Reversible cerebral vasoconstriction syndrome: a rare arteriopathy of childhood

Headache is a common condition of childhood. Although often benign, it may be a sentinel sign of a range of serious neurological conditions associated with significant morbidity and mortality. Durrleman et al. report a case series of children with system lupus erythematosus who presented with severe headache and no or mild hypertension, in association with magnetic resonance angiography (MRA) findings that detected anterior and posterior circulation large vessel stenosis. Escalation of immunosuppressive therapies occurred before diagnosis of reversible vasoconstriction syndrome and there was institution of calcium channel antagonist therapy in one participant. Subcortical and cortical infarction were sustained in two children, with complete normalization of MRA findings occurring by 5 months. This paper highlights the challenges and importance of accurate diagnosis in the management of headache in childhood. An approach to the differential diagnosis of headache in childhood is provided by several publications including the 2013 International Classification of Headache Disorders. Headache associated with vasculopathy is recognized as one of the secondary headache syndromes. Arteriopathy is commonly associated with childhood arterial ischaemic stroke. It is a predictor of stroke recurrence and poor neurological outcome. In a recent study by the collaborative network of International Paediatric Stroke Study investigators of over 2000 children who reported headache at stroke onset, children with arteriopathy and acute or chronic conditions of the head and neck were more likely to report headache than those without. This study highlights the importance of headache and its association with arteriopathy in children presenting with stroke. Arteriopathy diagnosis is key and required for the provision of aetiology-guided therapies. However, specific arteriopathy diagnosis in the acute setting is a challenge. This is because the common arteriopathies of childhood typically affect the carotid T junction, i.e. the distal internal carotid arteries, proximal middle cerebral, and anterior cerebral arteries. Neuroimaging approaches to aid differentiation between the three most common conditions – focal cerebral arteriopathy/transient cerebral arteriopathy, moyamoya, and dissection – in the absence of other features such as collaterals, has been advanced by the application of magnetic resonance imaging (MRI) techniques, such as high-resolution vessel wall imaging. The MRI findings of bilateral, multifocal, anterior, and posterior circulation involvement in this case series was suggestive of a diffuse process, such as reversible vasoconstrictive syndrome, posterior reversible leukencephalopathy, or intracranial large vessel vasculitis. Clinical work-up resulted in a posteriori diagnosis of reversible cerebral vasoconstrictive syndrome. Reversible cerebral vasoconstrictive syndrome is an aetiologically heterogenous arteriopathy that is underrecognized in childhood. Although the underlying pathology is not fully understood, transient dysregulation of cerebral vascular tone due to sympathetic hyperactivity is suggested. Advances in current approaches in imaging have not yet been demonstrated to reliably aid in the classification of these rarer arteriopathies. An over-reliance on MRA findings and a reluctance to conduct conventional catheter angiography continue to pose a barrier to the management of these conditions in childhood. This case series highlights the specific challenges that present themselves when managing a child with severe headache associated with cerebral arteriopathy and MRI evidence of stroke. It highlights the need and importance of aetiological diagnosis of headache in association with arteriopathy as a guide to the direction of care. Increased awareness of this less common childhood condition and the development of improved non-invasive approaches in vascular imaging would facilitate advances in the management of this rare arteriopathy of childhood.