POEMS Syndrome Diagnosed 10 Years after Disabling Peripheral Neuropathy (original) (raw)
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Thrombocytosis distinguishes POEMS syndrome from chronic inflammatory demyelinating polyneuropathy
Muscle & Nerve, 2015
OEMS is a rare syndrome characterized by the unique constellation of polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes. Correct diagnosis is often delayed in early stages of the syndrome when patients exhibit only isolated polyneuropathy due to the clinical and electrodiagnostic similarities with chronic inflammatory demyelinating polyneuropathy. We describe a case in which early suspicion for POEMS uncovered underlying malignancy, and we review the clinical, electrophysiological, pathological, and laboratory findings characteristic of POEMS. The importance of high clinical suspicion is key in the proper diagnosis and management of this complex syndrome.
Journal of Neurology, Neurosurgery & Psychiatry, 2012
Background POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome, a rare cause of demyelinating neuropathy associated with multiorgan involvement, has been increasingly recognised. Polyneuropathy is often an initial manifestation and therefore the disorder can be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP). Objective To elucidate whether POEMS syndrome and CIDP are differentiated based on profiles of neuropathy. Methods Clinical and electrophysiological data were reviewed in consecutive POEMS syndrome (n¼51) and typical CIDP (n¼46) patients in a single Japanese hospital between 2000 and 2010.
An update on the diagnosis and management of the polyneuropathy of POEMS syndrome
Journal of Neurology
POEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells disorder and skin changes. Diagnosis relies on the fulfillment of a set of clinical criteria of which polyneuropathy and a monoclonal plasma cell dyscrasia are early and essential features. Treatment may be either local or systemic and is aimed at the monoclonal plasma cell disorder. Our knowledge of the pathogenesis underlying the POEMS syndrome has advanced greatly over the past years, favoring an important progression in the recognition and management of this disorder. Here, we discuss the recent literature that has advanced our knowledge of the pathogenesis and clinical management of the polyneuropathy in POEMS syndrome.
An Atypical Case of POEMS Syndrome Associated with Autonomic Dysfunction
The Open Neurology Journal, 2019
A 52 year old patient showed, for two years, symptoms compatible with sensorimotor polyneuropathy preventing him from walking. An electroneuromyography found a demyelinating pattern suggesting Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). The patient did not respond to corticosteroid therapy and later suffered sexual Dysfunction, Swelling, Lymphadenopathy, Hypotension, astrointestinal dysmotility, urinary retention and neuropathic pain. Analysis of the Cerebrospinal Fluid (CSF) revealed elevated protein levels and Computed Tomography (CT) scan found sclerotic bone lesions. High Vascular Endothelial Growth Factor (VEGF) levels and the results of Lambda light-Chain monoclonal gammopathy in urine protein Electrophoresis Suggested a Diagnosis of POEMS syndrome. The most striking feature, in this case, was the patient’s heightened and atypical polyneuropathy without axonal injury even after an extended period of time, and significant and atypical dysautonomia.
Journal of Neurology Neurosurgery and Psychiatry, 2010
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome is a rare cause of demyelinating neuropathy with monoclonal plasma cell proliferation, and POEMS neuropathy is usually chronically progressive. Herein, the authors report a 34-year-old woman with POEMS syndrome presenting as acute polyneuropathy. Within 2 weeks of disease onset, she became unable to walk with electrodiagnostic features of demyelination and was initially diagnosed as having GuillaneBarré syndrome. Other systemic features (oedema and skin changes) developed later, and an elevated serum level of vascular endothelial growth factor led to the diagnosis of POEMS syndrome. She received high-dose chemotherapy with autologous peripheral blood stem cell transplantation, resulting in good recovery. The authors also reviewed patterns and speed of progression of neuropathy in the 30 patients with POEMS syndrome; 22 (73%) of them were unable to walk independently with the median period of 9.5 months from POEMS onset (range 0.5e51 months). Whereas the speed of neuropathy progression varies considerably among patients, some POEMS patients can show acute or subacute polyneuropathy. The early diagnosis and treatment could result in rapid improvement as shown in the present patient. < Additional tables are published online only. To view these files please visit the journal online
Diseases which cause generalized peripheral neuropathy: a systematic review
Scandinavian Journal of Gastroenterology, 2021
