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

Case contributed by Ali Hekmatnia
Diagnosis almost certain


Progressive altered facial sensation and limb paresis

Patient Data

Age: 35 years
Gender: Female

Brain MRI with GAD showing multiple foci of punctate brainstem and in particular pontine enhancement (peppering) suggestive of perivascular distribution with extension to centrum semiovale and internal capsule and basal gangelia and cerebellar peduncles.

Similar foci of gadolinium enhancement seen in cervical and upper thorasic cord.

Case Discussion

Punctate and curvilinear gadolinium enhancement of the pons is characteristic feature of CLIPPERS. A specific CSF or serum biomarker for this disorder is not known. CSF studies of this patient were negative, markers of vasculitis, and tumor markers and paraneoplastic antibodies were unrevealing.

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