Chorea
Huntington Disease
Rheumatic Fever
Chorea Gravidarum
Anti-Dyskinesia Agents
Autoimmune Diseases of the Nervous System
Familial essential ("benign") chorea. (1/216)
A family is described with essential non-progressive chorea occurring in an autosomal dominant inheritance pattern over four generations. A few families with an apparently similar disorder have been reported previously. This condition is characterized by early childhood onset of chorea which is not progressive and is compatible with a long life. It is not associated with dementia, seizures, rigidity, or ataxia. It is a socially embarrassing condition and may, sometimes, be associated with behavioural problems and learning difficulties. For genetic counselling, it is important to distinguish this disorder from Huntington's disease and other hereditary disorders associated with chorea. (+info)Rheumatic chorea in northern Australia: a clinical and epidemiological study. (2/216)
To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years at first episode. Chorea occurred in 28% of cases of acute rheumatic fever, carditis occurred in 25% of episodes of chorea, and arthritis in 8%. Patients with carditis or arthritis tended to have raised acute phase reactants and streptococcal serology. Two episodes lasted at least 30 months. Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence. Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea. Differences in the patterns of chorea and other manifestations of acute rheumatic fever in different populations may hold clues to its pathogenesis. Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence. (+info)Anticonvulsant-induced dyskinesias: a comparison with dyskinesias induced by neuroleptics. (3/216)
Anticonvulsants cause dyskinesias more commonly than has been appreciated. Diphenylhydantoin (DPH), carbamazepine, primidone, and phenobarbitone may cause asterixis. DPH, but not other anticonvulsants, may cause orofacial dyskinesias, limb chorea, and dystonia in intoxicated patients. These dyskinesias are similar to those caused by neuroleptic drugs and may be related to dopamine antagonistic properties possessed by DPH. (+info)Paroxysmal kinesigenic choreoathetosis associated with frontotemporal arachnoid cyst--case report. (4/216)
A 17-year-old male presented with paroxysmal kinesigenic choreoathetosis (PKC) associated with frontotemporal arachnoid cyst. Xenon-133 single photon emission computed tomography detected a slight but equivocal decrease in regional cerebral blood flow in the vicinity of basal ganglia associated with the PKC episodes. PKC continued after surgical removal of the cyst but was well controlled by oral administration of carbamazepine. Whether the pathogenesis of symptomatic PKC was associated with the cortical lesion could not be determined in the present case. (+info)Intracortical inhibition of the motor cortex is normal in chorea. (5/216)
Intracortical inhibition of the motor cortex was investigated using a paired pulse magnetic stimulation method in 14 patients with chorea caused by various aetiologies (six patients with Huntington's disease, one with chorea acanthocytosis, a patient with systemic lupus erythematosus with a vascular lesion in the caudate, three with senile chorea and three with chorea of unknown aetiology). The time course and amount of inhibition was the same in the patients as in normal subjects, suggesting that the inhibitory mechanisms of the motor cortex studied with this method are intact in chorea. This is in striking contrast with the abnormal inhibition seen in patients with Parkinson's disease or focal hand dystonia, or those with a lesion in the putamen or globus pallidus. It is concluded that the pathophysiological mechanisms responsible for chorea are different from those producing other involuntary movements. (+info)Movement disorders caused by brain tumours. (6/216)
Movement disorders are uncommon presenting features of brain tumours. Early recognition of such lesions is important to arrest further deficit. We treated seven patients with movement disorders secondary to brain tumours over a period of seven years. Only two of these were intrinsic thalamic tumours (astrocytomas) while the rest were extrinsic tumours. The intrinsic tumours were accompanied by hemichorea. Among the extrinsic tumours, there was one pituitary macroadenoma with hemiballismus and four meningiomas with parkinsonism. Symptoms were unilateral in all patients except one with anterior third falcine meningioma who had bilateral rest tremors. There was relief in movement disorders observed after surgery. Imaging by computed tomography or magnetic resonance imaging is mandatory in the evaluation of movement disorders, especially if the presentation is atypical, unilateral and/or accompanied by long tract signs. (+info)From off-period dystonia to peak-dose chorea. The clinical spectrum of varying subthalamic nucleus activity. (7/216)
The effect of chronic bilateral high-frequency stimulation of the subthalamic nucleus (STN) on levodopa-induced dyskinaesias was investigated in eight patients with fluctuating Parkinson's disease complicated by functionally disabling off-period dystonia. All of the patients also had severe diphasic and peak-dose chorea, so that it was possible to study the effect of high-frequency stimulation on the different types of levodopa-induced dyskinaesias. Off-period fixed dystonia was reduced by 90% and off-period pain by 66%. After acute levodopa challenge, high-frequency stimulation of the STN reduced diphasic mobile dystonia by 50% and peak-dose choreic dyskinaesias by 30%. The effect of bilateral high-frequency stimulation of the STN on the Unified Parkinson's Disease Rating Scale motor score had the same magnitude as the preoperative effect of levodopa. This allowed the levodopa dose to be reduced by 47%. The combination