarni, Pooja; Nagrajan, Anjana; Badhe, Prerna

    2013-01-01

    Cerebral palsy (CP) is one of the non-progressive neurological diseases caused by damage to the brain tissue at birth, which leads to physical, cognitive and perceptive symptoms. Even after lifelong medical and therapeutic management there are residual deficits which affect the quality of life of the patients and their families. We examined a maximally rehabilitated, 20 year old male suffering from CP and Mental Retardation (MR). He had diplegic gait and Intelligence Quotient (IQ) score of 44...

  10. Neuroimaging in Cerebral Palsy – Report from North India

    Directory of Open Access Journals (Sweden)

    Anju AGGARWAL

    2013-11-01

    Full Text Available How to Cite This Article: Aggarwal A, Mittal H, Debnath SKR, Rai A. Neuroimaging in Cerebral Palsy–Report from North India. Iran J Child Neurol. 2013 Autumn; 7(3:41- 46. ObjectiveOnly few Indian reports exist on neuroimaging abnormalities in children with cerebral palsy (CP from India. Materials & MethodsWe studied the clinico-radiological profile of 98 children diagnosed as CP at a tertiary centre in North India. Relevant investigations were carried out to determine the etiology. ResultsAmong the 98 children studied, 80.5% were males and 22.2% were premature. History of birth asphyxia was present in 41.9%. Quadriplegic CP was seen in 77.5%, hemiplegic in 11.5%, and diplegic in 10.5%. Other abnormalities were microcephaly (60.5%, epilepsy (42%, visual abnormality (37%, and hearing abnormality (20%. Neuroimaging was abnormal in 94/98 (95.91%.Abnormalities were periventricular white matter abnormalities (34%, deep grey matter abnormalities (47.8%, malformations (11.7%, and miscellaneous lesions (6.4%. Neuroimaging findings did not relate to the presence of birth asphyxia, sex, epilepsy, gestation, type of CP, or microcephaly. ConclusionsNeuroimaging is helpful for etiological diagnosis, especially malformations.  ReferencesSinghi PD, Ray M, Suri G. Clinical spectrum of cerebral palsy in north India-an analysis of 1000 cases. J Trop Pediatr 2002 48(3; 162-6.Sharma P, Sharma U, Kabra A. Cerebral Palsy-Clinical Profile and Predisposing Factors. Indian Pediatr 1999;36(10:1038-42.Nelson KB, Ellenberg JH. Antecedents of cerebral palsy. Multivariate analysis of risk. N Engl J Med 1986 315(2:81-6.Krägeloh-Mann I, Horber V. The role of magnetic resonance imaging in elucidating the pathogenesis of cerebral palsy: a systematic review. Dev Med Child Neurol 2007; 49(2:144-51.Rosenbaum P, Paneth N, Leviton A, Goldstein M, Bax M, Damiano D, et al. A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol Suppl 2007

  11. Sciatic nerve palsy associated with total hip arthroplasty.

    Science.gov (United States)

    Dhillon, M S; Nagi, O N

    1992-01-01

    Six cases of clinically evident sciatic or peroneal nerve palsy occurred in a consecutive series of 380 total hip arthroplasties (THA). An additional eight cases of peroneal nerve palsy due to pressure from Thomas splint or tight bandages were seen. Factors apparently causing nerve palsy were significant lateralization and lengthening in four cases and dislocation of the hip in one case. The cases with neuroapraxia of the peroneal nerve were seen from the third to the fifth day of Thomas splint immobilization. EMG studies were conducted in all six group 1 patients; at the end of one year the results were good in two cases, fair in three cases, and poor in one case. The results suggest that limb lengthening should be limited to 4 cm to minimize this complication. It was also seen that patients with peroneal nerve palsy due to local compression do well, though some are bothered by mild residual dysesthesia over the dorsum of the foot. In contrast, patients with sciatic nerve palsy do not have such a good outlook. PMID:1345646

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

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    Lee Hyun

    2003-06-01

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

  13. 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.

  14. 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.

  15. The Role of Information Systems to Manage Cerebral Palsy

    Science.gov (United States)

    AJAMI, Sima; MAGHSOUDLORAD, Ali Akbar

    2016-01-01

    Objective In healthcare system, it is necessary to have exact and accurate information in order to address health care needs and requirements of society members as well as expectations of policy makers, planners and decision makers. The aim of this narrative review article was to explain the role of information systems in cerebral palsy management and identify the advantages and barriers to the development of cerebral palsy registry system. Data were collected using databases such as of Science Direct, PubMed, Proquest, Springer, and SID (Scientific Information Database). Overall, 65 sources were selected. One of the biggest challenges for children with physical and motor disabilities in rehabilitation center is access to a system, which provides a comprehensive data set reflecting all information on a patient’s care. Thus, data and information management in children with physical and motor disability such as cerebral palsy facilitates access to data and cerebral palsy data comparison as well as the monitoring incidence rate of cerebral palsy, enhancing health care quality; however, there are always numerous barriers to establish the system. One of the ways to overcome these problems is the establishment of a standard framework of minimum data sets and exact definition of its data components. Reliable standards in the use of applications as well as user-friendly software will ensure patients’ data extraction and registration. PMID:27247578

  16. 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.

  17. CT findings of cerebral palsy and behaviour development

    International Nuclear Information System (INIS)

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

  18. 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.

  19. The Role of Information Systems to Manage Cerebral Palsy.

    Science.gov (United States)

    Ajami, Sima; Maghsoudlorad, Ali Akbar

    2016-01-01

    Objective In healthcare system, it is necessary to have exact and accurate information in order to address health care needs and requirements of society members as well as expectations of policy makers, planners and decision makers. The aim of this narrative review article was to explain the role of information systems in cerebral palsy management and identify the advantages and barriers to the development of cerebral palsy registry system. Data were collected using databases such as of Science Direct, PubMed, Proquest, Springer, and SID (Scientific Information Database). Overall, 65 sources were selected. One of the biggest challenges for children with physical and motor disabilities in rehabilitation center is access to a system, which provides a comprehensive data set reflecting all information on a patient's care. Thus, data and information management in children with physical and motor disability such as cerebral palsy facilitates access to data and cerebral palsy data comparison as well as the monitoring incidence rate of cerebral palsy, enhancing health care quality; however, there are always numerous barriers to establish the system. One of the ways to overcome these problems is the establishment of a standard framework of minimum data sets and exact definition of its data components. Reliable standards in the use of applications as well as user-friendly software will ensure patients' data extraction and registration. PMID:27247578

  20. EVALUATION OF NEUROIMAGING IN CEREBRAL PALSY

    Directory of Open Access Journals (Sweden)

    S.H. Hasanpour avanji

    2008-11-01

    Full Text Available ObjectiveCerebral palsy (CP, a common static motor neurological disorder of childhood with wide spectrum of underlying etiologies, can be demonstrated with different neuro imaging techniques. We undertook this study to investigate the diagnosis of intracranial lesions in children with CP and its correlation between clinical deficits and neuroradiological findings.Materials and methodsIn this prospective hospital-based study, the data of 120 patients with CP, aged below 18 years, referring to the neurology clinic of the Ali Asghar Pediatric hospital in Tehran was studied; data on their cranial neuroimaging findings was analyzed any possible association(s between the gestational ages, prenatal history and neurological deficits were investigated.ResultsOf the 120 patients, 72 (60% were male; 75% were aged below 7 years.Common predisposing factors were prenatal asphyxia, LBW, prematurity and toxemia of pregnancy. Of the 120 cases, 90%(107 had spastic CP, with the quadriplegic type being the most common (54%, followed by spastic paraplegia (21%; twenty-four patients (20% had significant Preventricular Leucomalacia (PVL, a finding more common among those born pre-term.Sixteen patients had hemiplegic CP, 14 of whom showed unilateral lesions on brain MRI imaging. Ten (8% had extra pyramidal CP, a condition more common among term born infants, while six of the 10(72% showed significant abnormalities on the basal ganglia. Cerebral atrophy was seen in 60 (50% of patients and PVL in 20%; encephalomalacia, gliosis, middle cerebral artery infarcts, PVL and gliosis indicated hypoxia as a risk factor for CP. Extent of MRI lesions correlated with the severity of neurological deficits in CP lesions, which were more extensive in Quadriplegics and double hemiplegics rather than paraplegics, and among those delivered preterm as compared to those born at term.ConclusionRadiological findings were found to be closely related to the type of CP and the neurological

  1. 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.

  2. 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. PMID:16272661

  3. Birth brachial plexus palsy: a race against time.

    Science.gov (United States)

    Patra, Sambeet; Narayana Kurup, Jayakrishnan K; Acharya, Ashwath M; Bhat, Anil K

    2016-01-01

    A 5-year-old child presented to us with weakness of the left upper limb since birth. With the given history of obstetric trauma and limb examination, a diagnosis of birth brachial plexus palsy was made. Brachial plexus exploration along with microsurgery was performed at the same time which included extrinsic neurolysis of the roots and trunks and nerve transfer for better shoulder external rotation and elbow flexion. Both the movements were severely restricted previously due to co-contractures with the shoulder internal rotators and triceps. The problem of birth brachial plexus palsy is proving to be a global health burden both in developed countries and in developing countries such as India. The lack of awareness among the general public and primary healthcare providers and inadequate orthopaedic and neurosurgeons trained to treat the condition have worsened the prognosis. This case lays stress on the delayed complications in birth brachial palsy and its effective management. PMID:27402656

  4. 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...

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

    International Nuclear Information System (INIS)

    The CT findings in 120 cerebral palsied children are analysed. The 72.5% positive findings are correlated with the clinical types, as well as the aetiological basis for the cerebral palsy. The spastic type, 83.3% of the total number of children, had the highest positive findings. The yield was increased in children with seizures (91.3%) and those in the postnatal group (90%), as well as those with birth trauma and neonatal asphyxia (94%). The findings were those of atrophy in 30.8%, hydrocephalus, in 10%, infarct in 11.6%, porencephaly in 8.3% and others. The atropic changes and their patterns are explained. Treatable lesions, such as tumour, hydrocephalus, subdural haematoma, porencephaly and hygroma were identified in 22.5% of cases. It is concluded that CT scan is definitely efficacious in the management of cerebral palsied children. (orig.)

  7. 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.

  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. Isolated Abducens Nerve Palsy: Update on Evaluation and Diagnosis.

