Cases reported "Muscle Spasticity"

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1/69. Successful intrathecal ethanol block for intractable spasticity of AIDS-related progressive multifocal leukoencephalopathy.

    OBJECTIVE: To study the efficacy of intrathecal ethanol block to relieve intractable spasticity in AIDS-related progressive multifocal leukoencephalopathy (PML) when long-term intrathecal baclofen infusion cannot be used. methods: A 33-year-old man with AIDS-related PML developed very severe spastic paraparesis (Ashworth rigidity score, 4) and painful muscle spasms. The patient was unable to sit in his wheelchair and remained bed bound. Combined oral baclofen and tizanidine at therapeutical doses were used without any effect on the spasticity. The patient refused the placement of an intrathecal catheter for long-term baclofen infusion. A single intrathecal ethanol (6 ml) injection in the L2-L3 intervertebral space with the patient placed in a lateral Trendelenburg (40 C) position was performed. RESULTS: The procedure was very effective in improving the stiffness (Ashworth rigidity score. 2, after the technique) and the muscle spasms disappeared. No side effects during or after the injection were observed. CONCLUSION: Intrathecal ethanol block is a last but very useful choice for treatment of intractable spasticity in PML and other neurologic disorders in AIDS patients when other oral treatments have failed and intrathecal baclofen infusion is not suitable.
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2/69. hypotension following the initiation of tizanidine in a patient treated with an angiotensin converting enzyme inhibitor for chronic hypertension.

    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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keywords = block
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3/69. Radioisotopic control for baclofen pump catheter failure.

    STUDY DESIGN: Case report of baclofen pump catheter failure investigated by radioisotope injection. OBJECTIVES: To report a safe and reliable method for evaluating catheter dysfunction. SETTING: france. methods: Single case report of failure of baclofen pump investigated by radioisotope injection. RESULTS: The injection demonstrated the block in the catheter. The catheter failure was not visualised by plain X-ray nor by filling the pump with radio-opaque solution. CONCLUSION: Catheter failure is a common cause of intrathecal drug delivery problems and may be difficult to diagnose. When catheter disconnection, kink, or dislodgement is not visible on X-ray, radioisotopic control is a safe and reliable method for assessment.
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4/69. friedreich ataxia with minimal GAA expansion presenting as adult-onset spastic ataxia.

    Around a quarter of friedreich ataxia (FA) patients, despite being homozygous for GAA expansion within the FRDA gene, show atypical presentations. Our aim is to describe the case of three brothers with long-term follow-up suffering from late onset FA manifested with spastic ataxia. The three patients belong to a family with occipital dysplasia (OD) and Chiari I malformation previously reported by us. We have carried out serial examinations since 1977. Electrophysiological and neuroimaging studies, and molecular genetic analyses of hereditary ataxias are available in all three patients. Onset of symptoms occurred between 25 and 35 years. The clinical picture consisted of progressive spastic gait, truncal and limb ataxia, dysarthria, nystagmus, hyperreflexia with knee and ankle clonus and extensor plantar response, and mild hypopallesthesia. Ages at present vary between 50 and 59. One patient is wheelchair-bound but the other two are able to walk with support. Leaving OD aside, skeletal anomalies are not prominent. All three patients showed cardiomyopathy. MR imaging revealed atrophy of the cerebellum and spinal cord. Motor and sensory nerve conduction velocities were normal. Central conduction time of both motor and sensory pathways was delayed or unobtainable. All three patients were homozygous for the GAA expansion, the smaller expanded allele ranging between 131 and 156 repeats. Four heterozygotic carriers were detected among non-ataxic relatives including one with OD; furthermore, an asymptomatic OD patient showed normal genotype. We conclude that adult onset spastic ataxia is a distinctive FA phenotype associated with minimal GAA expansion. This phenotype represents a new cause of selective distal degeneration of central sensory axons. The present concurrence of OD and FA reflects coincidental cosegregation of two different inherited disorders.
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ranking = 0.21974744538489
keywords = nerve
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5/69. multiple sclerosis and oral care.

    multiple sclerosis is a complex neurological condition affecting sensory and motor nerve transmission. Its progression and symptoms are unpredictable and vary from person to person as well as over time. Common early symptoms include visual disturbances, facial pain or trigeminal neuralgia and paraesthesia or numbness of feet, legs, hands and arms. These, plus symptoms of spasticity, spasms, tremor, fatigue, depression and progressive disability, impact on the individual's ability to maintain oral health, cope with dental treatment and access dental services. Also, many of the medications used in the symptomatic management of the condition have the potential to cause dry mouth and associated oral disease. There is no cure for multiple sclerosis, and treatment focuses on prevention of disability and maintenance of quality of life. Increasingly a multi-disciplinary team approach is used where the individual, if appropriate his/her carer, and the specialist nurse are key figures. The dental team plays an essential role in ensuring that oral health impacts positively on general health.
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keywords = nerve
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6/69. Report of a Turkish child with Sjoren-Larsson syndrome associated with peripheral nerve involvement.

