Neurological Complications during Treatment of Middle East Respiratory Syndrome

BACKGROUND AND PURPOSE: Middle East respiratory syndrome (MERS) has a high mortality rate and pandemic potential. However, the neurological manifestations of MERS have rarely been reported since it first emerged in 2012. METHODS: We evaluated four patients with laboratory-confirmed MERS coronavirus (CoV) infections who showed neurological complications during MERS treatment. These 4 patients were from a cohort of 23 patients who were treated at a single designated hospital during the 2015 outbreak in the Republic of Korea. The clinical presentations, laboratory findings, and prognoses are described. RESULTS: Four of the 23 admitted MERS patients reported neurological symptoms during or after MERS-CoV treatment. The potential diagnoses in these four cases included Bickerstaff's encephalitis overlapping with Guillain-Barré syndrome, intensive-care-unit-acquired weakness, or other toxic or infectious neuropathies. Neurological complications did not appear concomitantly with respiratory symptoms, instead being delayed by 2–3 weeks. CONCLUSIONS: Neuromuscular complications are not rare during MERS treatment, and they may have previously been underdiagnosed. Understanding the neurological manifestations is important in an infectious disease such as MERS, because these symptoms are rarely evaluated thoroughly during treatment, and they may interfere with the prognosis or require treatment modification.

Euthyroid Graves' Ophthalmopathy with Negative Autoantibodies

Graves' ophthalmopathy occurs in 25-50% of patients with Graves' disease. Although patients with Graves' ophthalmopathy mostly present with hyperthyroidism, a minority of patients have euthyroid or hypothyroid characteristics, which may delay a correct diagnosis. Here, we report a case of euthyroid Graves' ophthalmopathy that was initially negative for thyroid autoantibodies, but later changed to positivity.

Antiplatelet Therapy for Secondary Stroke Prevention: 2012 Focused Update of Korean Clinical Practice Guidelines for Stroke / 대한뇌졸중학회지

Writing Committee of Korean clinical practice guidelines for secondary prevention of stroke has reviewed recent randomized controlled trials of cilostazol published after the first edition of Korean clinical practice guidelines that considered evidences published before June 2007. Two clinical trials and 1 meta-analysis which compared cilostazol directly with aspirin in the prevention of stroke in patients with cerebral infarction or transient ischemic attack (TIA) were identified and included for the current guideline update. Review of findings indicates that cilostazol as compared to aspirin achieved a greater reduction of stroke as well as composite vascular events of stroke, myocardial infarction, and vascular death. For safety, cilostazol was associated with fewer major bleeding events than aspirin. Accordingly, new recommendations for cilostazol are made for prevention of stroke in the setting of noncardioembolic stroke or TIA. Changes in the guidelines necessitated by new evidences will be continuously reflected in future guidelines.

Abdominal Wall Protrusion Following Herpes Zoster

No abstract available.

Prevalence of Risk Factors for Ischemic Stroke in Korean: A Systematic Review

BACKGROUND: This study examined the prevalence of the major risk factors of ischemic stroke in a Korean population. METHODS: Two investigators conducted an independent literature search of previously published reports on the prevalence of hypertension, diabetes, hypercholesterolemia, smoking, atrial fibrillation, obesity, ischemic heart disease, and history of stroke in Koreans. A study was considered eligible for inclusion if it was a population-based cross-sectional survey published between January 1996 and June 2007. RESULTS: The inclusion criteria were satisfied by 14 publications on hypertension, 7 on diabetes, 4 on hypercholesterolemia, 3 on smoking, 3 on obesity, 2 on atrial fibrillation, 3 on ischemic heart disease, and 3 on stroke. The prevalence of risk factors varied between studies, but it increased with age in most studies. Applying the estimates to the projected population in 2030 revealed a large increase in the prevalence of risk factors. CONCLUSIONS: Considering the rapid increase in the elderly population, in which major risk factors for ischemic stroke are prevalent, there is an urgent need to develop strategies for preventing this condition among Koreans.

