QUANTITATIVE STUDY OF INTERMEDIOLATERAL COLUMN CELL COUNTS IN MACHADO-JOSEPH-DISEASE

The number of intermediolateral column (ILC) neurons in 6 alternating segments from the 2nd to 12th thoracic segment of the spinal cord were studied in 4 cases with Machado-Joseph disease (MJD), 3 cases with ol ivopontocerebellar atrophy (OPCA), a case with Shy-Drager syndrome (SD S), and 5 normal controls. We counted the number of ILC neurons with c learly defined nucleoli in 12 sections of each segment, each section 2 0 mu m thick and taken at 100 mu m intervals and then divided the 6 al ternating segments into 3 groups, upper (Th2, 4), middle (Th6, 8) and lower (Th10, 12). In each of the three groups of normal control cases, the number of TLC neurons had decreased with aging. In all MJD cases, the number of ILC neurons had moderately decreased in comparison with age-matched controls. One of the MJD cases showed a marked decrease i n the number of ILC neurons, as did the SDS case. The ILCs of the enti re thoracic spinal cord in the MJD cases were moderately involved.