myelin-basic-protein and Leigh-Disease

We present a young child with an illness characterized by remissions and exacerbations and signs of disseminated lesions in the central nervous system. Visual evoked response and brainstem auditory evoked potential gave abnormal findings showing lesions also of the optic nerves and brainstem. The CSF IgG index was slightly elevated and myelin basic protein was high. There was also intrathecal antibody production against different viruses, the highest against measles. The HLA type was A3, B7, Dr2. The T4/T8 ratio reflected an immunological active disease. All these signs could have been diagnosed as multiple sclerosis. However, there were also lower motoneuron lesions and metabolic studies showed disturbances in pyruvate metabolism as in Leigh's disease. Cerebral tomography and magnetic resonance imaging showed scattered lesions in the nuclei lentiformis and capsula interna. We would like to stress the importance of careful etiological studies in cases of MS in childhood.

Topics: Brain Diseases, Metabolic; Child; Chronic Disease; Diagnosis, Differential; Humans; Immunoglobulin G; Leigh Disease; Male; Myelin Basic Protein; Pyruvate Dehydrogenase Complex; Recurrence; T-Lymphocytes, Helper-Inducer