The Association of Ovarian Teratoma and Anti-N-Methyl-D-Aspartate Receptor Encephalitis: An Updated Integrative Review.

Ovarian teratomas are by far the most common ovarian germ cell tumor. Most teratomas are benign unless a somatic transformation occurs. The designation of teratoma refers to a neoplasm that differentiates toward somatic-type cell populations. Recent research shows a striking association between ovarian teratomas and anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, a rare and understudied paraneoplastic neurological syndrome (PNS). Among teratomas, mature teratomas are thought to have a greater relevance with those neurological impairments. PNS is described as a neurologic deficit triggered by an underlying remote tumor, whereas anti-NMDAR encephalitis is characterized by a complex neuropsychiatric syndrome and the presence of autoantibodies in cerebral spinal fluid against the GluN1 subunit of the NMDAR. This review aims to summarize recent reports on the association between anti-NMDAR encephalitis and ovarian teratoma. In particular, the molecular pathway of pathogenesis and the updated mechanism and disease models would be discussed. We hope to provide an in-depth review of this issue and, therefore, to better understand its epidemiology, diagnostic approach, and treatment strategies.

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.

Anaesthetic management of a patient with a unique combination of anti-N-methyl-D-aspartate receptor encephalitis and stiff-person syndrome.

Stiff-person syndrome (SPS) and anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are rare paraneoplastic syndromes caused by antibodies that target the central nervous system. Here, we describe a 26-year-old woman who presented with psychosis, amnesia, rigidity and fever. After extensive diagnostic and laboratory workup, she was diagnosed with an ovarian teratoma which was causing the symptoms of anti-NMDAR encephalitis and SPS. The patient was successfully treated with laparoscopic removal of the ovarian tumour under general anaesthesia. She was placed on immunosuppressant medications preoperatively and postoperatively, and her symptoms gradually resolved. Although there are case reports regarding the anaesthetic management of SPS and anti-NMDAR encephalitis, our study is the first report of a patient afflicted with both conditions.

A case of reversible anti-NMDA-receptor encephalitis: neuropsychological and neuroradiological features.

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune encephalitis mainly affecting young women. We report a case of a mild paraneoplastic anti-NMDAR encephalitis in a 31-year-old female with an ovarian immature teratoma. The patient exhibited a severe short-term episodic memory impairment and psychiatric symptoms. A detailed diagnostic work-up including complete clinical and laboratory examinations, neuropsychological assessments, and neuroradiological investigations has been done at the onset and during follow-up. The amnestic syndrome and MRI medial-temporal abnormalities reversed after medical and surgical treatment. The present report indicates that the disease can be rapidly reversible if promptly diagnosed and treated. While the disease has already been described elsewhere, the course of neurospychological deficits in adults is not as much known. Usually, when the diagnosis of anti-NMDAR encephalitis is made, the severity of the disease makes the assessment of the neuropsycological profile particulary challenging. The present report is of interest because it describes the complete neuropsychological profile of a mild form of anti-NMDAR encephalitis.

Assessment of pain and stress intensity among women with ovarian endometriomas versus teratomas.

BACKGROUND: In recent years, numerous studies have considered endometriosis to be a subclinical, local inflammatory process in the pelvic peritoneum, the main symptom of which is pain. OBJECTIVES: To assess pain intensity and pain-related stress in women with ovarian endometriomas versus teratomas. METHODS: In total, 860 women (18 to 38 years of age) treated laparoscopically for lesions in the adnexa between September 2006 and November 2013 were included in the present study. After an intraoperative review of their histopathological lesions, the patients were divided into two study groups: group E (n=480), with histopathologically confirmed ovarian endometriomas; and group T (n=380), after laparoscopic treatment of ovarian teratomas. A questionnaire was generated for the study and completed by each group. Statistical analysis was performed using the Mann-Whitney U test (P≤0.05). RESULTS: Median pain scores for group E versus group T were as follows: pain during menstruation, 6 versus 3 (P=0.001); pain outside of menstruation (in professional life), 2 versus 2 (P=0.014); and pain during sexual intercourse, 3 versus 1 (P=0.006). Pain-related stress scores were higher in group T versus group E (5 versus 3; P=0.007). CONCLUSION: Ovarian endometriomas caused more pain than ovarian teratomas, likely due to the endometrial tissue component and not a mass effect. The assessment of pain and pain-related stress associated with the pelvis minor showed a high level of pain intensity and lower level of pain-related stress among patients with ovarian endometriomas.

