Neurology: Case of the Month

Test Yourself — Patient 5

Primary Angiitis of CNS (PACNS)

  1. Select the true statement with respect to histological findings in PACNS:
    • [ A ] The presence of granulomatous lesions in the CNS vessels excludes the diagnosis.
    • [ B ] The histological appearance is unique and readily distinguishable from findings seen in other types of vasculitis.
    • [ C ] Lesions show contiguous vasculitis and skip lesions are rare.
    • [ D ] The histological appearance lacks total diagnostic specificity.
  2. All of the following are true regarding the clinical presentation of PACNS EXCEPT:
    • [ A ] If untreated, most patients will go into coma and die within a week or two.
    • [ B ] Non-focal symptoms (i.e. headaches, confusion, and decreased cognitive function) are common.
    • [ C ] Fever, weight loss, arthralgia, and myalgia are uncommon.
    • [ D ] A wide variety of focal neurologic deficits may occur.
  3. Select the true statement about diagnostic tests for PACNS:
    • [ A ] The two most common CSF findings are the presence of oligoclonal bands and increase in the IgG synthesis rate.
    • [ B ] MRI of the brain may show evidence of bilateral ischemia of the cortex and deep white matter.
    • [ C ] A normal MRI excludes the diagnosis of PACNS.
    • [ D ] Cerebral angiography is diagnostic in greater than 80% of patients.
  4. All of the following statements are true regarding PACNS EXCEPT:
    • [ A ] Less than 5% of biopsy procedures result in false-negative outcomes.
    • [ B ] Both leptomeningeal and cortical biopsies are usually obtained.
    • [ C ] Histologic confirmation is the gold standard to confirm the diagnosis.
    • [ D ] Biopsy of the non-dominant temporal lobe tip is usually recommended in patients without focal lesions.
  5. The following criteria have been suggested to establish the antemortem diagnosis of PACNS:
    • [ A ] Headaches and multifocal neurologic deficits present for at least six months (unless the onset is devastating), and exclusion of systemic inflammation or infection.
    • [ B ] Cerebral angiography demonstrating several areas of segmental arterial narrowing.
    • [ C ] Leptomeningeal/parenchymal biopsy to demonstrate vascular inflammation and exclude infection, atherosclerosis, and neoplasia.
    • [ D ] All of the above.

 

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