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DystoniaDDystoniaDystoniaEnglishNeurologyChild (0-12 years);Teen (13-18 years)BodyBrainConditions and diseasesCaregivers Adult (19+)NA2020-02-27T05:00:00Z9.9000000000000047.30000000000002029.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Learn about dystonia, a brain disorder that causes muscle stiffness and abnormal postures. </p><h2>What are movement disorders?</h2> <p>Movement disorders are conditions that cause involuntary body movements. With all movement disorders, abnormal signals from the brain cause patients to have trouble controlling their muscles. </p> <p>There are several different types of movement disorders that affect people in different ways. Dystonia, Parkinson's disease, tics and Tourette syndrome are all movement disorders. </p> <h2>What is dystonia?</h2> <p>Dystonia (say: diss-TOE-nee-uh) is a movement disorder that causes sustained muscle contractions. In a person with dystonia, the muscles tense up, often causing twisting and repetitive movements or abnormal postures. In some children, the movements may be painful. There are several different types of dystonia, each with its own characteristics. </p> <p>The condition can vary from very mild to severe. Dystonia may get worse over time or it may stay the same or get better.</p> <p>Some children are born with dystonia. Dystonia may also appear as a child or an adult gets older.</p> <h3>How much of the body and how many muscles are affected by dystonia can vary</h3> <p>In some children, only one part of the body is affected; in other children, several or many parts are affected. How dystonia is treated depends on the type of dystonia, its cause and how severe the case is. </p><h2>Key points</h2> <ul> <li>Dystonia is a movement disorder that causes muscle control problems.</li> <li>There are many different types of dystonia. Some forms are mild and some are more severe. </li> <li>Dystonia can get worse over time. </li> <li>There are several different types of treatments for dystonia, such as medications and surgery.<br></li> </ul><h2>Symptoms of dystonia can differ depending on the part of the body that is affected</h2><p>Posturing due to abnormal muscle contraction can be present in only one region of the body (focal dystonia), such as the leg, the arm or the neck, or it may affect the entire body (generalized dystonia). The following are examples of the ways dystonia affects different parts of the body.</p><ul><li>Cervical dystonia involves the neck and can cause abnormal posturing of the head.</li><li>Limb (upper or lower) dystonia can cause difficulties with walking or fine motor skills, such as those required for writing or playing an instrument.</li><li>Oromandibular dystonia involves the tongue or jaw and can cause slurred speech or chewing or swallowing difficulties, which could lead to reduced food intake and dietary problems.</li><li>Spasmodic dystonia involves the vocal cord and can cause sudden changes in voice pitch and even difficulties breathing.</li></ul><h2>Dystonia begins in the brain</h2><p>Like all movement disorders, dystonia is a neurological condition. The problem begins in the brain. The brain sends abnormal signals to muscles in the body. These signals cause the uncontrolled movements. </p><p>What causes the brain to send these signals is not fully understood. However, dystonia can have both genetic and other causes. </p><p>Sometimes, children with dystonia may also have other neurological problems, such as <a href="/article?contentid=847&language=english">cerebral palsy</a>.</p> <figure> <span class="asset-image-title">Nervous system</span> <img src="https://assets.aboutkidshealth.ca/akhassets/Nervous_system_MED_ILL_EN.jpg" alt="The brain, spinal cord and nerves in a young girl" /> </figure><h2>Diagnosing dystonia</h2> <p>Symptoms of dystonia may appear at any age from young toddlers to older children. Symptoms are usually mild at the beginning and gradually get worse. Very rarely, symptoms appear abruptly and severely. </p> <p>At first, parents usually notice problems with their child's legs. A child may seem clumsy, and may fall or trip a lot. A child may start walking later than normal and may have trouble keeping up with other kids. </p> <p>In some children, dystonia progresses though the day; in the morning, they have a surprising amount of control over their muscles, but in the evening the symptoms are more severe. </p> <p>If your child's doctor suspects your child might have dystonia, your child will be examined by a neurologist. A neurologist is a doctor who specializes in brain, nerve and muscle function. The neurologist will check