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Antibody-mediated inflammatory brain disordersAAntibody-mediated inflammatory brain disordersAntibody-mediated inflammatory brain disordersEnglishImmunologyChild (0-12 years);Teen (13-18 years)BrainImmune systemConditions and diseasesCaregivers Adult (19+)NA2013-05-13T04:00:00ZSusanne Benseler, MD;Manisha Sickand, MSc;Marinka Twilt, MD13.000000000000024.00000000000001053.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Learn about the different types antibody-mediated inflammatory brain disorders and what the signs and symptoms are for each.</p><h2>What are antibody-mediated inflammatory brain disorders?</h2><p>In antibody-mediated inflammatory disease, B cells (unique white blood cells) produce antibodies against the body's own structures; these are called autoantibodies. When autoantibodies bind to these structures, they induce unnecessary inflammation that is directed against healthy tissue. Autoantibodies directed against structures in the brain lead to irritation and swelling of brain tissue. If not treated, long standing inflammation can lead to permanent brain damage and dysfunction.</p> <figure class="asset-c-80"> <span class="asset-image-title">Antibody-mediated normal immune response</span> <img src="https://assets.aboutkidshealth.ca/akhassets/IMD_antibody_mediated_normal_immune_response_EN.jpg" alt="" /> <figcaption class="asset-image-caption">Your body’s immune system produces antibodies to "mark" harmful things that enters your body. Special immune cells know what to attack by finding antibodies attached to the surface of these harmful things.</figcaption> </figure> <p>Antibody-mediated inflammatory brain diseases are relatively new conditions that were previously under-diagnosed. Recognition and awareness of these conditions is growing however. The following is a list of the currently known antibody-mediated inflammatory brain disorders:</p><ul><li>Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis</li><li>Neuromyelitis optica (NMO)</li><li>Limbic encephalitis</li><li>Hashimoto encephalitis</li><li>Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS)</li></ul> <figure class="asset-c-80"> <span class="asset-image-title">Antibody-mediated autoimmune response</span> <img src="https://assets.aboutkidshealth.ca/akhassets/IMD_antibody_mediated_autoimmune_response_EN.jpg" alt="" /> <figcaption class="asset-image-caption">In antibody-mediated inflammatory diseases, the immune system starts creating antibodies against healthy cells. Once marked "bad", the healthy cells become the target of the immune cells’ attack.</figcaption> </figure> <p>Your child's condition is not your fault. It is important for you to understand that your child's disease is not due to anything you or a family member may have done. The exact cause of auto-antibody production is not well understood. Various theories have been proposed including the role of infection, malignancy, environmental triggers and genetics.</p><h2>Key points</h2> <ul> <li>In antibody-mediated inflammatory disease, antibodies are produced that act against the body's own structures. When this happens against structures in the brain this can lead to irritation and swelling of brain tissue. </li> <li>Currently there are five known antibody-mediated inflammatory brain disorders: anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis; neuromyelitis optica (NMO); limbic encephalitis; hashimoto encephalitis; and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS).</li> </ul>
Maladies inflammatoires du cerveau liées à la présence d’anticorpsMMaladies inflammatoires du cerveau liées à la présence d’anticorpsAntibody-mediated inflammatory brain disordersFrenchImmunologyChild (0-12 years);Teen (13-18 years)BrainImmune systemConditions and diseasesCaregivers Adult (19+)NA2013-05-13T04:00:00ZSusanne Benseler, MD;Manisha Sickand, MSc;Marinka Twilt, MD13.000000000000024.00000000000001053.