• Annals of Clinical Neurophysiology
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• v.4 no.2
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• pp.98-107
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• 2002

Multifocal motor neuropathy (MMN) is a chronic immune-mediated peripheral myelinopathy. The major clinical features include slowly progressive, painless, and asymmetric weakness, usually of distal limb muscle. Early in the course of the disease, weakness is not necessarily associated with muscle atrophy, owing to the initial primary involvement of peripheral myelin. Chronic progressive weakness is often associated with some degree of concurrent axonal loss and subsequent muscle atrophy. Sensory symptoms are usually mild or absent, and involvement of cranial and respiratory muscles is rare. The findings of multifocal motor conduction block, abnormal temporal dispersion, and focal conduction slowing at segments not at risk for common entrapment or compression injury, associated with normal sensory conduction studies along the same segments, are the hallmark electrophysiologic features of MMN. The slow progression and absence of upper motor neuron signs are the major clinical points that separate MMN from amyotrophic lateral sclerosis. The role of GM1 antibodies, found in high titers in 22~84% of MMN patients, remains uncertain. The contention that MMN is an autoimmune disorder is largely based on the often dramatic improvement in symptoms following the administration of intravenuos immunoglobulin or cyclophosphamide.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.6-15
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• 2006

Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has been used in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.

• Annals of Clinical Neurophysiology
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• v.20 no.2
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• pp.101-104
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• 2018

Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is a variant of chronic acquired demyelinating polyneuropathy. A 65-year-old women presented with upper arm weakness. A nerve conduction study showed conduction blocks over intermediate segments with sparing of distal compound action potentials. Magnetic resonance imaging revealed asymmetric hypertrophy of the brachial plexus on the affected side. These findings represent important electrophysiological and radiological evidence of MADSAM neuropathy. The condition of the patient began to improve after starting intravenous immunoglobulin administration.

• Annals of Clinical Neurophysiology
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• v.7 no.2
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• pp.127-129
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• 2005

We report a 29-year old female who developed bilateral femoral neuropathy combined with multiple abscesses in both thigh muscles. She was present with weakness in both lower extremities for 15 days and intermittent chilling sense for 3 months. Nerve conduction study showed complete absence of compound muscle action potentials in bilateral femoral nerves when stimulated at inguinal area. Electromyographic examination revealed no motor unit action potentials in both rectus femoris and vastus medialis muscles. CT revealed multifocal abscesses in bilateral thigh muscles. After antibiotic treatment, the patient's neurologic symptoms were improved.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.48-52
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• 2006

Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.102-105
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• 2006

We present a case with stepwise weakness and sensory involvement of both hands for more than 2 months. His nerve conduction study findings revealed prolonged terminal latencies, decreased motor and sensory conduction velocities and conduction blocks of both ulnar nerves, more severely on left side. And there were other abnormalities manifested with mononeuropathy multiplex. Increased cerebrospinal fluid protein was found. We diagnosed him as Lewis-Sumner syndrome and tried high dose oral steroid therapy for 2 months. He showed improvement of motor functioning with persistent conduction block.