Hemispherectomy for seizures

Hemispherectomy, or hemidecorticectomy as it is more accurately described, has been highly effective in reducing or eliminating medically intractable seizures associated with hemiplegia. Because of late onset postoperative superficial cerebral hemosiderosis and its associated neurologic deterioration, this procedure was all but abandoned for years. With improved surgical techniques and diagnostic testing, some medical centers with a special interest in epilepsy are again using hemispherectomy as a treatment for uncontrolled seizures associated with Rasmussen's encephalitis, as well as other etiologies. Specialized nursing care throughout the hospital course is essential to a positive outcome for the patient and family.     

抗N-甲基-D-天冬氨酸受体脑炎八例分析

目的：探讨抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎患者的临床表现、辅助检查特点、治疗和预后。 方法：回顾性分析我院8例确诊为抗NMDAR脑炎患者的临床资料。结果：5例患者出现前驱症状;所有患者 临床症状均出现快速进展的精神行为异常、认知障碍,4例为首发症状,此外表现有言语障碍、癫痫发作、运 动障碍、意识水平下降及自主神经功能障碍等;8例患者脑脊液抗NMDAR抗体阳性,3例头MRI检查显示异 常病灶,位于大脑皮质、丘脑、海马、脑干等部位。6例患者脑电图异常,为弥漫性慢波或局灶性痫样放电。 所有患者均接受一线免疫治疗,延误诊治的1例患者对治疗反应差并出现复发。结论：抗NMDAR脑炎临床 表现复杂多样,但具有其特点,对于出现不明原因的精神行为异常或认知障碍的青年患者,及时行抗NMDAR抗体筛查十分必要,早期治疗预后良好。     

Four-year-old anti-N-methyl-D-aspartate receptor encephalitis patient with ovarian teratoma: A case report

BACKGROUNDA population-based comparative study in United States shows that the prevalence and incidence of autoimmune encephalitis are comparable to those of infectious encephalitis and its detection is increasing over time. Some patients are complicated with ovarian teratoma. The younger the patient is, the less likely a tumor will be present.CASE SUMMARYThis case report describes the successful treatment of anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis by early laparoscopic ovarian cystectomy and immunotherapy in a 4-year-old female child. And to the best of our knowledge, this detailed case report describes the youngest patient to date with anti-NMDAR encephalitis who underwent laparoscopic ovarian cystectomy.CONCLUSIONAlthough the younger the patient is, the less likely a tumor will be detected, we still emphasize that all patients with suspected or confirmed anti-NMDAR encephalitis should be screened for ovarian tumors if possible. Prompt initiation of immunotherapy and tumor removal are crucial for good outcomes.     

Tick-borne encephalitis: A review of epidemiology,clinical characteristics,and management

Tick-borne encephalitis is an infection of central nervous system caused by tick-borne encephalitis virus transmitted to humans predominantly by tick bites. During the last few decades the incidence of the disease has been increasing and poses a growing health problem in almost all endemic European and Asian countries. Most cases occur during the highest period of tick activity, in Central Europe mainly from April to November. Tickborne encephalitis is more common in adults than in children. Clinical spectrum of the disease ranges from mild meningitis to severe meningoencephalitis with or without paralysis. Rare clinical manifestations are an abortive form of the disease and a chronic progressive form. A post-encephalitic syndrome, causing long-lasting morbidity that often affects the quality of life develops in up to 50% of patients after acute tick-borne encephalitis. Clinical course and outcome vary by subtype of tick-borne encephalitis virus(the disease caused by the European subtype has milder course and better outcome than the disease caused by Siberian and Far-Easter subtypes), age of patients(increasing age is associated with less favorable outcome), and host genetic factors. Since clinical features and laboratory results of blood and cerebrospinal fluid are nonspecific, the diagnosis must be confirmed by microbiologic findings. The routine laboratory confirmation of the tick-borne encephalitis virus infection is based mainly on the detection of specific Ig M and Ig G antibodies in serum(and cerebrospinal fluid), usually by enzyme-linked immunosorbent assay. There is no specific antiviral treatment for tick-borne encephalitis. Vaccination can effectively prevent the disease and is indicated for persons living in or visiting tick-borne encephalitis endemic areas.     