    Science.gov (United States)

    Elder, Christopher; Hainline, Clotilde; Galetta, Steven L; Balcer, Laura J; Rucker, Janet C

    2016-08-01

    Abducens nerve palsy is a common clinical finding in neurology practice. In many instances, the origin is obvious and management straightforward; however, the list of possible etiologies and mimics is vast and diverse and diagnostic decisions can be challenging and even controversial. This is especially true when the abducens nerve is affected in isolation, since in the current era of cost-effective medicine, it is critical to accurately diagnose etiologies that may lead to major morbidity or mortality with efficiency. Topics for highlighted updates in this review include management of isolated abducens nerve palsy with a high likelihood of a microvascular ischemic etiology; common imaging pitfalls and current state-of-the-art neuroimaging; and abducens palsy mimics. PMID:27306521

  10. Computerized tomographic studies in cerebral palsy

    International Nuclear Information System (INIS)

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

  11. Cerebral Palsy: Comprehensive Review and Update

    International Nuclear Information System (INIS)

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

  12. 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. PMID:24648650

  13. Cerebral palsy: definition, assessment and rehabilitation.

    Science.gov (United States)

    Richards, Carol L; Malouin, Francine

    2013-01-01

    Over the last 25 years the definition and classification of cerebral palsy (CP) have evolved, as well as the approach to rehabilitation. CP is a disorder of the development of movement and posture, causing activity limitations attributed to nonprogressive disturbances of the fetal or infant brain that may also affect sensation, perception, cognition, communication, and behavior. Motor control during reaching, grasping, and walking are disturbed by spasticity, dyskinesia, hyperreflexia, excessive coactivation of antagonist muscles, retained developmental reactions, and secondary musculoskeletal malformations, together with paresis and defective programing. Weakness and hypoextensibility of the muscles are due not only to inadequate recruitment of motor units, but also to changes in mechanical stresses and hormonal factors. Two methods, the General Movements Assessment and the Test of Infant Motor Performance, now permit the early detection of CP, while the development of valid and reliable outcome measures, particularly the Gross Motor Function Measure (GMFM), have made it possible to evaluate change over time and the effects of clinical interventions. The GMFM has further led to the development of predictive curves of motor function while the Gross Motor Classification System and the Manual Ability Classification System provide standardized means to classify the severity of the movement disability. With the emergence of the task-oriented approach, the focus of therapy in rehabilitation has shifted from eliminating deficits to enhancing function across all performance domains by emphasizing fitness, function, participation, and quality of life. There is growing evidence supporting selected interventions and interest for the therapy and social integration of adults with CP.

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

    Science.gov (United States)

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

    2016-01-01

    Cerebral Palsy (CP) is a disabling condition that affects a child’s life and his/her family irreversibly. It is usually a non-progressive condition but improvement over time is rarely seen. The condition can be due to prenatal hypoxia, metabolic, genetic, infectious, traumatic or other causes. It is therefore a heterogeneous group that results in functional motor disability associated with different degrees of cognitive abnormalities. There are no treatments that can cure or even improve CP and the best available approach aims at functional, social and nutritional supportive care and counseling. In this paper, we report 17 sequential patients with CP treated with intrathecal administration of Bone Marrow Mononuclear Cells (BMMC). All patients had an uneventful post-injection course with 73% of the evaluable patients treated having a good response using the Gross Motor Function Classification System (GMFCS). The average improvement was 1.3 levels on the GMFCS with cognitive improvements as well. PMID:27426090

  15. 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...

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

    OpenAIRE

    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 ea...

  17. When is facial paralysis Bell palsy? Current diagnosis and treatment.

    Science.gov (United States)

    Ahmed, Anwar

    2005-05-01

    Bell palsy is largely a diagnosis of exclusion, but certain features in the history and physical examination help distinguish it from facial paralysis due to other conditions: eg, abrupt onset with complete, unilateral facial weakness at 24 to 72 hours, and, on the affected side, numbness or pain around the ear, a reduction in taste, and hypersensitivity to sounds. Corticosteroids and antivirals given within 10 days of onset have been shown to help. But Bell palsy resolves spontaneously without treatment in most patients within 6 months.

  18. [Treatment of idiopathic peripheral facial nerve paralysis (Bell's palsy)].

    Science.gov (United States)

    Meyer, Martin Willy; Hahn, Christoffer Holst

    2013-01-28

    Bell's palsy is defined as an idiopathic peripheral facial nerve paralysis of sudden onset. It affects 11-40 persons per 100,000 per annum. Many patients recover without intervention; however, up to 30% have poor recovery of facial muscle control and experience facial disfigurement. The aim of this study was to make an overview of which pharmacological treatments have been used to improve outcomes. The available evidence from randomized controlled trials shows significant benefit from treating Bell's palsy with corticosteroids but shows no benefit from antivirals.

  19. Complete oculomotor palsy caused by persistent trigeminal artery.

    Science.gov (United States)

    Bosco, Domenico; Consoli, Domenico; Lanza, Pier Luigi; Plastino, Massimiliano; Nicoletti, Francesco; Ceccotti, Claudio

    2010-10-01

    Primitive trigeminal artery (PTA) is the most frequent embryonic communication between the carotid and vertebro-basilar system. PTA is a pathophysiology phenomenon which has been implicated as a rare cause of cranial nerve dysfunction. We report the case of a 40-year-old woman who developed a complete oculomotor nerve palsy caused by a persistent ecstatic trigeminal artery. Brain MRI and MRA studies documented a neurovascular conflict between the oculomotor nerve and a PTA. To the best of our knowledge there is no report about complete third cranial nerve palsy NC due to a PTA. A role of this rare vascular condition is discussed. PMID:20552240

  20. Two Cases of Elderly-Onset Hereditary Neuropathy with Liability to Pressure Palsy Manifesting Bilateral Peroneal Nerve Palsies

    Directory of Open Access Journals (Sweden)

    Norihiko Kawaguchi

    2012-10-01

    Full Text Available Hereditary neuropathy with liability to pressure palsy (HNPP is characterized by recurrent focal neuropathies, which usually become symptomatic in the second or third decade of life. However, clinical phenotypic heterogeneity among patients with HNPP has recently been reported. Certain patients show polyneuropathy-type diffuse nerve injuries, whereas others remain asymptomatic at older ages. We present two cases of elderly-onset bilateral peroneal nerve palsies with diffuse muscle weakness in the lower limbs and glove-and-stocking type sensory disturbance. Both patients were diagnosed with HNPP by genetic analyses that detected deletions of chromosome 17p11.2 in peripheral myelin protein 22 genes. Their clinical courses suggested that the Japanese sitting style termed ‘seiza’, a way of sitting on the floor with the lower legs crossed under the thighs, was a precipitating factor for the bilateral peroneal nerve palsies.

  1. 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.

  2. Observation of the aesthetic effects of treating eyelid spots and bulbar conjunctiva brown spots by operation%手术治疗眼睑裂斑及球结膜褐色斑美容效果观察

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Objective To investigate the beautifying eyes by removing eyelid spots and bulbar conjunctiva brown spots with operation. Methods From November 2009 to April 2014,We treated 98 cases with eyelid spots and bulbar conjunctiva brown spots by diseased tissues resection.And the wound did not sew up. Results Followed up for 1-2 years after the operation,93 patients in 98 cases received satisfactory effects,5 cases had more satisfactory results. Conclusion It has good eye aesthetic effects by removing eyelid spots and bulbar conjunctiva brown spots with operation.%目的:探讨通过手术方法去除眼睑裂斑及球结膜褐色斑,达到眼部美学效果。方法:2009年11月-2015年4月,笔者对98例眼睑裂斑、球结膜褐色斑患者,采用表面麻醉和肿胀浸润麻醉后,将病变处球结膜浅层充分分离后剪除病变组织,创口边缘不缝合,自行愈合。结果:术后随访1~2年,98例中93例获得了理想的术后效果,术区洁白、光滑,5例效果比较理想。结论:通过手术方法去除眼睑裂斑及球结膜褐色斑可达到良好的美容效果。

  3. The cranial MRI in severe cerebral palsy

    International Nuclear Information System (INIS)

    The magnetic resonance examination was performed in 38 patients with severe cerebral palsy (CP; 15 males and 23 females) who had both motor delay (unable to move anywhere) and mental retardation (I.Q. or D.Q. below 30). Neuroimaging findings were compared with the CP type, etiology, and grade of understanding of language. Cranial magnetic resonance imagings (MRI) in CP were divided into five types. In type 1, nine predominantly showed cyst-liked ventricles and periventricular hyperintensity on T2-weighted imaging (PVH) and only scarred basal ganglia and thalamus were visible. All suffered from neonatal asphyxia and the clinical type was rigospastic tetraplegia (RST). In type 2, eleven predominantly showed PVH and hyperintensity on T2-weighted (HT2) in basal ganglia and thalamus. All suffered from neonatal asphyxia and the clinical type was RST or rigospastic diplegia. In type 3, five showed PVH and three had cortical atrophy. All suffered from neonatal asphyxia and the clinical type was spastic diplegia. In type 4, four predominantly showed HT2 in putamen and thalamus. Three had cortical atrophy. All suffered from neonatal asphyxia. The clinical type was athetotic CP (ATH). In type 5, nine predominantly showed HT2 in globus pallidus. Four had cortical atrophy and two had hippocampal atrophy. All suffered from neonatal jaundice and the clinical type was ATH. All patients who suffered from neonatal asphyxia and spastic CP had MRI in PVH. All patients who suffered from neonatal asphyxia and ATH showed HT2 in putamen and thalamus. Almost patients who suffered from neonatal jaundice and ATH showed HT2 in globus pallidus. With athetotic CP, cases with atrophy of the cerebral cortex and/or hippocampus were lower grade of understanding of language than no atrophy of both. The results of studies of MRI are in agreement with neuropathological findings. (author)

  4. Parálisis cerebral Cerebral palsy

    Directory of Open Access Journals (Sweden)

    Jorge Malagon Valdez

    2007-01-01

    Full Text Available El término parálisis cerebral (PC engloba a un gran número de síndromes neurológicos clínicos, de etiología diversa. Estos síndromes se caracterizan por tener una sintomatología común: los trastornos motores. Algunos autores prefieren manejar términos como "encefalopatía fija", "encefalopatías no evolutivas". Se mencionan la utilidad de programas de intervención temprana y métodos especiales de rehabilitación, así como el manejo de las deficiencias asociadas como la epilepsia, deficiencia mental, trastornos del lenguaje, audición, visión, déficit de la atención que mejoran el pronóstico de manera significativa. El pronóstico también depende de la gravedad del padecimiento y de las manifestaciones asociadas.The term cerebral palsy (CP, is used for a great number of clinical neurological syndromes. The syndromes are characterized by having a common cause, motor defects. It is important, because they can cause a brain damage by presenting motor defects and some associated deficiencies, such as mental deficiency, epilepsy, language and visual defects and pseudobulbar paralysis, with the nonevolving fact. Some authors prefer using terms such as "non-evolving encephalopathies". In the treatment the utility of prevention programs of early stimulation and special rehabilitation methods, and treatment of associated deficiencies such as epilepsy, mental deficiency, language, audition and visual problems, and the attention deficit improve the prognosis in an important way. The prognosis depends on the severity of the disease and the associated manifestations.

