Thymectomy for treatment of anti-NMDA receptor encephalitis.

In this case report, we present a 25-year-old woman who was diagnosed with anti-N-methyl-D-aspartate (NMDA) receptor encephalitis and subsequently was found to have thymic hyperplasia. She underwent robotic bilateral access thymectomy for treatment of her anti-NMDA receptor encephalitis and has remained asymptomatic after her operation without any medication. This is only the second reported case of thymectomy for treatment of this condition. This case may further suggest thymic tissue is involved in the development of anti-NMDA receptor encephalitis and that thymectomy is an appropriate treatment for a subset of patients with this disease.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated with Ovarian Teratoma in South China-Clinical Features, Treatment, Immunopathology, and Surgical Outcomes of 21 Cases.

OBJECTIVE: To study the clinical characteristics and surgical outcomes of anti-NMDAR encephalitis and the immunopathology of associated teratomas. METHODS: Twenty-one patients were enrolled in this retrospective study, who were diagnosed with anti-NMDAR encephalitis with ovarian teratoma and admitted to two tertiary hospitals in South China from July 2014 to December 2019. The clinical data of patients were reviewed. Comparisons were made between the patients with different outcomes after surgery. Immunohistochemical analyses of associated ovarian teratomas were performed. RESULTS: The mean age of the patients was 24.33 ± 5.12 years. The peak seasons of disease onset were autumn and winter (30.61% and 32.65%). The symptoms could be divided into 8 categories, including psychiatric abnormalities, seizures, movement dysfunction, consciousness disorders, autonomic dysregulation, speech disturbance, central hypoventilation, and memory deficits. All patients developed four or more categories of symptoms within the first four weeks. Twelve patients (57.1%) had a maximum mRS of 5, and 11 patients (52.4%) were admitted to ICU. Twenty patients received surgery, and only 3 patients were diagnosed pathologically with immature ovarian teratomas, while the other 17 patients had mature ovarian teratomas. After surgery, 17 patients (85.0%) got clinical improvement. The central hypoventilation symptom and mature ovarian teratomas were associated with surgical outcome. Immunohistochemical analysis revealed that there were NMDAR-positive neural tissues in all 8 teratomas and in which 3 cases also contained large numbers of NMDAR-positive sebaceous glands and squamous epithelial tissues. CONCLUSION: The disease is of high prevalence in autumn and winter. The central hypoventilation symptom and mature ovarian teratomas were associated with surgical outcome. NMDAR-positive neural tissue is not the only etiological factor of encephalitis. We speculate that encephalitis development in some patients may result from NMDAR expression in sebaceous glands and squamous epithelial tissues.

Battey's operation as a treatment for hysteria: a review of a series of cases in the nineteenth century.

Ovarian resection as a treatment for hysteria, called 'Battey's operation' or 'normal ovariotomy', was performed in the nineteenth century. Battey later reported that the resected ovaries appeared to have 'cystic degeneration'. Currently, patients with acute neuropsychiatric symptoms are screened for teratomas for the differential diagnosis of anti-NMDA receptor encephalitis. There is now a hypothesis that ovarian lesions resulting in paraneoplastic encephalitis were among the patients who underwent Battey's operation. We identified 94 published cases of Battey's operation for neuropsychiatric symptoms in the late nineteenth century. Among 36 cases with detailed descriptions, we found 3 patients who showed acute onset neuropsychiatric symptoms with macropathological ovarian findings that were compatible with teratoma. They showed favourable prognoses after surgery and might have motivated the surgeons to perform the operation.

Resection of melanocytic nevi as a potential treatment of anti-NMDAR encephalitis patients without tumor: report of three cases.

The most common underlying tumor associated with anti-N-methyl D-aspartate-receptor (NMDAR) encephalitis is ovarian teratoma. Resection of the underlying tumor may decrease exposure of autoantigen and make for faster response of immunotherapy and less relapse frequency. Similar to teratoma, expression of NMDAR in human epidermal melanocytes was suspected recently. The dense melanocytes in melanocytic nevus may serve as potential autoantigens and are prone to increase relapse frequency in the tumor-negative patients. Three patients with confirmed diagnosis of anti-NMDAR encephalitis were described here. They shared common features that the screening tests for an ovarian teratoma or other tumors were all negative, while they were found to have prominent melanocytic nevi on the skin and resection of the nevi likely played a positive effect on their persistent recovery. This is a report on treatment of anti-NMDAR encephalitis patients without underlying tumor through resection of melanocytic nevi. More clinical and experimental investigations are needed to prove its validity.