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Découvrez les différents types de maladies inflammatoires du cerveau liées à la présence d’anticorps, ainsi que les signes et les symptômes de chacune d’entre elles. </p><h2>En quoi consistent les maladies inflammatoires du cerveau liées à la présence d’anticorps? </h2><p>Chez les personnes atteintes de ces maladies, les lymphocytes B (qui sont des globules blancs particuliers) produisent des anticorps ciblant les structures mêmes du corps. Lorsque les anticorps, nommés auto anticorps, se lient à ces structures, elles entraînent une inflammation anormale des tissus sains. Les auto anticorps se fixant aux structures du cerveau y provoquent l’irritation et l’enflure des tissus. Si elle n’est pas soignée, une inflammation de longue durée peut causer un dommage et un dysfonctionnement permanent du cerveau. </p> <figure class="asset-c-80"> <span class="asset-image-title">Réponse immunitaire normale en présence d'éléments nuisibles</span> <img src="https://assets.aboutkidshealth.ca/akhassets/IMD_antibody_mediated_normal_immune_response_FR.jpg" alt="" /> <figcaption class="asset-image-caption">Votre système immunitaire produit des anticorps pour « marquer » les éléments nuisibles qui pénètrent dans votre corps. Les cellules immunitaires spéciales savent à quoi s'attaquer, car les anticorps se sont fixés à la surface des éléments nuisibles.</figcaption> </figure> <p>Les maladies inflammatoires du cerveau liées à la présence d’anticorps sont des affections relativement nouvelles qui étaient auparavant sous diagnostiquées. Toutefois, elles font l’objet d’une reconnaissance et d’une sensibilisation grandissantes. Voici les maladies de ce type actuellement connues : </p><ul><li>encéphalite à anticorps antirécepteurs N méthyl D aspartate (NMDA), </li><li>neuromyélite optique (NMO), </li><li>encéphalite limbique, </li><li>encéphalite d’Hashimoto, </li><li>troubles neuropsychiatriques auto-immuns pédiatriques associés aux infections à Streptocoques (PANDAS). </li></ul> <figure class="asset-c-80"> <span class="asset-image-title">Réponse auto-immune liée à la présence d'anticorps</span> <img src="https://assets.aboutkidshealth.ca/akhassets/IMD_antibody_mediated_autoimmune_response_FR.jpg" alt="" /> <figcaption class="asset-image-caption">Dans les maladies inflammatoires liées à la présence d'anticorps, le système immunitaire commence à créer des anticorps ciblant les cellules saines. Une fois que les anticorps « marquent » les cellules saines comme étant malades, les cellules immunitaires s’y attaquent.</figcaption> </figure> <p>Vous n’êtes pas responsable de l’état de votre enfant. Il est important que vous reconnaissiez que ni vous ni aucun membre de votre famille n’auriez pu faire quoi que ce soit pour le provoquer. La cause exacte de la production d’auto-anticorps n’est pas bien comprise. Diverses théories ont été avancées concernant l’origine du phénomène, dont le rôle de l’infection, de la malignité, de déclencheurs environnementaux et de la génétique dans l’affection.</p><h2>À retenir<br></h2><ul><li> En ce qui concerne les maladies inflammatoires du cerveau liées à la présence d’anticorps, les anticorps s’attaquent aux structures mêmes de l’organisme. Quand ces anticorps s’attaquent aux structures du cerveau, ils peuvent irriter et enflammer le tissu cérébral.</li><li>Les cinq maladies inflammatoires du cerveau liées à la présence d’anticorps actuellement connues sont l’encéphalite à anticorps antirécepteurs N méthyl D aspartate (NMDA), la neuromyélite optique (NMO), l’encéphalite limbique, l’encéphalite d’Hashimoto et les troubles neuropsychiatriques auto-immuns pédiatriques associés aux infections à streptocoques (PANDAS).</li></ul>

 

 

Antibody-mediated inflammatory brain disorders927.000000000000Antibody-mediated inflammatory brain disordersAntibody-mediated inflammatory brain disordersAEnglishImmunologyChild (0-12 years);Teen (13-18 years)BrainImmune systemConditions and diseasesCaregivers Adult (19+)NA2013-05-13T04:00:00ZSusanne Benseler, MD;Manisha Sickand, MSc;Marinka Twilt, MD13.000000000000024.00000000000001053.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Learn about the different types antibody-mediated inflammatory brain disorders and what the signs and symptoms are for each.</p><h2>What are antibody-mediated inflammatory brain disorders?</h2><p>In antibody-mediated inflammatory disease, B cells (unique white blood cells) produce antibodies against the body's own structures; these are called autoantibodies. When autoantibodies bind to these structures, they induce unnecessary inflammation that is directed against healthy tissue. Autoantibodies directed against structures in the brain lead to irritation and swelling of brain tissue. If not treated, long standing inflammation can lead to permanent brain damage and dysfunction.