Slow virus diseases of the central nervous system

Slow virus diseases are characterized by a long asymptomatic period, often months or years in duration, between the introduction of the infectious agent and the appearance of clinical illness. Two distinct groups cause serious degenerative diseases of the brain and spinal cord. The first to be identified are those caused by "unconventional agents," kuru and Creutzfeldt-Jakob disease. The second category, "conventional virus diseases," include SSPE (subacute sclerosing panencephalitis), PML (progressive multifocal leukoencephalopathy), progressive rubella encephalitis, and HIV encephalopathy. The universal focus on acquired immune deficiency syndrome (AIDS) has stimulated new research on slow viruses. The extreme neurological deficits, the chronic nature of these diseases, and the possible concern with infection control make patients with these diseases a challenge to nursing.     

Cerebral toxoplasmosis in patients with AIDS

Toxoplasma gondii, a previously rare cause of central nervous system infection, has become a major cause of encephalitis in patients with acquired immunodeficiency syndrome. The diagnosis of cerebral toxoplasmosis should be kept in mind when neurologic signs and symptoms develop in patients with AIDS. Patients with positive serology and characteristic lesions on computed tomographic scans should receive two weeks of antitoxoplasma therapy. Biopsy is recommended for patients with atypical presentation, negative serology, progressive clinical deterioration or differential response of lesions to empiric therapy.     

Plasma exchange in Rasmussen's encephalitis.

The authors observed a 4-year-old girl who has Rasmussen's encephalitis. She started with frequent localized and generalized seizures. Standard antiepileptic treatment was almost ineffective. The frequency of the generalized seizures decreased, but the myoclonic jerks of the left part of the body persisted. An EEG showed partial status epilepticus. The results of the CT scan were normal. Antibodies to viruses were absent from the blood and cerebrospinal fluid. An MR scan showed a T2-weighted hypersignal zone in the right frontal region. Intravenous bolus injections of corticosteroids and drips of immunoglobulins were inefficient, and we started plasma exchanges which have continued for 9 months. The clinical state stabilized, and the images on the MR scan improved, but the results of the EEG did not improve. The authors discuss the effect of the plasma exchange, the use of which is questionable in this disease.     

Encéphalites paranéoplasiques et auto-immunes : quand y penser, comment en faire le diagnostic

The field of limbic paraneoplastic and autoimmune encephalitis has been greatly enhanced during the last decade by the discovery of neuronal cell-surface directed autoimmunity in the serum and/or CSF of limbic encephalitis patients. The main antibodies target the glutamate NMDA receptor and the voltage-gated potassium channel (VGKC), now known to be mainly LGI-1 and CASPR2 proteins. The recent discovery that a number of acute encephalitis of unknown cause are due to anti-NMDAR or anti-VGKC encephalitis has induced a great interest in these pathologies from critical care physicians because they display specific clinical patterns and can be cured by surgical removal of a tumour, if applicable, and immunotherapy. Neurological paraneoplastic syndromes, classic limbic encephalitis, cellsurface antibody-associated encephalitis are described and the most recent literature is reviewed.     

电压门控钾离子通道复合物相关抗体脑炎7例分析

目的:探讨电压门控钾离子通道复合物(VGKC)相关抗体脑炎的临床特点及诊治。方法:回顾性分析VGKC相关抗体脑炎7例的临床资料。结果:患者发病前无前驱症状;出现癫痫发作6例,精神症状3例,认知障碍2例;伴睡眠障碍者4例,心慌、多汗1例,低钠血症者3例,面-肩臂肌张力障碍者1例。脑脊液富亮氨酸胶质瘤失活蛋白1(LGI-1)抗体阳性4例(其中2例同时血清LGI-1抗体阳性),血清LGI-1抗体阳性1例,脑脊液和血清接触蛋白相关蛋白2(Caspr2)抗体阳性1例,血清Caspr2抗体阳性1例。头MRI平扫表现为主要累及颞叶内侧、海马区伴或不伴基底节区T2和FLAIR序列高信号;视频脑电图监测异常3例。均未发现肿瘤。应用丙种球蛋白、甲泼尼龙冲击或二者联合治疗,随访3个月,好转6例,复发、放弃治疗1例。结论:VGKC相关抗体脑炎首发症状多为癫痫,LGI-1脑炎多伴有低钠血症、睡眠障碍,Caspr2脑炎多伴有自主神经功能障碍。头颅MRI不能鉴别脑炎的类型,脑电图无特征性的改变,脑脊液和血清自免脑抗体检测有助于确定诊断。多数病例早期免疫治疗有效,少数病例可复发。     

