Neuromyelitis optica (Devic's syndrome) typically occurs in a sporadic pattern. We studied the disorder in two sisters, ages 6 and 10 years, who presented with sudden loss of vision at age 3 years, followed 5 months later by a transverse myelopathy. The older girl had only light perception in either eye, persistent paraparesis and progressive scoliosis; her sister had a visual acuity of 20/200 in both eyes and mild spinal cord dysfunction. Results of electrodiagnostic tests correlated well with the severity of clinical neurologic deficits and provided objective estimates of the extent of tissue damage in both the optic nerves and the spinal cord. The presence of Devic's syndrome in these two sisters could be coincidental; however, the possibility of a shared genetic factor(s) should be considered.

The electrophysiological features of 3 brothers with the classic form of Pelizaeus-Merzbacher disease (PMD) were studied. Two consecutive overnight polygraphic sleep recordings indicated a gross distortion of rapid-eye-movement (REM) sleep for all patients. A lower than normal percentage of REM sleep in these patients was consistent with their retarded intellectual development, which supports current thinking that REM sleep may be a sensitive index of brain function integrity. Non-rapid-eye-movement (NREM) sleep, in contrast to reported findings in 1 patient with PMD, was uniformly characterized by distinct stages in which the electroencephalograms contained frequent vertex waves and spindles. Tests of peripheral nerve conduction velocity, acoustic brainstem reflexes, and visual and auditory evoked potentials did not indicate any abnormalities, nor did electroencephalograms obtained during wakefulness. One patient did have epileptiform spikes and spike waves recorded during an all-night EEG, an unusual finding in a child with cerebral white matter disease.

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