Isolated Angiitis of the Central Nervous System in Childhood

Abstract

Isolated angiitis of the central nervous system, a rare inflammatory condition of the nervous system, characterized by vasculitis of the small vessels, has not, to the best of our knowledge, been described in childhood. It usually presents with a diversity of neurological symptoms in the fifth to eighth decades of life. We reviewed the case of an 8 year old male with autopsy proven isolated cerebral angiitis, to encourage the consideration of this disorder in children; and to emphasize the lack of sensitivity of present modalities of neurological investigation in the diagnosis. RESUME: Angeite isolee du systeme nerveux central chez I'enfant L'angeite isolee du systeme nerveux central, une affection inflammatoire rare du systeme nerveux, caracterisee par une vasculite des petits vaisseaux, n'a jamais, au meilleur de notre connaissance, ete decrite dans l'enfance. Elle se presente avec divers symptomes neurologiques entre la cinquieme et la huitieme decade de vie. Nous avons revu le cas d'un jeune gar?on de 8 ans avec angfiite ceVebrale isolee prouv£e a l'autopsie, pour inciter les cliniciens a considerer cette maladie chez les enfants et pour souligner le manque de sensibilite des modalites actuelles d'investigation neurologique dans ce diagnostic.Can. J. Neurol. Sci. 1990; 17:151-154 Isolated angiitis of the central nervous system is a rare vasculitic process involving only the small vessels of the brain. There have been approximately 36 histopathologically confirmed cases since 1922. 1 The clinical spectrum of the disease has been well described by several authors, and the majority of the cases have occurred in the fifth to eighth decades of life.'" 5 We present the case of an eight year old boy with post-mortem confirmation of this disorder. This case expands our understanding of the clinical spectrum of the disease, in that, to the best of our knowledge, he is the youngest case described, his clinical course was complicated by recurrent intracerebral bleeds, and his investigations included the use of magnetic resonance imaging. CASE REPORT

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

“…Our experience of rapidly progressing neurologic deficits including the addition of new and/or worsening of preexisting deficits is supported by the pediatric literature and is in contrast to a more indolent course reported in biopsy-proven granulomatous vasculitis as reported in the adult literature (4,9). In the pediatric cases of CNS vasculitis reported in the literature, disease progression was most commonly rapid and led to a fatal outcome (9,14). Our cohort was small and therefore may not capture the entire spectrum of clinical features at the time of diagnosis of small-vessel cPACNS.…”

“…Earlier reports suggested a poor prognosis (14,18,(24)(25)(26). More recent case reports have described a good response to steroids plus cyclophosphamide (9,10).…”

“…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).…”

“…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).…”

Primary central nervous system vasculitis in children

“…The presenting features in children have been poorly characterized, and there are few published case series (1)(2)(3). The diagnostic features of cPACNS have been based on rare autopsy findings (4) and, more recently, on angiographic findings (1).…”