Involvement of CD45RO+ T Lymphocyte Infiltration in a Patient with Primary Angiitis of the Central Nervous System Restricted to Small Vessels

Iwase, Tamaki; Ojika, Kosei; Mitake, Shigehisa; Katada, Eiichi; Katano, Hiroyuki; Mase, Mitsuhito; Yoshida, Shigeaki; Ueda, Ryuzo

doi:10.1159/000052120

Cited by 37 publications

(19 citation statements)

References 9 publications

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“…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%

“…In the majority of patients with PACNS, abnormalities, albeit nonspecific, are present on cerebral angiograms (4). Brain biopsies in adult patients with PACNS frequently show granulomatous vasculitis of parenchymal and leptomeningeal arterial vessels in the brain (5); however, reports of nongranulomatous, lymphocytic CNS vasculitis have been published (6)(7)(8).…”

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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

143

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%

mentioning

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

143

110

View full text Add to dashboard Cite

show abstract

“…Leukocytes, in particular memory T cells, concentrated in the vessel wall of PCNSV samples may indicate the presence of a cross-reacting antigenic trigger [42]. Metalloproteinase 9 seems to play an important role in vessel wall damage in animal models of vasculitis [43]. …”

Section: Discussionmentioning

confidence: 99%

The Role of Catheter Angiography in the Diagnosis of Central Nervous System Vasculitis

et al. 2016

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Background: Central nervous system vasculitis (CNSV) is a rare disorder, the pathophysiology of which is not fully understood. It involves a combination of inflammation and thrombosis. CNSV is most commonly associated with headache, gradual changes in mental status, and focal neurological symptoms. Diagnosis requires the effective use of history, laboratory testing, imaging, and biopsy. Catheter angiography can be a powerful tool in the diagnosis when common and low-frequency angiographic manifestations of CNSV are considered. We review these manifestations and their place in the diagnostic algorithm of CNSV. Summary: We reviewed the PubMed database for case series of CNSV that included 5 or more patients. Demographic and angiographic findings were collected. Angiographic findings were dichotomized between common and low-frequency findings. A system for incorporating these findings into clinical decision-making is proposed. Key Message: CNSV is a diagnostic challenge due to the absence of a true gold standard test. In the absence of such a test, catheter angiography remains a central piece of the diagnostic puzzle when appropriately employed and interpreted.

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“…There is evidence that it is a parainfectious phenomenon, triggered by microorganisms through molecular mimicry, generating an antigenspecific immune response, with an inflammatory process in the walls of cerebral vessels by infiltration of CD4 + T lymphocytes, memory cells of the cellular immune system [5]. In children with CNPSV, evidence in immunohistochemical biopsy studies shows an inflammatory infiltrate with a predominance of cytotoxic CD8 + T lymphocytes [6].…”

Section: Pathophysiologymentioning

confidence: 99%

Primary central nervous system vasculitis: a review of this challenging diagnosis

Ftm¹,

Lfo²

2017

Rheumatol Orthop Med

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The primary vasculitis of the central nervous system is still a controversial subject and in constant study for a better understanding of all its aspects. It is a pathophysiology disease that is not yet fully understood, and with probable immunological and biological mechanisms of very high complexity, which go beyond the current technical and scientific limits. Its clinical manifestations are challenging even for the most experienced neurologists, which requires, in addition to academic knowledge, a unique ability to interpret clinically and to weigh the results presented in complementary tests. This is because we do not have any non-invasive diagnostic tool that confirms, or discards, with full security, the possibility of VPSNC. In practice, the confirmatory examination is considered the gold standard and is still the brain biopsy, which is usually performed only in exceptional cases.

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Involvement of CD45RO+ T Lymphocyte Infiltration in a Patient with Primary Angiitis of the Central Nervous System Restricted to Small Vessels

Cited by 37 publications

References 9 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

The Role of Catheter Angiography in the Diagnosis of Central Nervous System Vasculitis

Primary central nervous system vasculitis: a review of this challenging diagnosis

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