</p> <figure class="asset-c-80"> <span class="asset-image-title">Antibody-mediated normal immune response</span> <img src="https://assets.aboutkidshealth.ca/akhassets/IMD_antibody_mediated_normal_immune_response_EN.jpg" alt="" /> <figcaption class="asset-image-caption">Your body’s immune system produces antibodies to "mark" harmful things that enters your body. Special immune cells know what to attack by finding antibodies attached to the surface of these harmful things.</figcaption> </figure> <p>Antibody-mediated inflammatory brain diseases are relatively new conditions that were previously under-diagnosed. Recognition and awareness of these conditions is growing however. The following is a list of the currently known antibody-mediated inflammatory brain disorders:</p><ul><li>Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis</li><li>Neuromyelitis optica (NMO)</li><li>Limbic encephalitis</li><li>Hashimoto encephalitis</li><li>Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS)</li></ul> <figure class="asset-c-80"> <span class="asset-image-title">Antibody-mediated autoimmune response</span> <img src="https://assets.aboutkidshealth.ca/akhassets/IMD_antibody_mediated_autoimmune_response_EN.jpg" alt="" /> <figcaption class="asset-image-caption">In antibody-mediated inflammatory diseases, the immune system starts creating antibodies against healthy cells. Once marked "bad", the healthy cells become the target of the immune cells’ attack.</figcaption> </figure> <p>Your child's condition is not your fault. It is important for you to understand that your child's disease is not due to anything you or a family member may have done. The exact cause of auto-antibody production is not well understood. Various theories have been proposed including the role of infection, malignancy, environmental triggers and genetics.</p><h2>Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis</h2><p>In NMDAR encephalitis, auto-antibodies target the NMDA receptor found on the surface of brain cells. Receptors are very important for cell-to-cell signaling in the brain. It is thought that the action of antibodies binding to the NMDA receptor reduces the number of available receptors on the surface of the brain cells. This has deleterious effects on neuronal signaling, which increase in severity as the number of antibodies increase and the depletion of receptors worsens. </p><p>NMDA receptor encephalitis is a relatively new disease, initially discovered in 2007 in female adults. Its incidence has since increased, with more than 400 cases discovered. With such rapid accrual, it is thought to be the most common antibody-mediated brain disease. It primarily affects young adults and children and also occurs with a female predominance of 80 per cent. In adults, the disease is more commonly associated with an ovarian tumour, however this association is rarely seen in young children and infants. </p><h3>Signs and symptoms</h3><p>The disease typically manifests as a continuum of symptoms as the effects of the auto-antibodies on the receptors increase: </p><ul><li>cold symptoms: <a href="/Article?contentid=29&language=English">headache</a>, <a href="/Article?contentid=30&language=English">fever</a>, nausea, <a href="/Article?contentid=7&language=English">diarrhea</a> </li><li>psychiatric symptoms: behaviour abnormalities, hallucinations, paranoia, temper tantrums, insomnia </li><li>memory loss, speech problems </li><li>decreased responsiveness </li><li>seizures, abnormal movements of face and limbs </li><li>autonomic dysfunction: breathing instability, rapid heartbeat, high blood pressure, increased saliva production. </li></ul><p>In young children, the first noticeable presentation is often that of seizures or abnormal movements, preceded by temper tantrums, irritability or hyperactivity. This is likely because young children and infants cannot communicate exactly what they are feeling. The disease is thought to resolve in reverse order, as antibodies are depleted and receptors return to the surface of neuronal cells. </p><h2>Neuromyelitis optica (NMO)</h2><p>In NMO, auto-antibodies target the aquaporin-4 water channel (AQP4), a protein important in maintaining brain water volume. This causes inflammation, degradation of the channel itself and irritation of the surrounding brain tissue. </p><p>NMO typically presents