Chorea gravidarum

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Chorea gravidarum
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Chorea gravidarum is a rare [1] type of chorea which presents with involuntary abnormal movement, characterized by abrupt, brief, nonrhythmic, nonrepetitive movement of any limb, often associated with nonpatterned facial grimaces. It is a complication of pregnancy which can be associated with eclampsia and its effects upon the basal ganglia. It is not a causal or pathologically distinct entity but a generic term for chorea of any cause starting during pregnancy. It is associated with history of Sydenham's chorea. It mostly occurs in young patients; the average age is 22 years. [2] [3]

Contents

Recently there has been a decline in incidence which is probably the result of a decline in rheumatic fever (RF), which was a major cause of chorea gravidarum before the use of antibiotics for streptococcal pharyngitis.[ citation needed ]

Pathophysiology

Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven. History of either rheumatic fever or chorea is suspected: [2] [3] the suggestion is that estrogens and progesterone may sensitize dopamine receptors (presumably at a striatal level) and induce chorea in individuals who are vulnerable to this complication by virtue of preexisting pathology in the basal ganglia. The relation to rheumatic fever was strengthened by many studies that showed that women with normal pregnancies before rheumatic fever developed chorea in subsequent pregnancies. [4] [5] At least 35% of patients have a definite history of acute rheumatic fever and Sydenham chorea; 4% of those with chorea gravidarum had acute rheumatic fever. [2] [3]

It has been suggested that use of oral contraceptives is an infrequent cause of chorea. A patient developed this chorea with no definite evidence of previous Sydenham's chorea or recent streptococcal infections, but had anti-basal ganglia antibodies, suggesting immunological basis for the pathophysiology of this chorea. [6] [7]

Diagnosis

Differential diagnoses

Chorea can also be a manifestation of drug toxicity (for example, anticonvulsants, antiparkinson agents, neuroleptics, steroids, and estrogen), or a result of an infectious disease such as meningovascular syphilis, Lyme disease, viral encephalitis, and many others.[ citation needed ]

Treatment

Drug treatment is indicated for patients with severe disabling chorea. It is treated with haloperidol, [9] [10] [11] chlorpromazine alone or in combination with diazepam, and also pimozide, which is another neuroleptic drug which may have fewer adverse effects than haloperidol. [12] Valproic acid, chloral hydrate, risperidone, or phenobarbital can also be used. [13]

See also

Related Research Articles

Group A streptococcal infection Medical condition

A group A streptococcal infection is an infection with group A streptococcus (GAS). Streptococcus pyogenes comprises the vast majority of the Lancefield group A streptococci, and is often used as a synonym for GAS. However, S. dysgalactiae can also be group A. S. pyogenes is a beta-hemolytic species of Gram positive bacteria that is responsible for a wide range of both invasive and noninvasive infections.

Scarlet fever Infectious disease

Scarlet fever is a disease resulting from a group A streptococcus infection, also known as Streptococcus pyogenes. The signs and symptoms include a sore throat, fever, headaches, swollen lymph nodes, and a characteristic rash. The rash is red and feels like sandpaper and the tongue may be red and bumpy. It most commonly affects children between five and 15 years of age.

Streptococcal pharyngitis Medical condition

Streptococcal pharyngitis, also known as strep throat, is an infection of the back of the throat including the tonsils caused by group A streptococcus (GAS). Common symptoms include fever, sore throat, red tonsils, and enlarged lymph nodes in the neck. A headache, and nausea or vomiting may also occur. Some develop a sandpaper-like rash which is known as scarlet fever. Symptoms typically begin one to three days after exposure and last seven to ten days.

Neuroleptic malignant syndrome Medical condition

Neuroleptic malignant syndrome (NMS) is a rare but life-threatening reaction that can occur in response to neuroleptic or antipsychotic medication. Symptoms include high fever, confusion, rigid muscles, variable blood pressure, sweating, and fast heart rate. Complications may include rhabdomyolysis, high blood potassium, kidney failure, or seizures.

