El síndrome de Ofelia describe la asociación entre pérdida de memoria y enfermedad de Hodgkin, en memoria del personaje de La Tragedia de Hamlet, Príncipe de Dinamarca, de William Shakespeare. One was later diagnosed with breast cancer and the other with lung cancer. (Funded by the Canadian Cancer Society and the National Cancer Institute; HD.6 ClinicalTrials.gov number, NCT00002561.). Paraneoplastic disorders in Hodgkin lymphoma is a very rare presentation. There are common therapeutic themes emerging. The main risk factors are severe underlying disease, chemotherapy management (especially corticosteroids), elderly patients. Methods: Turk J Pediatr 2019; 61: 139-141. In nosologic aspect, mental disorders in HL are classified as somatogenic and adjustement disorders. Two remarkable things can be derived from Carr's article: his presaging of neuronal autoantibodies before the first association between autoantibodies and limbic encephalitis had been made 2, and giving memory loss secondary to Hodgkin's disease, the eponym “Ophelia syndrome”, in reminiscence of Shakespeare's character, Ophelia, in Hamlet. It‟s fine to say, “Learn to think for yourself,” and it‟s … Hodgkin's lymphoma treatment typically includes chemotherapy or radiotherapy with potential treatment-related complications affecting the nervous system. Both patients presented with fairly sudden onset of profound and persistent memory loss in the absence of other neurological symptoms. Subsequent seizure recurrence and worsening of memory led to the discovery of widespread metastases. Although there was a long preneoplastic history, and no inflammatory changes were found at autopsy, the present case of hippocampal sclerosis could be included in the category of paraneoplastic limbic encephalitis associated with Hodgkin's lymphoma or the Ophelia syndrome. Neurologic complications of Hodgkin's Lymphoma can be separated into those that result directly from the disease, indirectly from the disease, or from its treatment. : CNS autoimmunity: new findings and pending issues. No significant myocardial dysfunction as a result of previous combined doxorubicin treatment and chest RT was identified. The Delay of Hamlet Essay 1558 Words | 7 Pages. Video-EEG on the first patient recorded multifocal sharp waves and bilateral independent seizure onsets. Complete remission from PNLE may occur and seems to be associated with pure anterograde amnesia without evidence of structural hippocampal damage in MRI. The remarkable scope of neurology…in 83 revealing numbers. Grade III/IV neutropenia was present on the scheduled day of treatment in 187 (20.9%) treatments in 64 (79%) patients. Methods: Immunohistochemistry with brain tissue and cultures of rat hippocampal neurons were used to demonstrate antibodies. Treatment of the tumour appeared to have more effect on the neurological outcome than the use of immune modulation. Several other cases of paraneoplastic limbic encephalitis without an identified autoantibody have been reported in association with HL [33, When uncomplicated neutropenia during doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine (ABVD) chemotherapy for the treatment of Hodgkin's lymphoma is encountered, it is unclear whether or not treatment should be modified. 25 updated practical neurology checklists. In most cases of adjustment disorders there was observed a combination of several risk factors (both general and specific). People fail to think independently due to the presence of authority figures or different forms of the media. The Othello syndrome affects males and, less often, females. This blog has visited this topic several times before such as with the posts titled What are the dreadful autoimmune disorders that plague neurology? Lancet 1:: 844, 1982-845, Medline, Google Scholar 2. None of the survivors in this sample showed overt congestive heart failure, a finding in contrast with larger studies. Abstract Introduction: Hodgkin lymphoma is a potentially curable lymphoma which has unique biological, clinical and histological characteristics. 