Vagal mononeuropathy is very rare. Diabetes mellitus is one of the causes of this rare disease condition. Here we report a 44-year-old woman who presented with an idiopathic vagal mononeuropathy and was finally diagnosed with diabetic vagal mononeuropathy. She presented with isolated dysphagia without hoarseness or other symptoms related with vagal dysfunction. Except for diabetes mellitus, no abnormalities were found by routine and specific checkups including brain imaging, gastroscopy, electromyography, and laryngoscopy. Finally, 12 days later, she abruptly developed hoarseness without other cranial nerve dysfunction.We suggest that her neurological symptoms originated from diabetes affecting the vagus nerve in isolation. Clinicians should pay attention to this association, especially when they encounter a patient with diabetes mellitus with sudden idiopathic dysphagia even without problems of vocalization.
Diabetes Mellitus

PURPOSE: We report one case of Purtscher's retinopathy related to chest compression by safety belts during motor vehicle accident. METHODS: A 31 old year man was referred to our ophthalmologic department because of diminished vision in the left eye at 6 days after motor vehicle accident. Best uncorrected visual acuity (UCVA) was right eye 1.0 and left eye 0.5. RESULTS: Best corrected visual acuity (BCVA) was both 1.0. In the left fundus there was multiple cotton wool spots, retinal hemorrhages and macular edema. At 15 days after accident, multiple cotton wool spots and retinal hemorrhages still be seen. Fluorescein angiography showed capillary nonperfusions and blocked fluorescence by retinal hemorrhages in the arteriovenous phase, leakage of dye in the region of capillary nonperfusion in the late venous phase. At 58 days after accident, multiple cotton wool spots remarkably decreased, however vision remained as 0.2. At 78 days after accident, left eye vision improved to 0.5. CONCLUSIONS: This case suggested to consider a safety belt injury as the cause of Purtscher's retinopathy.
Capillaries ; Fluorescein Angiography ; Fluorescence ; Macular Edema ; Motor Vehicles ; Retinal Hemorrhage ; Thorax ; Vision, Low ; Visual Acuity ; Wool

BACKGROUND: The use of prednisone in the treatment of myasthenia gravis (MG) has been required circumspection because of its tendency to produce exacerbations of weakness in the early stages of treatment, often requiring critical management. However, factors influencing the exacerbation has not been defined well. The purpose of this study was to evaluate the clinical factors predicting the exacerbation by prednisone in the early stage of treatment in MG. METHODS: Fifty five patients, first-ever prescribed high dose prednsone (40-80 mg) during hospitalization in Samsung Medical Center were included. Prednisone induced exacerbation was defined as significant exacerbation of objective neurologic signs of MG within 4 weeks after prednisone addition by utilizing Myasthnia gravis Severity Scale (MSS). We investigate the differences between the exacerbated and nonexacerbated groups in the clinical, laboratory and electrophysiological features. RESULTS: Twenty three patients (42%) experienced definite exacerbation after prednisone treatment. Old age, presence of bulbar symptom, and severe neurologic finding reflected by MSS score were significant predictors of prednisone induced exacerbation in multivariate logistic regression analysis. Higher prednisone dosage per body weight (Kg) was neither a significant predictor of exacerbation nor related with the early improvement in bivariate correlation. CONCLUSIONS: Prednisone induced exacerbation in MG is a frequent challenging problem to clinician. Clinicans should keep in mind the possibility of exacerbation of MG when prescribing prednisone especially, to old, bulbar dominant, severe and disable myasthenic patients.
Body Weight ; Hospitalization ; Humans ; Logistic Models ; Myasthenia Gravis* ; Neurologic Manifestations ; Prednisone

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogenous group of acquired peripheral neuropathies. A subset of CIDP involves predominantly distal parts of the limbs, which is similar to axonal polyneuropathy. The clinical course or response to treatment may be different in this group. We investigated the clinical course and electrodiagnostic findings of the distal CIDP. METHODS: Twenty five CIDP cases were reviewed retrospectively. Patients with proximal as well as distal involvement were grouped as typical CIDP, and patients with predominantly distal involvement as distal CIDP. We compared the clinical, laboratory and electrophysiological findings of these two groups. RESULTS: Sixteen patients had typical CIDP and nine had distal CIDP. Distal CIDP differed significantly from typical CIDP; later age of onset (p=0.049), less frequent relapses (p=0.041), more rapidly progressive to maximal disability (p =0.01), low disability score at the diagnosis (p=0.02) and after treatment (p=0.01), poor response to immunomodulating therapy (p=0.02), and infrequent conduction blocks or abnormal temporal dispersions (p<0.01). CONCLUSIONS: Distal CIDP is a distinctive variant of CIDP different from typical CIDP in clinical and electrophysiological features. Identification of the pathogenesis underlying this new entity may lead to better understanding of the heterogeneous acquired demyelinating neuropathies.
Age of Onset ; Axons ; Diagnosis ; Extremities ; Humans ; Peripheral Nervous System Diseases ; Polyneuropathies ; Polyradiculoneuropathy ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* ; Recurrence ; Retrospective Studies