Testosterone Relaxes Rabbit Seminal Vesicle by Calcium Channel Inhibition

Recent studies have documented that testosterone relaxes several smooth muscles by modulating K+channel activities. Smooth muscles of seminal vesicles play a fundamental role in ejaculation, which might involve testosterone. This study was aimed to assess the role of testosterone in seminal vesicular motility by studying its effects on contractile agents and on the ion channels of single vesicular myocytes in a rabbit model. The contractile responses of circular smooth muscle strips of rabbit seminal vesicles to norepinephrine (10 micrometer), a high concentration of KCl (70 mM), and testosterone (10 micrometer were observed. Single vesicular myocytes of rabbit were isolated using proteolytic enzymes including collagenase and papain. Inside-out, attached, and whole-cell configurations were examined using the patch clamp technique. The applications of 10 micrometernorepinephrine or 70 mM KCl induced tonic contractions, and 10 micrometertestosterone (pharmacological concentration) evoked dose-dependent relaxations of these precontracted strips. Various K+channel blockers, such as tetraethylammonium (TEA; 10 mM), iberiotoxin (0.1 micrometer), 4-aminopyridine (4-AP, 10 micrometer, or glibenclamide (10 micrometer rarely affected these relaxations. Single channel data (of inside-out and attached configurations) of BK channel activity were also hardly affected by testosterone (10 micrometer). On the other hand, however, testosterone reduced L-type Ca2+currents significantly, and found to induce acute relaxation of seminal vesicular smooth muscle and this was mediated, at least in part, by Ca2+current inhibition in rabbit.

Heat shock protein 70 alters the endosome-lysosomal localization of huntingtin

Huntington's disease is caused by CAG trinucleotide expansions in the gene encoding huntingtin. N- terminal fragments of huntingtin with polyglutamine produce aggregates in the endosome-lysosomal system, where proteolytic fragments of huntingtin is generated. Heat shock protein 70 (HSP70) prevents the formation of protein aggregates, but the effect of HSP70 on the huntingtin in the endosome-lysosomal system is unknown. This study was to determine whether HSP70 alters the distribution of huntingtin in endosome-lysosomal system. HSP70 expressing stable cells (NIH/3T3 or cerebral hybrid cell line A1) were generated, and mutant [(CAG)100] huntingtin was transiently overexpressed. Analysis of subcellular distribution by immnuocytochemistry or proteolysis cleavage by Western blotting was performed. 18 CAG repeat wild type [WT; (CAG)18] huntingtin was used as a control. Cells with huntingtin showed patterns of endosome- lysosomal accumulation, from a 'dispersed vacuole (DV)' type into a coalescent 'perinuclear vacuole (PV)' type over time. In WT huntingtin, HSP70 increased the cells with the PV types that enhanced the proteolytic cleavage of huntingtin. However, HSP70 reduced cells of the DV and PV types expressing mutant huntingtin, that result in less proteolysis than that of control. In addition, intranuclear inclusions were formed only in mutant cells, which was not affected by HSP70. These results suggest that HSP70 alters the distribution of huntingtin in the endosome-lysosomal system, and that this contributes to huntingtin proteolysis.

A Case of Primary CNS Lymphoma Presenting as a Huge Intraventricular Mass

No abstract available.

Vasculitic Neuropathy in Ulcerative Colitis

No abstract available.

Contingent Negative Variation of Pre- and Post-Hemodialysis in Patient with End Stage Renal Disease

BACKGROUND: The contingent negative variation (CNV) reflects neuronal activities related to sensorimotor integration and motor planning or execution and is probably originated from the frontal-subcortical circuit. The aim of this study is to investigate the neurophysiologic changes in uremia and the effect of hemodialysis to them by utilizing the CNV test. METHODS: Fifteen right-handed healthy subjects and 12 patients with end stage renal disease (ESRD) were studied. CNV was recorded from Fz, Cz, and Pz referenced to linked ear lobes by using an S1 (click)-S2 (flashes)-key press paradigm. The amplitude of initial CNV (iCNV) was calculated as the average amplitude of 550~750 msec after S1. The amplitude of late CNV (lCNV) was calculated as the average amplitude between 200 msec before S1 and S2. The test was repeated for the patients group at the time of pre- and post-hemodialysis. Neuropsychological measurements, the trail making test (TMT) and mini-mental state score (MMSE), were conducted at the time of each test. RESULTS: Both the mean amplitudes of iCNV and lCNV at the vertex (Cz) were significantly lower in the patient group than those in the control group (p<0.05). The MMSE score and TMT were also significantly different between the patient and control group (p<0.05). There was no significant correlation between the values of neuropsychological tests and the parameters of CNV. Both iCNV and lCNV were not significantly different between the pre- and post-dialysis test. CONCLUSIONS: It appears that CNV negativity in uremia reflects dysfunctions in the frontal-subcortical circuit. In addition, hemodialysis seems to have no significant effect on it in patients with ESRD.