111 - 120 of 500 . We report an unusual case of paraneoplastic limbic encephalitis (PLE) in a 17-year-old adolescent with classical Hodgkin lymphoma. Encephalitis was reversible in both patients receiving tumor treatment. These cases illustrate that patients with paraneoplastic neurological syndromes may present with psychiatric symptoms which can hamper an early diagnosis. Direct neurologic dysfunction from Hodgkin's Lymphoma results from metastatic intracranial spinal disease, epidural metastases causing spinal cord/cauda equina compression, leptomeningeal metastases, or intradural intramedullary spinal cord metastases. The antigens recognized by these antibodies are not expressed in lymphoma cells suggesting the tumor itself does not trigger the PNS. HL is the third most common cause of LE after SCLC and testicular germ cell tumors. The attraction of autoimmune neurological diseases lies in part in the, progressive loss of memory, depression, hallucinations, and bizarre, These symptoms aptly describe Ophelia’s deluded and obsessional attraction to the equally deluded and murderous, View ibrahimimam2000’s profile on Facebook, View ibrahimimam2000’s profile on Pinterest, View ibrahimimam2000’s profile on LinkedIn, When Shakespeare meets neurology: Hamlet, Ophelia and autoimmune encephalitis, Follow The Neurology Lounge on WordPress.com. Www3.uakron.edu Reviving Ophelia (1998) Treating women as objects through the sexualized gaze is common in the media as well as directly from men and, premenstrual syndrome, postpartum depression, depression with miscarriage, and postabortion syndrome. Most clinicians use first-line therapy (steroids, intravenous immunoglobulin, plasma exchange), and if severe or refractory, second-line therapy (rituximab, cyclophosphamide). Deanna will be performing live from the basement Saturday in Ophelia Syndrome’s jam space. Keywords: Ophelia syndrome; Limbic encephalitis; Hodgkin lymphoma. Based strictly on the script, her role in the story and relationship with other characters, particularly Hamlet, come off as ambiguous. Yes, you read it correctly. It may be possible to maintain dose intensity in the face of uncomplicated neutropenia during ABVD therapy. Among patients with Hodgkin's lymphoma, ABVD therapy alone, as compared with treatment that included subtotal nodal radiation therapy, was associated with a higher rate of overall survival owing to a lower rate of death from other causes. Firstly, patients given immune therapy do better and relapse less than patients given no treatment. Graus F et coll. Enter your email address to follow this blog and receive notifications of new posts by email. Conclusions: Antibodies to mGluR5 should be considered in patients with symptoms of limbic encephalitis and HL (Ophelia syndrome). Objective: To review the origins of the Ophelia’s syndrome through the original description and its relation to Shakespeare’s character, and the related autoantibodies. These metaphors describe people at various stages along the way from Ophelia to individuation. Neuropathological examination revealed that the pathology was confined to the hippocampus, both hippocampi showing sclerosis without inflammation. are the dreadful autoimmune disorders that plague neurology? To date, five years after initial presentation, the young man is able to walk with walking aids and orthoses and is still on oral prednisolone therapy. Sin embargo, existen diferencias entre ambos. A 9-year-old boy receiving chemotherapy for Hodgkin lymphoma, developed headache, temporal lobe seizures, anxiety, hallucinations, short-term memory loss and autonomic disturbances. Are parasites the simple solution to the problem of MS? Many of these diseases include a typical movement disorder that can be a powerful aid to diagnosis. Limbic encephalitis should be considered in the differential diagnosis of intractable partial epilepsy, particularly if accompanied by severe memory loss and cognitive decline. A few more catchy titles from the world of neurology, Anti-epileptic drug monitoring, ILAE, 2008, Ataxias and spastic paraplegias, EFNS, 2010, Facioscapulohumeral muscular dystrophy, AAN, 2015, IVIg in neuromuscular disorders, AAN, 2012, Limb girdle muscular dystrophies, EFNS, 2007, Neuromuscular transmission disorders, EFNS, 2010, Tuberous Sclerosis management recommendations, Arteriovenous malformations (AVMs), Choi and Mohr, 2005, Autoimmune encephalopathy, Vincent et al, 2011, Cerebral amyloid angiopathy (CAA), Rannikmae et al, 2013, Cerebral microbleeds, Cordonnier et al, 2007, Cervical artery dissection (CAD), Debette and Markus, 2009, Charcot Marie Tooth disease (CMT), Pareyson and Marchesi, 2009, Clinically Isolated Syndrome (CIS), Miller et al, 2012, Dementia in the young, Rossor et al, 2010, Duchenne Muscular Dystrophy (DMD), Bushby et al, 2010, Frontotemporal dementia (FTD), Seelar et al, 2011, Guillain Barre Syndrome (GBS), van Doorn et al, 2008, Hereditary spastic paraparesis (HSP), Salinas et al, 2008, Huntington’s disease, Novak and Tabrizi, 2010, Idiopathic Intracranial Hypertension (IIH), Biosse et al, 2012, Lambert Eaton myasthenic syndrome (LEMS), Gilhus, 2011, Motor Neurone Disease (MND), Miller et al, 2009, Movement disorders emergencies, Munhoz et al, 2012, Multiple Sclerosis (MS) treatment, Castro-Borrero et al, 2012, Myasthenia Gravis (MG), Jayam Trouth et al, 2012, Myasthenia Gravis (MG), myasthenic crisis, Wendell and Levine, 2011, Myotonic Dystrophy, Turner and Hilton-Jones, 2014, Neuromyelitis Optica (NMO), Palace et al, 2012, Neurosarcoidosis, Joseph and Scolding, 2009, Parkinson’s Disease (PD), Massano and Bhatia, 2012, Periodic paralysis (primary), Finsterer, 2008, Posterior Reversible Encephalopathy Syndrome (PRES), Roth and Ferbert, 2011, Postural Tachycardia Syndrome (POTS), Carew et al, 2009, Primary angiitis of the CNS, Hajj-Ali, 2011, Primary Progressive Multiple Sclerosis (PPMS), Rice et al, 2013, Progressive Multifocal Leukoencephalopathy (PML), Hunt and Giovannoni, 2012, REM sleep Behaviour Disorder (RBD), Boeve, 2010, Reversible Cerebral Vasoconstriction Syndrome (RCVS), Sattar et al, 2010, Sudden Unexpected Death in Epilepsy (SUDEP), So et al, 2009, Thunderclap headache (TCH), Ducros and Bousser, 2013, Transient Epileptic Amnesia (TEA), Zeman and Butler, 2010, Venous Sinus Thrombosis (VST), Piazza, 2012, Essential Parkinson’s Disease Drug Packaged in Engineered Tomatoes for Ease and Affordability, Immune Cell Identified That Contributes to Brain Damage During Viral Encephalitis, Tackling in children's rugby must be banned to curb dementia risks, The nautilus’s impressive memory has survived five mass extinctions, Antiepileptic drug reduces motor neuron excitability in ALS, How Do We Separate the Factual from the Possible? On thorough cardiac assessment a mean of 21 years after their diagnosis, none of the 53 unselected asymptomatic HLS showed physical signs or significant ECG abnormalities during or after the stress echo test. Immune cross-reactivity between malignant and normal tissues causes the rare, so called paraneoplastic syndrome (PS). Results: Common signs and symptoms during an episode may include severe headache, fever, nausea, vomiting, sensitivity to light (photophobia), and stiff neck. © 2008-2020 ResearchGate GmbH. Another thought of herself as a dog jumping through a hoop. Ophelia is worse than naive. He describes this syndrome as people choosing to be reliant on another to tell them what to think and how to feel. Their cardiac assessment included physical examination, electrocardiogram (ECG), and resting and postexercise echocardiograms. There is another constellation of signs and symptoms named after Ophelia, known as the Ophelia complex (and not Ophelia syndrome), in psychiatry. Recognition of this disorder is important because it can affect young individuals and is reversible. In the group of patients without anti-Hu or anti-Ta antibodies, the tumour distribution was diverse, with cancer of the lung the most common (36%); 57% had positive MRI. 16,18-29 The association of LE with HL is also known as Ophelia syndrome. Keywords: Ophelia syndrome; Limbic encephalitis; Hodgkin lymphoma Introduction . Mollaret meningitis is a type of meningitis due to a viral infection (aseptic meningitis) that occurs multiple times. The diagnosis of PLE required neuropathological examination or the presence of the four following criteria: (i) a compatible clinical picture; (ii) an interval of <4 years between the development of neurological symptoms and tumour diagnosis; (iii) exclusion of other neuro-oncological complications; and (iv) at least one of the following: CSF with inflammatory changes but negative cytology; MRI demonstrating temporal lobe abnormalities; EEG showing epileptic activity in the temporal lobes. PWYC. Paraneoplastic neurological syndromes (PNS) rarely associate with Hodgkin lymphoma (HL) and non-Hodgkin lymphomas (NHL). 16,[18][19][20][21][22][23][24][25]. Patients in the ABVD-only group, both those with a favorable risk profile and those with an unfavorable risk profile, received four to six cycles of ABVD. Among CNS lesions that may cause MD in patients with BD, refers to "Ophelia Syndrome" -paraneoplastic limbic encephalitis described in patients with Hodgkin's disease, ... HL is the third most common cause of LE after SCLC and testicular germ cell tumors. There is no other way. After radiochemotherapy, the lymphoma subsided except for involvement of the spleen, but the dementia remained. The Ophelia Syndrome - Essay by … Diagnosing and treating the ophelia syndrome How to write a article for magazine rabbit proof fence summary outline of an informative speech diagnosing and treating By Thomas G Plummer BYU TODAY September 1989 (Excerpt…) “S I Hayakawa describes symptoms of the Ophelia Syndrome in his essay, “What Does … Shinohara T, Kojima H, Nakamura N, et al: Pathology of pure hippocampal sclerosis in a patient with dementia and Hodgkin's