Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026827 (hypotonia)
 5,860 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

After the stereotactic treatment of patients with Parkinson's disease, a correlative study between the site of the deep subcortical lesions and subsequent cerebellar signs (dysmetria and hypotonia) was made. Cerebellar signs appeared in 27 cases (40.8%) of subthalamotomy and in 3 cases (8.6%) of VIM thalamotomy 2 weeks after an operation. The appearance of these signs after an operation was independent from the operative effect on tremor. Thus, we concluded that VIM thalamotomy might be better than subthalamotomy for the relief of tremor in Parkinson's disease.
 ...
PMID:Slight cerebellar signs in stereotactic thalamotomy and subthalamotomy for parkinsonism. 80 61

Forty patients have been treated with Sinemet (L-carbidopa/L-dopa) for a period of up to two years. The results are in agreement with those in the literature. In two-thirds of cases a good to very good improvement was obtained. The principal side effects were dyskinesia, hypotonia, and gastrointestinal and psychotic symptoms, though they seldom necessitated treatment interruption. L-carbidopa/L-dopa affords a real alternative therapy in the modern treatment of Parkinson's disease with L-dopa and a decarboxylase inhibitor. Generally the dosage range was up to a maximum of one tablet three times daily. Sinemet tablets are simple and convenient to handle for both doctor and patient. Dosage titration to therapeutic efficacy can be achieved in one week to ten days without complications, though we recommended a slower titration based on individual patient reaction and requirements.
 ...
PMID:[Treatment of Parkinson's disease with the combination drug L-carbidopa/L-dopa. Report on a 2 years study]. 84 50

Neurophysiological studies were performed on 8 patients with group A xeroderma pigmentosum during early childhood. EEG, ABR and NCV were normal during this period. In contrast, various sleep parameters detected by polysomnography showed abnormal findings even in the neurologically normal patient. Decreased % sleep REM was seen in a case, and decreased frequency of REMs were seen in another. Body movements were extremely high or low in frequency in 3 cases in whole night sleep. The distribution of body movements were abnormal; in control subjects, the frequency was higher in SREM and stage 1 than in slow wave sleep; in 7 cases, it was higher in slow wave sleep than in stage 1 or 2, or body movements were extremely frequent. Neurological examination revealed soft signs in various systems in early childhood. All cases except one showed hypotonia. Many cases were slow in learning to walk and the gait was unstable. Speech delay and decreased deep tendon reflexes, especially of patella, were seen in most cases. Since the neural deficits in XP may be related to the DNA repair defect, these findings indicate the possibility that some endogenous compounds distributing all over the nervous system might produce the DNA damages. Because the body movements during sleep are controlled by the nigrostriatal dopaminergic system, present data indicate that the basal ganglia might be one of the earliest degenerative systems in the CNS. Recently, some studies have suggested the possibility that oxygen radical mechanisms might be involved in the development of the dopamine neurodegenerative process in Parkinson's disease.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Neurophysiological studies on group A xeroderma pigmentosum in early childhood]. 217 31

The paralysis due to lesions of the cortico-spinal tract disturb movements in their different modes while dissociated paralysis result from cortical and particular biopercular lesions. Movements cannot be executed on command whereas they are accomplished in their total complexity when they accompanied certain emotional states or when they are part of an automatic activity. Such a dissociation suggest that the motor program which does not respond to voluntary command will eventually be applied thanks to emotional or affective stimuli. Basal ganglia lesions induce other symptoms. The first to be observed concern the movements and postures implied in automatic series after learning. Akinesias which results from these lesions can be partially controlled by a voluntary command, particularly when helped by sensori stimuli. Such symptoms are observed sometimes from its beginning in the Parkinson's disease. Emotion and sleep are also able to improve these akinesias: therefore they appear either on a deficitary form implying a sensitive reinforcement or on a hyperactive form, which is alleviated by hypotonia and sleep. The localized lesions of striatum or of pallidum provoke identical symptoms but at the interruption of akinesia, a distortion of movement appears, contrary to what is observed in Parkinson's disease. Mechanisms of anticipation and adjustment of postural reaction are disturbed concerning not only the body axis but also the different segments of the limbs. This disturbance provokes unsteadiness and sometimes falls. At the level of the limbs the anomaly of postural reactions slows down the movement and disturbs specially the repetitive gestures.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Akinesia and associated motor involvement in Parkinson's disease and basal ganglia lesions]. 226 22