Postherpetic Anti-N-methyl-D-aspartate Receptor Encephalitis after Hemispherotomy in a Patient with Intractable Startle Epilepsy.

Herpes simplex encephalitis (HSE) has been increasingly reported after neurosurgical procedures, mostly after tumor resections in patients with a prior history of HSE. Early detection and appropriate treatment are essential to prevent high mortality of the disease; however, there are diagnostic difficulties due to nonspecific prodromal symptoms. In addition, anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been reported after HSE as an immunological relapse. Here, we report a case of postherpetic anti-NMDAR encephalitis following right hemispherotomy for intractable startle-induced seizures, to emphasize the importance of early diagnosis and appropriate treatment. To our knowledge, this is the first reported case of anti-NMDAR encephalitis after postoperative HSE, and the third reported case of hemispherotomy as a curative treatment for startle epilepsy.

Recidiva de teratoma ovárico tras encefalitis anti-NMDA y manejo de la fertilidad: caso clínico / Ovarian teratoma recurrence after anti-NMDA encephalitis and fertility management: a clinical case

RESUMEN CASO CLÍNICO: la encefalitis anti receptor N-metil D-aspartato (NMDAR) es un trastorno autoinmune con un amplio espectro de síntomas neuropsiquiátricos. Se presenta el caso de una mujer de 22 años con una encefalitis anti-NMDA que cursó con amnesia, crisis parciales complejas y alteraciones del comportamiento asociado a un teratoma ovárico. La evolución fue adecuada con cirugía e inmunosupresores. A los tres años se objetivó un teratoma contralateral, sin recidiva de encefalitis; que fue extirpado tras estimulación ovárica para criopreservación de ovocitos. CONCLUSIONES: el teratoma ovárico debe ser sospechado ante la presencia de una encefalitis atípica. Antes de realizar cirugías ováricas repetidas, debe valorarse la opción de vitrificación ovocitaria si los deseos genésicos de la mujer no están cumplidos.

ABSTRACT CLINICAL CASE: anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis is an autoimmune disorder with a wide sprectrum of neuropsyquiatric symtoms. A case of a 22 years old woman with NMDAR encephalitis presenting with amnesia, focal complex seizures and behavior disturbances associated with an ovarian teratoma is reported. Three years after the first episode, a contralateral teratoma was observed, with no recurrence of the encephalitis; teratoma excision was performed, after controlled ovarian stimulation for oocyte cryopreservation. CONCLUSIONS: ovarian teratoma must be suspected when atypical encephalitis occurs. Before performing repetead ovarian surgeries, oocyte vitrificaction must be considered as a fertility-sparing option in women who have not completed their childbearing wishes.

NMDA receptor encephalitis with cancer of unknown primary origin.

PURPOSE: N-methyl-D-aspartate receptor (NMDAR) encephalitis may present as a paraneoplastic syndrome in young women and is often associated with ovarian teratoma. METHODS: We report 2 male cases of NMDAR encephalitis presenting with metastatic cancer of unknown primary origin. RESULTS: Both patients showed cognitive dysfunction as well as other neurological symptoms, slow waves on EEG, and NMDAR antibodies in sera and CSF. Symptoms were effectively treated by pulse steroid and intravenous immunoglobulin treatment. The patients developed metastatic small cell neuroendocrine carcinoma of the parotid gland and inguinal metastatic squamous cell cancer shortly after their neurological episodes. Follow-up PET studies showed small cell lung cancer in the first patient while no primary origin could be found in the second patient. CONLUSIONS: Our cases imply that NMDAR encephalitis may present with metastatic cancers that display slow progression rates and occur after encephalitis attacks.

A case of anti-NMDAR encephalitis induced by ovarian teratoma.

