Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)

Diagnosis:

Clinical features, MRI and occasionally biopsy

Clinical features:

Episodic diplopia or facial paresthesias with subsequent brainstem and occasionally myelopathic symptoms and had a favourable initial response to high dose glucocorticosteroids

Findings on Investigations:

+MRI:

  • Symmetric curvilinear gadolinium enhancement peppering the pons and extending variably into the medulla, brachium pontis, cerebellum, midbrain and occasionally spinal cord

Pathology:

white matter perivascular, predominantly T lymphocytic, infiltrate without granulomas

Treatment:

Glucocorticosteroids, if unable to taper add on other immunosuppressants