A 45-year-old woman with a history of probable perinatal craniocerebral trauma resulting in mild asymptomatic right hemiatrophy developed right leg weakness and hypotonia alternating with dystonia only after prolonged exertion at age 12. At age 27, she developed right-sided parkinsonism. Exertional paresis and dystonia and parkinsonism responded completely to levodopa; however, she developed a progressive reduction in the duration of action of levodopa over the first 4 years of treatment. Investigations including computed tomography, magnetic resonance imaging, [18F]fluorodopa, and [18F]fluorodeoxyglucose positron emission tomography scans suggested a static lesion involving the left substantia nigra. This unusual exertion-induced weakness and hypotonia alternating with hypertonia and dystonia has not been reported previously. The role of dopamine deficiency in dystonia and the role of levodopa in the development of fluctuations in Parkinson's disease are discussed. Review of the literature, including this patient, emphasizes the heterogeneity of the syndrome of hemiparkinsonism-hemiatrophy.
 ...
PMID:Hemiatrophy, juvenile-onset exertional alternating leg paresis, hypotonia, and hemidystonia and adult-onset hemiparkinsonism: the spectrum of hemiparkinsonism-hemiatrophy syndrome. 756 31

In the present study, we investigated the ability of NMDA receptor antagonists to inhibit catalepsy induced by haloperidol, or SCH23390 and clebopride, selective dopamine D1 and D2 receptor antagonists respectively. Catalepsy was measured by recording the time the animal remained with its forepaws placed over a rod 6 cm above the bench. Pretreatment with either the non-competitive NMDA receptor antagonist, MK-801 (0.25-0.5 mg/kg i.p.) or the competitive antagonist, LY274614 (10-20 mg/kg i.p.) reduced the cataleptic response produced by haloperidol (10 mg/kg), SCH23390 (2.5-10 mg/kp i.p.) or clebopride (5-20 mg/kg i.p.). This demonstrates that NMDA receptor antagonists will reduce both dopamine D1 and D2 receptor antagonist-induced catalepsy. Muscle relaxant doses of chlordiazepoxide (10 mg/kg i.p.) failed to reduce the catalepsy induced by haloperidol, suggesting that the anticataleptic effect of the NMDA receptor antagonists was not due to a non-specific action. These results support the hypothesis that NMDA receptor antagonists may have beneficial effects in disorders involving reduced dopaminergic function, such as Parkinson's disease.
 ...
PMID:NMDA receptor antagonists inhibit catalepsy induced by either dopamine D1 or D2 receptor antagonists. 835 5

Application of dopamine agonists in the therapy of Parkinson's disease constitutes significant progress. The adverse effects of dopamine agonists in the treatment of this disease are caused by various action mechanisms and depend upon the composition and pharmacological characteristics of the drug, its impact upon the dopaminergic and non-dopaminergic receptors, as well as dosage and duration of treatment. The most important ones include: nausea and vomiting, orthostatic hypotonia, psychiatric syndromes, sleep disturbances, pleuropulmonary and retroperitoneal fibrosis, vasoconstrictive properties, oedema, hormonal disturbances, as well as dyskinesias. Knowledge of such adverse effects is necessary for each physician, although in Poland dopamine agonists are used in a minority of patients due to the significant costs of the treatment.
 ...
PMID:[Adverse effects of dopamine agonists]. 1794 57