Conduction Slowing in Painful versus Painless Diabetic Neuropathy

BACKGROUND: Motor conduction slowing in diabetic distal symmetrical polyneuropathy (DSP) generally exceeds that in distal axonal polyneuropathy. Additional mechanisms secondary to axonal injury may contribute towards this slowing. However, clinical and pathophysiological significances of motor conduction slowing have been rarely discussed. The purpose of this study is to evaluate the clinical and pathophysiological significance of conduction slowing in DSP. METHODS: We analyzed motor conduction studies of 39 patients with symptomatic painful DSP and 24 patients with asymptomatic painless DSP. Motor conduction studies of 39 patients with amyotrophic lateral sclerosis (ALS) were used as controls for the amplitude-dependent slowing of conduction. Percentages of normal limits were calculated for the compound muscle action potential amplitude (CMAP), distal motor latency (DL), and conduction velocity (CV), and converted to a square root (SQRT) form. The changes of SQRT-DL or SQRT-CV according to SQRT-CMAP changes were plotted and analyzed. RESULTS: Regression analysis showed that DL and CV were amplitude-dependent in both painless DSP and ALS. The changes of DL and CV in painful DSP did not show amplitude-dependency except DL in the lower extremities. CONCLUSIONS: This data supports the hypothesis that the mechanism of slowing is similar in both painless DSP and ALS and results from the loss of large, fast-conducting fibers. Lack of amplitude-dependency of conduction slowing in painful DSP may reflect the combined axonal and demyelinating changes, possibly due to inflammation.

A Case of Vocal Cord Palsy Following Disulfiram Intoxication

A 49 year-old woman, who took a high dose of disulfiram for the purpose of suicide, presented with severe hoarseness, quadriparesis, and sensory loss of distal limbs. Laryngeal electromyography showed ample denervation potentials. Nerve conduction study was consistent with severe sensorimotor axonal polyneuropathy. She was diagnosed with an acute palsy of recurrent laryngeal nerve and superimposed severe acute sensorimotor axonal polyneuropathy due to high dose disulfiram intoxication.

A Case of Oculopharyngeal Muscular Dystrophy Due to a Novel Mutation of the PABPN1 Gene

We recently encountered a Korean patient with oculopharyngeal muscular dystrophy (OPMD). His major clinical manifestations were late onset bilateral ptosis, dysarthria, and dysphagia. Direct sequencing analysis of the PABPN1 gene demonstrated a heterozygous insertion of 9 bp sequence [(GCG)(GCA)(GCA); c.28insGCGGCA GCA], resulting in an in-frame insertion of 3 alanines (p. A10insAAA). To our knowledge, this is the first report of a genetically confirmed case of OPMD in Korea.

Ataxic Variant of Guillain-Barre Syndrome Associated with Anti-GD1b Ig G Antibody

It is known that anti GD1b antibody bind to the cerebellar granular layer or spinocerebellar Ia fiber. We recently encountered a patient of Guillain Barr syndrome (GBS) showing marked cerebellar ataxia and relatively mild quadriparesis but completely intact extraocular eye movement. Markedly high IgG anti GD1b antibody titer was identified from the patient's serum. The nerve conduction study showed reduction of compound muscle action potential without evidence of perpheral nerve demyelination. We report an ataxic variat of GBS associated with anti GD1b IgG antibody.

Therapeutic Effect of Different Tables in Spinal Type of Decompression Sickness

BACKGROUND: Recompression therapy is well established in the treatment of decompression sickness (DCS) including spinal type. However it was not confirm whether each United State Navy (USN) treatment tables had different therapeutic effect in spinal type of DCS. The purpose of this study is to find if the use of different tables results in a different outcome in spinal type of DCS. And we want to reconfirm an unsatisfactory cure rate of USN table 6. METHODS: We applied different USN treatment tables, 6, 6a and 6a1, to spinal type of DCS for 10 years. 68 patients with spinal type of DCS were treated with recompression therapy, 16 patients by table 6, 31 by 6a, and 21 by 6a1. We reviewed the outcome of motor power before and after each treatment. And we investigated age, diving experience, depth of dive, bottom time of dive, and onset of treatment before the treatment in each treatment group. RESULTS: There were no significant differences between each treatment group in age, diving experience, depth of dive, bottom time of dive, onset of treatment and the grade of motor power before the treatment. Marked motor power improvements were noticed in 59.6% (table 6; 18.7%, 6a; 74.2%, and 6a1; 81%). There were significant differences of motor power improvement between groups 6 and 6a (p=0.0057), 6 and 6a1 (p=0.0053) and 6a and 6a1 (p=0.0139). CONCLUSIONS: We think that table 6a1 should be used in spinal type of DCS. And table 6 is not useful in spinal type DCS.

