Affiliations: Department of Pediatrics, Pediatric Neurology Unit,
Hospital de Santa Maria, Lisboa, Portugal | Department of Neurology, Neuropathology Laboratory,
Hospital de Santa Maria, Lisboa, Portugal
Note: [] Correspondence: Dr. Sara Pinto, Department of Pediatrics,
Hospital de Santa Maria, Av. Professor Egas Moniz, 1649-035 Lisboa, Portugal.
Tel.: +351 21 7805016; Fax: +351 21 7805623; E-mail: pintosara@gmail.com
Abstract: Primary angiitis of the central nervous system (CNS) is a recently
described and rare disease in children. It is confined to the CNS and its
etiology is unknown. A 10-year-old boy, previously healthy, presented with
fever, headache and focal seizures, followed by left hemiparesis and central
facial palsy. Brain magnetic resonance imaging (MRI) showed a large
parietooccipital, cortical and subcortical lesion, hyperintense in
T2-weighted/fluid attenuated inversion recovery imaging. Subsequently, he
presented a rapid neurological deterioration by weekly relapses, having several
focal seizures, worsening of previous deficits and appearance of new ones on
the right hemibody. He became aphasic and developed encephalopathy.
Accordingly, MRI showed an extension of the previous lesion and appearance of
new ones on the left hemisphere. He was initially treated with broad spectrum
antibiotics and later immunoglobulin, methylprednisolone pulses and daily oral
prednisolone, without sustained improvement. Etiologic investigations,
including infectious or neurometabolic disease and systemic vasculitis were
negative. Cerebral angiography was normal. A lesional biopsy confirmed a small
vessel primary angiitis of the CNS. Seven wk after symptom onset, he was
started on monthly cyclophosphamide (seven pulses) with prednisolone, achieving
clinical stabilization. Mycophenolate mophetil was then substituted for
cyclosphosphamide for 18 months. During a 2-year follow-up, no new clinical or
imaging deterioration has been noted. Motor deficits have improved
significantly but severe expression aphasia and cognitive deficit remains. He
also developed refractory multifocal epilepsy. Small vessel primary angiitis of
the CNS should be considered in a child with rapidly progressing acquired
neurologic deterioration, multifocal inflammatory lesions on MRI and normal
angiography. Prompt diagnosis and aggressive treatment is crucial for a favorable outcome.
Keywords: Angiitis, vasculitis, small vessels, CNS, primary, child
