INTRATHECAL IGA SYNTHESIS IN X-LINKED CEREBRAL ADRENOLEUKODYSTROPHY

Cerebrospinal fluid data of 25 patients suffering from various phenoty pes of X-linked adrenoleukodystrophy have been evaluated neurochemical ly including cerebrospinal fluid/serum quotient diagrams. Intrathecal IgA production in 13 of 14 patients with cerebral adrenoleukodystrophy was the most sensitive parameter in cerebrospinal fluid and was not s een in any of the neurologically asymptomatic patients or in the patie nts with adrenomyeloneuropathy. A blood-cerebrospinal fluid barrier dy sfunction was found in 9 of these 14 patients. Additional intrathecal IgG or IgM synthesis was observed in 3 patients each. In 1 patient wit h lumbar punctures before and after onset of neurologic symptoms intra thecal IgA synthesis was seen only after the appearance of neurologic symptoms. Repetition of lumbar punctures in 5 neurologically symptomat ic patients with cerebral adrenoleukodystrophy revealed a similar patt ern of intrathecal IgA synthesis with a tendency to decreasing IgA con centration. The pathophysiologic aspects of intrathecal IgA synthesis are discussed in relation to other demyelinating and inflammatory neur ologic diseases.