disease: The Ophelia syndrome. View Ophelia Syndrome.pdf from STDEV 117 at Brigham Young University. Secondly, patients given early treatment do better. Two women aged 33 and 61 presented with uncontrolled complex partial seizures, profound memory loss and cognitive decline. "Is it such a bad thing to emulate teachers? What are the emerging treatments for neurofibromatosis? Antibody against mGLUR5 seems to be associated for this presentation. What are the pitfalls and perils of intracranial pressure? Paraneoplastic syndromes in Hodgkin lymphoma (HL) can be seen with different symptoms and organ findings within a significant time before definite diagnosis of the primary disease. The Electra complex is a term used to describe the female version of the Oedipus complex.. Among the patients randomly assigned to ABVD alone, 6 patients died from Hodgkin's lymphoma or an early treatment complication and 6 died from another cause; among those receiving radiation therapy, 4 deaths were related to Hodgkin's lymphoma or early toxic effects from the treatment and 20 were related to another cause. Most studies are retrospective cohorts, and there are no randomised controlled trials. She is described as a helpless child and has a “Lord” or authoritative figure that forces her even lower to the status of an infant. The clinical picture with this antibody is a form of encephalitis which may manifest with prosopagnosia (difficulty recognising faces), and with the curious Ophelia syndrome. Hippocampal hypermetabolism may be a common feature on PET, and may indicate subclinical seizure activity. Adequate data was available on 894 treatments in 81 patients with Hodgkin's lymphoma treated with ABVD chemotherapy. Neurons of sector CA2 were relatively well preserved and the subiculum was intact. We examined the neurological symptoms and the causal tumours in 50 patients with PLE to determine the utility of paraneoplastic antibodies and other tests. (RT). Hodgkin's lymphoma is a hematolymphoid neoplasm, primarily of B cell lineage, that has unique histologic, immunophenotypic, and clinical features. Nos résultats dans les troubles bipolaires et la schizophrénie montraient une prévalence du syndrome métabolique chez les patients respectivement de 20% et de 24%. What is the place of magnesium in the treatment of migraine? Ophelia syndrome is named after Shakespeare’s unfortunate Danish maiden, and it was first described by Dr. Ian Carr whose daughter, at the age of 15, developed progressive loss of memory, depression, hallucinations, and bizarre behaviour. Carr I : The Ophelia syndrome: memory loss in Hodgkin's disease. Which anti-MOG antibody treatment has the lowest relapse risk? Yes, you read it correctly. It remains to study the mental disorders and personality disorders, which are formed in patients with specific malignant and nonmalignant (hemophilia, thrombocytopathy etc.) For example, some young girls feel the pressure to be thin or to change their bodies because. Psychotic disorders observed in patients with HL include affective, delusional conditions, delirium. Conclusions: The presented data of literature suggests clinical heterogeneity and a wide range (from asthenic reactions to delirious states) of psychopathological disorders detected in blood diseases patients. This literature review differentially describes mental disorders in patients with Hodgkin’s lymphoma (HL) with an emphasis on psychotic disorders, affective states and adjustment disorders. The combination of symptoms, MRI findings and paraneoplastic antibodies established the diagnosis of PLE in 78% of the patients. Patients with anti-Ta (also called anti-Ma2) antibodies were young men with testicular tumours (100%), frequent hypothalamic involvement (70%) and a poor neurological outcome. The first patient suffered from a mood disorder, personality changes and complained of several, hitherto unexplained physical symptoms. All Ages. Immune therapy in autoimmune encephalitis: A systematic review, Unusual Presentation of Hodgkin Lymphoma with Limbic Encephalitis (Ophelia Syndrome), Autoimmune Movement Disorders in Children, Psychohematology. Third, unlike patients with solid tumors in patients with lymphoma the PNS often develops at advanced stages of the disease. The median length of follow-up was 11.3 years. When present, tumours should be removed. Of 1047 patients with neurological symptoms, whose sera or CSF were examined for paraneoplastic antibodies, 79 had the presumptive diagnosis of limbic encephalitis, dementia, cognitive dysfunction, or confusion. The fairly well-recognised ‘conventional’ antibodies are those against VGKC (Caspr 2 and LGI1), NMDA, and AMPA.