Guillain-Barré Syndrome Associated with Shiga Toxin-Producing and Enterotoxigenic Escherichia coli Infection

GuillainBarré syndrome (GBS) is the inflammatory neuropathy that affects the myelin and nodal or paranodal areas of peripheral nerves. Immunoglobulin G GM1 antibody is well known as the cause of GBS associated with Campylobacter jejuni infection. However, the relationship between other specific infectious agents and autoantibodies is not yet well elucidated in patients with GBS. Recently we have experienced a case with GBS associated with antiGM1 and phosphatidic acid complex antibody that occurred after Shiga toxinproducing and enterotoxigenic Escherichia coli enteritis.

Anti-Myelin Oligodendrocyte Glycoprotein Syndrome with Findings Resembling “Snake-Eye Appearance”: a Case Report

Anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndrome is an immunemediated inflammatory condition of the central nervous system, which usually involves spinal cord and optic nerves. Herein, we studied the case of a 57-yearold female patient who presented with acute/subacute symptoms of sphincter dysfunction, paraparesis, and ocular pain. The patient was diagnosed with anti-MOG syndrome with findings resembling snake-eye appearance (SEA), characterized by nearly symmetrical round high signal intensity lesions located at anterior horns (gray matter) on T2-weighted image.

Anti-Myelin Oligodendrocyte Glycoprotein Syndrome with Findings Resembling “Snake-Eye Appearance”: a Case Report

Anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndrome is an immunemediated inflammatory condition of the central nervous system, which usually involves spinal cord and optic nerves. Herein, we studied the case of a 57-yearold female patient who presented with acute/subacute symptoms of sphincter dysfunction, paraparesis, and ocular pain. The patient was diagnosed with anti-MOG syndrome with findings resembling snake-eye appearance (SEA), characterized by nearly symmetrical round high signal intensity lesions located at anterior horns (gray matter) on T2-weighted image.

Pivotal Role of Subcortical Structures as a Network Hub in Focal Epilepsy: Evidence from Graph Theoretical Analysis Based on Diffusion-Tensor Imaging

BACKGROUND AND PURPOSE: There is accumulating evidence that epilepsy is caused by network dysfunction. We evaluated the hub reorganization of subcortical structures in patients with focal epilepsy using graph theoretical analysis based on diffusion-tensor imaging (DTI). In addition, we investigated differences in the values of diffusion tensors and scalars, fractional anisotropy (FA), and mean diffusivity (MD) of subcortical structures between patients with focal epilepsy and healthy subjects. METHODS: One hundred patients with focal epilepsy and normal magnetic resonance imaging (MRI) findings and 80 age- and sex-matched healthy subjects were recruited prospectively. All subjects underwent DTI to obtain data suitable for graph theoretical analysis. We investigated the differences in the node strength, cluster coefficient, eigenvector centrality, page-rank centrality measures, FA, and MD of subcortical structures between patients with epilepsy and healthy subjects. RESULTS: After performing multiple corrections, the cluster coefficient and the eigenvector centrality of the globus pallidus were higher in patients with epilepsy than in healthy subjects (p=0.006 and p=0.008, respectively). In addition, the strength and the page-rank centrality of the globus pallidus tended to be higher in patients with epilepsy than in healthy subjects (p=0.092 and p=0.032, respectively). The cluster coefficient of the putamen was lower in patients with epilepsy than in healthy subjects (p=0.004). The FA values of the caudate nucleus and thalamus were significantly lower in patients with epilepsy than in healthy subjects (p=0.009 and p=0.007, respectively), whereas the MD value of the thalamus was higher than that in healthy subjects (p=0.005). CONCLUSIONS: We discovered the presence of hub reorganization of subcortical structures in focal epilepsy patients with normal MRI findings, suggesting that subcortical structures play a pivotal role as a hub in the epilepsy network. These findings further reinforce the idea that epilepsy is a network disease.