of reduced medication and continuous high-frequency stimulation of the STN reduced the duration of on-period diphasic and peak-dose dyskinaesias by 52% and the intensity by 68%. Acute high-frequency stimulation of the STN mimics an acute levodopa challenge, concerning both parkinsonism and dyskinaesias, and suppresses off-period dystonia. Increasing the voltage can induce repetitive dystonic dyskinaesias, mimicking diphasic levodopa-induced dyskinaesias. A further increase in voltage leads to a shift from a diphasic-pattern dystonia to a peak-dose pattern choreodystonia. Chronic high-frequency stimulation of the STN also mimics the benefit of levodopa on parkinsonism and improves all kinds of levodopa-induced dyskinaesias to varying degrees. Off-period dystonia, associated with neuronal hyperactivity in the STN is directly affected by stimulation and disappears immediately. The effect of chronic high-frequency stimulation of the STN on diphasic and peak-dose dyskinaesias is more complex and is related directly to the functional inhibition of the STN and indirectly to the replacement of the pulsatile dopaminergic stimulation by continuous functional inhibition of the STN. Chronic high-frequency stimulation of the STN allows a very gradual increase in stimulation parameters with increasing beneficial effect on parkinsonism while reducing the threshold for the elicitation of stimulation-induced dyskinaesias. In parallel with improvement of parkinsonism, the levodopa dose can be gradually decreased. As diphasic dystonic dyskinaesias are improved to a greater degree than peak-dose dyskinaesias, both direct and indirect mechanisms may be involved. Peak-dose choreatic dyskinaesias, associated with little evidence of parkinsonism and thus with low neuronal activity in the STN, are improved, mostly indirectly. Fixed off-period dystonia, mobile diphasic dystonia and peak-dose choreodystonia seem to represent a continuous clinical spectrum reflecting a continuous spectrum of underlying activity patterns of STN neurons. (+info)Paroxysmal kinesigenic choreoathetosis locus maps to chromosome 16p11.2-q12.1. (8/216)
Paroxysmal kinesigenic choreoathetosis (PKC), the most frequently described type of paroxysmal dyskinesia, is characterized by recurrent, brief attacks of involuntary movements induced by sudden voluntary movements. Some patients with PKC have a history of infantile afebrile convulsions with a favorable outcome. To localize the PKC locus, we performed genomewide linkage analysis on eight Japanese families with autosomal dominant PKC. Two-point linkage analysis provided a maximum LOD score of 10.27 (recombination fraction [theta] =.00; penetrance [p] =.7) at marker D16S3081, and a maximum multipoint LOD score for a subset of markers was calculated to be 11.51 (p = 0.8) at D16S3080. Haplotype analysis defined the disease locus within a region of approximately 12.4 cM between D16S3093 and D16S416. P1-derived artificial chromosome clones containing loci D16S3093 and D16S416 were mapped, by use of FISH, to 16p11.2 and 16q12.1, respectively. Thus, in the eight families studied, the chromosomal localization of the PKC critical region (PKCR) is 16p11.2-q12.1. The PKCR overlaps with a region responsible for "infantile convulsions and paroxysmal choreoathetosis" (MIM 602066), a recently recognized clinical entity with benign infantile convulsions and nonkinesigenic paroxysmal dyskinesias. (+info)Chorea is a medical term that describes an involuntary movement disorder characterized by brief, irregular, and abrupt jerky movements. These movements often occur randomly and can affect any part of the body. Chorea can also cause difficulty with coordination and balance, and can sometimes be accompanied by muscle weakness or rigidity.
The term "chorea" comes from the Greek word "χορεία" (khoréia), which means "dance," reflecting the graceful, dance-like movements that are characteristic of this condition. Chorea can occur as a symptom of various underlying medical conditions, including neurological disorders such as Huntington's disease, Sydenham's chorea, and cerebral palsy, as well as metabolic disorders, infections, and certain medications.
Treatment for chorea depends on the underlying cause of the condition and may include medications to help control the involuntary movements, physical therapy to improve coordination and balance, and lifestyle modifications to reduce the risk of injury from falls or other accidents. In some cases, surgery may be recommended as a last resort for severe or refractory chorea.
Huntington Disease (HD) is a genetic neurodegenerative disorder that affects both cognitive and motor functions. It is characterized by the progressive loss of neurons in various areas of the brain, particularly in the striatum and cortex. The disease is caused by an autosomal dominant mutation in the HTT gene, which codes for the huntingtin protein. The most common mutation is a CAG repeat expansion in this gene, leading to the production of an abnormal form of the huntingtin protein that is toxic to nerve cells.
The symptoms of HD typically appear between the ages of 30 and 50, but they can start earlier or later in life. The early signs of HD may include subtle changes in mood, cognition, and coordination. As the disease progresses, individuals with HD experience uncontrolled movements (chorea), emotional disturbances, cognitive decline, and difficulties with communication and swallowing. Eventually, they become dependent on others for their daily needs and lose their ability to walk, talk, and care for themselves.
There is currently no cure for HD, but medications and therapies can help manage the symptoms of the disease and improve quality of life. Genetic testing is available to confirm the diagnosis and provide information about the risk of passing the disease on to future generations.