Neurocognitive evaluation of long term survivors of atypical teratoid rhabdoid tumors (ATRT): The Canadian registry experience.

BACKGROUND: Because atypical teratoid rhabdoid tumor(ATRT) is a rare disease of infancy carrying a grim prognosis, focus on long-term outcome, especially neurocognitive remained very limited. With new era of multimodality therapy, an increasing proportion of patients are now long-term survivors. PROCEDURE: Retrospective review of neuropsychological (NP) status of survivors from the Canadian ATRT registry. RESULTS: Among 77 patients diagnosed between 1995-2012, 16(22%) were survivors. Formal NP assessments were available in eight patients. Partial information on academic achievement was available on three additional patients. There were four girls and seven boys diagnosed at a median age of 27.5 months. Seven patients underwent complete resection and three had metastatic disease. All but one received sequential high dose chemotherapy. Five patients received intrathecal chemotherapy. Three patients underwent radiation. Median age at time of formal NP assessment was 7.6 years (3.9-9.8). Full Scale Intellectual Quotient (FSIQ) ranged from less than 50-119 (mean 78). Simple expressive and receptive language appeared relatively preserved. Three recently diagnosed patients (median time assessment from diagnosis 2.6 years (1.6-4.7)) had average to high average FSIQ, academic and visual spatial skills, visual, and verbal memory. Five other patients diagnosed earlier and tested at a median time of 4.9 years (3.3-8.3) post-diagnosis had a FSIQ ranging from <50 to 71. Approximately 50% of their scores were in the impaired range. CONCLUSION: Overall this cohort appears significantly impaired at school age despite the absence of systematic radiotherapy. Larger series focusing on neurocognitive outcome are needed in the current context where treatment strategies include adjuvant radiation.

Psicosis parece, encefalitis es (o un caso de inicio con síntomas psiquiátricos en una encefalitis límbica) / Psychiatric symptoms as initial presentation in limbic encephalitis: case report

Introducción: La encefalitis límbica esuna enfermedad autoinmune y con frecuencia paraneoplásica,caracterizada por síntomas neuropsiquiátricosy asociada con frecuencia a diferentes tumorescomo el cáncer de células pequeñas de pulmón, timoma,teratoma testicular o cáncer de mama. Se hanidentificado varios autoanticuerpos, uno de los cualeses anti-NMDAR, asociado con más frecuencia a teratomaovárico y síntomas psiquiátricos en pacientesjóvenes. Caso clínico: Una paciente de 14 años conalucinaciones auditivas, problemas de lenguaje, agitación,movimientos anormales y pobre respuesta aantipsicóticos es finalmente diagnosticada de encefalitisanti-NMDAR, encontrando y tratando despuésen ella un teratoma ovárico. Discusión: La encefalitisanti-NMDAR es tratada con frecuencia primero porpsiquiatras por su presentación con cambios de personalidad,alucinaciones, agitación etc. Los psiquiatrasdeberíamos conocer esta enfermedad autoinmune paradar a los pacientes un correcto y temprano diagnósticoy tratamiento (AU)

Introduction: Limbic encephalitis isan autoimmune and often paraneoplastic disorder,characterized by neuropsychiatric symptoms andfrequently associated with several tumors such assmall cell lung cancer, thymoma, testicular teratomaor breast cancer. Several autoantibodies havebeen identified linked to limbic encephalitis, i.e.the anti-NMDAR, frequently associated with ovarianteratoma and psychiatric symptoms in youngpatients. Case report: A 14-year-old girl with auditoryhallucinations, speech problems, agitation,abnormal movements and poor response to antipsychoticswas diagnosed of anti-NMDAR encephalitis,and there after an ovarian teratoma wasdetected and treated. Discussion: Anti-NMDARencephalitis is often first treated by psychiatristsbecause of its presentation with personality changes,hallucinations, agitation etc. Psychiatristsshould consider this autoimmune disorder in orderto give patients correct and early diagnosis andtreatment (AU)

The long-term psychosocial outcomes following excision of sacrococcygeal teratoma: a national study.

