Conclusion: This case emphasis the need to investigate for paraneoplastic disorders when patients with Hodgkin lymphoma presents with neurological changes. Secondly, patients given early treatment do better. At 12 years, the rate of overall survival was 94% among those receiving ABVD alone, as compared with 87% among those receiving subtotal nodal radiation therapy (hazard ratio for death with ABVD alone, 0.50; 95% confidence interval [CI], 0.25 to 0.99; P=0.04); the rates of freedom from disease progression were 87% and 92% in the two groups, respectively (hazard ratio for disease progression, 1.91; 95% CI, 0.99 to 3.69; P=0.05); and the rates of event-free survival were 85% and 80%, respectively (hazard ratio for event, 0.88; 95% CI, 0.54 to 1.43; P=0.60). It is characterized by low self esteem, anxiety, inability to take decisions and increased risk for depression — … The purpose of this review is to summarize the clinical characteristics, diagnosis, and treatment of movement disorders that occur in pediatric autoimmune disorders. These questions are raised by an article I read called "Diagnosing and Treating the Ophelia Syndrome" by Thomas G. Plummer. One reading of Ophelia's suicide later in Hamlet suggests that because she has no thoughts of her own, because she listened only to the contradictory voices of the men around her-- Laertes, Polonius, and Hamlet-- she reaches a breaking point. "Is it such a bad thing to emulate teachers? 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? Firstly, patients given immune therapy do better and relapse less than patients given no treatment. A 9-year-old boy receiving chemotherapy for Hodgkin lymphoma, developed headache, temporal lobe seizures, anxiety, hallucinations, short-term memory loss and autonomic disturbances. The aim of our study was to assess clinical and subclinical cardiac sequelae in a cohort of childhood HLS treated in the 1980s with doxorubicin, bleomycin, vinblastine, and dacarbazine (the ABVD regimen) and limited-field radiotherapy, Chemotherapy plus radiation treatment is effective in controlling stage IA or IIA nonbulky Hodgkin's lymphoma in 90% of patients but is associated with late treatment-related deaths. Deanna will be performing live from the basement Saturday in Ophelia Syndrome’s jam space. Paraneoplastic neurological syndromes (PNS) rarely associate with Hodgkin lymphoma (HL) and non-Hodgkin lymphomas (NHL). Purpose: This article describes studies involving mental and personality disorders in patients with blood diseases - disorders typology, prevalence, risk factors for developing of mental disturbances and their treatment. Given that early detection and treatment with immunotherapy may confer improved outcomes, recognizing these patterns of abnormal movements is essential for child neurologists. 18 The cases reported in the English literature are summarized in Table 3. [106] However, poor outcome with death in Ophelia syndrome has been reported in other cases, with [113] or without, ... Our patient's case fulfilled all the criteria required for diagnosis of Ophelia syndrome. We searched for subtle cardiac abnormalities in a subgroup of asymptomatic individuals, who underwent rest and exercise echocardiography at least 20 years after completing their therapies. In the second patient, the lung cancer was irradiated with resolution of seizures and improvement of memory function. Another thought of herself as a dog jumping through a hoop. In 1982, Dr. Ian Carr described personality changes and memory loss in his 15-year-old daughter, who had limbic encephalitis and Hodgkin lymphoma (HL).¹ He assumed “a circulating neurotransmitter-like molecule produced by the neoplasm” causing the brain disease, and noted that it “may be reversible and can be remembered as the Ophelia syndrome.” Almost 30 years later, Lancaster et al.² identified antibodies against the metabotropic glutamate receptor 5 (mGluR5) as Carr's neurotransmitter-like molecule in 2 patients with limbic encephalitis and HL. The Othello syndrome affects males and, less often, females. Sometimes it may be the only presentation of the underlying tumor which poses a diagnostic challenge to the physicians. None of the survivors in this sample showed overt congestive heart failure, a finding in contrast with larger studies. The fairly well-recognised ‘conventional’ antibodies are those against VGKC (Caspr 2 and LGI1), NMDA, and AMPA. Summary In his article “Diagnosing and Treating the Ophelia Syndrome,” (1990/2012) Thomas G. Plummer, a professor of Germanic and Slavic languages, gives an insight into what he calls the Ophelia Syndrome. A PET scan six months later was normal. Neurons of sector cornu ammonis (CA) 1 were completely lost whereas moderate neuron loss was also observed in sectors CA3 and 4, and the dentate gyrus. 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. 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. Chemotherapy for Hodgkin's lymphoma may cause cerebral infarction (due to emboli from anthracycline-induced cardiomyopathy) and peripheral neuropathy. Neutropenia and febrile neutropenia in patients with Hodgkin's lymphoma treated with doxorubicin (Ad... Nodular sclerosis Hodgkin lymphoma with classic Reed-Sternberg cells, Clinical and Subclinical Cardiac Late Effects in Pediatric Hodgkin's Lymphoma Survivors, ABVD Alone versus Radiation-Based Therapy in Limited Stage Hodgkin’ s Lymphoma. Ophelia Syndrome is when a person is "chronically ignorant, chronically dependent, and chronically submissive." The patient improved with aggressive immunotherapy. The present study shows that long-term cardiac effects are common in HLS treated with the ABVD regimen and RT. Treatment of the tumour appeared to have more effect on the neurological outcome than the use of immune modulation. There is no other way. Patients with anti-Hu antibodies usually had small-cell lung cancer (94%), multifocal neurological symptoms (78%) and a poor neurological outcome. What's wrong with a cage? Abstract Introduction: Hodgkin lymphoma is a potentially curable lymphoma which has unique biological, clinical and histological characteristics. 