Herpetic brainstem encephalitis.

A fatal case of brainstem encephalitis presented as an acute lateral medullary syndrome. Pathologic examination showed a focal encephalitis along the roots and the intramedullary portion of the right vagus and glossopharyngeal nerves. Herpesvirus particles were demonstrated by electronmicroscopy of formol-fixed material. This is the second known case of herpetic brainstem encephalitis confirmed by postmortem examination and the only one in which the infection was limited to the brainstem. This case and a review of previously reported cases suggest that the viral infection could proceed to the brainstem through the vagus and glossopharyngeal nerves, probably from a source of latent herpetic infection in the respective ganglia.

Successful treatment of familial idiopathic lipid storage myopathy with L-carnitine and modified lipid diet.

We describe the clinical presentation, course, pathologic findings, and biochemical abnormalities found in three adult siblings with an idiopathic lipid storage myopathy. The major presenting symptoms were weakness and cramping, which were profound in one patient, moderate in the second patient, and mild in the third. All three individuals exhibited true myotonic discharges on EMG, normal or mildly depressed muscle free carnitine levels, and borderline delayed ketosis (40 hours) with fasting. Muscle biopsies from all three showed neutral lipid storage. Polymorphonuclear leukocytes and macrophages were vacuolated. No systemic abnormalities of glucose or fat metabolism were identified at rest, with fasting, or with exercise. The two more severely affected patients have responded to medium-chain triglyceride diet and oral carnitine with increased strength and muscle bulk and decreased histochemically observed neutral lipid stores in muscle. One patient has had a resolution of the electrical myotonia. Prednisone therapy in one patient resulted in a loss of cramping sensations but not improvement in strength. We suggest that some cases of idiopathic lipid storage myopathy may be safely and effectively treated with carnitine and medium-chain triglyceride diet.

Persistent retrograde memory deficit after transient global amnesia.

A 64-year-old woman suddenly had an attack of confusion and amnesia that suggested transient global amnesia. However, her loss of memory for recent events lasted more than ten days and was accompanied by psychomotor agitation and transient alteration of sexual behavior. The patient had no other neurologic signs during the episode. She recovered completely from the recent memory deficit, but was left with a persistent retrograde amnesia for a period of five to ten years and total amnesia for the acute episode. The EEG was suggestive of a left medial temporal lobe lesion.