A case report of CLIPPERS syndrome with gait and cognitive impairment

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) syndrome is a rare, chronic inflammatory central nervous system (CNS) disorder. It primarily involves the brainstem and cerebellum, presenting with subacute neurological symptoms.

A 44-year-old male presented with progressive gait imbalance, cognitive dysfunction, and behavioral changes over six weeks. Neurological examination revealed a wide-based unsteady gait, cerebellar dysmetria, and mild cognitive deficits. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis and elevated protein, while infectious and neoplastic causes were ruled out. Brain MRI demonstrated nodular and patchy lesions involving the cerebellum, brainstem, and thalamus, with characteristic pepper-like post-contrast enhancement consistent with CLIPPERS syndrome.

High-dose intravenous methylprednisolone (1 g/day for 5 days) led to significant clinical improvement, including resolution of gait disturbance and cognitive deficits. Follow-up MRI showed a marked reduction in lesion size. Maintenance therapy with oral methotrexate was initiated to sustain remission.

CLIPPERS syndrome should be considered in patients presenting with subacute pontocerebellar dysfunction and characteristic MRI findings. Early diagnosis and prompt corticosteroid therapy can lead to favorable outcomes. Long-term immunosuppressive treatment is essential to prevent relapse. Careful follow-up is required to monitor for recurrence and potential malignant transformation.