Aminergic neurotransmitter disorders occurring in childhood include metabolic disorders of pteridine and tyrosine hydroxylase (TH). Pteridine metabolic disorders cause a deficiency of serotonin (5-HT) and dopamine (DA) and TH disorder causes a deficiency of noradrenaline (NA) and DA in the terminals of each aminergic neuron. The activities of TH or DA in the terminals are marked in early childhood, and then they show an exponential age-dependent decrement and achieve stationary or minimal levels in the twenties. As observed in Segawa disease, TH or DA activities in these disorders follow this age-related decrease with levels around 20% of normal, and patients develop symptoms age-dependently, with onset in childhood, progression by the late teens, and a stationary period after the twenties, but this does not cause morphological changes. These phenomena may occur with other neurotransmitters. So replacement therapies are effective irrespective of the clinical course. However, early-onset cases in infancy or early childhood showing a marked decrement of 5-HT or NA activities show postural hypotonia and failed locomotion. These cause failure in atonia restriction in the REM stage and induce dysfunction of the pedunculopontine nucleus, and, consequently induce dysfunction or failure in the development of DA neurons in the sutbstantia nigra and ventrotegmental area. These relate to failure in the development of higher cortical functions. Thus, assessing of ages at onset and activities of antigravity muscles and locomotion in infancy is cardinal for the treatment the neurotransmitter disorders occurring in infancy and early childhood. PARK2 with deficiency of DA in the substantia nigra leads to dystonia in the teens and Parkinson disease after 20 years, although these respond to 1-Dopa favorably but induce D2 receptor upregulation and intractable dyskinesia. A decrease of DA in the perikaryon leads to symptoms after 10 years and causes dysfunction of the target structures.
 ...
PMID:[Neurotransmitter disorders in children--special reference to Segawa disease]. 2194 41

Striatum is the central structure controlling movement. It plays a pivotal role in the regulation of voluntary movement, unconscious movement, muscle tone, posture adjustment and fine movement. Dysfunction of striatum causes a variety of movement disorders ranging from the hypokinetic disorders with increased muscle tone, such as Parkinson's disease, to the hyperkinetic disorders with decreased muscle tone, such as Huntington's disease. It is generally recognized that striatum receives the neural movement signals from the motor cortex, and then processes and modifies these signals and subsequently transfers the signals back to the motor cortex via thalamus for execution of the movement through pyramidal system. The movement control function of striatum depends on a complex neural circuit system. In this review, the studies on the movement control function of striatum as well as the striatal neural circuit system are summarized with an emphasis on the progress made during recent years for better understanding the mechanism underlying the movement control function as well as the disease association of striatum.
 ...
PMID:[Movement Control of Striatum Neural Pathway]. 2988 93

Satisfactory management of Parkinson's disease is a challenge that requires a tailored approach for each individual. In the advanced phase of the disease, patients may experience motor complications despite optimized pharmacological therapy. Apomorphine, a short-acting D1- and D2-like receptor agonist, is the only drug proven to have an efficacy equal to that of levodopa, albeit with a shorter time to onset and effect duration. Clinical trials have shown that intermittent apomorphine injections provide rapid and effective relief from unpredictable "off" periods. Continuous apomorphine infusion reduced around 50% of the daily "off" time in several studies. Dopaminergic side effects such as nausea, somnolence and hypotonia, as well as administration site reactions, are often mild or treatable, but somnolence and skin reactions in particular can sometimes be reasons for premature discontinuation. We provide an overview of the pharmacological mechanism of action of the drug in light of its effects on Parkinson's disease symptoms. We then summarize the evidence regarding the efficacy and tolerability of apomorphine, both in its established formulations (subcutaneous intermittent injection and continuous infusion) and in the new preparations currently under investigation.
 ...
PMID:Apomorphine for Parkinson's Disease: Efficacy and Safety of Current and New Formulations. 3147 80


1