Haloperidol Typical antipsychotic medication

Haloperidol, sold under the brand name Haldol among others, is a typical antipsychotic medication. Haloperidol is used in the treatment of schizophrenia, tics in Tourette syndrome, mania in bipolar disorder, delirium, agitation, acute psychosis, and hallucinations in alcohol withdrawal. It may be used by mouth or injection into a muscle or a vein. Haloperidol typically works within 30 to 60 minutes. A long-acting formulation may be used as an injection every four weeks in people with schizophrenia or related illnesses, who either forget or refuse to take the medication by mouth.

Pimozide

Pimozide is an antipsychotic drug of the diphenylbutylpiperidine class. It was discovered at Janssen Pharmaceutica in 1963. It has a high potency compared to chlorpromazine. On a weight basis it is even more potent than haloperidol. It also has special neurologic indications for Tourette syndrome and resistant tics. The side effects include akathisia, tardive dyskinesia, and, more rarely, neuroleptic malignant syndrome and prolongation of the QT interval.

Encephalitis lethargica Form of encephalitis (sleeping sickness)

Encephalitis lethargica is an atypical form of encephalitis. Also known as "sleeping sickness" or "sleepy sickness", it was first described in 1917 by the neurologist Constantin von Economo and the pathologist Jean-René Cruchet.

Rheumatic fever Post-streptococcal inflammatory disease

Rheumatic fever (RF) is an inflammatory disease that can involve the heart, joints, skin, and brain. The disease typically develops two to four weeks after a streptococcal throat infection. Signs and symptoms include fever, multiple painful joints, involuntary muscle movements, and occasionally a characteristic non-itchy rash known as erythema marginatum. The heart is involved in about half of the cases. Damage to the heart valves, known as rheumatic heart disease (RHD), usually occurs after repeated attacks but can sometimes occur after one. The damaged valves may result in heart failure, atrial fibrillation and infection of the valves.

Chorea is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias. The term chorea is derived from the Ancient Greek: χορεία, as the quick movements of the feet or hands are comparable to dancing.

Sydenham's chorea, also known as chorea minor and historically and occasionally referred to as St Vitus' dance, is a disorder characterized by rapid, uncoordinated jerking movements primarily affecting the face, hands and feet. Sydenham's chorea is an autoimmune disease that results from childhood infection with Group A beta-haemolytic Streptococcus. It is reported to occur in 20–30% of people with acute rheumatic fever and is one of the major criteria for it, although it sometimes occurs in isolation. The disease occurs typically a few weeks, but up to 6 months, after the acute infection, which may have been a simple sore throat (pharyngitis).

Hyperkinesia Excessive movements due to basal ganglia dysfunction

Hyperkinesia refers to an increase in muscular activity that can result in excessive abnormal movements, excessive normal movements or a combination of both. Hyperkinesia is a state of excessive restlessness which is featured in a large variety of disorders that affect the ability to control motor movement, such as Huntington's disease. It is the opposite of hypokinesia, which refers to decreased bodily movement, as commonly manifested in Parkinson's disease.

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Hemiballismus or hemiballism is a basal ganglia syndrome resulting from damage to the subthalamic nucleus in the basal ganglia. Hemiballismus is a rare hyperkinetic movement disorder, that is characterized by violent involuntary limb movements, on one side of the body, and can cause significant disability. Ballismus affects both sides of the body and is much rarer. Symptoms can decrease during sleep.

PANDAS hypothesis in pediatric medicine

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is a hypothesis that there exists a subset of children with rapid onset of obsessive-compulsive disorder (OCD) or tic disorders and these symptoms are caused by group A beta-hemolytic streptococcal (GABHS) infections. The proposed link between infection and these disorders is that an initial autoimmune reaction to a GABHS infection produces antibodies that interfere with basal ganglia function, causing symptom exacerbations. It has been proposed that this autoimmune response can result in a broad range of neuropsychiatric symptoms. The PANDAS hypothesis was based on observations in clinical case studies at the US National Institutes of Health and in subsequent clinical trials where children appeared to have dramatic and sudden OCD exacerbations and tic disorders following infections.

Neuroacanthocytosis is a label applied to several genetic neurological conditions in which the blood contains misshapen, spiculated red blood cells called acanthocytes.

Tourette syndrome is an inherited neurodevelopmental disorder that begins in childhood or adolescence, characterized by the presence of motor and phonic tics. The management of Tourette syndrome has the goal of managing symptoms to achieve optimum functioning, rather than eliminating symptoms; not all persons with Tourette's require treatment, and there is no cure or universally effective medication. Explanation and reassurance alone are often sufficient treatment; education is an important part of any treatment plan.

