IVIG in childhood primary angiitis of the central nervous system: A case report
| Autor: | Satoko Kumada, Kenji Inoue, Takashi Komori, Hideaki Mashimo, Keisuke Takai, Harushi Mori, Mitsumasa Fukuda, Hiromi Suzuki, Hiroya Nishida, Michiharu Morino, Yasuhiro Nakata, Atsuko Arisaka |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Male
medicine.medical_specialty Biopsy 03 medical and health sciences 0302 clinical medicine Cerebrospinal fluid Developmental Neuroscience Prednisone medicine Humans Child Vasculitis Central Nervous System Pleocytosis medicine.diagnostic_test business.industry Brain biopsy Headache Brain Immunoglobulins Intravenous General Medicine medicine.disease Magnetic Resonance Imaging Hyperintensity Pediatrics Perinatology and Child Health Angiography Immunotherapy Neurology (clinical) Radiology Nervous System Diseases business Vasculitis Immunosuppressive Agents 030217 neurology & neurosurgery medicine.drug |
| Zdroj: | Brain and Development. 42:675-679 |
| ISSN: | 0387-7604 |
| DOI: | 10.1016/j.braindev.2020.06.007 |
| Popis: | Aggressive immunosuppressive therapies have been proposed to treat primary angiitis of the central nervous system (PACNS). Here, we report the first successfully stabilized case of childhood, small-vessel PACNS with intravenous immunoglobulin (IVIG) therapy. A 12-year-old boy was admitted to our hospital complaining of recurrent headaches and upper-left homonymous quadrantanopia, since the age of 11 years. Brain computed tomography scans revealed fine calcification in the right temporal and occipital lobes. Brain magnetic resonance imaging scans revealed white matter lesions, with gadolinium enhancement, which waxed, waned, and migrated for 1 year, without immunomodulatory therapies. A cerebrospinal fluid study showed pleocytosis (12 cells per µl). No clinical or serological findings suggested systemic inflammation or vasculitis. Brain angiography was unremarkable. Brain biopsy revealed thickened and hyalinized small vessels, with intramural infiltration of inflammatory cells, which confirmed the diagnosis of small-vessel PACNS. Because the patient developed surgical site infection following biopsy, the administration of monthly IVIG (2 g/kg) was prescribed, instead of immunosuppressive agents. After IVIG therapy, the patient remained stable, except for a single episode of mild radiological exacerbation at 16 months, which occurred when the IVIG interval was expanded. Oral prednisone was added and gradually tapered. At 50 months, his intellectual abilities and motor functions were normal, although he showed residual upper-left homonymous quadrantanopia and post-exercise headache. A temporary headache, associated with the immunoglobulin infusion, was resolved by slowing the infusion rate. PACNS should be treated aggressively to improve prognosis. However, when immunosuppressants are contraindicated, IVIG may be an alternative therapeutic option. |
| Databáze: | OpenAIRE |
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