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DEJERINE-SOTTAS SYNDROME IN CHILDREN

(HYPERTROPHIC INTERSTITIAL POLYNEURITIS)

C. V. G. KRISHNA RAO M.D.1, C. R. FITZ M.D., and D. C. HARWOOD-NASH M.D., F.R.C.P.(C)

1 Division of Neuroradiology, Department of Radiology, Tufts-New England Medical Center, Boston, Massachusetts.

The occurrence of hypertrophic interstitial polyneuritis (H.I.P.) in 2 children, each of whom had characteristic roentgenographic features, is presented.

H.I.P. rarely presents in childhood.

Myelography is recommended for all children having bizarre and progressive gait abnormalities, deficits in motor or sensory abilities, or both. These abnormalities often resemble those caused by Friedreich’s ataxia or peripheral neuropathy. However, we feel that the myelographic findings, taken together with the minor spinal changes, are quite characteristic of H.I.P. in this age group.


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