Recurrent leukoencephalopathy with microhemorrhages: gradient-echo MRI study diagnostic value in CNS primary angiitis

Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion reco...

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Bibliographic Details
Published in:Multiple sclerosis 2008-09, Vol.14 (8), p.1139-1141
Main Authors: Arias, M, Osorio, XR, Dapena, D, Arias-Rivas, S, Vázquez, F
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Cerebral Hemorrhage - pathology
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Humans
Investigative techniques, diagnostic techniques (general aspects)
Leukoencephalitis, Acute Hemorrhagic - pathology
Magnetic Resonance Imaging
Male
Medical sciences
Nervous system
Neurology
Radiodiagnosis. Nmr imagery. Nmr spectrometry
Recurrence
Vasculitis, Central Nervous System - diagnosis
Vasculitis, Central Nervous System - pathology
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Summary:Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS.
ISSN:1352-4585
1477-0970
DOI:10.1177/1352458508094642