Spinal ganglia and peripheral nerves from a patent with Tay-Sachs disease. Morphological and ganglioside studies

We examined the spinal ganglia and peripheral nerves from a patient with Tay-Sachs disease, comparing the other nervous tissues morphologically and lipid-biochemically. The spinal ganglia and peripheral nerves showed numerous membranous cytoplasmic inclusion bodies (MCBs), which are characteristic of GM2-ganglioside storage in the neuronal cell bodies of the patient brains. In spinal ganglia, all neurons and satellite cells around the neurons contained membrane-bound lipid materials. In peripheral nerves, Schwann cells and myelinated axons except for enlarged axons were devoid of MCBs. Major ganglioside stored in both spinal ganglia and peripheral nerve was also GM2-ganglioside. The contents of ganglioside in the spinal ganglia and peripheral nerves were 50 and 10 times more than those from normal tissues, respectively. The spinal cord contained a slightly higher amount of gangliosides than the normal control. The cerebral white matter totally demyelinated in this patient accumulated a much higher amount of gangliosides than the cerebral gray matter. The retinal tissue showed GM2-ganglioside as the major one also.