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)

Theories on "Coping" try to explain which resources people use to master crises and conflict situations. Reactions to challenges of life are considered as "coping strategies". In psychosomatic research, the notion of "coping" serves to describe how patients can handle physical or mental illness. Myatrophic (or amyothrophic) lateral sclerosis (ALS) is a rare chronic progressive disease of the nervous system with a gradual loss of motor neurons, resulting in muscular atrophy, weakness and spasticity. Since the etiology is unknown and no curative treatment available, most patients die from respiratory failure within a few years. In a follow-up study on physical disability, medical care and social support in 21 patients with advanced ALS, we tried to address the question how patients get along with such a threatening condition. After semi-standardized clinical interviews with patients and close relatives, two investigators gave independent descriptions of reported or observed "coping strategies" (rated as "not at all-barely-possibly-probably-very probably present"). Considering 17 patients assessed by both raters, significant agreement (Kendall's W) was achieved in three patients only. A comparison of aggregated answers (chi 2-Test) revealed different response sets, since one rater tended to choose extreme scale points, while the other preferred undecided answers. Differences of mean scores were observed in 8 out of 21 items, while significant correlations between investigators were obtained in another 8, including, though, only 2 of those 5 items which both had rated as "probably present" in no less than 50% of the patient sample ("Dejection & Flight-Brooding-Keeping the Situation Open-Distraction-Self-Isolation").(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Assessment of "coping with disease" in patients with amyotrophic lateral sclerosis (ALS): on the use of an interviewer assessment rating scale]. 223 71

We present a case history of a patient with definite multiple sclerosis who developed an abrupt onset of unilateral diaphragmatic paralysis, minor increase in lower extremity spasticity and complaint of marked neck stiffness. Her vital capacity during this episode was 600 mL and she was in impending respiratory failure. The diaphragmatic paralysis was demonstrated by radiographic plain films and fluoroscopy. Phrenic nerve stimulation was performed during fluoroscopy and the evoked motor response from the diaphragm recorded. There was a normal amplitude diaphragmatic twitch observed with an evoked motor response latency of 1 ms and amplitude of 300 microV. After high dose intravenous steroids, her neck stiffness and spasticity improved, her vital capacity improved to 1500 mL and her diaphragm regained its normal position and movement confirmed by followup radiographic plain films and fluoroscopy. We postulate the presence of a demyelinating plaque in the brainstem fibers descending to the phrenic nucleus as the etiology of the diaphragmatic paralysis. We are unaware of any other case reports of unilateral "upper motor neuron" phrenic nerve paralysis secondary to multiple sclerosis.
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PMID:Complete hemidiaphragmatic paralysis in a patient with multiple sclerosis. 340 62

Presented here is a 17-month-old adenosine deaminase-deficient, severe combined immunodeficient patient with chest symptoms, oral ulcer, neurologic manifestations, head lag, spasticity and developmental delay in motor functions. Antibiotics, systemic antifungal agents, intravenous immunoglobulins and partial exchange transfusions with irradiated fresh red cells were given. No other mode of therapy for adenosine deaminase (ADA) deficiency was available at that time. Amelioration of neurologic manifestations within one month of therapy with irradiated fresh red cell exchange transfusions suggests that these manifestations may have resulted from accumulated toxic metabolites. However, no improvement was seen in the course of infection and oral ulcer, and the patient died of respiratory failure on the 48th day of admission.
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PMID:A case of adenosine deaminase-negative severe combined immunodeficiency with neurological abnormalities. 856 Jun 6

Amyotrophic lateral sclerosis (ALS) is a motor neuron disease with evidence of both anterior horn cell and corticospinal tract degeneration. The incidence of ALS is 1 to 2.5 cases per 100,000 population and the disease occurs primarily in adult life. The etiology of sporadic ALS remains unknown, although 5 to 10% of cases are familial. The diagnosis of ALS requires the presence of both upper and lower motor neuron findings and progressive motor dysfunction. Several theories regarding the pathogenesis of ALS have emerged including glutamate excitotoxicity, free radical oxidative stress, neurofilament accumulation, and autoimmunity. Clinical trials involving antiglutamate agents, antioxidants, immunosuppressants, and growth factors have shown no substantial benefit in slowing progression, with death usually occurring 2 to 5 years following the onset of symptoms. The management of ALS patients requires a multidisciplinary team that can provide the numerous medical and physical interventions necessary to treat weakness and fatigue, bulbar dysfunction, spasticity and pain, depression, and respiratory failure.
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PMID:Amyotrophic lateral sclerosis. 956 65