your child's bones, joints and muscles. Reflexes will also be checked. </p> <p>Other conditions can also cause symptoms similar to dystonia. To diagnose dystonia, the doctor will need to consider these other conditions. </p><h2>Treatment of dystonia</h2><p>How dystonia is treated depends on the type and severity of the condition. There are no treatments to cure dystonia. Treatments target specific symptoms and are intended to relieve muscle spasms, pain and discomfort. Treatment is individualized to each patient — no specific treatment is appropriate for every patient. Children who are still growing need treatment before the dystonia causes permanent bone deformities. </p><p>In general, there are three ways to treat dystonia:</p><ul><li>medicines that are taken by mouth<br></li><li>injections of Botulinum toxin (Botox) </li><li>surgery </li></ul><h2>Medicines taken by mouth<br></h2><p>All medicines for dystonia have side effects, and they must be used carefully according to your doctor's instructions. For more information, please speak to your doctor or pharmacist. </p><h3>Levodopa and carbidopa</h3><p>Many children with dystonia are first given a trial of a medicine called levodopa (L-dopa). Levodopa often works well at restoring muscle control for dopa-responsive dystonia (DRD). Some patients with other types of dystonia can also show some improvement with this medicine. </p><p>Sometimes, levodopa is combined with a medicine called carbidopa. Both these medicines increase dopamine in the brain. Dopamine is a neurotransmitter that regulates movement. </p><p>These medicines may produce side effects such as skin flushing, a drop in blood pressure, nausea, vomiting and diarrhea, among others.</p><h3>Other oral medicines</h3><p>Other medicines can also be used to treat dystonia. They include:</p><ul><li>trihexyphenidyl and other anticholinergic (say: an-tee-call-in-URGE-ic) medicines, which act on neurotransmitters </li><li> <a href="/Article?contentid=82&language=English">baclofen</a>, a muscle relaxant — in children with cerebral palsy, this is often the first line of medication treatment for dystonia<br></li><li>benzodiazepines, such as <a href="/Article?contentid=107&language=English">clonazepam</a> or <a href="/article?contentid=123&language=english">diazepam</a>, which act as a muscle relaxant and sedative </li></ul><p>In more severe cases, a combination of these medicines may be used.</p><h2>Injections of Botulinum toxin (Botox)</h2><p>Most people know that Botox injections can be used to reduce wrinkles. Because Botox works by temporarily paralyzing tissues around an injected area, it can also be used to treat dystonia. </p><p>Botox injections are the treatment of choice for most forms of focal dystonia. However, these conditions are not frequently seen in children. In children, botulinum toxin is used in generalized dystonia patients when targeting a certain part of the body. It is often used when there is spasticity as well as dystonia, particularly in the legs. Spasticity means the brain is constantly telling the muscles to tighten or contract. </p><p>The effect of Botox injections usually lasts about three to four months. After this, more injections will be needed.</p><p>Botox injections are limited in what they can accomplish. If too many places are injected, for example, the child can temporarily lose control of movement altogether. </p><h2>Surgical treatment of dystonia</h2><p>If dystonia does not respond to other treatments, the neurologist or developmental paediatrician may suggest a surgical treatment.</p><h3>Intra-thecal baclofen</h3><p>Patients with generalized dystonia may be treated with <a href="/Article?contentid=81&language=English">intra-thecal baclofen</a> (ITB). Like the medicines by mouth discussed above, baclofen is a medicine that relaxes muscles by acting on neurotransmitters in the brain. </p><p>Intra-thecal baclofen is given through a small tube that is surgically placed into the spine. The tube is attached to a pump that is placed in the soft tissue of the abdominal wall. After the operation, the pump is programmed to deliver a continuous amount of baclofen into the fluid around the spine. The pump can be re-programmed by the doctor to adjust for a child's specific needs.