Announcement

Collapse
No announcement yet.

Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

Collapse
X
 
  • Filter
  • Time
  • Show
Clear All
new posts

  • #31
    Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

    A Young Man with Anti-NMDAR Encephalitis following Guillain-Barr? Syndrome
    Case Rep Neurol. 2011 Jan?Apr; 3(1): 7?13.
    Published online 2011 January 11. doi: 10.1159/000323751.

    PMCID: PMC3037987
    Copyright ? 2011 by S. Karger AG, Basel
    Kana Tojo,a Kazuhito Nitta,a Wataru Ishii,a Yoshiki Sekijima,a Hiroshi Morita,a Yukitoshi Takahashi,b Keiko Tanaka,c and Shu-ichi Ikedaa
    aDepartment of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan
    bDepartment of Pediatrics, National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan
    cDepartment of Neurology, Kanazawa Medical University, Ishikawa, Japan

    This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License (http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

    Abstract
    A 19-year-old man developed rapidly progressive muscle weakness and dysesthesia in the extremities, and dyspnea after a flu-like episode. Nerve conduction studies showed reduced motor nerve conduction velocities with conduction block, and sensory nerve action potentials could not be evoked. The patient was diagnosed as having Guillain-Barr? syndrome (GBS), and was treated with 2 cycles of intravenous immunoglobulin (IVIg) therapy and was assisted by mechanical ventilation. During the recovery course of the illness, he experienced several attacks of psychomotor agitation from the 37th hospital day, and generalized tonic convulsive seizures suddenly developed on the 42nd hospital day. Brain MRI showed high-intensity lesions in the bilateral thalamus and medial temporal lobes. The convulsions were controlled by continuous thiopental infusion (until the 50th hospital day) and mechanical ventilation (until the 84th hospital day). Intravenous methylprednisolone pulse therapy (1,000 mg/day) for 3 days followed by dexamethasone (16 mg/day) was added. After relief of convulsive seizures, prominent orolingual dyskinesia appeared, and on MRI marked atrophy of the bilateral medial temporal lobes was seen. Anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in serum and cerebrospinal fluid were positive on the 92nd hospital day. Anti-NMDAR encephalitis usually affects young females but a small number of male cases with this disease have been reported. Our male patient was unique in having GBS, a post-infectious autoimmune disease, as a preceding disease, suggesting that anti-NMDAR encephalitis itself is caused by a parainfectious autoimmune mechanism....
    Full text available at link.
    _____________________________________________

    Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

    i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

    "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

    (My posts are not intended as advice or professional assessments of any kind.)
    Never forget Excalibur.

    Comment


    • #32
      Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

      A couple of case history reports from the Facebook support group:

      http://www.facebook.com/topic.php?ui...77&topic=15516
      ARTICLE FROM 24 November, 2010 from the Netherlands (NL)
      Complex Illness: A young female resident from Steenbergen (NL), TOGETHER WITH HER PARENTS IS BATTLING against Anti NMDAR Encephalites.


      Wendy goes down only one step at a time

      Simply like that, out of the blue, misfortune struck....
      http://www.facebook.com/topic.php?ui...77&topic=15610
      A Mother's Account of her daughter's battle with Anti NMDA
      The mother of a teen who wishes to remain anonymous, has sent me the following account of her daughter's illness. It is well documented and what struck me, is how quickly the physicians intervened, in comparison with other case reports and accounts I have read.

      My sincere thanks to the contributor, whose hope it is that this may help someone in some way....
      Both stories can be read in full at the links.
      _____________________________________________

      Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

      i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

      "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

      (My posts are not intended as advice or professional assessments of any kind.)
      Never forget Excalibur.