BACKGROUND: It has been well known that absolute and interpeak latencies of brainstem auditory evoked potentials (BAEP) are usually prolonged in diabetics. However, Its etiology is still controversial. We tried to identify whether the cause is structural or metabolic in origin by performing BAEP and brain MRI in the diabetic patients. METHODS: BAEP were performed in both the diabetic patients (DM) group (16 males and 15 females) and the normal control group (25 males and 33 female). A brain MRI was performed in the DM group on those who showed abnormal BAEP and com-pared the results of BAEP of the DM group with those of the control group. RESULTS: 7 patients (22.6%) showed abnor-mal BAEP (male; 6, female; 1, unilateral; 4, bilateral; 3) when abnormal BAEP was defined as being larger than two and a half standard deviations of the control group BAEP results. Two males of the DM group who showed abnormal structural lesions of the pons in their brain MRI were not included in the statistical analysis. The remaining 14 diabetic male patients (mean age: 58.7 +/-9.1 years, mean disease duration: 6.1 +/-4.7 years, mean hemoglobin (Hb) A1C: 7.7 +/- 2.0%) and 15 diabetic female patients (mean age: 60.6 +/-10.8 years, mean disease duration: 5.4 +/-5.1 years, mean HgA1C: 7.8 +/-2.1%) were stastistically analyzed. Interpeak latencies of I-III, III-V, and I-V were found to be signifi-cantly prolonged in the DM group. The prolongation of interpeak latencies of I-III and I-V were found to be signifi-cantly correlated with the disease duration only in the diabetic female patients, but not with age and HbA1c. CONCLUSIONS: These findings suggest that both metabolic derangement and structural lesion contribute to prolonging the central conduction time on BAEP pathway in diabetics.
Brain ; Brain Stem* ; Diabetes Mellitus ; Evoked Potentials, Auditory, Brain Stem* ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Pons

Hirayama disease usually selectively involves lower cervical myotomes (C8, T1). Thus, patients usually manifest with atrophy and weakness of small hand muscle. Predominant isolated involvement of proximal arm is rarely reported in Hirayama disease. Here, we report a case of Hirayama disease who had focal weakness and wasting, mainly confined to right biceps brachii muscle, with prominent shifting of dural sac in C4-5 segment by dynamic flexion magnetic resonance imaging (dfMRI), which may explain this unusual distribution of the disease.
Amyotrophy, monomelic

Outcome measurements are essential to monitor the clinical course or the treatment response of peripheral neuropathy. Even though there are no designated standard scale for peripheral neuropathy currently, several clinical scales were validated to use for outcome measurements based on many researches. Here, we reviewed clinical scales commonly used and fulfilled clinimetric properties in peripheral neuropathy, especially focusing on inflammatory neuropathy (Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy). Each scale was classified according to the International Classification of outcome measure model - the International Classification of Functioning, Disability and Health to achieve a comprehensive concept of clinical scale in peripheral neuropathy.

Outcome measurements are essential to monitor the clinical course or the treatment response of peripheral neuropathy. Even though there are no designated standard scale for peripheral neuropathy currently, several clinical scales were validated to use for outcome measurements based on many researches. Here, we reviewed clinical scales commonly used and fulfilled clinimetric properties in peripheral neuropathy, especially focusing on inflammatory neuropathy (Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy). Each scale was classified according to the International Classification of outcome measure model - the International Classification of Functioning, Disability and Health to achieve a comprehensive concept of clinical scale in peripheral neuropathy.

Guillain-Barré syndrome (GBS) is a representative form of post-infectious autoimmune neuropathy with heterogenous manifestations. It was originally considered as an ascending demyelinating polyneuropathy in Western countries. However, the discovery of anti-ganglioside antibodies on the basis of molecular mimicry theory could help us better understand various kinds of focal and regional variants as well as axonal type of GBS those were frequently found from Asian countries. Recent development of new techniques about anti-ganglioside complex antibodies is making more detailed descriptions for specific or unusual clinical manifestations. It has been regarded that GBS has good prognosis if treated properly as early as possible, but it still shows high mortality and morbidity rate with frequent long term neurologic and medical complications. Unfortunately, there are only two options for medical treatment, intravenous immunoglobulin and plasmapheresis, for the last 100 years. Several clinical studies on new immunotherapy targeting complement activating system with background of molecular mimicry using animal model are underway. We hope that these new treatments will be helpful for the future patients.
Antibodies ; Asian Continental Ancestry Group ; Axons ; Complement System Proteins ; Gangliosides ; Guillain-Barre Syndrome ; Hope ; Humans ; Immunoglobulins ; Immunotherapy ; Miller Fisher Syndrome ; Models, Animal ; Molecular Mimicry ; Mortality ; Plasmapheresis ; Polyneuropathies ; Prognosis

Pain in Guillain-Barré syndrome (GBS) is known as a common symptom, experienced by about 72% of patients. Various types of pain are associated with GBS, including paresthesia, dysesthesia, radicular pain, meningism, myalgia and visceral pain. Pain in GBS can vary from mild to severe, often under-recognized and poorly managed. This article reviews the various pains associated with Guillain-Barré syndrome and their management.