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)

Toxins are increasingly being used as valuable tools for analysis of cellular physiology, and some are used medicinally for treatment of human diseases. In particular, botulinum toxin, the most poisonous biological substance known, is used for treatment of a myriad of human neuromuscular disorders characterized by involuntary muscle contractions. Since approval of type-A botulinum toxin by the US Food and Drug Administration in December 1989 for three disorders (strabismus, blepharospasm, and hemifacial spasm), the number of indications being treated has increased greatly to include numerous focal dystonias, spasticity, tremors, cosmetic applications, migraine and tension headaches, and other maladies. Many of these diseases were previously refractory to pharmacological and surgical treatments. The remarkable therapeutic utility of botulinum toxin lies in its ability to specifically and potently inhibit involuntary muscle activity for an extended duration. The clostridia produce more protein toxins than any other bacterial genus and are a rich reservoir of toxins for research and medicinal uses. Research is underway to use clostridial toxins or toxin domains for drug delivery, prevention of food poisoning, and the treatment of cancer and other diseases. The remarkable success of botulinum toxin as a therapeutic agent has created a new field of investigation in microbiology.
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PMID:Clostridial toxins as therapeutic agents: benefits of nature's most toxic proteins. 1054 1

Botulinum toxin (BTX), a potent biologic neurotoxin, commonly is associated with lethal outbreaks of food poisoning; however, it also plays a role as a therapeutic agent. Since the 1970s physicians have investigated BTX therapy in patients with neurologic disorders. The number of applications greatly expanded over the years to include certain focal dystonias (blepharospasm, torticollis, laryngeal dystonias, writer's cramp), strabismus, and a wide variety of other indications (gastrointestinal disorders, cosmetic wrinkle correction, spasticity, hyperhidrosis). BTX's safety and efficacy are reviewed.
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PMID:Pharmacotherapy with botulinum toxin: harnessing nature's most potent neurotoxin. 1099 1

Botulinum toxin is more and more frequently used as a therapeutic agent. The toxin blocks selectively and reversibly the neuromuscular junction, causing a muscle relaxation. Indications are mainly muscular hypercontraction, such as dystonia, blepharospasm, focal spasticity, strabismus or tics. The range of action extend to focal hyperhydrosis, palmar, axillary or plantar. It seems now that some painful syndrome such as migraine or tension headache may benefit from toxin injections. Esthetic indications constitute an extension to the pure medical indications.
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PMID:[Current indications for the treatment with botulin toxin]. 1172 14

After a brief review of the pharmacological properties of the botulinum toxin (BT), its mechanism of action on the nerve endings of the neuromuscular junctions, and the general therapeutic principles and adverse side effects, we discuss the advantages of interventional neurophysiology for the treatment of focal motor disorders by means of botulinum toxin A (BTA) muscle infiltration. Electromyography (EMG) provides a valuable objective information in the diagnosis of many motor disturbances and enables the precise identification of the muscles that contribute to the abnormal movement or posture. The use of EMG guidance for BTA injection seems advisable in every muscle but it become indispensable in those difficult to access, deeply located or partially atrophied by previous toxin infiltrations. The EMG study also serves to localise the areas with the highest abnormal activity and the motor point of the muscle, where the injection of toxin exerts its maximal effect. Consequently, lower doses of BTA can be employed without decreasing the efficacy of treatment but reducing the potential risk of side effects, antibody production and the cost of treatment. Electrophysiological diagnosis and BTA treatment may be performed during the same exploration. Considerations on the particular aspects and lines of action are given referring to the main focal muscular hyperactivity motor disorders such as cervical, oromandibular and laryngeal dystonias, blepharospasm, writer's cramp, hemifacial and hemimasticatory spasms, infantile and adult forms of spasticity and some other focal disturbances such as strabismus, detrusor-sphincter dyssynergia and anismus.
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PMID:Botulinum toxin in motor disorders: practical considerations with emphasis on interventional neurophysiology. 1159 29

We present two siblings with pontocerebellar hypoplasia who have progressive microcephaly, mental and motor retardation, truncal ataxia, strabismus, and progressive spasticity and hyperreflexia of the lower limbs. Extrapyramidal dyskinesia and epilepsy, other main clinical features of pontocerebellar hypoplasia, are absent. The older sibling also has a high arched palate, triangular-shaped face, thoracolumbar scoliosis, pectus carinatum, kyphosis, cubitus valgus, arachnodactyly, long extremities, and a tall stature, which were not previously reported in association with pontocerebellar hypoplasia. The clinical phenotype should be expanded, especially within type II, with the reports of additional cases.
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PMID:Pontocerebellar hypoplasia in two siblings with dysmorphic features. 1191 77

