• Journal of The Korean Society of Emergency Medicine
• /
• v.29 no.5
• /
• pp.519-528
• /
• 2018

Objective: Delayed neuropsychiatric sequelae (DNS) following carbon monoxide (CO) poisoning, which may result from a demyelinating leukoencephalopathy, is a disease with a poor prognosis. This study examined the factors affecting the long-term prognosis of DNS and the efficacy of hyperbaric oxygen therapy (HBOT) in patients with DNS. Methods: This retrospective study included 84 patients with DNS following CO poisoning from January 2013 to June 2016. HBOT was given to 24 patients. The patients were divided into an improvement group and non-improvement group based on their clinical condition on a telephone interview at intervals between 3 months and 3 years after the onset of DNS. The improvement group was defined as having Cerebral Performance Category (CPC) scores in their daily life that improve to 1 or 2 grade. Results: Of the 594 patients, DNS were found in 18.2%, and 70.2% (59 of 84) of the patients with DNS improved. The prognostic factors for the improvement of DNS were an age of 45 years or less (odds ratio [OR], 12.068; 95% confidence interval [CI], 2.393-60.858; P<0.005), CPC score of 1 or 2 group at the time of DNS onset (OR, 12.361; 95% CI, 3.161-48.330; P<0.005), and a lucid interval longer than 20 days (OR, 5.164; 95% CI, 1.393-19.141; P<0.01). HBOT was not associated with the improvement of DNS in CO poisoning (OR, 0.467; 95% CI, 0.172-1.269; P>0.1). Conclusion: Patients aged less than 45 years, low grade CPC score of 1 and 2, and lucid interval longer than 20 days are more likely to have a good prognosis. On the other hand, HBOT failed to produce a benefit for DNS patients.

• Annals of Clinical Neurophysiology
• /
• v.25 no.2
• /
• pp.84-92
• /
• 2023

Background: Clinical spectrum of immunoglobulin M (IgM) monoclonal gammopathy varies from IgM monoclonal gammopathy of unknown significance (IgM-MGUS) to hematological malignancies. We evaluated the clinical features, electrophysiological characteristics, and prognosis of patients with peripheral neuropathy associated with IgM monoclonal gammopathy (PN-IgM MG). Methods: We retrospectively evaluated 25 patients with PN-IgM MG. Peripheral neuropathy was classified as axonal, demyelinating, or undetermined, based on electrophysiological studies. We classified the enrolled patients into the IgM-MGUS and malignancy groups, and compared the clinical and electrophysiological features between the groups. Results: Fifteen patients had IgM-MGUS and 10 had hematologic malignancies (Waldenström's macroglobulinemia: two and B-cell non-Hodgkin's lymphoma: eight). In the electrophysiological evaluation, the nerve conduction study (NCS) criteria for demyelination were met in 86.7% of the IgM-MGUS group and 10.0% of the malignancy group. In particular, the distal latencies of the motor NCS in the IgM-MGUS group were significantly prolonged compared to those in the malignancy group (median, 9.1 ± 5.1 [IgM-MGUS], 4.2 ± 1.3 [malignancy], p = 0.003; ulnar, 5.4 ± 1.9 [IgM-MGUS], 2.9 ± 0.9 [malignancy], p = 0.001; fibular, 9.3 ± 5.1 [IgM-MGUS], 3.8 ± 0.3 [malignancy], p = 0.01; P-posterior tibial, 8.3 ± 5.4 [IgM-MGUS], 4.4 ± 1.0 [malignancy], p = 0.04). Overall treatment responses were significantly worse in the malignancy group than in the IgM-MGUS group (p = 0.004), and the modified Rankin Scale score at the last visit was higher in the malignancy group than in the IgM-MGUS group (2.0 ± 1.1 [IgM-MGUS], 4.2 ± 1.7 [malignancy], p = 0.001), although there was no significant difference at the initial assessment. Conclusions: The risk of hematological malignancy should be carefully assessed in patients with PN-IgM MG without electrophysiological demyelination features.

