Anti-leucine-rich glioma inactivated-1 (LGI1) limbic encephalitis (LE) is a rare neurological disorder that has a subacute course of progressive encephalopathy and fasciobrachial dystonic seizures. We report a patient with anti-LGI1 LE that presented with atypical manifestations that complicated the diagnosis. A 62-year-old woman presented with a chronic course of memory disturbance and a subsequent relapse with an altered mental status after 10 months. The patient reported frequent chest pain of squeezing and dull nature, typically lasting 10-30 seconds. The chest pain was related to partial seizures, which were confirmed by video-EEG monitoring. Anti-LGI1 antibody was identified in serum and CSF. The patient's symptoms improved by immune modulation treatment. Patients with anti-LGI1 LE can experience atypical partial seizures, and a chronic relapsing course. Clinical suspicions and video-EEG monitoring are helpful for the early diagnosis and effective immune modulation.
Chest Pain ; Diagnosis ; Early Diagnosis ; Female ; Glioma ; Humans ; Limbic Encephalitis* ; Memory ; Middle Aged ; Nervous System Diseases ; Recurrence ; Seizures

Tryptophan metabolites regulate a variety of physiological processes, and their downstream metabolites enter the kynurenine pathway. Age-related changes of metabolites and activities of associated enzymes in this pathway are suggestable and would be potential intervention targets. Blood levels of serum tryptophan metabolites in C57BL/6 mice of different ages, ranging from 6 weeks to 10 months, were assessed using high-performance liquid chromatography, and the enzyme activities for each metabolic step were estimated using the ratio of appropriate metabolite levels. Mice were subjected to voluntary chronic aerobic exercise or high-fat diet to assess their ability to rescue age-related alterations in the kynurenine pathway. The ratio of serum kynurenic acid (KYNA) to 3-hydroxylkynurenine (3-HK) decreased with advancing age. Voluntary chronic aerobic exercise and high-fat diet rescued the decreased KYNA/3-HK ratio in the 6-month-old and 8-month-old mice groups. Tryptophan metabolites and their associated enzyme activities were significantly altered during aging, and the KYNA/3-HK ratio was a meaningful indicator of aging. Exercise and high-fat diet could potentially recover the reduction of the KYNA/3-HK ratio in the elderly.
Aged ; Aging ; Animals ; Chromatography, Liquid ; Diet, High-Fat* ; Exercise* ; Humans ; Infant ; Kynurenic Acid ; Kynurenine* ; Mice ; Physiological Processes ; Tryptophan*

BACKGROUND: Intracerebral hemorrhage (ICH) is associated with a considerable proportion of stroke and head injuries, but except for supportive care, there is no medical therapy available. Transplantation of human neural stem cells (NSCs) can be used to reduce behavioral deficit in experimental ischemic infarct model. However, effect of stem cell transplantation in experimental intracerebral hemorrhage (ICH) is unknown. We hypothesized that NSCs could migrate and differentiate into neurons or glial cells, and improve functional outcome in ICH. METHODS: Experimental ICH was made by intrastriatal administration of bacterial collagenase in adult rats. Animals were randomized to receive intravenously either immortalized Lac-Z positive human NSCs (5x1 06 in 500microL, n=15) or same volume of saline (n=12) on the following day. Animals were evaluated for 8 weeks after surgery with behavioral test battery. After 8 weeks, animals were sacrificed and the brains were sectioned. Transplanted NSCs were detected by X-gal histochemistry or beta-gal immunohistochemistry, and differentiation of grafted NSCs were evaluated by double labeling of GFAP, NeuN, or neurofilament. RESULTS: Transplanted NSCs migrated to the side of peri-hematomal areas, and differentiated into neurons and astrocytes. NSCs injection group showed improved performances on rotarod test after 2 weeks and on limb placing test after 5 weeks compared with control group (p<0.05) and these effect persisted up to 8 weeks. CONCLUSIONS: Intravenously injected NSCs enter rat brain with ICH, and differentiate into astrocytes or neuronal cell, which lead to functional recovery. These findings show the possibility that NSCs can be used to reduce neurological deficits in the experimental ICH.
Adult ; Animals ; Astrocytes ; Brain ; Cerebral Hemorrhage* ; Collagenases ; Craniocerebral Trauma ; Extremities ; Humans* ; Immunohistochemistry ; Neural Stem Cells* ; Neuroglia ; Neurons ; Rats ; Rotarod Performance Test ; Stem Cell Transplantation ; Stroke ; Transplants

