Related Experiment Video

Updated: Aug 8, 2026

Targeting Alpha Synuclein Aggregates in Cutaneous Peripheral Nerve Fibers by Free-floating Immunofluorescence Assay
08:33

Targeting Alpha Synuclein Aggregates in Cutaneous Peripheral Nerve Fibers by Free-floating Immunofluorescence Assay

Published on: June 25, 2019

POEMS syndrome: clinical, pathological and immunological study of a case

G Orefice1, V B Morra, G De Michele

  • 1Department of Neurology, Federico II University.

Neurological Research
|December 1, 1994
PubMed

Insights

POEMS syndrome in a young Italian woman presented with plasma cell dyscrasia and a mixed sensorimotor neuropathy. High cerebrospinal fluid interleukin-6 levels were observed, with prednisone offering some clinical improvement.

Area of Science:

  • Neurology
  • Hematology
  • Immunology

Background:

  • POEMS syndrome is a rare multisystem disorder.
  • It is characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes.
  • This case highlights a presentation without overt multiple myeloma.

Observation:

  • A young Italian woman presented with POEMS syndrome.
  • She had plasma cell dyscrasia but no clinical or laboratory evidence of multiple myeloma.
  • Phenotypic analysis of bone marrow and lymphocytes was normal.

Findings:

  • Cerebrospinal fluid (CSF) demonstrated elevated interleukin-6 (IL-6) levels, while serum IL-6 was undetectable.
  • Electrophysiological studies and sural nerve biopsy revealed a mixed demyelinating-axonal sensorimotor neuropathy.
  • A significant loss of large myelinated fibers was noted.

Implications:

  • Elevated CSF IL-6 may be a key indicator in POEMS syndrome, even with normal serum levels.
  • This case underscores the importance of considering POEMS syndrome in young patients with unexplained neuropathy and plasma cell dyscrasia.
  • Prednisone treatment showed potential for clinical improvement in this patient.

More Related Videos

Isolating Human Peripheral Blood Mononuclear Cells and CD4+ T cells from Sézary Syndrome Patients for Transcriptomic Profiling09:08

Isolating Human Peripheral Blood Mononuclear Cells and CD4+ T cells from Sézary Syndrome Patients for Transcriptomic Profiling

Published on: October 14, 2021

Primary Sjogren's Syndrome Associated with Lung Adenocarcinoma: Probing the Potential Common Pathogenic Mechanisms and Experimental Verification10:21

Primary Sjogren's Syndrome Associated with Lung Adenocarcinoma: Probing the Potential Common Pathogenic Mechanisms and Experimental Verification

Published on: September 20, 2024

Related Concept Videos

Lysosomal Hydrolases01:22

Lysosomal Hydrolases

Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Myocarditis II: Clinical Features and Diagnostic Tests01:27

Myocarditis II: Clinical Features and Diagnostic Tests

Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
Introduction to Language of Pathophysiology l01:25

Introduction to Language of Pathophysiology l

Pathophysiology investigates how biological mechanisms—typically starting at the cellular level—disrupt normal bodily functions. It bridges anatomy and physiology to explain the progression of disease. With this foundation, it is important to understand the following key terms used to describe disease processes: Diagnosis:The process of identifying a disease using clinical evaluation, including signs (objective evidence like rashes), symptoms (subjective experiences like pain), laboratory test...
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...