Abstract
. Clinical, neurophysiological and certain other laboratory data are given for 10 children considered to represent sporadic cases of de- and remyelinating hereditary motor and sensory neuropathies (HMSN). In all 10 families the parents had been found to be non-affected and said to be non-consanguineous. Nine of the 10 families originated from the two most northerly Swedish counties, but none from the Gothenburg area. The median age at clinical onset was 3 years. Gait abnormalities and/or foot deformities were common reasons for referral. Scoliosis developed in all 10 and hand atrophies in 7 children. In the majority there was neurological deterioration through childhood. Nerve conduction velocities, both motor and sensory, were consistently and markedly reduced. The protein level in cerebrospinal fluid (medium 657 mg/l) was slightly to moderately raised in 7 of 9 examined children and ≧1500 mg/l in the remaining two. The fatty acid pattern of serum lecithin was consistently normal in all 10.
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