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A 57-year-old woman with biopsy proven glioblastoma multiforme with greater left-sided involvement, presented with subacute right hemiparesis. Her EEG showed triphasic waves with a left-sided predominance. Possible metabolic causes of triphasic waves were excluded. The association between triphasic waves and primary brain tumor is extremely rare, and the pathogenesis unknown.

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Monozygotic twin sisters from Qatar, concordant for the Rubinstein-Taybi syndrome, are described. Skeletal anomalies not previously seen in this syndrome are described. The mode of inheritance is reviewed.

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A 45 year old woman with probable bacterial endocarditis was admitted to hospital having presented with a deteriorating level of consciousness and a febrile illness. Among the numerous investigations she underwent was an EEG which showed classical triphasic waves at the peak of her illness, and which reverted to normal as did the patient after treatment with antibiotics. The association between triphasic waves and bacterial endocarditis has never been previously reported.

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We report 14 patients with a slowly progressive syndrome featuring ataxia, choreoathetosis, and ocular motor apraxia in both the horizontal and vertical planes. Although the neurological signs were indistinguishable from those of ataxia-telangiectasia, the onset tended to be later and none of the patients had evidence of multisystemic involvement. Specifically, there was no tendency to frequent infections, and immunoglobulins, alpha-fetoprotein, T- and B-lymphocyte markers, and chromosomes 7 and 14 were normal in all tested patients. The simultaneous absence of telangiectasias and of other nonneurological manifestations made ataxia-telangiectasia an unlikely diagnosis. We suggest that these patients suffer from an unusual type of spinocerebellar degeneration. This syndrome has been observed in different populations from three continents, with a genetic pattern suggesting recessive autosomal inheritance.

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An Arab woman developed recurrent chorea gravidarum in all four pregnancies that went to term, and none in the three pregnancies that ended in spontaneous abortion. She also developed an acute psychosis in one of these four pregnancies. A discussion of similar cases in the literature is undertaken.

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A recent questionnaire survey of the life-effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, compared to similarly distributed age and sex matched controls, documented multiple and marked effects on work, education, driving, accidents, recreation, personality, memory and other parameters. The data have now been further analysed according to the patients' geographic (culturo-genetic) origin and to a number of other patient and illness variables. The three different geographic populations showed few significant differences for the some 160 life-effects items in the questionnaire. This strongly indicates that these are an integral part of the disease or of the human reactions to it. Most of the few significant population differences appeared cultural in origin (e.g., concerning driving records, personality changes), although a few may possibly reflect genetic differences (e.g., visual problems). Analysis of the pooled data according to respondees' age, sex, age at illness onset, duration of illness and treatment led again to relatively few significant findings. It is concluded that, in general, once the disease has been diagnosed, all the major life effects are present and remain so. The results strongly support the contention that most life-effects are not related to the diagnostic 'tetrad' symptoms themselves but rather to excessive daytime sleepiness, the symptoms most resistant to ant-narcoleptic treatment.

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A questionnaire survey has been made of the life effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, with 180 similarly distributed age and sex matched controls. Life-effects were attributed by the patients to the primary symptoms of excessive daytime drowsiness, sleep attacks, cataplexy, vivid hypnagogic hallucinations and sleep paralysis, and also to other frequent symptoms such as visual problems (blurring, diplopia) and memory impairment. Occupational problems were prevalent (over 75%) and included statistically significant deleterious effects upon performance, promotion, earning capacity, fear of or actual job loss and increased disability insurance. Driving was greatly affected and patients fell asleep at the wheel more frequently (66%), had near or actual accidents from drowsiness or falling asleep at the wheel (67%), and could experience cataplexy (29%) or sleep paralysis (12%) while driving. Work or home accidents attributed to sleepiness or sleep (49%) or related to smoking (49%) were much more common in patients. There were also deleterious effects on education, recreation and personality related to disease. Narcolepsy can produce a variety of life-effects probably more serious and pervasive than, for instance, those of epilepsy, therefore emphasizing the importance of early diagnosis and treatment.

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The characteristic EEG complexes seen in subacute sclerosing panencephalitis and the periodic myoclonus accompanying them are well known. That either of these can occur unilaterally is less well appreciated. However, the combination of unilateral SSPE complexes and contralateral myoclonus is most unusual. Attention is drawn to such a case in this paper.

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The case of an 8-year-old boy with spontaneous spinal epidural haematoma and complete post-operative recovery is described. A review of the literature with particular emphasis on the paediatric age-group is given. The clinical presentation and aetiology are discussed.

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Spontaneous spinal epidural hematoma was confirmed by myelography in an 8-year-old boy. The hematoma was removed more than 21 hours after the onset of paraplegia, and there was no evidence of vascular malformation. The patient made a complete recovery.

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Clinical, electrophysiological, and histopathological studies of some members of a family with dominantly inherited hypertrophic neuropathy are presented. Twenty-five members were studied. Seventeen were abnormal on clinical examination. Their ages varied from 2 1/2 to 78 years. Age at onset in 14 of the 17 varied between 2 1/2 and 56 years. Pes cavus and palpable nerve thickening were present in more than half of the affected individuals. All patients had areflexia. Fifteen of the 17 had distal motor weakness as well as mild to moderate sensory impairment. Motor weakness affecting the proximal hip and shoulder girdle muscles was seen in 13 patients. Four patients gave a history of trigeminal neuralgia. Motor nerve conduction velocities were markedly impaired in all the clinically affected members. These studies were normal in the 8 unaffected members. Motor conduction velocities of the proximal segment of the ulnar nerve were slower compared to the distal segment in almost all the affected members. There was no significant correlation between the degree of clinical disability and the extent of impairment in the motor nerve conduction velocities. Sural nerve biopsies were studied. These observations are discussed.

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