  5. Hand Functioning in Children with Cerebral Palsy

    Science.gov (United States)

    Arnould, Carlyne; Bleyenheuft, Yannick; Thonnard, Jean-Louis

    2014-01-01

    Brain lesions may disturb hand functioning in children with cerebral palsy (CP), making it difficult or even impossible for them to perform several manual activities. Most conventional treatments for hand dysfunction in CP assume that reducing the hand dysfunctions will improve the capacity to manage activities (i.e., manual ability, MA). The aim of this study was to investigate the directional relationships (direct and indirect pathways) through which hand skills influence MA in children with CP. A total of 136 children with CP (mean age: 10 years; range: 6–16 years; 35 quadriplegics, 24 diplegics, 77 hemiplegics) were assessed. Six hand skills were measured on both hands: touch-pressure detection (Semmes–Weinstein esthesiometer), stereognosis (Manual Form Perception Test), proprioception (passive mobilization of the metacarpophalangeal joints), grip strength (GS) (Jamar dynamometer), gross manual dexterity (GMD) (Box and Block Test), and fine finger dexterity (Purdue Pegboard Test). MA was measured with the ABILHAND-Kids questionnaire. Correlation coefficients were used to determine the linear associations between observed variables. A path analysis of structural equation modeling was applied to test different models of causal relationships among the observed variables. Purely sensory impairments did seem not to play a significant role in the capacity to perform manual activities. According to path analysis, GMD in both hands and stereognosis in the dominant hand were directly related to MA, whereas GS was indirectly related to MA through its relationship with GMD. However, one-third of the variance in MA measures could not be explained by hand skills. It can be concluded that MA is not simply the integration of hand skills in daily activities and should be treated per se, supporting activity-based interventions. PMID:24782821

  6. Hand functioning in children with cerebral palsy

    Directory of Open Access Journals (Sweden)

    Carlyne eArnould

    2014-04-01

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

  7. Treating cerebral palsy with aculaser therapy

    Science.gov (United States)

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

    2008-03-01

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

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

    Directory of Open Access Journals (Sweden)

    Yun Jeong-hun

    2000-07-01

    Full Text Available This report was done to observe the effect of Hominis placenta herbal acupuncture on Bell's palsy. The study group comprised 16 patients who arrived at Woo-suk university oriental hospital from January, 1999 till January, 2000 for Bell's palsy. All patients were divided into two group. One was herbal acupunture group, and the other was control group. Acupunture group was done herbal acupuncture therapy on the facial acupuncture points. Followings are achievement and a term of each group. In herbal acupuncture group, 100% motor recovery was 7 case, 75% was 1 case, and 25% motor recovery term was 7.38±5.21 days, 50% was 11.00±6.16 days, 75% was 15.13±9.55 days, 100% was 23.14±7.97 days. In control group, 100% motor recovery was 4 case, 75% was 2 case, 25% below was 2 case and 25% motor recovery term was 11.17±4.96days, 50% was 18.17±6.82 days, 75% was 29.50±6.95 days, 100% was 44.00±11.49 days. The above results indicate that Hominis placenta herbal acupuncture is a useful effect on Bell's palsy. thus, continuous herbal acupunture study will be needed for more clinical application on Bell' palsy.

  9. Gait Training and Ankle Dorsiflexors in Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2015-03-01

    Full Text Available Investigators at University of Copenhagen, Denmark, evaluated whether 4 weeks of 30 min daily treadmill training with an incline may facilitate corticospinal transmission and improve control of the ankle joint in 16 children, aged 5-14 years, with cerebral palsy.

  10. Infant Motor Profile and cerebral palsy : promising associations

    NARCIS (Netherlands)

    Heineman, Kirsten R.; Bos, Arend F.; Hadders-Algra, Mijna

    2011-01-01

    AIM The Infant Motor Profile (IMP) is a novel qualitative assessment of motor behaviour in infancy. The aim of this study was to determine whether IMP scores throughout infancy differ between children with and without cerebral palsy (CP) at 18 months. Furthermore, we evaluated the predictive ability

  11. Executive Functions in Youth With Spastic Cerebral Palsy

    NARCIS (Netherlands)

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

    2011-01-01

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

  12. The Determinants of Daily Function in Children with Cerebral Palsy

    Science.gov (United States)

    Tseng, Mei-Hui; Chen, Kuan-Lin; Shieh, Jeng-Yi; Lu, Lu; Huang, Chien-Yu

    2011-01-01

    The aim of this study was to identify determinants of daily function in a population-based sample of children with cerebral palsy (CP). The study took into consideration factors from the entire scope of the International Classification of Functioning, Disability, and Health (ICF). Furthermore, the determinants of daily function were examined from…

  13. Evaluating rehabilitation interventions in preschool children with cerebral palsy

    NARCIS (Netherlands)

    Kruijsen-Terpstra, A.J.A.

    2015-01-01

    Children with cerebral palsy (CP) face limitations in their daily activities, in particular regarding mobility and self-care. Although many treatment ideas and approaches are available, evidence to show which intervention is the most effective for preschool children with CP is lacking. Furthermore,

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

    NARCIS (Netherlands)

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

    2007-01-01

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

  15. Orthodontic Treatment for a Patient with Cerebral Palsy

    Institute of Scientific and Technical Information of China (English)

    TANG Kai-hong; DUAN Chang-hua; WANG Zeng-quan

    2009-01-01

    @@ Cerebral palsy(CP) is a group of permanent disorders asso-ciated with the developmental brain injuries that occur during fe-tal development, at birth, or shortly after birth[1,2]. Very few of such patients had the chance to be treated their maloccluded teeth. So, many orthodontists have no experience on such cases.

  16. Predictors of participation of adolescents with cerebral palsy

    DEFF Research Database (Denmark)

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

    2014-01-01

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

  17. Retrospective Descriptive Study of Cerebral Palsy in Nepal

    Science.gov (United States)

    Thapa, Ritesh

    2016-01-01

    There is very little data pertaining to cerebral palsy (CP) from Nepal. In this retrospective study it was observed that dyskinetic CP was seen in 29% and the sex ratio of males to females was two in the study population of children with CP. Both of these are much higher than data from developed countries. Hence, further randomized cross-sectional…

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

    DEFF Research Database (Denmark)

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

    2011-01-01

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

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

    DEFF Research Database (Denmark)

    Reimers, J I; Latocha, J E

    1990-01-01

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

  20. Everyday life and social consequences of cerebral palsy

    DEFF Research Database (Denmark)

    Uldall, Peter

    2013-01-01

    The disclosure of diagnosis for a child with cerebral palsy (CP) is a highly stressful experience to the parents. The experience can be alleviated by clarity, empathy, and an emphasis on the child's resources and abilities. Despite chronic stress many families function well and manage to strengthen...

  1. Diagnosis of Bell palsy with gadolinium magnetic resonance imaging.

    Science.gov (United States)

    Becelli, R; Perugini, M; Carboni, A; Renzi, G

    2003-01-01

    Bell palsy is a condition resulting from a peripheral edematous compression on the nervous fibers of the facial nerve. This pathological condition often has clinical characteristics of no importance and spontaneously disappears in a short time in a high percentage of cases. Facial palsy concerning cranial nerve VII can also be caused by other conditions such as mastoid fracture, acoustic neurinoma, tumor spread to the temporal lobe (e.g., cholesteatoma), neoformation of the parotid gland, Melkersson-Rosenthal syndrome, and Ramsay-Hunt syndrome. Therefore, it is important to adopt an accurate diagnostic technique allowing the rapid detection of Bell palsy and the exclusion of causes of facial paralysis requiring surgical treatment. Magnetic resonance imaging (MRI) with medium contrast of the skull shows a marked increase in revealing lesions, even of small dimensions, inside the temporal bone and at the cerebellopontine angle. The authors present a clinical case to show the important role played by gadolinium MRI in reaching a diagnosis of Bell palsy in the differential diagnosis of the various conditions that determine paralysis of the facial nerve and in selecting the most suitable treatment or surgery to be adopted.

  2. Childhood Educational Experiences of Women with Cerebral Palsy

    Science.gov (United States)

    Freeborn, Donna; Mandleco, Barbara

    2010-01-01

    The purpose of this study was to examine the childhood experiences of women with cerebral palsy (CP), from the perspectives of these women. Using the feminist biographical method, eight women with CP participated in two in-depth interviews. Participants ranged in age from 22 to 55 years and had moderate to severe athetoid or spastic CP. Four…

  3. Rehabilitation of Bells' palsy from a multi-team perspective.

    Science.gov (United States)

    Hultcrantz, Malou

    2016-04-01

    Conclusions Defectively healed facial paralysis causes difficulties to talk and eat, involuntary spasms (synkinesis), and cosmetic deformities which can give rise both to severe psychological and physical trauma. A team consisting of Ear-Nose-Throat specialists, Plastic surgeons and Physiotherapists can offer better care, treatment and outcome for patients suffering from Bells' palsy. Objectives Patients suffering from Bells' palsy from all ENT hospitals in Sweden and the University Hospital in Helsinki has been included. Methods Results have been drawn and statistically processed for different outcomes from a prospective, double blind cross over study. Results from a pilot surgical study and therapeutic results from physiotherapy studies have been included. Ideas concerning different kinds of surgery will be reviewed and the role of physiotherapy discussed. Results According to common results, treatment with Prednisolone enhances the recovery rate and should, if possible, be used early in the course. Sunnybrook grading at 1 month after onset most accurately predicts non-recovery at 12 months in Bells' palsy and a risk factor curve will be presented in order to predict outcome and selection of patients for undergoing facial surgery. This report is focusing on how to handle patients with Bells' palsy from a multi-rehabilitation team point of view, and what will be recommended to provide these patients with the best clinical and surgical help. PMID:26634395

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

    Science.gov (United States)

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

    2009-01-01

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

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

    Science.gov (United States)

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

    2009-01-01

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

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

    Science.gov (United States)

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

    2009-01-01

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

  7. Language and Motor Speech Skills in Children with Cerebral Palsy

    Science.gov (United States)

    Pirila, Silja; van der Meere, Jaap; Pentikainen, Taina; Ruusu-Niemi, Pirjo; Korpela, Raija; Kilpinen, Jenni; Nieminen, Pirkko

    2007-01-01

    The aim of the study was to investigate associations between the severity of motor limitations, cognitive difficulties, language and motor speech problems in children with cerebral palsy. Also, the predictive power of neonatal cranial ultrasound findings on later outcome was investigated. For this purpose, 36 children (age range 1 year 10 months…

  8. Operant Control of Pathological Tongue Thrust in Spastic Cerebral Palsy.

    Science.gov (United States)

    Thompson, George A., Jr.

