Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One male and two female cases in a family of Machado-Joseph disease were reported. Two cases showed typical symptoms that are characterized by bulging eyes, ophthalmoplegia, dystonia, ataxia, spasticity of extremities and amyotrophy, and were consistent with Type II (Rosenberg et al). But another one lacked diversity of the symptoms, showing mainly progressive cerebellar ataxia for over 10 years. We pointed out the existence of a new type of MJD case exhibiting only progressive cerebellar ataxia over a long period. A female patient had dyspnea and insomnia after 20 years in her clinical course, and central sleep apnea was revealed by respiratory monitor. But, the apnea and irregular respiration appeared in both awake and sleep stages. We described the importance of attention to the apnea as a new complication of Machado-Joseph disease.
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PMID:[A family of Machado-Joseph disease with a patient having frequent apnea in all day]. 191 27

Multiple sclerosis patients with motor involvement of the lower extremities and the trunk often experience exertional dyspnea and generalized or leg fatigue on walking, and their walking performance is reduced. It has recently been suggested that a high energy cost of walking (Cw) may be an important contributing factor to the observed dyspnea and fatigue. The purpose of this study was to determine which factors influence Cw. Clinical tests were used to assess the major alterations of the motor system. Thirty-three patients (mean age 41 years, mean maximal speed 2.8 km/h, range 1.2 to 6.2 km/h) in a stable phase of their disease were examined. Cost of walking (mean +/- SE) at 1.8 km/h was 0.287 +/- 0.018 ml 02.kg-1.m-1 (normal value 0.163 +/- 0.007, p less than 0.001). A multivariate regression analysis showed that Cw was significantly related to spasticity of the lower extremities, whereas lower extremity and truncal weakness did not contribute to the observed high Cw.
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PMID:Increased energy cost of walking in multiple sclerosis: effect of spasticity, ataxia, and weakness. 317 52

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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PMID:Eosinophilic pleural effusion due to dantrolene: resolution with steroid therapy. 1137 1

Amyotrophic lateral sclerosis (ALS) is the most common form of degenerative motor neuron disease in adulthood. The clinical picture was accurately described by Charcot over 125 years ago and consists of generalized fasciculations, progressive atrophy and weakness of the skeletal muscles, spasticity and pyramidal tract signs, dysarthria, dysphagia, and dyspnea. Pseudobulbar affect is common. Disease-specific treatment options are still unsatisfactory. However, therapeutic nihilism is not justified as a large array of palliative measures is available to enhance the quality of life of patients and their families. Palliative care in ALS is a multidisciplinary effort requiring careful coordination. An open and frank disclosure of the diagnosis is of paramount importance. Nutritional deficiency due to pronounced dysphagia can be relieved by a percutaneous endoscopic gastrostomy. Respiratory insufficiency can be effectively treated by noninvasive home mechanical ventilation. The terminal phase of the disease should be discussed, at the latest, when symptoms of dyspnea appear in order to prevent unwarranted fears of "choking to death." Collaboration with hospice and completion of advance directives can be of invaluable help in the terminal phase.
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PMID:Clinical characteristics and management of ALS. 1144 24

Amyotrophic lateral sclerosis (ALS) is the most common degenerative motor neuron disease in adults. The clinical picture consists of generalized fasciculations, progressive atrophy and weakness of the skeletal muscles, spasticity and pyramidal tract signs, dysarthria, dysphagia, and dyspnea. Pseudobulbar affect is common. Disease-specific treatment options are still unsatisfactory. Therapeutic nihilism is not justified as a large array of palliative measures available to enhance the quality of life of patients and their families. Because of its clinical characteristics, ALS represents a paradigm for palliative care in neurological diseases. Numerous projects are being undertaken worldwide in an effort to enlarge the evidence base for palliative interventions in ALS. Palliative care in ALS is a multidisciplinary effort requiring careful coordination. An open and empathic disclosure of the diagnosis is essential. Nutritional deficiency caused by dysphagia can be relieved by a percutaneous endoscopic gastrostomy. Respiratory insufficiency can be effectively treated by non-invasive home mechanical ventilation. The terminal phase of the disease should be discussed at the latest when symptoms of dyspnea appear, in order to prevent unwarranted fears of "choking to death." Psychological and spiritual care of patients and families are important. Collaboration with hospice institutions and completion of advance directives can be of invaluable help in the terminal phase.
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PMID:Palliative care in amyotrophic lateral sclerosis. 1185 2

