Interstitial hyper-trophic neuritis of Dejerine and Sottas. A report of three cases

Wolf, Abner; Rubinowitz, A.H.; Burchell, S.C.

Bull Neurol Inst New York 2(3): 373-428


Accession: 024888226

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The 40 cases of this disease found in the literature are reviewed. Pathologically, the essential features of the disease are (1) uniform gross thickening of the nerve trunks, of wide distribution; and (2) characteristic hypertrophy of the Schwann sheaths which produces the concentric laminated structures called "onion-bulbs." Secondary histological features are: (1) degeneration and shrinkage of the myelin away from the axis cylinder and toward the outer sheath; (2) degeneration and proliferation of the axis cylinder; (3) secondary degeneration in the dorsal columns; (4) occasional gross thickening of the spinal roots, but more frequently microscopic involvement. The histopathological findings in biopsy specimens of nerves from 3 new cases are presented. The patients were 2 young men and a woman in whom the disease began at the ages of 12, 13 and 26 yrs. There was no familial history in any of the 3 cases.