[Idiopathic intracranial hypertension during treatment of Cushing's disease with mitotane].
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Biomedical subjects
Publications and source records attributed to L Boissonnot.
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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.
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Eighty-seven patients (mean age 73 years) with histologically proven Horton's disease were followed up in 5 different specialized hospital units from 1970 to 1984. Except for one patient treated exclusively with antimalarials, all were under corticosteroids. The mean initial dose of prednisone or equivalent was 0.7 mg/kg/day and the mean maintenance dose, 10 mg/day. Steroids could be discontinued in 21 cases after 36 months on average; the other patients could not be weaned, even though 4 of them had been on steroids for more than 10 years. Side-effects were noted in 43 cases. Twenty-four patients died, mostly of cardiovascular diseases. Survival rates were 89 +/- 6.35% at 1 year, 60.2 +/- 8.7% at 5 years and 48 +/- 11.3% at 10 years. In the long run, mortality in our series proved to be exactly the same as in the general population. Nineteen patients had ocular manifestations of giant cell arteritis always from the onset, except for a fall in visual acuity; 26 relapses were observed in 18 patients, either during reduction of steroid dosage (21 cases) or after withdrawal (5 cases). In 2 cases histology showed typical lesions of giant cell arteritis after 41 and 50 months respectively under corticosteroid therapy.
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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The blink reflex obtained from 66 patients with multiple sclerosis (MS) shows 73% pathologic responses with MS considered as definite, 50% with MS considered as probable and only 18% with possible MS. These findings are of great diagnostical help, particularly in the absence of clinical evidence of involvement of the brain-stem. Among 8 electrological syndromes of alteration of the blink reflex, those due to large lesions of the brain-stem are often associated with definite MS, but small alterations of the blink reflex are more valuable for multifocal characterisation of the illness.
The study of 430 cerebrospinal fluids resulting from a neurological department showed 67 pathological medical cases according to following tests: intrathecal IgG synthesis and/or restricted heterogeneity with immuno-electrophoresis. We established a biological classification to approach the different etiologies. This classification was used for multiple sclerosis for which various strictly clinical classifications are not always able to diagnose the illness, as we show it for one of them.
112 echocardiographies and 91 long-term Holter recordings were performed looking for an emboligenic cardiac lesion in patients in sinus rhythm and free of any major carotid atherosclerosis. The results were evaluated in relation to the emboligenic potential of the lesions observed. 5 positive results were obtained (4.46%) on echocardiography (3 aneurysms of the interatrial septum, 1 myxoma and 1 endocarditis) and 10 positive results (10.99%) were demonstrated on the Holter monitor. Among the 45 (40.18%) echocardiographic anomalies capable of constituting a cardiac site of origin of emboli, there were 18 cases of dilatation or hypertrophy of the left chambers of the heart, 13 cases of calcification of the aortic valve, 8 cases of prolapse of the mitral valve and 8 cases of calcification of the mitral ring. Finally, 55.35 per cent of the echocardiographic examinations and 52.74 per cent of the Holter examinations were found to be normal. Although the yield of these examinations is low, the anomalies discovered were definitely responsible for the cerebral emboli and could only have been demonstrated by such investigations.
A 11 cases study of hematoma of the iliacus muscle underlines better results obtained by C.T. Scanner compared to those of ultrasonography examination in term of definite diagnoses. Comparison of density values and repeated examination were useful to assert diagnosis and to appreciate the course of the illness. Only C.T. Scanner was able to show which portion of the muscle was concerned. Authors correlated the C.T. Scanner results to the nature of the entrapment neuropathy clinically found: an isolated femoral nerve palsy or association with an obturator nerve dysfunction.
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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.
The blink reflex combined with stimulo-detection of the facial nerve was studied in 50 patients presenting Guillain-Barré polyradiculoneuritis. The reflex was normal in more than half the cases. The most frequent electrological picture consisted in bilateral lengthening of the early and late reflex responses of the blink reflex with unilateral or bilateral increase of motor facial latency. The blink reflex showed various abnormalities in slightly more than one third of the cases of polyradiculoneuritis presenting no clinical signs of facial involvement, thus constituting subclinical lesional evidence of the reflex arc.
67 patients presented with a syncope of unknown origin following the usual exploration and were the subject of a more elaborated study: echocardiography (echo 2D) and Holter. 3.5 per cent of the Holter recordings enabled to ascribe to the heart the cause of the syncope. More than half of the Holters disclosed abnormalities of the cardiac rhythm leading to an anti-arrhythmic therapy in some cases, even when the cardiac origin of the loss of consciousness (LC) could not be confirmed. Echocardiograms could not lead to the diagnosis and less than one fourth of them disclosed abnormalities. In a developing study (14.8 +/- 8.7 months) that was carried out, 3 out of 4 patients did not present with any more LC; 3 died (6%) and 9 (18%) had LC again. On the other hand, in 5 patients (10%) a diagnosis could be evoked at the end of this follow-up. This study confirms the difficulties of diagnosis of this group of syncope of unknown etiology, as evidenced by the low results of intense and costly explorations and the little information obtained, even after a follow-up of more than 1 year.
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Two years after surgery followed by chemotherapy and radiotherapy of a temporal glioblastoma, a 51 year-old woman presented pluriradicular symptoms ascribed to compression of the left part of the cauda equina. The second operation confirmed the metastatic nature of the compression, and histological investigation proved the similarity of the neoplastic tissues taken from the medullary and cerebral regions. The authors stress the rarity of the clinical forms of medullary metastases of glioblastomas. The factors conducive to this fluid effusion are trauma and ventricular effraction by the original tumour. Neither screening nor prophylactic irradiation seem necessary in view of the low frequency of these metastases.