To assess the clinical impact of microcephaly among children with developmental disabilities, we reviewed the charts of 1393 consecutive patients from birth to 5 years of age referred to our child development center. Comparisons were made between normal and low IQ microcephalic patients and between children with cerebral palsy with and without small head circumference. Microcephaly was detected in 15.4% of patients. Although mental retardation was more common among microcephalic children (P < .001), almost half had normal intelligence. Prematurity (P < .001), perinatal asphyxia (P < .001), small for gestational age (P < .001), respiratory distress syndrome (P < .001), and brain hemorrhage (P < .001) were associated with microcephaly. Hypotonia (P < .001) and spasticity (P < .001) were the most common neurologic findings. Cerebral palsy (P < .001), growth retardation (P < .001), epilepsy (P < .001), and strabismus (P < .001) were the main associated diagnoses found. Mental retardation was significantly more common among microcephalic patients with cerebral palsy than among normocephalic ones (P < .0004). Microcephaly is common among children evaluated for developmental disabilities. Many of these patients have normal or borderline IQ. Of several perinatal conditions associated with later microcephaly, respiratory distress syndrome and intraventricular hemorrhage show the strongest correlation. Mental retardation is not a risk factor for other neurologic complications in microcephalic children. However, in children with cerebral palsy, microcephaly is a risk factor for mental retardation.
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PMID:Significance of microcephaly among children with developmental disabilities. 1195 71

Oculodentodigital dysplasia (ODDD) (MIM 164200) is a rare autosomal dominant inherited disorder affecting the development of the face, eyes, limbs and dentition. Neurological complications are thought to be occasional manifestations of the disorder. This report illustrates the neurological manifestations by a pedigree of two ODDD patients with spastic paraparesis, cerebral white matter hyperintensity and basal ganglia hypointensity. A systematic review of the English, French, German and Italian literature on ODDD is also provided to summarize the neurological manifestations of the disorder. 243 previously described ODDD cases presented a spectrum of neurological manifestation including spasticity (25), subcortical white matter lesions (9) and basal ganglia changes (6) on MRI. Additional findings consisted of gaze palsy and squinting (28), bladder and bowel disturbances (21), visual loss (20) and blindness (4), hearing loss (15), ataxia (11), nystagmus (9), muscle weakness (5) and paresthesias (3). Neurological manifestations, including spasticity associated with MRI changes, are an underrecognized feature in the ODDD phenotype. A clinical guide to the neurological manifestations of ODDD may assist in the assessment of patients with this condition.
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PMID:Neurological manifestations of the oculodentodigital dysplasia syndrome. 1202 49

Botulinum toxin is a dreaded biological toxin elaborated by Clostridium botulinum. The action of this toxin is to cause paralysis of both voluntary and involuntary muscles. The unique property of paralysing capability of muscles has been used for the benefit of human beings. Dr Allan Scot, an ophthalmologist, first used the toxin in a patient with squint in 1981 and since then the botulinum toxin is being used in various disorders characterised by muscle overactivity such as spasticity in both children and adult, dystonic conditions such as blepharospasm, cervical dystonia, spasmodic dysphonia, writer's cramp, etc, hemifacial spasm and headache. Its main action is at the terminal nerve endings of myoneural junction and it prevents release of acetylcholine from vesicles thus causing chemical denervation. Its action persists for 3 to 4 months on an average. Its side effects such as drooping, diplopia, dysphagia, depending on the sites of injection, are few and usually transient. Generalised anaphylaxis is almost unknown. Now botulinum toxin is being used in non-neurological conditions where muscles are under spasmodic state such as achalasia cardia, anal fissure, spasm of urethral sphincter, etc. Because of wider safety range and fewer complications, botulinum toxin has been an important therapeutic armamentarium in different branches of medicine and surgery.
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PMID:Botulinum toxin: a dreaded toxin for use in human being. 1245 15

Botulinum toxin, the most potent known biological neurotoxin, holds great promise in the therapy of many diseases. It has been used effectively to treat strabismus, dystonias and other movement disorders, and spasticity. However, a number of potential new therapeutic indications have emerged and attracted a considerable amount of interest from the scientific community. These emerging indications included treatment for conditions associated with pain (e.g. headaches, myofascial pain, chronic low back pain), hypersecretion of glands (e.g. hyperhidrosis, sialorrhea, intrinsic rhinitis), and excessive or dyssynergic muscle contraction, and for cosmesis (e.g. myokymia, bruxism, anal fissure). There is a need for more controlled clinical trials, dose-ranging studies to determine optimal treatment, validated clinical scales and studies developed to assess the value of electromyographic guidance and skill of investigators on the outcome of treatment for some of these diseases. The long-term cost effectiveness of treatment and immunoresistance from repeated injections are also important clinical issues to address.
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PMID:Emerging therapeutic applications of botulinum toxin. 1260 5

To get a comprehensive picture of prematurity and neonatal death or survival, all factors on the maternal and fetal records that might be pertinent were recorded. This information, having to do with factors such as age, gravida of the mother, blood group incompatibility, period of gestation, weight at birth, type of delivery, medication and anesthesia administered to the mother, is presented in tabular form. In the study of perinatal mortality it was noted that failure to establish normal pulmonary ventilation is the most common cause of death in the neonatal period. Maternal history of relative infertility, that is, previous abortions, stillbirths and premature deliveries, was the most impressive finding in the stillborn series. In the follow-up study of premature infants who survived the neonatal period, 8.5 per cent were found to be severely handicapped. In four cases the handicap was due to congenital anomalies, in two others probably to infection, leaving 12 with complications possibly ascribable to prematurity. Six of these had retrolental fibroplasia as a major handicap. In seven, mental retardation was the presenting problem. Most of the handicapped children had multiple handicaps, which included spasticity, delayed motor development, strabismus, etc. The incidence of the necessity for corrective measures for feet and legs appeared relatively high. In general, survivors compared favorably with the rest of the childhood population. Complications and twinning were associated inordinately often.
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PMID:A five-year study of prematurity. 1330 92


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