    1979-01-01

    The behavior modification procedure, carried out at mealtime with a ten-year-old retarded boy who had spastic cerebral palsy, consisted of differential reinforcement and punishment, and resulted in substantial decreases in tongue thrust (reverse swallowing) and food expulsion, and a large increase in observed chewing. (Author/DLS)

  9. Behaviour in Children with Cerebral Palsy with and without Epilepsy

    Science.gov (United States)

    Carlsson, Malin; Olsson, Ingrid; Hagberg, Gudrun; Beckung, Eva

    2008-01-01

    The aim of the study was to describe behavioural problems in children with cerebral palsy (CP) with and without epilepsy. The children were sampled from the Western Sweden CP register and were part of a European Union project. The Strength and Difficulties Questionnaire and questions on epilepsy were answered by one parent of each child. Medical…

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

    Science.gov (United States)

    van der Linden, Marietta

    2012-01-01

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

  11. Hansen's disease and HIV coinfection with facial nerve palsy.

    Science.gov (United States)

    Yadav, Nidhi; Kar, Sumit; Madke, Bhushan; Gangane, Nitin

    2015-01-01

    There are very few published reports of HIV leprosy co infection in India in spite of having a large burden of both leprosy and HIV. Herein we are reporting a case of co-infection of Hansen's disease and HIV with facial nerve palsy. PMID:25883486

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

    Science.gov (United States)

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

    2012-01-01

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

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

    Science.gov (United States)

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

    2012-01-01

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

  14. Aerobic Capacity in Children and Adolescents with Cerebral Palsy

    Science.gov (United States)

    Verschuren, Olaf; Takken, Tim

    2010-01-01

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

  15. Tactile Assessment in Children with Cerebral Palsy: A Clinimetric Review

    Science.gov (United States)

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

    2011-01-01

    This review evaluates the clinimetric properties of tactile assessments for children with cerebral palsy. Assessment of registration was reported using Semmes Weinstein Monofilaments (SWMs) or exteroception. Assessment of two-point discrimination was reported using the Disk-Criminator[R] or paperclip methods; Single point localization and double…

  16. Drooling in cerebral palsy: hypersalivation or dysfunctional oral motor control?

    NARCIS (Netherlands)

    Erasmus, C.E.; Hulst, K. van; Rotteveel, L.J.C.; Jongerius, P.H.; Hoogen, F.J.A. van den; Roeleveld, N.; Rotteveel, J.J.

    2009-01-01

    AIM: To investigate whether drooling in children with cerebral palsy (CP) in general and in CP subtypes is due to hypersalivation. METHOD: Saliva was collected from 61 healthy children (30 males, mean age 9y 5mo [SD 11mo]; 31 females, mean age 9y 6mo [1y 2mo]) and 100 children with CP who drooled (5

  17. Physical activity in young children with cerebral palsy

    NARCIS (Netherlands)

    Zwier, J. Nathalie; van Schie, Petra E. M.; Becher, Jules G.; Smits, Dirk-Wouter; Gorter, Jan Willem; Dallmeijer, Annet J.

    2010-01-01

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

  18. Aerobic capacity in children and adolescents with cerebral palsy

    NARCIS (Netherlands)

    Verschuren, Olaf; Takken, Tim

    2010-01-01

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

  19. Trunk recruitment during spoon use in tetraparetic cerebral palsy

    NARCIS (Netherlands)

    Roon, D.B. van; Steenbergen, B.; Meulenbroek, R.G.J.

    2004-01-01

    In the present study we investigated the extent to which individuals suffering from spastic tetraparesis as a consequence of cerebral palsy tune their trunk involvement to accuracy demands in a spoon-handling task. Twenty-two participants (ten adolescents with spastic tetraparesis and 12 control par

  20. Intermittent versus Continuous Physiotherapy in Children with Cerebral Palsy

    Science.gov (United States)

    Christiansen, Annette Sandahl; Lange, Christa

    2008-01-01

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

  1. Neuro-ophthalmological approach to facial nerve palsy.

    Science.gov (United States)

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

    2015-01-01

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

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

    DEFF Research Database (Denmark)

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

    2002-01-01

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

  3. Quality of life in children with cerebral palsy: implications for practice.

    Science.gov (United States)

    Gilson, Kim-Michelle; Davis, Elise; Reddihough, Dinah; Graham, Kerr; Waters, Elizabeth

    2014-08-01

    The ability to assess the quality of life of children with cerebral palsy to inform and evaluate individual care plans, service planning, interventions, and policies is crucial. In this article, the recent evidence on quality of life in children with cerebral palsy is reviewed, with attention to the determinants of quality of life and role of this construct as a practical outcome indicator in clinical trials. Quality of life measurement advances for children with cerebral palsy are discussed with a focus on condition-specific quality of life measures, particularly, the Cerebral Palsy Quality of Life-Child, which is the first condition-specific quality of life measure for children with cerebral palsy. The article presents an overview for clinicians and researchers intending to use quality of life measures on children with cerebral palsy and provides recommendations for future research that will better inform practice in the field.

  4. Incidence and management of diaphragmatic palsy in patients after cardiac surgery

    OpenAIRE

    Mehta Yatin; Vats Mayank; Singh Ajmer; Trehan Naresh

    2008-01-01

    Background: Diaphragm is the most important part of the respiratory system. Diaphragmatic palsy following cardiac surgery is not uncommon and can cause deterioration of pulmonary functions and attendant pulmonary complications. Objectives: Aim of this study was to observe the incidence of diaphragmatic palsy after off pump coronary artery bypass grafting (OPCAB) as compared to conventional CABG and to assess the efficacy of chest physiotherapy on diaphragmatic palsy in post cardiac surgica...

  5. SOMATOTYPE PROFILES AND CHANGES DEPENDING ON TREADMILL EXERCISE IN CHILDREN WITH CEREBRAL PALSY

    OpenAIRE

    Nazmi; Betül; Hanife; Mustafa

    2014-01-01

    Objective. The postponement of the brain development as a result of brain lesion causes some functional inabilities affecting the whole body of the children with cerebral palsy compared to their peers. In this study, it was aimed to investigate the effects of the treadmill exercise on somatotype profiles and some variables in disabled children diagnosed with cerebral palsy. Methods. The subjects of the study were 37 children with cerebral palsy whose ages range from 7 to 15 and they were t...

  6. Demographic features of children with cerebral palsy in Şanlıurfa and neighbor counties

    OpenAIRE

    ALTINDAĞ, Özlem; SORAN, Neslihan; akcan, sAİT

    2009-01-01

    Cerebral palsy is the most common cause of disability in childhood. There are several factors in etiopathogenesis of cerebral palsy. Evaluation of etiology, the type of disease, and associated clinical features are important for the result of rehabilitation program and the participation of family. This study was planned for the determination of demographical characteristics of 50 patients with cerebral palsy from Sanliurfa and its neighbor counties.

  7. Isolated Bell’s palsy - An unusual presentation of dengue infection

    Institute of Scientific and Technical Information of China (English)

    Peter S; Malhotra N; Peter P; Sood R

    2013-01-01

    Dengue fever is a very common arthropod – borne infection in tropical countries. Neurological complications in dengue fever are relatively uncommon and among these, isolated cranial neuropathies have been reported only very rarely. We present an unusual neurological complication of Bell’s palsy (lower motor neuron 7 th nerve palsy) associated with dengue infection. To the best of our knowledge, there have been very few documented cases of Flavivirus causing isolated Bell’s palsy.

  8. Does the risk of cerebral palsy increase or decrease with increasing gestational age?

    OpenAIRE

    Murphy-Kaulbeck Lynn; Lutfi Samawal; Allen Alexander C; Joseph K S; Vincer Michael J; Wood Ellen

    2003-01-01

    Abstract Background It is generally accepted that the risk of cerebral palsy decreases with increasing gestational age of live born infants. However, recent studies have shown that cerebral palsy often has prenatal antecedents including congenital malformations, vascular insults and maternal infection. Cerebral palsy is therefore better viewed as occurring among fetuses, rather than among infants. We explored the epidemiologic implications of this change in perspective. Methods We used recent...

  9. Three-dimensional In Vivo Quantification of Knee Kinematics in Cerebral Palsy

    OpenAIRE

    Sheehan, Frances T.; Seisler, Andrea R.; Alter, Katharine E.

    2008-01-01

    Cerebral palsy is the most common disabling condition in childhood, involving a diverse group of movement and posture disorders of varying etiologies. Yet, much is unknown about how cerebral palsy affects individual joints because currently applied techniques cannot quantify the three-dimensional kinematic parameters at the joint level. We quantified the effects of cerebral palsy at the knee using fast phase contrast MRI, with the ultimate intent of improving the assessment of joint impairmen...

  10. Transient facial nerve paralysis (Bell's palsy) following administration of hepatitis B recombinant vaccine: a case report.

    Science.gov (United States)

    Paul, R; Stassen, L F A

    2014-01-01

    Bell's palsy is the sudden onset of unilateral transient paralysis of facial muscles resulting from dysfunction of the seventh cranial nerve. Presented here is a 26-year-old female patient with right lower motor neurone facial palsy following hepatitis B vaccination. Readers' attention is drawn to an uncommon cause of Bell's palsy, as a possible rare complication of hepatitis B vaccination, and steps taken to manage such a presentation.

