Abstract Details

Title Severe painful neuropathy as the presenting feature of POEMS syndrome

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P13 - Poster Session 13 (8:00 AM-9:00 AM)

Poster/Presentation
Number 10-012

Objective POEMS is a rare and disabling paraneoplastic syndrome, with a plasma cell disorder background and a multisystem dysfunction. The acronym, first coined in 1980 by Bardwick, refers to polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Not all of the features in this acronym are required to establish the diagnosis and other ones, not included in the name, are of utmost importance, such as elevated vascular endothelial growth factor (VEGF) levels, sclerotic bone lesions, extravascular volume overload, thrombocytosis and papilledema¹.
The diagnosis of POEMS syndrome is based upon the presence of the two mandatory major criteria (polyneuropathy and monoclonal gammopathy), at least 1 other major criteria (Castleman disease, sclerotic bone lesions and VEGF elevation) and at least 1 minor criteria (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema and thrombocytosis/polycythemia)².

Background A 60-year-old man presented with a two-year history of severe neuropathic pain in limbs, distal weakness and hypoesthesia for pain and light touch. Due to a suspicion of a myeloradiculopathy, the patient underwent a cervical arthrodesis. On neurological examination there was distal muscular atrophy, a steppage gait, hyporeflexia in the lower limbs and allodynia in the feet. Complementary etiological investigation of neuropathy showed IgG lambda monoclonal gammopathy. MRI showed sclerotic bone lesion in the lumbosacral spine, and a mielography was compatible with plasma cell neoplasms. The patient began cyclophosphamide chemotherapy with significant improvement, and now waits for hematopoietic stem cell transplantation.

Design/Methods NA

Results NA

Conclusions The neuropathy is commonly the first clinical finding in POEMS and is usually demyelinating, sensory-motor, length-dependent and may be painful (65%). In this case report we reinforce not only the importance of a throughout clinical evaluation, but also an extensive etiologic investigation when facing neuropathies, as they can be the unique early manifestation of a serious treatable disorder, as POEMS syndrome.

Authors/Disclosures
Thiago D. Pinheiro, MD (Uff) PRESENTER	Dr. Pinheiro has nothing to disclose.
	No disclosure on file
Lya M. Marques, MD (Hospital Universitário Antônio Pedro)	Dr. Marques has nothing to disclose.
Tairine L. Silva, MBBS	Dr. Silva has nothing to disclose.
Caroline B. Braune, MD (Fluminense Federal University Department of Neurology)	Dr. Braune has nothing to disclose.

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