Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an increasingly recognized entity affecting the brainstem, particularly the pons. An entity with similar pathophysiology in the supratentorial region has also been described- supratentorial lymphocytic inflammation with parenchymal perivascular enhancement responsive to steroids (SLIPPERS). We describe a rare case of CLIPPERS/SLIPPERS combination.

A 62-year-old man presented to the neurology movement disorder clinic with symptoms of tremors in hands, gait abnormality, and cognitive dysfunction for two years. His family members had noticed increased irritability and short-term memory impairment like difficulty in recalling details of conversation. He denied sensory deficit and vision changes. Neurological examination was significant for ataxia in finger-nose-finger testing bilaterally, intention tremor, hyperreflexia, and impaired Luria test.

Contrasted MRI of the brain showed diffuse punctate lesions involving the bilateral periventricular and subcortical white matter, deep gray nuclei, brainstem and cerebellum with accompanying patchy and linear enhancement. Workups for other etiologies including malignancy, demyelinating, rheumatological, paraneoplastic, and infectious conditions were negative. The patient started high-dose oral prednisone therapy with a slow taper over 6 months and continued prednisone 10mg daily. Repeat MRI brain showed improvement in the supratentorial and infratentorial lesions. The diagnosis of CLIPPERS/SLIPPERS combination was made based on both clinical and radiographic improvements. The patient was planned for start of immunosuppression with mycophenolate mofetil, as he developed intolerance to high-dose steroid regimen, and continued to have slight radiographic recrudescence when tapered off steroid regimen.

This case highlights the rare findings of CLIPPERS/SLIPPERS combination as well as atypical neurologic presentation initially concerning for movement disorder pathology. The lack of unified diagnostic criteria and prolonged treatment courses with steroids before improvement in symptoms are a significant hurdle in diagnosis of these conditions. However, clinical success can still be achieved with late intervention by steroids and immunosuppression.