<br></p><p>The treatment can be effective for most people, but for about one in 10 patients (10%), the treatment will stop working within the first year. </p><p>Complications of ITB may include:</p><ul><li>infection </li><li>decreased head and neck control </li><li>drowsiness </li><li>leakage of cerebral spinal fluid (CSF) </li><li>catheter or pump malfunction </li><li>baclofen withdrawal </li><li>overdose </li></ul><p>These complications can be very dangerous. Patients and families must learn about the signs and symptoms for these complications, so that they can seek medical attention as soon as possible. </p><h3>Deep brain stimulation</h3><p>Deep brain stimulation (DBS) is a relatively new treatment for children. It tends to work best on the genetic forms of dystonia but also works in other forms of dystonia. </p><p>DBS controls dystonia by putting a small amount of electricity into the brain through tiny electrodes. The electrodes are attached to a battery. The brain is not surgically opened; instead, a tiny hole is made in the skull. </p><p>Sometimes, it can take several months for the treatment to become effective. Often, the effect improves over a period of six months to a year.<br></p><p>Complications of DBS may include:</p><ul><li>mechanical problems such as broken wires, displaced electrodes, or battery failure </li><li>infections </li><li>bleeding in the brain </li><li>stroke<br></li></ul><p>These complications can be very dangerous. Patients and families must learn about the signs and symptoms for these complications, so that they can seek medical attention as soon as possible. </p><h2>Potential complications of dystonia</h2><p>The complications your child may experience will depend on the type of dystonia they have. Potential complications of dystonia include:</p><ul><li>difficulties performing daily activities or specific tasks (writing, playing an instrument, etc.)</li><li>in severe cases, fixed contraction (tightening) of a joint (wrist, ankle, etc.) or scoliosis</li><li>difficulties chewing and swallowing, which may eventually lead to malnutrition</li><li>depression, anxiety</li></ul><h2>Take your child to the nearest emergency department if they experience dystonic storm</h2><p>Dystonic storm is a rare presentation of dystonia that is primarily seen in children with pre-existing motor abnormalities. This usually occurs as a result of an acute illness or after general anaesthesia in a child with a history of dystonia. The child will present with continuous dystonia that can last for a few hours. The child might be diaphoretic (covered with sweat) and in pain. If this occurs, take your child to the nearest emergency department.</p><h2>Resources</h2><p><a href="https://www.dystoniacanada.org/">Dystonia Medical Research Foundation Canada</a></p><p><a href="https://dystonia-foundation.org/">Dystonia Medical Research Foundation (USA)</a></p>
DystonieDDystonieDystoniaFrenchNeurologyChild (0-12 years);Teen (13-18 years)BodyBrainConditions and diseasesCaregivers Adult (19+)NA2009-11-06T05:00:00Z10.000000000000044.00000000000001700.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Apprenez-en davantage sur la dystonie, un trouble du cerveau qui provoque une perte de contrôle musculaire.</p><h2>Que sont les troubles du mouvement?</h2> <p>Les troubles du mouvement désignent des états qui provoquent des mouvements involontaires du corps. Dans tous les cas de troubles du mouvement, les signaux anormaux du cerveau font que les patients ont du mal à contrôler leurs muscles.</p> <p>Il existe différents types de troubles du mouvement qui peuvent toucher les personnes atteintes de diverses façons. La dystonie, la maladie de Parkinson, les tics et le syndrome de la Tourette constituent des troubles du mouvement.</p> <h2>Qu'est-ce que la dystonie?</h2> <p>La dystonie est un trouble du mouvement qui provoque des contractions musculaires soutenues. Chez une personne atteinte de dystonie, les muscles se tendent, ce qui entraîne souvent des contorsions et des mouvements répétitifs ou des postures anormales. Chez certains enfants, ces mouvements peuvent être douloureux. Il existe divers types de dystonie, chacune avec ses particularités.</p> <p>L’état peut aller de léger à grave. La dystonie peut s’aggraver avec le temps, ne pas changer, ou encore, s’améliorer.</p> <p>L’état peut aller de léger à grave. La dystonie peut s’aggraver avec le temps, ne pas changer, ou encore, s’améliorer.</p> <h3>La proportion corporelle et le nombres de muscles touchés par une dystonie peuvent varier</h3> <p>Chez certains enfants, seule une partie du corps est touchée; chez d’autres, plusieurs parties ou de nombreuses parties du corps sont touchées. Le traitement de la dystonie et le pronostic dépendent du type de dystonie et de la gravité du cas. </p><h2>À retenir</h2> <ul> <li>La dystonie est un trouble du mouvement qui entraîne des problèmes de contrôle musculaire.</li> <li>Il existe de nombreux types de dystonies, certaines légères et d’autres graves.</li> <li>La dystonie peut s’aggraver avec le temps.