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CLIPPERS — SCE Neurology MCQ

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HardNeurogenetics and NeuroimmunologyCLIPPERSSCE Neurology

A subacute pontocerebellar syndrome causes gait ataxia, diplopia and multiple cranial neuropathies. MRI shows numerous punctate and curvilinear gadolinium-enhancing lesions peppering the pons; symptoms improve briskly with corticosteroids. What must accompany a diagnosis of CLIPPERS?

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Correct answer: E — A systematic search for infectious, with these neuroanatomical findings

The best answer is “A systematic search for infectious, with these neuroanatomical findings”. CLIPPERS is a clinicoradiological syndrome and diagnosis of exclusion; lymphoma, infection, vasculitis and other inflammatory disease can imitate it. “A positive aquaporin-4 antibody result” can be reasonable in another presentation, but it does not account for the defining feature here. “A diagnostic serum ACE concentration alone” can be reasonable in another presentation, but it does not account for the defining feature here. “Permanent steroid treatment without tissue or interval reassessment” can be reasonable in another presentation, but it does not account for the defining feature here. “A normal spinal MRI proving the diagnosis” can be reasonable in another presentation, but it does not account for the defining feature here.

Reference: CLIPPERS diagnostic criteria: https://pubmed.ncbi.nlm.nih.gov/29029857/