  11. Epilepsy in children with cerebral palsy Epilepsia em crianças com paralisia cerebral

    OpenAIRE

    Isac Bruck; Sérgio Antônio Antoniuk; Adriane Spessatto; Ricardo Schmitt de Bem; Romeu Hausberger; Carlos Gustavo Pacheco

    2001-01-01

    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 w...

  12. Resting position of the head and malocclusion in a group of patients with cerebral palsy

    OpenAIRE

    Martinez-Mihi, Victoria; Silvestre, Francisco J.; Orellana, Lorena M.; Silvestre-Rangil, Javier

    2014-01-01

    Cerebral palsy are found as a result of these disorders, along with associated neuromuscular functional alterations that affect the resting position of the head. In this context, the resting position of the head could be responsible for several skeletal and dental occlusal disorders among patients with cerebral palsy. Objective: To assess the presence of malocclusions in patients with cerebral palsy, define the most frequent types of malocclusions, and evaluate how the resting position of the...

  13. Normalization of muscle tone in children with cerebral palsy in special schools.

    Directory of Open Access Journals (Sweden)

    Kruglyak M.

    2011-08-01

    Full Text Available The features of using exercise to reduce muscle tone in children with cerebral palsy in training at a special school. The study involved children with cerebral palsy 7-10 years. Presents the results of evaluation of motor function and muscle tone. The efficacy of the use of physical rehabilitation for children with cerebral palsy, a positive effect of using special exercise in stretching the muscles with increased tone, improve motor function and normalization of muscle tone.

  14. New Tendon Transfer for Correction of Drop-foot in Common Peroneal Nerve Palsy

    OpenAIRE

    Vigasio, Adolfo; Marcoccio, Ignazio; Patelli, Alberto; Mattiuzzo, Valerio; Prestini, Greta

    2008-01-01

    Common peroneal nerve palsy has been reported to be the most frequent lower extremity palsy characterized by a supinated equinovarus foot deformity and foot drop. Dynamic tendon transposition represents the gold standard for surgical restoration of dorsiflexion of a permanently paralyzed foot. Between 1998 and 2005, we operated on a selected series of 16 patients with traumatic complete common peroneal nerve palsy. In all cases, we performed a double tendon transfer through the interosseous m...

  15. 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

    dorsiflexion, plantarflexion, leg press, hamstring curls, abdominal curls and back extension 3 days/week for 12 weeks, with 3 sets per exercise and progressing during the training period from 12-6 RM. RFDdf, 3-D gait analysis, functional performance and ankle joint passive- and reflex-mediated muscle stiffness...... to the increased RFDdf (r=0.73). No other between-group differences were observed.These findings suggest that explosive PRT may increase RFDdf and facilitate larger range of movement in the ankle joint during gait. Explosive PRT should be tested in clinical practice as part of a long-term training program......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...

  16. Clinical Observation on Acupuncture Treatment of 30 Cases with Apoplectic Pseudobulbar Palsy

    Institute of Scientific and Technical Information of China (English)

    彭拥军; 李忠仁; 杨永清; 黄国琪

    2006-01-01

    目的:探讨影响针刺治疗中风假性球麻痹(PBP)患者疗效的因素.方法:将60例风痰阻络型中风假性球麻痹住院患者随机分为治疗组和对照组,对疗效进行观察.结果与结论:针刺效果优于对照组(P<0.05).研究中发现随着脑卒中发生次数的增多,发生重度吞咽困难的机率有增高的趋势,发生吞咽困难的时间相继提前,提示对脑卒中进行早期治疗和预防的重要性.%Objective: To investigate the factors influencing the therapeutic effect in acupuncture treatment of apoplectic pseudobulbar palsy (PBP). Methods: Sixty patients with apoplectic pseudobulbar palsy in pattern of obstruction of wind and phlegm in the meridians were randomly divided into the treatment group and control group, to observe the therapeutic effect. Results and Conclusion: The therapeutic effect was significantly better in the treatment group than in the control group (P<0.05). It has been found in the study that with increase in the occurrence of cerebral apoplexy, the incidence rate of severe dysphagia increased and dysphagia took place progressively earlier, indicating the importance of early treatment and prevention of cerebral apoplexy.

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

    Directory of Open Access Journals (Sweden)

    Mohammad Ghasem Shams

    2010-12-01

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

  18. FACIAL PALSY AS FIRST PRESENTATION OF ACUTE LYMPHOBLASTIC LEUKEMIA: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    S. Inaloo

    2008-11-01

    Full Text Available ObjectiveFacial paralysis in children is very often idiopathic and isolated facial nerve palsy, resulting from leukemic infiltration is a rare occurrence. Here we present the case of a 14 year-old boy with acute lymphobastic leukemia, who first presented with isolated right side peripheral facial nerve paralysis and was initially diagnosed with Bell's palsy.ConclusionThe presence of Bell's palsy in young children requires a complete evaluation, keeping in mind the possibility of leptomeningeal disease.Key words: Lymphoblastic Leukemia, Facial nerve palsy, Children.

  19. TORCH infection and cerebral palsy%TORCH感染与小儿脑性瘫痪

    Institute of Scientific and Technical Information of China (English)

    张蔚; 覃蓉

    2001-01-01

    @@Background: Cerebral palsy is a group of disorders that are related but probably have different causes. Children who have cerebral palsy acquire the disorder before or during or after birth. Objective: To discuss the relationship between the TORCH infection and cerebral palsy onset . Design : A TORCH screen is given to the children who have cerebral palsy in our hospital(1996.1~ 1998.6). A TORCH screen checks to see if the baby has been infected by any of the common causes.

  20. Cerebral palsy: social class differences in prevalence in relation to birthweight and severity of disability.

    OpenAIRE

    Dowding, V M; Barry, C.

    1990-01-01

    STUDY OBJECTIVE--The aim of the study was to examine the possible influence of social class on the prevalence of cerebral palsy. DESIGN--The study was a retrospective population based survey of all cases of cerebral palsy. SETTING--The study involved all cases of cerebral palsy born to residents in the Eastern Health Board area of the Republic of Ireland between 1976 and 1981 inclusive. PATIENTS--There were 289 cases of cerebral palsy during the study period. Thirty one were excluded because ...

  1. Postoperative recovery from posterior communicating aneurysm complicated by oculomotor palsy

    Institute of Scientific and Technical Information of China (English)

    YANG Ming-qi; WANG Shuo; ZHAO Yuan-li; ZHANG Dong; ZHAO Ji-zong

    2008-01-01

    Background Oculomotor palsy is a common complication in patients with posterior communicating aneurysm.This study was conducted to investigate the postoperative recovery of patients with posterior communicating aneurysm complicated with oculomotor palsy and to analyze the factors influencing length of recovery.Methods From 2000 to 2006,148 patients with posterior communicating aneurysm were treated at our hospital,with 74 of them having concurrent unilateral oculomotor palsy.All of the patients underwent craniotomy after the diagnosis by means of whole-brain digital subtraction angiography (DSA).The patients were divided into two groups for observation of postoperative recovery during the follow-up period.Patients in group A were treated with simple pedicle clipping of the aneurysm while patients in group B were treated with pedicle clipping of the aneurysm and decompression of the oculomotor nerve.Results Of the 40 patients in group A.20 underwent surgery within 14 days and completely recovered from oculomotor palsy in 10-40 days.Fourteen patients underwent surgery within 14-30 days.of whom 12 completely recovered within 30-90 days and 2 cases recovered incompletely.The remaining six patients underwent surgery after more than 30 days:of these.four patients recovered completely and two recovered incompletely.Of the 34 cases in group B,15 underwent surgery within 14 days and completely recovered from oculomotor palsy in 10-40 days.Sixteen patients underwent surgery in 14-30 days.of whom 14 completely recovered in 30-90 days and 2 recovered incompletely.The remaining three patients underwent surgery after more than 30 days,of whom two patients recovered completely and one recovered incompletely.Conclusions Early diagnosis and surgical treatment of patients with unilateral oculomotor palsy induced by posterior communicating aneurysm are important to full postoperative recovery of the oculomotor nerve.No correlation was found,however,between decompression of the

  2. Adults with cerebral palsy: a workshop to define the challenges of treating and preventing secondary musculoskeletal and neuromuscular complications in this rapidly growing population.

    Science.gov (United States)

    Tosi, Laura L; Maher, Nancy; Moore, D Winslow; Goldstein, Murray; Aisen, Mindy L

    2009-10-01

    Although the neurological injury associated with cerebral palsy (CP) is non-progressive, adults with the disorder often develop musculoskeletal and neurological symptoms, such as severe pain, chronic fatigue, and a premature decline in mobility and function, as they age. Little is known about how to manage, much less prevent, these symptoms. This paper summarizes the findings of a multi-disciplinary workshop, sponsored by the Cerebral Palsy International Research Foundation, the American Academy for Cerebral Palsy and Developmental Medicine, and Reaching for the Stars, convened to review current knowledge and begin to develop a blueprint for future research. The goals of the workshop were to (1) define the current incidence and prevalence of CP, (2) review the known complications for persons aging with CP, (3) review current understanding of physiological processes that may contribute to loss of function and premature aging in CP, (4) evaluate current treatment interventions in terms of long-term outcomes, (5) identify cutting-edge technologies in neurorehabilitation that may help prevent or treat the effects of accelerated aging for persons diagnosed with CP, and (6) identify strategies to ensure that individuals with CP receive evidence-based care as they transition from pediatric to adult-care services. PMID:19740204

  3. Bell's palsy: data from a study of 70 cases.

    Science.gov (United States)

    Cirpaciu, D; Goanta, C M

    2014-01-01

    Bell's palsy is a condition that affects the facial nerve, which is one of the twelve cranial nerves. Its main function is to control all the muscles of the facial expression. It is a unilateral, acute, partial or complete paralysis of the facial nerve. Bell's palsy remains the most common cause of facial nerve paralysis, more often encountered in females aged 17 to 30 years, recurrent in many cases and with poor associations with other pathologic conditions. In modern literature, the suspected etiology could be due to the reactivation of the latent herpes viral infections in the geniculate ganglia, and their subsequent migration to the facial nerve but, favorable outcome by using vasodilators, neurotrophic and corticosteroid therapy was recorded. PMID:25870668