• Clinical and Experimental Vaccine Research
• /
• v.12 no.2
• /
• pp.143-155
• /
• 2023

Purpose: An association between Guillain-Barré syndrome (GBS) and severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) vaccination has been reported. We aimed to summarize the clinical features of GBS associated with SARS-CoV-2 vaccination and determine the contrasting features from coronavirus disease-19 (COVID-19) associated GBS and GBS following other causes. Materials and Methods: We performed PubMed search for articles published between 1 December 2020 and 27 January 2022 using search terms related to "SARS-CoV-2 vaccination" and "GBS". Reference searching of the eligible studies was performed. Sociodemographic and vaccination data, clinical and laboratory features, and outcomes were extracted. We compared these findings with post-COVID-19 GBS and International GBS Outcome Study (IGOS) (GBS from other causes) cohorts. Results: We included 100 patients in the analysis. Mean age was 56.88 years, and 53% were males. Six-eight received non-replicating virus vector and 30 took messenger RNA (mRNA) vaccines. The median interval between the vaccination and the GBS onset was 11 days. Limb weakness, facial palsy, sensory symptoms, dysautonomia, and respiratory insufficiency were seen in 78.65%, 53.3%, 77.4%, 23.5%, and 25%, respectively. The commonest clinical and electrodiagnostic subtype were sensory-motor variant (68%) and acute inflammatory demyelinating polyneuropathy (61.4%), respectively. And 43.9% had poor outcome (GBS outcome score ≥3). Pain was common with virus vector than mRNA vaccine, and the latter had severe disease at presentation (Hughes grade ≥3). Sensory phenomenon and facial weakness were common in vaccination cohort than post-COVID-19 and IGOS. Conclusion: There are distinct differences between GBS associated with SARS-CoV-2 vaccination and GBS due to other causes. Facial weakness and sensory symptoms were commonly seen in the former and outcomes poor.

• Journal of the Korean Society of Radiology
• /
• v.81 no.1
• /
• pp.243-247
• /
• 2020

We report a rare case of hyperglycemia-related osmotic demyelination syndrome (ODS) with focus on the imaging findings. A 61-year-old man with diabetes was admitted for general weakness and severe thirst. A few days later, he complained of dysarthria, dysphasia, and dysmetria. Laboratory examinations showed significant hyperglycemia, but normal electrolyte levels. Brain MRI revealed T2-signal abnormalities that were symmetrical, non-space occupying, and located in the central pons with a peripheral sparing pattern, which were suggestive of ODS. In addition, subsequent MRI revealed progression of signal hyperintensity; however, the patient's symptoms improved.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.12 no.1
• /
• pp.49-53
• /
• 2012

X-linked adrenoleukodystrophy (ALD) is a rare inherited metabolic disease which results in impaired peroxisomal ${\beta}$-oxidation and the accumulation of very long chain fatty acids (VLCFA) in the adrenal cortex, the myelin of the central nervous system, and the testes. X-linked ALD is caused by mutations in the ABCD1 gene encoding an ATP-binding cassette transporter superfamily located in the peroxisomal membrane. This disease is characterized by a variety of phenotypes. The classic childhood cerebral ALD is a rapidly progressive demyelinating condition affecting the cerebral white matter before the age of 10 years in boys. We report the case of a 8-year-old with childhood cerebral X-linked ALD who developed inattention, hyperactivity, motor incoordination and hemiparesis. We diagnosed ALD with elevated plasma very long chain fatty acid level and diffuse high signal intensity lesions in both parieto-occipital white matter and cerebellar white matter in brain MRI. We identified a novel c.983delT (p.Met329CysfsX7) mutation of the ABCD1 gene. There is no correlation between X-ALD phenotype and mutations in the ABCD1 gene. Further studies for searching additional non-genetic factor which determine the phenotypic variation will be needed.

