Chorea gravidarum
Encyclopedia
Chorea gravidarum is a rare 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 an etiologically or pathologically distinct morbid 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.
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.
History of either rheumatic fever
or chorea is suspected: 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. 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.
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 streptoccocal infections, but had anti-basal ganglia antibodies, suggesting immunological
basis for the pathophysiology of this chorea.
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.
, chlorpromazine
alone or in combination with diazepam
, and also pimozide
, which is another neuroleptic drug which may have fewer adverse effects than haloperidol. Valproic acid
, chloral hydrate
, risperidone
, or phenobarbital
can also be used.
Chorea (disease)
Choreia is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias. The term choreia is derived from the Greek word χορεία , see choreia , as the quick movements of the feet or hands are vaguely comparable to dancing or piano playing.The term...
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
Pregnancy
Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...
which can be associated with eclampsia
Eclampsia
Eclampsia , an acute and life-threatening complication of pregnancy, is characterized by the appearance of tonic-clonic seizures, usually in a patient who had developed pre-eclampsia...
and its effects upon the basal ganglia
Basal ganglia
The basal ganglia are a group of nuclei of varied origin in the brains of vertebrates that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex, thalamus and other brain areas...
. It is not an etiologically or pathologically distinct morbid entity but a generic term for chorea of any cause starting during pregnancy. It is associated with history of Sydenham's chorea
Sydenham's chorea
Sydenham's chorea or chorea minor is a disease characterized by rapid, uncoordinated jerking movements affecting primarily the face, feet and hands. Sydenham's chorea results from childhood infection with Group A beta-hemolytic Streptococci and is reported to occur in 20-30% of patients with...
. It mostly occurs in young patients; the average age is 22 years.
Recently there has been a decline in incidence which is probably the result of a decline in rheumatic fever
Rheumatic fever
Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...
(RF
Rheumatic fever
Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...
), which was a major cause of chorea gravidarum before the use of antibiotics for streptococcal pharyngitis.
Pathophysiology
Several pathogenetic mechanisms for chorea gravidarum have been offered, but none have been proven.History of either rheumatic fever
Rheumatic fever
Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain, the illness typically develops two to three weeks after...
or chorea is suspected: the suggestion is that estrogens and progesterone
Progesterone
Progesterone also known as P4 is a C-21 steroid hormone involved in the female menstrual cycle, pregnancy and embryogenesis of humans and other species...
may sensitize dopamine
Dopamine
Dopamine is a catecholamine neurotransmitter present in a wide variety of animals, including both vertebrates and invertebrates. In the brain, this substituted phenethylamine functions as a neurotransmitter, activating the five known types of dopamine receptors—D1, D2, D3, D4, and D5—and their...
receptors (presumably at a striatal level
Striatum
The striatum, also known as the neostriatum or striate nucleus, is a subcortical part of the forebrain. It is the major input station of the basal ganglia system. The striatum, in turn, gets input from the cerebral cortex...
) and induce chorea in individuals who are vulnerable to this complication by virtue of preexisting pathology
Pathology
Pathology is the precise study and diagnosis of disease. The word pathology is from Ancient Greek , pathos, "feeling, suffering"; and , -logia, "the study of". Pathologization, to pathologize, refers to the process of defining a condition or behavior as pathological, e.g. pathological gambling....
in the basal ganglia
Basal ganglia
The basal ganglia are a group of nuclei of varied origin in the brains of vertebrates that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex, thalamus and other brain areas...
.
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. 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.
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 streptoccocal infections, but had anti-basal ganglia antibodies, suggesting immunological
Autoimmune disease
Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. In other words, the body actually attacks its own cells. The immune system mistakes some part of the body as a pathogen and attacks it. This may be restricted to...
basis for the pathophysiology of this chorea.
Differential Diagnoses
- Cerebellar Hemorrhage
- Pelizaeus-Merzbacher DiseasePelizaeus-Merzbacher diseasePelizaeus–Merzbacher disease is a rare central nervous system disorder in which coordination, motor abilities, and intellectual function are delayed to variable extents.-Classification:...
- Hallervorden-Spatz DiseasePantothenate kinase-associated neurodegenerationPantothenate kinase-associated neurodegeneration , also known as neurodegeneration with brain iron accumulation 1 and formerly called Hallervorden-Spatz syndrome Pantothenate kinase-associated neurodegeneration (PKAN), also known as neurodegeneration with brain iron accumulation 1 (NBIA1) and...
- Ramsay Hunt SyndromeRamsay Hunt syndromesRamsay Hunt syndrome type 2 also known as herpes zoster oticus is a disorder that is caused by the reactivation of pre-existing herpes zoster virus in a nerve cell bundle in the head...
- Huntington Disease
- Striatonigral DegenerationMultiple system atrophyMultiple system atrophy is a degenerative neurological disorder. MSA is associated with the degeneration of nerve cells in specific areas of the brain. This cell degeneration causes problems with movement, balance and other autonomic functions of the body such as bladder control or blood pressure...
