A case of Déjerine-Sottas disease with schizophrenic symptoms. A clinical and pathological study

A case of hypertrophic interstitial neuritis with a disturbance of intelligence and schizophrenic symptoms was reported. The patient, a 41-year-old male, showed monologia at the age of 14 and auditory hallucination at the age of 21. He was diagnosed as schizophrenia. Torsion of the head to the right side and muscular atrophy were also noticed at 21 years. Atrophy of the muscles progressed gradually, particularly in the distal parts of the upper and lower extremeties. Psychiatric examination revealed schizophrenic symptoms such as auditory hallucination, thought disorder, lack of spontaneity, autism, apathy, etc. There was also a disturbance of intelligence (imbecility). In neurological examination, the torsion of the head to the right side, slight exaggeration of the peripheral reflexes, muscular atrophy in the distal parts of the extremeties were noted. The bilateral ulnar nerves were markedly hypertrophied. The biopsy of the right ulnar nerve revealed a remarkable thickening of the endoneuria and "onion bulb" formations. A large amount of the interstitial accumulation of PAS positive substance, which showed metachromasia in cresyl violet staining, was also noted. Schwann cell processes were proved electron-microscopically to surround the myelin sheaths and some of them meandered and extended and into the metachromatic substance. The basement membrane of the sheath cell was hypertrophic in some portions and elongated into interstitium. The nucleus of the sheath cell was hyperchromic and showed marked atrophy. Histological examination of the biopsy specimen obtained from the gastrocnemius muscle suggested neurogenic muscular atrophy. The relationship among the hypertrophic interstitial neuritis, schizophrenic symptoms, disturbance of intelligence and torsion of the head was also discussed.