Neurology: Case of the Month

Test Yourself — Patient 36

Mononeuropathy Multiplex due to Mixed Cryoglobulinemia

1. Which of the following statements are CORRECT:
   • [ A ] Essential cryoglobulinemias are not associated with a lymphoproliferative disorder.
   • [ B ] Essential cryoglobulinemias may be associated with a systemic vasculitis involving small and medium sized vessels.
   • [ C ] A peripheral neuropathy can be the sole presenting sign of cryoglobulinemia in ~15% of patients.
   • [ D ] Peripheral neuropathy associated with essential cryoglobulinemias is typically diagnosed by finding evidence of a demyelinating process on sural nerve biopsy, with electrophysiologic evidence of normal to minimally decreased amplitudes and significant slowing of nerve conduction.
   • [ E ] A, B, and C
   • [ F ] A and C
   • [ G ] B and D
2. Which of the following statements are INCORRECT — Patients with hepatitis C infection and cryoglobulinemia frequently have:
   • [ A ] Only peripheral nerve and cutaneous involvement.
   • [ B ] Involvement of skin, elevations in serum alanine aminotransferase and rheumatoid factor, a decrease in CH50, and greater histologic liver lesions than patients without concomitant hepatitis C infection.
   • [ C ] A less severe peripheral neuropathy than patients with cryoglobulinemia not associated with hepatitis C infection.
   • [ D ] Direct and indirect signs of a more severe vasculitis than patients with cryoglobulinemia not associated with hepatitis C infection.
   • [ E ] A, B, and C
   • [ F ] A and C
   • [ G ] C and D
3. Nerve biopsy is indicated in the presence of data suggestive of an immunologic or infectious cause in a patient with an acute or subacute polyneuropathy.
   • [ A ] True
   • [ B ] False
4. Muscle and skin biopsy increases the yield for diagnosis when there is evidence for systemic vasculitic involvement.
   • [ A ] True
   • [ B ] False
5. Choose the INCORRECT statement — Interferon A is the only approved therapy for chronic hepatitis C infection and
   • [ A ] According to the majority of studies, interferon treatment typically results in significant improvement of peripheral neuropathy in patients with HCV infection and cryoglobulinemia.
   • [ B ] Some reports have documented improvement of prednisone-resistant mononeuropathy multiplex following treatment with interferon alpha alone, or with interferon alpha given as an adjunct to other immunosuppressive therapy.
   • [ C ] Other reports warn that interferon alpha may exacerbate extrahepatic manifestations of cryoglobulinemia, including peripheral neuropathy.
   • [ D ] The usual treatment for cryoglobulinemia, though not supported by randomized, controlled clinical trials, involves immunosuppressive therapies such as prednisone and plasma exchange.

 

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