</li> <li>Il existe divers traitements qui peuvent réduire les effets de nombreuses dystonies. </li> </ul><h2>La dystonie commence dans le cerveau</h2><p>Comme pour tous les troubles du mouvement, la dystonie est une affection neurologique. Cela signifie que le problème commence avec le cerveau qui envoie des signaux anormaux aux muscles du corps. Ces signaux entraînent des mouvements incontrôlés. </p><p>On ne comprend pas complètement pourquoi le cerveau envoie de tels signaux. Cependant, nous savons que la dystonie peut avoir à la fois des causes génétiques et environnementales.</p><p>Parfois, les enfants atteints de dystonie ont également d’autres problèmes neurologiques. Ces autres problèmes neurologiques peuvent affecter la capacité de penser d’un enfant. Dans d’autres cas, seuls les muscles et les mouvements sont touchés.</p> <figure> <span class="asset-image-title">Système nerveux</span> <img src="https://assets.aboutkidshealth.ca/akhassets/Nervous_system_MED_ILL_FR.jpg" alt="Le cerveau, la moelle épinière et les nerfs dans une jeune fille" /> </figure><br><h2>Diagnostic de la dystonie</h2> <p>Les symptômes de la dystonie peuvent apparaître à n’importe quel âge, du plus jeune au plus vieux. Les symptômes sont habituellement légers au début, puis ils s’aggravent. Très rarement, les symptômes apparaissent soudainement et sont sévères.</p> <p>Au début, les parents constatent habituellement des problèmes avec les jambes de leur enfant. L'enfant peut sembler maladroit et il peut tomber ou trébucher souvent. L'enfant peut commencer à marcher plus tard que la normale et peut avoir du mal à suivre les autres enfants.</p> <p>Chez certains enfants, la dystonie progresse au cours de la journée; le matin, ils ont un bon contrôle de leurs muscles, mais le soir, les symptômes sont plus graves.</p> <p>Si le médecin de votre enfant pense qu’il peut être atteint de dystonie, votre enfant sera examiné par un neurologue. Un neurologue est un médecin spécialiste du cerveau, du système nerveux et des fonctions musculaires. Ce médecin examinera les os, les articulations et les muscles de votre enfant, ainsi que ses réflexes et ses sensations.</p> <p>D’autres états de santé peuvent également causer des symptômes semblables à la dystonie. Pour diagnostiquer une dystonie, le médecin devra tenir compte de ces autres maladies. </p><h2>Traitement de la dystonie</h2> <p>La manière dont la dystonie est traitée dépend de son type et de sa gravité, ainsi que du patient. Certaines dystonies répondent bien à différents traitements. D’autres offrent peu d’options de traitement.</p> <p>En général, il existe trois façons de traiter la dystonie :</p> <ul> <li>Médicaments par voie orale (par la bouche)</li> <li>Injections de toxine botulinique (Botox)</li> <li>Chirurgie</li> </ul> <h2>Médicaments par voie orale</h2> <p>Tous les médicaments pour la dystonie ont des effets secondaires, et ils doivent être pris soigneusement selon les instructions de votre médecin. Pour plus de renseignements, consultez votre médecin ou votre pharmacien.</p> <h3>Lévodopa et carbidopa</h3> <p>De nombreux enfants atteints de dystonie essayent tou d'abord un médicament appelé lévodopa (L‑dopa). Souvent, la lévodopa arrive bien à restaurer le contrôle musculaire dans les cas de dystonie dopa-sensible. Certains patients avec d’autres types de dystonie peuvent également constater une amélioration avec ce médicament. </p> <p>Parfois, la lévodopa est combinée à un autre médicament appelé carbidopa. Ces deux médicaments augmentent la dopamine dans le cerveau. La dopamine est un neurotransmetteur qui régule le mouvement. </p> <p>Ces médicaments peuvent avoir des effets secondaires comme, entre autres, des rougeurs cutanées, une baisse de la pression artérielle et des nausées.</p> <h3>Autres médicaments par voie orale</h3> <p>D’autres médicaments peuvent également être utilisés pour traiter la dystonie. Ils comprennent :</p> <ul> <li>la trihexyphénidyle et d’autres anticholinergiques (prononcez : an-ti-co-li-ner-gique), qui agissent sur les neurotransmetteurs;</li> <li>le baclofène, un relaxant musculaire;</li> <li>la clonazépam, une benzodiazépine qui agit comme un relaxant musculaire et comme sédatif.</li> </ul> <p>Dans les cas les plus graves, il est possible d’utiliser une combinaison de ces médicaments.