Unusual aggressive central nervous system involvement in CLIPPERS syndrome

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids known as CLIPPERS syndrome is a rare and recently described inflammatory disorder of the central nervous system. Ataxia and cognitive dysfunction are usual symptoms and are dramatically responsive to steroid treatment. We present a case of a 58-year-old female patient, clinically diagnosed CLIPPERS syndrome with unusual aggressive involvement of supratentorial white matter and spinal cord that showed complete recovery with significant atrophy after steroid therapy.