Anti-myelin oligodendrocyte glycoprotein antibody associated neuromyelitis optica spectrum disorder presenting homonymous hemianopia

@#Anti-aquaporin 4 antibody is the most common cause of neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oliogodendrocyte glycoprotein (MOG) antibody recently has emerged as another cause of NMOSD. Visual field defect can be observed as a manifestation of optic neuritis in patients with multiple sclerosis and NMOSD. However, homonymous hemianopia associated with a visual cortex lesion has been rarely reported in patients with multiple sclerosis and anti-aquaporin 4-positive NMOSD. Recently, we experienced a case of MOG-positive NMOSD who presented with a homonymous hemianopia associated with a visual cortex lesion

Autoimmune Encephalitis Associated with Thymoma

No abstract available.

Ictal Epileptic Headache in an Elderly Patient with a Hippocampal Tumor

No abstract available.

Questioning the Diagnosis of Recurrent Optic Neuritis Associated with Neuro-Behçet's Disease

No abstract available.

Bilateral Optic Neuritis Mimicking Bilateral Optic Perineuritis

Optic perineuritis (OPN) is a rare form of orbital inflammatory disease of optic nerve sheath. The clinical presentation of OPN are known as distinct from demyelinating optic neuritis (ON). Recently, we have experienced a patient with bilateral idiopathic ON who had initial magnetic resonance imaging (MRI) feature of OPN. Serial MRI and funduscopic examination exhibited the bilateral ON findings. Serial MRI and funduscopic examination are needed in diagnosing OPN to differentiate to the temporal change of ON.

Vasculitis Findings for the Anterior Choroidal Artery in 3D-Time-of-Flight Magnetic Resonance Angiography

No abstract available.

Neurosyphilis Exhibiting Similar MRI Findings of Multiple Sclerosis

No abstract available.

Procalcitonin as a Diagnostic and Prognostic Factor for Tuberculosis Meningitis

BACKGROUND AND PURPOSE: We investigated the potential role of serum procalcitonin in differentiating tuberculosis meningitis from bacterial and viral meningitis, and in predicting the prognosis of tuberculosis meningitis. METHODS: This was a retrospective study of 26 patients with tuberculosis meningitis. In addition, 70 patients with bacterial meningitis and 49 patients with viral meningitis were included as the disease control groups for comparison. The serum procalcitonin level was measured in all patients at admission. Differences in demographic and laboratory data, including the procalcitonin level, were analyzed among the three groups. In addition, we analyzed the predictive factors for a prognosis of tuberculosis meningitis using the Glasgow Coma Scale (GCS) at discharge, and the correlation between the level of procalcitonin and the GCS score at discharge. RESULTS: Multiple logistic regression analysis showed that a low level of procalcitonin (≤1.27 ng/mL) independently distinguished tuberculosis meningitis from bacterial meningitis. The sensitivity and specificity for distinguishing tuberculosis meningitis from bacterial meningitis were 96.2% and 62.9%, respectively. However, the level of procalcitonin in patients with tuberculosis meningitis did not differ significantly from that in patients with viral meningitis. In patients with tuberculosis meningitis, a high level of procalcitonin (>0.4 ng/mL) was a predictor of a poor prognosis, and the level of procalcitonin was negatively correlated with the GCS score at discharge (r=-0.437, p=0.026). CONCLUSIONS: We found that serum procalcitonin is a useful marker for differentiating tuberculosis meningitis from bacterial meningitis and is also valuable for predicting the prognosis of tuberculosis meningitis.