- Lesch-Nyhan SyndromeLesch-Nyhan syndromeLesch–Nyhan syndrome , also known as Nyhan's syndrome, Kelley-Seegmiller syndrome and Juvenile gout, is a rare inherited disorder caused by a deficiency of the enzyme hypoxanthine-guanine phosphoribosyltransferase , produced by mutations in the HPRT gene located on X chromosome. LNS affects about...
- Systemic Lupus ErythematosusSystemic lupus erythematosusSystemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...
- Lyme DiseaseLyme diseaseLyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...
- TorticollisTorticollisTorticollis, or wryneck, is a stiff neck associated with muscle spasm, classically causing lateral flexion contracture of the cervical spine musculature...
- Multiple System AtrophyMultiple system atrophyMultiple system atrophy is a degenerative neurological disorder. MSA is associated with the degeneration of nerve cells in specific areas of the brain. This cell degeneration causes problems with movement, balance and other autonomic functions of the body such as bladder control or blood pressure...
- Tourette SyndromeTourette syndromeTourette syndrome is an inherited neuropsychiatric disorder with onset in childhood, characterized by multiple physical tics and at least one vocal tic; these tics characteristically wax and wane...
and Other Tic Disorders - NeuroacanthocytosisNeuroacanthocytosisNeuroacanthocytosis is a term that refers to a group of genetically diverse conditions complicated by movement disorders, neurological problems and spiculated red blood cells...
- Viral EncephalitisViral encephalitisViral encephalitis refers to a type of Encephalitis caused by a virus.Encephalitis may be caused by a variety of afflictions.Types include:*Arbovirus encephalitis**La Crosse encephalitis**California encephalitis virus**Japanese Encephalitis**St...
- Neuronal Ceroid Lipofuscinoses
- Wilson disease
- Olivopontocerebellar AtrophyOlivopontocerebellar atrophyOlivopontocerebellar atrophy is a term used to define neuronal degeneration in the cerebellum, pontine nuclei, and inferior olivary nucleus...
- Familial paroxysmal choreoathetosis
- Benign hereditary chorea
Chorea can also be a manifestation of drug toxicity (for example anticonvulsants, antiparkinson agents
Antiparkinson drug
An antiparkinson, or antiparkinsonian, is a type of drug which is intended to treat and relieve the symptoms of Parkinson's disease or Parkinsonism...
, neuroleptics, steroids, and estrogen
Estrogen
Estrogens , oestrogens , or œstrogens, are a group of compounds named for their importance in the estrous cycle of humans and other animals. They are the primary female sex hormones. Natural estrogens are steroid hormones, while some synthetic ones are non-steroidal...
), or a result of an infectious disease such as meningovascular syphilis, Lyme disease
Lyme disease
Lyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...
, viral encephalitis
Viral encephalitis
Viral encephalitis refers to a type of Encephalitis caused by a virus.Encephalitis may be caused by a variety of afflictions.Types include:*Arbovirus encephalitis**La Crosse encephalitis**California encephalitis virus**Japanese Encephalitis**St...
, and many others.
Treatment
Drug treatment is indicated for patients with severe disabling chorea. It is treated with haloperidolHaloperidol
Haloperidol is a typical antipsychotic. It is in the butyrophenone class of antipsychotic medications and has pharmacological effects similar to the phenothiazines....
, chlorpromazine
Chlorpromazine
Chlorpromazine is a typical antipsychotic...
alone or in combination with diazepam
Diazepam
Diazepam , first marketed as Valium by Hoffmann-La Roche is a benzodiazepine drug. Diazepam is also marketed in Australia as Antenex. It is commonly used for treating anxiety, insomnia, seizures including status epilepticus, muscle spasms , restless legs syndrome, alcohol withdrawal,...
, and also pimozide
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...
, which is another neuroleptic drug which may have fewer adverse effects than haloperidol. Valproic acid
Valproic acid
Valproic acid is a chemical compound that has found clinical use as an anticonvulsant and mood-stabilizing drug, primarily in the treatment of epilepsy, bipolar disorder, and, less commonly, major depression. It is also used to treat migraine headaches and schizophrenia...
, chloral hydrate
Chloral hydrate
Chloral hydrate is a sedative and hypnotic drug as well as a chemical reagent and precursor. The name chloral hydrate indicates that it is formed from chloral by the addition of one molecule of water. Its chemical formula is C2H3Cl3O2....
, risperidone
Risperidone
Risperidone is a second generation or atypical antipsychotic, sold under the trade name . It is used to treat schizophrenia , schizoaffective disorder, the mixed and manic states associated with bipolar disorder, and irritability in people with autism...
, or phenobarbital
Phenobarbital
Phenobarbital or phenobarbitone is a barbiturate, first marketed as Luminal by Friedr. Bayer et comp. It is the most widely used anticonvulsant worldwide, and the oldest still commonly used. It also has sedative and hypnotic properties but, as with other barbiturates, has been superseded by the...
can also be used.