ICNC Abstracts, ICNC 2018

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A NEW NEUROINFLAMMATORY DISORDER IN CHILDREN: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)
tarishi nemani, PURVA KENI KARNAVAT, EPARI SRIDHAR, ANAITA UDWADIA HEGDE

Last modified: 2018-09-09

Abstract


Introduction: CLIPPERS is a recently described, rare neuro-inflammatory disorder. It is diagnosed by clinical symptoms involving the brainstem with a distinct pattern on neuro-imaging and a perivascular T-lymphocyte infiltrate on brain biopsy. It is a condition usually described in adults in the 4th-5th decade. We report the youngest case of an Indian boy presenting at 7 years of age.

Case report: A 13-year old boy, first presented with bilateral esotropia at 7 years of age. MRI brain showed white matter signal changes in bilateral cerebellar hemispheres, cerebellar peduncles, pons and midbrain. He was treated as a case of acute disseminated encephalomyelitis with methylprednisolone, following which he completely recovered and was asymptomatic. Six years later he presented with 3 episodes of diplopia, esotropia and ataxia at an interval of 6-8 months. Subsequent MRI brain showed gadolinium enhancing punctate lesions peppering pons and cerebellum. Further investigations excluded infective, neoplastic, autoimmune and demyelinating conditions. In addition, histopathology and neuro-immunological testing showed polyclonal T-cell infiltration suggesting the diagnosis of CLIPPERS. Clinico-radiological improvement following immunosuppressive treatment with steroids and methotrexate also supported the diagnosis.

Conclusion:

  • CLIPPERS, recognized as a rare neuroinflammatory disorder of adults, this case highlights its paediatric onset.
  • Characteristically diagnosed by neuroimaging and immune-histopathology.
  • Treatment with steroids and particularly Methotrexate provides a promising outcome.

Keywords


PEDIATRIC;CLIPPERS; NEUROINFLAMMATORY

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