      Comment


      • #33
        Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

        This is an audio documentary made by Aviva Ziegler about NMDAR encephalitis. Patients, family members, and Dr. Dalmau are interviewed.

        http://mpegmedia.abc.net.au/rn/podca...10606_0830.mp3

        Here's a recent article about a patient who recovered in Japan:
        (The full text and a movie are available.)

        http://www.jstage.jst.go.jp/article/...0_627/_article
        Akiko Uchino; Takahiro Iizuka; Yoshiaki Urano; Masahide Arai; Atsuko Hara; Junichi Hamada; Ryuichi Hirose; Josep Dalmau and Hideki Mochizuki, Pseudo-Piano Playing Motions and Nocturnal Hypoventilation in Anti-NMDA Receptor Encephalitis: Response to Prompt Tumor Removal and Immunotherapy . Intern. Med. 50: 627-630, 2011 .
        _____________________________________________

        Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

        i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

        "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

        (My posts are not intended as advice or professional assessments of any kind.)
        Never forget Excalibur.

        Comment


        • #34
          Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

          http://mynorthwest.com/?nid=11&sid=504515
          Updated Jun 29, 2011 - 12:57 pm
          Rare brain disease puzzles doctors

          By Ursula Reutin
          97.3 KIRO FM Reporter
          (Audio interview and report at link)
          Imagine losing your mind for a month or even longer. It's a terrifying reality for a growing number of people who have a newly-recognized disease that attacks their brain, plunging them into madness. KIRO Radio's URSULA REUTIN reports it especially affects young women.
          A young woman begins acting strangely, her parents think she's depressed, or maybe on drugs. Instead, it turns out she's suffering from a newly-recognized disease....
          Turned out to be anti-NMDAR encephalitis. Concern is expressed that there may be thousands of people suffering undiagnosed.
          _____________________________________________

          Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

          i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

          "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

          (My posts are not intended as advice or professional assessments of any kind.)
          Never forget Excalibur.

          Comment


          • #35
            Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

            http://www.capmh.com/content/5/1/15
            Child and Adolescent Psychiatry and Mental Health 2011, 5:15 doi:10.1186/1753-2000-5-15

            The electronic version of this article is the complete one and can be found online at: http://www.capmh.com/content/5/1/15

            Received: 21 January 2011
            Accepted: 13 May 2011
            Published: 13 May 2011

            ? 2011 Consoli et al; licensee BioMed Central Ltd.

            This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

            Malignant catatonia due to anti-NMDA-receptor encephalitis in a 17-year-old girl: case report


            Ang?le Consoli1, Karine Ronen1, Isabelle An-Gourfinkel2, Martine Barbeau1, Donata Marra3, Nathalie Costedoat-Chalumeau3, Delphine Montefiore5, Philippe Maksud4, Olivier Bonnot1, Adrien Didelot6, Zahir Amoura3, Marie Vidailhet2 and David Cohen1*

            Abstract

            Anti-NMDA-Receptor encephalitis is a severe form of encephalitis that was recently identified in the context of acute neuropsychiatric presentation. Here, we describe the case of a 17-year-old girl referred for an acute mania with psychotic features and a clinical picture deteriorated to a catatonic state. Positive diagnosis of anti-NMDA-receptor encephalitis suggested specific treatment. She improved after plasma exchange and immunosuppressive therapy. Post-cognitive sequelae (memory impairment) disappeared within 2-year follow-up and intensive cognitive rehabilitation.

            [snip]
            Background
            [snip]
            ...The exact incidence of anti-NMDAR encephalitis is unknown, but it seems to be more frequent than any other known paraneoplastic encephalitis [4].

            Conclusions
            [snip]
            This case report emphasizes the importance to search for a medical condition in catatonic syndrome of young people to treat and avoid severe neurological sequelae or death. The proposal of the DSM-V workgroup to make catatonia a "specifier" added as a fifth digit to other diagnoses seems likely to reduce rather than enhance clinician awareness of importance of recognizing this syndrome and researching for medical condition, particularly during psychiatric training. By contrast, a large group of experts advocated a unique and broadly-defined code for catatonia as a syndrome, which can be diagnosed acutely in addition to any suspected or established associated disorders [24]. In this case, the initial psychiatric clinical presentation was complicated by a malignant catatonic state, which is now well-described in anti-NMDA-Receptor encephalitis. Child psychiatrists need to know that anti-NMDA-Receptor encephalitis occurs frequently in children and adolescents. Plasma exchanges and immunosuppressive therapy treatments can dramatically improve catatonic syndrome associated with autoimmune dysfunction such as SLE [17,18], PANDAS [25] and NMDA-receptor encephalitis.
            _____________________________________________

            Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

            i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

            "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

            (My posts are not intended as advice or professional assessments of any kind.)
            Never forget Excalibur.