28, 34 Although some patients with neuromyotonia have Caspr2 antibodies, the large majority of patients are antibody negative. 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. 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. People fail to think independently due to the presence of authority figures or different forms of the media. One was later diagnosed with breast cancer and the other with lung cancer. Yes, you read it correctly. She is an adult who chooses to be a baby, one who does not know her own opinions and who would not express them to an authority if she did. An archive autopsy case of a 50-year-old man who died of Hodgkin's lymphoma had a 4-year, 4-month history of dementia. Early treatment of the underlying tumor helps in successful neurological recovery. Magnetic resonance imaging (MRI) was normal in both patients. Thus, multiple courses of multidrug immunosuppressive therapy were administered. People with Down syndrome are subject to a variety of medical problems including heart abnormalities and thyroid gland dysfunction. 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. 18 The cases reported in the English literature are summarized in Table 3. Subsequently, further cases of Ophelia syndrome were reported (Hodgkin's lymphoma and limbic encephalitis), [107][108] [109] and in 2011 anti-mGluR5 antibodies were detected in two patients. All Ages. The antigens recognized by these antibodies are not expressed in lymphoma cells suggesting the tumor itself does not trigger the PNS. All Ages. First, a person must have been “confronted with an event or events that involved actual or threatened death or serious injury, or a threat to … With grade III/IV neutropenia on the day of therapy, 158 treatments were administered without dose reduction or dose delay with one subsequent episode of febrile neutropenia. 16,18-29 The association of LE with HL is also known as Ophelia syndrome. Which anti-MOG antibody treatment has the lowest relapse risk? There are common therapeutic themes emerging. The memory of patient 1 recovered fully and he died from an unrelated cause 1 year later; neuropsychological testing showed a severe, but isolated, anterograde amnesia, brain MRI was normal and HMPAO-SPECT showed left medial temporal hypoperfusion. ... 67,68 Myoclonus has been reported in adults with Ophelia syndrome, but movement disorders have not yet been recognized in childhood cases. The primary end point was 12-year overall survival. Their subsequently diagnosed small cell lung cancer was treated with a combination of radiotherapy and chemotherapy, leading to remission of the tumour. The Delay of Hamlet Essay 1558 Words | 7 Pages. Treatment of the underlying cancer may be lead to improved seizure control. Post was not sent - check your email addresses! First, some PNS such as sensory neuronopathy or Lambert-Eaton myasthenic syndrome (LEMS) rarely occur in lymphomas whereas others, such as granulomatous angiitis are only described in HL. Indirect neurologic dysfunction may be caused by paraneoplastic disorders (such as paraneoplastic cerebellar degeneration or limbic encephalitis) and primary angiitis of the central nervous system. Both patients presented with fairly sudden onset of profound and persistent memory loss in the absence of other neurological symptoms. She is chronically ignorant, chronically dependent, and chronically submissive. Read More . Analyses of the CSF and serum revealed anti SOX-1 antibodies at initial presentation but PCA-2 antibodies seven months after diagnosis. He presented with a variety of neurologic and neuropsychiatric symptoms, profound B-symptoms and typical MRI findings including hyperintense lesions with contrast enhancement in the medial temporal lobe and limbic system. DIAGNOSING AND TREATING THE OPHELIA SYNDROME By Thomas G. Plummer; BYU Magazine; In Hamlet, Act 1, Scene 3, Laertes warns his sister, Ophelia, to avoid falling in love with Hamlet, whose advances, he claims, are prompted by fleeting, youthful lust. The most frequent complications observed in this sample were essentially coronary artery disease and valvular abnormalities. Results: 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. When present, tumours should be removed. 111 - 120 of 500 . Conversion disorders and behavioral disorders that lead to non-compliance in patients with HL are described as well. What is the state of gene therapy for Parkinson’s disease? : CNS autoimmunity: new findings and pending issues. Hippocampal hypermetabolism may be a common feature on PET, and may indicate subclinical seizure activity. Conclusion: 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 … Get on it. Frequently, movement disorders in autoimmune conditions are the sole or among a few presenting symptoms, allowing for earlier diagnosis of an underlying malignancy or systemic autoimmune disease. The Ophelia Syndrome is not processing the ability to create one’s own opinion. Most studies are retrospective cohorts, and there are no randomised controlled trials. Twenty-five of 44 (57%) patients with MRI studies had signal abnormalities in the limbic system. What is the last word on migraine and PFOs? "Ophelia Syndrome Commentary" Essays and Research Papers . Ophelia Syndrome is best described as thinking or feeling a certain way because a person is told to. Many of these diseases include a typical movement disorder that can be a powerful aid to diagnosis. Get on it. By contrast, cognitive deficits beyond severe anterograde amnesia and evidence of destructive medial temporal lobe pathology on MRI seem to be poor prognostic features. Methods: Immunoprecipitation, mass spectrometry, and mGluR5 -null mice served to … 25 updated practical neurology checklists. 