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Intracranial hypertension as an unusual presentation of adrenoleukodystrophy
Author(s) -
Smadja D.,
Cabre Ph.,
Kujas M.,
Vernant J.C.,
Rivierez M.
Publication year - 1997
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.1997.tb00371.x
Subject(s) - medicine , adrenoleukodystrophy , status epilepticus , presentation (obstetrics) , adrenal insufficiency , epilepsy , brain biopsy , intracranial pressure , family history , computed tomography , radiology , magnetic resonance imaging , neuroimaging , pathology , pediatrics , psychiatry , peroxisome , receptor
A 19‐year‐old man with a history of adrenal insufficiency and epilepsy for several years presented with headache, obtundation, status epilepticus and papilloedema. A computed tomography‐scan was consistent with a fronto‐collosal glioma. However, this patient's condition spontaneously improved after a brain biopsy. Therefore, the diagnosis of adrenoleukodystrophy (ALD) was established, based on histological findings, positive family history and plasma fatty acid analysis. In ALD, an acute and reversible worsening of brain demyelination with increased intracranial pressure may be evocative of a cerebral neoplasm.