Rheumatic fever is a systemic inflammatory disease that may occur following an untreated Group A streptococcal infection, such as strep throat. It primarily affects children between the ages of 5 and 15, but it can occur at any age. The condition is characterized by inflammation in various parts of the body, including the heart (carditis), joints (arthritis), skin (erythema marginatum, subcutaneous nodules), and brain (Sydenham's chorea).
The onset of rheumatic fever usually occurs 2-4 weeks after a streptococcal infection. The exact cause of the immune system's overreaction leading to rheumatic fever is not fully understood, but it involves molecular mimicry between streptococcal antigens and host tissues.
The Jones Criteria are used to diagnose rheumatic fever, which include:
1. Evidence of a preceding streptococcal infection (e.g., positive throat culture or rapid strep test, elevated or rising anti-streptolysin O titer)
2. Carditis (heart inflammation), including new murmurs or changes in existing murmurs, electrocardiogram abnormalities, or evidence of heart failure
3. Polyarthritis (inflammation of multiple joints) – typically large joints like the knees and ankles, migratory, and may be associated with warmth, swelling, and pain
4. Erythema marginatum (a skin rash characterized by pink or red, irregularly shaped macules or rings that blanch in the center and spread outward)
5. Subcutaneous nodules (firm, round, mobile lumps under the skin, usually over bony prominences)
6. Sydenham's chorea (involuntary, rapid, irregular movements, often affecting the face, hands, and feet)
Treatment of rheumatic fever typically involves antibiotics to eliminate any residual streptococcal infection, anti-inflammatory medications like corticosteroids or nonsteroidal anti-inflammatory drugs (NSAIDs) to manage symptoms and prevent long-term heart complications, and secondary prophylaxis with regular antibiotic administration to prevent recurrent streptococcal infections.
Chorea gravidarum is a rare condition characterized by involuntary, irregular, and abrupt movements known as chorea, which typically affect the face, arms, and legs. This condition usually occurs during pregnancy or after childbirth. The exact cause of chorea gravidarum is not well understood, but it is believed to be related to hormonal changes and fluctuations during pregnancy.
Chorea gravidarum can range from mild to severe in its presentation. In some cases, the movements may be barely noticeable, while in others, they can interfere with daily activities and quality of life. The condition may also cause emotional lability, which refers to rapid and unpredictable shifts in mood or behavior.
Chorea gravidarum is typically treated with medications that help to reduce the severity of the movements and improve symptoms. These medications may include anticholinergics, dopamine-blocking agents, or benzodiazepines. In some cases, supportive therapies such as physical therapy or occupational therapy may also be recommended to help manage symptoms and improve function.
It is important to note that chorea gravidarum can pose risks to both the mother and the developing fetus. Women who experience this condition should be closely monitored by their healthcare provider throughout pregnancy and postpartum period to ensure the best possible outcomes for both.
Anti-dyskinetic agents are a class of medications that are used to treat or manage dyskinesias, which are involuntary movements or abnormal muscle contractions. These medications work by blocking or reducing the activity of dopamine, a neurotransmitter in the brain that is involved in movement control.
Dyskinetic symptoms can occur as a side effect of long-term use of levodopa therapy in patients with Parkinson's disease. Anti-dyskinetic agents such as amantadine, anticholinergics, and dopamine agonists may be used to manage these symptoms.
Amantadine works by increasing the release of dopamine and blocking its reuptake, which can help reduce dyskinesias. Anticholinergic medications such as trihexyphenidyl and benztropine work by blocking the action of acetylcholine, another neurotransmitter that can contribute to dyskinesias. Dopamine agonists such as pramipexole and ropinirole mimic the effects of dopamine in the brain and can help reduce dyskinesias by reducing the dose of levodopa required for symptom control.
It is important to note that anti-dyskinetic agents may have side effects, and their use should be carefully monitored by a healthcare provider.
Autoimmune diseases of the nervous system are a group of conditions that occur when the body's immune system mistakenly attacks healthy tissue in the brain, spinal cord, or nerves. These diseases can cause inflammation, damage to nerve cells, and interference with the transmission of nerve impulses, leading to various neurological symptoms.
Examples of autoimmune diseases that affect the nervous system include:
1. Multiple sclerosis (MS): A chronic disease characterized by damage to the protective covering of nerve fibers in the brain and spinal cord, causing a variety of neurological symptoms such as muscle weakness, vision problems, and difficulty with coordination and balance.
2. Myasthenia gravis: A condition that causes muscle weakness and fatigue, particularly affecting the eyes, face, and neck muscles. It occurs when the immune system attacks the receptors that transmit signals between nerves and muscles.
3. Guillain-Barré syndrome: A rare disorder in which the body's immune system attacks the nerves, causing muscle weakness, tingling, and numbness that can spread throughout the body. In severe cases, it can lead to paralysis and respiratory failure.
4. Neuromyelitis optica (NMO): A rare autoimmune disease that affects the optic nerve and spinal cord, causing vision loss, muscle weakness, and other neurological symptoms.
5. Autoimmune encephalitis: A group of conditions characterized by inflammation of the brain, caused by an overactive immune response. Symptoms can include seizures, memory loss, confusion, and behavioral changes.
