A Case of Severe Encephalitis with Mycoplasma pneumoniae Infection in a 4-Year-Old Boy / 대한소아신경학회지

Mycoplasma pneumoniae (MP) infection can result in extrapulmonary and respiratory manifestations. The direct invasion by MP and the indirect invasion by immune-mediated response have been suggested as the pathogenesis of extrapulmonary manifestations. Neurologic manifestations are the most common among the extrapulmonary manifestations associated with MP infection. We report the case of a 4-year-old previously healthy boy with encephalitis accompanied by MP pneumonia. The patient's respiratory manifestations appeared 14 days before the neurological manifestations. Leukocytosis was observed in the patient's cerebrospinal fluid, but the result of the MP polymerase chain reaction was negative. The magnetic resonance imaging of the patient's brain showed high signal intensity at bilateral basal ganglia. The chest radiograph confirmed the presence of lobar pneumonia. The serological test on MP-specific immunoglobulin M titer revealed a positive result. The clinical course improved with the administration of immunomodulatory therapies, but the patient subsequently developed spastic quadriplegic cerebral palsy. MP is a common pathogen in children and may induce aggravating neurologic diseases. Thus, MP should be considered a causative agent of encephalitis in children. Immunomodulatory drugs are the recommended therapeutic option for severe MP encephalitis.

An Outbreak of Mumps Meningitis Among Adolescents / 대한소아신경학회지

PURPOSE: We report sharp rise of incidence of mumps meningitis at our institute and describe the clinical manifestations, laboratory findings, and complications. METHODS: This is a retrospective study involving 34 patients with mumps meningitis at Eulji University Hospital from July 2012 to June 2014. They were diagnosed by definite parotid gland swelling and leukocytosis in cerebrospinal fluid (CSF) study. RESULTS: Nineteen (55.9%) of the 34 patients with mumps meningitis were confirmed as positive by the mumps virus-specific immunoglobulin M (IgM) antibody in serum or CSF and the remaining 15 patients (44.1%) were classified as probable cases. We identified 31 boys and 3 girls clearly showing male predominance. The median age of the patients was 15.8 years and all patients were in the middle school and high school age group. Sixteen patients (47.1%) had unilateral mumps and 29 patients (85.3%) were diagnosed with meningitis after parotid swelling. Six patients (17.6%) were accompanied by testicular involvement and one (2.9%) showed hearing loss. The positivity of mumps virus specific IgM was 17 of 34 (50.0%) in serum and 4 of 34 (11.8%) in CSF. The total duration of illness including hospitalization was 5 to 21 days, median being 11 days. There was no serious neurologic deficit but 5 of 34 (14.7%) complained dizziness after discharge. It caused delay in back-to-work. CONCLUSION: It is worthy to notice that recent outbreak of mumps among adolescents are more complicated. We need to be more aggressive about prevention of mumps outbreak which bring on substantial socioeconomic loss.

Clinical outcome of patients with refractory Kawasaki disease based on treatment modalities / 소아과

PURPOSE: Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. METHODS: We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. RESULTS: A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. CONCLUSION: No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients.

Clinical Significance of Repetitive Compound Muscle Action Potentials in Patients with Myasthenia Gravis: A Predictor for Cholinergic Side Effects of Acetylcholinesterase Inhibitors

BACKGROUND AND PURPOSE: Acetylcholinesterase inhibitors (AChEIs) are widely used to treat myasthenia gravis (MG). Although AChEIs are usually tolerated well, some MG patients suffer from side effects. Furthermore, a small proportion of MG patients show cholinergic hypersensitivity and cannot tolerate AChEIs. Because repetitive compound muscle action potentials (R-CMAPs) are an electrophysiologic feature of cholinergic neuromuscular hyperactivity, we investigated the clinical characteristics of MG patients with R-CMAPs to identify their clinical usefulness in therapeutic decision-making. METHODS: We retrospectively reviewed the clinical records and electrodiagnostic findings of MG patients who underwent electrodiagnostic studies and diagnostic neostigmine testing (NT). RESULTS: Among 71 MG patients, 9 could not tolerate oral pyridostigmine bromide (PB) and 17 experienced side effects of PB. R-CMAPs developed in 24 patients after NT. The highest daily dose of PB was lower in the patients with R-CMAPs (240 mg/day vs. 480 mg/day, p<0.001). The frequencies of PB intolerance and side effects were higher in the patients with R-CMAPs than in those without R-CMAPs [37.5% vs. 0% (p<0.001) and 45.8% vs. 12.8% (p=0.002), respectively]. The MG Foundation of America postintervention status did not differ significantly between MG patients with and without R-CMAPs, and the response to immunotherapy was also good in both groups. CONCLUSIONS: Side effects of and intolerance to AChEIs are more common in MG patients with R-CMAPs than in those without R-CMAPs. AChEIs should be used carefully in MG patients with R-CMAPs. The presence of R-CMAPs after NT may be a good indicator of the risks of PB side effects and intolerance.

