Myeloneuropathy in POEMS syndrome
โ Scribed by Raul N. Mandler; Molly K. King
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- English
- Weight
- 307 KB
- Volume
- 19
- Category
- Article
- ISSN
- 0148-639X
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โฆ Synopsis
POEMS, the acronym for "peripheral neuropathy, organomegaly, endocrinopathy, monoclonal Mprotein, and skin lesions," is an underrecognized syndrome of relevance for the neuromuscular specialist.' The full development of this condition is often heralded by a sensorimotor, acquired axonal and demyelinating peripheral neuropathy in young and middle-age patient^.^'^'^^^ Rarely, POEMS presents with additional clinical manifestations not included in the acronym, as in the present report.
CASE REPORT
A 37-year-old, right-handed, previously healthy man was referred for evaluation of a 10-month leg weakness and sensory loss, accompanied by impotence and hand tremors. On exam, mental status and cranial nerves were normal. Sensory ataxia and bilateral foot drop were present. Marked distal, predominantly lower extremity weakness was symmetrically found, with better proximal strength. The lower extremities were spastic. Enlarged peripheral nerves were not palpated. Deep tendon reflexes were absent in all joints. Babinski sign was not found, but stimulation of the soles elicited a triple flexor response. The sensory exam, abnormal for all modalities in a rather symmetric, distal manner, showed vibration and position senses to be more affected than pain and light touch. No truncal sensory level was found. A small, brown macular lesion (3 x 2 cm diameter) appeared on the left cheek a few weeks prior to hospitalization, and hyperpigmentation of
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