Neuropathological study and chemico-pathoiogical correlation in sibling cases of Sanfilippo syndrome type B
K Tamagawa, Y Morimatsu, K Fujisawa, A Hara… - Brain and …, 1985 - Elsevier
Pathological and biochemical studies of two sibling cases (18-year-old girl and 16-year-old
boy) with Sanfilippo syndrome type B are presented. Pathological study revealed distended
neurons and neuronal processes with inclusions throughout the CNS and the visceral
organs, and neuronal loss and corresponding gliosis. The authors paid particular attention
to atrophic changes in the thalamus and to “dissociation gliomyélinique” in the cerebral
white matter. Histochemistry and electron microscopy revealed regional differences in stored …
boy) with Sanfilippo syndrome type B are presented. Pathological study revealed distended
neurons and neuronal processes with inclusions throughout the CNS and the visceral
organs, and neuronal loss and corresponding gliosis. The authors paid particular attention
to atrophic changes in the thalamus and to “dissociation gliomyélinique” in the cerebral
white matter. Histochemistry and electron microscopy revealed regional differences in stored …
