[P1-133] Efficacy of Intravenous Immunoglobulin Therapy for Childhood Primary Angiitis of the Central Nervous System: A Case Report
[Background]
Primary angiitis of the central nervous system (PACNS) is a rare autoimmune vasculitis affecting vessels within the brain. It is classified into two types according to the size of the affected vessels: large- and medium-vessel PACNS and small-vessel PACNS. Children with small-vessel PACNS show poor outcomes, and a strong immunosuppressive therapeutic protocol with a combination of a prednisone and intravenous cyclophosphamide followed by mycophenolate mofetil is recommended. We herein report a child with PACNS who was successfully treated with intravenous immunoglobulin therapy (IVIg) for the first time.
[Case presentation]
A 12-year-old boy developed a seizure followed by recurrent headache and hemianopsia. Serum markers for inflammatory and autoimmune diseases were negative, but cerebrospinal fluid examination revealed a mildly elevated cell count and protein level. Brain magnetic resonance imaging (MRI) showed gadolinium-enhanced white matter lesions that continued to wax, wane, and migrate. Brain computed tomography revealed progressive white matter calcification. Brain angiography showed no remarkable findings. The diagnosis of small-vessel PACNS was confirmed by brain biopsy. IVIg was administered instead of strong immunosuppressive therapy because of a surgical site infection. His neurological status remained stable for 14 months. The MRI lesions diminished in size, and no new lesions were observed.
[Conclusions]
Diagnosis of small-vessel PACNS is challenging because the clinical, laboratory, and radiological features are nonspecific and brain biopsy is required for confirmation. Although rarely seen in children, pediatricians should be aware of this condition because early treatment can improve its outcome. IVIg may be an effective and safe treatment option.
Primary angiitis of the central nervous system (PACNS) is a rare autoimmune vasculitis affecting vessels within the brain. It is classified into two types according to the size of the affected vessels: large- and medium-vessel PACNS and small-vessel PACNS. Children with small-vessel PACNS show poor outcomes, and a strong immunosuppressive therapeutic protocol with a combination of a prednisone and intravenous cyclophosphamide followed by mycophenolate mofetil is recommended. We herein report a child with PACNS who was successfully treated with intravenous immunoglobulin therapy (IVIg) for the first time.
[Case presentation]
A 12-year-old boy developed a seizure followed by recurrent headache and hemianopsia. Serum markers for inflammatory and autoimmune diseases were negative, but cerebrospinal fluid examination revealed a mildly elevated cell count and protein level. Brain magnetic resonance imaging (MRI) showed gadolinium-enhanced white matter lesions that continued to wax, wane, and migrate. Brain computed tomography revealed progressive white matter calcification. Brain angiography showed no remarkable findings. The diagnosis of small-vessel PACNS was confirmed by brain biopsy. IVIg was administered instead of strong immunosuppressive therapy because of a surgical site infection. His neurological status remained stable for 14 months. The MRI lesions diminished in size, and no new lesions were observed.
[Conclusions]
Diagnosis of small-vessel PACNS is challenging because the clinical, laboratory, and radiological features are nonspecific and brain biopsy is required for confirmation. Although rarely seen in children, pediatricians should be aware of this condition because early treatment can improve its outcome. IVIg may be an effective and safe treatment option.