Biomedical subjects
Publications and source records attributed to J P Neau.
The authors report on the case of a young woman, seven months gone, with an history of sudden onset of successive lobar intracerebral hematomas. They revealed a Rendu-Osler-Weber disease. The mechanism is discussed.
A case of meningo-radiculitis consecutive to tick bite with isolation, for the first time in France, of a Borrelia burgdorferi strain prompted us to review the records of patients admitted to our hospital department with Lyme disease. Meningo-radiculitis was the predominant feature of the clinical syndrome. It seems to be one of the usual forms of Lyme disease in Europe.
Three cases of spinal cord tumor, revealed by hydrocephalus, are reported. Two patients showed symptoms of increased intracranial pressure and the third one presented himself with ataxic gait without intellectual impairment nor incontinence. In two cases ventriculo atrial shunt was initially inserted and the correct diagnosis was made only later on myelogram because lombosciatalgia or syndrome of the cauda equina. Clinical outcome was satisfying after surgical removal of the tumor. The nature of which was a neurinoma of the cauda equina; a lumbar intradural granuloma and an ependymoma of the cauda equina. The mechanism by which spinal cord tumors raised intracranial hypertension is discussed.
A case of carpal tunnel syndrome with skin ulcerations and osteolysis is reported. Such cases are unusual. There was a sensory and motor deficit with chronic ulcerations on the palmar surface of the third phalanx of the medius. The distal phalanx of the forefinger had been lost due to osteolysis. Surgical treatment is said to be followed by a healing of the ulcerations.
Twenty-one diabetic subjects were studied; all manifested fasting hyperglycemia and elevated Hb A1C. Motor conduction velocity of the median, ulnar and peroneal nerves; F wave of the peroneal nerve; sensory conduction velocity and sensory potential amplitude of the median and ulnar nerves and H reflex were determinated immediately before and after two days of strict glucose control with an artificial endocrine pancreas. A significant and acute improvement of some electrophysiological parameters led us to presume that repair of structural lesions would be unlikely. Only a metabolic disturbance, due to hyperglycemia, can be rapidly corrected.
A 22 year old woman had a seizure, and the skull X-ray showed an intracranial arched calcification. CT scan permitted diagnosis of dermoid cyst. Rupture of the cyst was testified by low density areas disseminated on the cortical surface and intra-ventricular fat level. Surgical removal confirmed dermoid cyst with rupture and fat fluid inside. Authors underline infrequency of such intra-ventricular fat level. CT scan, with low density of lipidic origin promote considerably such diagnosis both of the real nature of the cyst and of its rupture.
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Dapsone has been used since 1976 in the treatment of relapsing polychondritis. A critical analysis of its therapeutic effectiveness based on 2 personal cases and 14 cases reported in the literature, all treated with dapsone alone, showed that treatment had to be discontinued in 4 on account of side effects (haemolytic anaemia, erythema multiforme, somnolence, headache, nausea); 1 patient showed no improvement and 5 relapsed; 6 responded favourably and without relapse during a 3 months' to 4 years' follow-up. Considering the unpredictable course of relapsing polychondritis and the fact that some of its clinical manifestations, notably auricular chondritis, may spontaneously resolve, the response of the disease to dapsone is difficult to establish and requires to be confirmed by a controlled clinical trial.
