HE, Hematoxylin and Eosin Stain. He presented to us with a history of recurrent ataxia, bilateral persistent esotropia, and diplopia with no other neurological and systemic symptoms. (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a recently described, rare inflammatory disorder of the central nervous system. This condition was first described by Pittock et al1 in 2010 2010 as clinically and radiologically distinct pontine-predominant encephalomyelitis. It is diagnosed by clinical symptoms involving the brain stem with a distinct pattern of magnetic resonance imaging (MRI) changes characterized by punctate and curvilinear gadolinium enhancement peppering the brain stem (mainly Tonapofylline the pons) and a perivascular T-lymphocyte infiltrate on brain biopsy.1,2 It is reported to be a steroid-dependent condition with a relapsing, remitting course requiring glucocorticoid therapy along with long-term Tonapofylline immunosuppression.1,3 Here, we report a 13-year-old boy with CLIPPERS to raise awareness about this rare condition, enabling early diagnosis and treatment. To our knowledge, this would be the first pediatric case from India. Case Report A 13-year-old male child was referred to us for complaints of recurring episodes of ataxia and diplopia for last 6 years. He was born of nonconsanguineous marriage with normal birth history and normal development and was asymptomatic till 6.5 years of age. He presented in a private hospital, with complaints of intermittent fever associated with vomiting and giddiness for a period of 1 1 month. This was followed by medial deviation of both eyes. On examination, he had bilateral sixth cranial nerve palsy with papilledema and unequal pupils; rest of his central nervous system examination was normal. Lumbar puncture showed 30 cells with 90% lymphocytes and normal protein, sugar, and lactate. Magnetic resonance imaging of the brain (Figure 1A) suggested altered signal intensities, hyperintense on T2 in subcortical and deep white matter over parieto-occipital and bilateral cerebellar regions. A probable diagnosis of acute disseminated Tonapofylline encephalomyelitis was made, and he was treated with intravenous methylprednisolone followed by tapering doses of oral steroids over 1 month. Improvement was noted within a month, total recovery without sequelae, and the child was asymptomatic for next 6 years. Open in a separate window Figure 1. A, Magnetic resonance imaging (MRI) Rabbit Polyclonal to XRCC4 of the brain suggested altered signal intensities, hyperintense on T2 in subcortical and deep white matter over parieto-occipital and bilateral cerebellar region. B, The MRI of the brain suggested white matter signal changes in bilateral cerebellar hemispheres, cerebellar peduncles, pons, and midbrain. Similar changes in left thalamus, adjacent posterior limb of internal capsule, parahippocampal gyrus, and parietal regions Tonapofylline on both sides. Small changes in the left frontoparietal Tonapofylline subcortical white matter. C, Significant decrease in hyperintensities as compared to (C), but not totally cleared. D, Reappearance of lesions and patchy involvement of pons, midbrain, cerebellar peduncles, and cerebellum. At 12 years, he returned with complaints of medial deviation of left eye and diplopia. There was no history of fever, vomiting, headache, seizures, or altered sensorium. Magnetic resonance imaging of the brain (Figure 1B) suggested white matter signal changes in bilateral cerebellar hemispheres, cerebellar peduncles, pons, and midbrain. Similar changes were seen in left thalamus, adjacent posterior limb of internal capsule, parahippocampal gyrus, and parietal regions on both sides with few changes in the left frontoparietal subcortical white matter. Complete blood count and serum biochemistry were normal. Cerebrospinal fluid routine, aquaporin 4, and anti-N-methyl-D-aspartate receptor (NMDAR) antibody were negative. Immunoglobulin E level-1140 (N 200) was raised (Table 1A). Diagnosis of multiphasic demyelinating encephalomyelitis or probable evolving multiple sclerosis was considered. He was again treated with.