6. Chronic inflammatory demyelinating polyneuropathy (CIDP): A rare disorder that causes progressive weakness and numbness in the legs and arms due to damage to the nerves' protective covering.
Treatment for autoimmune diseases of the nervous system typically involves medications to suppress the immune system and reduce inflammation, as well as physical therapy and other supportive measures to manage symptoms and maintain function.
Hyperglycemic Hyperosmolar Nonketotic Coma (HHNC) is a serious complication of diabetes, specifically type 2, that occurs when blood glucose levels rise to extremely high levels, typically above 600 mg/dL. This condition is often accompanied by severe dehydration due to excessive urination and an inability to consume adequate fluids.
The term "hyperosmolar" refers to the high concentration of glucose in the blood, which increases the osmolality (or osmotic pressure) of the blood. This can lead to water moving out of cells and into the bloodstream to try to balance out the concentration, causing severe dehydration.
The term "nonketotic" means that there is no significant production of ketone bodies, which are produced when the body breaks down fat for energy in the absence of sufficient insulin. This differentiates HHNC from diabetic ketoacidosis (DKA), another serious complication of diabetes.
The "coma" part of the term refers to the fact that HHNC can cause altered mental status, ranging from confusion and disorientation to coma, due to the effects of dehydration and high blood glucose levels on the brain.
HHNC is a medical emergency that requires immediate treatment in a hospital setting. Treatment typically involves administering fluids to rehydrate the body, insulin to lower blood glucose levels, and addressing any other underlying conditions or complications. If left untreated, HHNC can be life-threatening.
Huntington's17
- citation needed] Huntington's disease is a neurodegenerative disease and most common inherited cause of chorea. (wikipedia.org)
- The condition was formerly called Huntington's chorea but was renamed because of the important non-choreic features including cognitive decline and behavioural change. (wikipedia.org)
- The FDA announced Aug. 15 that it has approved Prestwick Pharmaceuticals Inc.'s new drug Xenazine (generic name tetrabenazine) for the treatment of chorea in people with Huntington's disease, heralding the first treatment to receive U.S. approval for any of the disease's symptoms ( Medical News Today , Aug. 18). (aapc.com)
- Chorea itself isn't life-threatening, but it could be a sign of a neurological disease such as Huntington's disease . (clevelandclinic.org)
- About 30,000 people in the United States have Huntington's disease (a genetic condition that causes chorea). (clevelandclinic.org)
- Chorea is the most common symptom of Huntington's disease. (clevelandclinic.org)
- There are two types of chorea, Huntington's chorea and Sydenham's chorea. (luriechildrens.org)
- Huntington's chorea appears in adults and is hereditary, progressive, and ultimately fatal. (luriechildrens.org)
- Chorea is a primary feature of Huntington's disease, a progressive, hereditary movement disorder that appears in adults, but it may also occur in a variety of other conditions. (brainfacts.org)
- Treatment for Huntington's disease is supportive, while treatment for Syndenham's chorea usually involves antibiotic drugs to treat the infection, followed by drug therapy to prevent recurrence. (brainfacts.org)
- Huntington's chorea, or Huntington's disease, named after Ohio physician George Huntington in 1872, is an inherited disorder characterized by abnormal body movements called chorea, and loss of memory. (writework.com)
- Understanding and treating Huntington's chorea can help save countless lives and individuals. (writework.com)
- Huntington's Chorea" WriteWork.com. (writework.com)
- Huntington's Disease Huntington's disease, or Huntngton's chorea, is a genetic disease that causes selective neural cell death, which results in chorea, or irregular, jerking movements of the limbs caused by involuntary muscle contractions, and dementia. (writework.com)
- We suggest that although benign hereditary chorea and Huntington's disease may have some clinical similarities they are probably at two different loci. (bmj.com)
- involuntary movements (chorea) of Huntington's disease. (ingrezza.com)
- 1. Neurocrine Biosciences Announces FDA Approval of INGREZZA® (valbenazine) Capsules for the Treatment of Chorea Associated With Huntington's Disease. (neurologylive.com)
Involuntary16
- Chorea (or choreia, occasionally) is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias. (wikipedia.org)
- Chorea refers to the involuntary jerking movements made by people with this disorder. (medlineplus.gov)
- In addition to chorea, another common feature of chorea-acanthocytosis is involuntary tensing of various muscles (dystonia), such as those in the limbs, face, mouth, tongue, and throat. (medlineplus.gov)
- Chorea is an involuntary abnormal movement, characterized by abrupt, brief, nonrhythmic, nonrepetitive movement of any limb, often associated with nonpatterned facial grimaces. (medscape.com)
- Chorea is defined as random-appearing, continuous (while awake), involuntary movements which can affect the entire body. (rarediseases.org)
- Initially, doctors may misattribute the restless movements and involuntary facial expressions of Sydenham chorea to a child being extremely fidgety, hyperactive, clumsy and/or purposely uncooperative. (rarediseases.org)