in the fourth or fifth decade of life and is less commonly seen in children and seniors. In all age groups, NMO occurs with a female predominance. NMO is most common in people who are non-Caucasian. </p><h3>Signs and symptoms </h3><p>Due to the location of the AQP4 channel in the optic nerves and spinal cord, typical symptoms are reflective of damage to these areas: </p><ul><li>hiccups</li><li>nausea and uncontrollable <a href="/Article?contentid=746&language=English">vomiting</a> </li><li>breathing difficulties </li><li>blurry vision and eye pain </li><li>muscle spasms </li><li>burning pain, numbness or paralysis. </li></ul><p>Other areas of the brain can also be affected, resulting in symptoms such as:</p><ul><li>changes in sleep patterns </li><li>hypothermia (decrease in core body temperature) </li><li>confusion</li><li>changes in level of consciousness </li></ul><h2>Limbic encephalitis</h2><p>In limbic encephalitis, auto-antibodies attack a <a href="/Article?contentid=220&language=English">potassium</a> channel in the limbic area of the brain, the part of the brain responsible for emotion and memory. This leads to a reduction in the number of potassium channels which decreases the electrical activity of the brain. </p> <p>Limbic encephalitis is relatively rare in children and adolescents. Two forms exist, one associated with a tumour, seen most often in adults and one that is not associated with a tumour.</p><h3>Signs and symptoms </h3><ul><li>rapid cognitive decline </li><li>behavioural abnormalities </li><li>memory loss </li><li>seizures.</li></ul><h2>Hashimoto encephalitis</h2><p>In Hashimotos encephalitis no auto-antibodies specific to neuronal proteins have been identified. However, the condition is usually seen alongside Hashimoto's thyroiditis, an autoimmune disorder associated with auto-antibodies against the thyroid gland. Yet, despite the presence of autoantibodies, it is unclear whether they are responsible for patients' neurologic symptoms. Nevertheless, most patients respond to immune-based therapies. This suggests that the disorder does have an underlying, un-identified autoimmune cause. </p><p>Hashimoto's encephalitis is a rare complication of Hashimoto's thyroiditis in adults. Its incidence in children is even rarer, but occurs with a female predominance in both populations. </p><h3>Signs and symptoms </h3><p>Symptoms span the neurological and psychiatric spectrum, including: </p><ul><li>seizures </li><li>confusion and hallucinations </li><li>headache </li><li>loss of movement (ataxia) </li><li>coma. </li></ul><h2>Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS)</h2><p>In PANDAS, no neuronal autoantibodies have been identified. Nevertheless, the condition is still thought be an autoimmune response to a streptococcal infection in which children suddenly develop obsessive compulsive (OCD) and/or tic disorders. By definition, PANDAS occur in the paediatric population only. </p><h3>Signs and symptoms</h3><p>Children experience a sudden and dramatic onset of OCD or tic symptoms such as: </p><ul><li>persistent thoughts, images or impulses </li><li>repetitive behaviours: excessive washing, checking, touching, counting, ordering </li><li>abnormal movement </li><li>hyperactivity. </li></ul><p>Other psychiatric/behavioural symptoms may also be present suddenly such as: </p><ul><li><a href="https://akhpub.aboutkidshealth.ca/article?contentid=1922&language=English">attention deficit hyperactivity disorder (ADHD)</a></li><li><a href="https://akhpub.aboutkidshealth.ca/article?contentid=18&language=English">anxiety</a></li><li>nighttime fears </li><li>emotional volatility.</li></ul><h2>Key points</h2> <ul> <li>In antibody-mediated inflammatory disease, antibodies are produced that act against the body's own structures. When this happens against structures in the brain this can lead to irritation and swelling of brain tissue. </li> <li>Currently there are five known antibody-mediated inflammatory brain disorders: anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis; neuromyelitis optica (NMO); limbic encephalitis; hashimoto encephalitis; and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS).</li> </ul>https://assets.aboutkidshealth.ca/akhassets/IMD_antibody_mediated_normal_immune_response_EN.jpgAntibody-mediated inflammatory brain disordersFalse

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