Vasculitis in the central nervous system

Calabrese, Leonard H.; Duna, George F.; Lie, J.T.

doi:10.1002/1529-0131(199707)40:7<1189::aid-art2>3.0.co;2-4

Cited by 245 publications

(35 citation statements)

References 63 publications

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“…Over time, it became apparent to workers in the field that PACNS was a heterogeneous entity, with different subsets based on clinical, laboratory, angiographic and pathological findings 2. Granulomatous angiitis of the CNS (GACNS) is considered the most severe subset of PACNS; it accounts for approximately 20% of all patients with PACNS 3 4. GACNS has also been described in association with cerebral amyloid angiopathy (CAA),5 – 7 referred to as amyloid-β related angiitis (ABRA) or CAA-related inflammation.…”

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confidence: 99%

Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Molloy

Singhal

Calabrese

2008

Annals of the Rheumatic Diseases

108

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Although rare, PACNS should be considered in the differential diagnosis of ML; greater awareness of this manifestation may facilitate more prompt diagnosis and treatment. Biopsy evidence of angiitis is required for diagnosis; specimens should routinely be stained for amyloid. While excision of the lesion may be curative, aggressive immunosuppressive therapy is associated with favourable outcomes and may obviate the need for surgery.

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confidence: 99%

Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Molloy

Singhal

Calabrese

2008

Annals of the Rheumatic Diseases

108

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“…In a series of 116 patients with pathologically confirmed disease, nearly 70% of patients presented with diffuse neurological dysfunction which included: Decreased cognition (83%) Headache (56%) Seizure (30%) Stroke (14%) Cerebral haemorrhage (12%)4 …”

Section: Discussionmentioning

confidence: 99%

Cerebral vasculitis presenting as a stroke

2012

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SummaryA 57-year-old man was admitted with right arm weakness and numbness on the background of intermittent headaches. On examination he was found to have mildly decreased sensation, power was 4/5 on the right side. He had dyspraxia in the right hand and was unable to spell his name. His speech was hesitant and he had left-sided visual field impairment as well as some photophobia. MRI and CT revealed multiple areas of haemorrhage and infarctions raising the possibility of primary angitis of brain. The biopsy confirmed the diagnosis. The patient responded to steroids and immunosuppressants partially. BACKGROUND

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“…Corticosteroids associated with intravenous cyclophosphamide is the usual recommended therapeutic regimen 2 4. Such treatment may lead to iatrogenic complications such as infections and neoplasia because of the alkylating properties of cyclophosphamide 8.…”

Section: Discussionmentioning

confidence: 99%

“…Treatment guidelines are based on empirical data and usually combine glucocorticosteroids and cyclophosphamide. Recommended doses, route and duration are highly variable 3 4. We report the case of a patient with PACNS, characterised by unusually prominent involvement of the posterior cerebral fossa and successful treatment with mycophenolate mofetil (MMF) in combination with steroids.…”

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confidence: 99%

Primary angiitis of the central nervous system: response to mycophenolate mofetil

Chenevier

Renoux

Marignier

et al. 2009

Journal of Neurology, Neurosurgery & Psychiatry

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Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease restricted to the CNS of unknown cause. Clinical presentation and evolution are highly variable with potentially fluctuating signs and symptoms. Brain imaging often shows supratentorial ischaemic lesions. Definite diagnosis is established by brain biopsy. Treatment usually combines glucocorticosteroids and cyclophosphamide. A case of PACNS is reported here, which was proved by a brain biopsy and characterised by unusually prominent involvement of the posterior cerebral fossa. Successful treatment with mycophenolate mofetil in combination with steroids is described.

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Vasculitis in the central nervous system

Cited by 245 publications

References 63 publications

Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Cerebral vasculitis presenting as a stroke

Primary angiitis of the central nervous system: response to mycophenolate mofetil

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