- Chorea is a movement disorder that causes involuntary, irregular, unpredictable muscle movements. (clevelandclinic.org)
- Chorea is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias, which are caused by overactivity of the neurotransmitter dopamine in the areas of the brain that control movement. (brainfacts.org)
- Intensive propulsion, involuntary muscle movements (limbs and face), muscle weakness of left upper and lower limbs, and "walk dance" in 38-year-old man with tick-borne encephalitis and chorea, Poland. (cdc.gov)
- HD is the most common genetic cause of abnormal involuntary writhing movements called chorea. (tevausa.com)
- Absent subthalamic nucleus inhibition enhances motor activity through the motor thalamus, resulting in abnormal involuntary movements such as dystonia, chorea, and tics. (medscape.com)
- After excluding the other possible causes of chorea, phenytoin was withdrawn which resulted in a dramatic subsidence of chorea .On rechallenge with phenytoin patient again developed chorieform movements which again subsided after drug withdrawl, thus implicating phenytoin as the possible etiological agent for chorea Conclusion:Phenytoin rarely induces involuntary movements as adverse effect.During phenytoin therapy,if a patient develops involuntary movements,phenytoin toxicity should be suspected. (authorea.com)
- Signs and symptoms in infants may include low muscle tone, involuntary movements (chorea), lung infections, and respiratory distress. (nih.gov)
- Chorea (Greek for 'dance') refers to widespread arrhythmic involuntary movements of a forcible, jerky and restless fashion. (nih.gov)
- Chorea results in involuntary movements like jerking and writhing, typically beginning in the person's fingers, feet, face, or torso. (bestmindbodyhealth.com)
- Chorea is a neurological disorder characterized by abrupt, purposeless, non-rhythmic, involuntary movements that is often associated with muscle weakness and emotional lability. (cdc.gov)
Rheumatic14
- Twenty percent (20%) of children and adolescents with rheumatic fever develop Sydenham's chorea as a complication. (wikipedia.org)
- Rheumatic fever is no longer a major cause of chorea gravidarum (CG) and the pathophysiology of CG in current times is unclear. (medscape.com)
- Sydenham chorea usually develops within weeks to months following group A beta-hemolytic streptococcal infection and may occur as an isolated finding or as a major complication of acute rheumatic fever. (rarediseases.org)
- Because Sydenham chorea is a complication of rheumatic fever, some individuals will have additional symptoms of joint arthritis or arthralgia, inflammation of the heart valves causing permanent damage to the valves, and ongoing fever. (rarediseases.org)
- In the United States, about 4,000 kids a year develop Sydenham chorea after having rheumatic fever . (clevelandclinic.org)
- Kids and adolescents can develop Sydenham chorea after rheumatic fever, which is a complication of untreated strep throat. (clevelandclinic.org)
- Around one to eight months after having rheumatic fever, children can develop Sydenham chorea (also called St. Vitus dance). (clevelandclinic.org)
- Sydenham's chorea appears in about 20 percent of children and young adults as a complication of rheumatic fever, a complication of untreated strep throat or scarlet fever. (luriechildrens.org)
- Syndenham's chorea occurs in a small percentage (20 percent) of children and adolescents as a complication of rheumatic fever. (brainfacts.org)
- Chorea gravidarum occurs during pregnancy, often in patients who have had rheumatic fever. (merckmanuals.com)
- Sydenham chorea, or rheumatic chorea, is a movement disorder that is more prevalent among young people, with a mean age at symptom onset between 8 and 9 years. (openneurologyjournal.com)
- Sydenham chorea is associated with rheumatic fever and is considered the most common cause of acute chorea in children. (openneurologyjournal.com)
- Sydenham chorea (SC) is one of the main diagnostic criteria for rheumatic fever. (openneurologyjournal.com)
- Chorea often appears after the other manifestations of acute rheumatic fever. (cdc.gov)
Athetosis9
- These 'dance-like' movements of chorea often occur with athetosis, which adds twisting and writhing movements. (wikipedia.org)
- Chorea often occurs with athetosis, which adds twisting and writhing movements. (brainfacts.org)
- Athetosis (slow chorea) is nonrhythmic, slow, writhing, sinuous movements predominantly in distal muscles, often alternating with postures of the proximal limbs. (merckmanuals.com)
- many experts believe that when they occur together (as choreoathetosis), athetosis is a dystonia superimposed on chorea. (merckmanuals.com)
- Chorea and athetosis result from impaired inhibition of thalamocortical neurons by the basal ganglia. (merckmanuals.com)
- Mutations in the myofibrillogenesis regulator gene (MR-1) causes paroxysmal non -kinesigenic dyskinesia (PNKD) characterized by episodic (paroxysmal) attacks of any combination of chorea, dystonia and athetosis (MR-1/PNKD) [1]. (medgenome.com)
- Any discussion of chorea must also address the related terms athetosis, choreoathetosis, and ballism (also known as ballismus). (medscape.com)
- Choreoathetosis is essentially an intermediate form (ie, a bit more rapid than the usual athetosis, slower than the usual chorea, or a mingling of chorea and athetosis within the same patient at different times or in different limbs). (medscape.com)