OBJECTIVE: The overall effect of the reported long-term sequelae following sacrococcygeal teratoma (SCT) excision on the psychosocial and sexual development has not been addressed appropriately in the literature. The aim of this study was to evaluate the psychosocial adjustment of a national cohort of SCT patients using a validated psychosocial questionnaire. METHODS: Three paediatric surgery centres in Scotland were contacted to identify those SCT patients who were now 5 years or older. The main outcome measure was the Derriford Appearance Scale 59 (DAS-59) which assesses concerns of everyday living, personal relationships, self-esteem and emotional distress. Following ethical approval, age appropriate invitation letters and information sheets for both parents and patients were sent to prospective participants. Parents/patients were contacted after 2 weeks to arrange an interview to complete the questionnaire. RESULTS: 31/48 (65%) of patients identified with SCT completed the DAS-59. Participant ages ranged from 5 to 35 years (median 12 years). 5/31 (16%) were malignant cases. There were 25 (81%) female participants and 12 (39%) patients older than 16 years. 9/31 (29%) participants indicated concern over their appearance. However, we found low levels of appearance-related distress and overall participants showed positive adjustment to personal relationships and everyday living on the DAS-59. CONCLUSIONS: This is the first study looking at the psychosocial adjustment of patients with SCT using a validated psychosocial questionnaire. Encouragingly, we found low levels of appearance-related distress. Future research could identify those factors associated with increased risk of poorer outcomes and highlight those in need of psychological intervention.

[Psychiatric symptoms of a paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis: A case report]. / Symptômes psychiatriques d'une encéphalite paranéoplasique à anticorps antirécepteurs NMDA : à propos d'un cas.

INTRODUCTION: We describe the case of a young woman affected by a benign ovarian teratoma with paraneoplastic encephalitis. Several cases have already been reported, but it is the first article that focuses on details of the psychiatric symptoms of this disorder. BACKGROUND: Paraneoplastic encephalitis usually begins with a prodromal phase, followed first by prominent psychiatric symptoms or, less frequently, short-term memory loss, seizure, catatonia-like symptoms, dyskynesias and, secondly, by autonomic instability and central hypoventilation requiring intensive care. In our case and to our knowledge, for the first time in the literature, the patient was hospitalized in a psychiatric unit for a suspected manic episode with psychotic features, in association with short-term memory impairment and anxiety. It has been shown that patients suffering from paraneoplastic encephalitis associated with ovarian teratoma display antibodies for anti-N-methyl-D-aspartate (NMDA) receptors in CSF or plasma (more specifically for the NR1 subunit of the NRl/NR2 heteromers required to form a functional NMDA receptor). The NR1/NR2B heteromers are preferentially expressed in the adult hippocampus/forebrain, which are brain regions involved in the pathogenesis of various psychiatric, psychotic in particular, symptoms. Furthermore, the glutamatergic NMDA receptors are the major mediator of excitotoxicity and their dysfunction had been associated with neurologic disorders, but also with schizophrenia and, more recently, with mood disorders. CASE REPORT: This case supports the idea that the dysfunction of NMDA receptors may play a major role in psychiatric disorders, especially in psychosis and affective disorders. This article will briefly summarize the different evidences and hypotheses reported in the literature on NMDA receptors implication and will report how these receptors may serve as therapeutic targets.

[From psychiatric symptoms to paraneoplastic syndrome]. / Van psychiatrisch symptoom tot paraneoplastisch syndroom.

Two patients, a 38-year-old man and a 32-year-old woman, were admitted to a psychiatric ward. The first patient suffered from a mood disorder, personality changes and complained of several, hitherto unexplained physical symptoms. Finally the patient was diagnosed with paraneoplastic cerebellar degeneration associated with Hodgkin's disease. The second patient presented with psychosis and panic disorders, but the condition was later found to be caused by paraneoplastic limbic encephalitis due to ovarian teratomas. These cases illustrate that patients with paraneoplastic neurological syndromes may present with psychiatric symptoms which can hamper an early diagnosis.