    Sjoren-Larsson syndrome is a rare hereditary neurocutaneous disorder characterized by ichthyosis, spastic di- or tetra-plegia, and mild to moderate mental retardation. In this article, we present a nine-year-old girl with the classical features of the syndrome associated with peripheral nerve involvement because of its rare presentation. To the best of our knowledge, only three cases of Sjoren-Larsson syndrome with peripheral nerve involvement have been previously reported in the literature. We assume that Sjoren-Larsson syndrome involves extensive disorders of the ectodermal tissues, including the peripheral nerves as well as the skin and the central nervous system.
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ranking = 1.5382321176942
keywords = nerve
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7/69. Breathlessness associated with abdominal spastic contraction in a patient with C4 tetraplegia: a case report.

    A tetraplegic patient with C4 cervical cord injury reported breathlessness during episodes of spastic contraction of the abdominal muscles. To determine the mechanism, we performed electrophysiologic testing of the phrenic nerves. We measured abdominal pressure, esophageal pressure, and transdiaphragmatic pressure (Pdi) during a maximal inspiratory effort (Pdi max), a maximal sniff maneuver (sniff Pdi) during resting breathing, and during the episodes of breathlessness. Electrophysiologic testing of the phrenic nerves showed axonal neuropathy on the left. Sniff Pdi and Pdi max were 38cmH(2)O and 42cmH(2)O, respectively. Transient spastic contractions of abdominal muscles were associated with an increase in abdominal pressure greater than 30cmH(2)O, with a decrease in abdominal volume; this rise in abdominal pressure was transmitted to the esophageal pressure. Inspiration became effective only when esophageal pressure fell below the resting baseline value. Achieving this decrease required an increase in inspiratory effort, characterized by swings in esophageal pressure and Pdi of 30cmH(2)O and 40cmH(2)O (approximately 100% of Pdi max), respectively. During these periods, minute ventilation was markedly reduced. This is the first report that spastic abdominal muscle contractions can impose a significant load on the diaphragm, uncovering moderate diaphragmatic weakness. This has important clinical implications; abolition of the spastic abdominal muscle contraction in this patient completely resolved her intermittent respiratory symptoms.
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ranking = 0.43949489076978
keywords = nerve
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8/69. phenol block for hip flexor muscle spasticity under ultrasonic monitoring.

    Hip flexor spasticity, which is often associated with central nervous system (CNS) diseases, is a major impediment in rehabilitation. In order to cope with this problem, lumbar nerve blocking techniques developed by Meelhuysen and major and minor psoas muscle blocking techniques developed by Awad have been used in combination with physical therapies. Based on these techniques, we conducted major and minor psoas muscle phenol block (motor point block or intramuscular nerve block) under ultrasonic monitoring. phenol block was conducted in nine patients with cerebral infarction (13 blocking procedures) and three with spinal cord injuries (six blocking procedures) while keeping them in a lateral position with the operation side upside. The beginning of the femoral nerves and part of the lumbar artery were visualized by ultrasound in some patients. As a result of the improvement of hip flexor spasticity, the range of hip joint motion (determined by the Mundale technique, prone hip extension and Thomas test) improved shortly after blocking. When physical therapy was conducted after blocking, improvement of skin care management was observed in eight cases, ability to keep in a stable sitting position in nine, improvement of a standing posture in three, increases in the ability to walk in two and alleviation of pain in three. Although nerve block is reported to result in hematoma, decreases in muscle force, pain, cystic/rectal disorders and hypogonadism, we have observed no such complication in our patients.
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ranking = 93.006406716339
keywords = nerve block, nerve, block
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9/69. Implantable baclofen pump as an adjuvant in treatment of pressure sores.

    Currently, the success of ulcer treatment is limited by the high recurrence and complication rates. Spasticity is an important contributing factor to ulcer recurrence, and intrathecal baclofen is an effective method to reduce spasticity. Spasticity creates friction, shear, and mobility impairment resulting in wound dehiscence, flap loss, infection, and hematoma. Spasticity can be managed pharmacologically and surgically; baclofen is the drug of choice. baclofen inhibits spasticity by blocking excitatory neurotransmitters in the spinal dorsal horn. Intrathecal baclofen maximizes the dose delivered to spinal receptors and minimizes the side effects associated with oral baclofen. case reports of intrathecal baclofen used in patients with pressure sores demonstrate the use of intrathecal baclofen to improve reconstructive outcomes in spastic patients.
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10/69. Hereditary spastic dystonia with Leber's hereditary optic neuropathy: neuropathological findings.

    Neuropathological findings in a 59-year-old male case of hereditary spastic dystonia with Leber's hereditary optic atrophy included: marked depletion of myelinated nerve fibres in the posterior funiculi, corticopontine tracts and striatum; practically complete neuronal depletion in the putamen and lateral part of the caudate, and mild cell loss in the substantia nigra. The putamina had changed into a spongy fibrillary scar, the pallidal fibres and laminae were practically all degenerated. Moreover, there was generalised mild fibre degeneration of the white matter. The optic nerve showed marked, predominantly central, loss of nerve fibres with demyelination.
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