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CIDP — SCE Neurology MCQ

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HardNeuromuscularCIDPSCE Neurology

A 62-year-old has multiple cranial neuropathies, headache and a new superior sagittal sinus thrombosis. MRI shows diffuse hypertrophic pachymeningitis. CSF is mildly inflammatory; serum IgG4 is normal and systemic imaging is unrevealing. Which investigation is now most likely to establish a treatable diagnosis?

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Correct answer: B — Dural biopsy with IgG4 immunostaining

Explanation lettering: B = shown as A · A = shown as B

A is correct: IgG4-related hypertrophic pachymeningitis can be isolated to the nervous system and serum IgG4 may be normal. When accessible and safe, dural biopsy can demonstrate lymphoplasmacytic inflammation, storiform fibrosis and increased IgG4-positive plasma cells while excluding infection and neoplasia. Repeating serum alone cannot rule in or out isolated disease. CSF ACE is neither sensitive nor specific for neurosarcoidosis. PET can identify another biopsy target but is not a histological diagnosis.

Reference: IgG4-related hypertrophic pachymeningitis presenting with cerebral venous sinus thrombosis: https://pn.bmj.com/content/26/3/288