Biomedical subjects
Publications and source records attributed to B Bady.
In a patient with chronic active hepatitis (CAH) due to B-virus, a polyneuropathy developed following a cure of vidarabine. The pathophysiology of this neuropathy remains unclear as it appears not to occur in patients with diseases other than CAH. Muscular pains and paresthesias reported in such patients treated with vidarabine could be the consequence of peripheral nerve involvement.
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EMG and nerve conduction studies have limitations and require particular consideration in children. The indications and the main results are considered from the study of 1624 EMGs in 1385 children under 15 years old seen over a period of 3 years. Classification of cases was based on clinical criteria. The diagnostic yield of EMG is emphasized in the evaluation of 122 children with hypotonia and weakness (all under 3 years old); abnormalities were demonstrated in 48% leading to the diagnosis of spinal muscular atrophy in 16%, of congenital myopathies in 11%, and of peripheral neuropathies in 8%. In 103 children with hypotonia associated with mental deficiency and/or seizures, evidence of a peripheral neuropathy was shown in 20%. EMG abnormalities allowing the diagnosis of hereditary motor and sensory neuropathies or myopathies were demonstrated in a wide range of clinical conditions. EMG appears to be useful for the early detection of hereditary myopathies or neuropathies in asymptomatic children. It may have also a prognostic value as in adults, especially in nerve traumas.
The authors tried to test the diagnostic yield of muscle and nerve biopsies in children and studied the relationships between clinical, electrophysiological and histopathological findings in 97 cases seen over a 3-year period. Specific histopathological lesions were described in 17.9% of muscle biopsies and 27.2% of nerve biopsies. In 65% of the cases, the biopsy revealed or confirmed the diagnosis, or specified the process (neurogenic or myogenic). Histopathology was concordant with clinical and electrophysiological findings in 83.5% of the cases.
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After voluntary inhalation of a domestic solvent containing N-Hexane and N-Heptane for three months, a 23-year-old woman developed motor deficit of the lower limbs, sensory symptoms and areflexia. Clinical disorders continued to progress after discontinuation of the intoxication, with a parallel aggravation of the E.M.G. disturbances. A nerve biopsy with ultrastructural study showed axon dilatation with accumulation of neurofilaments. The clinical, electrophysiological and pathological features of neuropathies induced by hexacarbon solvents are reviewed and their pathogenesis is discussed.
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Three children referred for electromyographic investigations presented difficulty in standing upright and in walking. They walked on the points of the feet. The disorder had appeared several years after the development of normal walking. Electrophysiological tests demonstrated a severe peripheral neuropathy with slowing of motor conduction in all four limbs and severe disturbances of sensory conduction. The purely peripheral nature of the lesion was confirmed by studying cortical somatesthetic potentials. E.M.G. and stimulus-detection examinations in family ascendants free from any clinical disorders revealed a similar neuropathy. Biopsy confirmed the presence of a demyelinating neuropathy with schwannian hyperplasia in adult subjects. Biological tests showed anomalies of blood saturated/unsaturated fatty acid ratios. This represents therefore a rather unusual initial clinical presentation of a hypertrophic form of Charcot-Marie's disease with dominant transmission, or form I in Dyck's classification of sensory-motor hereditary neuropathies. Families of children presenting this type of clinical or electrical picture should undergo routine electrophysiological tests. In this way, a whole series of peripheral neuropathies previously considered as sporadic or transmitted in a recessive mode will be found to form part of the group of dominant forms.
A case of chronic disabling sensory-motor polyradiculitis occurring in a female patient and followed up for 18 months is reported. Clinical findings were papilledema, hypertrichosis, abnormal pigmentation of the skin, generalized edema, and spontaneous cutaneous necrosis. The diagnosis of a plasmocytic dyscrasia to IgA Lambda was made at a late stage of the disease. There were no signs of a solitary plasmocytoma or of diffuse myeloma. At autopsy, there were mild lesions in the peripheral nervous system, but no plasmocytic infiltration or amylosis was seen on optical or electron microscopy. This case is similar to other clinical cases reported mainly by Japanese authors.
Two cases of mainly interstitial myositis with epitheloid and giant cells are reported. In the first case, the late amyotrophic-type paralysis of the two girdles, of the clinical myositis type, was associated with severe myocardial damage, the atrioventricular conduction disorder requiring a pace-maker. This observation would appear to be a true polymyositis of the collagen diseases, in view of the associated skin affection, death occurring within a year, a large increase in serum muscle enzymes and urine creatine, the existence of a rheumatoid factor and striated muscle antibodies, and a large increase in M and G immunoglobulins. In the 2nd case, the amyotrophic affection of the pelvic girdle was associated with progressive blindness, diabetes insipidus, and anterior pituitary insufficiency. Death occurred after two years and autopsy showed a suprasellar dysgerminoma (ectopic pinealoma) without any visceral localization of sarcoidosis. The authors discuss the concept of granulomatous polymyositis, autonomous with respect to the sarcoidosis, and sometimes symptomatic of an inflammatory connective tissue condition or a malignant tumor.
Five cases of peripheral neuropathies occurring with monoclonal gammapathies are studied by means of nerve biopsies. Immunofluorescence and HRP-immunolabelling with the electron microscope are performed. The direct pathogenic role of a subperineurial immunoglobulin deposit is pointed out. The relationship of the heavy chain monoclonal immunoglobulin with amyloid fibrils is discussed.
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Analysis of 105 peripheral and central nervous system complications in 1062 monoclonal gammapathies draws attention to two types of phenomena. The possibility of pure neurological manifestations of IgM monoclonal gammapathies with macroglobulinorachia leads to discussion of their nosological position in relation to Waldenström's disease, Burkitt's lymphoma and Marek's disease. It is suggested that these cases should be reclassified under the heading "secreting neurolymphomatosis". Immunofluorescence and electron microscopy of 10 biopsies of the peripheral nerve showed deposits of monoclonal immunoglobulin whose function in determining peripheral neuropathies is discussed. The simultaneous presence of lymphoid infiltration, amyloid deposits and the monoclonal immunoglobulin (M component) suggests that this immunoglobulin could be the link between the cellular infiltrate secreting it and amyloid infiltration which would be the visible manifestation of it.
Twenty-five cases of amyotrophic lateral sclerosis occurring before the age of 40, collected between 1963 and 1973 in the Neurological Hospital, Lyons, have prompted the authors to make the following observations. -The incidence of disease seems to have been on the increase during this period both in absolute terms and relatively speaking (relative to the total number of patients hospitalized and to the total number of patients hospitalized for amyotrophic lateral sclerosis after the age of forty). -The 25 cases involved mostly women--the opposite of what was found in cases of amyotrophic lateral sclerosis occurring after forty. -Only one was possibly familial. -The onset is mostly with purely pyramidal features or of the topographically circumscribed peripheral type. Development appears to take longer than the average for amyotrophic lateral sclerosis. It would be worth while confirming these findings by analysis of other similar groups.
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