Unihemispheric cerebral vasculitis mimicking Rasmussen’s encephalitis

Derry, Christine; Dale, Russell C.; Thom, Maria; Miller, D.H.; Giovannoni, Gavin

doi:10.1212/wnl.58.2.327

Cited by 37 publications

(33 citation statements)

References 5 publications

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“…Abnormal levels of systemic inflammation markers are infrequently reported in adult PACNS (3,15) and in the limited literature of pediatric PACNS (10,16). However, CSF abnormalities are common in adult PACNS (13,17), whereas CSF data from previous pediatric case reports are controversial (14,18,19).…”

Section: Angiography-negative Primary Cns Vasculitis In Childrenmentioning

confidence: 99%

“…The histologic characteristics of granulomatous CNS vasculitis include a transmural infiltrate with well-defined granulomas, evidence of giant cells, and areas of vessel wall necrosis (23,24). Lymphocytic, nongranulomatous vasculitis is characterized by a transmural, predominantly T cell and B cell infiltrate, without evidence of granulomas (14,19). However, most patients have positive results of angiography, and few case reports of angiography-negative CNS lymphocytic vasculitis have been published in the adult literature (6,7).…”

Section: Angiography-negative Primary Cns Vasculitis In Childrenmentioning

confidence: 99%

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Angiography‐negative primary central nervous system vasculitis in children: A newly recognized inflammatory central nervous system disease

Benseler¹,

deVeber²,

Hawkins³

et al. 2005

Arthritis & Rheumatism

146

110

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Inflammatory central nervous system (CNS) diseases in childhood comprise a wide spectrum of heterogeneous conditions. We studied 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. We determined that angiography-negative, biopsy-confirmed primary smallvessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. Use of immunosuppressive therapy plus aspirin leads to an excellent neurologic outcome.

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Section: Angiography-negative Primary Cns Vasculitis In Childrenmentioning

confidence: 99%

Section: Angiography-negative Primary Cns Vasculitis In Childrenmentioning

confidence: 99%

Angiography‐negative primary central nervous system vasculitis in children: A newly recognized inflammatory central nervous system disease

Benseler¹,

deVeber²,

Hawkins³

et al. 2005

Arthritis & Rheumatism

146

110

View full text Add to dashboard Cite

show abstract

“…1 Four children treated with corticosteroids for at least 6 months, of which 3 also received cytotoxic agents for 6 to 22 months, experienced no recurrence during a follow-up of 14 to 59 months. 2 However, case 3 and other reports 5,7 reveal that nongranulomatous SV PACNS can recur despite prolonged immunosuppressant treatment that combines corticosteroids and cytotoxic agents.…”

Section: Discussionmentioning

confidence: 92%

Primary Angiitis of the Central Nervous System: Neurologic Deterioration Despite Treatment

Tiège

Bogaert²,

Aeby³

et al. 2011

Pediatrics

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Primary angiitis of the central nervous system (PACNS) is an idiopathic vasculitis confined to the central nervous system. In children with PACNS, small-vessel (SV) involvement is characterized clinically by progressive neurologic symptoms, multifocal lesions on brain imaging, occasional pseudo-tumor presentation, and normal angiogram results in most patients. Small case series of patients with SV PACNS with short follow-up usually reveal favorable outcomes in children treated with immunosuppressive therapy. We report here the cases of 3 children with biopsy-confirmed SV PACNS and long-term follow-up who developed different patterns of neurologic deterioration despite immunosuppressive therapy. One patient had transient ischemic attacks shortly after initiation of corticosteroid treatment. Early ischemic events probably result from residual thrombogenicity or residual inflammation of recently affected vessels, which supports the use of antiplatelet agents and suggests potential benefits of stronger immunosuppressive therapy. In contrast, the other 2 patients had later neurologic deterioration after corticosteroid withdrawal, which suggests failure of initial immunosuppressant treatment and the need for stronger agents, longer treatment duration, or both. All patients responded to long-term treatment with corticosteroids combined with cytotoxic agents. This particular combination is probably indicated in many cases of SV PACNS, including those with neurologic deterioration that occurs during maintenance corticotherapy or after corticosteroid withdrawal. In 1 case, SV PACNS recurred several years after discontinuation of combination therapy. Long-term relapses may reflect intrinsic predispositions to SV PACNS rather than treatment failure. These cases highlight different chronological patterns of neurologic deterioration despite immunosuppressive therapy, which supports the relevance of monitoring clinical, laboratory, and radiologic responses to treatment and of long-term follow-up of patients with SV PACNS.

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“…The unilateral necrosing form is very uncommon and has been reported in adults and children (Derry et al 2002;Benseler et al 2006). …”

Section: Unilateral Formmentioning

confidence: 99%

Primary Central Nervous System Angiitis

Bouhaouala

Naggara

Ghorbel

et al. 2011

Systemic Vasculitis

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Unihemispheric cerebral vasculitis mimicking Rasmussen’s encephalitis

Cited by 37 publications

References 5 publications

Angiography‐negative primary central nervous system vasculitis in children: A newly recognized inflammatory central nervous system disease

Angiography‐negative primary central nervous system vasculitis in children: A newly recognized inflammatory central nervous system disease

Primary Angiitis of the Central Nervous System: Neurologic Deterioration Despite Treatment

Primary Central Nervous System Angiitis

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