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24 ULTRASTRUCTURAL FINDINGS OF SKIN AND PERIPHERAL NERVE BIOPSIES IN DEGENATIVE DISORDERS OF THE CENTRAL NERVE SYSTEM

In South America and, particularly in Brazil technical problems make difficult the performance of the majority of the lysosomal enzymes determinations and chemical analysis of central nervous system tissues. Thus, the correct diagnosis of most of the lyso-somial diseases is done by clinical and morphological data. Furthermore, the diagnosis of some degenerative diseases of the central nervous system whose biochemical background is unknown is based only on morphological data. This paper shows the ultrastructural findings in 70 consecutive skin and peripheral nerve biopsies from patients with a clinical history of a cerebral degenerative disorder. Positive results were obtained mainly in lysosomal diseases, i.e., the several forms of mucopolysaccharidosis, neurolipidosis and ceroid lipofuscinosis.