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)

A double-blind cross-over trial of the effects of baclofen and placebo was carried out in 20 female patients suffering from neuroleptic-induced tardive dyskinesia. After 14 days of treatment 15 patients showed improvement of baclofen, whereas none showed improvement on placebo; baclofen was thus significantly more effective than placebo. Baclofen is a GABA-like drug which passes through the blood-brain barrier and which reduces the neuroleptic-induced increase of dopamine turn-over. In tardive dyskinesia is found dopaminergic hypersensitivity, and baclofen is supposed to exert its action by inhibiting the dopamine activity. Side effects, although temporary, were observed in the form of sedation, muscular hypotonia, dizziness, vomiting, and muscular rigidity. One patient developed a depression. Baclofen or other gabergic drugs used in the treatment of dyskinesias do not increase the dopaminergic hypersensitivity, which is part of the pathogenesis of these conditions; gabergic therapy must therefore be preferred to treatment with dopamine receptor blocking drugs.
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PMID:Baclofen (Lioresal) in the treatment ofneuroleptic-induced tardive dyskinesia. 78 59

The neuroleptic effect and tolerability of roxindole (EMD 49,980), an agonist of the dopamine-D2 autoreceptor, was studied during a 4 week treatment period in 7 patients with paranoid-hallucinatory schizophrenia (ICD-9: 295.3). In patients with a daily dosage of up to 4.5 mg/day, there was no improvement as measured with the total score of the BPRS scale. In contrast, patients with a daily dosage of up to 30 mg/day showed a slight improvement, especially in items associated with negative symptoms. In 3 patients there were slight adverse events (dizziness, hypersalivation, hypotonia, nausea/vomiting, miction disturbance) which were probably connected with the intake of roxindole.
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PMID:Early clinical results with the neuroleptic roxindole (EMD 49,980) in the treatment of schizophrenia--an open study. 135 88

The indications for administering clozapine to 21 adolescent inpatients with schizophrenia were insufficient response to other neuroleptic agents, likelihood of psychotic symptoms becoming chronic and extensive extrapyramidal side-effects from other neuroleptic agents. Marked improvement in or complete disappearance of most of the remaining psychopathological symptoms was seen in 11 of the 21 subjects, with at least some improvement seen in an additional 6. Vegetative side-effects such as daytime weariness, dizziness, orthostatic hypotonia and hypersalivation were usually transient. Deviations from the normal range of values for the hematological and serum enzyme values assessed were of no clinical significance and occurred no more frequently than with other neuroleptic agents, and the values returned to normal when treatment was continued. On the whole, clozapine is a useful addition to the group of drugs available for treating adolescents with schizophrenia.
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PMID:[Results of treatment with clozapine in schizophrenic adolescents]. 376 97

The aim of this study was to determine the efficacy, safety, and cost-effectiveness of intrathecal baclofen delivered by a programmable pump for the chronic treatment of severe spasticity. Sixty-six patients with severe spasticity of spinal cord origin that was refractory to oral baclofen or who experienced intolerable side effects with this form of the drug were screened. The first nine participated in a double-blinded, randomized, placebo (normal saline)-controlled trial to determine response to a bolus dose of intrathecal baclofen. Subsequent patients were enrolled in an open-label treatment protocol without a placebo trial. All passed the screening, and the pump was implanted in 59 patients. Spasticity scores and medical costs before and after surgery were analyzed. In all patients, the mean Ashworth score for rigidity decreased from 4.3 preoperatively to 1.4 (p < 0.0005) with use of intrathecal baclofen. The spasm frequency score decreased from a mean of 3.6 to 0.5 (p < 0.0005). Activities of daily living, sleep, and skin integrity improved, and pain was eradicated in some. Constipation occurred in six patients. A reduction in dosage was necessitated by muscular hypotonia in three ambulatory patients, areflexic bladder and urinary retention in three others, and nausea, dizziness, and drowsiness in one. Catheter-related problems occurred 19 times in 15 patients. One pump was explanted because of infection in the pump pocket, and one was removed after it eroded through the skin. There were no pump failures. The use of intrathecal baclofen resulted in a decrease in the average length of subsequent hospitalizations. It is concluded that intrathecal baclofen delivered by an implanted programmable pump is a safe, effective, and cost-efficient method for treatment of severe intractable spinal spasticity.
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PMID:Chronic intrathecal delivery of baclofen by a programmable pump for the treatment of severe spasticity. 912 19

