A case report of CLIPPERS involving brainstem, cerebellum, white matter of cerebral hemisphere, spinal cord and corpus callosum

CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a rare inflammatory disorder of the central nervous system. We describe a case with an atypical disease course and unusual imaging features, broadening the radiological spectrum of this condition. An adult male presented with acute gait instability. Neurological examination revealed upper motor neuron signs, including hyperreflexia and patellar/ankle clonus. Brain and spinal MRI demonstrated characteristic punctate, gadolinium-enhancing lesions in the pons, cerebellum, and spinal cord. Symptoms improved markedly with high-dose corticosteroid therapy. However, over a 5-year follow-up, the patient experienced several clinical relapses, each associated with steroid taper or discontinuation. Subsequent MRI during relapses revealed new, atypical lesions within the corpus callosum in addition to the classic findings. Long-term disease control was ultimately achieved with mycophenolate mofetil, an immunosuppressive agent, which maintained clinical and radiological remission. This case highlights that probable CLIPPERS can involve atypical structures such as the corpus callosum, which should not preclude the diagnosis. The relapsing-remitting course underscores the importance of long-term monitoring and the critical role of immunosuppressive agents in sustaining remission. Early initiation of steroid-sparing immunosuppressants should be considered for patients with a relapsing disease course.