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Neurology — UKMLA MCQ

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HardNeurologyUKMLAMRCEM SBAMRCP Part 1

A patient develops a subacute severe demyelinating polyradiculoneuropathy with disabling neuropathic pain, sensory ataxia, facial weakness and poor response to IVIG. Which antibody is most closely associated with this pain-predominant autoimmune nodopathy?

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Correct answer: D — IgG antibody to contactin-associated protein 1

The best answer is “IgG antibody to contactin-associated protein 1”. Antibodies to contactin-associated protein 1, or Caspr1, define an autoimmune nodopathy that may resemble aggressive CIDP or GBS. Severe neuropathic pain is a particularly useful discriminator, with sensory ataxia, cranial involvement and poor IVIG response also reported. NF155 disease more characteristically causes distal weakness, tremor and ataxia without the same pain association. Anti-MAG causes a chronic distal sensory phenotype, GM1 is linked to motor neuropathy, and MOG antibodies cause central demyelination rather than a paranodal peripheral neuropathy.

Reference: Antibody-mediated nodo- and paranodopathies review: https://pmc.ncbi.nlm.nih.gov/articles/PMC11477122/