</p> <h2>Injections de toxine botulinique (Botox)</h2> <p>La plupart des gens savent que des injections de Botox peuvent être utilisées pour réduire les rides. Le Botox agit en paralysant de façon temporaire les tissus autour de la zone de l’injection, et il peut donc être également utilisé pour traiter la dystonie. </p> <p>Les injections de Botox constituent le traitement de choix pour la plupart des formes de dystonies focales. Cependant, on ne rencontre pas souvent ce type de dystonie chez les enfants. Chez les enfants, la toxine botulinique est utilisée pour traiter les patients atteints de dystonie généralisée lorsqu’on cible une certaine partie du corps. Elle est souvent utilisée dans le cas de spasticité combinée à la dystonie, surtout dans les jambes. La spasticité signifie que le cerveau demande constamment aux muscles de se contracter.</p> <p>L’effet des injections de Botox dure en général de 3 à 4 mois. Ensuite, il faut de nouvelles injections.</p> <p>Veuillez noter que ce que les injections de Botox permettent d’accomplir est limité. Par exemple, si trop d’endroits reçoivent des injections, l’enfant pourrait perdre temporairement le contrôle complet du mouvement. </p> <h2>Traitement chirurgical de la dystonie</h2> <p>Si la dystonie ne répond pas aux autres traitements, le neurologue peut envisager un traitement chirurgical.</p> <h3>Baclofène intrathécal</h3> <p>Les patients atteints d’une dystonie généralisée peuvent être traités avec du baclofène intrathécal. Tout comme les autres médicaments présentés ci-dessus, le baclofène est un médicament qui relaxe les muscles en agissant sur les neurotransmetteurs du cerveau.</p> <p>Le baclofène intrathécal est souvent administré en utilisant un cathéter (un tube mince) placé lors d’une chirurgie dans la colonne vertébrale. Le cathéter est relié à une pompe insérée dans les tissus mous de la paroi abdominale. Après l’opération, la pompe est programmée pour administrer une quantité continue de baclofène. </p> <p>Le traitement peut être efficace pour la plupart des gens, mais chez environ 1 patient sur 10 (10 %), le traitement cesse de fonctionner au cours de la première année. </p> <p>Les complications associées à l’utilisation du baclofène intrathécal peuvent comprendre :</p> <ul> <li>une infection,</li> <li>une diminution du contrôle de la tête et du cou,</li> <li>de la somnolence,</li> <li>une fuite du liquide céphalo-rachidien (LCR),</li> <li>un défaut de fonctionnement du cathéter ou de la pompe,</li> <li>un sevrage au baclofène,</li> <li>une surdose. </li> </ul> <p>Ces complications peuvent être très dangereuses. Les patients et les familles doivent apprendre à reconnaître les signes et les symptômes de ces complications afin de pouvoir obtenir de l’aide médicale dès que possible.</p> <h3>Stimulation cérébrale profonde</h3> <p>La stimulation cérébrale profonde est un traitement relativement nouveau pour les enfants. Elle semble fonctionner le mieux sur les formes génétiques (primaires) de dystonies, maisfonctionne également sur d’autres formes de dystonies.</p> <p>La stimulation cérébrale profonde contrôle la dystonie en induisant une petite quantité d’électricité dans le cerveau à l’aide de petites électrodes. Les électrodes sont reliées à un accumulateur. Aucune chirurgie n’est requise pour ouvrir le cerveau; à la place, on pratique un tout petit trou dans le crâne.</p> <p>Parfois, il peut s’écouler plusieurs mois avant que le traitement devienne efficace. Souvent, l’effet s’améliore sur une période de 6 mois à un an. Les enfants en croissance doivent être traités avant que la dystonie cause des déformations permanentes des os.</p> <p>Les complications associées à l’utilisation de la stimulation cérébrale profonde peuvent comprendre :</p> <ul> <li>problèmes mécaniques tels que des fils électriques cassés, des électrodes déplacées ou un problème de baterie,</li> <li>infections,</li> <li>saignements dans le cerveau,</li> <li>accident vasculaire cérébral,</li> <li>mort (rare).</li> </ul> <p>On a également rapporté quelques cas de problèmes psychiatriques comme la dépression et le suicide.</p> <p>Ces complications peuvent être très dangereuses. Les patients et les familles doivent apprendre à reconnaître les signes et les symptômes de ces complications afin de pouvoir obtenir de l’aide médicale dès que possible.</p>

 

 

 

 

