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Antibody Mediated Diseases

Anti-NMDA Receptor Encephalitis

  • Anti-NMDA receptor encephalitis is a recently recognized antibody-mediated inflammatory brain disease
  • Patients have neuropsychiatric symptoms, including changes in mental status, bizarre behavior, and seizures which are common among many infectious and metabolic diseases, and this makes the diagnosis much harder[1].
  • Patients initially appear to have a mental illness, but they soon exhibit recurrent seizures and movement disorders[1].
  • Testing for the anti-N-methyl-D-asparatate (NMDA) receptor antibodies can help diagnose this disease, and lead to reversal of symptoms with appropriate treatment.
  • Treatment includes immunosuppressive therapy, anti-psychotic medication, and oncologic or infectious treatment to target the cause (if necessary)[2].

References

  1. Tumbi A, Gilani A, Scarff JR, Kaur G, Lippmann S. Anti-N-methyl-D Encephalitis. Innov Clin Neurosci, 2011. 8(9): p. 24-5.
  2. Peery HE, Day GS, Dunn S, Fritzler MJ, Pruss H, De Souza C, Doja A, Mossman K, Resch L, Xia C, Sakic B, Belbeck L, Foster WG. Anti-NMDA receptor encephalitis. The disorder, the diagnosis and the immunobiology. Autoimmun Rev, 2012. 11(12): p. 863-72.
  3. Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol, 2011. 10(1): p. 63-74.

Limbic Encephalitis

  • Limbic encephalitis is a condition marked by the inflammation of the limbic system and other parts of the brain.
  • It is caused by specific antibodies that attack the brain tissue, particularly the limbic areas.
  • These antibodies are associated with the potassium channel, a protein crucial for proper neurological function. This causes a reduction in the number of potassium channels, decreasing the control over electrical activity of the brain[3].
  • The typical symptoms are cognitive decline, behavioral abnormalities, memory loss and seizures[1].
  • The diagnosis is confirmed by cerebrospinal fluid (CSF) inflammatory findings, Electroencephalography (EEG) or Magnetic Resonance Imaging (MRI) abnormalities in the temporal lobes, and antineuronal antibodies[2].
  • There are two forms of autoimmune limbic encephalitis:
    • Non-Paraneoplastic Limbic Encephalitis (NPLE)
      • Caused by specific antibodies that attack different antigens in the brain, particularly the limbic areas.
      • Occurs in patients without carcinomas.
      • Responds well to treatment and health improves with immunosuppressive therapy[1].
    • Paraneoplastic Limbic Encephalitis (PLE)
      • Occurs rarely in patients with cancer, most commonly in small cell lung cancer and seminomas[3].
      • Antibodies are made by the patients in their attempt to fight the cancer, but the antibodies inadvertently attack the brain tissue[3].
      • Treatment is made based on the underlying oncologic condition and prognosis is poor.

References:

  1. Derry CP, Wilkie MD, Al-Shahi Salman R, Davenport RJ. Autoimmune limbic encephalitis. Clinical Medicine, 2011. 11(5): p. 476-8.
  2. Tüzün E, Dalmau J. Limbic encephalitis and variants: classification, diagnosis and treatment. Neurologist, 2007. 13(5): p. 261-71.
  3. Irani S, Vincent A, Buckley C. Limbic Encephalitis. The Encephalitis Society, Web. 26 June 2012. <http://www.encephalitis info/images/iPdf/Resources/FactSheets/FS013LimbicEncephalitis.pdf>.

Hashimoto’s Encephalitis

  • Hashimoto’s encephalitis (HE) is a neuroendocrine autoimmune disease.
  • HE involves inflammation of the brain, and is characterized by a high presence of thyroid antibodies similar to those present in Hashimoto’s thyroiditis.
  • Patients may be euthyroid or hypothyroid upon onset[1].
  • The etiology is unknown. The thyroid antibodies are thought to be a marker of the disease, rather than the cause of the problem[2].
  • Symptoms are similar to other encephalopathic conditions; Clinical manifestations of HE may include seizures, behavioral and psychiatric manifestations, movement disorders, and coma[1,2].
  • Treatment is very effective. The symptoms respond very well to immunosuppressive treatment[1,2].
  • Thyroid replacement drugs may be needed depending on the condition of the thyroid.

References:

  1. Holanda NC, de Lima DD, Cavalcanti TB, Lucena CS, Bandeira F. Hashimoto's Encephalopathy: Systematic Review of the Literature and an Additional Case. J Neuropsychiatry Clin Neurosci, 2011. 23: p. 384-390.
  2. Aji, Baba. “Hashimoto’s Encephalitis.” The Encephalitis Society. Web. 26 June 2012.<http://www.encephalitis.info/images/iPdf/Resources/FactSheets/fs005Hashimoto.pdf>.

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