- Given that the only difference between chorea, choreoathetosis, and athetosis is the speed of movement, some neurologists argue that the term athetosis is unnecessary and even confusing. (medscape.com)
Symptoms16
- Psychological symptoms may precede or accompany this acquired chorea and may be relapsing and remitting. (wikipedia.org)
- Chorea gravidarum refers to choreic symptoms that occur during pregnancy. (wikipedia.org)
- The signs and symptoms of chorea-acanthocytosis usually begin in early to mid-adulthood. (medlineplus.gov)
- Additional symptoms of Sydenham chorea may include slurring of speech and difficulty maintaining steady hand grip. (rarediseases.org)
- The severity of chorea and the presence of non-chorea symptoms of Sydenham chorea may vary greatly from one person to another. (rarediseases.org)
- Symptoms of Sydenham chorea may appear anywhere from 1 week to 6 months following streptococcal infection. (rarediseases.org)
- The abnormal movements in Sydenham chorea range from subtle symptoms, affecting coordination and tasks such as writing, to severe symptoms, disrupting walking, talking, and performing basic tasks such as dressing, eating, or simply holding objects. (rarediseases.org)
- Sydenham chorea symptoms usually resolve within three weeks to six months. (rarediseases.org)
- Occasionally, the symptoms of Sydenham chorea have recurred later during adult life, particularly in young women during the first trimester of pregnancy (so-called chorea gravidarum, which may represent a recurrence of Sydenham chorea in some cases). (rarediseases.org)
- Metabolic and endocrine-related choreas are treated according to the cause(s) of symptoms. (brainfacts.org)
- Chorea is a rare but potentially reversible neurological manifestation of coeliac disease, usually presenting insidiously and often presumed to be associated with typical gastrointestinal symptoms. (bmj.com)
- We report a patient with rapidly progressive generalised chorea, but without preceding gastrointestinal symptoms, who was subsequently diagnosed with coeliac disease. (bmj.com)
- We report here the case of a 14-year-old girl with symptoms of acute chorea that emerged 15 days after treatment resolution of COVID-19 (SARS-CoV-2 or severe acute respiratory syndrome coronavirus 2). (openneurologyjournal.com)
- Therefore, we report here the case of a 14-year-old girl with symptoms of acute chorea that emerged 15 days after treatment resolution of COVID-19 (SARS-CoV-2). (openneurologyjournal.com)
- One of the most common, and likely most noticeable symptoms, is chorea. (bestmindbodyhealth.com)
- We believe that this is a first report of adult-onset Sydenham chorea accompanied with psychiatric symptoms. (elsevierpure.com)
Huntington6
- Historically, choreas like Huntington disease and Sydenham's chorea were called Saint Vitus' dance, related to a series of social phenomena of the same name. (wikipedia.org)
- Teva Pharmaceutical Industries Ltd. (NYSE and TASE:TEVA) today announced that the U.S. Food and Drug Administration (FDA) has accepted the resubmission of the New Drug Application (NDA) for SD-809 (deutetrabenazine) for the treatment of chorea associated with Huntington disease (HD). (tevausa.com)
- SD-809 is being developed for the treatment of chorea associated with Huntington disease, a neurodegenerative movement disorder that impacts cognition, behavior, and movements. (tevausa.com)
- In a busy movement disorder center, levodopa-induced chorea is the most common movement disorder, followed by Huntington disease (HD). (medscape.com)
- This episode, 'Addition of Valbenazine to Treat Huntington Disease Chorea,' features an exclusive interview with Erin Furr-Stimming, MD, FAAN, FANA, a professor of neurology at McGovern Medical School of UTHealth Houston, and principal investigator of the phase 3 KINECT-HD studies. (neurologylive.com)
- Neurocrine Biosciences) to include the treatment of chorea associated with Huntington disease (HD), offering her immediate reaction to the news. (neurologylive.com)
Gravidarum3
- Chorea gravidarum (CG) is the term given to chorea occurring during pregnancy. (medscape.com)
- Movement disorders rarely occur during reproductive years, therefore, clinicians are not very familiar with chorea gravidarum (CG). (medscape.com)
- Though rare, a type of chorea called chorea gravidarum can occur during pregnancy. (clevelandclinic.org)
Hereditary3
- Absence of close linkage between benign hereditary chorea and the locus D4S10 (probe G8). (bmj.com)
- A genetic linkage study between benign hereditary chorea and the locus D4S10 using the DNA probe G8 has shown two recombinations in five small families. (bmj.com)
- Benign hereditary chorea (BHC) is a rare movement disorder that beginsin infancy or childhood. (nih.gov)
Sydenham's Chorea3
- Sydenham's chorea occurs as a complication of streptococcal infection. (wikipedia.org)
- A brief description of the disease Sydenham's Chorea also called St. Vitus Dance. (writework.com)
- Chorea, also called Sydenham's chorea or St. Vitus dance, is the major central nervous system manifestation. (cdc.gov)
Vitus2
- Chorea, once called St. Vitus' Dance, is characterized by brief, irregular contractions that appear to flow from one muscle to the next. (aapc.com)
