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Multifocal motor neuropathy (MMN) and motor syndromes with
serum anti-GM1 antibodies.
A. Clinical Syndromes
The
immune-mediated motor neuropathies are characterized by
asymmetric, slowly progressive weakness that most commonly
begins in the hands & arms. The age of onset is generally
between 20 and 75. Men are affected somewhat more commonly
than women. Motor findings include asymmetric weakness and
variable degrees of atrophy. Patients with prominent
conduction block may present with weakness in muscles with
relatively normal bulk. Rarely, patients have had cranial
nerve signs including external ophthalmoplegia and
unilateral tongue weakness and atrophy. Some patients report
paresthesias, but sensory signs are usually absent or
clinically insignificant. In regions with normal strength
tendon reflexes are often preserved. In areas of weakness,
reflexes may initially be normal but can become reduced with
progression of the disease. Fasciculations are not uncommon,
and may add to diagnostic confusion between MMN and variants
of amyotrophic lateral sclerosis (ALS) with only lower motor
neuron signs. However, hyperreflexia and spasticity typical
of ALS never occur in MMN
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