Clinical Usefulness of a Cell-based Assay for Detecting Myelin Oligodendrocyte Glycoprotein Antibodies in Central Nervous System Inflammatory Disorders

Background@#The clinical implications of myelin oligodendrocyte glycoprotein autoantibodies (MOG-Abs) are increasing. Establishing MOG-Ab assays is essential for effectively treating patients with MOG-Abs. We established an in-house cell-based assay (CBA) to detect MOG-Abs to identify correlations with patients’ clinical characteristics. @*Methods@#We established the CBA using HEK 293 cells transiently overexpressing fulllength human MOG, tested it against 166 samples from a multicenter registry of central nervous system (CNS) inflammatory disorders, and compared the results with those of the Oxford MOG-Ab-based CBA and a commercial MOG-Ab CBA kit. We recruited additional patients with MOG-Abs and compared the clinical characteristics of MOG-Ab-associated disease (MOGAD) with those of neuromyelitis optica spectrum disorder (NMOSD). @*Results@#Of 166 samples tested, 10 tested positive for MOG-Abs, with optic neuritis (ON) being the most common manifestation (4/15, 26.7%). The in-house and Oxford MOG-Ab CBAs agreed for 164/166 (98.8%) samples (κ = 0.883, P < 0.001); two patients (2/166, 1.2%) were only positive in our in-house CBA, and the CBA scores of the two laboratories correlated well (r = 0.663, P < 0.001). The commercial MOG-Ab CBA kit showed one falsenegative and three false-positive results. The clinical presentation at disease onset differed between MOGAD and NMOSD; ON was the most frequent manifestation in MOGAD, and transverse myelitis was most frequent in NMOSD. @*Conclusions@#The in-house CBA for MOG-Abs demonstrated reliable results and can potentially be used to evaluate CNS inflammatory disorders. A comprehensive, long-term study with a large patient population would clarify the clinical significance of MOG-Abs.

A Case of Anterior Communicating Artery Aneurysm Impending Rupture Presenting with Isolated Painful Adduction Palsy Mimicking Ischemic Internuclear Ophthalmoplegia

Anterior communicating artery (ACoA) aneurysms may rarely lead to oculomotor nerve palsy. We present here interesting cases in which isolated unilateral adduction paresis mimicking internuclear ophthalmoplegia (INO) was one of the symptoms of suspicious impending ruptured aneurysm of the ACoA. Careful neurologic examination is crucial for early discrimination with INO and oculomotor palsy.

A Case of Chiari Type 1 Malformation and Syringomyelia in Neurofibromatosis Type 1 Patient

A 26-year-old woman presented with skin pigmentation and numbness on the upper arm. More than six café au lait spots over 15 mm diameter could be seen throughout her skin. Neurofibromatosis (NF) type 1 was diagnosed based on genetic study. The brain magnetic resonance imaging (MRI) showed Chiari type 1 malformation and syringomyelia was noted in the C2 to T7 level in the spinal MRI. We suggest Chiari malformation and syringomyelia could be a rare manifestation in a patient with NF type 1.

Oculomotor Nerve Palsy that Developed after Endovascular Treatment in an Acute Stroke Patient with a Dissecting Occlusion of the Extracranial Internal Carotid Artery

Oculomotor nerve palsy is underrecognized clinical manifestation of extracranial internal carotid artery (ICA) dissection. Herein we report a case of complete oculomotor nerve palsy following endovascular revascularization, which developed in a patient with acute stroke due to extracranial ICA dissecting occlusion. We also discuss about the development mechanism of oculomotor nerve palsy, considering the vascular anatomy and the possibility of periprocedural complications during endovascular treatment.

Severe Hyponatremia during Acyclovir Treatment in Shingles / 대한신경근육질환학회지

Acyclovir is effective in treating the herpes virus infection, especially chickenpox and shingles. Hyponatremia caused by acyclovir has been rarely reported. We present a shingles patient with symptomatic hyponatremia that was presumably caused by acyclovir affecting the hypothalamus or posterior pituitary gland. This case demonstrates that the serum electrolyte levels should be checked regularly and careful observation of symptoms such as nausea, convulsions, or mentality change is necessary in patients treated with acyclovir.

