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

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

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

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

Microscopic polyangiitis (MPA) is a systemic small vessel vasculitis with few or no immune deposits and no granulomatous inflammation. Peripheral neuropathy occurs in approximately 20%–30% of patients with MPA. We report a case of a 66-year-old woman who presented with paresthesia and motor weakness of the extremities and rapidly progressive glomerulonephritis. She was later diagnosed with MPA based on the findings of positive perinuclear antineutrophil cytoplasmic antibody along with findings on kidney biopsy. Nerve conduction study showed symmetric sensorimotor polyneuropathy. We followed the patient for 3 years, and she showed good functional outcome after immune-modulating therapy although Five-Factor Score more than 2 at diagnosis.
Aged ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Diagnosis ; Extremities ; Female ; Follow-Up Studies* ; Glomerulonephritis ; Humans ; Inflammation ; Kidney ; Microscopic Polyangiitis* ; Neural Conduction ; Paresthesia ; Peripheral Nervous System Diseases* ; Polyneuropathies ; Vasculitis

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

OBJECTIVE: To determine whether patients with lumbosacral (LS) radiculopathy and peripheral polyneuropathy (PPNP) exhibit sudomotor abnormalities and whether SUDOSCAN (Impeto Medical, Paris, France) can complement nerve conduction study (NCS) and electromyography (EMG). METHODS: Outpatients with lower extremity dysesthesia underwent electrophysiologic studies and SUDOSCAN. They were classified as normal (group A), LS radiculopathy (group B), or PPNP (group C). Pain severity was measured by the Michigan Neuropathy Screening Instrument (MNSI) and visual analogue scale (VAS). Demographic features, electrochemical skin conductance (ESC) values on hands and feet, and SUDOSCAN-risk scores were analyzed. RESULTS: There were no statistical differences in MNSI and VAS among the three groups. Feet-ESC and hands-ESC values in group C were lower than group A and B. SUDOSCAN-risk score in group B and C was higher than group A. With a cut-off at 48 microSiemens of feet-ESC, PPNP was detected with 57.1% sensitivity and 94.2% specificity (area under the curve [AUC]=0.780; 95% confidence interval [CI], 0646–0.915). With a SUDOSCAN-risk score cut-off at 29%, NCS and EMG abnormalities related to LS radiculopathy and PPNP were detected with 64.1% sensitivity and 84.2% specificity (AUC=0.750; 95% CI, 0.674–0.886). CONCLUSION: SUDOSCAN can discriminate outpatients with abnormal electrophysiological findings and sudomotor dysfunction. This technology may be a complementary tool to NCS and EMG in outpatients with lower extremity dysesthesia.
Complement System Proteins ; Cross-Sectional Studies* ; Diabetes Mellitus ; Diagnosis* ; Electromyography ; Erythromelalgia ; Foot ; Galvanic Skin Response ; Hand ; Humans ; Lower Extremity* ; Mass Screening ; Michigan ; Neural Conduction ; Outpatients ; Paresthesia* ; Polyneuropathies ; Radiculopathy ; Sensitivity and Specificity ; Skin

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