Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): case presentation

Case presentation: 52-year-old female patient, previously healthy, presented to the ataxia outpatient clinic with a history of progressive imbalance for 8 years. She developed paresthesia in her extremities in the past 4 months, and dysphagia for liquids and solids in the last 2 months. The physical examination revealed dysarthria, Parinaud’s syndrome, Collier’s sign, global cerebellar ataxia, hyperreflexia in the lower limbs and bradykinesia at alternating movements. Magnetic Resonance Imaging (MRI) revealed a punctate and curvilinear T2/FLAIR hyperintensity on the central portion of the pons and extending to the midbrain with gadolinium enhancement. Cerebrospinal fluid analysis, electroneuromyography and electroencephalography showed no abnormalities. Due to the clinical and laboratory findings, the diagnosis of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) was suggested. The patient was treated with methylprednisolone 1g/day for 5 days, progressing with moderate improvement of the imbalance, dysarthria and dysphagia. Discussion: CLIPPERS is a rare chronic inflammatory disorder of the central nervous system (CNS). The disease can occur in all age groups, and both men and women can be affected. Its etiology and pathogenesis remain unknown and most patients have no specific triggering factors. Diagnosis is based on clinical, radiological and pathological criteria. Clinical findings include subacute pontocerebellar dysfunction such as ataxia, diplopia and facial paresthesia, as well as clinical responsiveness to corticosteroid therapy. MRI of the pons shows punctate images with a “salt and pepper” appearance with contrast enhancement. Biopsy, when performed, shows infiltration of the perivascular spaces by T cells. The cornerstone of the treatment is a corticosteroids pulsotherapy, which typically leads to significant improvement in symptoms. No study has shown that CLIPPERS can be cured, so, long-term steroid maintenance therapy is necessary to prevent recurrence of the disease. Final comments: Clinical, radiological and pathological findings of CLIPPERS are non-specific and occur in several corticosteroid-sensitive neurological diseases and there is still a lack of specific biomarkers for its diagnosis. Therefore, diagnosis of CLIPPERS is challenging and is a disease yet to be explored due to its rarity. This diagnosis should be kept in mid in patients with subacute or chronic history of cerebellar ataxia.