Thoracic Endovascular Repair of Spinal Cord Infarction Resulting from Impending Rupture of the Thoracic Aortic Aneurysm

No abstract available.

Venous Thromboembolism Following Acute Ischemic Stroke: A Prospective Incidence Study

BACKGROUND: Asians were known to have a relatively lower incidence of venous thromboembolism (VTE), and there is insufficient evidence to suggest a specific D-dimer threshold level for screening VTE in patients with acute stroke. METHODS: We prospectively enrolled patients with acute ischemic stroke admitted to Jeju National University Hospital. The inclusion criteria were: 1) aged ≥18 years, 2) admission within seven days of symptom onset, and 3) an initial National Institute of Health Stroke Scale (NIHSS) score >1 for the affected lower limb. Ultrasound scans of the lower limbs and plasma D-dimer assays were performed on days 7–14 and 15–28 after stroke onset. RESULTS: Of 285 patients admitted during the study period, 52 patients met inclusion criteria (mean age 74.5, male 40.4%, median initial NIHSS score 12, and unable to walk unassisted at discharge 76.9%). During 7–14 days, 23 of 52 patients (44.2%) had a D-dimer level above 1.57 mg/L, and 9.6% had a level above 5.50 mg/L. Proximal deep vein thrombosis (DVT) was detected in 3 patients (5.8%, 95% confidence Interval 1.2–16.0%) on ultrasound examination. All DVTs were found in elderly female patients with severe leg weakness. No patient was diagnosed with pulmonary embolism during the study period. CONCLUSION: The incidence of VTE seems to be very low among Korean patients with acute ischemic stroke. Advanced age, female sex, and severe leg weakness were important risk factors for developing DVT in this study.

Polymyositis Associated with Autoimmune Hepatitis

A 46-year old woman presented with progressive limb weakness and fatigue. Laboratory tests showed abnormalities consistent with autoimmune hepatitis and she was diagnosed with biopsy-proven inflammatory myopathy. The patient was commenced on immunosuppressive therapy with azathioprine 50 mg and prednisolone 1 mg/kg. At the six-month follow up, her symptoms subsided and aminotransferase and muscle enzymes were normalized. This case presents rare case of concomitant development of polymyositis and autoimmune hepatitis.

Sinking Skin Flap Syndrome after Decompressive Craniotomy

Sinking skin-flap syndrome is a rare complication of a skull defect after craniotomy. A 69-year-old woman visited our hospital and was diagnosed with a left middle cerebral artery infarction. Decompressive craniotomy was performed for cerebral herniation on the following day. However, on day 25 of hospitalization her mental status worsened and her scalp appeared sunken. This case suggests that sinking skin-flap syndrome can develop from a skull defect after decompressive craniotomy.

The Impacts of Influenza Infection and Vaccination on Exacerbation of Myasthenia Gravis

BACKGROUND AND PURPOSE: Upper respiratory infection (URI), including influenza, may exacerbate the symptoms of myasthenia gravis (MG), which is an autoimmune disease that causes muscle weakness. There is also concern that the influenza vaccine may trigger or worsen autoimmune diseases. The objective of this study was to determine the impacts of influenza infection and vaccination on symptom severity in MG patients. METHODS: Patients diagnosed with MG were enrolled from 10 university-affiliated hospitals between March and August 2015. Subjects completed a questionnaire at the first routine follow-up visit after enrolling in the study. The patient history was obtained to determine whether a URI had been experienced during the previous winter, if an influenza vaccination had been administered before the previous winter, and whether their MG symptoms were exacerbated during or following either a URI or vaccination. Influenza-like illness (ILI) was defined and differentiated from the common cold as a fever of ≥38℃ accompanied by a cough and/or a sore throat. RESULTS: Of the 258 enrolled patients [aged 54.1±15.2 years (mean±SD), 112 men, and 185 with generalized MG], 133 (51.6%) had received an influenza vaccination and 121 (46.9%) had experienced a common cold (96 patients) or ILI (25 patients) during the analysis period. MG symptoms were aggravated in 10 (40%) patients after ILI, whereas only 2 (1.5%) experienced aggravation following influenza vaccination. The rate of symptom aggravation was significantly higher in patients experiencing an ILI (10/25, 40%) than in those with the common cold (15/96, 15.6%, p=0.006). CONCLUSIONS: The results of this study suggest that the potential risk of aggravating autoimmune disease is higher for ILI than for influenza vaccination, which further suggests that influenza vaccination can be offered to patients with MG.

