Subject(s)
Adult , Humans , Male , Athetosis/chemically induced , Chelating Agents/therapeutic use , Chorea/chemically induced , Lead Poisoning/complications , Wounds, Gunshot/complications , Athetosis/drug therapy , Chorea/drug therapy , Dimercaprol/therapeutic use , Edetic Acid/therapeutic use , Lead Poisoning/drug therapy , Lead/blood , Magnetic Resonance Imaging , Tomography, X-Ray ComputedABSTRACT
Paroxysmal kinesigenic choreoathetosis (PKC) is characterized by short paroxysms of focal or generalized involuntary movement induced by sudden movements, and is a well-known disease in the neurologic literature, but only 4 cases have been reported in Korea. The purpose of the presentation is to clarify the clinical features of PKC in Korea. We clinically analyzed 20 patients with PKC between 1986 and 1994 at Yongdong Severance Hospital, Yonsei Medical Center, with a minimum of a 1 to 2 year follow-up period. There were 14 men and 6 women. The age at onset of the condition ranged from 8 to 17 years (mean, 13.1 years). Six patients (30%) had a family history of the condition and the mode of inheritance was suggestive of an autosomal recessive pattern. The involuntary movements seemed to be dystonic rather than choreoathetonic upon a mild attack, and the paroxysms were precipitated by sudden movements. The attacks occurred on one or both sides, and were often associated with dysarthria, upward gaze and sensory aura. Consciousness was never lost. Their duration were usually 10 to 30 seconds, and never more than two minutes. All laboratory tests including electroencephalographic and neuroimaging studies showed no abnormality. All patients responded well to diphenylhydantoin. PKC is not rare in Korea and has a benign course.
Subject(s)
Adolescent , Child , Female , Humans , Male , Athetosis/drug therapy , Chorea/drug therapy , Movement , Phenytoin/therapeutic use , RecurrenceABSTRACT
A coreatetose paroxística (CP) é entidade rara. Até mesmo profissionais que estudam desordens do movimento näo costumam vê-la com freqüência. A ocorrência paroxística de moviemtnos distônicos, coréicos e atetósicos é a apresentaçäo típica da síndrome. O início costuma ser abrupto e os ataques podem durar de alguns segundos até horas. Casos esporádicos e, mais frequentemente,casos familiares têm sido relatados. A abordagem terapêutica com anticonvulsivantes, como a carbamazepina, nem sempre tem sucesso. Com esta droga, porém, geralmente há boa resposta na variante cinesiogênica da CP. É relatado o caso de um paciente jovem do sexo masculino com essa variante da CP. O início da doença se deu na puberdade. O exame neurológico era normal entre os ataques. A investigaçäo laboratorial, EEG, TC de crânio e RNM de segmento cefálico foram normais. Carbamazepina em doses baixas (100 mg/dia) foi eficaz no manejo dos ataques
Subject(s)
Adult , Humans , Male , Athetosis/drug therapy , Carbamazepine/therapeutic use , Chorea/drug therapy , Movement Disorders/classificationABSTRACT
É descrito um caso de hemicoreo-atetose aguda de etiologia vascular (hematomas da gânglia basal esquerda), seguido tardiamente por distonia de torçäo do tipo deformante e fixo durante o tratamento com butirofenoma, em uma paciente de 75 anos. A paciente era portadora de hipertensäo arterial e Diabetis mellitus, compensados clinicamente. O diagnóstico foi baseado na história clínica, exame neurológico e exames complementares (Radiografias do crânio, eletroencefalografia, líquido céfalo-raqueano e tomografia cerebral computadorizada)