Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intrathecal administration of baclofen has proved to be an effective treatment of spasticity related to CNS damage. Especially patients with spinal spasticity due to traumatic spinal cord injury or transverse myelitis showed a dramatic reduction of spasticity and improvement of their Ashworth scores. The results are, however, often disappointing in patients with muscular hypertension of the extensor muscles, which is frequently found in patients with multiple sclerosis or cerebral hypoxia. In the latter, using intrathecal baclofen may be restricted by serious side effects. Botulinumtoxin A is widely used in patients with various forms of dystonia. It has also been studied in spastic disorders, where local injections were valuable in relieving focal spasticity in hemiparetic patients and in infantile cerebral palsy. It is used only cautiously in severe paraspasticity. The case reports of 4 patients with incomplete and complete paraparesis due to spinal cord injury, neurodegenerative pyramidal disorder, and cerebral hypoxia demonstrate that a combination of intrathecal baclofen and botulinumtoxin A can improve clinical benefits and reduce side effects.
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PMID:[Optimized therapy of spastic syndrome by combination intrathecal baclofen with botulinum toxin]. 1113 87

Centrally acting alpha-2 adrenergic agonists are one of several pharmacologic agents used in the treatment of spasticity related to disorders of the central nervous system. In addition to their effects on spasticity, certain adverse cardiorespiratory effects have been reported. Adults chronically treated with angiotensin converting enzyme inhibitors may have a limited ability to respond to hypotension when the sympathetic response is simultaneously blocked. The authors present a 10-year-old boy chronically treated with lisinopril, an angiotensin converting enzyme inhibitor, to control hypertension who developed hypotension following the addition of tizanidine, an alpha-2 agonist, for the treatment of spasticity. The possible interaction of tizanidine and other antihypertensive agents should be kept in mind when prescribing therapy to treat either hypertension or spasticity in such patients.
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PMID:Hypotension following the initiation of tizanidine in a patient treated with an angiotensin converting enzyme inhibitor for chronic hypertension. 1119 99

Atypical phenotypes of CADASIL and corresponding anatomical data in two cases are described in 6 members of 2 new French families. In the first family, 4 cases in the same kindred were probably affected, two of them with a predominant psychiatric presentation, two others with dementia and a pseudo-bulbar syndrome of progressive evolution. No history of migraine or ischemic event were documented in any. In the propositus, the diagnosis was documented by skin biopsy, Notch 3 gene mutation and autopsy after the patient had died when 67 years old, 8 years after onset. Brain examination showed a widespread leukoencephalopathy with subcortical infarcts. Characteristic granular lesions of the small arteries of the brain and other organs were observed. In the second family, two cases are reported. One patient died when 63 years old after a subacute evolution mimicking intracranial hypertension. The anatomical diagnosis was retrospectively proven typical of CADASIL with Notch 3 immunostaining of arterial smooth muscle cells. The other case had a progressive evolution over 20 years of limb paresthesia with a mild spasticity diagnosed as a progressive form of multiple sclerosis. It was followed by a pseudo-bulbar syndrome and a mild subcortical dementia without acute ischemic attack. The diagnosis was confirmed by skin biopsy and mutation of the Notch 3 gene. This report illustrates
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PMID:[Atypical CADASIL phenotypes and pathological findings in two new French families]. 1145 85

Intrathecal baclofen is used as a muscle relaxant and antispasmodic in cases of spasticity resulting from central nervous system trauma. The baclofen withdrawal syndrome may include hyperthermia, tachycardia, hypertension, seizures, altered mental status, and psychomotor agitation. We report a case in which the removal of a baclofen pump lead tothe development of severe withdrawal symptoms despite oral baclofen replacement therapy. In order to avoid the development of withdrawal, adequate doses of GABA agonist agents should be administered immediately prior to, and following, baclofen pump removal.
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PMID:Baclofen withdrawal following removal of an intrathecal baclofen pump despite oral baclofen replacement. 1470 58

Baclofen (Lioresal) is a drug of choice to treat spasticity and is increasingly being administered intrathecally via an implantable pump in cases refractory to oral therapy. Emergency physicians will likely treat patients with baclofen withdrawal or overdose as this treatment becomes more widespread. The syndrome of baclofen withdrawal presents with altered mental status, fever, tachycardia, hypertension or hypotension, seizures, and rebound spasticity, and may be fatal if not treated appropriately. Baclofen withdrawal may mimic other diseases including sepsis, meningitis, autonomic dysreflexia, malignant hyperthermia, or neuroleptic malignant syndrome. Treatment consists of supportive care, reinstitution of baclofen, benzodiazepines, and diagnosis and eventual repair of intrathecal pump and catheter malfunction.
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PMID:Intrathecal baclofen withdrawal mimicking sepsis. 1274 45

The spinal cord represents a complex system that serves in the encoding of sensory information and organization of autonomic and somatomotor outflow. As such, it has become a target of investigation for subjects ranging from pain to hypertension to motor spasticity. A primary method in such investigations is the specific delivery of drugs into the spinal intrathecal space to assess such agents with the actions limited to the spinal cord. The rat has served as a primary model in these investigations. It provides for the ability to deliver such agents both acutely and chronically in the absence of anesthesia or restraint. These goals can be accomplished by the placement of intrathecal catheters in the spinal space. This model, first demonstrating the feasibility of routinely catheterizing the lumbar intrathecal space in 1976, led directly to enabling a large number of studies focusing on spinal drug actions. As a test model, it has provided for the acquisition of an exceptional amount of information on the pharmacological and physiological mechanisms of spinal function, drug screening, drug efficacy and safety studies, and pharmacokinteics of spinally delivered drugs. The modified surgical method of intrathecal catheter placement, microinjection drug delivery, and behavioral parameters are described in detail.
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PMID:Intrathecal catheterization and drug delivery in the rat. 1513 33