Clinical Applications of Transcranial Direct Current Stimulation in Neurological Disorders

Transcranial direct current stimulation (tDCS) is non-invasive brain stimulation technique increasingly used for modulation of central nervous system excitability in humans. The use of non-invasive brain stimulation has significant advantages, such as not involving surgical procedures and having relatively mild adverse effects. In recent years there has been an exponential rise in the number of studies employing tDCS as a means of gaining an improvement on motor and cognitive function in patients with neurological diseases. In the present review, we will first introduce a brief background on the basic principles of tDCS. We also summarize recent studies with tDCS that aimed at enhancing behavioral outcome or disease-specific symptoms in patients suffering from stroke, movement disorders, Alzheimer disease, and epilepsy. Although outcomes of tDCS trials include some conflicting results, the evidence supports that tDCS might have a therapeutic value in different neurological conditions.

Periodic Limb Movements During Sleep Associated with Human T-Lymphotropic Virus Type I-Associated Myelopathy

Periodic limb movements during sleep (PLMS) are frequently observed in the general population, although such movements may be associated with a variety of medical and neurological disorders. Human T-lymphotropic virus type I-associated myelopathy (HAM) is a rare progressive disease in which abnormalities are rarely observed on spinal images. We present the case of a 55-year-old woman with PLMS who was later diag-nosed with HAM. The current case indicates that HAM can be considered a possible cause of PLMS.

Concurrent Demyelinating Polyneuropathy Associated with Recurrent Cervical Myelitis

No abstract available.

Sensory Polyneuropathy Associated with Pyridoxine Overdose

Pyridoxine deficiency and excess have both been implicated as causes of peripheral neuropathy. A 74-year-old man presented with paresthesia in both legs that first appeared 2 months previously. A nerve conduction study revealed axonal sensory polyneuropathy. He had consumed 100 milligrams of pyridoxine every day for 1 year, in the form of vitamin tablets. His blood levels of vitamin B6 were markedly elevated to above 250 nmol/L. This case indicates that the consumption of high-dose pyridoxine can cause sensory polyneuropathy.

Bilateral Adduction Palsy in a Patient with Myotonic Dystrophy Type 1

Myotonic dystrophy type 1 (DM1) is caused by CTG repeat expansion in the DMPK gene in chromosome 19q13.3. External ophthalmoplegia is a rare manifestation in DM1. We report a DM1 patient confirmed by the presence of 650 CTG triplet expansions in the DMPK gene and had limitation of adduction gaze bilaterally. Brain MRI showed bilateral medial rectus muscles atrophy. Our patient provides additional evidence of ocular motor muscle involvement in DM1.

Myasthenia Gravis Presenting with Facial Diplegia without Ocular Muscle Involvement / 대한임상신경생리학회지

No abstract available.

