Lysosomal abnormalities in cultured schwann cells from a patient with peripheral neuropathy and continuous muscle fiber activity

Schwann cell cultures were established from a sural nerve containing large membrane-bound vacuoles in its Schwann cells, obtained from a patient with neuropathy and continuous muscle fiber activity. Cultured Schwann cells contained many large membrane-bound vacuoles, presumably lysosomes, resembling those present in the biopsied nerve. The acid phosphatase reaction was excessive in the patient's cultured Schwann cells but practically negative in normal cultured Schwann cells. This study indicates that the patient's neuropathy is primary dysschwannian with abnormal lysosomes as a major abnormality.