Early Electrodiagnostic Features of Upper Extremity Sensory Nerves Can Differentiate Axonal Guillain-Barré Syndrome from Acute Inflammatory Demyelinating Polyneuropathy

BACKGROUND AND PURPOSE: Serial nerve conduction studies (NCSs) are recommended for differentiating axonal and demyelinating Guillain-Barré syndrome (GBS), but this approach is not suitable for early diagnoses. This study was designed to identify possible NCS parameters for differentiating GBS subtypes. METHODS: We retrospectively reviewed the medical records of 70 patients with GBS who underwent NCS within 10 days of symptom onset. Patients with axonal GBS and acute inflammatory demyelinating polyneuropathy (AIDP) were selected based on clinical characteristics and serial NCSs. An antiganglioside antibody study was used to increase the diagnostic certainty. RESULTS: The amplitudes of median and ulnar nerve sensory nerve action potentials (SNAPs) were significantly smaller in the AIDP group than in the axonal-GBS group. Classification and regression-tree analysis revealed that the distal ulnar sensory nerve SNAP amplitude was the best predictor of axonal GBS. CONCLUSIONS: Early upper extremity sensory NCS findings are helpful in differentiating axonal-GBS patients with antiganglioside antibodies from AIDP patients.

Has Snoring Significance for Predicting Obstructive Sleep Apnea Severity? / 대한임상신경생리학회지

BACKGROUND: The aim of this study was to investigate the predictive value of snoring frequency in the diagnosis and severity of obstructive sleep apnea. METHODS: Patients who underwent polysomnography with one or more of the following characteristics were included: 1) sleepiness, non-restorative sleep, fatigue, or insomnia symptoms; 2) arousal due to cessation of breathing or the occurrence of gasping or choking when waking up; and 3) habitual snoring, breathing interruptions, or both, noted by a bed partner or other observer. We analyzed the differences in clinical and polysomnographic variables between patients with and without obstructive sleep apnea and investigated the associations of those variables with obstructive sleep apnea severity. RESULTS: One hundred ninety-three patients met the inclusion criteria, and 145 of the 193 patients were diagnosed with obstructive sleep apnea. Multiple logistic regression analysis showed that large neck circumference (p = 0.0054) and high snoring index (p = 0.0119) were independent predictors for obstructive sleep apnea. Moreover, between the obstructive sleep apnea severity groups, there was a strong tendency of difference in body mass index (p = 0.0441) and neck circumference (p = 0.0846). However, there was no significant difference in snoring frequency according to obstructive sleep apnea severity (p = 0.4914). CONCLUSIONS: We confirmed that snoring frequency is a predictor of obstructive sleep apnea. In addition, we showed for the first time that snoring frequency is not associated with obstructive sleep apnea severity, thus it is not a valuable marker for predicting obstructive sleep apnea severity.

Hashimoto's Encephalopathy Presenting with Ocular Flutter

Hashimoto's encephalopathy is a neurological disorder associated with high titers of antithyroid antibodies. The common neurologic symptoms are seizure, stroke-like episode, or mental change. However other atypical presentations, such as aphasia, myoclonus, ataxia, and cognitive dysfunction have also been described. We report hear a 44-year-old woman with Hashimoto's encephalopathy. The patient presented with ocular flutter as a principal manifestation, which is rare in Hashimoto's encephalopathy.

Miller-Fisher Syndrome with Blink Reflex Abnormality

No abstract available.

Predictors for Presence and Severity of Obstructive Sleep Apnea in Snoring Patients: Significance of Neck Circumference

OBJECTIVES: Documented risk factors for obstructive sleep apnea include advanced age, male gender, hypertension, large neck circumference, and obesity; but some controversy remains regarding the risk factors, especially in Asians. In this study, we evaluated the risk factors for obstructive sleep apnea in snoring patients, and also analyzed the risk factors that could predict the severity of obstructive sleep apnea. METHODS: The inclusion criteria were patients 1) who visited our hospital with a chief complaint of snoring as witnessed by a sleep partner and 2) who underwent overnight polysomnography. The primary endpoint was the presence of obstructive sleep apnea as a dependent variable. RESULTS: One hundred forty-seven patients met the inclusion criteria. Of the 147 patients, 109 patients were diagnosed with obstructive sleep apnea. Multivariate analysis showed that old age and large neck circumference were significant independent variables for predicting the presence of obstructive sleep apnea, whereas hypertension and large neck circumference were independent variables for predicting the severity of obstructive sleep apnea. CONCLUSIONS: We demonstrated that neck circumference can be used to predict the presence as well as the severity of obstructive sleep apnea in snoring Asian patients.

