| Cerebral and pulmonary histiocytosis X. Neurologic manifestations disclosing a pseudotumoral formation on the floor of the 4th ventricle | |
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MedLine Citation:
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PMID: 6335282 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The authors report a case of histiocytosis X which presented with neurological manifestations in a 20 year old man: episodes of headache with vomiting, followed, three months later, by the development of paralysis of the right 6th and 7th cranial nerves associated with nystagmus. The CT scan revealed a tumour-like lesion on the floor of the IVth ventricle with a long axis of 18 mm. The chest x-ray revealed diffuse nodular opacities in the pleural and apical regions with features suggestive of histiocytosis X. The diagnosis was confirmed by surgical biopsy of the typical pulmonary nodules which were rich in histiocytes with X bodies on electron microscopy. The neurological signs disappeared after one month of treatment with Prednisone (1 mg/kg/day) and Vincaleukoblastine (10 mg/week). By the 3rd month, the pulmonary lesions were reduced and the intra-ventricular formation had regressed by 40%. In the authors' series of 29 cases of confirmed histiocytosis X in adults, the present case is the only one with a clinical neurological presentation, apart from 3 cases of diabetes insipidus. A review of the literature confirms the rarity of this type of presentation. The suggestion of the diagnosis by the chest x-ray appearance enabled a dangerous neuro-surgical operation to be avoided. |
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Authors:
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P Allard; J Kermarec; J Goasguen; M Ferry; H de Muizon; P Girard; R Herning |
Publication Detail:
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Type: Case Reports; English Abstract; Journal Article |
Journal Detail:
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Title: Revue de pneumologie clinique Volume: 40 ISSN: 0761-8417 ISO Abbreviation: Rev Pneumol Clin Publication Date: 1984 |
Date Detail:
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Created Date: 1985-03-06 Completed Date: 1985-03-06 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8406312 Medline TA: Rev Pneumol Clin Country: FRANCE |
Other Details:
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Languages: fre Pagination: 305-9 Citation Subset: IM |
Vernacular Title:
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Histiocytose X cérébrale et pulmonaire. Manifestations neurologiques révélatrices d'une formation pseudo-tumorale du plancher du IVe ventricule. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abducens Nerve Adult Brain Diseases / complications, diagnosis*, pathology Cerebral Ventricle Neoplasms / diagnosis* Cranial Nerve Diseases / etiology Diabetes Insipidus / etiology Diagnosis, Differential Facial Nerve Diseases / etiology Histiocytosis, Langerhans-Cell / diagnosis*, therapy Humans Lung Diseases / diagnosis*, pathology Male Meningeal Neoplasms / diagnosis Meningioma / diagnosis Prognosis Tomography, X-Ray Computed |
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