POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs: 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.
Bone Marrow ; Diagnosis ; Flow Cytometry ; Humans ; Hyperplasia ; Medical Records ; Megakaryocytes ; Paraneoplastic Syndromes ; Paraproteinemias ; Plasma Cells ; POEMS Syndrome ; Polyneuropathies ; Skin

Swallowing can be affected by a variety of systemic diseases. The etiology of dysphagia in the geriatric population is usually overlooked due mainly to a presumed diagnosis of presbyphagia or difficulty in revealing the direct cause. On the other hand, dysphagia can be a meaningful clinical sign of premalignant systemic disease. A 78-year-old man, without any prior medical or family history, was admitted with the chief complaint of dysphagia with recent aspiration pneumonia. Instrumental swallowing tests revealed a severe degree of dysphagia due to decreased laryngopharyngeal sensation and weakness of the pharyngeal constrictor muscles. Extensive workup, including electromyography and laboratory tests, revealed severe sensorimotor peripheral polyneuropathy related to monoclonal gammopathy. Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant precursor of multiple myeloma, which is characterized by the proliferation of monoclonal proteins. These conditions are often associated with peripheral polyneuropathy, ataxia, and sometimes even muscle weakness. Although dysphagia can occur in other systemic disorders, such as vasculitis or paraneoplastic syndrome-related malignancies, there are few reports of dysphagia related to MGUS. The patient was followed up for three years. The MGUS showed no further progression, but the patient showed no improvement, indicating a protracted clinical course and poor prognosis when dysphagia is related to MGUS.
Aged ; Ataxia ; Deglutition ; Deglutition Disorders ; Diagnosis ; Electromyography ; Hand ; Humans ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Muscle Weakness ; Muscles ; Paraproteinemias ; Pneumonia, Aspiration ; Polyneuropathies ; Prognosis ; Sensation ; Vasculitis

Electrodiagnostic tests (EDX) is essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). EDX could provide information about demyelinating pathology in the peripheral nerves. According to phenotypes, CIDP could be classified several phenotypes, which has different clinical manifestations, EDX could present a different distribution pattern of demyelinating lesions. In addition, EDX could be useful markers for predicting treatment response of prognosis of CIDP.
Classification ; Diagnosis ; Electrodiagnosis ; Neural Conduction ; Pathology ; Peripheral Nerves ; Phenotype ; Polyneuropathies ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating ; Prognosis

Early recognition and appropriate management of diabetic peripheral polyneuropathy (DPNP) is important. We evaluated the necessity of simple, non-invasive tests for DPNP detection in clinical practice. We enrolled 136 randomly-chosen patients with type 2 diabetes mellitus and examined them with the 10-g Semmes-Weinstein monofilament examination, the 128-Hz tuning-fork, ankle-reflex, and pinprick tests; the Total Symptom Score and the 15-item self-administered questionnaire of the Michigan Neuropathy Screening Instrument. Among 136 patients, 48 had subjective neuropathic symptoms and 88 did not. The abnormal-response rates varied depending on the methods used according to the presence of subjective neuropathic symptoms (18.8% vs. 5.7%, P < 0.05; 58.3% vs. 28.4%, P < 0.005; 81.3% vs. 54.5%, P < 0.005; 12.5% vs. 5.7%, P=0.195; 41.7% vs. 2.3%, P < 0.001; and 77.1% vs. 9.1%, P < 0.001; respectively). The largest abnormal response was derived by combining all methods. Moreover, these tests should be implemented more extensively in diabetic patients without neuropathic symptoms to detect DPNP early.
Diabetes Mellitus ; Diabetes Mellitus, Type 2* ; Diabetic Neuropathies ; Diagnosis ; Humans ; Mass Screening ; Michigan ; Neurologic Examination ; Peripheral Nervous System Diseases* ; Polyneuropathies ; Surveys and Questionnaires

No abstract available.
Diagnosis ; Myotonia ; Polyneuropathies ; Relaxation

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis that commonly affects the peripheral nervous system. EGPA rarely presents with acute polyneuropathy resembling Guillain-Barré syndrome (GBS). A 51-year-old female patient with a history of asthma suddenly developed bilateral lower extremityparesthesia that progressed to asymmetric ascending paralysis within 10 days of onset. Nerve conduction study results were compatible with acute motor sensory axonal neuropathy, consistent with a GBS subtype. A clinical and neurophysiological diagnosis of GBS was made, and high-dose intravenous immunoglobulins were administered. However, the patient's painful motor weakness persisted. Furthermore, she had newly developed skin lesions on her back, face, and arms. Her blood test revealed marked eosinophilia (>60%). In addition, antineutrophil cytoplasmic antibodies were reported positive. A Water's view radiographic image showed bilateral maxillary sinusitis. Considering the history of asthma, we suspected EGPA-associated polyneuropathy and started steroid treatment. The patient's strength and eosinophilia improved rapidly and dramatically. EGPA can mimic GBS and should be differentiated because of different treatment strategies. Early diagnosis and prompt treatment help achieve a good outcome.
Antibodies, Antineutrophil Cytoplasmic ; Arm ; Asthma ; Axons ; Diagnosis ; Early Diagnosis ; Eosinophilia ; Eosinophils ; Female ; Granulomatosis with Polyangiitis ; Guillain-Barre Syndrome ; Hematologic Tests ; Humans ; Immunoglobulins, Intravenous ; Maxillary Sinus ; Maxillary Sinusitis ; Middle Aged ; Neural Conduction ; Paralysis ; Peripheral Nervous System ; Polyneuropathies ; Skin ; Systemic Vasculitis

