Five-Year Retention of Perampanel and Polytherapy Patterns:328 Patients From a Single Center in South Korea

Background@#and Purpose Perampanel (PER) is an α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid antagonist used to treat focal and generalized epilepsy. Comprehensive data from real-world settings with long-term follow-ups are still scarce. This study aimed to determine the factors related to PER retention and the polytherapy pattern with PER. @*Methods@#We reviewed all patients with epilepsy with a history of PER prescription during 2008–2017 and over a follow-up of >3 years. PER usage patterns and associated factors were analyzed. @*Results@#Among the 2,655 patients in the cohort, 328 (150 females, 178 males) were enrolled.The ages at onset and diagnosis were 21.1±14.7 years and 25.6±16.1 years (mean±standard deviation), respectively. The age at the first visit to our center was 31.8±13.8 years. Seizure types were focal, generalized, and unknown onset in 83.8%, 15.9%, and 0.3% of patients, respectively. The most common etiology was structural (n=109, 33.2%). The maintenance duration of PER was 22.6±19.2 months (range=1–66 months). The initial number of concomitant antiseizure medications was 2.4±1.4 (range=0–9). The most common regimen was PER plus levetiracetam (n=41, 12.5%). The median number of 1-year seizures before PER usage was 8 (range=0–1,400). A seizure reduction of >50% was recorded in 34.7% of patients (52.0% and 29.2% in generalized and focal seizures, respectively). The 1-, 2-, 3-, 4-, and 5-year retention rates for PER were 65.3%, 50.4%, 40.4%, 35.3%, and 21.5%, respectively. A multivariate analysis indicated that lower age at onset was associated with longer retention (p=0.01). @*Conclusions@#PER was safely used in patients with diverse characteristics and was maintained for a long time in a real-world setting, especially in patients with a lower age at onset.

Lesion Detection Through MRI Postprocessing in Pathology-Proven Focal Cortical Dysplasia:Experience at a Single Institution in the Republic of Korea

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.

Clinical Characteristics and Treatment Outcomes of De Novo Nonconvulsive Status Epilepticus:A Retrospective Study

Background@#and Purpose Nonconvulsive status epilepticus (NCSE) is challenging to diagnose. This study aimed to describe and classify the clinical features and electroencephalography (EEG) findings of patients with de novo NCSE and to correlate them with clinical outcomes. @*Methods@#We retrospectively reviewed the medical and EEG records of patients admitted to our institution with altered mentation and EEG abnormalities from January 1, 2013 to December 31, 2018. We evaluated premorbid modified Rankin Scale (mRS) scores, underlying disorders, precipitating factors, clinical manifestations, laboratory tests, and outcomes after a 3-month follow-up. Patients who met the Salzburg Consensus Criteria for NCSE were categorized into good-outcome and poor-outcome groups. A good outcome was defined as 1) clinical and electrographic seizures ceasing after treatment, and 2) an mRS score of ≤2 or remaining unchanged during the 3-month follow-up. A poor outcome was defined as 1) death, 2) seizures continuing despite treatment, or 3) a follow-up mRS score of ≥3 in a patient with a premorbid mRS score of ≤2, or a follow-up mRS score that increased in a patient with a premorbid mRS score of ≥3. @*Results@#The 48 included patients comprised 37 categorized into the good-outcome group and 11 into the poor-outcome group. The presence of acute metabolic disturbances was significantly correlated with poor outcome (p=0.036), while the other analyzed variables were not significantly correlated with outcomes. @*Conclusions@#Acute metabolic disturbances in NCSE are associated with poor outcomes. Adequate treatment of underlying reversible disorders alongside controlling seizures is critical for patients with NCSE.

Comorbid Depression Is Associated with a Negative Treatment Response in Idiopathic REM Sleep Behavior Disorder

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.

Case of Rickettsia typhi-induced Brain Abscess Mimicking Brain Tumor

Murine typhus is one of the most prevalent rickettsial infections in the world, caused by the bacterial genus Rickettsia. Though the disease manifests a relatively benign clinical course with fever, rash, and headache being the 3 classic symptoms, neurological complications may arise in patients that could become permanent. In this case study, a patient with a brain abscess caused by R typhi infection is described. Based upon the recent reemergence of arthropod-borne disease, the findings in this case are significant; R typhi can cause a brain abscess that mimics a brain tumor, which delays the diagnosis and appropriate management of the disease. Murine typhus should always be considered when performing the differential diagnosis of brain abscesses in South Korea.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Korea: Clinical Features, Treatment, and Outcome

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.

Positive Result in the Early Passive Phase of the Tilt-table Test: A Predictor of Neurocardiogenic Syncope in Young Men

BACKGROUND/AIMS: This study elucidated the prognostic factors for neurocardiogenic syncope in males in their late teens and early twenties. METHODS: Tilt-table testing (TTT) was performed on 665 males (age range, 17 to 27 years) following the Italian protocol. The subjects were tilted head-up at a 70degrees angle on a table for 30 minutes during the passive phase. If the passive phase was negative, the subjects were given sublingual nitroglycerin and tilted to the same angle for 20 minutes during the drug-provocation phase. The subjects with positive results were followed without medication. We analyzed factors related to the recurrence rate of syncope. RESULTS: Of 305 subjects (45.8%) with positive results, 223 (age range, 18 to 26 years) were followed for 12 months. The frequency of previous syncopal episodes > or = 4 (p = 0.001) and a positive result during the passive phase (p = 0.022) were significantly related to a high recurrence rate. A positive result during the early passive phase ( 12 minutes; p = 0.011). CONCLUSIONS: A positive result during the early passive phase of TTT and frequent previous syncopal episodes were prognostic factors for neurocardiogenic syncope in men in their late teens and early twenties.

Cataract Following Long-term Use of Carbamazepine in Young Adult

No abstract available.

Neuropathologic and Clinical Features of Human Medial Temporal Lobe Epilepsy

BACKGROUND AND PURPOSE: There is recent evidence of various types of morphological changes in the hippocampus of a rodent model of medial temporal lobe epilepsy (mTLE). However, little is known about such changes in humans. We examined the histological changes [i.e., neuronal loss, cell genesis, and granule cell dispersion (GCD)] in surgical hippocampal specimens taken from patients with mTLE. METHODS: Nissl staining, and nestin and Prox1 immunohistochemistry were performed on human hippocampal specimens obtained from patients with medically intractable mTLE, thus allowing the analysis of neuronal loss, cell genesis, and GCD, respectively. We also assessed the correlations between clinical parameters and the histopathologic findings. RESULTS: The degree of cell genesis in the granule cell layer was significantly correlated with the severity of GCD, history of childhood febrile seizures, and frequent generalized seizures. Cell genesis was not correlated with cell death, age at seizure onset, duration of epilepsy, or the mean frequency of all seizures. CONCLUSIONS: Our results indicate that cell genesis in the dentate gyrus of patients with mTLE is associated with GCD and is influenced by the presence of febrile seizures during childhood and the frequency of episodes of generalized seizures.

Cluster-like Headache Secondary to Cerebral Venous Thrombosis

Cluster headache (CH) is considered a primary headache syndrome. However, symptomatic cases that resemble CH have also been reported. A patient with cerebral venous thrombosis presented with ipsilateral frontal pain accompanied by ophthalmoparesis, nasal congestion, and lacrimation. The patient's headache showed a dramatic response to oxygen. He experienced no further cluster-like headaches after treatment with an anticoagulant. This case suggests the possible role of venous stasis of the cavernous sinus in cluster-like headache.