• Korean Journal of Veterinary Research
• /
• v.46 no.3
• /
• pp.177-184
• /
• 2006

Experimental autoimmune encephalomyelitis (EAE) is a disease model of multiple sclerosis (MS) that is characterized by remittance and relapse of the disease and autoimmune and demyelinating lesions in the central nervous system (CNS). Autoimmune inflammation is maintained by secretion of a large number of protein. Previous studies have suggested that transcripts encoding osteopontin (OPN) are frequently detected in the mRNA population of MS plaques. To elucidate the functional role of OPN in initiation and development of EAE, we examined the expression and localization of OPN in the spinal cord during acute EAE. We demonstrated that OPN significantly increased at the early stage of EAE and slightly declined thereafter by western blot analysis. An immunohistochemical study revealed that OPN was constitutively expressed in some glial cells (microglia, astrocytes) of white matter and neurons in the CNS of control rats. OPN expression was shown to be increased in the same cells at the early and peak stage of EAE. To identity cells expressing OPN by double-immunofluorescence labeling, we labeled rat spinal cord sections for OPN with a monoclonal OPN antibody and with mAbs for astrocyte (GFAP), microglia/macrophage (OX42)-specific markers. The major cell types of OPN-expressing cells were activated astrocytes and microglia in the adjacent inflammatory lesions. Interestingly, OPN was mainly expressed in the end feet of astrocytes around vascular cell adhesion molecule-1 (VCAM-1) expressing endothelial cells of CNS blood vessel. These findings suggest that increased levels of OPN in activated glial cell may play an important role in the recruitment of inflammatory cells into the CNS parenchyma during EAE.

• Journal of the Korean Child Neurology Society
• /
• v.23 no.2
• /
• pp.45-50
• /
• 2015

Purpose: X-linked adrenoleukodystrophy (X-ALD) is a fatal, axonal demyelinating, neurodegenerative disease, and is caused by mutations the in ABCD1 (ATP-binding cassette transporter subfamily D member 1). Oxidative damage of proteins caused by very long chain fatty acid accumulating in X-ALD, is an early event in the neurodegenerative cascade. We evaluated valproic acid (VPA) as a possible option for oxidative damage in X-ALD. Method: We generated fibroblast of the childhood cerebral ALD from patient. We evaluated mRNA (ribonucleic acid) level of ABCD2 by real-time polymerase chain reaction, and reactive oxygen species (ROS) levels by flow cytometry. Results: VPA increased expression of ABCD2 in both control and ALD fibroblast. ABCD2 gene mRNA expression was increased 1.76 fold in normal fibroblasts, and 2.22 fold in the X-ALD fibroblasts. ROS levels were decreased in VPA treated X-ALD fibroblast, especially in treated with 1 mM of VPA. ROS levels revealed 13.7 in control fibroblast, on the other hand, 5.83 in X-ALD fibroblast treated with 1 mM of VPA. Conclusion: We propose VPA as a promising novel therapeutic approach in oxidant damage that warrants further clinical investigation in X-ALD.

• Journal of the Korean Society of Radiology
• /
• v.85 no.2
• /
• pp.381-393
• /
• 2024

Purpose Metabolic abnormalities in hepatic encephalopathy (HE) cause brain edema or demyelinating disease, resulting in symmetric regional cerebral edema (SRCE) on MRI. This study aimed to investigate the usefulness of the clustering analysis of SRCE in predicting the development of brain failure. Materials and Methods MR findings and clinical data of 98 consecutive patients with HE were retrospectively analyzed. The correlation between the 12 regions of SRCE was calculated using the phi (φ) coefficient, and the pattern was classified using hierarchical clustering using the φ² distance measure and Ward's method. The classified patterns of SRCE were correlated with clinical parameters such as the model for end-stage liver disease (MELD) score and HE grade. Results Significant associations were found between 22 pairs of regions of interest, including the red nucleus and corpus callosum (φ = 0.81, p < 0.001), crus cerebri and red nucleus (φ = 0.72, p < 0.001), and red nucleus and dentate nucleus (φ = 0.66, p < 0.001). After hierarchical clustering, 24 cases were classified into Group I, 35 into Group II, and 39 into Group III. Group III had a higher MELD score (p = 0.04) and HE grade (p = 0.002) than Group I. Conclusion Our study demonstrates that the SRCE patterns can be useful in predicting hepatic preservation and the occurrence of cerebral failure in HE.