Whole-Body Muscle MRI in Patients with Hyperkalemic Periodic Paralysis Carrying the SCN4A Mutation T704M: Evidence for Chronic Progressive Myopathy with Selective Muscle Involvement

BACKGROUND AND PURPOSE: Hyperkalemic periodic paralysis (hyperKPP) is a muscle sodium-ion channelopathy characterized by recurrent paralytic attacks. A proportion of affected individuals develop fixed or chronic progressive weakness that results in significant disability. However, little is known about the pathology of hyperKPP-induced fixed weakness, including the pattern of muscle involvement. The aim of this study was to characterize the patterns of muscle involvement in hyperKPP by whole-body magnetic resonance imaging (MRI). METHODS: We performed whole-body muscle MRI in seven hyperKPP patients carrying the T704M mutation in the SCN4A skeletal sodium-channel gene. Muscle fat infiltration, suggestive of chronic progressive myopathy, was analyzed qualitatively using a grading system and was quantified by the two-point Dixon technique. RESULTS: Whole-body muscle MRI analysis revealed muscle atrophy and fatty infiltration in hyperKPP patients, especially in older individuals. Muscle involvement followed a selective pattern, primarily affecting the posterior compartment of the lower leg and anterior thigh muscles. The muscle fat fraction increased with patient age in the anterior thigh (r=0.669, p=0.009), in the deep posterior compartment of the lower leg (r=0.617, p=0.019), and in the superficial posterior compartment of the lower leg (r=0.777, p=0.001). CONCLUSIONS: Our whole-body muscle MRI findings provide evidence for chronic progressive myopathy in hyperKPP patients. The reported data suggest that a selective pattern of muscle involvement-affecting the posterior compartment of the lower leg and the anterior thigh-is characteristic of chronic progressive myopathy in hyperKPP.

Effects of the C3G/D3G anthocyanins-rich black soybean testa extracts on improvement of lipid profiles in STZ-induced diabetic rats / 한국영양학회지

PURPOSE: The purpose of this study was to examine the effect of black soybean (CJ-3) testa extracts on lipid profiles in streptozotocin (STZ)-induced diabetic rats. METHODS: One control group and four STZ-induced diabetic groups with different doses of black soybean (CJ-3) testa extracts treatment [0 mg/kg (diabetic control, EX), 250 mg/kg (EX-250), 500 mg/kg (EX-500), 1,000 mg/kg (EX-1000)] were orally administered for 4 weeks. RESULTS: All CJ-3 treatment groups had remarkably lower serum triglyceride (TG) levels than that of EX group (p < 0.05) whereas hepatic TG contents did not show any differences. Results from serum total cholesterol (TC) concentrations of EX-250 and EX-1000 groups were decreased compared to EX group (p < 0.05). Furthermore, protein levels of 3-hydroxy-3-methyl-glutaryl-Coenzyme A (HMG-CoA) reductase from the liver decreased in all treatment groups (p < 0.05). However, significant differences were not observed in serum glucose and insulin, and glucose transporter 4 (GLUT-4) protein expression in skeletal muscle tissue. CONCLUSION: These results suggest that black soybean testa extracts could be useful for improvement of hyperlipidemia and hypercholesteremia in diabetes.

