Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dipropylacetic acid (DPA), gamma-aminobutyric acid (GABA), physostigmine, CB 154 and butyrophenones were administered to 26 patients affected by Huntington's chorea. The evaluation of the pharmacologic activity of the different drugs was determined by means of some clinical parameters and motor performance tests. Butyrophenones succeeded to ameliorate the hyperkinesias and the motor dexterity, CB 154 provoked a worsening of the motor signs of the disorder, while DPA, GABA and physostigmine did not modify the clinical pattern. The relationship between these results and the Hungtington's chorea physiopathology is discussed and a hypersensitivity of DA receptors is suggested.
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PMID:Pharmacology of Huntington's chorea. Personal experience. 15 Mar 32

Huntington's Chorea has multiple neurologic and psychiatric manifestations, and its treatment has been the subject of much debate. It appears that no single agent is effective for all patients with Chorea. Furthermore, different biochemical substances may be lacking in various Huntington's patients, i.e., gamma aminobutyric acid (GABA) in some, or a deficiency of acetylcholine (ACH) receptors in others. We approach this disease as a possible "spectrum illness," characterized by a relative deficiency of one substance, or a relative excess of another. If patients do not respond to one family of drugs, they may still benefit from a trial with another drug group.
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PMID:Huntington's Chorea and dimethylaminoethanol (deanol). 15 Apr 13

The habituation index is a quantitative expression of the ability of the orbicularis oculi (blink reflex) to adapt to a series of electrical stimuli applied to the supraorbital region. This parameter has been studied in a group of normal control subjects, and the results compared with those in cases of idiopathic and drug-induced Parkinsonism, states of dementia, and dyskinesias such as Huntington's chorea and senile chorea. Patients with Huntington's chorea showed a tendency for the reflex to habituate readily in contrast to patients with dementia caused by cortical atrophy and those with Parkinson's disease. Younger patients with Huntington's chorea had indices within the normal range. It seems unlikely that this test will prove of value in the detection of clinically unaffected relatives. Where dementia was associated with a reversible intracranial lesion, the habituation index was studied before and after treatment. Failure of habituation in this condition appears to be due to the release of a primitive protective reflex.
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PMID:Habituation of the orbicularis oculi reflex in dementia and dyskinetic states. 15 Nov 27

Huntington's chorea is a dominantly inherited disorder that usually leads to involuntary movements in the third or fourth decade. On gross pathological examination of the post-mortem brain there is a marked atrophy of the caudate nucleus and putamen. Histological examination reveals cell loss in most regions of the brain, although the hippocampus is usually remarkably free of any abnormalities. Studies to detect a biochemical defect in patients with chorea have been largely unrewarding. Since chorea appears to be the clinical counterpart of Parkinson's disease a number of investigations on dopamine metabolism have been carried out by measuring dopamine in the post-mortem choreic brain, and HVA, a metabolite of dopamine, in the CSF of patients. Most studies have found the dopamine concentrations to be normal or sometimes decreased and the activity of the biosynthetic enzyme for dopamine, tyrosine hydroxylase, is normal. The discovery that the inhibitory neurotransmitter, GABA, and the biosynthetic enzyme GAD are greatly decreased in the post-mortem choreic brain provides some rational explanation for the uncontrolled movements in this disorder. The other significant abnormality found in many, but not all, choreic post-mortem brains has been a decrease in the biosynthetic enzyme for acetylcholine, choline acetyl transferase. The evidence that GABA receptors are intact in choreic brain provides an added stimulus for the development of useful GABA-mimetic drugs. For the ultimate eradication of this distressing disorder, however, a search must continue for the primary defect in order that this can be detected before the onset of symptoms, or hopefully in amniotic fluid.
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PMID:Neurochemical findings in Huntington's chorea. 15 97

Muscimol, a gamma-aminobutyric acid (GABA) analogue that exerts potent and specific agonist effects on GABA receptors, was administered orally to 10 patients with Huntington's disease. In this double-blind study, muscimol treatment did not result in improvement in these patients' motor or cognitive functions. However, muscimol administration did ameliorate chorea in the most severely hyperkinetic patient, and it was associated with the appearance of dystonic features, electroencephalographic changes, and behavioral alterations in some patients. These latter observations support a functional relationship between GABA-ergic activity and the genesis of both systonia and EEG abnormalities in humans. The therapeutic failure of muscimol indicates that the GABA disturbances in Huntington's disease does not alone account for the clinical features of this disorder.
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PMID:Huntington's disease: treatment with muscimol, a GABA-mimetic drug. 15 2

The effects of Baclofen, a gabamimetic drug, have been studied in 4 patients affected by Huntington's Chorea. The biochemical bases of Chorea are discussed in relation to the HVA modifications in the cerebrospinal fluid induced by the drug. Some interesting data on the extrapyramidal physiology may be achieved with further investigations in a greater group of patients.
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PMID:[Choreic dyskinesia and GABA - pharmaco-clinical approach. Preliminary note]. 15 81

Clinical features suggesting a diagnosis of Huntington's chorea in several siblings in a Black Rhodesian family are described. A progressive and eventually fatal mental deterioration accompanied by gradually worsening choreiform movements has until the present time occurred in only a single generation of this family, which precludes a definitive diagnosis based on the triad of family history, chorea and dementia. It is suggested that Huntington's chorea is the most likely diagnosis.
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PMID:Huntington's chorea in a black Rhodesian family. 15 74

The involuntary movements of Huntington disease may be related to cholinergic hypofunction in the striatum. For this reason, the effect of a direct cholinergic agonist, arecoline, was studied in six patients with this disorder. Rather than improving the chorea, arecoline tended to exacerbate the choreic movements. Arecoline did produce significant alterations of blood pressure, heart rate, and body temperature, probably by central cholinergic stimulation.
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PMID:Treatment of Huntington disease with a cholinergic agonist. 15 24

A 56-year-old woman had a 50-year history of childhood-onset chorea without progressive neurologic deficit. The patient's father had a lifelong extrapyramidal disorder characterized by a head-nodding tremor and involuntary movements especially evident with anxiety. The computerized tomographic scan was normal, without evidence of cortical or caudate atrophy. The computerized tomographic scan supports the notion of a functional rather than a structural lesion, and may aid in the discrimination of hereditary nonprogressive chorea from more devastating forms of hereditary chorea.
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PMID:Computerized tomography in hereditary nonprogressive chorea. 15 81

The cerebrospinal fluid (CSF) content of homovanillic acid (HVA) was assayed in 10 patients with Huntington disease. On doses of less than 40 mg of bromocriptine daily, there was clinical improvement and the CSF HVA concentration increased. On higher doses of bromocriptine, chorea worsened and the CSF HVA concentration decreased. Bromocriptine at low dosage seems to act as a partial dopamine antagonist, with phenothiazine-like effects, and at higher doses it acts as a direct dopamine-receptor stimulating agent.
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PMID:Bromocriptine and dopaminergic function in Huntington disease. 15 84


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