We report the neurophysiological findings from 24 subjects with myotonic dystrophy of Steinert and the histological findings in two of them. The conduction data is compared with that of a group of subjects with Landouzy‐Déjérine muscular dystrophy. In 46% of cases, the electrophysiological data revealed slight and generalized axonal neuropathy. Histological results of sural nerve confirmed axonal damage of sensory fibres. The neuropathy was not correlated with age of patients, duration or onset of the disease, nor with the state of the deep reflexes; it did not show signs of progressing and is probably one of the multisystemic manifestations of gene pleiotropism.
The effect of oral treatment with acyclovir (ACV) on sensory axonal neuropathy, segmental motor paresis and postherpetic neuralgia (PHN) was studied in 105 patients with herpes zoster. Forty-seven patients were treated with ACV at a dose of 4 g/day in 5 doses for at least a week; the others did not undergo any kind of treatment. Electrodiagnostic examination of the nerves and muscles corresponding to the dermatomeric lesions was performed, including sensory and motor nerve conduction studies, blink reflex and electromyography (EMG). The patients treated with ACV showed a significant reduction in the number of cases in which there was electrophysiological evidence of axonal damage in afferent fibres of nerves arising from roots corresponding to affected dermatomes. The treated group also showed a smaller incidence of segmental motor neuritis, clinically evident or only detectable by EMG as denervation. However, there was no significant difference between groups as far as the incidence of PHN was concerned. Oral treatment with ACV therefore reduces peripheral sensory axonopathy due to ganglion damage and prevents the possibility of spread to anterior roots and spinal motoneurones. In this way it reduces the incidence of segmental motor neuritis, but does not reduce the incidence of PHN.
We report the electrophysiological findings and the management of 5 subjects with progressive systemic sclerosis (PSS) and clinical evidence of nerve entrapment. Three had carpal tunnel syndrome (CTS), 1 bilateral CTS and right tarsal tunnel syndrome (TTS) and 1 Guyon's canal syndrome. Only 1 patient (with CTS) showed significant clinical improvement after surgical decompression; the other 4 demonstrated a slight recovery of conduction without lasting clinical relief after conventional treatment. To explain these failures we hypothesized that these entrapment syndromes were the clinical expression of underlying diffuse damage to the peripheral nervous system (PNS). The conduction values of nerves unaffected by entrapment syndromes were within normal limits, but almost all distal velocities were below the mean of controls. Such subclinical distal peripheral neuropathy was also verified in a selected sample of 17 patients with PSS, without clinical symptoms or signs of PNS involvement. In these 17 cases the mean distal sensory and motor conduction findings of the median, ulnar, sural and tibial nerves were significantly lower than those of a control group, while no significant differences were found in the more proximal tracts of the same nerves. Furthermore, 3 of the 17 patients showed classical electrophysiological evidence of TCS and TTS without any clinical symptoms. We conclude that the subjects with PSS had subclinical polyneuropathy which may become plain polyneuropathy or nerve entrapment syndromes perhaps induced by other risk factors.
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