  4. Clinical Trial of Erythropoietin in Young Children With Cerebral Palsy.

    Science.gov (United States)

    Cho, Kye Hee; Min, Kyunghoon; Lee, Seung Hoon; Lee, SunHee; An, SeongSoo A; Kim, MinYoung

    2016-09-01

    This study was conducted to assess the safety and efficacy of recombinant human erythropoietin in young children with cerebral palsy aged between 6 months and 3 years. All participants received subcutaneous recombinant human erythropoietin and 8 weeks of rehabilitation therapy. Adverse events, changes of vital signs, and hematologic tests were monitored up to 8 weeks postinjection. Functional measures of development at 4 and 8 weeks postinjection were compared with baseline values, and improvements were compared with those of an age-matched historical control group. Nine participants completed the trial from June 2012 to February 2015. No adverse events were related to recombinant human erythropoietin. Erythropoiesis was noted, although within normal range. Functional improvements were observed in all participants (P cerebral palsy. PMID:27233796

  5. A Diagnostic Approach for Cerebral Palsy in the Genomic Era

    OpenAIRE

    Lee, Ryan W; Poretti, Andrea; Cohen, Julie S.; Levey, Eric; Gwynn, Hilary; Johnston, Michael V.; HOON, ALEXANDER H; Fatemi, Ali

    2014-01-01

    An ongoing challenge in children presenting with motor delay/impairment early in life is to identify neurogenetic disorders with a clinical phenotype which can be misdiagnosed as cerebral palsy (CP). To help distinguish patients in these two groups, conventional magnetic resonance imaging (MRI) of the brain has been of great benefit in “unmasking” many of these genetic etiologies and has provided important clues to differential diagnosis in others. Recent advances in molecular genetics such a...

  6. Growth and Nutrition Disorders in Children with Cerebral Palsy

    OpenAIRE

    KUPERMINC, MICHELLE N; Stevenson, Richard D.

    2008-01-01

    Growth and nutrition disorders are common secondary health conditions in children with cerebral palsy (CP). Poor growth and malnutrition in CP merit study because of their impact on health, including psychological and physiological function, healthcare utilization, societal participation, motor function, and survival. Understanding the etiology of poor growth has led to a variety of interventions to improve growth. One of the major causes of poor growth, malnutrition, is the best-studied cont...

  7. Bruxism Control in a Child with Cerebral Palsy

    OpenAIRE

    Gloria Fernanda Castro; Laura Salignac Guimarães Primo; Maristela Barbosa Portela; Oliveira, Cristiana Aroeira G. R.; Viviane Andrade Cancio de Paula

    2010-01-01

    Cerebral palsy (CP) is one of the most severe childhood disabilities due to a lesion in the developing brain. Oral conditions often observed in this pathogenic are a tendency for the delayed eruption of permanent molars, higher percentages of malocclusion and parafunctional habits, including bruxism. The significance of oral conditions observed in CP patients demonstrates the need for intensive home and professional care for these individuals. This paper presents a 7-year-old boy, with cerebr...

  8. FEEDING, GROWTH AND NUTRITION DISORDERS IN CEREBRAL PALSY

    OpenAIRE

    Rosulescu Eugenia; Ilona Ilinca; Mihaela Zăvăleanu; Costin Nanu

    2009-01-01

    Objective: To evaluate the growth, physical development and nutrition status for a sample of cerebral palsied children with spastic, dyskinetic and ataxic type.Material and methods: A total of 81 children with CP, who were rehabilitated in the pediatrics rehabilitation clinic between 2005 - 2008 years, were included. Children’s assessments included: anthropometric measures (height H, recumbent length L, weight W), anthropometric indicators (weight fot length WL, body mass indexBMI) and was ca...

  9. Hemiplegic peripheral neuropathy accompanied with multiple cranial nerve palsy

    Directory of Open Access Journals (Sweden)

    Hirohisa Okuma

    2012-03-01

    Full Text Available A 32-year-old man experienced double vision around January, 2010, followed by weakness of his left upper and lower extremities. Articulation disorders and loss of hearing in his left ear developed, and he was admitted to our hospital on February 14, 2010. Physical examination was normal, and neurological examination showed clear consciousness with no impairment of cognitive function, but with articulation disorders. Olfactory sensation was reduced. Left ptosis and left gaze palsy, complete left facial palsy, perceptive deafness of the left ear, and muscle weakness of the left trapezius muscle were observed. Paresis in the left upper and lower extremities was graded 4/5 through manual muscle testing. Sensory system evaluation revealed complete left-side palsy, including the face. Deep tendon reflexes were slightly diminished equally on both sides; no pathologic reflex was seen. No abnormality of the brain parenchyma, cerebral nerves or cervicothoracolumbar region was found on brain magnetic resonance imaging. On electroencephalogram, alpha waves in the main frequency band of 8 to 9 Hz were recorded, indicating normal findings. Brain single photon emission computed tomography (SPECT scan showed reduced blood flow in the right inner frontal lobe and both occipital lobes. Nerve biopsy (left sural nerve showed reduction of nerve density by 30%, with demyelination. The patient also showed manifestations of multiple cranial nerve disorder, i.e., of the trigeminal nerve, glossopharyngeal nerve, vagus nerve, and hypoglossal nerve. Whole-body examination was negative. Finally, based on ischemic brain SPECT images, spinal fluid findings and nerve biopsy results, peripheral neuropathy accompanied with multiple cranial nerve palsy was diagnosed.

  10. Bilateral facial palsy as a manifestation of Japanese encephalitis

    OpenAIRE

    Verma, Rajesh; Praharaj, Heramba Narayan

    2012-01-01

    Japanese encephalitis is a cause of substantial morbidity and mortality, prevalent mainly in South East nations. It is caused by group B arbovirus and transmitted with bite of infected culex mosquitoes. The clinical features described are: headache, vomiting, altered sensorium, convulsions and both hypokinetic and hyperkinetic movement disorders. In this submission, we described a 68-year-old man suffering from Japanese encephalitis, who presented with bilateral facial palsy with encephalitic...

  11. Stigma Against Mental Illness and Cerebral Palsy in China

    OpenAIRE

    Shen, Liying

    2016-01-01

    This dissertation examines the stigmatization of two health conditions: mental disability and physical disability in the context of China. In particular, it addresses two main themes: the processes and impacts of stigma, and the variables that moderate the association of stigma with social attributes. The first paper applied a qualitative approach to identify the sources of burdens of raising a child with cerebral palsy in China and how stigma and “face” as a cultural factor affect childr...

  12. Cerebral venous thrombosis presenting as multiple lower cranial nerve palsies

    OpenAIRE

    Byju, N.; James Jose; Saifudheen, K; V Abdul Gafoor; P Jithendranath

    2012-01-01

    Cerebral venous thrombosis (CVT) is a well-recognized entity, but its clinical presentation is varied and often mimics many neurological disorders, making it a diagnostic challenge. Cerebral venous thrombosis has a wide spectrum of signs and symptoms, which may evolve suddenly or over weeks. It mimics many neurological conditions such as meningitis, encephalopathy, idiopathic intracranial hypertension, and stroke. Cerebral venous thrombosis presenting as multiple lower cranial nerve palsies, ...

  13. RESULTS OF ADDUCTORS MUSCLE TENOTOMY IN SPASTIC CEREBRAL PALSY

    OpenAIRE

    Guglielmetti, Luiz Gabriel Betoni; Santos, Ruy Mesquita Maranhao; Mendonça, Rodrigo Góes Medea de; Yamada, Helder Henzo; Assumpçao, Rodrigo Montezuma César de; Fucs, Patricia Maria de Moraes Barros

    2015-01-01

    Objective: Radiographic evaluation of the evolution of hips that underwent soft-tissue release. Methods: This was a retrospective evaluation on 101 spastic cerebral palsy patients who underwent soft-tissue release between 1991 and 2006. Forty-four patients met the inclusion criteria: 23 boys and 21 girls; 34 diparetic and 10 quadriparetic. Functionally, 29 were non-walkers, five were able to walk at home and 10 were able to walk within the community. Reimers' index (RI) and the acetabular ind...

  14. Aquatic exercise in the treatment of children with cerebral palsy

    OpenAIRE

    Dimitrijević Lidija; Bjelaković Bojko; Lazović Milica; Stanković Ivona; Čolović Hristina; Kocić Mirjana; Zlatanović Dragan

    2012-01-01

    Introduction. Aquatic exercise is one of the most popular supplementary treatments for children with neuro-motor impairment, especially for cerebral palsy (CP). As water reduces gravity force which increases postural stability, a child with CP exercises more easily in water than on land. Objective. The aim of the study was to examine aquatic exercise effects on gross motor functioning, muscle tone and cardiorespiratory endurance in children with spastic CP. Methods. The study included 1...

  15. Program Layanan Bimbingan Perilaku Seksual bagi Siswa Cerebral Palsy

    OpenAIRE

    Ari Wahyudi

    2016-01-01

    This colaborative action research aims to implement a counselling program to deal with the sexual behaviors of students with cerebral palsy. The program was developed collaboratively by the resercher and advisory teachers. Data were collected from observation to the students and interviuews with the students, teachers, and parents. The results of the qualitative analysis indicate that the counselling program helps effectively develop the students’ sexual behaviors in accordance with the local...

  16. Pulmonary sequestration cyst in a patient of cerebral palsy

    OpenAIRE

    Bilal Mirza; Muhammad Saleem; Lubna Ijaz; Arsalan Qureshi; Afzal Sheikh

    2011-01-01

    Pulmonary sequestration cyst is a rare entity in pediatric patients. Most of the time, it is diagnosed as an incidental finding. It is associated with other congenital anomalies, especially congenital diaphragmatic hernia. We report a patient of cerebral palsy presented with vomiting and recurrent chest infections. He was diagnosed to have hiatal hernia on computed tomography scan of chest. At operation, a pulmonary sequestration cyst along with hiatal hernia, malrotation, and meckel′s divert...

  17. Early Diagnosis and Early Intervention in Cerebral Palsy

    OpenAIRE

    Hadders-Algra, Mijna

    2014-01-01

    This paper reviews the opportunities and challenges for early diagnosis and early intervention in cerebral palsy (CP). CP describes a group of disorders of the development of movement and posture, causing activity limitation that is attributed to disturbances that occurred in the fetal or infant brain. Therefore, the paper starts with a summary of relevant information from developmental neuroscience. Most lesions underlying CP occur in the second half of gestation, when developmental activity...