A young woman was received in the hospital with gradually worsening neurological symptoms consistent with encephalitis. After several cerebrospinal fluid examinations, infectious encephalitis (viral, bacterial, or linked to tuberculosis or parasites) and autoimmune encephalitis were ruled out. The possibility of autoantibody-mediated encephalitis was considered, and anti-N-methyl-D-aspartate receptor (anti-NMDAR) antibodies were detected in cerebrospinal fluid and blood, thus confirming the diagnosis of anti-NMDAR encephalitis. After initial successful treatment, the disease relapsed and the repeated ultrasound investigation revealed teratoma in the left ovary, which was not observed at initial examination. Tumor was removed by laparoscopic oophorocystectomy, and the treatment with hormones and gamma globulin was continued after the surgery. The patient's conditions were gradually improving after the treatment. Correct diagnostic and prompt treatment of anti-NMDAR encephalitis remains a serious clinical challenge due to its unspecific manifestations and varying response to treatments. This article describes the details of a recent complicated case of a patient with this condition. The information will be of interest to clinicians working with encephalitis patients.

Resective surgery in the treatment of super-refractory partial status epilepticus secondary to NMDAR antibody encephalitis.

BACKGROUND: Anti-NMDAR encephalitis is an increasingly described clinical entity in children, comprising 40% of all cases. We present a case of super-refractory status epilepticus secondary to anti-NMDAR encephalitis treated with emergent resective surgery. CASE STUDY: A 7 years-old boy presented with progressive abnormal irritability. On the day after admission he had multiple seizures, characterized by head and eye version to the right. EEG revealed left parietal-occipital continuous paroxysmal activity. Anti-NMDAR antibodies were positive in CSF and serum. After almost 3 months in the Intensive Care Unit, in barbituric coma, and given the failure of all treatment regimens, a preoperative evaluation was conducted. Ictal SPECT showed significant hiperperfusion and brain FDG-PET a cortical hypometabolism in the left occipital lobe; a left occipital lobectomy was performed. In the next days it was possible to progressively suspend Thiopental. Currently, patient presents right homonymous hemianopsia, eats by his own hand but needs help in almost all other activities. DISCUSSION: Status epilepticus (SE) in the setting of anti-NMDAR encephalitis is unusual but described. Whilst the role of surgery in the management of refractory focal epilepsy is established, it is seldom used in the treatment of SE. In the patient with refractory SE (RSE), awareness of surgery as a potentially life saving treatment is an important issue. To our knowledge, this is the first report of a partial RSE secondary to anti-NMDAR encephalitis treated with resective surgery and illustrates the need to consider anti-NMDAR encephalitis as a cause of super-refractory SE.

Clinically significant response to zolpidem in disorders of consciousness secondary to anti-N-methyl-D-aspartate receptor encephalitis in a teenager: a case report.

BACKGROUND: Anti-N-methyl-d-aspartate receptor encephalitis has been associated with a prolonged neuropsychiatric phase that may last for months to years. PATIENT: We report the case of a 16-year-old girl who was diagnosed with anti-N-methyl-d-aspartate receptor encephalitis resulting from left ovarian mature teratoma 2 weeks after presentation with psychosis. Following tumor removal and immunotherapy, recovery from a minimally conscious state was accelerated significantly by zolpidem that was used for her sleep disturbance. Our patient was discharged home 8 weeks after admission with marked improvement in her neurological function. Zolpidem has been reported to improve arousal in disorders of consciousness but there are no previous reports of its benefit among patients with anti-N-methyl-d-aspartate receptor encephalitis. CONCLUSION: Zolpidem would be a reasonable consideration as an adjunctive treatment in anti-N-methyl-d-aspartate receptor encephalitis after tumor removal and immunotherapy to accelerate recovery and rehabilitation.

Anti-N-methyl-aspartate receptor encephalitis in identical twin sisters: role for oophorectomy.

BACKGROUND: Anti-N-methyl-aspartate receptor encephalitis is a potentially fatal form of encephalitis and frequently associated with ovarian teratomas. Surgical removal of ovarian teratomas improves clinical outcome, but it is unclear whether bilateral salpingo-oophorectomy for normal-appearing ovaries is of clinical benefit. CASE: Our report describes a unique clinical scenario of identical twin sisters with anti-N-methyl-aspartate receptor encephalitis. Neither patient responded to immunosuppressive therapy. Imaging studies showed normal-appearing ovaries. The first twin continued on medical therapy only and died of the disease. The second twin underwent a bilateral salpingo-oophorectomy followed by gradual recovery. CONCLUSION: Based on our experience in two genetically identical individuals, we suggest considering the removal of normal-appearing ovaries in patients with anti-N-methyl-aspartate receptor encephalitis who fail to respond to medical treatment.

