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Spirit to autoantibodies: Journey of limbic disorders from philosophy to affective neuroscience

Limbic encephalitis usually presents with neuropychiatric symptomatology, often remain undiagnosed, treated as psychiatry disorders with various antipsychotic medications and die. Early diagnosis and treatment of autoimmune limbic encephalitis has good outcome, establishing these patients back into the society

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Spirit to autoantibodies: Journey of limbic disorders from philosophy to affective neuroscience

  1. 1. SPIRIT TO AUTOANTIBODIES JOURNEY OF LIMBIC DISORDERS FROM PHILOSOPHY TO AFFECTIVE NEUROSICENCE Dr. Ubaidur Rahaman M.D. Internist and Critical Care Specialist
  2. 2. “Education is a progressive discovery of our own ignorance” Will Durant
  3. 3. CASE VIGNETTE • 28 Y F • History of psychiatric illness on medications, • Presented with headache, vomiting, convulsions and altered mentation, • Routine Lab revealed no abnormality, • CSF examination inconclusive, • CT brain unremarkable • MRI brain showed dubious hyperintenisity in temporal lobes, as metal artifact related to dental brace
  4. 4. LIMBIC SYSTEM ARISTOTLE Heart as center of intelligence and emotions GALEN Brain as center of intelligence and emotions BROCA, PAPEZ AND MCLEAN Objective scientific explanation PHILOSOPHY SPIRIT AFFECTIVE NEUROSCIENCE NEUROCHEMICALS
  5. 5. LIMBIC SYSTEM “a visceral brain that interprets and gives expression to its incoming information in terms of feeling, being incapable perhaps of getting at the meaning of things at the level of symbolic language.” Maclean, 1952 “The hypothalamus, the anterior thalamic nucleus, the cingulate gyrus, the hippocampus and their interconnections, constitute a harmonious mechanism which may elaborate the functions of central emotion as well as participate in the emotional expression.” James Papez, 1937
  6. 6. More of a functional concept than anatomical Ring of interconnected structures around the "stem" that connects the cerebral hemispheres to the top end of the brain stem. It function include EMOTION, behavior, motivation, memory, LIMBIC SYSTEM
  7. 7. LIMBIC SYSTEM DISEASES NEUROPYCHIATRIC MANIFESTATIONS • ADHD • OCD • ANXIEITY DISORDER • SCHIZOPHRENIA • AFFECTIVE DISORDERS • EPILEPSY • LIMBIC ENCEPHALITIS • KORSAKOFF PSYCHOSIS • AUSTISM • KLUVER BACHY SYNDROME • DEMENTIA
  8. 8. LIMBIC ENCEPHALITIS ERA of HISTOPATHOLOGY: Paraneoplastic LE 1960 1968 3 Patients of atypical encephalitis Abnormal behavior and motor movement Autopsy showed inflammation in limbic area 3 Patients of bronchial CA: 1 with severe short term amnesia, 2 with amnesia and dementia Autopsy: inflammation and degeneration mainly in limbic area Reviewed 8 previously reported cases and suggested relationship between LE and CA
  9. 9. PARANEOPLASTIC LIMBIC ENCEPHALITIS (PLE) CLINICO-PATHO-RADIOLOGICAL DIAGNOSIS Characterized by neuropsychiatric symptoms like personality changes, irritability, depression, seizures, memory loss and sometimes dementia. Associated with neoplasms: lung (50%), testis (20%) and breast (8%). The diagnosis is difficult because clinical markers are often lacking, and symptoms usually precede the diagnosis of cancer in 60% of patients Distinct radiologic and pathologic finding in temporal lobe
  10. 10. AUTOIMMUNE LIMBIC ENCEPHALITIS Era of NEUROCHEMISTY AND NEURORADIOLOGY • Thirty (60%) patients had antineuronal antibodies (18 anti-Hu, 10 anti-Ta, 2 anti-Ma), and 20 were antibody-negative or had uncharacterized antibodies • Treatment of the tumor appeared to have more effect on the neurological outcome than the use of immune modulation • Improvement was observed in 38% of anti-Hu patients, 30% of anti-Ta patients and 64% of patients without these antibodies Brain. 2000 Jul;123 ( Pt 7):1481-94. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J
  11. 11. AUTOIMMUNE LIMBIC ENCEPHALITIS Era of NEUROCHEMISTY AND NEURORADIOLOGY Paraneoplastic encephalitis and anti-Hu antibodies. Atypical limbic encephalitis and VGKC antibodies. FLAIRIMAGEMRIoflimbicencephalitis The Neurologist 2007;13: 261–271 Limbic Encephalitis and Variants: Classification, Diagnosis and Treatment Erdem Tu¨zu¨n, MD, and Josep Dalmau, MD, PhD
  12. 12. AUTOIMMUNE LIMBIC ENCEPHALITIS NOT A UNCOMMON DISEASE ERA OF NEURORAGIOLOGY AND NEUROCHEMISTRY Neurologia. 2005 Jan-Feb;20(1):24-30. [Limbic encephalitis: a probably under-recognized syndrome]. [Article in Spanish] Graus F, Saiz A • Recognition and reporting of Autoimmune LE of varied neuronal antibodies • Excellent response to immunosuppressive therapy • if diagnosis is confirmed by the clinical picture and MRI findings, • Start immunosuppressive therapy, • without waiting for the antibody results as its negativity does not exclude the diagnosis