The neurology of human immunodeficiency virus infection

HIV-infected patients are at markedly increased risk for neurological dysfunction, which may occur at any level of the neuraxis (see Table 1). The most common syndromes--AIDS dementia complex, vacuolar myelopathy, and possibly distal symmetric peripheral neuropathy--appear to be related to HIV infection within the nervous system, rather than due to the immunoincompetence caused by HIV. However, the mechanism(s) by which HIV causes these syndromes, e.g., infecting neurons or oligodendroglia directly, interfering with neurotrophic factors, effecting toxic monokine production, etc., is unknown. Early, albeit incomplete, success with azidothymidine is encouraging. Less commonly, neurological syndromes may be secondary to the immunoincompetence produced by HIV. Many different etiologies--most of which are treatable--have been encountered, but a few of these (cerebral toxoplasmosis, cryptococcal meningitis, primary CNS lymphoma, and progressive multifocal leukoencephalopathy) are responsible for most of the opportunistic complications. Marked differences in symptoms and signs between AIDS patients and immunologically normal patients may complicate recognition of some of these diseases (e.g., herpes simplex encephalitis). Finally, some HIV-associated syndromes, e.g., inflammatory demyelinating polyradiculoneuropathy and retinal microvasculopathy, are of unknown etiology.     

Clinical management of dilated cardiomyopathy: current knowledge and future perspectives

Dilated cardiomyopathy (DCM) is a primary heart muscle disease characterized by a progressive dilation and dysfunction of either the left or both ventricles. The management of DCM is currently challenging for clinicians. The persistent lack of knowledge about the etiology and pathophysiology of this disease continues to determine important fields of uncertainty in managing this condition. Molecular cardiology and genetics currently represent the most crucial horizon of increasing knowledge. Understanding the mechanisms underlying the disease allows clinicians to treat this disease more effectively and to further improve outcomes of DCM patients through advancements in etiologic characterization, prognostic stratification and individualized therapy. Left ventricular reverse remodeling predicts a lower rate of major cardiac adverse events independently from other factors. Optimized medical treatment and device implantation are pivotal in inducing left ventricular reverse remodeling. Newly identified targets, such as angiotensin–neprilysin inhibition, phosphodiesterase inhibition and calcium sensitizing are important in improving prognosis in patients affected by DCM.     

Encéphalites auto-immunes à anticorps antirécepteurs-NMDA, une cause fréquente d’encéphalite en réanimation

Paraneoplastic autoimmune encephalitis results in rapidly evolving symptoms encompassing behavior changes, mood disorders, seizures, memory deficits, and possible decline of consciousness associated to an inflammation of the cerebrospinal fluid (CSF) and abnormalities on cerebral imaging. Among these encephalitis, the recently described anti-NMDA-receptor encephalitis seems more common. According to an epidemiological prospective study, this encephalitis represents up to 4% of all causes of encephalitis; the 5th cause of encephalitis after Herpes simplex virus, Varicelle zona virus (VZV), Mycobacterium tuberculosis infections, and acute demyelinating encephalomyelitis (ADEM), and thus the second cause of autoimmune encephalitis after ADEM. Anti-NMDA-receptor encephalitis predominantly affects young people, especially women. The clinical presentation is characteristic and includes rapidly evolving behavioral changes, prominent psychiatric symptoms with delusion and psychosis, seizures, abnormal movements, autonomic instability, and central hypoventilation. Admission to the intensive care unit is common for hypoventilation or decreased consciousness. Presentation is associated with the presence of an ovarian teratoma in two-third of the cases. Diagnosis is assessed based on the identification of specific antibodies against NMDA-receptor in the CSF, as well as indirect data obtained using electroencephalography and cerebral imaging. Treatment relies on immunotherapy, corticosteroids associated to intravenous immunoglobulins or plasma exchange, and resection of the germinal tumor. If diagnosed and treated early, anti-NMDA-receptor encephalitis has a mortality rate of 4% and a favorable outcome in about two-third of the patients.     

