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.

Background: and PurposeThe first-line medications for the symptomatic treatment of rapid eye movement sleep behavior disorder (RBD) are clonazepam and melatonin taken at bedtime. We aimed to identify the association between depression and treatment response in patients with idiopathic RBD (iRBD). Methods: We reviewed the medical records of 123 consecutive patients (76 males; age, 66.0±7.7 years; and symptom duration, 4.1±4.0 years) with iRBD who were treated with clonazepam and/or melatonin. Clonazepam and melatonin were initially administered at 0.25–0.50 and 2 mg/day, respectively, at bedtime, and the doses were subsequently titrated according to the response of individual patients. Treatment response was defined according to the presence or absence of any improvement in dream-enacting behaviors or unpleasant dreams after treatment. Results: Forty (32.5%) patients were treated with clonazepam, 56 (45.5%) with melatonin, and 27 (22.0%) with combination therapy. The doses of clonazepam and melatonin at followup were 0.5±0.3 and 2.3±0.7 mg, respectively. Ninety-six (78.0%) patients reported improvement in their RBD symptoms during a mean follow-up period of 17.7 months. After adjusting for potential confounders, depression was significantly associated with a negative treatment response (odds ratio=3.76, 95% confidence interval=1.15–12.32, p=0.029). Conclusions We found that comorbid depression is significantly associated with a negative response to clonazepam and/or melatonin in patients with iRBD. Further research with larger numbers of patients is needed to verify our observations and to determine the clinical implications of comorbid depression in the pathophysiology of iRBD.

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

We report here an ectopic case of Fasciola hepatica infection confirmed by recovery of an adult worm in the mesocolon. A 56-year-old female was admitted to our hospital with discomfort and pain in the left lower quadrant of the abdomen. Abdominal CT showed 3 abscesses in the left upper quadrant, mesentery, and pelvic cavity. On surgical exploration, abscess pockets were found in the mesocolon of the sigmoid colon and transverse colon. A leaf-like worm found in the abscess pocket of the mesocolon of the left colon was diagnosed as an adult fluke of F. hepatica. Histologically, numerous eggs of F. hepatica were noted with acute and chronic granulomatous inflammations in the subserosa and pericolic adipose tissues. Conclusively, a rare case of ectopic fascioliasis has been confirmed in this study by the adult worm recovery of F. hepatica in the mesocolon.
Animals ; Fasciola hepatica/genetics/*isolation & purification ; Fascioliasis/diagnosis/*parasitology ; Female ; Humans ; Mesocolon/*parasitology ; Middle Aged

BACKGROUND AND PURPOSE: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune synaptic encephalitis and it often responds to treatment. We analyzed the clinical characteristics of anti-NMDAR encephalitis in Korea. METHODS: Serum and/or cerebrospinal fluid (CSF) of adult patients (aged > or =18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-based indirect immunofluorescence assay. The patients came from 41 university hospitals. RESULTS: Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies and clinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-two patients (68.8%) presented with psychiatric symptoms, 16 (50%) with seizures, 13 (40.6%) with movement disorders, 15 (46.9%) with dysautonomia, 11 (34.4%) with memory disturbance, and 11 (34.4%) with speech disturbance. Magnetic resonance imaging, electroencephalography, and CSF examinations yielded nonspecific findings. Tumor information was only available for 22 patients: 5 patients had tumors, and 2 of these patients had ovarian teratomas. Twenty-two patients received immunotherapy and/or surgery, and therapeutic responses were analyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score on the modified Rankin Scale of 0-2). CONCLUSIONS: This study investigated the clinical characteristics of adult anti-NMDAR encephalitis in Korea. Currently, elderly patients who do not have tumors are commonly diagnosed with this condition. Understanding the detailed clinical characteristics of this disease will improve the early detection of anti-NMDAR encephalitis in patients both young and old.
Adult ; Aged ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis* ; Antibodies ; Cerebrospinal Fluid ; Electroencephalography ; Encephalitis ; Fluorescent Antibody Technique, Indirect ; Hospitals, University ; Humans ; Immunotherapy ; Korea ; Magnetic Resonance Imaging ; Memory ; Movement Disorders ; Primary Dysautonomias ; Seizures ; Teratoma

