Cases reported "Athetosis"

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11/171. A new neurological syndrome with mental retardation, choreoathetosis, and abnormal behavior maps to chromosome Xp11.

    Choreoathetosis is a major clinical feature in only a small number of hereditary neurological disorders. We define a new X-linked syndrome with a unique clinical picture characterized by mild mental retardation, choreoathetosis, and abnormal behavior. We mapped the disease in a four-generation pedigree to chromosome Xp11 by linkage analysis and defined a candidate region containing a number of genes possibly involved in neuronal signaling, including a potassium channel gene and a neuronal G protein-coupled receptor. ( info)

12/171. Circumferential cervical surgery for spondylostenosis with kyphosis in two patients with athetoid cerebral palsy.

    BACKGROUND: patients with athetoid cerebral palsy may develop severe degenerative changes in the cervical spine decades earlier than their normal counterparts due to abnormal cervical motion. methods: Two patients, 48 and 52 years of age, presented with moderate to severe myelopathy (Nurick Grades IV and V). MR and 3-dimensional CT studies demonstrated severe spondylostenosis with kyphosis in both patients. This necessitated multilevel anterior corpectomy with fusion (C2-C7, C3-C7) using fibula and iliac crest autograft and Orion plating, followed by posterior wiring, fusion using Songer cables, and halo placement. RESULTS: Postoperatively, both patients improved, demonstrating only mild or mild to moderate (Nurick Grades II and III) residual myelopathy. Although both fused posteriorly within 3.5 months, the patient with the fibula graft developed a fracture of the anterior C7 body with mild anterior graft migration, and inferior plate extrusion into the C7-T1 interspace. However, because he has remained asymptomatic for 9 months postoperatively, without dysphagia, removal of the plate has not yet been necessary. CONCLUSIONS: patients with athetoid cerebral palsy should undergo early prospective cervical evaluations looking for impending cord compromise. When surgery is indicated, circumferential surgery offers the maximal degree of cord decompression and stabilization with the highest rate of fusion. ( info)

13/171. 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. ( info)

14/171. Chronic cerebellar stimulation in cerebral palsy.

    Data are presented for the first 50 patients with cerebral palsy who underwent chronic cerebellar stimulation for symptom alleviation. We observed significant shorter and longer term improvement in spasticity as well as athetosis, speech, and functional status. Continuing increments in improvement were noted as a function of time on stimulation. In many instances, psychometric test scores and behavior also were improved. There was one death in this series. There were no neurologic complications due to cerebellar stimulation. The results of this study warrant the judicious use of cerebellar stimulation for symptomatic and functional relief in cerebral palsy. ( info)

15/171. Recurrent artery of Heubner infarction in infancy.

    Classically, acquired occlusion of the recurrent artery of Heubner (RAH) results in hemiparesis with faciobrachial predominance. infarction in the territory of the RAH represents a specific stroke syndrome not yet described in infancy with a range of motor and functional manifestations. An infant is described with apparent congenital infarction of the recurrent artery of Heubner. The child had prominent involvement of the contralateral upper extremity with athetosis. neuroimaging changes were evident in the vascular territory classically attributed to the RAH. The clinician should suspect congenital RAH infarction in those infants with congenital upper-extremity athetosis. ( info)

16/171. 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. ( info)

17/171. Acute neurologic decompensation in an infant with cobalamin deficiency exposed to nitrous oxide.

    After exposure to nitrous oxide, an infant with unrecognized cobalamin deficiency developed neurologic deterioration and pancytopenia. Hematologic recovery and partial resolution of his neurologic changes followed repletion of the vitamin. nitrous oxide depletes bioavailable cobalamin and may be a dangerous anesthetic in patients with cobalamin deficiency. ( info)

18/171. Possible mechanisms in infants for selective basal ganglia damage from asphyxia, kernicterus, or mitochondrial encephalopathies.

    magnetic resonance imaging and neuropathologic studies have demonstrated remarkably selective patterns of injury to subregions of the basal ganglia in children. Examples are kernicterus and certain mitochondrial encephalopathies, which cause selective injury to the globus pallidus, and near-total perinatal asphyxia, which causes lesions in the putamen and thalamus. To explain the differential vulnerability of nuclei within millimeters of each other, we hypothesize that their locations within the neurotransmitter-specific circuitry of the basal ganglia motor loop are important. In severe hypoxic-ischemic encephalopathy, excitatory glutamatergic pathways into the putamen and thalamus are overactive, but the globus pallidus might be protected because its activity is silenced by inhibitory neuronal activity. In contrast, the relatively high resting neuronal activity in the globus pallidus might make it more vulnerable to less intense, subacute oxidative stresses from mitochondrial toxins such as bilirubin or from genetic mitochondrial disorders. This hypothesis has implications for designing neuroprotective therapies and for treating associated chronic movement disorders. ( info)

19/171. Hemidystonia and hemichoreoathetosis as an initial manifestation of moyamoya disease.

    OBJECTIVE: To describe hemidystonia and hemichoreoathetosis in an adult patient with moyamoya disease without a previous history of cerebrovascular accident. DESIGN: Case report. SETTING: Tertiary care center. PATIENT: A 22-year-old woman suddenly developed dystonic spasms in her left hand and left foot after a severe emotional stress. The dyskinesia gradually subsided over the next 4 months. Five months after the onset, she suddenly developed choreoathetoid movement in her right hand and right foot. MAIN OUTCOME AND RESULTS: The patient had both somatic and cortical sensory deficits in the right hand and right foot. magnetic resonance imaging of the brain showed an infarction at the right putamen and lesions involving the right frontal lobe and the left frontotemporoparietal lobe. Magnetic resonance cerebral angiography showed severe stenoses of both internal carotid arteries at the supraclinoid portion and numerous collateral vessels, compatible with moyamoya disease. Single photon emission tomography of the brain showed hypoperfused areas at the right frontal and left frontotemporoparietal lobes. The choreoathetosis of the right limbs improved markedly, along with improvement of sensory deficits. CONCLUSIONS: To our knowledge, this is the first report of an adult patient presenting with hemidystonia and hemichoreoathetosis as the initial manifestations of moyamoya disease. Arch Neurol. 2000;57:1510-1512 ( info)

20/171. Co-segregation of benign infantile convulsions and paroxysmal kinesigenic choreoathetosis.

    We report seven families and two sporadic cases in which benign infantile convulsions and paroxysmal kinesigenic choreoathetosis were co-segregated. Clinical investigations included physical and neurological examinations, blood electrolyte values, interictal and ictal electroencephalograms, and computed tomography or magnetic resonance imaging of the brain. The family pedigree was confirmed and the clinical history of the relatives was obtained. Seventeen individuals developed infantile convulsions followed by paroxysmal dyskinesias during childhood or adolescence. Six had only infantile convulsions, and two had only paroxysmal dyskinesias. The seizures never persisted into childhood or recurred in adulthood. The seizure type was a complex partial seizure, with or without secondary generalization, in nine of 14 patients. Paroxysmal dyskinesias, a subgroup of paroxysmal kinesigenic choreoathetosis, occurred for less than 5 min. The attacks of dyskinesias began at age 5-12 years in most patients, and tended to remit in adulthood. The mode of inheritance was apparently autosomal dominant in four of the families (17 affected individuals), who were diagnosed with ICCA syndrome (infantile convulsions and paroxysmal choreoathetosis). However, the condition occurred only among siblings in three families (six patients), and sporadically in two patients, suggesting genetic heterogeneity in this distinct co-segregation. ( info)
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