Distinguishing CLIPPERS from Non-CLIPPERS Etiologies

Summary and Comment |
October 6, 2017

Distinguishing CLIPPERS from Non-CLIPPERS Etiologies

  1. Jaime Toro, MD

An in-depth assessment of potential cases leads to new diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement, a condition that responds to steroids.

  1. Jaime Toro, MD

In 2010, Pittock and colleagues described a condition that they termed chronic lymphocytic inflammation with perivascular enhancement responsive to steroids (CLIPPERS). Clinical symptoms are related to brainstem involvement and include gait ataxia and diplopia. Other symptoms observed are dysarthria, altered sensation and paresthesia of the face, dizziness, nystagmus, spastic paraparesis, and pseudobulbar affect. The radiologic distribution is particularly focused in the pons and adjacent rhomboencephalitis structures such as cerebellar peduncules, cerebellum, medulla, and midbrain.

The lack of formalized diagnostic criteria for this syndrome has led to reports in the literature that often don't correspond to the disease. In this study, the authors evaluated 35 patients who were evaluated for suspected CLIPPERS. They studied clinical, radiologic, and pathologic features and propose diagnostic criteria to discriminate CLIPPERS from non-CLIPPERS etiologies.

CLIPPERS was diagnosed in 23 patients (18 male, 5 female; median age at onset, 58; median follow-up, 44 months); 12 other patients had a non-CLIPPER diagnosis (median age at onset, 52; median follow-up, 27 months). CLIPPERS and non-CLIPPERS patients did not differ significantly in the proportions with clinical symptoms such as ataxia, diplopia, cognitive impairment, and facial paresthesia. Clinical and radiologic corticosteroid responsiveness was seen in CLIPPERS, while in non-CLIPPERS many but not all responded well to corticosteroids, and on discontinuing the medication not all worsened. On magnetic resonance imaging, CLIPPERS patients had perivascular gadolinium-enhancing lesions in the brainstem, whereas several non-CLIPPERS patients had heterogeneous gadolinium enhancement, heterogeneous T2 signal, or nonenhancing center. Neuropathologic findings showed marked CD3-positive T lymphocytes, mildly elevated CD20-positive B-lymphocytes, and moderate macrophage infiltrates with perivascular predominance.

Comment

CLIPPERS is a rather newly described pontine-centric inflammatory disorder with distinct clinical and radiologic features that respond well to corticosteroids. The new criteria will help clinicians do a more precise diagnosis and differentiate CLIPPERS from non-CLIPPERS patients. Further studies are required to better understand the pathogenesis of CLIPPERS.

Editor Disclosures at Time of Publication

  • Disclosures for Jaime Toro, MD at time of publication Editorial boards Multiple Sclerosis and Related Disorders

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