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Background: New-onset refractory status epilepticus (NORSE) occurs in people without a history of seizures. In these cases, the seizure causes are unclear, and the seizures are not controlled by standard treatment. Autoimmune encephalitis (AIE) can be a cause of NORSE. Cryptogenic NORSE may be associated with AIE, but antibodies associated with the condition have not yet been identified. Primary immunotherapy may not be effective for AIE. Rituximab has improved the prognosis in some cases. Case Report: We treated a cryptogenic NORSE patient with a combination of antiepileptic drugs and immunotherapy. On the 13th hospital day, the seizures were controlled, but the patient remained in a coma. The patient rapidly recovered after administration of rituximab started on the 26th hospital day. Conclusions: Rituximab may be helpful for cryptogenic NORSE patients in whom primary immunotherapy controls seizures, but fails to improve consciousness.
본 논문에서는 3차와 4와의 큐뮬런트를 이용하여 FIR 시스템의 파라메터 추정을 위한 회귀 추정 알고리듬을 제안한다. 제안한 FIR 파라메터 회귀 추정 알고리듬에서 3차와 4차의 큐뮬런트 관계식으로부터 Overdetermined Recurisive Instrumental Variable (ORIV) 형태의 회귀 추정 알고리듬으로 변환할 수 있도록 출력신호로 구성된 행렬식을 얻어낸 후, 이를 전개하여 회귀 추정 알고리듬을 개발한다. 제안한 회귀 추정 알고리듬은 기존의 비회귀 알고리듬의 확장으로 적은 데이터로 수렴이 가능하며, 시변 시스템의 추정에도 용이하다. 또한 3차와 4차의 순수 고차 큐뮬런트로 구성됨에 따라 기존의 2차의 자기상관함수를 이용한 회귀 추정 알고리듬에 비해 가산 가우시안 잡음에 의한 추정 오차를 줄일 수 있는 장점이 있다. In this paper, a recursive estimation algorithm for FIR systems is proposed using the 3rd and 4th order cumulants. To obtain the Overdetermined Recursive Instrumental Variable(ORIV) method type algorithm, we transform the 3'th and 4'th order cumulant relationship to a certain matrix form which is consist of only output data. From the matrix form, we induce the proposed algorithm procedure following the ORIV method. The proposed algorithm provides improved estimation accuracy with smaller data and can be applied to a time varying system as well. In addition, it reduces the estimation error due to the additive Gaussian noise compared to conventional 2'rd order based algorithms since it only uses higher than 2'rd order cumulant. Simulation results are presented to compare the performance with other HOS-based algorithms.
Myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS) are distinct disorders. ALS affects motor neurons that control muscle movement, while MG controls communication between neurons and muscles, which occurs at neuromuscular junctions. However, on rare occasions, ALS develops after MG and vice versa. The coexistence of the two diseases represents a diagnostic challenge and requires thoughtful interpretation of clinical features. We present the case of a 53-year-old Korean male who developed ALS after MG, confirmed by clinical and electrophysiological follow-up.
Background: Brain death is a clinical diagnosis that implies irreversible loss of function of the entire brain, including the brainstem and both hemispheres. Based on previous reports, it is not rare for reflex and spontaneous movements to occur in patients during the process of determinin brain death. However, reports of the frequency and common types of these movements vary from study to study. Thus, we evaluated adult patients with impending brain death in Korea to determine the frequency and characteristics of reflex and spontaneous movements. Methods: Brain dead patients who were admitted to 15 hospitals in the Yeongnam region (Southeast) of Korea were recruited prospectively from January 2013 to September 2016. All patients met the criteria for brain death as established by the Korea Medical Association. All body movements occurred during the process of diagnosing brain death and were assessed by physicians and trained organ transplant coordinators. The frequency and characteristics of these movements were identified and the demographic and clinical factors of impending brain dead patients with and without these movements were compared. Results: A total of 436 patients who met the criteria for brain death were enrolled during the study period. Of these patients, 74 (17.0%) exhibited either reflex or spontaneous movements Of this subset, 45 (60.8%) exhibited reflex movements only, 18 (24.3%) exhibited spontaneou movements only, and 11 (14.9%) exhibited both reflex and spontaneous movements. The mos common reflex movements were the flexor/extensor plantar response and spinal myoclonus. Of the 74 patients, 52 (70.3%) exhibited one movement of the same pattern and 22 (29.7%) exhibited two or more different movement patterns. In addition, 45 (60.8%) exhibited these movements only on a limited area of the body with the leg being most common (n = 26, 57.8%). Patients with hypoxic brain damage and a higher systolic blood pressure exhibited significantly more reflex or spontaneous movements. Conclusion: Movements associated with brain dead patients are not rare and thus an awareness of these movements is important to brain death diagnosis. Physicians who perform brain death examinations should understand the frequency and characteristics of these movements to reduce delays in determining brain death.
Objective: To elucidate the response to steroid therapy and prognosis in patients with acquired idiopathic generalized anhidrosis (AIGA). Method: We reviewed the medical records of patients seen at a tertiary neurology clinic between March 2017 and July 2019. Inclusion criteria required the diagnosis of widely distributed anhidrosis, affecting at least 50% of the entire body, in the absence of any other autonomic or neurologic dysfunction. The diagnosis and distribution of generalized anhidrosis were confirmed using thermoregulatory sweat test. Results: Six male patients were included with a mean age at onset of 32 years (range, 16~43 years). All patients received corticosteroid therapy with five consecutive days of intravenous 1g methylprednisolone and oral prednisolone. Five patients showed good response to steroid therapy. However, decreased sweating reappeared in more than a half of patients (N = 3/5) with a mean interval of 32 days (standard deviation, 7.5) until recurrence. The differentiated findings in patients with recurrence compared to those without were anhidrosis over a wider part of body on quantitative sudomotor axon reflex test and decreased heart rate variability to deep breathing. Conclusion: Corticosteroid therapy was effective in improving AIGA symptoms. However, recurrence rates seem to be high after treatment discontinuation and prognostic factors need to be confirmed.