Motor conduction studies were performed serially in 10 patients, ages 10-62 years, with clinical and electrophysiological criteria of hereditary motor and sensory neuropathy type 1 (HMSN-1) over periods of 11-19 years. Median nerve conduction velocity (MNCV) and distal motor latency showed no significant change on serial studies. Mean median compound muscle… (More)
During surgical correction of scoliosis, 63 patients had somatosensory evoked potential (SEP) monitoring of the spinal cord. Tibial nerves were unilaterally stimulated, and the potentials were recorded from the midcervical spine with surface and epidural needle electrodes. Over 85% had no significant change in their SEP and no postoperative neurologic… (More)
We report a third family with six members with features of the syndrome of familial parkinsonism, depression, weight loss, and central hypoventilation. Patients with this syndrome typically die suddenly, presumably from central respiratory failure. Following multiple respiratory arrests, one affected member was successfully managed with aggressive pulmonary… (More)
A large cystic olfactory bulb was discovered incidentally at autopsy. There was no evidence of central nervous system trauma or infection. Hemispheric malformations did not exist except for a dorsal displacement of the overlying gyrus rectus. We interpreted the cyst to be a persistent olfactory bulb ventricle.