• IMMUNE NETWORK
• /
• v.20 no.5
• /
• pp.40.1-40.15
• /
• 2020

The protein encoded by the Gene Associated with Retinoid-Interferon-Induced Mortality-19 (GRIM-19) is located in the mitochondrial inner membrane and is homologous to the NADH dehydrogenase 1-alpha subcomplex subunit 13 of the electron transport chain. Multiple sclerosis (MS) is a demyelinating disease that damages the brain and spinal cord. Although both the cause and mechanism of MS progression remain unclear, it is accepted that an immune disorder is involved. We explored whether GRIM-19 ameliorated MS by increasing the levels of inflammatory cytokines and immune cells; we used a mouse model of experimental autoimmune encephalomyelitis (EAE) to this end. Six-to-eight-week-old male C57BL/6, IFNγ-knockout (KO), and GRIM-19 transgenic mice were used; EAE was induced in all strains. A GRIM-19 overexpression vector (GRIM19 OVN) was electrophoretically injected intravenously. The levels of Th1 and Th17 cells were measured via flow cytometry, immunofluorescence, and immunohistochemical analysis. IL-17A and IFNγ expression levels were assessed via ELISA and quantitative PCR. IL-17A expression decreased and IFNγ expression increased in EAE mice that received injections of the GRIM-19 OVN. GRIM19 transgenic mice expressed more IFNγ than did wild-type mice; this inhibited EAE development. However, the effect of GRIM-19 overexpression on the EAE of IFNγ-KO mice did not differ from that of the empty vector. GRIM-19 expression was therapeutic for EAE mice, elevating the IFNγ level. GRIM-19 regulated the Th17/Treg cell balance.

• Clinical and Experimental Pediatrics
• /
• v.50 no.9
• /
• pp.891-895
• /
• 2007

Purpose : Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system and mostly develops after viral illness or vaccinations. We investigated the clinical differences and neurologic outcomes according to the distribution of the lesions on brain MRI. Methods : The study group was composed of 21 patients from January 1995 to August 2003 in Kyunghee University hospital. We grouped the patients according to the MRI findings as follows. Group I (14 cases): Multi- or unifocal lesions only in the cerebral white matter. Group II (7 cases): lesions in the gray matter with or without white matter involvement. Results : 1. Preceding events were as follows: no defined prodrome (38.1%), upper respiratory tract infection (28.6%), nonspecific febrile illness (19.0%), gastointestinal disturbance and vaccination. 2. Presenting symptoms were as follows: seizures (76.2%), headache/vomiting (47.6%), altered consciousness (38.1%), hemiparesis, cerebellar ataxia, visual disturbance and facial nerve palsy. 3. Laboratory findings were as follows: CSF pleocytosis (76.2%), leucocytosis (38.1%) and elevated CSF protein (28.6%). 4. Fifteen patients were recovered completely without neurological sequelae. Three patients in group I and 1 patient in group II had intractable seizures. Two patients in group I and 2 patients in group II had motor disturbance. Conclusion : There were no statistically significant differences in preceding events, presenting symptoms, and neurological outcomes according to the distribution of the lesions on brain MRI. However, the ADEM have quite diverse clinical manifestations and neuroimage findings. MRI plays an important role in making diagnosis of the patients who are suspected of ADEM.