- disease gathered in the chapels of St. Vitus in belief that this Catholic patron saint of dancing had miraculous curative powers.1,2 This type of chorea is a movement disorder as other types of chorea are. (writework.com)
Type of chorea2
- Treatment depends on the type of chorea and the associated disease. (wikipedia.org)
- The prognosis for individuals with chorea varies depending on the type of chorea and the associated disease. (brainfacts.org)
Case of chorea3
- In 2004, Miranda et al reported of a case of chorea associated with the use of the oral contraceptives, in which anti-basal ganglia antibodies were detected, suggesting a possible immunological basis to the pathogenesis of this disorder. (medscape.com)
- We describe a case of chorea in the course of TBE in a man in Poland. (cdc.gov)
- ABSTRACT Introduction:We describe a case of chorea induced by phenytoin in an young indian male. (authorea.com)
Neurology1
- The ad hoc Committee on Classification of the World Federation of Neurology has defined chorea as "a state of excessive, spontaneous movements, irregularly timed, non-repetitive, randomly distributed and abrupt in character. (medscape.com)
Cause chorea5
- The broader spectrum of paediatric autoimmune neuropsychiatric disorders associated with streptococcal infection can cause chorea and are collectively referred to as PANDAS. (wikipedia.org)
- Mutations in the VPS13A gene cause chorea-acanthocytosis. (medlineplus.gov)
- Depending on the underlying cause, chorea may be temporary or be ongoing and get worse over time. (clevelandclinic.org)
- Autoimmune diseases (such as lupus) and hormonal disorders like hyperthyroidism and metabolic disorders such as hypoglycemia can cause chorea. (clevelandclinic.org)
- There is a long list of other disorders that can cause chorea. (clevelandclinic.org)
Autoimmune4
- Dozens of genetic conditions, autoimmune and infectious diseases, endocrine disorders, medications and even pregnancy can have chorea as a symptom. (clevelandclinic.org)
- Dozens of genetic conditions, autoimmune diseases, metabolic disorders, infections and medications can affect basal ganglia and lead to the onset of chorea. (clevelandclinic.org)
- 60 should not be assumed to be senile chorea but should be thoroughly evaluated to identify the cause (eg, toxic, metabolic, autoimmune, paraneoplastic, drug-induced). (merckmanuals.com)
- An Autoimmune Basis for Diabetes-Related Chorea? (elsevierpure.com)
Acute3
- Sydenham chorea is a major sign of acute RF. (medlineplus.gov)
- A tumor or an infarct in the striatum (caudate or putamen) can cause acute unilateral chorea (hemichorea) on the opposite side of the body. (merckmanuals.com)
- Case presentation: A twenty one year old indian male patient who is a known case of generalized epilepsy for past 2 years on sodium valproate presented with acute onset chorea, four days after starting phenytoin sodium with normal serum phenytoin levels. (authorea.com)
Drug-induced1
- Adjusting medication dosages can treat drug-induced chorea. (brainfacts.org)
Hyperkinetic movement disorder2
- Thus, chorea is said to be a hyperkinetic movement disorder. (wikipedia.org)
- Chorea is a hyperkinetic movement disorder resulting from dysfunction of striatal medium spiny neurons (MSNs), which form the main output projections from the basal ganglia. (northwestern.edu)
Genetic2
- Here, we used whole-exome sequencing to unravel the underlying genetic cause in three unrelated individuals with a very similar and unique clinical presentation of childhood-onset chorea and characteristic brain MRI showing symmetrical bilateral striatal lesions. (northwestern.edu)
- Chorea can be genetic or acquired, and often leads to a challenging diagnostic conundrum. (bmj.com)
Sydenham Chorea15
- Sydenham chorea is a movement disorder that occurs after infection with specific bacteria called group A streptococcus. (medlineplus.gov)
- Sydenham chorea is caused by an infection with bacteria called group A streptococcus. (medlineplus.gov)
- Sydenham chorea may be the only sign of RF in some people. (medlineplus.gov)
- Sydenham chorea occurs most often in girls before puberty, but may be seen in boys. (medlineplus.gov)
- Sydenham chorea usually clears up in a few months. (medlineplus.gov)
- In rare cases, an unusual form of Sydenham chorea may begin later in life. (medlineplus.gov)
- Sydenham chorea is a rare neurological disorder characterized by sudden onset chorea, usually in childhood. (rarediseases.org)
- Sydenham chorea most often affects children over the age of 5 years and adolescents. (rarediseases.org)
- The abnormal movements (chorea) that characterize Sydenham chorea usually emerge over hours, peaking within a few hours or days. (rarediseases.org)
- Most of the time, kids with Sydenham chorea get better without treatment in less than two years. (clevelandclinic.org)
- Sydenham chorea and chorea due to infarcts of the caudate nucleus often lessen over time without treatment. (merckmanuals.com)
- We believe that the present case is worth reporting since the occurrence of Sydenham chorea as a post-COVID-19 sequela has not been described in Brazil. (openneurologyjournal.com)
- Sydenham chorea is usually self-limiting and lasts on average 2 to 3 months, although cases with a duration of more than 2 years have been reported. (openneurologyjournal.com)
- We report a 56-year-old man with adult-onset Sydenham chorea. (elsevierpure.com)
- The present case was diagnosed as Sydenham chorea, because of the presence of arthritis, chorea, fever, increased erythrocyte sedimentation rate and elevated CRP. (elsevierpure.com)