Comparison of the Demographic and Laboratory Profiles of Patients with Aseptic Meningitis and Encephalitis: Significance of Age and C-reactive Protein / 대한임상신경생리학회지

BACKGROUND: Viruses can cause either meningitis or encephalitis. It is unclear why some people suffer from aseptic meningitis, and others acquire aseptic encephalitis when infected with the same viral pathogens. The aim of this study was to compare demographic and laboratory factors between patients with aseptic meningitis and encephalitis. METHODS: The demographic and laboratory differences were analyzed according to age, sex, diabetes, hypertension, C-reactive protein in the blood, white blood cell and protein in the cerebrospinal fluid, and glucose ratio (cerebrospinal fluid/blood). Additionally, we analyzed the nation-wide differencesin age between the patients with aseptic meningitis and those with encephalitis in Korea. RESULTS: The patients with aseptic encephalitis were older, more likely to have hypertension, and had higher levels of C-reactive protein than did the patients with aseptic meningitis. However, the numbers of white blood cells in the cerebrospinal fluid were significantly higher in the patients with meningitis than in the patients with encephalitis. Multivariable analysis revealed that age >49 years, hypertension and a C-reactive protein level >5.81 mg/dL were independent and significant variables in the prediction of aseptic encephalitis. Additionally, the patients with aseptic encephalitis were older than those with aseptic meningitis in the nation-wide Korean database. CONCLUSIONS: Older age, hypertension, and higher levels of C-reactive protein are useful factors for the prediction of aseptic encephalitis.

Diabetes mellitus as a predictor for late recovery of vestibular neuritis

The time course of recovery in vestibular neuritis varies between individuals. The aim of this study was to identify the predictors for the early or late recovery of vestibular neuritis. The inclusion criteria were patients 1) who had an acute onset of vertigo lasting at least 24 hours, 2) with a horizontal-torsional unidirectional spontaneous nystagmus, and 3) with a canal paresis of 20% or more on the bithermal caloric tests. The primary endpoint for this study was an early or late recovery of vestibular neuritis as a dependent variable. A functional level scale was used to define the late recovery (5 or more points) at seven days after the symptom onset. The secondary endpoint was the duration of hospitalization. One hundred twenty eight patients met the inclusion criteria for this study, and among them, 71 patients had an early recovery. Multiple logistic regression analysis showed that diabetes mellitus was the only independent significant variable for the prediction of a late recovery of vestibular neuritis. In addition, the diabetes mellitus was a predicting variable for long duration of hospitalization. Diabetes mellitus was a predictor for a late recovery of vestibular neuritis.

A novel mutation of CACNA1A gene in episodic ataxia type 2 family in Korea

Episodic ataxia type 2 (EA-2) is a rare disorder presenting with paroxysmal vertigo and cerebellar dysfunction. EA-2 is known to be caused by mutations of the CACNA1A gene on chromosome 19q13. We examined a family of EA-2 with a novel mutation of the CACNA1A gene showing characteristic ocular symptoms. A-36-year woman visited our hospital with paroxysmal vertigo. When she experienced vertigo attack, she also suffered from gait disturbance, dysarthria, and ataxia. She complained that she could not ride in a car or a train that moved fast, because she could not visually follow the moving objects. Her mother, grandmother, and uncle also complained of similar symptoms. Video nystagmographic findings showed loss of optokinetic nystagmus. We found a novel missense mutation, R279C (c.835C>T), on exon 6 in the CACNAIA gene. This is the first report of a family with new mutation of EA-2 in Korea.