Cases reported "Movement Disorders"

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1/31. Bilateral ballism in a patient with overlapping Fisher's and Guillain-Barre syndromes.

    A 29 year old woman developed diplopia and ataxic gait. Neurological examination showed total ophthalmoplegia, cerebellar ataxia, and areflexia. Moreover, there was muscle weakness in all four limbs. An overlap of Fisher's and Guillain-Barre syndromes was dignosed. On day 5 she suddenly developed involuntary flinging movements that affected the face and four limbs. Surface EMG showed 1.5-2 Hz rhythmic grouping discharges. The involuntary movements were considered ballism. This is the first report of a patient with guillain-barre syndrome and a related disorder who showed ballism.
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ranking = 1
keywords = ophthalmoplegia
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2/31. Familial dysequilibrium-diplegia with T-lymphocyte deficiency.

    A second family is described with a combination of defective thymus-dependent immunity and cerebral palsy. The cerebral palsy comprised nonprogressive dysequilibrium and mild spastic diplegia without limb ataxia. This genetic entity of presumed autosomal recessive inheritance is clearly distinguished from ataxia-telangiectasia. Immunological abnormalities should be sought in other familial or unexplained cerebral palsy syndromes.
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ranking = 0.099959537067346
keywords = palsy
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3/31. Ballistic movements of the arm in systemic lupus erythematosis.

    The case of a 24-year old woman with systemic lupus erythematosis and central nervous system disease manifested by ballistic movements of the left arm is presented. She also had bilateral cerebral infarcts with pseudobulbar palsy. Ballismus is not generally known to be a manifestation of systemic lupus although there have been been reports of chorea in this disease which has responded to treatment with corticosteroids. In the present case, the ballismus disappeared when very large doses of steroids were given. This suggests that massive doses of corticosteroids may be needed for effective treatment of central nervous system lupus.
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ranking = 0.033319845689115
keywords = palsy
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4/31. An unusual family with multiple movement disorders.

    Multiple movement disorders presenting in the same family are rare. We present an unusual family where generalized dystonia, Huntington's disease, progressive supranuclear palsy and secondary paroxysmal dyskinesia co-exist. The index case presented with young-onset dystonia and tested negative for the DYT1 gene deletion. Her father was similarly affected. The father's brother (paternal uncle of the index) also had abnormal movements-a mixture of chorea and dystonia-and tested positive for the HD expansion. His son had secondary paroxysmal dyskinesia, and tested negative for the HD expansion. The index case and her father were also negative for the HD expansion. A paternal aunt of two of the cases had a clinical diagnosis of progressive supranuclear palsy.dystonia is known to be a genetically heterogeneous condition. The co-existence of inherited generalized dystonia with other movement disorders may provide clues to its genetic localization.
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ranking = 394.44310192743
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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5/31. Bobble-head doll syndrome: some atypical features with a new lesion and review of the literature.

    Bobble-head doll syndrome is a rare and unique movement disorder encountered in children. It is characterized by continuous or episodic involuntary forward and backward and side to side movement of the head at the frequency of 2-3 Hz. neuroimaging in most of the cases reveals third ventricular tumors, suprasellar arachnoid cysts, aqueductal stenosis and other lesions in the region of the third ventricle along with communicating hydrocephalus. In most of the circumstances, the problem starts in the first decade of life and diversion of cerebrospinal fluid by shunt operation is very often accompanied by dramatic improvement. We report one case where bobbing of the head started at around 12 years of age. Additionally, there was evidence of partial left abducens nerve palsy, tremor in the outstretched hands, difficulty in finger-nose test and tandem walking, hyperreflexia and extensor plantar response. He was unconscious on two occasions and there was evidence of gross hydrocephalus along with a thin membranous web, running transversely across the lower part of the aqueduct of Sylvius without any cerebrospinal fluid flow void. Ventriculo-peritoneal shunt abolished the abnormal movements. We propose that the aqueductal web was the offending agent for the pathogenesis of bobble-head doll syndrome in our case and this lesion has not been identified in the cases reported so far. Relevant literature in this regard has also been reviewed.
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ranking = 0.033319845689115
keywords = palsy
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6/31. Motor outcome differences between two groups of children with spastic diplegia who received different intensities of early onset physiotherapy followed for 5 years.

    The objective of this study is to determine the clinical effectiveness of early onset long-term intensive physiotherapy on motor development in children with spastic diplegic cerebral palsy (CP). The study was a non-randomized cohort study with 62 months (mean) follow-up. The participants were ten infants who were first examined before 3 months of age corrected for prematurity. All had a gestational age of less than 33 weeks and a birth weight of less than 2000 g. Brain magnetic resonance imaging revealed periventricular white matter injury in nine subjects and moderate grade bilateral porencephaly in one. Five completed a full course of training of 52 months (mean), two did not receive therapy, and three received an insufficient course of therapy. The study was conducted at the Regional Center for Children with Disabilities including outpatient clinics and a school for children with special needs. The Vojta Method was used, which is an extensive family oriented physiotherapy program which uses isometric strengthening of muscles with tactile stimulation. Subjects were evaluated for the highest motor developmental level at the outcome evaluation 59 months (mean) after initiation of therapy. Four of the five who completed training could either stand still for 5 s or walk at the time of the outcome evaluation 52 months after the beginning of the therapy program. None of the five subjects with no training or insufficient training could accomplish this task when evaluated 64 months following therapy initiation. This was a statistically significant difference (P = 0.0278). A consistently applied physiotherapy program resulted in better motor outcomes in this group of children at risk for developing spastic diplegic CP.
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ranking = 0.033319845689115
keywords = palsy
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7/31. Primary progressive freezing gait: a syndrome with many causes.