A 31 year-old inhabitant of French Guiana was prescribed mercuric iodide per os for two and a half months. Shortly before the end of the treatment he developed fasciculations in the trunk and particularly the lower limb muscles, distal painful paresthesias with vasomotor disorders, episodes of excessive perspiration and palmoplantar erythema, moderate fluctuating hypertension, progressive loss of weight and irritability with insomnia. Clinical and electrical signs of neuropathy were lacking. The clinical picture was that of Morvan's fibrillary chorea with acrodynia, the conditions of onset strongly suggesting a mercurial intoxication. Blood and particularly urine mercury levels were elevated. Administration of dimercaprol (BAL) considerably increased urinary excretion of mercury and there was progressive improvement and finally recovery after two months of BAL treatment. This case exemplifies the possible co-existence of fibrillary chorea and acrodynia. Whereas in many cases of fibrillary chorea a precise etiology cannot be determined, the affection can be induced by mercury as by gold administration. The fact that cases of fibrillary chorea due to mercury poisoning are rarely reported may be the result of individual patient hypersensitivity or particular metabolic absorption and excretion features of mercury. This case cannot be included within the continuous activity syndrome of muscle fibers described by Isaacs, since muscle contractures were absent and there was associated acrodynia. Moreover, there was no latent polyneuropathy, in spite of the intense fasciculations. It must be concluded, therefore, that in spite of its rarity fibrillary chorea should keep its semiologic autonomy.
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Intraspinal tumors with subarachnoid hemorrhage are exceptional. They are most often localized at the cauda equina. Ependymoma is the most common tumor. Low back pain followed by headache (Fincher syndrome) should suggest hemorrhage originating from a low lesion.
The authors report the case of a 81 years-old-woman who had simultaneously atypical Cogan's syndrome, cutaneous vasculitis and diverticular sigmoiditis. The relations being between these three diseases are discussed.
Third, fourth and sixth cranial nerve palsy is a less common complication of giant cell arteritis than ischemic optic neuritis. Nevertheless, it occurs in at least 10% of cases. A case of giant cell arteritis with third nerve palsy which resolved under corticosteroid therapy is reported. The incidence of ocular complications is assessed from a review of the cases previously seen in the department and of data from the medical literature. The authors believe that the index patient has ischemic neuritis rather than myositis. It is concluded that giant cell arteritis should be considered at an early reversible stage.
Long latency event-related auditory evoked potentials, particularly the P300 wave, constitute an objective electrophysiological index of cognitive function. For this reason, these potentials have been studied in a series of 101 patients with multiple sclerosis (MS), classified according to McAlpine's criteria into definite, probable and possible cases. The patients were also classified as depressed or non-depressed according to the DSM-III and Research Diagnostic Criteria. They were also subjected to a battery of psychometric tests. In the patient population the N200 and P300 latencies were increased, as were the P200 latencies, when compared with a control population. This electrophysiological pattern had previously been observed in other conditions characterised by subcortical lesions. Partial correlations (at constant disease duration) between the disability score and the cognitive deficit were found to be significant. Patients with an increased P300 latency had a greater disability and the P300 latency was significantly correlated with the duration of the illness. The N200 and P300 latencies were increased in depressed MS subjects, but this increase did not reach the level of significance. Depression was more frequent in the more severely handicapped patients. This suggests that the origin of the depression seen in multiple sclerosis is only partly organic, and that it is one of the factors contributing to the subcortical cognitive deficit in multiple sclerosis. Progressive forms of the disease exhibited the most profound cognitive deficit, and the most marked increase in P300 latency.
A case of isolated trigeminal sensitive neuropathy revealed by a nasal ulceration is reported. Such case is quite unusual and requires a very thorough search for any local lesion or general pathology. The treatment is often disappointing.
The physiopathology of the syndrome of ophtalmoplegia-ataxia-areflexia-hyperproteinorachia, firstly described by FISHER in 1956, remains a matter of controversy among neurologists. We report a new case of recurrence of a MILLER-FISHER's syndrome. The involvement of peripheral and central structures is discussed according to recent knowledges about the immunopathology of inflammatory polyneuropathies.
Rendu-Osler-Weber disease is a generalized vascular dysplasia which also involves the central nervous system. The neurological manifestations of the disease are due either to primary intracranial or spinal vascular lesions or to neurological complications of other visceral lesions, notably those of the lung (arteriovenous fistulae). The prevention of ischaemic or infectious cerebral accidents rests on the anatomical (excision) or functional (selective embolization) exclusion of pulmonary arteriovenous fistulae, when present.
A case of acute respiratory failure revealing amyotrophic lateral sclerosis is reported. The other neurological diseases with diaphragmatic paralysis are recalled.