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Primary Central Nervous System Angiitis in a 10-year-old Girl

Published online by Cambridge University Press:  02 December 2014

Mubeen F. Rafay
Affiliation:
Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada
Trenna L. Sutcliffe
Affiliation:
Child Development Center, The Hospital for Sick Children, Toronto, Ontario, Canada
Manohar Shroff
Affiliation:
Division of Neuroradiology, The Hospital for Sick Children, Toronto, Ontario, Canada
Cynthia Hawkins
Affiliation:
Division of Neuropathology, The Hospital for Sick Children, Toronto, Ontario, Canada
Shelly Weiss
Affiliation:
Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada
Susanne M. Benseler
Affiliation:
Division of Rheumatology, The Hospital for Sick Children, Toronto, Ontario, Canada
Gabrielle deVeber
Affiliation:
Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada
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Extract

Primary angiitis of central nervous system (PACNS) is well reported, however its occurrence in the pediatric population is infrequent. We describe the clinical, neuroimaging and histopathological features of PACNS in a young girl.

A ten year-old, previously healthy girl presented with a threeweek history of progressive left hemiparesis and facial weakness. Other findings included left hemineglect, impaired concentration and memory. She had no evidence of systemic disease. Head computed tomography (CT), with and without contrast, revealed no abnormalities. Magnetic resonance imaging (MRI) demonstrated multifocal, bilateral signal abnormalities within basal ganglia, thalami, right frontal cortex and subcortical white matter on T2 and FLAIR images. However, these areas did not show restricted diffusion on diffusion weighted imaging (DWI). Cerebral angiography revealed normal intracranial vessels (Figure 1). Neuroimaging, three weeks later, showed progression of these abnormalities. The areas of abnormal signal showed heterogeneous and nodular enhancement on gadolinium enhanced T1 weighted images (Figure 2). Rheumatologic, metabolic, prothrombotic and cerebral spinal fluid investigations were normal.

Type
Neuroimaging Highlights
Copyright
Copyright © The Canadian Journal of Neurological 2005

References

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