Cases reported "Chorea"

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21/279. Acute onset of chorea and dystonia following a febrile illness in a 1-year-old boy.

    A 12-month-old boy with acute onset hemichorea and dystonia following a gastroenteritis has abnormal signal intensities of his basal ganglia on brain magnetic resonance imaging (MRI). A rigorous laboratory investigation is successful in diagnosing his rare condition. A discussion of the differential of abnormal basal ganglia on MRI is presented to help illustrate this case.
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22/279. chorea in juvenile primary antiphospholipid syndrome. Reversible decreased circulation in the basal ganglia visualised by single photon emission computed tomography.

    chorea was observed in a 12-year-old girl with primary antiphospholipid syndrome (APS). She developed severe chorea in a few weeks. On immunosuppressive treatment, including high doses of glucocorticoids and cyclophosphamide, she had a rapid clinical recovery. Single photon emission computed tomography (SPECT) of the brain showed decreased circulation in the basal ganglia and in the medial parts of both temporal lobes. One month after treatment, SPECT was completely normalised. APS in children has a variety of clinical manifestations, and should be suspected in cases of unexplained thromboembolic disease or obscure neurological symptoms.
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23/279. Non-ketotic hyperglycemia in a young woman, presenting as hemiballism-hemichorea.

    We report a 22-year-old girl presenting with acute onset left sided hemiballism-hemichorea (HH) and non-ketotic hyperglycemia (NKH). Initial brain CT revealed faint hyperdensities, sharply confined to the contralateral nucleus caudatus and putamen. Sequential MRI investigations yielded increasing hypersignal intensities on T1-weighted images and resolving hypodensities on T2-weighted images of the right striatum, leaving small sequelae in the head of the right caudate nucleus. NKH is an unusual cause of HH. The abnormalities seen in neuroimaging are rare, but seem to be quite specific to this syndrome. We give an update on current literature regarding the possible pathophysiological processes underlying this specific clinical entity.
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24/279. Pseudochoreoathetosis in four patients with hypesthetic ataxic hemiparesis in a thalamic lesion.

    Thalamic lesions give rise to a variety of clinical syndromes including choreoathetotic movements and ataxic hemiparesis as well as sensory deficits. We describe four patients exhibiting pseudochoreoathetosis, hypesthesia, and ataxic hemiparesis in the limbs contralateral to a thalamic lesion. Three of the four patients showed the involuntary movements within 10 days of stroke onset; the remaining patient was not seen until 4 years later. Three had infarction and the other one hemorrhage in the posterior and lateral thalamus. All the patients had both cerebellar and sensory ataxia. These cases suggest that failure to convey proprioceptive information be the basic pathophysiology of pseudochoreoathetosis.
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25/279. A combined pattern of movement disorders resulting from posterolateral thalamic lesions of a vascular nature: a syndrome with clinico-radiologic correlation.

    We report a series of seven patients in whom a combined pattern of complex movement disorders restricted to one upper extremity emerged as a result of posterolateral thalamic lesions of vascular origin. This disorder was mainly characterized by choreiform and dystonic movements associated with variable, rhythmic, alternating movements of low frequency (myorhythmia). All cases showed, on computed tomography scan and/or magnetic resonance imaging, focal lesions involving the posterolateral quadrant of the thalamus. review of similar cases reported with identical clinico-radiologic features allows us to conclude that it is possible to establish an accurate anatomoclinical correlation based on the clinical phenomenology, even before imaging studies are performed, in these cases. The opposite is not entirely possible, however, because lesions in the same quadrant of the thalamus are often associated with different patterns of abnormal movements or present without abnormal movements.
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26/279. Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome.

    Huntington's disease is characterised by hyperkinetic movements, mainly chorea, cognitive dysfunction, and psychiatric abnormalities. Non-dopa responsive parkinsonism occurs in the later stages of choreic disease or as the predominant feature of juvenile patients (Westphal variant). Late onset Huntington's disease presenting as levodopa responsive parkinsonism is rare. A series of four patients with late onset Huntington's disease presenting as levodopa responsive parkinsonism and cardiovascular dysautonomia, initially misdiagnosed as multiple system atrophy (MSA) in three patients, is reported. levodopa treatment did not unmask significant chorea. These cases suggest the presence of a distinct phenotypic variant of Huntington's disease to be added to the differential diagnosis of other akinetic rigid syndromes.
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27/279. Gabapentin treatment in a child with delayed-onset hemichorea/hemiballismus.

    A 13-year, 6-month-old female was evaluated for subacute onset of left-sided hemichorea/hemiballismus, with an old, right parietal, cortical, and subcortical stroke as the presumed cause. Treatment with gabapentin was initiated, with good results at 6-month follow-up. Discussion of the differential diagnosis and evaluation of delayed-onset movement disorders in children and the mechanism of action of gabapentin is included.
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28/279. Paroxysmal dystonic choreoathetosis: clinical features and investigation of pathophysiology in a large family.

    Paroxysmal dystonic choreoathetosis (PDC) is an unusual hyperkinetic movement disorder characterized by attacks of chorea, dystonia, and ballism with onset in childhood. We report a large British family with dominantly inherited PDC linked to chromosome 2q and describe the clinical features in 20 affected family members. Attacks were precipitated by a variety of factors, including caffeine, alcohol, or emotion, and could be relieved by short periods of sleep in most subjects. The clinical features in the family are compared with those of 11 other PDC families in the literature and a core phenotype for PDC suggested. CSF monoamine metabolites measured at baseline and during an attack in one subject were found to increase during the attack. magnetic resonance spectroscopy of brain and basal ganglia performed both during and between attacks was normal. Positron emission tomography using the D2 receptor ligand, 11C-raclopride, showed no abnormalities.
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29/279. Antiphospholipid- associated recurrent chorea and ballism in a child with cerebral palsy.

    We present the case of a 9-year-old female with cerebral palsy and repeated episodes of ballism associated with antiphospholipid and anticardiolipin antibodies. She was treated unsuccessfully with varying medications, including neuroleptics, anticholinergics, antiepileptics, dopamine, dopamine agonists, and monoamine oxidase inhibitors. Intravenous immunoglobulin and corticosteroids led to resolution of the movements. We postulate an immune mechanism of disease for ballism associated with antiphospholipid and anticardiolipin antibodies.
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30/279. role of vitamin e in rheumatic chorea.

    Rheumatic chorea is the sole neurologic manifestation of rheumatic fever. It is a debilitating illness lasting for weeks to months. Drugs like diazepam, haloperidol, chlorpromazine take four to six weeks for functional improvement and can cause serious side effects. The authors investigated the role of vitamin e in reducing rheumatic chorea. A case series of patients of rheumatic chorea were administered vitamin e in the dose 50 IU daily for fifteen days. The various clinical signs of rheumatic chorea were scored with MAIMS score (Modified Abnormal Involuntary Movement Scale score) which is used for tardive dyskinesia. No other drug for abnormal movements was used. In all the 4 patients who received vitamin e, there was remarkable change by 7th day and almost complete functional improvement by 14th day. vitamin e is safer than the conventional drugs used for chorea in children. It was found effective in this case series. Its role needs further evaluation by a double-blind randomized controlled trial.
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