CEREBRAL MAGNETIC-RESONANCE-IMAGING PATHOLOGY AND CEREBROSPINAL-FLUIDPROTEIN IN SPORADIC AMYOTROPHIC-LATERAL-SCLEROSIS (SALS)

In 3 out of 20 patients with sporadic amyotrophic lateral sclerosis (s ALS), cranial magnetic resonance imaging detected multiple demyelinati ng lesions. All 3 patients died from definite upper and lower motor ne uron degeneration. In all 3 cases total cerebro-spinal fluid (CSF) pro tein remained within normal ranges, and a blood-CSF barrier dysfunctio n was not detectable. In one of the patients multifocal CNS demyelinat ion coincided with an intrathecal synthesis of immunoglobulin-G and au tochthonous CSF oligoclonal IgG banding (OCB) early in disease. Neithe r absolute or age-corrected survival nor disease progression differed for patients with and without cerebral MR lesions, or normal vs. eleva ted CSF total protein. Evaluating the CSF in an extended patient sampl e (n = 29), we found the total CSF protein elevated in 5 of 16 men and none of 13 women (p < 0.05). The mean age-corrected CSF protein conte nt [practical reference limit = (age x 3.3) + 300 mg/l] was higher in male (465 mg/l +/- 32 SE) than in female (350 mg/l +/- 26 SE) sALS pat ients (p < 0.01). This coincides with a male preponderance in sALS.