1/6. 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.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/6. Dystonic attacks related to sleep and exercise.Two unrelated children displayed attacks of paroxysmal jerky 'puppet-like' movements lasting 2-3 min. The attacks were not kinesigenic and occurred during wakefulness precipitated by physical exercise and during NREM sleep, spontaneous or upon arousal and awakenings. Paroxysmal dystonic choreoathetosis was excluded by the absent family history, and paroxysmal kinesigenic dystonia by the absence of triggering effects by sudden movements and efficacy of anticonvulsants. Pattern and duration of involuntary movements were not those typical of nocturnal paroxysmal dystonia. Our cases emphasize that overlap exists among the different clinical categories of paroxysmal dyskinesia.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/6. Sydenham's chorea.P, an Indonesian boy, 5 years old, 15 kg of body weight and 110 cm of body length, was admitted to the PTP IX Hospital on March 30, 1987 with cerebral palsy. The patient had fever since 10 days before admission which lasted 7 days; he showed restlessness by involuntary movements when catching, thus when holding something in his hand, it fell off. He had difficulty in walking and talking. On physical examination, were hyperemic the tonsils and pharynx; there was muscular hypotonia, flexed arms and metacarpophalangeal extension. Laboratory findings showed leukocytosis, increased ASTO titer, positive CRP and normal cerebral spinal fluid. head CT scan showed no abnormality. Electrocardiogram and EEG were in normal limits. Throat swab culture produced Group A streptococcus beta hemolyticus. The diagnosis of sydenham's chorea was established. procaine penicillin, phenobarbital and vitamins were administered to the patient. After 5 weeks in the ward the patient was doing well, and now he is working and speaking normally; he is on ambulatory treatment with 1.2 million units/month of long-acting benzathine penicillin, at the Department of child health, Tembakau Deli Hospital, Medan.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/6. Hereditary progressive chorea without dementia.A family with hereditary non-Huntington's chorea is presented. Transmission was autosomal dominant with variable penetrance. chorea commenced in childhood and affected predominantly the head, face and upper limbs. dysarthria appeared later, followed in two family members by elements of an axial dystonia. There was no intellectual impairment. Unlike previously described families, symptoms progressed steadily up to the eighth decade, causing considerable physical disability.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/6. Paroxysmal choreoathetosis following head injury.A 33-year-old man developed paroxysmal choreoathetosis following a severe head injury. phenobarbital therapy was promptly effective. The family history as well as physical examinations and laboratory studies were unremarkable.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/6. Paroxysmal choreoathetosis: report of five cases and review of the literature.Paroxysmal choreoathetosis is a rare, involuntary movement disorder. Attacks occur spontaneously or may be induced by movement, startle, or anxiety. The movements are tonic, dystonic, or choreoathetotic. Sporadic and, more commonly, familial cases have been reported. Onset occurs most often during childhood, and the course is nonprogressive. Response to anticonvulsant therapy is usually excellent. Five cases of the sporadic form of paroxysmal choreoathetosis are reported. Three of the five patients had attacks after initiation of movements such as rising from a chair. Results of physical examination were normal in four patients. One child had mild hemiatrophy and unilateral hyper-reflexia. Results of laboratory studies, including determinations of serum calcium and ceruloplasmin levels, EEGs and CAT scans of the head, were normal. The attacks ceased in all patients after treatment with either phenytoin or carbamazepine.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |