Neurology: Case of the Month

Test Yourself — Patient 77

Vasculitic neuropathy;
Crescentic glomerulonephritis, associated with anti-glomerular basement membrane antibodies and anti-neutrophil cytoplasmic antibodies

  1. Which of the following is the LEAST common clinical presentation for a vasculitic neuropathy?
    • [ A ] Mononeuritis multiplex
    • [ B ] Cranial mononeuropathy
    • [ C ] Symmetric length-dependent polyneuropathy
    • [ D ] Asymmetric polyneuropathy
  2. Which of the following studies is MOST useful to make the diagnosis of vasculitic neuropathy?
    • [ A ] Nerve conduction studies and electromyography documenting multiple mononeuropathies
    • [ B ] Nerve biopsy
    • [ C ] Cerebrospinal fluid analysis demonstrating elevated protein and increased immunoglobulins
    • [ D ] Serologic studies identifying high titer anti-neutrophil cytoplasmic antibodies (ANCA) and antibodies to proteinase-3
  3. Which of the following histological findings is the MOST specific for vasculitic neuropathy?
    • [ A ] Focal regions of nerve fiber loss
    • [ B ] Neovascularization
    • [ C ] Perivascular and transmural inflammatory cell infiltrate
    • [ D ] Thrombosis and recanalization of vessels
  4. Which of the following statements about the pathophysiology of vasculitic neuropathy is TRUE?
    • [ A ] The predominant cell infiltrates in histological samples are T cells and macrophages.
    • [ B ] Humoral mechanisms alone mediate the vessel injuries.
    • [ C ] Expression of cell surface molecules does not contribute to the vasculitic process.
    • [ D ] Schwann cells are the major sites of immunologic attack.
  5. Which of the following statements about vasculitic neuropathy is TRUE?
    • [ A ] Clinically isolated, biopsy-proven vasculitic neuropathy is invariably benign and requires no further evaluation.
    • [ B ] Identification of an antibody associated with vasculitis, such as anti-neutrophil cytoplasmic antibody (ANCA), renders nerve biopsy unnecessary for diagnosis of vasculitic neuropathy.
    • [ C ] Persistence of neuropathic pain is a valid marker of unsuccessful immune suppression in patients with vasculitic neuropathy.
    • [ D ] Vasculitic involvement of peripheral nerve precludes involvement of the central nervous system by the vasculitic process.
    • [ E ] None of the above statements are true.

 

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