Decompression Sickness with Cortical Blindness Responsive to Hyperbaric Oxygen Therapy

SCUBA diving has become increasingly popular, but it is a sport with high risks. It has been widely accepted that the neurologic sequelae of decompression illness (DCI) are confined to the spinal cord. However, cortical blindness is very rare in the central nervous system DCI. We report a case of DCI who showed cortical blindness and MRI abnormalities. The cortical blindness improved after serial hyperbaric oxygenation therapy with the USN table 6A protocol. (J Korean Neurol Assoc 19(4):404~406, 2001)

Clinical Characteristics of the Subtypes of Guillain-Barre Syndrome according to the Electrodiagnositic Criteria

BACKGROUND: Guillain-Barre syndrome (GBS) is defined as a recognizable clinical entity that is characterized by rapidly evolving symmetric limb weakness, loss of tendon reflexes, absent or mild sensory signs, and variable autonomic dysfunctions. Recently, GBS has been classified as a classical demyelinating (acute imflammatory demyelinating polyradiculoneuropathy, AIDP) and two axonal (acute motor axonal neuropathy, AMAN, and acute motor sensory axonal neuropathy, AMSAN) forms. The clinical pattern and prognosis according to type is not clear. METHODS: Forty-one patients clinically diagnosed as GBS were enrolled and classified as AIDP, AMAN, and AMSAN according to electrodiagnostic criteria. We analyzed the clinical data of each subgroup; age, sex, seasonal distribution, history of previous illness, cranial nerve involvement, respiratory involvement, and motor weakness. RESULTS: Forty-one patients with GBS were comprised of 19 patients (46.3%) with AIDP, 12 patients (29.2%) with AMAN, and 10 patients (24.3%) with AMSAN. AIDP was found more frequently in males and in winter (42.1%) while axonal forms of GBS showed neither gender nor seasonal predominance. Frequency of cranial nerve involvement was not different between the sub-groups of GBS, whereas respiratory involvement was more frequent in AMSAN (50%). Upper limbs were weaker in axonal than in demyelinating types of GBS. CONCLUSIONS: Axonal forms of GBS showed some clinical characteristics distinctive from the demyelinating forms, which might be useful in the differential diagnosis of subgroups of GBS. (J Korean Neurol Assoc 19(5):503~508, 2001)

The accessory deep peroneal nerve : frequency and electrophysiological findings

The accessory deep peroneal(ADP) nerve is known as a common anatomical variant. It may alter the usual clinical and electrophysiological charateristics of peroneal nerve lesions. The purpose of the study was to investigate the frequency of occurrence and the electrophysiologic characteristics of the ADP nerve. We performed peroneal motor nerve conduction studies in 434 patiets with conventional method. When the CMAP amplitudes evoked by distal peroneal stimulation is smaller than that by proximal stimulation, we searched ADP nerve by stimulation at posterior to the lateral malleolus. In 60 patients, we searched ADP nerve regardless of CMAP amplitude difference. Additionally, we routinely stimulated the region of posterior to the lateral malleolus with recording at the medial and lateral extensor digitorum brevis(EDB) muscles using multi channel EMG in 34 patients. In conventional peroneal nerve conduction study, ADP nerves were detected in 27(8.2%) patients out of 330 studied patients(right 7, left 15, both 5). Mean amplitue of ADP nerve was 1. 52mV (right 1. 90, left 1. 84). In routine ADP stimulation study, ADP nerve was detected in 5(21.7%) out of 23 patients(left 2, both 3). In 4 of them, the distal peroneal amplitudes were greater than the proximal. Mean amplitude was 1.86mV(right 1.38, left 1.65). In conclusion, we confirmed that the accessary deep peroneal nerve is a relatively common variant and its presence may not be predicted by the difference of amplitudes between the distal and proximal peroneal segments in conventional peroneal nerve conduction study. So in cases of suspected peroneal nerve lesions, ADP nerve should be searched.