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Axonal Multifocal Motor Neuropathy

without conduction block

 Read about axonal EMG findings in CIDP

Axonal multifocal motor neuropathy without conduction block or demyelination

J.S. Katz, MD;, R.J. Barohn, MD;,S. Kojan, MD;, G.I. Wolfe, MD;, S.P. Nations, MD;, D.S. Saperstein, MD; and A.A. Amato, MD
From the Departments of Neurology, Palo Alto VA Medical Center and Stanford University (Dr. Katz), Palo Alto, CA; University of Kansas Medical Center (Dr. Barohn), Kansas City; University of Texas Southwestern Medical Center (Drs. Kojan, Wolfe, Nations, and Saperstein), Dallas; and Brigham and Women's Hospital (Dr. Amato), Harvard Medical School, Boston, MA.

Address correspondence and reprint requests to Dr. Jonathan Katz, Department of Neurology (127), Department of Veterans Affairs, 3801 Miranda Ave., Palo Alto, CA 94304;
 Background: Conduction block is considered an essential finding for the distinction between motor neuropathies and lower motor neuron disorders. Only a small number of reports describe patients with multifocal motor neuropathies who lack overt conduction block, although in these cases other features of demyelination still suggest the presence of a demyelinating disorder. In contrast,a purely axonal multifocal motor neuropathy has not been described.

Methods: This report describes nine patients with slowly or nonprogressive multifocal motor neuropathies who had purely axonal electrodiagnostic features.

Results: GM1 antibodies titers were normal in all nine cases. Six patients were treated with either prednisone or IV immunoglobulin and three showed convincing improvement.

Conclusions: These findings suggest an immune-mediated motor neuropathy with axonal electrophysiologic features that appears to be distinct from both multifocal motor neuropathy and established motor neuron disorders.

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Stories links

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  • Salutatory conduction
  • Nerve physiology
  • Nerve fiber speeds
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    Neuropathies are a significant cause of morbidity worldwide, mainly from diabetes mellitus, HIV infection and leprosy. Many are treatable with immunosuppression or intravenous immunoglobulin. Tight glycaemic control slows progression of diabetic neuropathy. Even when the underlying disorder is untreatable, making a specific diagnosis and appropriate management to avoid complications and neuropathic pain can be rewarding.

  • AUTOIMMUNE EPIDEMIC

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    The simplest treatment for any neuropathy or CIDP is a gluten free diet, 30-40 % diseasefor reversal. See our celiac section of cidpusa website. We provide cures for ailing humanity.

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    celiac neuropathy