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Primary angiitis of CNS — SCE Rheumatology MCQ

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HardVasculitisPrimary angiitis of CNSSCE Rheumatology

A 41-year-old man has subacute headaches, cognitive change and multifocal neurological deficits over 6 weeks. MRI brain shows multiple infarcts of different ages. ESR is normal, ANCA is negative and echocardiography is unremarkable. CSF shows lymphocytic pleocytosis and high protein. What is the most appropriate investigation?

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Correct answer: D — Catheter cerebral angiography with consideration of brain biopsy

Explanation lettering: B = shown as A · A = shown as B · D = shown as C · E = shown as D · C = shown as E

Catheter cerebral angiography with consideration of brain biopsy is best because suspected primary CNS vasculitis requires specialist vascular imaging and sometimes biopsy because blood tests may be normal. A is less suitable because dual-energy CT evaluates urate deposition; B is less suitable because temporal ultrasound assesses cranial GCA rather than intraparenchymal CNS vasculitis; C is less suitable because DEXA assesses fracture risk; D is less suitable because Schirmer test evaluates ocular dryness. Clinical pearl: CNS vasculitis is a diagnosis requiring exclusion of infection, embolic disease and mimics.

Reference: Oxford Textbook of Rheumatology; EULAR vasculitis recommendations