            Comment


            • #36
              Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

              http://www.thelancet.com/journals/la...548-5/fulltext
              The Lancet, Volume 378, Issue 9785, Page 98, 2 July 2011
              <Previous Article
              doi:10.1016/S0140-6736(11)60548-5Cite or Link Using DOI
              Late onset autism and anti-NMDA-receptor encephalitis
              Caroline Creten MD a, Sanne van der Zwaan BSc a, Roos J Blankespoor BSc a, Arjen Maatkamp MD b, Joost Nicolai PhD b, Prof Jim van Os PhD a, Dr Jan NM Schieveld PhD

              In December, 2009, a 9-year-old boy was admitted to our hospital with an acute onset of secondary generalised seizures. He had no medical or psychiatric history and functioned very well socially and academically. He presented with speech and swallowing difficulties, which after 10 days developed into a severely agitated catatonic state with opisthotonic posturing, tonic posturing of limbs, insomnia, and dyskinesia....
              Same article - HTML copy available:
              http://74.125.127.132/scholar?q=cach...48&as_ylo=2011

              Childhood disintegrative disorder, early onset schizo-
              phrenia, late onset autism and all stages of anti-NMDA-
              receptor encephalitis share core symptoms, as in our
              patient. We suggest that anti-NMDA-receptor encephalitis
              might be a possible organic cause underlying these three
              disorders. Patients previously diagnosed with these
              diagnoses might need to be re-examined for anti-NMDA-
              receptor encephalitis. We suggest that forthcoming
              editions of DSM-5 and ICD-11 exclude and define cases of
              regressive autism spectrum disorders due to anti-NMDA-receptor encehpalitis.
              _____________________________________________

              Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

              i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

              "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

              (My posts are not intended as advice or professional assessments of any kind.)
              Never forget Excalibur.

              Comment


              • #37
                Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

                22 yr-old Kiera Echols, her family, and surgeon Dr. Ed Richards continue their work to educate others about anti-NMDAR encephalitis:

                http://nky.cincinnati.com/article/AB...tion-her-story
                "The reason God had this happen is for me to help get the word out," Echols said Saturday as 10 hours of filming came to an end. "This doesn't need to be something that nobody knows about."
                _____________________________________________

                Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

                i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

                "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

                (My posts are not intended as advice or professional assessments of any kind.)
                Never forget Excalibur.

                Comment


                • #38
                  Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

                  In December of 2009 an 8-year-old girl in Schwenksville, PA got anti-NMDAR encephalitis and is still having a difficult time battling the disease, even though an ovarian teratoma was removed in January 2010:


                  http://www.pottsmerc.com/articles/20...0009012950.txt
                  Annual 'If you build it, they will run' race supports local girl

                  Published: Tuesday, August 10, 2010; Last Updated: Tue. Aug 10, 2010, 3:13pm


                  Updates:
                  http://www.newhanoverumc.org/updates...arlotte_wendel

                  http://pottsmerc.com/articles/2011/0...mode=fullstory
                  _____________________________________________

                  Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

                  i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

                  "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

                  (My posts are not intended as advice or professional assessments of any kind.)
                  Never forget Excalibur.

                  Comment


                  • #39
                    Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease



                    in this paper, you can see some RMI, easy to understand.
                    ( White " hyppocampus" on the floor of brain )

                    Comment


                    • #40
                      Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

                      Thanks for sharing that paper, Anne. It seems that the young woman in that case was diagnosed with a related autoimmune disorder involving antibodies to the AMPA receptors in the brain:

                      An antibody syndrome
                      antiphospholipid has been eliminated, a dosage of autoantibodies
                      antineuronaux nuclear (anti-VGKC,
                      anti-NMDA, anti-GABA [B], Hu, Ri, Yo, Amphysin, CV2,
                      Ta/Ma2, Ma and recoverin) is entirely negative. finally,
                      assay of antibody-anti-AMPA-R is positive.

