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1504 - A case of CLIPPERS Syndrome in acute inpatient rehabilitation

Friday, February 28, 2025

5:00 PM - 6:30 PM MST

Presenting Author(s)

AD

Alaba Danagogo, BSc

Medical Student
Renaissance School of Medicine, Stony Brook University
East-Setauket, New York, United States

Co-Author(s)

AN

Adam Nessim, MD

Resident
Montefiore
New York, New York, United States

Corresponding Author(s)

AW

Aye Mon Win, DO

Assistant Professor
Montefiore Medical Center/Albert Einstein College of Medicine
Bronx, New York, United States

Case Diagnosis: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroid (CLIPPERS) Syndrome is a rare CNS inflammatory disorder of the brainstem with T-cell predominant pathology that has a relapsing/remitting course and is responsive to immunosuppression by steroids. It has an unknown etiology with about 100 cases reported since 2010. Brain MRI findings classically show punctate and curvilinear perivascular gadolinium enhancement peppering the pons and parts of the rhombencephalon, including the medulla, midbrain and cerebellum.

Case Description: The patient is an 84 year old woman who initially presented with right facial weakness, dysarthria, gait ataxia and dysmetria. She has a history of HFpEF, CKD3, T2DM, HTN, HLD, left hip and bilateral knee OA. An MRI showed a right pontine and bilateral medullary stroke. At acute inpatient rehab two weeks later, she presented with worsening facial paralysis, new blurry vision, and dysmetria on finger to nose. Two repeat MRIs showed unclear evolving diffuse pontine, cerebellar and midbrain pathology overlying her stroke infarcts. Workups for rheumatology, cancer and infectious disease were negative. Her symptoms improved with solumedrol followed by dexamethasone 2 mg q12hr.

Discussions:
A diagnosis of CLIPPERS syndrome was proposed where the subtle peppering enhancement may be hidden by previous stroke. It often presents with a combination of cranial nerve and cerebellar symptoms (eg dysarthria, gait ataxia, nystagmus, etc), infarcted long tract/spinal cord symptoms (eg spasticity, paresis, altered sensory input, etc), and cognitive symptoms (eg cognitive deficits, dysexecutive syndrome, etc).

Conclusions:
It is a diagnosis of exclusion based on symptoms and imaging, with a fluctuating course that is lethal without steroids. Start pulse corticosteroid treatment promptly during a relapse to limit clinical worsening, followed by progressive tapering and eventual long-term low dose therapy. In patients who react adversely to corticosteroids, trial and error of immunosuppressant maintenance therapy can be attempted.