Arthur K. Shapiro, M.D., was a psychiatrist and expert on Tourette syndrome. His "contributions to the understanding of Tourette syndrome completely changed the prevailing view of this disorder"; he has been described as "the father of modern tic disorder research" and is "revered by his colleagues as the first dean of modern Tourette syndrome researchers".

Tourettism refers to the presence of Tourette-like symptoms in the absence of Tourette syndrome, as the result of other diseases or conditions, known as "secondary causes".

A paraneoplastic syndrome is a syndrome that is the consequence of cancer in the body, specifically due to the production of chemical signaling molecules by tumor cells or by an immune response against the tumor. Unlike a mass effect, it is not due to the local presence of cancer cells.

Basal ganglia disease Group of physical problems resulting from basal ganglia dysfunction

Basal ganglia disease is a group of physical problems that occur when the group of nuclei in the brain known as the basal ganglia fail to properly suppress unwanted movements or to properly prime upper motor neuron circuits to initiate motor function. Research indicates that increased output of the basal ganglia inhibits thalamocortical projection neurons. Proper activation or deactivation of these neurons is an integral component for proper movement. If something causes too much basal ganglia output, then the ventral anterior (VA) and ventral lateral (VL) thalamocortical projection neurons become too inhibited, and one cannot initiate voluntary movement. These disorders are known as hypokinetic disorders. However, a disorder leading to abnormally low output of the basal ganglia leads to reduced inhibition, and thus excitation, of the thalamocortical projection neurons which synapse onto the cortex. This situation leads to an inability to suppress unwanted movements. These disorders are known as hyperkinetic disorders.

References

  1. Zegart, K. N.; Schwarz, R. H. (1968). "Chorea gravidarum". Obstetrics and Gynecology. 32 (1): 24–7. PMID   5742087.
  2. 1 2 3 Willson, Prentiss (1932). "Chorea Gravidarum". Archives of Internal Medicine. 49 (3): 471–533. doi:10.1001/archinte.1932.00150100128011.
  3. 1 2 3 Willson, Prentiss (1932). "Chorea Gravidarum". Archives of Internal Medicine. 49 (4): 671–97. doi:10.1001/archinte.1932.00150110134008.
  4. Black, M (1900). "Two cases of chorea in pregnancy". Glasgow Medical Journal: 441–4.
  5. Matthews, AA (1911). "Chorea complicating pregnancy". Northwest Med. 15: 372.
  6. Miranda, M; Cardoso, F; Giovannoni, G; Church, A (2004). "Oral contraceptive induced chorea: Another condition associated with anti-basal ganglia antibodies". Journal of Neurology, Neurosurgery, and Psychiatry. 75 (2): 327–8. doi:10.1136/jnnp.2003.019851. PMC   1738905 . PMID   14742621.
  7. Archelos, Juan J.; Hartung, Hans-Peter (2000). "Pathogenetic role of autoantibodies in neurological diseases". Trends in Neurosciences. 23 (7): 317–27. doi:10.1016/S0166-2236(00)01575-7. PMID   10856942.
  8. Chorea Gravidarum at eMedicine
  9. Axley, John (1972). "Rheumatic chorea controlled with haloperidol". The Journal of Pediatrics. 81 (6): 1216–7. doi:10.1016/S0022-3476(72)80272-5. PMID   4643046.
  10. Patterson, John F. (1979). "Treatment of Chorea Gravidarum with Haloperidol". Southern Medical Journal. 72 (9): 1220–1. doi:10.1097/00007611-197909000-00044. PMID   472859.
  11. Donaldson, J. O. (1982). "Control of chorea gravidarum with haloperidol". Obstetrics and Gynecology. 59 (3): 381–2. PMID   7078886.
  12. Shannon, K. M.; Fenichel, G. M. (1990). "Pimozide treatment of Sydenham's chorea". Neurology. 40 (1): 186. doi:10.1212/wnl.40.1.186. PMID   2296371.
  13. Chorea Gravidarum~treatment at eMedicine

Further reading

Classification
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