Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with seizures and central pyrexia, in a patient requiring tracheal intubation and mechanical ventilation: A case report

Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) has been described in multiple case reports since it was first described in the literature in 2010. The patient described is a 54-year-old man with no significant medical history who presented with ataxia, diplopia and hearing and taste disturbances. His GCS reduced to the point at which he required intubation and he was treated for 50 days on an intensive care unit. The patient's course also includes seizures, first described in 2014, and central pyrexia, which has not been previously described in case reports of CLIPPERS. Intravenous methylprednisolone resulted in a rapid and significant recovery; neurological rehabilitation is ongoing 10 months from diagnosis. The importance of recognition of unusual radiological findings and rapid treatment cannot be overemphasised in improving patient's long-term outcomes.

Hemidysgeusia, phantosmia and respiratory arrest: a case of CLIPPERS

“…The patient’s phantosmia may have been due to temporal lobe epilepsy. Seizures have previously been described in CLIPPERS due to cortical involvement 4. This suggests that even though the pontine is the most commonly affected area of the cerebrum, cortical involvement can present atypically.…”

“…CLIPPERS commonly presents with symptoms related to brainstem involvement such as ataxia, dysarthria, oculomotor abnormalities, tingling of the face and vertigo 2. Rarely, CLIPPERS may also present with cortical lesions and seizures as the initial presentation 3 4…”