Dystonia856.000000000000DystoniaDystoniaDEnglishNeurologyChild (0-12 years);Teen (13-18 years)BodyBrainConditions and diseasesCaregivers Adult (19+)NA2020-02-27T05:00:00Z9.9000000000000047.30000000000002029.00000000000Health (A-Z) - ConditionsHealth A-Z<p>Learn about dystonia, a brain disorder that causes muscle stiffness and abnormal postures. </p><h2>What are movement disorders?</h2> <p>Movement disorders are conditions that cause involuntary body movements. With all movement disorders, abnormal signals from the brain cause patients to have trouble controlling their muscles. </p> <p>There are several different types of movement disorders that affect people in different ways. Dystonia, Parkinson's disease, tics and Tourette syndrome are all movement disorders. </p> <h2>What is dystonia?</h2> <p>Dystonia (say: diss-TOE-nee-uh) is a movement disorder that causes sustained muscle contractions. In a person with dystonia, the muscles tense up, often causing twisting and repetitive movements or abnormal postures. In some children, the movements may be painful. There are several different types of dystonia, each with its own characteristics. </p> <p>The condition can vary from very mild to severe. Dystonia may get worse over time or it may stay the same or get better.</p> <p>Some children are born with dystonia. Dystonia may also appear as a child or an adult gets older.</p> <h3>How much of the body and how many muscles are affected by dystonia can vary</h3> <p>In some children, only one part of the body is affected; in other children, several or many parts are affected. How dystonia is treated depends on the type of dystonia, its cause and how severe the case is. </p><h2>Classifying dystonia</h2><p>Part of diagnosis is finding out what type of dystonia a child has. This is important because the type of dystonia will help determine which treatments may work best. Also, different types of dystonia will progress in different ways over time. </p><p>In general, dystonia is classified based on two factors:</p><ol><li> <strong>Clinical features</strong></li><ul><li>Age: how old the child is at the onset of symptoms</li><ul><li>infancy onset: birth to two years old</li><li>childhood onset: between three and 12 years old</li><li>adolescent onset: between 13 and 20 years old</li><li>adult onset: older than 20 years</li></ul><li>Body distribution of the dystonia: where and how much of the body is affected</li><ul><li>focal: only one part of the body, such as the leg, is affected</li><li>segmental: two adjacent parts of the body are affected, such as the head and neck</li><li>hemi-dystonia: different parts on the same side of the body are affected, such as the left hand and left foot</li><li>generalized: multiple parts of the body are affected, such as both arms and one leg</li></ul><li>Course of the disease: how the condition develops over time</li><ul><li>progressive: gets worse over time</li><li>episodic: comes and goes</li><li>static: does not get better or worse over time</li></ul></ul><li> <strong>Cause</strong></li><ul><li>Genetic dystonia is caused by an underlying genetic mutation.</li><li>Idiopathic generalized dystonia and dopa-responsive dystonia (DRD or Segawa's disease) are two types of primary dystonia, which are caused by genetic mutation.</li><li>Acquired dystonia is a result of the brain being injured by exposure to a toxin or drug, a stroke, infection, birth injury or other trauma.</li></ul></ol><h2>Key points</h2> <ul> <li>Dystonia is a movement disorder that causes muscle control problems.</li> <li>There are many different types of dystonia. Some forms are mild and some are more severe. </li> <li>Dystonia can get worse over time. </li> <li>There are several different types of treatments for dystonia, such as medications and surgery.<br></li> </ul><h2>Symptoms of dystonia can differ depending on the part of the body that is affected</h2><p>Posturing due to abnormal muscle contraction can be present in only one region of the body (focal dystonia), such as the leg, the arm or the neck, or it may affect the entire body (generalized dystonia). The following are examples of the ways dystonia affects different parts of the body.</p><ul><li>Cervical dystonia involves the neck and can cause abnormal posturing of the head.</li><li>Limb (upper or lower) dystonia can cause difficulties with walking or fine motor skills, such as those required for writing or playing an instrument.</li><li>Oromandibular dystonia involves the tongue or jaw and can cause slurred speech or chewing or swallowing difficulties, which could lead to reduced food intake and dietary problems.</li><li>Spasmodic dystonia involves the vocal cord and can cause sudden changes in voice pitch and even difficulties breathing.</li></ul><h2>Dystonia begins in the brain</h2><p>Like all movement disorders, dystonia is a neurological condition. The problem begins in the brain. The brain sends abnormal signals to muscles in the body. These signals cause the uncontrolled movements. </p><p>What causes the brain to send these signals is not fully understood. However, dystonia can have both genetic and other causes. </p><p>Sometimes, children with dystonia may also have other neurological problems, such as <a href="/article?contentid=847&language=english">cerebral palsy</a>.