BACKGROUND AND PURPOSE: Hypertrophic pachymeningitis (HP) is a rare disease caused by autoimmunity in the meninx that causes various neurologic symptoms, including headache, seizures, weakness, paresthesia, and cranial nerve palsies. Although the first-line therapy for HP is steroids, many HP cases are refractory to steroids or recur when the steroids are tapered. Here we report three HP cases that were successfully treated with rituximab (RTX). METHODS: From an institutional cohort recruited from April 2012 to July 2016, three HP cases that were identified to be steroid-refractory were treated with RTX (four weekly doses of 375 mg/m²). Clinical improvement was assessed by the number of relapses of any neurologic symptom and the largest dural thickness in MRI. RESULTS: All three patients were recurrence-free of neurologic symptoms and exhibited prominent decreases in the dural thickness after RTX treatment. No adverse events were observed in the patients. CONCLUSIONS: We suggest RTX as a second-line therapy for steroid-refractory HP. Further studies are warranted to confirm this observation in a larger population and to consider RTX as a first-line therapy.
Autoimmunity ; Cohort Studies ; Cranial Nerve Diseases ; Headache ; Humans ; Magnetic Resonance Imaging ; Meningitis* ; Neurologic Manifestations ; Paresthesia ; Rare Diseases ; Recurrence ; Rituximab* ; Seizures ; Steroids

Neurological disorders induced by long-term exposure to organic solvents typically have a slowly progressive clinical course, which may be arrested or even reversed following discontinuation of exposure. We report an unusual case of rapidly progressive toxic leukoencephalomyelopathy in a 29-year-old man who had worked at a chemical factory that used toluene for the manufacture of nylon 66 for 5 years. He presented with progressive weakness of legs, recurrent seizures, and cognitive decline. Widespread white-matter changes in the brain and spinal cord, and myelodysplastic syndrome were noted. He died 6 months after the onset of his symptoms, and autopsy showed discrete multifocal demyelination and necrosis in the central nervous system, and dysplastic cells of erythroid, myeloid, and megakaryotic lineages in blood vessels. The co-occurrence of leukoencephalomyelopathy and myelodysplastic syndrome highlights the vulnerability of the white matter and bone marrow to injury from organic solvents. Intravascular congestion of dysplastic hematopoietic cells might have led to his unusually rapid progression of leukoencephalomyelopathy.
Adult ; Autopsy ; Blood Vessels ; Bone Marrow ; Brain ; Central Nervous System ; Demyelinating Diseases ; Estrogens, Conjugated (USP) ; Humans ; Leg ; Myelodysplastic Syndromes* ; Necrosis ; Nervous System Diseases ; Nylons ; Seizures ; Solvents ; Spinal Cord ; Toluene

BACKGROUND AND PURPOSE: Herpes simplex encephalitis (HSE) is the most common type of sporadic encephalitis worldwide, and it remains fatal even when optimal antiviral therapy is applied. There is only a weak consensus on the clinical outcomes and prognostic factors in patients with HSE. This study examined whether the radiological and electrophysiological findings have a prognostic value in patients with HSE. METHODS: We retrospectively analyzed patients who were diagnosed with HSE by applying the polymerase chain reaction to cerebrospinal fluid and who received intravenous acyclovir at our hospital from 2000 to 2014. We evaluated the clinical outcomes at 6 months after onset and their correlations with initial and clinical findings, including the volume of lesions on MRI, the severity of EEG findings, and the presence of epileptic seizures at the initial presentation. RESULTS: Twenty-nine patients were enrolled (18 men and 11 women). Univariate analysis revealed that the presence of severe EEG abnormality and epileptic seizures at the initial presentation were significant correlated with a poor clinical outcome at 6 months (p=0.005 and p=0.009, respectively). In multivariate analysis, the presence of severe EEG abnormality was the only independent predictor of a poor outcome at 6 months (p=0.006). CONCLUSIONS: In cases of HSE, the initial EEG severity and seizure presentation may be useful predictive factors for the outcome at 6 months after acyclovir treatment.
Acyclovir ; Cerebrospinal Fluid ; Consensus ; Electroencephalography* ; Encephalitis ; Encephalitis, Herpes Simplex* ; Epilepsy ; Herpes Simplex* ; Humans ; Magnetic Resonance Imaging* ; Male ; Multivariate Analysis ; Polymerase Chain Reaction ; Retrospective Studies ; Seizures ; Simplexvirus

