To report a case of chronic lymphocytic inflammation with perivascular pontine enhancement responsive to steroids (CLIPPERS) associated with T-cell lymphoma.

 CLIPPERS is an inflammatory, central nervous system disorder that primarily involves the brainstem. Neurologic manifestations include neuropathies, dysarthria, ataxia, weakness, and altered mental status. The diagnosis is based on clinical presentation, imaging, responsiveness to steroids, and histopathology. It can occur separately or in the presence of neoplasm. To our knowledge only two cases with T-cell lymphoma are reported.

Case report

61-year-old man with no past medical history presented with three days of progressive left-sided weakness, dysarthria, and somnolence. Neurologic examination revealed a left hemiparesis with UMN facial weakness and dysarthria. MRI revealed multiple enhancing lesions in the brain and the brainstem. CSF testing was negative for malignancy or infection. Chest CT showed multiple, borderline enlarged cervical lymph nodes. FDG-PET scan revealed a single hypermetabolic right submandibular lymph node. Cervical lymph node excisional biopsy was consistent with CD4 T-cell non-Hodgkin lymphoma. A brain biopsy revealed focal parenchymal and perivascular mixed inflammatory infiltrate with astrogliosis and vascular injury inconsistent with neoplasm. Multiple courses of steroids (IV and PO) were given with marked improvement in symptoms followed by relapses. CLIPPERS was felt to be most likely, and the patient was started on mycophenolate mofetil after a poor response to azathioprine. The patient has experienced significant clinical and radiographic improvement with resolution of enhancing lesions on mycophenolate mofetil and has successfully tapered off steroids for over six months.  

CLIPPERS remains a challenging diagnosis that can occur both in the presence and absence of malignancy. CLIPPERS associated with T-cell lymphoma has been described twice and should be considered in the differential for multifocal enhancing infratentorial brain lesions, especially since it is treatable. Mycophenolate should be considered if relapsing and refractory to other steroid-sparing agents.