</p> <figure> <span class="asset-image-title">Nervous system</span> <img src="https://assets.aboutkidshealth.ca/akhassets/Nervous_system_MED_ILL_EN.jpg" alt="The brain, spinal cord and nerves in a young girl" /> </figure><h2>Course of dystonia</h2><p>The most common forms of dystonia that develop in childhood are caused by cerebral palsy. Cerebral palsy is a result of non-progressive damage (damage that does not get better or worse) to the developing brain of a fetus or infant. However, there are other forms of dystonia that develop in childhood that could have progressive symptoms. These forms result from genetic conditions. The exact progression of each form will vary depending on the underlying cause.</p><h2>Diagnosing dystonia</h2> <p>Symptoms of dystonia may appear at any age from young toddlers to older children. Symptoms are usually mild at the beginning and gradually get worse. Very rarely, symptoms appear abruptly and severely. </p> <p>At first, parents usually notice problems with their child's legs. A child may seem clumsy, and may fall or trip a lot. A child may start walking later than normal and may have trouble keeping up with other kids. </p> <p>In some children, dystonia progresses though the day; in the morning, they have a surprising amount of control over their muscles, but in the evening the symptoms are more severe. </p> <p>If your child's doctor suspects your child might have dystonia, your child will be examined by a neurologist. A neurologist is a doctor who specializes in brain, nerve and muscle function. The neurologist will check your child's bones, joints and muscles. Reflexes will also be checked. </p> <p>Other conditions can also cause symptoms similar to dystonia. To diagnose dystonia, the doctor will need to consider these other conditions. </p><h2>Treatment of dystonia</h2><p>How dystonia is treated depends on the type and severity of the condition. There are no treatments to cure dystonia. Treatments target specific symptoms and are intended to relieve muscle spasms, pain and discomfort. Treatment is individualized to each patient — no specific treatment is appropriate for every patient. Children who are still growing need treatment before the dystonia causes permanent bone deformities. </p><p>In general, there are three ways to treat dystonia:</p><ul><li>medicines that are taken by mouth<br></li><li>injections of Botulinum toxin (Botox) </li><li>surgery </li></ul><h2>Medicines taken by mouth<br></h2><p>All medicines for dystonia have side effects, and they must be used carefully according to your doctor's instructions. For more information, please speak to your doctor or pharmacist. </p><h3>Levodopa and carbidopa</h3><p>Many children with dystonia are first given a trial of a medicine called levodopa (L-dopa). Levodopa often works well at restoring muscle control for dopa-responsive dystonia (DRD). Some patients with other types of dystonia can also show some improvement with this medicine. </p><p>Sometimes, levodopa is combined with a medicine called carbidopa. Both these medicines increase dopamine in the brain. Dopamine is a neurotransmitter that regulates movement. </p><p>These medicines may produce side effects such as skin flushing, a drop in blood pressure, nausea, vomiting and diarrhea, among others.</p><h3>Other oral medicines</h3><p>Other medicines can also be used to treat dystonia. They include:</p><ul><li>trihexyphenidyl and other anticholinergic (say: an-tee-call-in-URGE-ic) medicines, which act on neurotransmitters </li><li> <a href="/Article?contentid=82&language=English">baclofen</a>, a muscle relaxant — in children with cerebral palsy, this is often the first line of medication treatment for dystonia<br></li><li>benzodiazepines, such as <a href="/Article?contentid=107&language=English">clonazepam</a> or <a href="/article?contentid=123&language=english">diazepam</a>, which act as a muscle relaxant and sedative </li></ul><p>In more severe cases, a combination of these medicines may be used.</p><h2>Injections of Botulinum toxin (Botox)</h2><p>Most people know that Botox injections can be used to reduce wrinkles. Because Botox works by temporarily paralyzing tissues around an injected area, it can also be used to treat dystonia. </p><p>Botox injections are the treatment of choice for most forms of focal dystonia. However, these conditions are not frequently seen in children. In children, botulinum toxin is used in generalized dystonia patients when targeting a certain part of the body. It is often used when there is spasticity as well as dystonia, particularly in the legs. Spasticity means the brain is constantly telling the muscles to tighten or contract. </p><p>The effect of Botox injections usually lasts about three to four months. After this, more injections will be needed.