  18. School process role for children with cerebral palsy

    OpenAIRE

    Žgur, Erna

    2015-01-01

    Abstract. Every child needs for successful school enteringspecific developmental maturation that includes developmental maturity oncognitive, emotional, social and motor area. Cerebral palsy (CP) at childcauses appearance of many difficulties that are shown on motor and otheractivities. Brain damage and also their associational connections cause a lotof defects, that are influencing on different school capabilities and skills:capability of thinking, concentration, listening, speech, reading,a...

  19. Pilot Study: Swimming for Children with Cerebral Palsy.

    OpenAIRE

    Declerck, Marlies; Daly, Daniel; Feys, Hilde

    2013-01-01

    The aim of this study was to investigate the effects of a swimming program on body function, activity and Quality of Life (QOL), in children diagnosed with Cerebral Palsy. Seven children (female/male: 4/3; Median 10.2 years old; Gross Motor Function Classification Scale I to III) participated in a 6-week swimming intervention using a case series design. Outcome measures were, for body function level: handgrip strength; for activity level: unimanual speed performance, walking capacity, gross m...

  20. Postural pattern recognition in children with unilateral cerebral palsy

    OpenAIRE

    Domagalska-Szopa M; Szopa A

    2014-01-01

    Małgorzata Domagalska-Szopa, Andrzej Szopa School of Health Sciences, Medical University of Silesia, Katowice, Poland Background: Several different strategies for maintaining upright standing posture in children with cerebral palsy (CP) were observed. Purpose: The purpose of the present study was to define two different postural patterns in children with unilateral CP, using moiré topography (MT) parameters. Additionally, another focus of this article was to outline some implicat...

  1. Gait improvement surgery in ambulatory children with diplegic cerebral palsy

    OpenAIRE

    Terjesen, Terje; Lofterød, Bjørn; Skaaret, Ingrid

    2015-01-01

    Background and purpose Instrumented 3-D gait analyses (GA) in children with cerebral palsy (CP) have shown improved gait function 1 year postoperatively. Using GA, we assessed the outcome after 5 years and evaluated parental satisfaction with the surgery and the need for additional surgery. Patients and methods 34 ambulatory children with spastic diplegia had preoperative GA. Based on this GA, the children underwent 195 orthopedic procedures on their lower limbs at a mean age of 11.6 (6–19) y...

  2. Neuroradiological and Neurophysiological Characteristics of Patients With Dyskinetic Cerebral Palsy

    OpenAIRE

    Park, Byung-Hyun; Park, Sung-Hee; Seo, Jeong-Hwan; Ko, Myoung-Hwan; Chung, Gyung-Ho

    2014-01-01

    Objective To investigate neuroradiological and neurophysiological characteristics of patients with dyskinetic cerebral palsy (CP), by using magnetic resonance imaging (MRI), voxel-based morphometry (VBM), diffusion tensor tractography (DTT), and motor evoked potential (MEP). Methods Twenty-three patients with dyskinetic CP (13 males, 10 females; mean age 34 years, range 16-50 years) were participated in this study. Functional evaluation was assessed by the Gross Motor Functional Classificatio...

  3. Gait maturation in children with cerebral palsy: A pilot study

    OpenAIRE

    J.S. Marques; P. Roquetti Fernandes; J. Fernandes Filho; F. B. Policarpo

    2011-01-01

    This study aimed at observing children with cerebral palsy's (CP) gait maturation and to correlate gait maturation's motor skill parameters. Podogram and video shooting of eight children's gait were used. Children up to seven years of age participated in the study. Cluster analysis was applied, dividing the sample into two groups, using the relation between pelvis’ width and the spreading of the ankles (REL) as parameters. Data were analyzed using t-test, analysis of Deltas, and Pearson’s cor...

  4. Platelet-Rich Plasma in a Patient with Cerebral Palsy

    OpenAIRE

    Alcaraz, Jesús; Oliver, Antonio; Sánchez, Juana María

    2015-01-01

    Patient: Male, 6 Final Diagnosis: Cerebral palsy secundary perinatal hypoxia Symptoms: Cognitive impairment • epilectic seizure Medication: Platelet rich plasma Clinical Procedure: Cognitive improvement with neuroestimulator and neuroregenerator power of platelet rich plasma injection Specialty: Hematology Objective: Unusual clinical course Background: The use of platelet-rich plasma is a now a common medical technique known as regenerative medicine, through power cell activation and differen...

  5. Therapeutic interventions for tone abnormalities in cerebral palsy

    OpenAIRE

    Tilton, Ann H.

    2006-01-01

    Cerebral palsy (CP) is a common cause of movement disorders in children. The upper motor neuron syndrome of CP leads to several types of muscle overactivity, including spasticity. Reduction of muscle overactivity may be an important treatment goal, to improve comfort, care, and active function and to prevent future musculoskeletal complications. After a comprehensive team evaluation, a treatment plan is generated. Treatments may include physical and occupational therapy, oral medications, bot...

  6. A case study on the Ayurvedic management of cerebral palsy

    OpenAIRE

    Sagar Mahendrabhai Bhinde

    2015-01-01

    Cerebral palsy (CP) is the leading cause of childhood disability affecting function and development. CP is defined as a nonprogressive neuromotor disorder of cerebral origin. It cannot be correlated with any single disease or condition in Ayurveda, as it is a multi-factorial disease with clinical features of a wide variation. According to Vāgbhaṭa, it is classified in the disease categories of sahaja (hereditary) and garbhaja (congenital) and jātaja (psychosomatic) type of diseases. Of the ma...

  7. Outpatient assessment of neurovisual functions in children with Cerebral Palsy

    OpenAIRE

    Barca, Laura; Cappelli, Francesca R.; Di Giulio, Paola; Staccioli, Susanna; Castelli, Enrico

    2010-01-01

    This study examined the feasibility of the Atkinson Battery for Child development for Examining Functional Vision (Atkinson, Anker, Rae, et al., 2002) to evaluate neurovisual functions of children with neurodevelopmental disorders in outpatient setting. A total of 90 patients underwent a comprehensive evaluation. Among these, a group of 33 children with Cerebral Palsy (CP), mean age 6 years, with different types of CP (26% diplegic, 37% hemiplegic and 37% tetraplegic) were selected to constit...

  8. Tibial Torsion in Cerebral Palsy: Validity and Reliability of Measurement

    OpenAIRE

    Lee, Sang Hyeong; Chung, Chin Youb; Park, Moon Seok; Choi, In Ho; Cho, Tae-Joon

    2009-01-01

    Physical examinations of tibial torsion are used for preoperative planning and to assess outcomes of tibial osteomy in patients with cerebral palsy (CP). The thigh-foot angle (TFA) and transmalleolar axis (TMA) are commonly used, and the second toe test recently was introduced. However, the validity and reliability of the three methods have not been clarified. This study was performed to evaluate the validity and reliability of these physical measures. We recruited 18 patients (36 limbs) with...

  9. Treatment of the spasticity in children with cerebral palsy.

    Science.gov (United States)

    Meholjić-Fetahović, Ajsa

    2007-11-01

    Botulinum toxin is a natural purified protein and one of the strongest biological poisons--neurotoxin. It is produced by the bacterium Clostridium botulinum. Its medical usage started in USA in 1981 and in Europe in 1992. There are seven different immune types of the toxin: A, B, C1, D, E, F and G. Toxin types A and B are used to decrease muscular spasticity. Botulinum toxin prevents the formation of acetylcholine from cholinergic nerve tissues in muscles, which in the end irreversibly destroys neuromuscular synapses. It is called temporary local chemodenervation. It does not affect the synthesis of acetylcholine. As it affects neuromuscular bond it also affects one of the symptoms of cerebral palsy--spasticity. Decreasing the spasticity of children with cerebral palsy leads to the improvement of conscious movements, muscles are less toned, passive mobility is improved, orthosis tolerance is also improved, and the child is enabled to perform easier and better motor functions such as crawling, standing and walking. Since the action of Botulinum toxin is limited to 2-6 months, new neural collaterals are formed and neuromuscular conductivity is reestablished which in the end once again develops a muscular spasm. This leads to a conclusion that botulinum toxin should again be applied into spastic muscles. It is very important for good effect of Botulinum toxin to set the goals of the therapy in advance. The goals include improvement of a function, prevention of contractions and deformities, ease of care and decrease of pain for children with cerebral palsy. After application of botulinum toxin, it is necessary to perform adequate and intensive physical treatment with regular monitoring of effects. This work shows a case of a boy with spastic form of cerebral palsy. After being rehabilitated using Vojta therapy and Bobath concept and the conduct of certain physical procedures, botulinum toxin is administered into his lower limbs' muscles and kinesiotherapy is

  10. Peroneal palsy after bariatric surgery: is nerve decompresion always necessary?

    Directory of Open Access Journals (Sweden)

    Ana M. Ramos-Leví

    2013-08-01

    Full Text Available We present two patients who underwent successful bariatric surgery and developed peroneal nerve palsy six months after the procedure. This is an unusual complication which determines a significant functional limitation, mainly because of foot drop, and its presence may be a hallmark of excessive and rapid weight loss. We discuss possible pathogenic mechanisms and therapeutic options, and we emphasize the important role of an adequate nutritional management, in order to avoid the need for a surgical nerve decompression.

  11. Food pattern and nutritional status of children with cerebral palsy

    Directory of Open Access Journals (Sweden)

    Patrícia Ayrosa C. Lopes

    2013-09-01

    Full Text Available OBJECTIVES To assess the food intake pattern and the nutritional status of children with cerebral palsy. METHODS Cross-sectional study with 90 children from two to 12.8 years with cerebral palsy in the following forms: hemiplegia, diplegia, and tetraplegia. Nutritional status was assessed by weight, height, and age data. Food intake was verified by the 24-hour recall and food frequency questionnaire. The ability to chew and/or swallowing, intestinal habits, and physical activity were also evaluated. RESULTS For 2-3 year-old age group, the mean energy intake followed the recommended range; in 4-6 year-old age group with hemiplegia and tetraplegia, energy intake was below the recommended limits. All children presented low intake of carbohydrates, adequate intake of proteins and high intake of lipids. The tetraplegia group had a higher prevalence of chewing (41% and swallowing (12.8% difficulties compared to 14.5 and 6.6% of children with hemiplegia, respectively. Most children of all groups had a daily intestinal habit. All children presented mild physical activity, while moderate activity was not practiced by any child of the tetraplegia group, which had a significantly lower height/age Z score than those with hemiplegia (-2.14 versus -1.05; p=0.003. CONCLUSIONS The children with cerebral palsy presented inadequate dietary pattern and impaired nutritional status, with special compromise of height. Tetraplegia imposes difficulties regarding chewing/swallowing and moderate physical activity practice.