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. Given the retrospective uncontrolled data, the literature has inherent bias, including severity and reporting bias. In nosologic aspect, mental disorders in HL are classified as somatogenic and adjustement disorders. Hodgkin's lymphoma is a hematolymphoid neoplasm, primarily of B cell lineage, that has unique histologic, immunophenotypic, and clinical features. Twenty-two (41%) of the 53 patients revealed valvular abnormalities, with mitral regurgitation in 28%, aortic regurgitation in 9%, and both in 4%. Third, unlike patients with solid tumors in patients with lymphoma the PNS often develops at advanced stages of the disease. For example, some young girls feel the pressure to be thin or to change their bodies because. Neurologic complications resulting from mantle-field radiotherapy include the "dropped head syndrome," acute brachial plexopathy, and transient ischemic attacks/cerebral infarcts. Conclusions: What if you are a student of biochemistry or German grammar? What are the pitfalls and perils of intracranial pressure? The attraction of autoimmune neurological diseases lies in part in the ever-expanding spectrum of the antibodies and the challenging symptoms and syndromes they produce. Adequate data was available on 894 treatments in 81 patients with Hodgkin's lymphoma treated with ABVD chemotherapy. Results: and dose modification, in a large university patient population. We have reviewed the literature of immune therapy in autoimmune encephalitis associated with antibodies to cell surface antigens including N-methyl-D-aspartate receptor (NMDAR), leucine-rich, glioma-inactivated protein-1 (LGI1), contactin-associated protein-2 (Caspr2), the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), γ-aminobutyric acid-A receptor (GABAAR), γ-aminobutyric acid-B receptor (GABABR), Glycine R and other rarer antigens. 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. are the dreadful autoimmune disorders that plague neurology? Attempt to Summarise: Vybornykh DE et al . Deanna will be answering your questions, premiering some new material, and giving a little behind the scenes intel. Ophelia syndrome is almost always associated with Hodgkins lymphoma and affects young people. 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. Ophelia is worse than naive. Thankfully Ophelia syndrome is a relatively mild disease without the Shakespearean tragic ending because it has a good outcome if recognised and treated. Similarly, Carr's daughter had a full recovery in the absence of immune therapy. For example, some young girls feel the pressure to be thin or to change their bodies because the media communicates in different ways what, by the mainstream media's … Tantabus Syndrome is a type of sickness that can be experienced by devote disciples of Princess Luna, i.e Ophelia Sparkle and Prince Somber Moon. There are different psychogenic states among the adjustment disorders. And thirdly, when patients fail first-line therapy, second-line therapy improves outcomes and reduces relapses. 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. In approximately 60% of the patients, various onconeural antibodies are detectable in the cerebrospinal fluid (CSF) and are associated with typical tumour entities. Hodgkin's lymphoma treatment typically includes chemotherapy or radiotherapy with potential treatment-related complications affecting the nervous system. Subsequent seizure recurrence and worsening of memory led to the discovery of widespread metastases. The clone, the chameleon personality is the Ophelia Syndrome in another form. There are several important differences in PNS when occurring in association with HL and NHL compared to those associated with solid tumors. Two women aged 33 and 61 presented with uncontrolled complex partial seizures, profound memory loss and cognitive decline. 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. It remains to study the mental disorders and personality disorders, which are formed in patients with specific malignant and nonmalignant (hemophilia, thrombocytopathy etc.) There was no evidence of global hypoxia, or of neurodegenerative disorders with pathological changes affecting the hippocampus. Which drug reduces the autoimmune risks of alemtuzumab? Two patients, a 38-year-old man and a 32-year-old woman, were admitted to a psychiatric ward. We examined the neurological symptoms and the causal tumours in 50 patients with PLE to determine the utility of paraneoplastic antibodies and other tests. Case Presentation: Here we describe a rare case of a 60 year old Caucasian lady newly diagnosed with stage 4B Hodgkin lymphoma who presented with progressive neurological deterioration. There is however an almost endless list of less familiar antibodies such as those against glycine, adenylate kinase 5, thyroid, GABA-A receptors, α-enolase, neurexin-3α, dipeptidyl-peptidase-like protein 6 (DPPX), and myelin oligodendrocyte glycoprotein (MOG). 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. No significant myocardial dysfunction as a result of previous combined doxorubicin treatment and chest RT was identified. View Ophelia Syndrome.pdf from STDEV 117 at Brigham Young University. Neurology can’t seem to get away from autoimmune disorders of the central nervous system. Cardiac late effects are responsible for a significant burden of mortality and morbidity among pediatric Hodgkin's lymphoma (HL) survivors (HLS). 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