Beitraege zu den klinisch‐ und histopathologischen Untersuchungen ueber die Faelle der Lafora‐aehnlichen Einschlusskoerperchen

SUMMARY In case 1, 41–year‐old male, developed progressive dementia, paretic gait disturbance and pyramidal signs with the duration of three years. The neuropathological study revealed systemic atrophy as type Pick‐disease i.e . lobal atrophy in the frontal and the parieto‐occipital regions, degenerative changes in the basal ganglia and in the thalamus, nerve cell loss in the substantianigra and myelin pallor in the pyramidal tract. Lafora‐like inclusions were found in the cerebral cortex and in the cochleal nucleus. In case 2, 45–year‐old male, showed character change, cerebellar symptomes and mental deterioration, and ulcers on the scrotum and on the oral mueosa during about 15 years long period. Neuropathological examination showed chronic encephalitis in the brain stein, vacuolar change in the neuron in the olivary nucleus and Lafora‐like inclusions in the cochlear nuclcus. Though neither generalized conversion nor myoclonus were clinically observed in these cases, the inclusions showed histo‐chemically strong similarity with that of the Lafora‐disease. These Lafora‐like inclusions were compared with those in the literatur, which were reported on various disease of CNS. Finally in respect of predilection of the inclusions, it is likely that the inclusions result from same metabolic disturbances in the cochlear neurons in the Lafora‐disease as well as in the present cases.