CLIPPERS is a rare chronic inflammatory disorder of unknown cause that primarily affects the brainstem.

The name is a mnemonic for the key findings:

(1) chronic lymphocytic inflammation (CLI), predominantly angiocentric

(2) pontine perivascular enhancement (PPE) on MRI

(3) responsive to steroids (RS) and immunosuppression


Additional findings:

(1) Extrapontine involvement is common.

(2) The serum IgE is elevated.

(3) The protein concentration in the CSF is elevated.


Clinical findings:

(1) gait ataxia

(2) intermittent diplopia and/or nystagmus

(3) perioral and facial parethesias

(4) dysarthria

(5) variable cognitive deficits

(6) variable spastic paraparesis


The diagnosis requires exclusion of other causes (infectious, autoimmune, toxic, paraneoplastic, malignancy).


Differential diagnosis:

(1) malignant lymphoma

(2) Bickerstaff brainstem encephalitis

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