Searching for safety signals: the experience of medical surveillance amongst men with testicular teratomas.

The aim of this study is to compare the experience of a group of men with Stage 1 testicular teratomas who were being managed through a surveillance programme (n=25) with a group of patients who had received chemotherapy for more advanced disease (n=22). The study employed a two-phase sequential design that combined quantitative and qualitative methods of data analysis. In the first phase, the hospital anxiety and depression scales (HADS) were used to screen for psychological morbidity. Twelve (48%) of the men assigned to the surveillance programme scored in the 'borderline' or 'clinical case' range on the anxiety subscale of the HADS, compared with six (27%) in the chemotherapy group. There was a significant negative correlation in the surveillance group between 'time since diagnosis' and an elevated anxiety subscale score on the HADS. Interviews were then conducted with 25 participants; a grounded theory approach was used to analyse the transcripts. The hypothesis that human beings are seekers of safety signals provided an explanatory model to account for the higher incidence of self-reported anxiety amongst the men in the surveillance programme.

Psychosocial adjustment after testicular cancer treatment.

PURPOSE: The high cure rate in testicular cancer has provoked investigations relating to the quality of life in long-term survivors. We determine the psychosocial consequences of the disease especially in regard to sexual and professional performance. MATERIALS AND METHODS: Among the testicular cancer patients treated with various treatment modalities 140 rendered free of disease for at least a year were included in this study. General Health Questionnaire 28 and a general survey were used to determine quality of life issues. RESULTS: Regarding the sexual life of these patients, problems related to libido, erection and ejaculation increased significantly during treatment and subsequently recovered but did not return to baseline after treatment. During treatment the frequency of sexual intercourse and/or masturbation decreased significantly in all patients. Of the single patients 35% thought that medical history would be a concern for the potential spouse. Regarding professional lives, 22.4% thought that they had better performance after treatment compared to before therapy, whereas only 6.1% reported it to be worse. When professional performance was analyzed according to the treatment modalities those who had received radiotherapy did worse. General Health Questionnaire scores indicated that patients with this disease had a positive view of life compared to that of the normal population. CONCLUSIONS: Although we observed a substantial recovery in sexual life after treatment, it was evident that therapy did have a negative effect on sexual functions. There was no effect on occupational performance and perspective of life, which may be related to the fact of having overcome a life threatening disease.

Paraneoplastic limbic encephalitis with immature ovarian teratoma--a case report.

Paraneoplastic limbic encephalitis (PLE) is a remote, nonmetastatic complication of carcinoma. Neuropsychiatric symptoms usually predate the diagnosis of cancer by 3 months to 6 years and very rarely the symptoms develop after the diagnosis of malignancy. We report the first case of limbic encephalitis associated with an immature ovarian teratoma. Within the month following the diagnosis of the tumor with pathologic stage Ia, somewhat acutely she developed neuropsychiatric symptoms that was exclusively a limbic disorder with impairments in almost every realm of limbic function. This case may show us that it is important to recognize the neuropsychiatric symptoms of PLE as the first manifestation of a very small malignant ovarian tumor and to aggressively try to identify the underlying cancer.

Quality of life after treatment for testicular cancer--the patient's view.

Twenty-eight patients cured of testicular cancer by cisplatin-based chemotherapy were asked for their own views of the long-term psychological and social effects of their treatment. Their views were compared with a group of 34 testicular cancer patients cured by radiotherapy who were matched for age, social class and time since treatment. A category rating type questionnaire was used with questions concerning general health, subjective side-effects of treatment, employment, relationships, reproduction and mood. The principal differences were (1) the chemotherapy group reported a greater prevalence of physical side-effects, (2) the radiotherapy group reported greater anxiety and depression since treatment and (3) a significant number of patients in the chemotherapy group felt that their illness had had beneficial effects on their relationships with family and friends.