Akademik Veri Yönetim Sistemi
Journal of Neurological Sciences, cilt.33, sa.1, ss.166-170, 2016 (SCI-Expanded)
© 2016, Ege University Press. All right reserved.Introduction: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) has recently been reported as an inflammatory disease of brainstem and cerebellum and responsive to corticosteroids. Case report: We report a 17-year-old man who was admitted to hospital because of right hemiparesis with gait ataxia for seven months. Muscle strength was 4/5 in right upper and lower extremity. He had right-sided dysmetria, dysdiadochokinesia and bilateral Babinski signs. Cranial MRI showed heterogeneous lesions with contrast enhancement in bilateral cerebellar hemispheres, pons, centrum semiovale and left cerebral deep white matter. The ratio of N-acetyl aspartate to creatine (NAA/Cr) was significantly decreased in magnetic resonance spectroscopy (MRS). VEP, cerebrospinal fluid examination, vasculitis markers, vitamin B12, folic acid, infections tests and ACE were normal. All the other possible causes were eliminated by laboratory and radiologic investigations as declared above. Cerebellar biopsy showed perivascular lymphohistiocytic infiltrate which contains CD3+, CD20+ and CD68+ B lymphocytes, T lymphocytes and histiocytes. We thought that the diagnosis is CLIPPERS. Some of his clinical signs responded to prednisone such as gait ataxia, right cerebellar tests, but right hemiparesis did not improve. The lesions in cranial MRI were regressed after prednisone treatment. He was put on azothioprine six months later. There were no new symptoms for two years. Discussion: CLIPPERS is newly described neuroinflammatory disease. We should think CLIPPERS if patient had signs of brainstem and cerebellum involvement and responsive to corticosteroids with nodular and punctate contrast enhancement of brachium pontis, pons and cerebellum on MRI.