There is nothing more discouraging than for a patient to be given a specific diagnosis, then to be told that there is nothing that can be done. Physicians are equally disheartened to see exponential progress being made in the understanding of the pathophysiology of a complex disorder but few direct benefits resulting for their patients. Over the past 5 years, molecular genetic research has completely revolutionized the way in which the progressive cerebellar ataxias are classified and diagnosed, but it has yet to produce effective gene-based, neuroprotective, or neurorestorative therapies. The treatment of cerebellar ataxia remains primarily a neurorehabilitation challenge, employing physical, occupational, speech, and swallowing therapy; adaptive equipment; driver safety training; and nutritional counseling. Modest additional gains are seen with the use of medications that can improve imbalance, incoordination, or dysarthria (amantadine, buspirone, acetazolamide); cerebellar tremor (clonazepam, propranolol); and cerebellar or central vestibular nystagmus (gabapentin, baclofen, clonazepam). Many of the progressive cerebellar syndromes have associated features involving other neurologic systems (eg, spasticity, dystonia or rigidity, resting or rubral tremor, chorea, motor unit weakness or fatigue, autonomic dysfunction, peripheral or posterior column sensory loss, neuropathic pain or cramping, double vision, vision and hearing loss, dementia, and bowel, bladder, and sexual dysfunction), which can impede the treatment of the ataxic symptoms or can worsen with the use of certain drugs. Treatment of the associated features themselves may in turn worsen the ataxia either directly (as side effects of medication) or indirectly (eg, relaxation of lower limb spasticity that was acting as a stabilizer for an ataxic gait). Secondary complications of progressive ataxia can include deconditioning or immobility, weight loss or gain, skin breakdown, recurrent pulmonary and urinary tract infections, aspiration, occult respiratory failure, and obstructive sleep apnea, all of which can be life threatening. Depression in the patient and family members is common. Although no cures exist for most of the causes of cerebellar ataxia and there are as yet no proven ways to protect neurons from premature cell death or to restore neuronal populations that have been lost, symptomatic treatment can greatly improve the quality of life of these patients and prevent complications that could hasten death. Supportive interventions should always be offered-- education about the disease itself, genetic counseling, individual and family counseling, referral to support groups and advocacy groups, and guidance to online resources. Misinformation, fear, depression, hopelessness, isolation, and financial and interpersonal stress can often cause more harm to the patient and caregiver than the ataxia itself.
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PMID:Cerebellar Ataxia. 1109 49

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neuromuscular disease that destroys both upper and lower motor neurons, resulting in spasticity, diffuse muscular atrophy, weakness, and ultimately death from respiratory failure. It is presumed that in the vast majority of cases, ALS is acquired and occurs sporadically, although the exact etiology is unknown. Recent, emerging evidence suggests that neuro-inflammation may be a pathological characteristic of this disease; this could represent a potential therapeutic target for a pharmacological agent to help treat this severe disease. This article reviews the current research in this area and discusses theoretical and clinical ramifications of these recent findings.
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PMID:Neuro-inflammation as a therapeutic target in amyotrophic lateral sclerosis. 1252 5

Amyotrophic lateral sclerosis (ALS) produces progressive weakness, muscular wasting, and spasticity leading to death from respiratory failure at a median of 3 years after onset. ALS and frontotemporal dementia (FTD) overlap in both familial and sporadic cases of ALS. When both occur in families, the affected members may have only ALS, only FTD, or both. This suggests a relationship in the cause of these disorders. We investigated the frequency of ALS in FTD patients and of FTD in ALS patients and found the overlap to be more common than had previously been reported. We report the features of cognitive impairment and pattern of motor involvement in these ALS-FTD patients, and the degree of overlap in both populations.
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PMID:Characterization of amyotrophic lateral sclerosis and frontotemporal dementia. 1517 49

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

The case of a term, male neonate (birthweight 3785g) with cephalic presentation, Caesarean-section (C-section) delivery, and failure to thrive is reported. The infant presented with generalized hypotonia and respiratory failure immediately following birth. An initial diagnosis of hypoxic-ischemic encephalopathy was made. However, ventilator dependency and slow recovery of generalized tonus over the following weeks could not be explained. Late cervical magnetic resonance imaging showed extensive syringomyelia from C2 to C7. To the authors' knowledge, this is the first report of syringomyelia after a C-section delivery following cephalic presentation without any associated abnormalities. Follow-up at 2 years of age revealed no improvement on neurological examination: poor head control, difficulty swallowing, flaccid paralysis of upper limbs, and spasticity of lower limbs with exacerbated deep reflexes and spontaneous clonus. Difficulties in establishing the diagnosis and managing the case are discussed.
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PMID:Syringomyelia and chronic respiratory failure in a term infant delivered by Caesarean section. 1759 28

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by progressive muscular paralysis reflecting degeneration of motor neurones in the primary motor cortex, corticospinal tracts, brainstem and spinal cord. Incidence (average 1.89 per 100,000/year) and prevalence (average 5.2 per 100,000) are relatively uniform in Western countries, although foci of higher frequency occur in the Western Pacific. The mean age of onset for sporadic ALS is about 60 years. Overall, there is a slight male prevalence (M:F ratio approximately 1.5:1). Approximately two thirds of patients with typical ALS have a spinal form of the disease (limb onset) and present with symptoms related to focal muscle weakness and wasting, where the symptoms may start either distally or proximally in the upper and lower limbs. Gradually, spasticity may develop in the weakened atrophic limbs, affecting manual dexterity and gait. Patients with bulbar onset ALS usually present with dysarthria and dysphagia for solid or liquids, and limbs symptoms can develop almost simultaneously with bulbar symptoms, and in the vast majority of cases will occur within 1-2 years. Paralysis is progressive and leads to death due to respiratory failure within 2-3 years for bulbar onset cases and 3-5 years for limb onset ALS cases. Most ALS cases are sporadic but 5-10% of cases are familial, and of these 20% have a mutation of the SOD1 gene and about 2-5% have mutations of the TARDBP (TDP-43) gene. Two percent of apparently sporadic patients have SOD1 mutations, and TARDBP mutations also occur in sporadic cases. The diagnosis is based on clinical history, examination, electromyography, and exclusion of 'ALS-mimics' (e.g. cervical spondylotic myelopathies, multifocal motor neuropathy, Kennedy's disease) by appropriate investigations. The pathological hallmarks comprise loss of motor neurones with intraneuronal ubiquitin-immunoreactive inclusions in upper motor neurones and TDP-43 immunoreactive inclusions in degenerating lower motor neurones. Signs of upper motor neurone and lower motor neurone damage not explained by any other disease process are suggestive of ALS. The management of ALS is supportive, palliative, and multidisciplinary. Non-invasive ventilation prolongs survival and improves quality of life. Riluzole is the only drug that has been shown to extend survival.
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PMID:Amyotrophic lateral sclerosis. 1919 1


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