A case of acute cerebellar ataxia caused by ECHO virus 30.5-year-old boy admitted to the Clinic of Gastroenterology and Nutrition in Warsaw, in September, 1996, complaining of headache, dizziness, weakness, somnolence, dysarthria and an unsteady walk. On neurological examination he had imparied coordination, rombergism, generalized hypotonia. There was no history of exposure to contagious diseases, ear discharge, convulsions, trauma. Parents suggested that the child could have swallowed an unidentified pill--toxicological tests ruled out poisoning. The diagnosis is based on the clinical examination and amplification ECHO virus from CSF.
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PMID:[Acute cerebellar ataxia in a 5-year-old boy. Clinical warnings]. 974 96

We began this study to determine the efficacy and safety of intrathecal baclofen (ITB) delivered by a programmable pump for the treatment of severe spasticity of spinal cord origin. One hundred fifty two patients with severe spasticity of spinal origin, refractory to oral baclofen, or who experienced intolerable side-effects were given a test dose of ITB. Only those who had a satisfactory response were considered to be appropriate for pump implantation. All but one of the 152 patients had a satisfactory response, and the pump was implanted in 131 patients. Pre- and postoperative spasticity scores were compared and analyzed. The mean Ashworth score for rigidity decreased from 4.2 preoperatively to 1.3 (p < 0.0005) on ITB. The spasm score decreased from a mean of 3.4 to 0.6 (p < 0.0005). Reduction of spasticity resulted in improved levels of physical activity, decreased pain, and augmentation of sleep. Drug-related complications included constipation, muscular hypotonia, urinary retention, erectile dysfunction, nausea, dizziness, drowsiness, hypotension and bradycardia as well as tolerance to baclofen. Some patients experienced post-spinal puncture headaches. Catheter-related problems included occlusions, breaks, punctures, and dislodgments. Superficial pump pocket infection, pocket erosion, cerebrospinal fluid (CSF) leak, post-spinal puncture headache, and meningitis were some of the procedure-related complications. Two pumps flipped and another pump valve was stuck. We conclude that long-term intrathecal baclofen by an implanted programmable pump is a safe and effective method of treating severe intractable spinal spasticity.
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PMID:Continuous intrathecal baclofen infusion by a programmable pump in 131 consecutive patients with severe spasticity of spinal origin. 2215 77

In recent years, there has been an increasing incidence of inflammatory bowel disease in children and adolescents. Neurologic involvement has been mainly reported in adults, and information in pediatrics is based primarily on individual case reports. In this study, we explored the prevalence and spectrum of neurologic manifestations of 50 children with inflammatory bowel disease in comparison to healthy controls. Based on clinical reports and neurologic evaluation, 34 patients (68%) exhibited neurologic manifestations compared with 10 children (23.8%) in the control group (P < .001). The main symptoms associated with inflammatory bowel disease in comparison to the control subjects were headache: 46% vs 3% (P < 0.001), dizziness: 26% vs none (P < .001), hypotonia: 10% vs none (P = .06), attention-deficit hyperactivity disorder (ADHD): 28% vs 7.1% (P < .001), tics and sensory complaints: 16% vs 2.4% (P = .036). Seizures and neuropsychiatric disorders were less characteristic. A larger-scale prospective study is required to further clarify this association.
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PMID:The neurologic profile of children and adolescents with inflammatory bowel disease. 2470 Jun 62

The cerebellum is involved in sensorimotor operations, cognitive tasks and affective processes. Here, we revisit the concept of the cerebellar syndrome in the light of recent advances in our understanding of cerebellar operations. The key symptoms and signs of cerebellar dysfunction, often grouped under the generic term of ataxia, are discussed. Vertigo, dizziness, and imbalance are associated with lesions of the vestibulo-cerebellar, vestibulo-spinal, or cerebellar ocular motor systems. The cerebellum plays a major role in the online to long-term control of eye movements (control of calibration, reduction of eye instability, maintenance of ocular alignment). Ocular instability, nystagmus, saccadic intrusions, impaired smooth pursuit, impaired vestibulo-ocular reflex (VOR), and ocular misalignment are at the core of oculomotor cerebellar deficits. As a motor speech disorder, ataxic dysarthria is highly suggestive of cerebellar pathology. Regarding motor control of limbs, hypotonia, a- or dysdiadochokinesia, dysmetria, grasping deficits and various tremor phenomenologies are observed in cerebellar disorders to varying degrees. There is clear evidence that the cerebellum participates in force perception and proprioceptive sense during active movements. Gait is staggering with a wide base, and tandem gait is very often impaired in cerebellar disorders. In terms of cognitive and affective operations, impairments are found in executive functions, visual-spatial processing, linguistic function, and affective regulation (Schmahmann's syndrome). Nonmotor linguistic deficits including disruption of articulatory and graphomotor planning, language dynamics, verbal fluency, phonological, and semantic word retrieval, expressive and receptive syntax, and various aspects of reading and writing may be impaired after cerebellar damage. The cerebellum is organized into (a) a primary sensorimotor region in the anterior lobe and adjacent part of lobule VI, (b) a second sensorimotor region in lobule VIII, and (c) cognitive and limbic regions located in the posterior lobe (lobule VI, lobule VIIA which includes crus I and crus II, and lobule VIIB). The limbic cerebellum is mainly represented in the posterior vermis. The cortico-ponto-cerebellar and cerebello-thalamo-cortical loops establish close functional connections between the cerebellum and the supratentorial motor, paralimbic and association cortices, and cerebellar symptoms are associated with a disruption of these loops.
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PMID:Consensus Paper: Revisiting the Symptoms and Signs of Cerebellar Syndrome. 2610 56

