Cases reported "Muscle Spasticity"

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1/93. Lessons to be learned: a case study approach: prolonged methaemoglobinaemia due to inadvertent dapsone poisoning; treatment with methylene blue and exchange transfusion.

    The authors present a case of methaemoglobinaemia of acute onset, with an unusually protracted course. The long persistence of this disorder led to a search for the cause which was eventually traced to medication with dapsone. The latter was found to be inappropriately being taken by the patient instead of an antispasmodic that had been prescribed for a spinal condition; this was because the tablets had been incorrectly labelled and dispensed in a pharmacy. The patient took increasing doses of the presumed 'antispasmodic' tablets as they seemed to lack clinical effect, thus further exacerbating the toxic consequences. Moreover, the patient brought his wrongly labelled tablets into hospital and was allowed to use them there, contrary to normal hospital policy. As treatment for the methaemoglobinaemia both bolus and continuous infusions of methylene blue were used, which probably contributed to the severe haemolysis which followed. Furthermore, the development of a rare side effect of dapsone toxicity, namely that of a sensorimotor neuropathy, is reported.
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ranking = 1
keywords = neuropathy
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2/93. 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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ranking = 0.3962651940855
keywords = nervous system
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3/93. Dysesthesia perceived as painful spasticity: A report of 3 cases.

    Lesions of the central nervous system often involve the pyramidal tracts and the sensory pathways to produce spasticity, paresthesias, and dysesthesia. Three patients with intractable spasticity were treated with intrathecal baclofen. Two had an implanted Medtronic SynchroMed pump for long-term delivery of the muscle relaxant. The third patient had undergone a screening trial in which the baclofen was delivered into the intrathecal space through a lumbar catheter. All had excellent relief of spasms on clinical examination, but they reported painful spasms particularly at night. Two of the patients were successfully treated for dysesthesia.
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ranking = 0.3962651940855
keywords = nervous system
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4/93. Eosinophilic pleural effusion due to dantrolene: resolution with steroid therapy.

    A quadriplegic patient with severe spasticity, treated with dantrolene (400 mg daily) for 5 years, had dyspnea, orthopnea, hypoxia, and right-sided opacity of the chest on radiograph. At thoracentesis, an exudative effusion containing 64% eosinophils was documented, with simultaneous peripheral eosinophilia of 11%. An allergic reaction to dantrolene was postulated. Despite withdrawal of the offending medication and repeated thoracenteses, symptomatic recurrence of effusion persisted for 4 days. After institution of prednisone therapy, rapid resolution of symptoms, signs, hypoxia, and radiologic abnormalities was observed. In contrast to five previously reported cases of dantrolene-associated eosinophilic pleural effusion (EPE), ours represents the first in which the patient was treated with steroids and suggests that steroid therapy may be of benefit in drug-related EPE.
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ranking = 2.1808733307309
keywords = peripheral
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5/93. 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 = 0.43789854278856
keywords = nerve
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6/93. Episodic coma in a new leukodystrophy.

    Among the leukodystrophies of a hypomyelinating nature, childhood ataxia with diffuse central nervous system hypomyelination exhibits the unique feature of rapid decrease in mental status after relatively minor head injuries or otherwise noncomplicated febrile illnesses. This article reports the case of a child with progressive spastic quadriparesis in whom unconsciousness developed repeatedly as a result of minor head trauma and required prolonged critical-care nursing. Although cognition is believed to be relatively preserved in this disorder, this child developed progressive cognitive decline. A detailed review of the literature is presented along with discussion of the potential mechanisms of neurologic deterioration.
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ranking = 0.3962651940855
keywords = nervous system
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7/93. Non-drug induced spasticity reduction achieved by coordination dynamic therapy in CNS injury.

    In two patients with severe central nervous system (CNS) lesions it could be demonstrated that spasticity reduction can be achieved by the coordination dynamic therapy method without administration of spasmolytic drugs. The reduction of spasticity in the short- and long-term memory was measured with the coordination dynamic recording method. When performing rhythmic coordinated movements, the mainly extensor spasticity reduced in the first case in the short-term memory within 35 min of assisted exercising by approximately 68% (easy treatable case). In the second case spasticity reduced in the short-term memory within 58 min of exercising by 15 up to 28% and in the long-term memory within 6 months by 16% (difficult treatable case) as judged by the improvement of the coordination dynamics when exercising on the special coordination dynamic therapy device. Reduction of spasticity without administration of drugs could be achieved in all of the more than 100 patients undergoing coordination dynamic therapy.
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ranking = 0.3962651940855
keywords = nervous system
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8/93. 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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ranking = 0.43789854278856
keywords = nerve
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9/93. baclofen withdrawal following removal of an intrathecal baclofen pump despite oral baclofen replacement.

    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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ranking = 0.3962651940855
keywords = nervous system
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10/93. 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 = 66.567697086608
keywords = peripheral nerve, peripheral, nerve, nervous system
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