Purpose: Peripheral autonomic neuropathy, including enteric neuropathy, may be subtle and unrecognized for several years. Diagnosis of enteric neuropathy demands complicated examinations such as full-thickness bowel biopsy. We hypothesized that knowledge about simultaneous occurrence of different types of neuropathy would lead to faster recognition and diagnosis of autonomic/enteric neuropathy. The aim of the present systematic review was to increase the awareness of disease groups causing autonomic and enteric neuropathy along with sensorimotor neuropathy. Methods: A systematic search strategy was used in PubMed, Embase and Web of Science. First, 4978 articles were identified. Review of titles/abstracts rendered exclusion of animal studies, articles not written in English or full-length, case reports, conference abstracts and duplicates until 357 articles remained. The full-length evaluation resulted in 35 studies (27 non-systematic reviews) which described objectively verified peripheral autonomic, enteric and sensorimotor neuropathy within the same disease. Results: Diabetes is the most common disease in society rendering generalized peripheral neuropathy. Accumulation of tissue deposits in amyloidosis, Lewy body disorders and sarcoidosis lead to widespread peripheral neuropathy. Several autoimmune disorders such as systemic sclerosis and primary Sj€ ogren's syndrome present themselves with neuropathy. Paraneoplastic neuropathy may appear prior to symptoms from the malignancy. Both the infection per se, as well as the autoimmune response to the infection, i.e., Guillain-Barr e syndrome, may lead to widespread peripheral neuropathy. Hereditary disorders with disturbed metabolism lead to intermittent attacks of neuropathy. Conclusions: The major causes of generalized peripheral neuropathy are diabetes, diseases with tissue deposits, autoimmunity, infections, malignancy and metabolic diseases.
Case 7-2010: A 49-Year-Old Man with Peripheral Neuropathy and Ascites
Pr e sen tat ion of C a se Dr. Caron A. Jacobson (Medical Oncology): A 49-year-old man was admitted to this hospital because of ascites. The patient had been well until approximately 4 years earlier, when paresthesias of both feet, weakness of the lower legs, and bilateral foot drop developed gradually. Three years before admission, he saw a neurologist at another hospital. Laboratory test results, including testing for autoantibodies, were reportedly normal. Electromyography (EMG) reportedly showed prolonged F responses in both feet, normal motor and sensory responses in the distal segments of the arms, and no motor responses in the intrinsic foot muscles. A diagnosis of chronic inflamma-tory demyelinating polyneuropathy (CIDP) was made, and therapy with intravenous immune globulin was begun. Two and a half years before admission, the patient was referred to the neurol-ogy clinic of this hospital. On examination, the Romberg test was markedly abnormal ; there was bilateral foot drop, and the patient was unable to walk on his toes or heels. He was able to sign his name but not without visual control. Strength (on a scale of 0 to 5, where 5 is normal) in the ankle dorsiflexors measured 1 to 2; in the muscles of ankle eversion and inversion, 4−; in the extensor hallucis longus, extensor digitorum brevis, and toe flexors, 0; and in the plantar flexors, 4− to 4. The left hand was mildly weak, particularly the abductor pollicis brevis, and strength was normal in the proximal arms. Deep-tendon reflexes were absent. Perception of vibration was absent in both first toes, decreased in both ankles, and normal at the knees and fingers. Position sense was diminished in both first toes. There was no muscle atrophy or fasciculation, and the remainder of the neurologic examination was normal. As compared with the previous study, EMG and nerve-conduction studies showed decreased sensory responses, absent sural-nerve responses, prolonged F-wave latencies, and more prominent denerva-tion in the tibialis anterior and medial gastrocnemius muscles, features consistent with a demyelinating polyneuropathy. During the next 6 months, weakness in the patient's legs and arms increased, and intermittent stabbing pain in the lower legs developed. Intravenous immune
POEMS syndrome with prominent acute axonal lesions
Journal of The Neurological Sciences, 2000
Polyneuropathy is a common presenting component of POEMS syndrome whose symptoms are attributed to an overproduction of vascular endothelial growth factor (VEGF). We report two female patients with POEMS syndrome presenting as a severe predominantly axonal neuropathy. A nerve biopsy was performed for these patients; pathological data confirmed unusual numerous acute axonal lesions associated with other classical signs of POEMS syndrome. POEMS syndrome is usually associated with demyelinating neuropathy (and secondary axonal loss); however, prominent axonal neuropathy (with acute axonal lesions on nerve biopsy) can also be observed in this disease. These observations illustrate the heterogeneity of peripheral nervous system involvement in POEMS syndrome.