Activating mutations in AVPR2 are associated with nephrogenic syndrome of inappropriate antidiuresis (NSIAD). NSIAD causes hyponatremia, decreased serum osmolality and clinical symptoms, which may present from birth or in infancy and include hypotonia, irritability, vomiting and/or seizures. Symptoms in later life are often less specific and include malaise, dizziness, confusion, tiredness and headache. NSIAD is a rare X-linked condition, which is associated with a variable phenotype in males, of whom some present in infancy but others do not become symptomatic until adulthood, or occasionally, never. Female carriers may present with episodes of hyponatremia, usually found incidentally. Literature in this field is limited; namely, two clinical reports describing a female proband, both diagnosed in infancy. We describe, for the first time, the case of an adult female proband with NSIAD, who had longstanding associated symptoms of tiredness, headache, temporary memory loss and mood changes as well as hyponatremia and decreased serum osmolality. A water load test demonstrated an inability to dilute urine and gene sequencing confirmed a recurrent activating mutation in AVPR2. The variant was inherited from the proband's mother who had had longstanding episodes of transient asymptomatic hyponatremia. This is the third report of a female proband with NSIAD and is the first female reported who sought medical treatment for chronic symptoms from adulthood. This case acts as a reminder of the importance of considering NSIAD as a diagnosis in females of all ages with unexplained hyponatremia.
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PMID:Adult female with symptomatic AVPR2-related nephrogenic syndrome of inappropriate antidiuresis (NSIAD). 2947 87

The Nipah virus was discovered twenty years ago, and there is considerable information available regarding the specificities surrounding this virus such as transmission, pathogenesis and genome. Belonging to the Henipavirus genus, this virus can cause fever, encephalitis and respiratory disorders. The first cases were reported in Malaysia and Singapore in 1998, when affected individuals presented with severe febrile encephalitis. Since then, much has been identified about this virus. These single-stranded RNA viruses gain entry into target cells via a process known as macropinocytosis. The viral genome is released into the cell cytoplasm via a cascade of processes that involves conformational changes in G and F proteins which allow for attachment of the viral membrane to the cell membrane. In addition to this, the natural reservoirs of this virus have been identified to be fruit bats from the genus Pteropus. Five of the 14 species of bats in Malaysia have been identified as carriers, and this virus affects horses, cats, dogs, pigs and humans. Various mechanisms of transmission have been proposed such as contamination of date palm saps by bat feces and saliva, nosocomial and human-to-human transmissions. Physical contact was identified as the strongest risk factor for developing an infection in the 2004 Faridpur outbreak. Geographically, the virus seems to favor the Indian sub-continent, Indonesia, Southeast Asia, Pakistan, southern China, northern Australia and the Philippines, as demonstrated by the multiple outbreaks in 2001, 2004, 2007, 2012 in Bangladesh, India and Pakistan as well as the initial outbreaks in Malaysia and Singapore. Multiple routes of the viremic spread in the human body have been identified such as the central nervous system (CNS) and respiratory system, while virus levels in the body remain low, detection in the cerebrospinal fluid is comparatively high. The virus follows an incubation period of 4 days to 2 weeks which is followed by the development of symptoms. The primary clinical signs include fever, headache, vomiting and dizziness, while the characteristic symptoms consist of segmental myoclonus, tachycardia, areflexia, hypotonia, abnormal pupillary reflexes and hypertension. The serum neutralization test (SNT) is the gold standard of diagnosis followed by ELISA if SNT cannot be carried out. On the other hand, treatment is supportive since there a lack of effective pharmacological therapy and only one equine vaccine is currently licensed for use. Prevention of outbreaks seems to be a more viable approach until specific therapeutic strategies are devised.
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PMID:Nipah virus - the rising epidemic: a review. 3120 33


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