Transient Cerebral Arteriopathy: A Disorder Recognized by Serial Angiograms in Children With Stroke

Abstract

Repeated clinical evaluation and cerebral arteriography during the evolution of ischemic strokes of idiopathic origin allowed us to characterize a transient cerebral arteriopathy. We retrospectively studied the clinical characteristics, course, and neuroimaging features of this disorder in nine children. Of 34 children with ischemic strokes seen consecutively between 1984 and 1995, 9 (26%) were diagnosed as having transient attack of the cerebral arterial wall, termed transient cerebral arteriopathy. All of these patients had previously been in good health. The mean age at the time of the first stroke was 6 years (range, 2 9/12 years to 13 4/12 years). All children presented with acute hemiplegia. A recurrence of the stroke took place 3 months at the latest after the initial infarct in three children (mean clinical follow-up 2 7/12 years). Cerebral imaging in all the patients showed small subcortical infarcts located in basal ganglia or internal capsule. Arteriography revealed multifocal lesions of the arterial wall (focal stenosis or segmental narrowing), mostly located in the initial parts of basal arteries of the carotid system. Longitudinal arteriographic follow-up showed initial worsening of these arterial lesions (n = 5) for a maximum duration of 7 months followed by complete regression (n = 2), improvement (n = 5), or stabilization of the lesions (n = 2). Five patients had a complete clinical recovery. Further studies are necessary to confirm a presumed inflammatory cause of this arteriopathy.

Primary central nervous system vasculitis in children

“…Previous reports described the association of stroke and cerebral vasculitis causing large vessel stenoses (16). The term transient cerebral arteriopathy was coined by Chabrier et al, who described a series of children presenting with arterial ischemic stroke and large vessel stenoses (17). The latter condition is thought to represent a localized cerebral vessel inflammatory condition that is provoked, in some cases, by varicella (17).…”

“…The term transient cerebral arteriopathy was coined by Chabrier et al, who described a series of children presenting with arterial ischemic stroke and large vessel stenoses (17). The latter condition is thought to represent a localized cerebral vessel inflammatory condition that is provoked, in some cases, by varicella (17). The clinical and neuroimaging features and course of transient cerebral arteriopathy and nonprogressive PACNS are similar, suggesting that they are likely to be the same illness (10).…”

Diagnostic pitfalls in paediatric ischaemic stroke

“…The course of the disease was one of the reasons to revise the diagnosis in three patients. Two patients with a lenticulostriate territory middle cerebral artery infarct showed the typical vascular abnormalities described in transient Diagnostic Pifalls in Paediatric Stroke Kees P J Braun et al 987 cerebral arteriopathy (TCA; Chabrier et al 1998). The initial aetiological diagnosis in these patients (unilateral moyamoya and intracranial fibromuscular dysplasia) was changed during the course of their disease because of stabilization or improvement of the arteriopathy with repeated vascular imaging, and because of increasing familiarity with the diagnosis TCA.…”

Transcranial Doppler in children

“…The spectrum differs from that in adults and includes moyamoya, vasculitis, dissection, and transient cerebral arteriopathy (TCA). TCA, first recognised as an important cause of childhood stroke in 1998 [56], is characterised by lenticulo-striate infarction due to non-progressive unilateral arterial disease affecting the supraclinoid internal carotid artery and its proximal branches. The course of the disease is characterised by the stabilisation, improvement or even normalisation of the Fig.…”