Childhood Central Nervous System Vasculitis
To learn about the presentation, pathogenesis, evaluation, differential diagnosis, and management of childhood central nervous system vasculitis
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Marinka Twilt and Susanne M. Benseler
Learning Objectives
1. To learn about the presentation, pathogenesis, evaluation, differential diagnosis, and management of childhood central nervous system vasculitis
Case
A 3-year-old previously healthy child presented to the emergency room with a 4-day history of bilateral leg pain with unwillingness to walk, headache, and enuresis/encopresis after having been toilet trained for the past 18 months. The
M. Twilt, MD, MSCE, PhD (*) Division of Rheumatology, Department of Pediatrics, Aarhus University Hospital, Palle Juul-Jensens Boulevard 99, Aarhus 8200, Denmark Rheumatology, Alberta Children’s Hospital for Sick Children, Faculty of Medicine, University of Calgary, 2888 Shaganappi Trail NW, Calgary, AB T3B 6A8, Canada e-mail: [email protected] S.M. Benseler, MD, MSCE, PhD Rheumatology, Alberta Children’s Hospital for Sick Children, Faculty of Medicine, University of Calgary, 2888 Shaganappi Trail NW, Calgary, AB T3B 6A8, Canada e-mail: [email protected]
parents reported that her behavior had changed dramatically; she was crying a lot and could not sleep. She had a mild cold 2 months ago, no systemic symptoms since, no fever, no rashes, and no other infectious symptoms. Her physical exam revealed normal vital signs; she has a normal general physical examination except for generalized pain in her arms and legs, and the musculoskeletal assessment demonstrated no evidence of arthritis, enthesitis, or tenosynovitis. The neurological examination revealed significant hyperreflexia with clonus of both ankles, decreased visual acuity, and a decreased tone of her anal sphincter muscle. While in the emergency room, she started seizing and was transferred to the intensive care unit, where she remained in a seizure status. Her inflammatory markers were raised, both CRP and ESR; the CSF reveals raised white blood count of 28; the protein was raised. She had evidence of oligoclonal banding. The extensive infectious workup was negative. The MRI showed bilateral asymmetrical T2/FLAIR lesions affecting both gray and white matter. She had bilateral optic nerve lesions, spinal cord lesions, and focal meningeal enhancement.
© Springer Science+Business Media Singapore 2017 S. Sawhney, A. Aggarwal (eds.), Pediatric Rheumatology, DOI 10.1007/978-981-10-1750-6_40
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Her lesional brain biopsy revealed a lymphocytic small vessel CNS vasculitis. The child was treated with corticosteroids and cyclophosphamide for 6 months and then switched to mycophenolate mofetil according to the small vessel CNS vasculitis BrainWorks protocol (http://www. sickkids.ca/pdfs/Research/BrainWorks/62046cPCNS%20Vasculitis.pdf) and made a complete recovery. She has been subsequently observed for 4 years and is developmentally entirely normal.
Introduction Central nervous system (CNS) vasculitis and other inflammatory brain diseases in childhood are reversible causes of childhood brain injury including strokes, seizures, and cognitive decline. New-onset neurological or psychiatric deficits in pre
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