                      A third MRI (2 months) shows a
                      again a shift of epicenters of achieving
                      with bilateral limbic reached a higher
                      the left side and a change in the achievement
                      right (Fig. 3 X). We retain finally the diagnosis
                      limbic encephalitis of autoimmune origin.
                      The paper below has a chart on page 2 that describes characteristics of all of the known antibody diseases affecting the brain. Unfortunately the case of the 8-yr-old with anti-NMDA receptor encephalitis would seem to have the best chance of recovery with the immune treatment and tumor removal that was done, yet so far that didn't happen.


                      Lancet Neurol 2011; 10: 759–72
                      Autoantibodies associated with diseases of the CNS: new
                      developments and future challenges

                      Angela Vincent, Christian G Bien, Sarosh R Irani, Patrick Waters
                      Several CNS disorders associated with specific antibodies to ion channels, receptors, and other synaptic proteins have
                      been recognised over the past 10 years, and can be often successfully treated with immunotherapies. Antibodies to
                      components of voltage-gated potassium channel complexes (VGKCs), NMDA receptors (NMDARs), AMPA receptors
                      (AMPARs), GABA type B receptors (GABABRs), and glycine receptors (GlyRs) can be identified in patients and are
                      associated with various clinical presentations, such as limbic encephalitis and complex and diffuse encephalopathies.
                      These diseases can be associated with tumours, but they are more often non-paraneoplastic, and antibody assays can
                      help with diagnosis. The new specialty of immunotherapy-responsive CNS disorders is likely to expand further as
                      more antibody targets are discovered. Recent findings raise many questions about the classification of these diseases,
                      the relation between antibodies and specific clinical phenotypes, the relative pathological roles of serum and intrathecal
                      antibodies, the mechanisms of autoantibody generation, and the development of optimum treatment strategies.
                      Last edited by Emily; August 15, 2011, 05:11 AM. Reason: Fixed text formatting
                      _____________________________________________

                      Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

                      i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

                      "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

                      (My posts are not intended as advice or professional assessments of any kind.)
                      Never forget Excalibur.

                      Comment


                      • #41
                        Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

                        thank you Emilie, before your file, I did'nt know that such pathology could exists.
                        I ve a paper about autoimmune epilepsy ( in french )...

                        Comment


                        • #42
                          Re: Anti-NMDA receptor encephalitis: an emerging autoimmune brain disease

                          Your paper on autoimmune epilepsy sounds interesting and I hope you'll post it here, Anne. I know there is a lot of research going on in France into these disorders.

                          I just noticed there are two recent cases of NMDA receptor encephalitis in very young children described by a mother in France here:

                          http://www.inspire.com/Pacobarth/jou...-encephalitis/

                          She mentions that both had viral infections before the neurological symptoms started.

                          (Maybe I'll change the title on this to include all related brain synaptic antibody diseases.)
                          Last edited by Emily; August 15, 2011, 05:05 AM. Reason: Added title change note
                          _____________________________________________

                          Ask Congress to Investigate COVID Origins and Government Response to Pandemic.

                          i love myself. the quietest. simplest. most powerful. revolution ever. ---- nayyirah waheed

                          "...there’s an obvious contest that’s happening between different sectors of the colonial ruling class in this country. And they would, if they could, lump us into their beef, their struggle." ---- Omali Yeshitela, African People’s Socialist Party

                          (My posts are not intended as advice or professional assessments of any kind.)
                          Never forget Excalibur.

                          Comment


                          • #43
                            Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

                            beginning and end
                            it's an automatic translation.

                            La Revue Médicale Suisse est la revue de formation continue de référence pour les médecins et les spécialistes des diverses disciplines médicales.