  12. Intrathecal baclofen for management of spastic cerebral palsy: multicenter trial.

    Science.gov (United States)

    Gilmartin, R; Bruce, D; Storrs, B B; Abbott, R; Krach, L; Ward, J; Bloom, K; Brooks, W H; Johnson, D L; Madsen, J R; McLaughlin, J F; Nadell, J

    2000-02-01

    Intrathecal baclofen infusion has demonstrated effectiveness in decreasing spasticity of spinal origin. Oral antispasticity medication is minimally effective or not well tolerated in cerebral palsy. This study assessed the effectiveness of intrathecal baclofen in reducing spasticity in cerebral palsy. Candidates were screened by randomized, double-blind, intrathecal injections of baclofen and placebo. Responders were defined as those who experienced an average reduction of 1.0 in the lower extremities on the Ashworth Scale for spasticity. Responders received intrathecal baclofen via the SynchroMed System and were followed for up to 43 months. Fifty-one patients completed screening and 44 entered open-label trials. Lower-extremity spasticity decreased from an average baseline score of 3.64 to 1.90 at 39 months. A decrease in upper extremity spasticity was evidenced over the same study period. Forty-two patients reported adverse events. Most common reports were hypotonia, seizures (no new onset), somnolence, and nausea or vomiting. Fifty-nine percent of the patients experienced procedural or system-related events. Spasticity in patients with cerebral palsy can be treated effectively by continuous intrathecal baclofen. Adverse events, although common, were manageable. PMID:10695888

  13. Improved Quality of Life in A Case of Cerebral Palsy after Bone Marrow Mononuclear Cell Transplantation.

    Science.gov (United States)

    Sharma, Alok; Sane, Hemangi; Kulkarni, Pooja; D'sa, Myola; Gokulchandran, Nandini; Badhe, Prerna

    2015-01-01

    Cerebral palsy (CP) is a non progressive, demyelinating disorder that affects a child's development and posture and may be associated with sensation, cognition, communication and perception abnormalities. In CP, cerebral white matter is injured resulting in the loss of oligodendrocytes. This causes damage to the myelin and disruption of nerve conduction. Cell therapy is being explored as an alternate therapeutic strategy as there is no treatment currently available for CP. To study the benefits of this treatment we have administered autologous bone marrow mononuclear cells (BMMNCs) to a 12-year-old CP case. He was clinically re-evaluated after six months and found to demonstrate positive clinical and functional outcomes. His trunk strength, upper limb control, hand functions, walking stability, balance, posture and coordination improved. His ability to perform activities of daily living improved. On repeating the Functional Independence Measure (FIM), the score increased from 90 to 113. A repeat positron emission tomography-computed tomography (PET-CT) scan of the brain six months after intervention showed progression of the mean standard deviation values towards normalization which correlated to the functional changes. At one year, all clinical improvements have remained. This indicated that cell transplantation may improve quality of life and have a potential for treatment of CP. PMID:26199918

  14. Improved Quality of Life in A Case of Cerebral Palsy after Bone Marrow Mononuclear Cell Transplantation

    Directory of Open Access Journals (Sweden)

    Alok Sharma

    2015-07-01

    Full Text Available Cerebral palsy (CP is a non progressive, demyelinating disorder that affects a child’s development and posture and may be associated with sensation, cognition, communication and perception abnormalities. In CP, cerebral white matter is injured resulting in the loss of oligodendrocytes. This causes damage to the myelin and disruption of nerve conduction. Cell therapy is being explored as an alternate therapeutic strategy as there is no treatment currently available for CP. To study the benefits of this treatment we have administered autologous bone marrow mononuclear cells (BMMNCs to a 12-year-old CP case. He was clinically re-evaluated after six months and found to demonstrate positive clinical and functional outcomes. His trunk strength, upper limb control, hand functions, walking stability, balance, posture and coordination improved. His ability to perform activities of daily living improved. On repeating the Functional Independence Measure (FIM, the score increased from 90 to 113. A repeat positron emission tomography- computed tomography (PET-CT scan of the brain six months after intervention showed progression of the mean standard deviation values towards normalization which correlated to the functional changes. At one year, all clinical improvements have remained. This indicated that cell transplantation may improve quality of life and have a potential for treatment of CP.

  15. Cerebral Palsy in 1-12 Year Old Children in Southern Iran

    Science.gov (United States)

    INALOO, Soroor; KATIBEH, Pegah; GHASEMOF, Masroor

    2016-01-01

    Objective Cerebral palsy (CP) is a non-progressive CNS disorder due to an insult to the growing brain, usually occurring in the first two years of life. During the recent years, its etiology has been changed; perinatal and postnatal insults are not considered as its main causes in developed countries any more. The aim of this study was to evaluate the causes of CP in children in southern Iran. Materials & Methods Overall, 200 children with CP aged 1-12 yr old referring to Pediatric Neurology Clinic affiliated to Shiraz University of Medical Sciences, Shiraz, Iran between 2012 and 2013 were enrolled. In addition, 200 healthy age and sex-matched children were considered as the control group. Exclusion criteria were isolated movement disorders with no other evidence of CP, progressive neurologic disorders, metabolic disorders, and incomplete or uncertain past history. After collecting the data on pregnancy period, prenatal history and past medical problems, they were analyzed with appropriate statistical methods. Results Maternal age, medical problems during pregnancy period, route of delivery, head circumference at birth, neonatal admission, neonatal jaundice, and prematurity were the main risk factors for CP. Discussion The distribution of risk factors of CP is different from that of developed countries in our region. Pre- and peri-natal etiologies are still among the common causes of CP in Iran. PMID:27057186

  16. A three-generation family with idiopathic facial palsy suggesting an autosomal dominant inheritance with high penetrance

    DEFF Research Database (Denmark)

    Larsen, Christian Grønhøj; Gyldenløve, Mette; Jønch, Aia Elise;

    2015-01-01

    Idiopathic facial palsy (IFP), also known as Bell's palsy, is a common neurologic disorder, but recurrent and familial forms are rare. This case series presents a three-generation family with idiopathic facial palsy. The mode of inheritance of IFP has previously been suggested as autosomal dominant...

  17. A 3-year review of cranial nerve palsies from the University of Port Harcourt Teaching Hospital Eye Clinic, Nigeria

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    Chinyere Nnenne Pedro-Egbe

    2014-01-01

    Conclusion: This is the first study in the literature on ocular cranial nerve palsies in Southern Nigeria. Third and sixth cranial nerve palsies were the most common cases to present to the University of Port Harcourt Teaching Hospital Eye Clinic. There was a statistically significant association to systemic disorders such as hypertension and DM and majority of cases with 6 th cranial nerve palsy.

  18. Effectiveness of Neurogenesis in treating Children with Cerebral Palsy

    Directory of Open Access Journals (Sweden)

    Susan AMIRSALARI

    2012-06-01

    Full Text Available How to Cite this Article: Amirsalary S, Dehghan L, Dalvand H, Haghgoo H. Effectiveness of Neurogenesis in treating children with Cerebral Palsy. Iran J Child Neurol 2012;6(2:1-8. objectiveTissue-specific stem cells divide to generate different cell types for the purpose oftissue repair in the adult. The aim of this study was to detect the significance ofneurogenesis in the central nervous system in patients with cerebral palsy (CP.Materials & MethodsA search was made in Medline, CINAHL, PubMed, ISI Web of Science andGoogle Scholar from 1995 to February 2011. The outcomes measured in thereview were classified to origins, proliferation, and migration of new neurons,and neurogenesis in CP.ResultsAccording to the review of articles, neurogenesis persists in specific brainregions throughout lifetime and can be enhanced from endogenous progenitorcells residing in the subventricular zone by growth factors or neurotrophicfactors and rehabilitation program.ConclusionMost of the studies have been conducted in the laboratory and on animals,more work is required at the basic level of stem cell biology, in the developmentof human models, and finally in well-conceived clinical trials. References1. Buonomano DV, Merzenich MM. Cortical plasticity: from synapses to maps. Annu Rev Neurosci 1998; 21:149-86.2. Haghgoo H. Fundemental of neurosciences. 1st ed. Tehran; USWR Press; 2011.3. Payne BR, Lomber SG. Reconstructing functional systems after lesions of cerebral cortex. Nat Rev Neurosci 2001 Dec;2(12:911-9.4. Bax M, Goldstein M, Rosenbaum P, Leviton A, Paneth N, Dan B, et al. Proposed definition and classification of cerebral palsy. Dev Med Child Neurol 2005 Apr;47(8:571-6.5. Joghataei M, Kazem M. Barresi sathe niazhaie jamee be khadamate behzisti colle keshvar [persian].Tehran: University of. Social Welfare and Rehabilitation Sciences; 1990.6. Johnson A. Prevalence and characteristics of children with cerebral palsy in Europe. Dev Med Child Neurol

  19. Advances in diagnosis and non-surgical treatment of Bell's palsy

    Institute of Scientific and Technical Information of China (English)

    Yang Zhao; Guodong Feng; Zhiqiang Gao

    2015-01-01

    Bell's palsy is a commonly seen cranial nerve disease and can result in compromised facial appearance and functions. Its etiology, prognosis and treatment are still being debated. This paper is a review of recent development in the understanding of etiology, diagnosis and non-surgical treatment of Bell's palsy.

  20. Handling the Cerebral Palsied Child: Multi-Level Skills Transfer in Pakistan.

    Science.gov (United States)

    Miles, M.; Frizzell, Yvonne

    1990-01-01

    The majority of children with cerebral palsy in developing countries have no access to trained therapists; for example, in Pakistan, there is less than one trained general physiotherapist per million population. In Pakistan, cerebral palsy handling skills were taught to a group of parents, teachers, and paraprofessionals in a series of practical…