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The deformity with which this neurological condition first shows itself is the pes cavus. During the period of growth a severely instable cavovarus deformity develops. The author avoids operations on children because the results are usually poor; the basic problem cannot be resolved with surgical measures. He recommends that the affected feet be immobilized by plastic boots until puberty is reached, at which time only minor bone resections for correcting the form of the feet are necessary. In cases of severe cavovarus deformity in early adulthood the author does not use the usual treatment, triple arthrodesis, but operates according to a technique developed by himself: a wedge osteotomy of the Chopart joints is performed to the degree necessary to permit an exact correction of the deformity. In cases of pronounced deformities, the wedge must include the entire navicular bone. After the resection the heel automatically assumes a neutral position and subtalar stability is achieved. In a number of cases the Achilles tendon must be lengthened after wedge resection. To make possible dorsal extension of the operated foot it is necessary to translocate the tendon of tibialis anterior to the base of metatarsus V. If the tibialis anterior muscle is weak or paralysed, we translocate the tendon of tibialis posterior. Instead of running this tendon through the interosseous route, we lead it subcutaneously to the distal part of the forefoot. Preoperative toe deformities are much less pronounced following the operation, and only in rare cases is correction of the first toe necessary.(ABSTRACT TRUNCATED AT 250 WORDS)

Severe hip dysplasia in children with Charcot-Marie-Tooth disease is asymptomatic or minimally symptomatic and usually goes undetected until early adolescence. We report five patients between the ages of 8 and 15 years who presented with hip dysplasia; all five required surgery. The dysplasia in two of these patients was detected only on routine screening because of their older sisters' problems. A multicenter study will be needed to determine the exact incidence of hip dysplasia associated with Charcot-Marie-Tooth disease.

Charcot-Marie-Tooth disease is a hereditary hypertrophic neuropathy. The finding of massive enlargement of the spinal nerve roots is demonstrated on a computed tomographic scan in a patient with this disease.

The myelographic findings of enlarged nerve roots in a patient with Charcot-Marie-Tooth disease is found to be identical to those of Hypertrophic Interstitial Neuritis and Neurofibromatosis. The hypertrophy of the roots may represent a single response to different noxious stimuli. Clinical and laboratory differentiation are given for the diseases.

Much confusion and disagreement exists regarding the classification and characteristics of inherited disorders manifesting neurogenic muscular atrophy. Many authors consider Charcot-Marie-Tooth syndrome (CMTS) and Roussy Levy syndrome (RLS) forme fruste or variants of Friedreich's ataxia (FA). Familial kyphoscoliosis has often been described in FA and RLS but not with CMTS. The purpose of this paper is to present detailed clinical and laboratory findings in a family with three cases of Scheuermann's kyphoscoliosis and CMTS in three generations. In all cases Scheuermann's kyphoscoliosis was associated with pes cavus, markedly diminished vibratory and position sensation in the lower extremities, absent deep tendon reflexes and muscular atrophy, predominantly of the distal muscles. Fine rhythmic tremor of outstretched hands and positive Romberg sign were present in one case only. Serum creating phosphokinase was elevated in two cases. Motor nerve conduction studies revealed impaired function in the median, ulnar, tibial and peroneal nerves. Sensory nerve conduction wal also impaired in median and ulnar nerves. There was evidence of left ventricular hypertrophy in one case only. The nosology and relationship between CMTS, RLS and FA are discussed.