Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy

Nasu, Saiko; Misawa, Sonoko; Sekiguchi, Yukari; Shibuya, Kazumoto; Kanai, Kazuaki; Fujimaki, Yumi; Ohmori, Satoshi; Mitsuma, Satsuki; Koga, Shunsuke; Kuwabara, Satoshi

doi:10.1136/jnnp-2011-301706

Cited by 165 publications

(148 citation statements)

References 22 publications

Supporting

Mentioning

137

Contrasting

Unclassified

Order By: Relevance

“…The neuropathy is the dominant characteristic. The quality and extent of the neuropathy, which is peripheral, ascending, symmetrical, and affecting both sensation and motor function should be elicited [50]; in our experience, pain may be a dominant feature in about 10-15% of patients, and in one report as many as 76% of patients had hyperesthesia [9,51]. Papilledema is present in at least one-third of patients.…”

Section: Diagnosismentioning

confidence: 77%

“…In contrast to CIDP, conduction block is rare [9,67,69]. The conduction findings could suggest that demyelination is predominant in the nerve trunk rather than the distal nerve terminals and axonal loss is predominant in the lower limb nerves [9].…”

Section: Diagnosismentioning

confidence: 94%

“…Nerve conduction studies in patients with POEMS syndrome show slowing of nerve conduction that is more predominant in the intermediate than distal nerve segments as compared to CIDP, and there is more severe attenuation of compound muscle action potentials in the lower than upper limbs [9,[66][67][68][69]. In contrast to CIDP, conduction block is rare [9,67,69].…”

Section: Diagnosismentioning

confidence: 98%

See 2 more Smart Citations

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

2015

View full text Add to dashboard Cite

Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome.Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy.Risk‐adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3–6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. The benefit of anti‐VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 90:951–962, 2015. © 2015 Wiley Periodicals, Inc.

show abstract

Section: Diagnosismentioning

confidence: 77%

Section: Diagnosismentioning

confidence: 94%

Section: Diagnosismentioning

confidence: 98%

See 1 more Smart Citation

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

2015

View full text Add to dashboard Cite

show abstract

“…Patients often describe dysesthesia followed by a progressive weakness that overshadows the sensory impairment. Associated cranial or autonomic system involvement is rare [5,6].…”

Section: Discussionmentioning

confidence: 99%

POEMS Syndrome: A Case Report of a patient with CIDP

Mansour¹,

Souissi²,

Beyrouti³

et al. 2017

J Neurol Disord

View full text Add to dashboard Cite

POEMS syndrome is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, a monoclonal protein (M-protein), and skin changes. Polyneuropathy is the most common initial symptom and it can hide other features of this disorder. We describe a patient with chronic inflammatory demyelinating polyneuropathy (CIDP) associated with extra-neurologic signs fulfilling the diagnosis criteria of POEMS syndrome to emphasize the importance of meticulous physical examination leading to challenging diagnosis. Keywords Case ReportA 67-year-old man, with a previous history of lumbar plasmocytoma diagnosed in 2004 and successfully treated with high radiation dose, presented in July 2016 with distal lower limbs paresthesia and weakness progressing proximally and to the upper limbs. Within 9 months he was unable to walk or stand. He also reported erectile dysfunction without any sphincter problems.Clinical examination revealed symmetric limbs weakness more pronounced distally and in the lower limbs with generalized hyporeflexia, impairment of touch, pain, and temperature sensation in both hands, a severe affection of position and vibration senses in the higher limbs and an ataxic gait with a positive Romberg's sign. Examination of the cranial nerves was normal. At the general physical examination, we noticed a bilateral pretibial edema, hypertrichosis and multiples skin angioma (Figure 1) [1].Neurophysiological testing showed a severe demyelinating polyneuropathy with significant secondary axonal loss fulfilling the EFNS/ PNS diagnostic criteria for CIDP. The cerebrospinal fluid protein level was of 0.91 g/l with 9 leucocytes.Full blood count, renal and hepatic function tests, glycaemia, autoantibody screen, cryoglobulin screen, vitamin B12 and folate levels and thyroid function tests were within normal ranges. We excluded infections of lyme, hepatitis and human immunodeficiency virus via serologic tests.Magnetic resonance imaging (MRI) of spine showed discrete lesions of low signal intensity in both T1 and T2-weighted images of a lumbar vertebra. Biopsy of this lesion demonstrated a plasmacytoma.Serum electrophoresis revealed an IgG-lambda monoclonal serum protein. There was no Bence-Jones proteinuria. Bone marrow biopsy showed normal cellularity and did not detect any bone marrow plasma cell involvement, which excluded multiple myeloma.

show abstract

“…POEMS syndrome, the other manifestations are subtle, and the primary problem is the demyelinating neuropathy. 32 Correct diagnosis was only made after the patients failed to respond to the standard treatment.…”

Section: Differential Diagnosismentioning

confidence: 99%

Chronic Inflammatory Demyelinating Polyradiculoneuropathy 101—Pitfalls and Pearls of Diagnosis and Treatment

Beydoun¹,

Brannagan²,

Donofrio³

et al. 2017

US Neurology

View full text Add to dashboard Cite

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which is caused by demyelination of the peripheral nerves, is characterized by progressive weakness and impaired sensory function in the arms and legs. CIDP is a treatable condition in which early diagnosis is crucial to limit chronic disability. CIDP can mimic other neuropathies and it is important to identify these in order to ensure prompt treatment. Patients with other causes of neuropathy should be suspected of having CIDP if there is rapid progress or proximal weakness. Intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange are first-line therapies. The IVIG CIDP Efficacy (ICE) trial, the largest trial reported of any CIDP treatment, demonstrated that IVIG therapy reduced disability and functional impairment, as well as improved quality of life. Autoantibodies against membrane proteins of the peripheral nerve axons or the myelin sheath have been reported recently, and an improved understanding of antibody responses in CIDP may enable the development of future targeted therapeutic interventions.

show abstract

Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy

Abstract: Before development of typical systemic manifestations, POEMS neuropathy can be distinguished from CIDP by the clinical profile and patterns of nerve conduction abnormalities. Recognition of these features leads to early diagnosis and proper treatment for POEMS syndrome.

Cited by 165 publications

References 22 publications

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

POEMS Syndrome: A Case Report of a patient with CIDP

Chronic Inflammatory Demyelinating Polyradiculoneuropathy 101—Pitfalls and Pearls of Diagnosis and Treatment

Contact Info

Product

Resources

About