A distinctive clinical EEG profile in herpes simplex encephalitis.

Two patients with herpes simplex encephalitis had clinical courses consisting of an acute febrile illness with early onset of seizures and rapidly progressive neurologic signs evolving to coma and death within 3 weeks. The electroencephalograms of both patients were similar and showed widespread, periodic, stereotyped sharp-and-slow-wave complexes occurring bilaterally over both hemispheres, as well as transient episodes of electrographic seizure activity occurring unilaterally, with suppression of the periodic activity on that side. Although the electroencephalographic findings are not pathognomonic, when associated with an acute encephalitic process, they would suggest the diagnosis of herpes simplex encephalitis.     

Unusual presentation of neurobrucellosis: a solitary intracranial mass lesion mimicking a cerebral tumor

Among the diverse presentations of neurobrucellosis, solitary intracranial mass lesions are extremely rare. To the best of our knowledge, we describe here the second case of neurobrucellosis mimicking a cerebral tumor caused by Brucella melitensis. The mass lesion was clinically and radiologically indistinguishable from a brain tumor. The diagnosis was established by isolating Brucella melitensis in a blood culture and a positive Wright’s agglutination test on the cerebrospinal fluid at 1:320 titers. Paraffin sections of the cerebral mass showed nongranulomatous encephalitis. We suggest that patients with an isolated intraparenchymal mass lesion with nongranulomatous encephalitis should also be studied for brucellosis in endemic areas.     

Antiviral drugs

Acute encephalitis is inflammation of the brain and meninges caused by direct invasion of pathogens into the central nervous system (CNS) (primary encephalitis) or immunopathological responses in the CNS (secondary encephalitis), while acute encephalopathy is characterized clinically by rapidly progressive brain damage following viral infection, and pathologically by brain edema in the absence of direct invasion of the virus and inflammatory cells. A number of pathogens have been associated with acute encephalitis/encephalopathy, including influenza viruses and human herpesviruses, although their involvement in the neuropathogenesis varies. Treatment of acute encephalitis/encephalopathy should be based on the etiological mechanism, and antiviral agents directly act against pathogens of primary encephalitis (e.g., herpes simplex encephalitis) and may alleviate encephalopathy by reducing antigenic burden (e.g., influenza-associated encephalopathy).     

Blood, urine and CSF analysis

This is an interpretative article concerning about the biomarkers of patients with acute encephalitis and acute encephalopathy. Firstly, examinations from blood and/or CSF specimens for the diagnosis of patients with encephalitis are considered using two guidelines. One is the guidelines for the management of encephalitis prepared by an Expert Panel of the Infectious Disease Society of America (IDSA). The other is those prepared by the European Federation of Neurological Societies (EFNS). CSF PCR is most useful to detect the pathogen (usually virus) of acute encephalitis. Secondly, a brief outline of acute encephalopathy associated with viral infections from the standpoint of examination is given. Finally, biomarkers of brain injury are considered through a systematic review in term neonatal encephalopathy.     

Leber's hereditary optic neuropathy: A genetic disorder of the eye

Leber's hereditary optic neuropathy is a rare, maternal linked, genetic disorder that is characterized by progressive bilateral optic nerve degeneration. Male offspring are predominately affected, and blindness occurs during the second or third decade of life. There is no known treatment; however, individuals affected early in life may show some visual recovery in later years. These patients require a supportive and caring environment to assist them; as this disease often results in devastating life style changes.     