                            Margitta Seeck, Andr&#233; Zacharia, Andrea O. Rossetti
                            Epilepsies d’origine auto-immune

                            Rev Med Suisse 2010;6:925-929


                            The diagnosis of drug-resistant epilepsy of autoimmune origin are becoming more frequent. It is reported more and more of limbic encephalopathy paraneoplastic origin not from the five to ten years. Three antibodies are now described: antibody anticanaux dependent potassium voltage (voltage-gated potassium channels (VGKC)), the antacid glutamic decarboxylase (GAD) and antir&#233;cepteur N-methyl-D-aspartate (NMDA). We review the clinical syndromes in association with the imaging findings and the laboratory. Note that most reported cases involve the adult population, although some cases are also seen in adolescents and children. Early recognition of such a syndrome is very important as an early immunomodulatory therapy appears to favorably modify the clinical course.

                            One third of patients with epilepsy will develop into a drug resistance (that is: a lack of adequate clinical response after the trial of two or more anti&#233;pilepeptiques). There are several reasons for this. Surgical treatment can be proposed that some of these patients. The origins of autoimmune increasingly recognized since the last five to ten years and should not be missed, since their detection may potentially lead to effective treatment. The limbic encephalitis is typically associated with temporal lobe seizures and is the most common cause of late-onset temporal lobe seizures (after twenty years) .1
                            Conclusion

                            There are more and more cases of epilepsy known as autoimmune although their study is still in its infancy. Most studies including a large group of patients have been published over the past five years and are mainly adults but also children.

                            Given the large number and rate of discovery of different antibodies, we expect the fact that more and more patients are diagnosed with epilepsy of autoimmune origin, in the coming years. Other autoantibodies are still discovered. For example, anti-AMPA were found in ten patients with limbic encephalitis (nine women, including seven with a tumor) .24 At the same time, new treatments have become available immunomodulators in the last decade as rituximab.Diagnostic tools show significant progress but it still lacks major systematic studies of different immunological treatments.

                            The evidence is increasingly clear that a good symptomatic recovery is related to early treatment. This is all the more important it may be young or very young patients.

                            Practical implications

                            > An autoimmune balance should be considered for epilepsy late, severe epileptic encephalopathy or to the presence of other neurological symptoms such as memory impairment, ataxia, rigidity, involuntary movements, psychiatric disorders, coma and rarely as status epilepticus
                            > There are painting and not paraneoplastic paraneoplastic
                            > The assessment includes a review of neuropsychological and neurological, EEG, MRI, lumbar puncture (LP), antibody
                            > A normal PL does not exclude the presence of an array of encephalopathy with autoimmune
                            > The treatment is based on an antiepileptic and immunomodulatory therapy
                            > A report in a specialized center is recommended

                            Comment


                            • #44
                              Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

                              Seizure. 2011 Apr;20(3):266-70. Epub 2010 Dec 10.


                              Anti-NMDA receptor encephalitis presenting with imaging findings and clinical features mimicking Rasmussen syndrome.

                              Greiner H, Leach JL, Lee KH, Krueger DA.
                              Source

                              Department of Pediatrics, Division of Child Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.

                              Abstract

                              BACKGROUND:

                              Antibody mediated anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a recently reported diagnosis of clinical importance. Recognition of the syndrome, especially in pediatric populations, is difficult and often undiagnosed and/or confused with neurological disorders with similar clinical features.
                              RESULTS:

                              We report a case of an 11 year old female with explosive-onset epilepsy and predominantly unilateral frontal lobe abnormalities on magnetic resonance imaging (MRI) and fluorodeoxyglucose positron emission tomography (FDG-PET) neuroimaging. A diagnosis of Rasmussen syndrome (RS) was considered. Cerebrospinal fluid analysis revealed strong positivity for NMDA receptor antibodies. Screening for occult ovarian teratoma with computed tomography (CT) and MRI initially did not demonstrate associated tumor. Treatment with steroids and plasma exchange improved her clinical course and subsequent MRI showed resolution.
                              CONCLUSION:

                              NMDA receptor encephalitis has variable neuroimaging manifestations, and can mimic other entities. We emphasize the clinical syndrome of NMDA receptor encephalitis and consideration of the diagnosis in evaluating a child with explosive-onset epilepsy, unilateral imaging abnormalities, and neurocognitive decline.
                              Copyright ? 2010 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.