</p><p>Botox injections are limited in what they can accomplish. If too many places are injected, for example, the child can temporarily lose control of movement altogether. </p><h2>Surgical treatment of dystonia</h2><p>If dystonia does not respond to other treatments, the neurologist or developmental paediatrician may suggest a surgical treatment.</p><h3>Intra-thecal baclofen</h3><p>Patients with generalized dystonia may be treated with <a href="/Article?contentid=81&language=English">intra-thecal baclofen</a> (ITB). Like the medicines by mouth discussed above, baclofen is a medicine that relaxes muscles by acting on neurotransmitters in the brain. </p><p>Intra-thecal baclofen is given through a small tube that is surgically placed into the spine. The tube is attached to a pump that is placed in the soft tissue of the abdominal wall. After the operation, the pump is programmed to deliver a continuous amount of baclofen into the fluid around the spine. The pump can be re-programmed by the doctor to adjust for a child's specific needs.<br></p><p>The treatment can be effective for most people, but for about one in 10 patients (10%), the treatment will stop working within the first year. </p><p>Complications of ITB may include:</p><ul><li>infection </li><li>decreased head and neck control </li><li>drowsiness </li><li>leakage of cerebral spinal fluid (CSF) </li><li>catheter or pump malfunction </li><li>baclofen withdrawal </li><li>overdose </li></ul><p>These complications can be very dangerous. Patients and families must learn about the signs and symptoms for these complications, so that they can seek medical attention as soon as possible. </p><h3>Deep brain stimulation</h3><p>Deep brain stimulation (DBS) is a relatively new treatment for children. It tends to work best on the genetic forms of dystonia but also works in other forms of dystonia. </p><p>DBS controls dystonia by putting a small amount of electricity into the brain through tiny electrodes. The electrodes are attached to a battery. The brain is not surgically opened; instead, a tiny hole is made in the skull. </p><p>Sometimes, it can take several months for the treatment to become effective. Often, the effect improves over a period of six months to a year.<br></p><p>Complications of DBS may include:</p><ul><li>mechanical problems such as broken wires, displaced electrodes, or battery failure </li><li>infections </li><li>bleeding in the brain </li><li>stroke<br></li></ul><p>These complications can be very dangerous. Patients and families must learn about the signs and symptoms for these complications, so that they can seek medical attention as soon as possible. </p><h2>Potential complications of dystonia</h2><p>The complications your child may experience will depend on the type of dystonia they have. Potential complications of dystonia include:</p><ul><li>difficulties performing daily activities or specific tasks (writing, playing an instrument, etc.)</li><li>in severe cases, fixed contraction (tightening) of a joint (wrist, ankle, etc.) or scoliosis</li><li>difficulties chewing and swallowing, which may eventually lead to malnutrition</li><li>depression, anxiety</li></ul><h2>Take your child to the nearest emergency department if they experience dystonic storm</h2><p>Dystonic storm is a rare presentation of dystonia that is primarily seen in children with pre-existing motor abnormalities. This usually occurs as a result of an acute illness or after general anaesthesia in a child with a history of dystonia. The child will present with continuous dystonia that can last for a few hours. The child might be diaphoretic (covered with sweat) and in pain. If this occurs, take your child to the nearest emergency department.</p><h2>What to expect in the future</h2><p>There are many different types of dystonia. There is no one set way the condition progresses. Ask your child's doctor what you can expect.</p><p>Typically, when dystonia is diagnosed in a child, the condition can slowly but steadily get worse over time. However, this does not mean that a patient's mental capacity or cognitive abilities will also get worse. In other words, other parts of the nervous system, may or may not be affected.</p><h2>Resources</h2><p><a href="https://www.dystoniacanada.org/">Dystonia Medical Research Foundation Canada</a></p><p><a href="https://dystonia-foundation.org/">Dystonia Medical Research Foundation (USA)</a></p>https://assets.aboutkidshealth.ca/akhassets/Nervous_system_MED_ILL_EN.jpgDystoniaFalse

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