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A case is presented of a 30-year-old, mentally retarded and epileptic patient, with a progressive hemifacial hypertrophy since birth. Repeated biopsies revealed the neurofibromatous nature of her facial lesion. Autopsy also revealed an ipsilateral hemimegalencephaly, as well as meningeal lipomas and osteomas. This combination of lesions represents an unusual variant within the spectrum of neurofibromatosis.

Forty patients with intractable seizures were studied in an epilepsy unit for an average of 8 weeks with video-electroencephalographic telemetry and continuous observation by trained personnel. Drugs were administered on the basis of antiepileptic drug measurements and seizure classification determined by clinical observation and telemetry. Seizure frequency was reduced in 24 patients (60%). Unrecognized seizure types were identified in 8 patients (20%), and diagnostic classification was changed in 19 patients (47.5). At least one antiepileptic drug was eliminated in 25 patients (60%), and the average drug reduction per patient--0.60--was highly significant (p less than 0.01). In patients with seizures refractory to conventional out-patient and hospital management, improvement in diagnostic accuracy and refinement in observation techniques result in significant reduction of seizure frequency, elimination of drugs, and limitation of toxicity.

We have attempted to identify those attitudes, skills, and competencies in the clinical neurosciences that every graduating medical student should possess. Curricular guidelines are provided that may act as a model educational outline, to be adapted and utilized as individual circumstances dictate and resources permit. A universal, although not lockstep, curriculum emerges from these considerations.

Four children in the same family have 47, +der (14), t(9;14) (p24;q21). Their mothers are sisters with 46,XX,t(9;14) (p24;q21). Clinical features of the children are similar to those of others reported to have partial 14 trisomy.

A 54-year-old woman with transient global amnesia (TGA) was found to have digitalis-induced bradyarrhythmia with atrioventricular dissociation. The amnesia cleared only upon resolution of the arrhythmia. Cardiac arrhythmia has been postulated as a cause, but TGA in the setting of cardiac arrhythmia has not been documented previously. Cardiac arrhythmia should be excluded in patients with TGA, and TGA, a syndrome diagnosed on clinical grounds alone, must be recognized as one possible manifestation of treatable, potentially serious cardiac or cerebrovascular disease.

To 468 women with epilepsy enrolled in the study, 171 children were born and evaluated prospectively. The mothers in this group were characterized by low socioeconomic status, high frequency of tonic-clonic generalized seizures, and use of combinations of anticonvulsants. Approximately 30% of infants exposed to diphenylhydantoin in utero had minor craniofacial and digital changes. The infants did not show an increased rate of growth retardation, mental retardation, or major malformations. It is suggested that the nature of the association of birth defects and maternal epilepsy is complex and that the teratogenicity of anticonvulsant drugs plays only a minor role in this association. The sample size necessary to demonstrate a two- to threefold increase in malformations is beyond the capability of a single center.

A 67-year-old organist and minister with diabetes mellitus had stereotyped focal seizures of the left lower face, jaw, and neck. Attacks occurred spontaneously or were induced when he played a specific hymn on the organ. The seizures were not induced by reading, singing, hearing, or playing the hymn silently. The patient had interictal weakness of the left lower face and left side of the tongue. Focal seizures were recorded on an electroencephalogram (EEG) at the right temporofrontal area. This patient illustrates partial seizures induced by playing music.

Dyskinesia is usually lifelong and progressive; therefore, physicians generally see the disorder in elderly patients. Medical treatment must be carefully selected on the basis of the cause of the dyskinesia. Parkinsonian dyskinesia is well controlled by drug therapy. However, patients can become less responsive to a drug after years of use and may experience unwelcome side effects. Cerebellar tremor is extremely disabling because it worsens with activity, but no satisfactory therapy is available. Senile, essential, and familial tremors are also intensified by action, but they can often be suppressed with a mild tranquilizer or a beta blocker. Drug treatment of blepharospasm and spastic dysphonia has been disappointing: Facial or laryngeal surgery is sometimes required. Tardive dyskinesia is caused by neuroleptic drugs, so the only therapy for the disorder is withdrawal of the offending drug.