抗γ-氨基丁酸B受体脑炎的临床与影像学特点

目的 探讨抗γ-氨基丁酸B(GABA-B)受体脑炎患者的临床表现、实验室检查及影像学特点。方法 选取我院2021年5月～2022年11月临床资料完整、符合《中国自身免疫性脑炎诊治专家共识（2022年版）》中自身免疫性边缘性脑炎的诊断标准,并经免疫学检查确诊为抗GABA-B受体脑炎的患者3例,收集其临床表现、脑脊液检查、免疫学检查、脑电图、影像学检查等资料进行回顾性分析。结果 3例患者均急性起病,以癫痫发作为首发症状或主要临床表现,伴不同程度的认知功能障碍及精神行为异常。3例患者发病时均合并肿瘤,分别为多发性骨髓瘤、肺癌及鼻咽癌。脑脊液及血清免疫学提示脑脊液和（或）抗GABA-B受体抗体阳性。脑电图均异常,呈癫痫样放电、散在分布或弥漫性慢波。影像学检查见病灶累及单侧海马,头颅CT呈低密度影,头颅MRI呈长T1、长T2信号,T2 FLAIR高信号,DWI高信号,ADC呈等或稍高信号。结论 抗GABA-B受体脑炎患者以癫痫发作为主要表现,易合并肿瘤。抗GABA-B受体脑炎合并鼻咽癌为首次报道,扩展了疾病谱。     

Clinical symptoms,diagnosis, treatment,and outcome of COVID‐19‐associated encephalitis: A systematic review of case reports and case series

IntroductionSince COVID‐19 outbreak, various studies mentioned the occurrence of neurological disorders. Of these, encephalitis is known as a critical neurological complication in COVID‐19 patients. Numerous case reports and case series have found encephalitis in relation to COVID‐19, which have not been systematically reviewed. This study aims to evaluate the clinical symptoms, diagnosis, treatment, and outcome of COVID‐19‐associated encephalitis.MethodsWe used the Pubmed/Medline, Embase, and Web of Science databases to search for reports on COVID‐19‐associated encephalitis from January 1, 2019, to March 7, 2021. The irrelevant studies were excluded based on screening and further evaluation. Then, the information relating diagnosis, treatment, clinical manifestations, comorbidities, and outcome was extracted and evaluated.ResultsFrom 4455 initial studies, 45 articles met our criteria and were selected for further evaluation. Included publications reported an overall number of 53 COVID‐19‐related encephalitis cases. MRI showed hyperintensity of brain regions including white matter (44.68%), temporal lobe (17.02%), and thalamus (12.76%). Also, brain CT scan revealed the hypodensity of the white matter (17.14%) and cerebral hemorrhages/hemorrhagic foci (11.42%) as the most frequent findings. The IV methylprednisolone/oral prednisone (36.11%), IV immunoglobulin (27.77%), and acyclovir (16.66%) were more preferred for COVID‐19 patients with encephalitis. From the 46 patients, 13 (28.26%) patients were died in the hospital.ConclusionIn this systematic review, characteristics of COVID‐19‐associated encephalitis including clinical symptoms, diagnosis, treatment, and outcome were described. COVID‐19‐associated encephalitis can accompany with other neurological symptoms and involve different brain. Although majority of encephalitis condition are reversible, but it can lead to life‐threatening status. Therefore, further investigation of COVID‐19‐associated encephalitis is required.     

Neurologic disease as the presenting manifestation of acquired immunodeficiency syndrome

Neurologic disease was the harbinger of the acquired immunodeficiency syndrome (AIDS) in 26 (20%) of 132 patients and ultimately developed in 83 (63%). The most common neurologic disorder heralding AIDS was toxoplasmic encephalitis (17 cases) followed by cryptococcal meningitis (six), retinitis (three), progressive multifocal leukoencephalopathy (two), neuropathy (one), myopathy (one), and subacute "viral" encephalitis (one). In four patients, more than one neurologic disorder coexisted at presentation. Recognition of underlying human T cell lymphotropic virus type III (HTLV-III) infection may allow a more expedient determination of the cause of the neurologic disease. Our study emphasizes the importance of testing for the presence of HTLV-III antibody and assessing the immunologic status of all patients at risk for AIDS who have neurologic illness.