Clinical and Electrophysiologic Responses to Acetylcholinesterase Inhibitors in MuSK-Antibody-Positive Myasthenia Gravis: Evidence for Cholinergic Neuromuscular Hyperactivity

BACKGROUND AND PURPOSE: Patients with muscle-specific tyrosine kinase (MuSK) antibody (MuSK-Ab)-positive myasthenia gravis (MG) show distinct responses to acetylcholinesterase inhibitors (AChEIs). Although clinical responses to AChEIs in MuSK-Ab MG are reasonably well known, little is known about the electrophysiologic responses to AChEIs. We therefore investigated the clinical and electrophysiologic responses to AChEIs in MuSK-Ab-positive MG patients. METHODS: We retrospectively reviewed the medical records and electrodiagnostic findings of 17 MG patients (10 MuSK-Ab-positive and 7 MuSK-Ab-negative patients) who underwent electrodiagnostic testing before and after a neostigmine test (NT). RESULTS: The frequency of intolerance to pyridostigmine bromide (PB) was higher in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (50% vs. 0%, respectively; p=0.044), while the maximum tolerable dose of PB was lower in the former (90 mg/day vs. 480 mg/day, p=0.023). The frequency of positive NT results was significantly lower in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (40% vs. 100%, p=0.035), while the nicotinic side effects of neostigmine were more frequent in the former (80% vs. 14.3%, p=0.015). Repetitive compound muscle action potentials (R-CMAPs) developed more frequently after NT in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (90% vs. 14.3%, p=0.004). The frequency of a high-frequency-stimulation-induced decrement-increment pattern (DIP) was higher in MuSK-Ab-positive patients than in MuSK-Ab-negative patients (100% vs. 17.7%, p=0.003). CONCLUSIONS: These results suggest that MuSK-Ab-positive MG patients exhibit unique and hyperactive responses to AChEIs. Furthermore, R-CMAP and DIP development on a standard AChEI dose may be a distinct neurophysiologic feature indicative of MuSK-Ab-positive MG.

X-linked Charcot-Marie-Tooth Disease Type 1 Harboring Unusual Electrophysiological Features

Charcot-Marie-Tooth X type 1 (CMTX1) is caused by mutations in the connexin 32 gene (Cx32) on the X chromosome. Electrophysiologically, CMTX1 is usually associated with intermediate slowing of conduction velocities and severe impairments in male patients. In addition, patients with CMTX1 rarely present conduction block and temporal dispersion, which are characteristic findings in acquired demyelinating neuropathy. We report herein, for the first time in Korea, two patients with Cx32 mutations who exhibited unusual electrophysiological findings.

Herpes Zoster Immune Reconstitution Inflammatory Syndrome in a HIV-infected Patient: Case Report and Literature Review / 감염과화학요법

According to current evidence, human immunodeficiency virus (HIV)-infected patients who have undergone treatment with antiretroviral therapy are at greater risk of developing herpes zoster, not when they are severely immunocompromised, but, paradoxically, when their immune system is recovering. This is a manifestation of the immune reconstitution inflammatory syndrome (IRIS). Here we report on a case of IRIS, presented as herpes zoster in a HIV-infected patient after undergoing highly active antiretroviral therapy (HAART).

Molluscum Contagiosum as a Skin Manifestation of Immune Reconstitution Inflammatory Syndrome in an AIDS Patient Who Is Receiving HAART / 가정의학회지

Highly active antiretroviral therapy (HAART), which restores specific immune responses, may paradoxically cause an inflammatory reaction known as immune reconstitution inflammatory syndrome (IRIS). We report a patient with acquired immune deficiency syndrome, who presented Molluscum contagiosum as IRIS after HAART, the first case in Korea.

Improvement of Bilateral Parkinsonian Symptoms After Unilateral Intracerebral Hemorrhage

No abstract available.

Tolosa-Hunt Syndrome With Facial Nerve Palsy

Tolosa-Hunt syndrome is a painful ophthalmoplegia due to nonspecific granulomatous inflammation in the cavernous sinus. A 16-year-old man developed Tolosa-Hunt syndrome accompanied by facial nerve palsy. MRI showed an inflammatory lesion in the cavernous sinus with gadolinium enhancement. This rare presentation suggests that Tolosa-Hunt syndrome is a localized form of idiopathic hypertrophic pachymeningitis.

Unilateral Abdominal Distension After Herpes Zoster Infection

No abstract available.