  13. 13. AUTOIMMUNE LIMBIC ENCEPHALITIS SYMPTOMATOLOGY Nephrologic Hyponatriaemia Neurological features Memory loss or amnesia Movement disorders & dystonia: motor or vocal tics, chorea, catatonia, parkinsonism, rigidity, acute dystonia Epilepsy: generalized, partial, myoclonus Aphasia, mutism Nystagmus, ataxia drowsiness, stupor, sleep inversion Decreased level of consciousness Autonomic features: instability of BT, hypoventilation, respiratory failure Neuropsychiatric symptoms Behavioral problems, changed personality Depression, anxiety, fear, psychosis, hallucinations ADHD, OCD Anorexia, bulimia Autoimmune encephalitis - History & current knowledge By Finn E. Somnier, M.D., D. Sc. (Med.), Department of Clinical Biochemistry, Immunology and Genetics, Staten’s Serum Institute, Copenhagen, Denmark
  14. 14. AUTOIMMUNE LIMBIC ENCEPHALITIS AUTOANTIBODIES PARANEOPLASTIC Anti-Hu Anti-CV2 (CRMP5) Anti-Ta (Ma2) Anti-Ri Anti-Amphiphysin Anti-mGluR5 NON NEOPLASTIC Anti-LGI1 (“anti-VGKC”) Anti-NMDAR (NR1) Anti-AMPAR (GluR1, GluR2) Anti-AMPAR (GluR3) Anti-GABABR1 Anti-AQP4 Anti-D1, anti-D2, anti-lyso-GM1 Anti-Alpha-enolase (ENO1) Anti-GAD Anti-GlyR alpha1 Autoimmune encephalitis - History & current knowledge By Finn E. Somnier, M.D., D. Sc. (Med.), Department of Clinical Biochemistry, Immunology and Genetics, Staten’s Serum Institute, Copenhagen, Denmark
  15. 15. LIMBIC ENCEPHALITIS DIFFERENTIAL DIAGNOSIS TheNeurologist2007;13:261–271 LimbicEncephalitisandVariants:Classification,DiagnosisandTreatment ErdemTu¨zu¨n,MD,andJosepDalmau,MD,PhD
  16. 16. ANTI NMDA RECETPOR ANTIBODY ENCEPHALITIS NMDAR ANTIBODY ENCEPHALITIS four young women with acute psychiatric symptoms and ovarian teratoma seizures, memory deficits, Decreased LOC and central hypoventilation Ann Neurol. 2005 October ; 58(4): 594–604. Paraneoplastic Encephalitis, Psychiatric Symptoms, And Hypoventilation in Ovarian Teratoma Roberta Vitaliani, Josep Dalmau, MD NMDA RECEPTOR N-methyl-D-aspartate receptor, excitatory glutamate receptor important in controlling synaptic plasticity and memory function
  17. 17. ANTI NMDA RECETPOR ANTIBODY ENCEPHALITIS NMDAR ANTIBODY ENCEPHALITIS Psychiatric symptoms • Diagnosed as anxiety, schizophrenia and admitted in psychiatric ward Neurologic al symptoms • Seizures, movement disorders, altered consciousness, • Autonomic instability • Hypoventilation Intubation Agitation or decrease LOC • Prolong weaning due to hypoventilation Death or Recovery Young Women USG Pelvic cyst (ovarian dermoid or teratoma) CSF Mild Increases protein Lymphocytic pleocytosis MRI Median temporal lobe hyperintensity (25%) TheNeurologist2007;13:261–271 LimbicEncephalitisandVariants:Classification,DiagnosisandTreatment ErdemTu¨zu¨n,MD,andJosepDalmau,MD,PhD
  18. 18. ANTI NMDA RECETPOR ANTIBODY ENCEPHALITIS NMDAR ANTIBODY ENCEPHALITIS SUSPICION • IF IT QUAKS LIKE DUCK, BUT REFUSES TO BEHAVE LIKE DUCK, ITS SOMETHING NOT DUCK IMMUNOTHE RAPY • CORTICOSTEROIDS, PLASMA EXCHANGE, IVIG, • CYCLOPHOSPHAMIDE, RITUXIMAB REMOVAL of TUMOUR MANAGEMENT TheNeurologist2007;13:261–271 LimbicEncephalitisandVariants:Classification,DiagnosisandTreatment ErdemTu¨zu¨n,MD,andJosepDalmau,MD,PhD Patients starts recovering 2-3 weeks of tumor excision and immunotherapy
  19. 19. ENCEPHALITIS RELEARNED….. EPIDEMIOLOGY Autoimmune encephalitis - History & current knowledge By Finn E. Somnier, M.D., D. Sc. (Med.), Department of Clinical Biochemistry, Immunology and Genetics, Staten’s Serum Institute, Copenhagen, Denmark
  20. 20. SOCIETY AND CULTURE People who fought Anti NMDAR Encephalitis ANNELIESE MICHEL, Genmany 1970 died following a year of exorcism by catholic church. Her symptoms were suggestive of anti NMDAR encephalitis. The movies "The Exorcism of Emily Rose" and "Requiem" are based on her. AMOBI OKOYE, football player (DALLAS COWBOY) Diagnosed anti-NMDAR encephalitis. Battled for 17 months. he experienced a 145-day memory gap and lost 78 pounds. He returned to practice on October 23, 2014. SUSANNAH CAHALAN, New York Post reporter suffered anti NMDAR encephalitis, and wrote a book titled Brain on Fire: My Month of Madness about her experience with the disease WILL GRAHAM, The protagonist of the Hannibal TV series diagnosed with anti-NMDAR encephalitis after an MRI scan in the 10th episode of the first season Source wikipedia
  21. 21. “Hello. My name is Leonard Lowe. It has been explained to me that I've been away for quite some time. I'm back.”

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