                              NMDA receptor encephalitis has variable neuroimaging manifestations, and can mimic other entities. We emphasize the clinical syndrome of NMDA receptor encephalitis and consideration of the diagnosis in evaluating a child with explosive-onset epilepsy, unilateral imaging abnormalities, and neurocogni …

                              Comment


                              • #45
                                Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases

                                it's not about NDMA, but it seems that Gille de la Tourette syndrome could be autoimmune
                                Background Similar to the model for Sydenham's chorea, antineuronal antibodies, which develop in response to a preceding streptococcal infection, have been speculated to have a role in the development of Tourette syndrome (TS). Methods Serum antibodies against human caudate, putamen, and globus pallidus (interna and externa) were assayed by enzyme-linked immunosorbent assay (ELISA) and Western blot techniques and results were correlated with clinical characteristics and markers of streptococcal infection. Subjects A total of 41 children with TS (mean age, 11.3 years) and 39 controls (mean age, 12.1 years) were included. Results Compared with controls, TS subjects had a significant increase in the mean ( p = 0.006) and median ( p = 0.002) ELISA optical density (OD) levels of serum antibodies against putamen, but not caudate or globus pallidus. Western blots on 20 control and 20 TS serum samples showed that specific antibodies to caudatelputamen occurred more frequently in TS subjects at 83, 67, and 60 kDa; antigens were present in a synaptosomal fraction. TS subjects with a positive family history of tics had higher OD values ( p ≤ 0.04), but no association was shown with age of tic onset, tic severity, sudden onset of tics, or presence of attention-deficit hyperactivity disorder or obsessive-compulsive disorder. Risk ratio calculations in TS and control groups and in study subjects dichotomized for high and low putamen OD values were similar for titers of antistreptolysin O ≥166 or antideoxyribonuclease B ≥ 170. A subgroup analysis limited to subjects with elevated streptococcal titers, however, showed a significantly ( p ≥ 0.004) larger number of TS subjects with elevated OD levels. Conclusion Children and adolescents with TS had significantly higher serum levels of antineuronal antibodies against putamen than did controls, but their relation to clinical characteristics and markers for streptococcal infection remains equivocal.

                                Antibodies against human putamen in children with Tourette syndrome



                                about lethargica :
                                We present a clinicopathological report of a recent fatal case of a 27-year-old woman whom we consider to have had encephalitis lethargica. Clinical features of note were a presentation with vertigo, persistent vomiting and sleep disturbance including marked daytime somnolence and vivid nightmares. …

                                Clin Neuropathol. 2001 Jan-Feb;20(1):2-7.
                                A contemporary case of encephalitis lethargica.

                                Kiley M, Esiri MM.
                                Source

                                Neurology, Oxford Radcliffe NHS Trust, UK.

                                Abstract

                                We present a clinicopathological report of a recent fatal case of a 27-year-old woman whom we consider to have had encephalitis lethargica. Clinical features of note were a presentation with vertigo, persistent vomiting and sleep disturbance including marked daytime somnolence and vivid nightmares. On examination, she had impaired slow pursuit vertical eye movements, dysarthria, an expressionless face and slow tongue movements. She went on to develop gross supranuclear gaze palsy, neck rigidity, bradykinesia, blepharospasm, profound somnolence and anarthria but no tremor, weakness or impairment of cognition. She died after an illness lasting 12 months. On investigation, the cerebrospinal fluid was found to contain a very high level of IgG with oligoclonal bands but no cells. Post-mortem examination revealed an active encephalitis, mainly centered on the upper brainstem and diencephalon with extensive Purkinje cell loss and marked plasma cell infiltrates and morula cells. No virus was recovered.

                                Comment

                                Working...
                                X