Anesthetic management and implications of pediatric patients with a diagnosis of anti-N-methyl-D-aspartate receptor encephalitis: two case reports.

This article discusses the anesthetic management and implications of 2 pediatric patients with a diagnosis of anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. Anti-NMDA receptor encephalitis has been described as an immune-meditated syndrome that triggers the production of antibodies to the NMDA receptor: a site of action for many commonly used anesthetic agents. Symptoms of this disease can be autonomic, neurologic, and psychological in nature. This disease process can pose a challenge to the anesthesia provider during all stages of the anesthetic. Thus, the anesthesia provider must incorporate an understanding of the administered anesthetic agent's potential pharmacologic effect on the affected NMDA receptor when formulating the patient's anesthetic plan.

Encefalitis por anticuerpos contra el receptor anti-N-metil-D-aspartato debido a teratoma ovárico / Anti-NMDA receptor encephalitis due to an ovarian teratoma

La presencia de anticuerpos dirigidos contra el receptor de N-metil-D-aspartato se ha asociado a un síndrome neurológico que se caracteriza por alteraciones de la consciencia, movimientos involuntarios y convulsiones. Se considera un síndrome paraneoplásico que se relaciona en las mujeres jóvenes con los tumores de ovario, pero puede ocurrir en ausencia de neoplasia. En la mayoría de los casos, la disautonomía, complejos movimientos involuntarios, hipertermia y insuficiencia respiratoria, requiere tratamiento en las unidades de cuidados intensivos. La resección del tumor conduce a la completa recuperación de las secuelas neurológicas a largo plazo (AU)

The presence of antibodies against the N-methyl-D-aspartate receptor has been associated with a neurological syndrome characterized by alterations of consciousness, involuntary movements, and seizures. These alterations are considered to be a paraneoplastic syndrome primarily associated with ovarian tumors in young women but can occur in the absence of neoplasia. In most patients, dysautonomia, complex involuntary movements, hyperthermia and respiratory failure require treatment in intensive care units. Tumoral resection of the tumor leads to complete neurological recovery in the long term (AU)

Not hysteria: ovarian teratoma-associated anti-N-methyl-D-aspartate receptor encephalitis.

We report a case of a 33-year-old nulliparous woman who, following a short prodromal illness, experienced a series of psychiatric and behavioural symptoms. These included states of terror, insomnia, delirium, self-harm and suicidal ideation, facial dyskinesias, verbigeration, cognitive impairment, reduced responsiveness, violence and paranoia. A diagnosis of anti-N-methyl-d-aspartate (NMDAR) encephalitis was made 50 days after symptom onset. Early tumour removal is associated with an improved prognosis and a laparoscopic oophorectomy was performed following detection of a dermoid cyst. Within 24 hours of the operation there was marked improvement in cognitive function and appetite.

A multidisciplinary approach to the treatment of anti-NMDA-receptor antibody encephalitis: a case and review of the literature.

Anti-NMDAR (N-methyl-d-aspartate receptor) encephalitis is a novel autoimmune and paraneoplastic disease often presenting as acute psychosis. Few studies exist in the psychiatric literature on neuroimmunity and behavioral management. This article reviews the epidemiology, diagnosis, pathophysiology, and management of this disease from a neuropsychiatric perspective. Patients have potential for near-complete recovery with early diagnosis and intervention. In addition to immune-suppression and tumor removal, electroconvulsive therapy may be an important tool in treatment of the underlying process in cases developing life-threatening catatonia. Psychiatrists should be familiar with treatment options, since they may be consulted within the context of a multispecialty team.

Anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian immature teratoma.

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a treatment-responsive encephalitis associated with anti-NMDAR antibodies. Unlike classic paraneoplastic encephalitis, this disorder usually develops in young women with ovarian teratoma who typically present with marked neuropsychiatric symptoms, followed by prolonged respiratory failure, clouding of consciousness, and bizarre dyskinesia. This disorder is often treatable by resection of ovarian tumor and immunotherapy, but, delayed diagnosis results in a worse condition and sometimes fatal outcome. However, some gynecologists are not familiar with this disorder. When physicians encounter a female patient with encephalitis showing marked neuropsychiatric symptoms, search for an ovarian tumor should be promptly initiated. We present a case of anti-NMDAR encephalitis associated with ovarian immature teratoma. The symptoms were dramatically relieved by tumor resection and immunotherapy.