Unihemispheric central nervous system vasculitis

Patients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy ma...

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Main Authors: Sikawat Thanaviratananich, Bashar Katirji
Format: Article
Language:English
Published: Elsevier 2017-06-01
Series:eNeurologicalSci
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2405650217300151
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author Sikawat Thanaviratananich
Bashar Katirji
author_facet Sikawat Thanaviratananich
Bashar Katirji
author_sort Sikawat Thanaviratananich
collection DOAJ
description Patients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. MRI brain revealed multiple hyperintense lesions confined to only the right hemisphere with contrast enhancement, involving both white and grey matters. He was treated with a methylprednisolone for 5 days followed by prednisone for suspected acute disseminated encephalomyelitis without improvements. He was presented again with left-sided weakness, transient dysarthria and black objects in left visual field. MRI brain was unchanged. MR angiogram and conventional cerebral angiogram were normal. Autoimmune work-ups were all negative. A brain biopsy showed evidence of PCNSV. He was then successfully treated with intravenous cyclophosphamide followed by oral azathioprine. On a follow-up 3 years later, he remains asymptomatic on azathioprine and a repeat MRI showed all areas of enhancement were gone.
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spelling doaj.art-72b5dac21961469db515f4835a95a5642022-12-21T19:48:22ZengElseviereNeurologicalSci2405-65022017-06-017C444810.1016/j.ensci.2017.05.003Unihemispheric central nervous system vasculitisSikawat ThanaviratananichBashar KatirjiPatients with primary central nervous system vasculitis (PCNSV) usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right) hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. MRI brain revealed multiple hyperintense lesions confined to only the right hemisphere with contrast enhancement, involving both white and grey matters. He was treated with a methylprednisolone for 5 days followed by prednisone for suspected acute disseminated encephalomyelitis without improvements. He was presented again with left-sided weakness, transient dysarthria and black objects in left visual field. MRI brain was unchanged. MR angiogram and conventional cerebral angiogram were normal. Autoimmune work-ups were all negative. A brain biopsy showed evidence of PCNSV. He was then successfully treated with intravenous cyclophosphamide followed by oral azathioprine. On a follow-up 3 years later, he remains asymptomatic on azathioprine and a repeat MRI showed all areas of enhancement were gone.http://www.sciencedirect.com/science/article/pii/S2405650217300151Central nervous system vasculitisVasculitisSeizureAutoimmune disease
spellingShingle Sikawat Thanaviratananich
Bashar Katirji
Unihemispheric central nervous system vasculitis
eNeurologicalSci
Central nervous system vasculitis
Vasculitis
Seizure
Autoimmune disease
title Unihemispheric central nervous system vasculitis
title_full Unihemispheric central nervous system vasculitis
title_fullStr Unihemispheric central nervous system vasculitis
title_full_unstemmed Unihemispheric central nervous system vasculitis
title_short Unihemispheric central nervous system vasculitis
title_sort unihemispheric central nervous system vasculitis
topic Central nervous system vasculitis
Vasculitis
Seizure
Autoimmune disease
url http://www.sciencedirect.com/science/article/pii/S2405650217300151
work_keys_str_mv AT sikawatthanaviratananich unihemisphericcentralnervoussystemvasculitis
AT basharkatirji unihemisphericcentralnervoussystemvasculitis