    Primary progressive freezing gait (PPFG) is characterized by early gait freezing and a stereotyped progression. Of nine patients followed up for 6 to 16 years, two were diagnosed pathologically: pallidonigroluysian degeneration (PNLD) and diffuse lewy body disease. Four others evolved clinically into progressive supranuclear palsy and corticobasal degeneration. PPFG is not a distinct disorder but a syndrome with diverse causes. Long-term follow-up (> or =10 years) and postmortem are required for accurate diagnosis. PNLD may be the primary form of disease.
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ranking = 197.22155096372
keywords = progressive supranuclear, supranuclear, supranuclear palsy, palsy
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8/31. The adult form of Niemann-Pick disease type C.

    Niemann-Pick disease type C (NPC) is a fatal neurovisceral lipid storage disease of autosomal inheritance resulting from mutations in either the NPC1 (95% of families) or NPC2 gene. The encoded proteins appear to be involved in lysosomal/late endosomal transport of cholesterol, glycolipids and other molecules but their exact function is still unknown. The clinical spectrum of the disease ranges from a neonatal rapidly fatal disorder to an adult-onset chronic neurodegenerative disease. Based upon a comprehensive study of 13 unrelated adult patients diagnosed in france over the past 20 years as well as the analysis of the 55 other cases published since 1969, we have attempted to delineate the major clinical, radiological, biochemical and genotypic characteristics of adult NPC. overall, mean age at onset ( /-SD) of neuropsychiatric symptoms was 25 /- 9.7 years. The diagnosis of NPC was established after a mean delay of 6.2 /- 6.4 years and the mean age at death (calculated from 20 cases) was 38 /- 10.2 years. Major clinical features included cerebellar ataxia (76%), vertical supranuclear ophthalmoplegia (VSO, 75%), dysarthria, (63%), cognitive troubles (61%), movement disorders (58%), splenomegaly (54%), psychiatric disorders (45%) and dysphagia (37%). Less frequent signs were epilepsy and cataplexy. During the course of the disease, clinical features could be subdivided into (i) visceral signs (hepatomegaly or splenomegaly), (ii) cortical signs (psychiatric cognitive disorders and epilepsy); and (iii) deep brain signs (VSO, ataxia, movement disorders, dysarthria, dysphagia, cataplexy) which exhibited different evolution patterns. Asymptomatic and non-evolutive visceral signs were often noticed since early childhood (38.5% of our patients), followed by mild cortical signs in childhood (learning difficulties) and early adulthood (62% of cases among which 38% were psychiatric disorders). Deep brain signs were observed in 96% of patients and were usually responsible for death. In general, there was a good correlation between clinical signs and the localization of brain atrophy on MRI. The 'variant' biochemical phenotype characterized by mild abnormalities of the cellular trafficking of endocytosed cholesterol was over-represented in the adult form of NPC and seemed associated with less frequent splenomegaly in childhood and lesser psychiatric signs. Involvement of the NPC1 gene was shown in 33 families and of the NPC2 gene in one. Improving the knowledge of the disease among psychiatrists and neurologists appears essential since emerging treatments should be more efficient at the visceral or cognitive/psychiatric stages of the disease, before the occurrence of widespread deep brain neurological lesions.
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ranking = 59.779740284986
keywords = supranuclear, ophthalmoplegia
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9/31. Alexander's disease: a report and reappraisal.

    A boy with juvenile onset of symptoms of Alexander's disease had a clinical picture of pseudobulbar palsy, ataxia, and spastic paraparesis. Pathologic examination of the central nervous system revealed the diffuse presence of Rosenthal fibers and patchy demyelination. This may be the first report of a case with significant neuronal changes and inflammation within the brain stem in this disease. The clinical picture of this entity is varied and age-dependent. Alexander's disease may be a motor system specific entity secondary to astrocytic dysfunction.
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ranking = 0.033319845689115
keywords = palsy
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10/31. Pure akinesia: an atypical manifestation of progressive supranuclear palsy.

    Two patients with "pure akinesia" who showed the characteristic changes of progressive supranuclear palsy (PSP) at necropsy are described. They had akinesia but no rigidity or tremor, and ophthalmoplegia was not observed during the course of illness. The symptoms of "pure akinesia" was not improved by levodopa therapy but was considerably improved by L-threo-3,4-dihydroxy-phenylserine. At necropsy, pathological findings were not different from those reported for PSP. It is suggested that "pure akinesia" is an atypical manifestation of PSP, and that norepinephrinergic neurons may be involved in some types of PSP.
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ranking = 987.10775481858
keywords = progressive supranuclear, supranuclear, supranuclear palsy, ophthalmoplegia, palsy
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