J Korean Med Sci.  2010 Jul;25(7):1101-1104. 10.3346/jkms.2010.25.7.1101.

A Case of Lewis-Sumner Syndrome Showing Dramatic Improvement after Plasma Exchange

• Park YE
• Yook JW
• Kim DS

• KMID: 1792967
• DOI: http://doi.org/10.3346/jkms.2010.25.7.1101

Abstract

We report a patient with Lewis-Sumner syndrome (LSS) who showed an improvement only with plasma exchange (PE). The patient, 32-yr old man, had progressive multifocal motor-sensory deficits with persistent, multiple conduction blocks and marked slowing of NCVs. Nerve pathology supported a diagnosis of demyelinating neuropathy by revealing marked loss of myelinated fibers with inter- and intrafascicular variation. Although the patient was refractory to treatment with corticosteroid and intravenous immunoglobulin, PE produced a dramatic improvement. Our experience strongly proposes that PE should be tried for refractory LSS.

Keyword

Lewis-Sumner Syndrome; Plasma Exchange; Conduction Block; Nerve Pathology; Cranial Nerve Diseases

MeSH Terms

Adrenal Cortex Hormones/therapeutic use
Adult
Demyelinating Diseases/*diagnosis/pathology/physiopathology/*therapy
Humans
Immunoglobulins, Intravenous/therapeutic use
Male
Neural Conduction/physiology
Peripheral Nerves/pathology/physiopathology
*Plasma Exchange
Syndrome
Treatment Outcome

Figure

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  Fig. 1 The sequential changes in the distributions of motor and sensory deficits of the patient.

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  Fig. 2 The first nerve conduction study in right median and left ulnar nerves show conduction blocks and temporal dispersion with mild slowing of nerve conduction velocities.

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  Fig. 3 Pathological findings of right superficial peroneal nerve. (A) A profound loss of myelinated fibers is seen in one fascicle, while they are relatively spared in others (modified Gomori-Trichome, ×40). (B) On semithin sections, marked nerve fiber hypertrophy with onion bulb formations is suggested (toluidine blue, ×400).

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