Idiopathic generalized myokymia (IGM) is a rare, heterogeneous, and poorly understood syndrome. We present analysis of 75 reported cases in the world literature. IGM affects men and women equally, with a mean age of onset 29 +/- 19 years. Patients' common presenting complaints are stiffness (60%), cramps (12%), weakness (12%), and muscle twitching (4%). Family history is positive in 30%. In addition to generalized clinical myokymia (92%), abnormal neurologic findings include: hyporeflexia (70%), weakness (45%), grip myotonia (39%), and calf hypertrophy (16%). Electrical activity consisting of spontaneous continuous motor unit activity and/or electrical myokymia was documented in all patients. When electrical myokymia was observed (66%), the grouped discharges where irregular and had an interburst frequency of 2-300 Hz. Both phenytoin and carbamazepine are effective treatments. We conclude that IGM has a wide spectrum of symptoms and severity and should be considered in all patients that present with stiffness, cramps, or muscle twitching. EMG greatly aids in diagnosis.

We describe conduction block as an unusual electrophysiologic manifestation in a patient with necrotizing angiopathy. The patient developed subacute symptoms over a 1-month period consisting of progressive pain, tingling, and weakness of the lower extremities. Physical examination revealed a pattern consistent with a polyneuropathy. Electrodiagnostic studies provided evidence of a conduction block in the left ulnar nerve. Pathologic studies confirmed the process to be a necrotizing angiopathy. This report establishes the role of conduction block in human nerve ischemia.

There have been major changes in neurology research during the past decade, but no systematic examination of the content, methods, funding, and research personnel. All scientific articles in the journals Annals of Neurology, Archives of Neurology, and Neurology were categorized by article type, number of authors, highest degree of first author, institution, disease area, research field, and source of support for the years 1980 and 1990. Original articles grew by 54% over the decade. The average number of authors increased by 1.3 to 5 (p greater than 0.0001). Groups of MDs and PhDs wrote 39% of articles in 1990 (24% in 1980). Contributions with a diagnostic or therapeutic focus rose from 7 to 14%, whereas there was a pronounced drop in case reports from nearly 40% to just under 20%. Articles on movement disorders and neurodegenerative diseases increased more than 100% and now together comprise more than 25% of original articles. Molecular biology increased by 1,600%, and there was significant growth in epidemiology, neuro-imaging, neurochemistry, and neuropsychology. The top 20 publishing institutions accounted for 40% of all original articles and 60% of US original articles. Foreign contributions doubled over the decade. The National Institutes of Health funded 46% of US articles in 1990 (35.5% in 1980). More than 80% of articles in 1990 on neurodegenerative disease were supported by the National Institutes of Health. The complexity of neurological research has driven greater collaboration between basic and clinical scientists. New areas of clinical and basic knowledge are rapidly emerging in neurology.

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