Diagnostic Sensitivity and Specificity of Residual Latency and Terminal Latency Index in the Diagnosis of Carpal Tunnel Syndrome

BACKGROUND: Conventional nerve conduction studies (NCS) are used in the diagnosis of carpal tunnel syndrome (CTS). The median terminal latency index (TLI) and median residual latency (RL) are parameters calculated to identify abnormalities in distal segments of the median motor nerve. The objective of this study was to determine the sensitivity and specificity of TLI and RL together with NCS in the diagnosis of CTS. METHODS: This prospective study involved 83 hands of 47 patients with suspected CTS. Conventional NCS were performed using Oh's method. Control data were obtained from the 68 hands of 40 healthy volunteers. The diagnostic sensitivity and specificity of TLI and RL were calculated and compared with those of conventional NCS. We divided the CTS patients into four groups based on their electrophysiological severity, and compared the TLI and RL values between these groups. RESULTS: TLI and RL were 0.20+/-0.03 (mean+/-SD) and 3.62+/-0.90, respectively, in the patients, while the corresponding values, in the healthy control, were 0.29+/-0.03 and 2.08+/-0.30. The sensitivities of TLI and RL in diagnosing CTS were 75.9% and 86.3%, respectively. Compared with median motor terminal latency, the sensitivities of TLI and RL in diagnosing CTS was found to be higher. Moreover, the diagnostic sensitivities of TLI and RL were significant better for the severe group than for the mild and moderate severity group. CONCLUSIONS: We conclude that measuring TLI and RL of the median nerve may increase the sensitivity in diagnosing CTS and also provide information about its electrophysiological severity.

Diagnostic Sensitivity and Specificity of Residual Latency and Terminal Latency Index in the Diagnosis of Carpal Tunnel Syndrome

BACKGROUND: Conventional nerve conduction studies (NCS) are used in the diagnosis of carpal tunnel syndrome (CTS). The median terminal latency index (TLI) and median residual latency (RL) are parameters calculated to identify abnormalities in distal segments of the median motor nerve. The objective of this study was to determine the sensitivity and specificity of TLI and RL together with NCS in the diagnosis of CTS. METHODS: This prospective study involved 83 hands of 47 patients with suspected CTS. Conventional NCS were performed using Oh's method. Control data were obtained from the 68 hands of 40 healthy volunteers. The diagnostic sensitivity and specificity of TLI and RL were calculated and compared with those of conventional NCS. We divided the CTS patients into four groups based on their electrophysiological severity, and compared the TLI and RL values between these groups. RESULTS: TLI and RL were 0.20+/-0.03 (mean+/-SD) and 3.62+/-0.90, respectively, in the patients, while the corresponding values, in the healthy control, were 0.29+/-0.03 and 2.08+/-0.30. The sensitivities of TLI and RL in diagnosing CTS were 75.9% and 86.3%, respectively. Compared with median motor terminal latency, the sensitivities of TLI and RL in diagnosing CTS was found to be higher. Moreover, the diagnostic sensitivities of TLI and RL were significant better for the severe group than for the mild and moderate severity group. CONCLUSIONS: We conclude that measuring TLI and RL of the median nerve may increase the sensitivity in diagnosing CTS and also provide information about its electrophysiological severity.

Arg460Cys Mutation in SPAST Gene in Patients with Hereditary Spastic Paraplegia

Hereditary spastic paraplegia is a group of genetically and clinically heterogeneous neurodegenerative disorder characterized by progressive lower extremity weakness and spasticity. A 49-year-old man presented with progressive gait disturbance. Neurological examination showed spasticity, hyperreflexia in lower extremity and bilateral ankle clonus. He had a family history consistent with autosomal dominant inheritance. Gene testing revealed a heterozygous missense mutation (c.1378C>T [p.Arg460Cys]) in SPAST gene. We report a first Korean family with Arg460Cys mutation in SPAST gene.

Takotsubo Cardiomyopathy Associated with Guillain-Barre Syndrome / 대한임상신경생리학회지

A 69-year-old woman presented with a progressive limb weakness. Both clinical and neurophysiological findings were consistent with diagnosis of Guillain-Barre syndrome (GBS). Two days after admission, the patient suffered from an acute coronary syndrome without stenosis at coronary arteriography. Echocardiography revealed left ventricular inferior wall and apical akinesia and decreased ejection fraction. A diagnosis of Takotsubo cardiomyopathy was then made. Left ventricular dysfunction and electrocardiography normalized within one month. Takotsubo cardiomyopathy can be developed as a complication of GBS.