The diagnosis of hypertrophic interstitial polyneuritis by myelography

Abstract

HYPERTROPHIC interstitial polyneuritis is a rare disease with a familial tendency. While its particular characteristic is thickening of the affected nerves, other changes in the extremities occur consequent on a slowly progressive peripheral neuritis. In 1889 Gombault and Mallet reported the first case. This followed the post-mortem examination of a patient previously thought to have had tabes dorsalis. At necropsy the peripheral nerves were recorded as larger than usual, and a gross hypertrophy of the nerve-roots attracted attention and was attributed to syphilis. Four years later D6j~rine and Sottas (1893) identified the full nature of this case in their report on the condition and its usual name is derived from their paper. The essential pathology is a hyperplasia of the sheath of Schwann which becomes increasingly widespread, and the enlargement may be clinically evident in various peripheral nerves. Biopsy should then prove the diagnosis. In fact palpable thickening was found in 13 out of 16 confirmed cases by De Bruyn and Stern when they reviewed the literature in 1929 . In the absence of such palpable thickening, however, the hypertrophic changes may affect only the spinal roots or the cauda equina, and a firm clinical diagnosis may not therefore be possible. In such circumstances the disease may clinically resemble the Charcot-Marie-Tooth type of peroneal atrophy, especially when the sensory loss is slight. Alternatively the picture of polyneuritis attributed to toxic or mttritional causes may be suggested. It is in such cases that marked changes within the spinal canal may enable myelography to give the correct diagnosis. I t is to illustrate this that two cases are reported, no reference to myelography in this condition having Fig. 233.--Case I. C o n t r o l f i lm t a k e n to exc l u d e s u b d u r a l i n j e c t i o n of m y o d i l , been found in the literature.