Electromyographic Studies in Peroneal Muscular Atrophy

Motor nerve conduction velocity in peroneal muscular atrophy: evidence for genetic heterogeneity

“…Our results are comparable with those of Dyck and Lambert (1968a, b), but conflict in certain respects with the findings obtained by Amick and Lemmi (1963) and Myrianthopoulos et al (1965). These authors reported individuals with reduced and with preserved conduction velocity in the same families.…”

“…Early reports of measurements of motor nerve conduction velocity in peroneal muscular atrophy (Henriksen, 1956;Gilliatt and Thomas, 1957) recorded gross reductions in velocity, and this was confirmed by Christie (1961), Amick and Lemmi (1963), Dyck et al (1963), Earl andJohnson (1963), Blom et al (1964) and later workers. Subsequent observations on sporadic cases by one of the present authors indicated that in clinically similar cases, conduction velocity could either be within normal limits or only slightly reduced, and that these differences did not depend upon the stage of the disease.…”

“…No supporting evidence for a progressive reduction in axon size has so far appeared. Measurements of motor nerve conduction velocity over periods of up to three years (Amick and Lemmi, 1963;Nielsen and Pilgaard, 1972) have shown no significant change, although this may be too short an interval for these very chronic disorders. Evidence against the axon progressively dwindling in size derives from the lack of any correlation between conduction velocity and age found in the present series.…”

Charcot‐Marie‐Tooth disease from first description to genetic localization of mutations

“…The heterogeneity of slowing in nerve conduction velocities was confirmed by a number of teams (Christie, 1961;Amick and Lemmi, 1963;Blom et ah, 1964;Myrianthopoulos et al, 1964;Humberstone, 1972;Thomas & Calne, 1974;Salisachs, 1974), and this led Dyck and Lambert (1968a, b) to suggest a classification of Charcot-Marie-Tooth disease in a larger nosologic domain called "hereditary motor and sensory neuropathies". In this classification, Charcot-Marie-Tooth disease is divided into five types: type I is the form of Charcot-Marie-Tooth disease known as the myelinic (hypertrophic) form with slowing of nerve conduction velocities and schwannian hypertrophy at nerve biopsy; type II is said to be the axonal (neuronal) form and is not accompanied by slowing of the nerve conduction velocities, nor any distinct histological abnormalities; type V is a form of Charcot-Marie-Tooth associated with spastic paraplegia; type VI is a form associated with optic atrophy and type VII is associated with retinitis pigmentosa.…”

Biology and Disease of the Peripheral Nerves

“…These appearances are common to the electromyographic picture of most cases of peripheral neuropathy. They are, however, much less frequent in the records of patients with Charcot-Marie-Tooth neuropathy (Amick and Lemmi, 1963). I have seen only two cases of hypertrophic neuropathy and three of a rare recurrent polyneuropathy of unknown cause which may be related to it (Nattrass, 1921 ;Austin, 1958).…”