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Multi Focal Motor neuropathy

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MOTOR NEUROPATHIES AND LOWER MOTOR NEURON SYNDROMES

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What is Multifocal Motor Neuropathy?
Multifocal motor neuropathy is a  weakness in the hands, with differences from one side of the body to the other in the specific muscles involved. It affects men much more than women. Symptoms also include muscle wasting, cramping, and involuntary contractions or twitching of the leg muscles. The disorder is sometimes mistaken for amyotrophic lateral sclerosis (ALS, or Lou Gehrig's disease) but unlike ALS, it is treatable. An early and accurate diagnosis allows patients to recover quickly.

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Is there any treatment?

Treatment consists of intravenous immunoglobulin (IVIg) Historical Aspects of Lower Motor Neuron Syndromes The original descriptions of pure motor syndromes without upper motor neuron signs were probably cases of "progressive muscular atrophy" in the writings of Duchenne, Aran and others during the 19th century . Benign, focal motor neuron disorders, such as monomelic amyotrophy, were subsequently reported . These syndromes were usually considered as variants of ALS, as early pathological studies suggested that the primary focus of the disease was on cell bodies in the ventral horn.
A pathological report by Rowland et al. first documented that a patient with a pure motor syndrome could have the primary site of disease along the course of the axon. This patient, with a lower motor neuron (LMN) syndrome and a serum IgM M-protein, had damage to motor axons but not cell bodies. Motor neuropathies were first diagnosed during life by electrodiagnostic testing. Nerve conduction studies showed blockade of impulses at focal sites along the course of motor axons (motor conduction block) . The phenomenon of conduction block had been described earlier in patients with sensory-motor neuropathies (chronic inflammatory demyelinating polyneuropathy (CIDP)) . Conduction block  results from focal regions of immune-mediated demyelination along the course of the nerve.
In 1986 a patient was reported with a LMN syndrome without conduction block, but with a serum IgM M-protein that bound to GM1 ganglioside. In this instance the association of the motor syndrome with an autoantibody directed against a neural antigen suggested that the disorder might be immune-mediated. However, attempts at immunosuppression had no effect on the progressive disease in that patient. A clinical response to immunotherapy remains a "gold standard", without which it is difficult to argue that a syndrome is immune mediated. In 1988 two patients with a multifocal motor neuropathy, motor conduction block, and serum IgM anti-GM1 antibodies were reported to improve after treatment with cyclophosphamide . It now appears that either motor conduction block or serum anti-GM1 antibodies alone can be markers for patients with LMN syndromes that often improve after immunomodulating therapy.

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