Causes of Huntington's Disease
Huntington's Disease Causes: Book Excerpts
Related information on causes of Huntington's Disease:
As with all medical conditions,
there may be many causal factors.
Further relevant information on causes of Huntington's Disease may be found in:
Causes of Huntington's Disease: Online Medical Books
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for more information about the causes of Huntington's Disease.
Chorea:
Differential Diagnosis
(In a Page: Signs and Symptoms)
Huntington's disease (chronic progressive
hereditary chorea)
–Autosomal dominant transmission
–Associated with psychiatric symptoms and
progressive dementia
–Caudate atrophy on neuroimaging studies
–Marker on chromosome 4
-
Sydenham's chorea
–Symptoms follow febrile illness (20–30%
of cases are associated with group A strep)
–Seen in rheumatic fever
–Peak ages: 5–13 years
–More common in females
Systemic lupus erythematosus
AIDS
Hyperthyroidism
-
Chorea gravidarum
–Develops in the first 4–5 months of
pregnancy
–Resolves following delivery
Drug-induced (e.g., levodopa, stimulants, anticonvulsants, antidepressants, neuroleptics, oral contraceptives)
Stroke
Neoplasm
-
Wilson's disease
–Autosomal recessive disorder
–Deficiency in copper metabolism
–Associated with hepatic dysfunction,
dystonia, dysarthria
Benign hereditary chorea
–Autosomal dominant
–Onset before age 5
–Symptoms are nonprogressive
-
Neuroacanthocytosis
–Etiology unknown
–Characterized by chorea and deformed
erythrocytes
DRPLA
–Most common in Japan
–Characterized by chorea, ataxia, epilepsy,
and dementia
» READ BOOK EXCERPT ONLINE »
Source: In a Page: Signs and Symptoms, 2004
Chorea:
Differential Diagnosis
(In A Page: Pediatric Signs and Symptoms)
-
Toxins
–Neuroleptics, phenytoin, antiemetics, oral contraceptives, theophylline, L-dopa, stimulants, lithium, carbon monoxide, manganese
-
Sydenham chorea (in rheumatic fever)
–Migratory chorea, hypotonia, dysarthria,
emotional liability
–Usually 4 months after group A β-hemolytic Streptococcus infection
–Molecular mimicry between streptococcal and CNS antigens results in formation of cross-reactive antibodies that disrupt basal ganglia function
–Carditis is present in 80% of Sydenham chorea patients
-
Inherited choreas
–Benign familial chorea
–Juvenile Huntington chorea (usually presents
with rigidity)
–Familial paroxysmal choreoathetosis
-
Postinfectious: Mycoplasma, HSV, EBV, echovirus 25, varicella
-
Encephalitis: viral, mycoplasma, Lyme
-
Post-cardiac surgery
–“Post-pump chorea”
–Usually 2 weeks after cardiac surgery
- Syndrome or disease associated
–Wilson disease
–Hallervorden-Spatz (disorder of iron metabolism with degeneration of globus pallidus)
–Fahr disease: Encephalopathy and
progressive calcification of basal ganglia
–Lesch-Nyhan syndrome
–Ataxia-telangiectasia
-
Endocrine: Hyperthyroidism, pregnancy (chorea gravidarum)
-
Acquired brain disorders
–Multiple sclerosis, basal ganglia stroke, hypoxic ischemic encephalopathy, neoplasm
-
Abetalipoproteinemia
-
Glutaric aciduria type I
-
Neuroacanthocytosis
-
Systemic lupus erythematosus
-
Kernicterus
-
Antiphospholipid antibody syndrome
-
Mitochondrial encephalopathies
» READ BOOK EXCERPT ONLINE »
Source: In A Page: Pediatric Signs and Symptoms, 2007
Huntington's disease:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
Huntingdon’s disease is inherited as a single faulty gene on chromosome #4 whereby part of the gene is repeated in multiple copies. It’s transmitted as an autosomal dominant trait; either sex can transmit and inherit it. Each child of a parent with this disease has a 50% chance of inheriting it.
The disease usually strikes people between ages 35 and 55; however, 2% of cases occur in children, and 5% of cases occur as late as age 60. Death usually results 10 to 15 years after onset, from suicide, heart failure, or pneumonia. Genetic testing is available for persons with a family history of the disease.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Chorea [Choreiform movements]:
Medical causes
(Professional Guide to Signs & Symptoms (Fifth Edition))
Cerebral infarction
An infarction that involves the thalamic area produces unilateral or bilateral chorea. The patient may also experience dysarthria, tremors, rigidity, weakness, and sensory disturbances such as paresthesia.