The neurological manifestations caused by Epstein-Barr virus (EBV) occur only rarely in association with its primary infection or reactivation. The mechanism by which EBV produces neurological disease is unknown. This article describes two cases of polymerase-chain-reaction-proven EBV brainstem encephalitis. The sera of both patients contained autoantibodies against N-methyl-D-aspartate receptor (NMDAR), suggesting the presence of a secondary immunological mechanism. Prospective studies are needed to reveal whether the subgroup of patients with EBV encephalitis and anti-NMDAR antibodies have different clinical presentations and would benefit from immunotherapy.
Antibodies ; Autoantibodies ; Brain Stem ; Encephalitis ; Herpesvirus 4, Human ; Humans ; Immunotherapy ; N-Methylaspartate ; Neurologic Manifestations

The neurological manifestations caused by Epstein-Barr virus (EBV) occur only rarely in association with its primary infection or reactivation. The mechanism by which EBV produces neurological disease is unknown. This article describes two cases of polymerase-chain-reaction-proven EBV brainstem encephalitis. The sera of both patients contained autoantibodies against N-methyl-D-aspartate receptor (NMDAR), suggesting the presence of a secondary immunological mechanism. Prospective studies are needed to reveal whether the subgroup of patients with EBV encephalitis and anti-NMDAR antibodies have different clinical presentations and would benefit from immunotherapy.
Antibodies ; Autoantibodies ; Brain Stem ; Encephalitis ; Herpesvirus 4, Human ; Humans ; Immunotherapy ; N-Methylaspartate ; Neurologic Manifestations

A 74-year-old man suffered from jejunal perforation and adhesion to sigmoid colon due to adenocarcinoma associated with intraductal papillary mucinous neoplasm (IPMN) arising in a jejunal heterotopic pancreas. The jejunal lesion showed direct extension to the sigmoid colon, which was mistaken as sigmoid colon cancer by surgeons. Malignant transformation is a rare complication of a heterotopic pancreas. About half of malignancies in reported cases were ductal adenocarcinoma arising in the stomach, and the jejunal location is extremely rare. Furthermore, IPMN is also uncommon finding in a heterotopic pancreas.
Adenocarcinoma ; Aged ; Colon, Sigmoid ; Humans ; Jejunum ; Mucins ; Pancreas ; Sigmoid Neoplasms ; Stomach

Autoimmune encephalitis is a group of disorders that predominantly affects the limbic system, with or without an associated neoplasm. Recently GABAB receptor antibody has been identified in a subset of encephalitides. We report a case of anti-GABAB receptor antibody encephalitis in a 64-year-old woman, who presented with transient, recurrent abnormal behavior and complex partial seizures. She had a history of breast cancer, which was in complete remission and her workup, including blood, imaging and routine cerebrospinal fluid studies was unremarkable. Indirect immunofluorescence assays with patient's serum revealed the presence of autoantibodies against GABAB receptor. She showed significant improvement after initiating immunotherapy. This case illustrates the importance of autoantibody testing in the diagnosis of encephalitis.
Autoantibodies ; Brain Diseases ; Breast Neoplasms ; Encephalitis ; Female ; Fluorescent Antibody Technique, Indirect ; Hashimoto Disease ; Humans ; Immunotherapy ; Limbic Encephalitis ; Limbic System ; Seizures

Carcinoma showing thymus-like differentiation (CASTLE) is a very rare malignant neoplasm of the thyroid, which resembles lymphoepithelioma or squamous cell carcinoma of the thymus. It originates from ectopic thymic tissue or remnants of the branchial pouches. We recently experienced a case of CASTLE in the thyroid gland of a 65-year-old woman. The patient presented with a non-tender mass in the right thyroid gland and dyspnea and coughing upon bending. The patient was diagnosed with ‘cystic change of adenomatous goiter’ of the thyroid by fine needle aspiration cytology. Right thyroidectomy was performed because of nodular hyperplasia on frozen biopsy. Histologic examination of the resected tumor showed that the tumor was lobulated and expansive growth pattern, with fibrous septa dividing the tumor and infiltrated by lymphocytes and plasma cells. Tumor cells possessed oval, large vesicular nuclei and prominent nucleoli, and the immunohistochemical staining was positive for CD5. The patient was diagnosed with thyroid CASTLE. We performed complete thyroidectomy. There has been no local regional recurrence.
Aged ; Biopsy ; Biopsy, Fine-Needle ; Carcinoma, Squamous Cell ; Cough ; Dyspnea ; Female ; Humans ; Hyperplasia ; Lymphocytes ; Plasma Cells ; Recurrence ; Thymus Gland* ; Thyroid Gland ; Thyroidectomy