Polyneuropathy includes a lot of diseases damaging peripheral nerves. It shows roughly the same areas on both sides of the body, featuring weakness, numbness, and burning pain. Polyneuropathy is known to usually begin in the hands and feet and progress to the arms and legs. Sometimes it can involve other parts of the body such as the autonomic nervous system. Lots of causes can induce acute or chronic polyneuropathy, so finding the original cause is most important for the treatment of polyneuropathy. There are too many different types of polyneuropathies to be discussed in this review, so we will discuss some of various acquired polyneuropathies such as diabetic neuropathy, vasculitic neuropathy, alcoholic neuropathy, Vitamin B12 deficiency neuropathy, and drug-induced neuropathy, with special focus on symptoms, pathogenesis, diagnosis, treatment, and prognosis.
Alcoholic Neuropathy ; Arm ; Autonomic Nervous System ; Burns ; Diabetic Neuropathies ; Diagnosis* ; Foot ; Hand ; Hypesthesia ; Leg ; Peripheral Nerves ; Polyneuropathies* ; Prognosis* ; Vitamin B 12 Deficiency

No abstract available.
Physical Phenomena ; Prostatitis ; Helicobacter Infections ; Helicobacter pylori ; Central Serous Chorioretinopathy ; Diabetes, Gestational ; Ophthalmologists ; Phosphatidylinositol 3-Kinases ; Diagnosis, Differential ; Physicians ; Writing ; Speech ; Thinking ; Prognosis ; Otolaryngology ; Internal Medicine ; Pelvic Pain ; Polyneuropathies ; Korea

BACKGROUND: Argyria is a rare irreversible cutaneous pigmentation disorder caused by prolonged exposure to silver. Herein, we report a case of generalized argyria that developed after chronic ingestion of soluble silver-nano particles and presented with muscle weakness. CASE PRESENTATION: A 74-year-old woman visited our emergency room, complaining of fever and mental deterioration. She was diagnosed with acute pyelonephritis and recovered after antibiotic therapy. At presentation, diffuse slate gray-bluish pigmented patches were noticed on her face and nails. Two months prior to visiting our hospital, she was diagnosed with inflammatory myopathy and given steroid therapy at another hospital. We performed a nerve conduction study that revealed polyneuropathy. In skin biopsies from pigmented areas of the forehead and nose, the histopathologic results showed brown-black granules in basement membranes of sweat gland epithelia, which are diagnostic findings of argyria. We reviewed pathology slides obtained from the left thigh muscles and found markedly degenerated myofibers with disorganization of myofibrils without inflammatory reactions, consistent with unspecified myopathy, rather than inflammatory myopathy. The patient was diagnosed with generalized argyria with polyneuropathy and myopathy and transferred to a rehabilitation institution after being tapered off of steroids. CONCLUSIONS: Clinicians should be aware of clinical manifestations of argyria and consider it in differential diagnosis when they examine patients who present with skin pigmentation and muscle weakness.
Aged ; Argyria* ; Basement Membrane ; Biopsy ; Diagnosis, Differential ; Eating ; Emergency Service, Hospital ; Female ; Fever ; Forehead ; Humans ; Muscle Weakness* ; Muscles ; Muscular Diseases ; Myofibrils ; Myositis ; Neural Conduction ; Nose ; Pathology ; Pigmentation Disorders ; Polyneuropathies ; Pyelonephritis ; Rehabilitation ; Silver ; Skin ; Skin Pigmentation ; Steroids ; Sweat Glands ; Thigh

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis that affect small and medium-sized blood vessels and is accompanied by asthma, eosinophilia, and peripheral neuropathy. This report describes a case of a 52-year-old man who had a history of sinusitis, asthma, and thymus cancer and who had complained of bilateral lower extremity paresthesia and weakness for a month. Peripheral neuropathy was detected by electrodiagnostic studies. Resection of a mediastinal mass, which was diagnosed as thymic neuroendocrine carcinoma, was performed five months before his visit. After thymectomy, peripheral blood tests revealed a gradual increase in eosinophils. Two months after surgery, he was admitted to the hospital for dyspnea, and nodules of focal consolidation were found in his chest X-ray. One month later, pyoderma occurred in the right shin, and the skin biopsy showed extravascular eosinophilic infiltration. He was diagnosed with CSS after thymectomy, and we report a very rare case of CSS presented with thymic neuroendocrine carcinoma.
Asthma ; Biopsy ; Blood Vessels ; Carcinoma, Neuroendocrine* ; Churg-Strauss Syndrome* ; Diagnosis* ; Dyspnea ; Eosinophilia ; Eosinophils ; Hematologic Tests ; Humans ; Lower Extremity ; Middle Aged ; Paresthesia ; Peripheral Nervous System Diseases ; Polyneuropathies ; Pyoderma ; Sinusitis ; Skin ; Systemic Vasculitis ; Thorax ; Thymectomy ; Thymus Neoplasms