Patients with amyotrophic lateral sclerosis (ALS) have symptoms of progressive muscle weakness, of disturbed speech and swallowing, and in the terminal phase those of respiratory weakness. Treatment options, in particular those for excessive weight loss and respiratory weakness, should be introduced to the patients and their families when the patient is emotionally capable and before dysarthria severely hampers communication. Special equipment for keeping the patient as mobile as possible should be made available much earlier than in the case of other diseases of the muscles as in ALS progression is much faster. Cramps, pathological crying or laughter, spasms, and spasticity can all be treated by medication. When speech can no longer be understood, adaptive strategies such as sign language, mime, posture and communication apparatus varying from a note pad to advanced computer systems can be used. Sialorrhoea, caused by difficulty swallowing with its accompanying danger of aspiration can be halted by the use of medication, by radiotherapy and by the injection into the salivary glands of botulin A toxin. Weight loss, also a result of dysphagia, can be avoided by eating frequent small meals or if necessary performing a percutaneous endoscopic or radiological gastroscopy. Excess mucus in the respiratory tract can be treated with anticholinergics. Difficulty in coughing up thick and sticky mucus cannot always be adequately helped. Respiratory weakness is treatable by external respiratory supportive therapy using a nasal mask, as well as invasive respiratory support via a trachcostoma and by treating the symptoms of respiratory weakness. The latter form of treatment is palliative and forms part of terminal care. During the terminal phase restlessness, anxiety, pain, and dyspnoea require the most attention. Treatment requires careful multidisciplinary cooperation.
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PMID:[The symptomatic treatment of amyotrophic lateral sclerosis]. 1519 69

We report on a patient with an incomplete tetraplegia below C2 who suffered from a post-traumatic abdominal spasticity, spasticity of the legs, and bladder contractions of high intensity. Breathing was possible during the day using accessory respiratory musculature. All standard therapeutic regimes against spasticity failed. Treatment was started with delta-9-tetrahydrocannabinol administered orally in a dosage of 2 x 2.5 mg/day. The spasticity of the legs and the bladder improved with the treatment. After 3 days, the patient complained about dyspnea and shortness of breath. Treatment with delta-9-tetrahydrocannabinol was discontinued after 5 days but the patient needed ventilatory support for 1 week. After 1 week, spontaneous breathing was possible again. The reasons for respiratory failure in endangered patients during treatment with delta-9-tetrahydrocannabinol could be effects such as sedation, combined treatment with baclofen, muscle weakness, or central nervous effects in the medulla oblongata.
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PMID:[Respiratory failure due to delta-9-tetrahydrocannabinol in a tetraplegic patient]. 1654 Dec 67

Cervicothoracic kyphotic deformity may inhibit horizontal gaze function, impede activities of daily living, and induce disabling pain. Eventually, some patients develop a chin-on-chest deformity that limits their ability to eat and drink; in the end stage, a few patients also may have difficulty breathing. Progressive kyphosis can stretch the spinal cord leading to myelopathy with progressive lower extremity spasticity and weakness. Indications for surgery include myelopathy, pain, dysphagia or dyspnea owing to kyphosis, and difficulty maintaining a functional horizontal gaze. Patients with unstable cervicothoracic fractures also require surgical fixation. For these patients, surgical goals include deformity correction with restoration of an acceptable forward gaze, re-establishment of sagittal balance, decompression of the spinal cord (if myelopathic), and stable fixation.
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PMID:Cervicothoracic kyphosis. 1687 28

Progressive infantile spastic tetraparesis spans a wide spectrum of partially rare differential diagnoses. Based on a clinical example the differential diagnostic thoughts are discussed in detail. Though juvenile motor neuron disease is a rare entity, it has to be kept in mind for differential diagnostics in cases of slowly progressive spastic tetraparesis, especially when a pseudobulbar palsy or distal amyotrophies add to the clinical picture. Electromyography can be helpful for early detection of lower motor neuron involvement. The glutamate antagonist riluzole slows the disease progression, but a causal treatment is not available, yet. Therefore symptomatic treatment of disturbing symptoms like muscle cramps, spasticity, pseudobulbar affect, dyspnea or dysphagia are of major interest.
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PMID:Differential diagnosis in progressive infantile spastic tetraparesis. 1900 70

This case report reviews the anesthesiological complications of intrathecal baclofen (ITB) therapy. An 11-year-old boy with spasticity and apallic syndrome needed general anesthesia for exchange of a baclofen pump but 2 h later he became increasingly hypothermic, hypotonic with bradycardy and dyspnea. The cause was an intra-operative bolus of ITB. Reduction of the baclofen administration rate caused disappearance of all symptoms without any residual effects. The ITB is an increasingly used therapeutic option for multiple sclerosis and cerebral palsy. Therefore, emergency personal and anesthesiologists must be aware of the possible side effects of this medication.
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PMID:[Intrathecal baclofen therapy. Overdose during replacement of a medication pump]. 1973 Jul 95


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