Manifestation1
- The patient reported here is the first well documented case of anti-phospholipid antibody syndrome presenting chorea as an initial manifestation in the Japanese literature. (elsevierpure.com)
Metabolic3
- Chorea can also be induced by drugs (levodopa, anti-convulsants, and anti-psychotics) metabolic and endocrine disorders, and vascular incidents. (brainfacts.org)
- Chorea due to hyperthyroidism or another metabolic cause (eg, hyperglycemia) usually lessens over time when thyroid function or blood glucose returns to normal. (merckmanuals.com)
- If chorea does not subside after several weeks of metabolic control, clinicians should check for another cause such as stroke. (merckmanuals.com)
Slow chorea1
- They argue a simpler nomenclature would delineate fast, intermediate, and slow chorea. (medscape.com)
Occurs2
- Chorea is a movement disorder that occurs in many different diseases and conditions. (clevelandclinic.org)
- Some people develop chorea if they have a stroke or a tumor that occurs in or near basal ganglia. (clevelandclinic.org)
Haloperidol2
- Regrettably, there is currently no cure for this condition, but there are treatment and medication options for symptom management, such as prescription medications like haloperidol, tetrabenazine, amantadine, and Austedo which specifically helps with chorea. (bestmindbodyhealth.com)
- The clinical diagnosis of chorea was made and haloperidol was administered with partial symptomatic improvements. (elsevierpure.com)
Movement disorders2
- The NINDS supports research on movement disorders such as chorea. (brainfacts.org)
- Pharmacological modulation of this pathway could offer promising etiologically targeted treatments for chorea and other hyperkinetic movement disorders. (northwestern.edu)
Syndrome1
- A 20 year old female patient with anti-phospholipid antibody syndrome who presented chorea as an initial symptom is described. (elsevierpure.com)
Severe7
- this form of severe chorea is referred to as ballism, or ballismus. (wikipedia.org)
- In some extremely rare cases (less than 2 percent), severe muscle weakness, irritability, or confusion may be profound and affected children may become bedridden, a condition sometimes referred to as paralytic chorea. (rarediseases.org)
- Hemiballismus may be considered a severe form of chorea. (merckmanuals.com)
- If treatment before delivery is necessary because chorea is severe, barbiturates are indicated because they have fewer fetal risks than other drugs used to manage chorea. (merckmanuals.com)
- Chorea is a severe, distressing, movement disorder characterised by excessive, purposeless movements of the limbs, head and orofacial muscles in a generalised and irregularly-timed fashion. (rcpe.ac.uk)
- Ballism or ballismus is considered a very severe form of chorea in which the movements have a violent, flinging quality. (medscape.com)
- Currently, ballism should be viewed as a severe form of chorea. (medscape.com)
Basal2
- Chorea is a neurological symptom that originates in an area of the brain called basal ganglia, which are collections of nerve cells deep inside the brain that control movement. (clevelandclinic.org)
- The identification of PDE10A mutations as a cause of chorea further motivates the study of cAMP signaling in MSNs and highlights the crucial role of striatal cAMP signaling in the regulation of basal ganglia circuitry. (northwestern.edu)
Behavior1
- On admission, neurological examination revealed dementia, emotional incontinence, abnormal behavior and chorea in four limbs. (elsevierpure.com)
Distal muscles1
- Chorea involves both proximal and distal muscles. (medscape.com)
Symptom1
- Chorea is usually a symptom of another disorder. (clevelandclinic.org)
Tetrabenazine1
- eg, risperidone , olanzapine ), and dopamine -depleting drugs (eg, deutetrabenazine , reserpine [no longer available in US], tetrabenazine ) can be used to treat chorea. (merckmanuals.com)
Pathophysiology1
- therefore, the pathophysiology of HD as it applies to chorea is the focus of the discussion that follows. (medscape.com)
Fidgety1
- The disorder can make you look like you're dancing (the word chorea comes from the Greek word for "dance") or look restless or fidgety. (clevelandclinic.org)
Degenerative1
- is the most common degenerative disorder causing chorea. (merckmanuals.com)
Hemiballismus1
- The term hemichorea refers to chorea of one side of the body, such as chorea of one arm but not both (analogous to hemiballismus). (wikipedia.org)
Pregnancy3
- This is not an etiologically or pathologically distinct entity but rather a generic term for chorea of any cause starting during pregnancy. (medscape.com)
- In 1950, Beresford and Graham postulated that, "It may be that pregnancy lowers the resistance of a patient who is inherently susceptible to chorea. (medscape.com)
- If pregnancy is the cause of the chorea, it may appear during the first three months of pregnancy and stop shortly after the birth of the baby. (clevelandclinic.org)
Disease1
- As the disease progresses, so does the degeneration of this area of the brain, thus explaining why chorea becomes more and more intense over time. (bestmindbodyhealth.com)
Bilateral1
- Hypermetabolism in bilateral caudate nuclei, especially on the right, was found on FDG-PET study, which was compatible with his left side-dominant chorea and might reflect inflammation as a nature. (elsevierpure.com)