Biomedical subjects
Publications and source records attributed to C Gardner-Thorpe.
A patient with McArdle's disease is reported in whome proximal muscle weakness began in the 7th decade. Other unusual features included the absence of muscle cramps at any stage, asymmetrical wasting of the shoulder girdle muscles and calf hypertrophy. Muscle biopsy revealed a vacuolar myopathy, absent phosphorylase staining and qualitatively low glycogen content.
The literature on complicated herpes zoster is summarized in this paper. The case histories of 18 patients with herpes zoster are presented. Two patients had encephalitis, 2 had myelitis and the other 14 patients had various types of lower motor neurone disturbance. Both patients with encephalitis--one of who developed choreo-athetosis during the illness--recovered fully. Only 1 of the 2 patients with myelitis recovered fully; the other remains severely paraparetic and the reason for her incomplete recovery may be related to the presence of generalized arteriolar disease associated with seronegative rheumatoid disease. One patient developed a Guillain-Barre syndrome 3 weeks after the onset of herpes zoster. Recovery in the 15 patients with lower motor neurone involvement has been slow butcomplete--or almost complete--in all but 1, a patient with persistent facial weakness as part of the Ramsay Hunt syndrome and who also had weakness of one upper limb. Seven other patients had lower limb weakness. In 2 patients the weakness was confined to abdominal myotomes and 2 other patients had urinary retention. Electromyographic abnormalities were found in the muscles which were weak and frequently also in muscles which appeared strong. It is emphasized that neurological disturbances other than sensory abnormalities may be found in patients with herpes zoster. Motor complications of various types are not uncommon.
One pair of male monozygous twins was examined: only 1 twin had multiple sclerosis. Differences in the life histories of the twins were identified. Haematological, biochemical and microbiological studies were performed. It is possible in theory to determine which environmental factors may have been responsible for the development of multiple sclerosis in 1 twin. Several differences in life history were found in this study.
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The case histories of two patients who developed an anterior interosseous nerve palsy apparently as a result of an external pressure injury are reported. Both patients recovered fully without surgical exploration, one 19 months and the other nine months after the onset. It is stressed that complete recovery may occur spontaneously.
The electroencephalogram in three patients with Jakob-Creutzfeldt disease showed two separate abnormalities-namely, progressive background suppression and periodic generalized synchronous triphasic sharp wave complexes which evolve to a uniform morphology and periodicity. The abnormalities, when found in the EEG of a patient in middle-age with a dementing illness, should not be confused with other periodic electroencephalographic phenomena. Since the neuropathological abnormalities of Jakob-Creutzfeldt disease are non-specific, the electroencephalogram is essential for the recognition of this disorder, although serial recordings may be necessary to establish the diagnosis. Modification of the electroencephalographic abnormalities occurs with afferent stimuli and with methylphenidate or diazepam, suggesting that the phenomenon of background suppression is independent of the presence of the periodic complexes. Modification of clinical activity with methylphenidate suggests that some degree of reversibility of function exists in this inexorably fatal disorder. Further detailed studies of the electroencephalogram in cases of Jakob-Creutzfeldt disease are indicated.
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Peripheral neuropathy and optic neuritis occurring in alcoholics are sometimes due to the toxic effects of disulfiram rather than to alcohol. Unless this is considered in the management of such patients it is likely that administration of the drug will be continued. In the past there have been reports of 16 cases of peripheral neuropathy and six cases of optic neuritis, possibly caused in this way; these reports are reviewed and a further six cases are described. The effects of disulfiram on the nervous system are briefly discussed.
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