Pelizaeus-Merzbacher disease is a rare X-linked disease characterized by defective central nervous system myelination owing to a mutation in the proteolipid protein 1 gene. Few studies report detailed clinical findings in children with genetic confirmation of mutations in the proteolipid protein 1 gene. We reviewed the records of 10 boys with Pelizaeus-Merzbacher disease and one symptomatic carrier girl. Their median age was 2 1/2 years (range 10 months to 20 years). Nine had proteolipid protein 1 gene duplications, one had a point mutation, and one had a single codon deletion. The families of eight patients reported perinatal complications, including maternal hypertension (three patients) and meconium aspiration (three patients). All of the patients were social and interactive, but all had difficulty with expressive speech. All patients presented with nystagmus and had hypotonia that progressed to spasticity, affecting the legs more than the arms; ataxia also contributed to motor impairment. Additional problems reported regarded feeding (eight patients) and sleep (three patients). Further work is needed to clarify the variations in disease course and the relationship of genotype to phenotype.
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PMID:Clinical findings in Pelizaeus-Merzbacher disease. 1522 5

Botulinum toxin A (BTX A) has been used for more than 20 years as a safe and effective treatment for numerous diseases characterized by pathological muscle hypertension. In patients suffering from dystonia or spasticity, it has been observed that use of BTX A results not only in muscle relaxation but also frequently relieves associated pain. This pain relief is often seen earlier and to a much greater extent than the muscular relaxation itself. This has led to extending the use of BTX A to treat various focal pain syndromes. The results of initial studies in specific musculoskeletal pain therapy suggest that BTX A infiltrations are effective in the treatment of chronic, therapy-resistant pain of the shoulder and back region. Furthermore, BTX A has been found to be a less invasive option for the treatment of chronic epicondylitis and similar tendonitis conditions. The healing process following rupture of tendons or muscle transfer operations may be improved. In adults with increased muscle tone and endoprostheses, the targeted relaxation of spastic muscles might increase the lifetime of the implant and diminish aseptic loosening. In children with cerebral palsy, prophylactic treatment of hip luxation appears possible. The doses used in pain therapy are low; if correctly applied, the tolerance and safety are high and the effect lasts for a number of weeks.
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PMID:[Botulinum toxin A in orthopedic pain therapy]. 1558 99

Intrathecal baclofen (ITB) therapy is being used increasingly to treat medically intractable spasticity in children with cerebral palsy and spinal cord injuries. Baclofen overdose and withdrawal are potentially life-threatening complications of pump and spinal catheter system malfunction. We report a case of a 12-year-old boy, on long-term ITB therapy, who presents to our emergency department with an overdose of ITB, which is followed by withdrawal symptoms. The patient initially presented obtunded and in respiratory arrest. His symptoms of respiratory arrest, obtundation, fixed pupils, and hypotension mimicked other diagnoses, such as head trauma. The history obtained from the family about the pump reservoir being refilled just before the onset of symptoms led to the diagnosis. During hospitalization, as the patient recovered from the overdose, he began to experience symptoms of baclofen withdrawal, including hypertension, hyperthermia, and hallucinations. The pump was found to be disconnected and was revised. The patient was discharged home without permanent sequelae. With increased use of ITB, emergency medicine physicians must be aware of the mechanics of these pumps and the management of baclofen toxicity and withdrawal.
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PMID:Intrathecal baclofen overdose and withdrawal. 1665 18

Community-based longitudinal study on stroke is rare in India. It has been predicted that the stroke incidence will be higher in developing countries than developed countries. Hence a five years prospective study was planned to carry out in the rural Bengal, India based on WHO protocol to determine the incidence rate, risk factors, morbidity and mortality profile of stroke. In a two-stage procedure, 20717 subjects out of 20842 people from a cluster of 12 villages was surveyed by house-to-house method and the screened cases were examined by a team of neurologists including post stroke surveillance for one year. Altogether 128 first ever stroke cases were detected over 5 years showing an average annual incidence rate of 123.57 per 100,000 persons [age adjusted incidence rate (AAIR)--262/100,000; based on USA population, 1990] and sex-specific AAIR (274/100,000) among women is slightly higher than men (253/100,000). Age-specific stroke incidence rate showed increasing rate from fourth decade up to seventh decade in both sexes when the rate was maximum. First 30 days mortality recorded was 18% with men suffering twice than women. Follow-up after one year revealed speech improvement in 47%, residual spasticity in 46% and independency in activities daily living in 62% of cases. Age and sex matched case control study has shown that hypertension (OR - 2.79), heart disease (OR - 6.20) and smoking (OR - 3.92) are significant risk factors. This study had indicated a higher age adjusted incidence rate of stroke in India as compared to that of developed country and hypertension, heart disease and smoking are important risk factors.
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PMID:A 5 years prospective study of incidence, morbidity and mortality profile of stroke in a rural community of eastern India. 1682 57


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