The present study was carried out to evaluate the effect of hypothermia and rewarming on the pattern of Cortical Spreading Depression(CSD) and predict the resultant vulnerability of the brain. Thirty four Sprague-Dawley rats, either sex, weighing 250-350gm, were used. They were devided into 4 groups, according to the degree of hypothermia and speed of rewarming, Profound Hypothermia & Slow Rewarming(G I), Profound Hypothermia & Rapid Rewarming(G II), Moderate Hypothermia & Slow Rewarming(G III), Moderate Hypothermia & Rapid Rewarming(G IV). CSD was elicited by local application of KC1 and identified by Direct Current Potential(DCP). We estimated the Frequency of DCP, Recovery time of negative shift of DCP, Area of negative shift of DCP according to the change of body temperature. The frequencies of DCP increased after rewarming compared with the normothermic state in all groups, statistical significance(P<0.05) was specially evident in Group II(Profound Hypothermia & Rapid Rewarming group), 5.1+/-0.3/30min.(mean+/-standard error) in normothermic state, while 9.3+/-0.8/30min in rewarming state. Recovery time(width) of DCP was delayed with hypothermia but decreased with rewarming but delayed state compared with normothermic state in all groups, i.e. 34.7+/-1.0sec at normothermic state, 59.9+/-4.3sec at 27.5 degrees C, 40+/-1.4sec at rewarming state in Group II. We can predict that the vulnerability of the brain tissue may be increased during the rewarming state following hypothermia in all groups. Such evidence is more significant in Group II(Profound Hypothermia & Rapid Rewarming) during the period of certain time(30-60min) after rewarming.
Animals ; Body Temperature ; Brain ; Cortical Spreading Depression* ; Hypothermia* ; Rats* ; Rats, Sprague-Dawley ; Rewarming*

Pheochromocytoma may cause anatomic and functional cardiac abnormalities. Various echocardiographic abnormalities have been observed, including systolic anterior movement, global hypokinesis as well as hypokinesis of the base and the apex of LV. There are no previous reports of serial echocardiographic finding in patients with pheochromocytoma. We experienced a 38-year-old woman with pheochromocytoma who suffered from acute myocardial injury. Echocardiography revealed that the acute myocardial injury started in the base in the early phase and subsequently extended to the mid-portion of left ventricule. The injury persisted longer in the base than the mid-portion. than 1.5 times the adjacent normal coronary artery.
Adult ; Coronary Vessels ; Echocardiography ; Female ; Humans ; Pheochromocytoma

Background: and Purpose Focal cortical dysplasia (FCD) is one of the most common causes of drug-resistant epilepsy, and necessitates a multimodal evaluation to ensure optimal surgical treatment. This study aimed to determine the supportive value of the morphometric analysis program (MAP) in detecting FCD using data from a single institution in Korea. Methods: To develop a standard reference for the MAP, normal-looking MRIs by two scanners that are frequently used in this center were chosen. Patients with drug-resistant epilepsy and FCD after surgery were candidates for the analysis. The three-dimensional T1-weighted MRI scans of the patients were analyzed as test cases using the MAP. Results: The MRI scans of 87 patients were included in the analysis. The radiologist detected abnormal findings correlated with FCD (RAD positive [RAD(+)]) in 34 cases (39.1%), while the MAP could detect FCD in 25.3% of cases. A combination of the MAP (MAP[+] cases) with interpretations by the radiologist increased the detection to 42.5% (37 cases). The lesion detection rate was not different according to the type of reference scanners except in one case. MAP(+)/RAD(-) presented in three cases, all of which had FCD type IIa. The detection rate was slightly higher using the same kind of scanner as a reference, but not significantly (35.0% vs. 22.4% p=0.26). Conclusions The results of postprocessing in the MAP for detecting FCD did not depend on the type of reference scanner, and the MAP was the strongest in detecting FCD IIa. We suggested that the MAP could be widely utilized without developing institutional standards and could become an effective tool for detecting FCD lesions.

The incidence of vancomycin-resistant enterococcus (VRE) meningitis is increasing. We present a 56-year-old man who suffered from VRE meningitis after cerebellar hemorrhage and intraventricular catheter insertion. The patient recovered completely after treatment with linezolid for 6 weeks. This is the first report on effectiveness of linezolid in treating VRE meningitis in Korea.
Acetamides ; Catheters ; Enterococcus ; Hemorrhage ; Humans ; Incidence ; Meningitis ; Middle Aged ; Oxazolidinones ; Linezolid