Encephalitis
Chorea may occur in the recovery phase of encephalitis. Low-grade fever and athetosis may also be present, in addition to such focal neurologic signs as hemiparesis, hemiplegia, and facial droop.
Huntington’s disease
In this inherited disease, chorea may be the first sign or it may accompany the intellectual decline that leads to emotional disturbances and dementia. The patient’s movements tend to be choreoathetotic and may be accompanied by dysarthria, dystonia, prancing gait, dysphagia, and facial grimacing.
Wilson’s disease
Chorea and dystonia affecting the arms and legs are early indicators of Wilson’s disease. The patient typically experiences dysarthria, tremors, hoarseness, dysphagia, and slowed body movements; he may also exhibit emotional and behavioral disturbances, drooling, rigidity, and mental deterioration. The pathognomonic Kayser-Fleischer ring in the cornea appears as the disease progresses.
Other causes
Carbon monoxide poisoning
A patient who survives severe carbon monoxide poisoning may have neurologic signs and symptoms, such as chorea, rigidity, dementia, impaired sensory function, masklike facies, generalized seizures, and myoclonus.
Drugs
Phenothiazines (especially the piperazine derivatives), haloperidol, thiothixene, and loxapine commonly produce chorea. Metoclopramide, metyrosine, hormonal contraceptives, levodopa, and phenytoin may also cause this sign.
Lead poisoning
In the later stages, lead poisoning produces chorea in addition to seizures, headache, memory lapses, and severe mental impairment. The patient may also develop masklike facies, footdrop, wristdrop, dizziness, ataxia, weakness, lethargy, abdominal pain, anorexia, nausea, vomiting, constipation, lead line on the gums, and a metallic taste in his mouth.
Manganese poisoning
In miners who have been exposed to manganese dioxide for prolonged periods, chorea characteristically occurs with a propulsive gait, dystonia, and rigidity. Initially, the patient may have masklike facies, a resting tremor, and personality changes; later, extreme muscle weakness and lethargy occur.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Signs & Symptoms (Fifth Edition), 2006
Huntington's disease:
Causes
(Handbook of Diseases)
Huntington’s disease is transmitted as an autosomal dominant trait, and either sex can transmit and inherit it. Each child of a parent with this disease has a 50% chance of inheriting it; the child who inherits it can pass it on to his own children.
Because of hereditary transmission, Huntington’s disease is prevalent in areas in which affected families have lived for several generations. Genetic testing is offered to those with a known family history of the disease.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
Chorea:
Medical causes
(Signs & Symptoms: A 2-in-1 Reference for Nurses)
Carbon monoxide poisoning
A patient who survives severe carbon monoxide poisoning may have neurologic signs and symptoms, such as chorea, rigidity, dementia, impaired sensory function, masklike facies, generalized seizures, and myoclonus.
Cerebral infarction
A cerebral infarction that involves the thalamic area produces unilateral or bilateral chorea. The patient may also experience dysarthria, tremors, rigidity, weakness, and sensory disturbances such as paresthesia.
Encephalitis
Chorea may occur in the recovery phase of encephalitis. Low-grade fever and athetosis may also be present, in addition to such focal neurologic signs as hemiparesis, hemiplegia, and facial droop. Other signs and symptoms include headache, vomiting, photophobia, stiff neck, confusion, and drowsiness.
Huntington’s disease
In Huntington’s disease, an inherited disease, chorea may be the first sign or may occur with the intellectual decline that leads to emotional disturbances and dementia. The patient’s movements tend to be choreoathetotic and may be accompanied by dysarthria, dystonia, prancing gait, dysphagia, and facial grimacing.
Lead poisoning
In the later stages of lead poisoning, chorea occurs in addition to seizures, headache, memory lapses, and severe mental impairment. The patient may also develop masklike facies, footdrop, wristdrop, dizziness, ataxia, weakness, lethargy, abdominal pain, anorexia, nausea, vomiting, constipation, lead line on his gums, and a metallic taste in his mouth.
Manganese poisoning
In miners who have been exposed to manganese dioxide for prolonged periods, chorea characteristically occurs with propulsive gait, dystonia, and rigidity. Initially, the patient may have masklike facies, a resting tremor, and personality changes; later, extreme muscle weakness and lethargy occur.
Other causes
Drugs
Such drugs as phenothiazines (especially the piperazine derivatives), haloperidol, thiothixene, and loxapine commonly produce chorea. Metoclopramide, metyrosine, hormonal contraceptives, levodopa, and phenytoin may also cause this sign.
» READ BOOK EXCERPT ONLINE »
Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007
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