Spastic cerebral palsy

In many cells, the capacity for de novo synthesis to supply purines and pyrimidines is insufficient, and the salvage pathway is essential for adequate nucleotide synthesis. In patients with Lesch-Nyhan disease, an enzyme for purine salvage (hypoxanthine guanine phosphoribosyl pyrophosphate transferase, HPRT) is absent. People with this genetic deficiency have CNS deterioration, mental retardation, and spastic cerebral palsy associated with compulsive self-mutilation, Cells in the basal ganglia of the brain (fine motor control) normally have very high HPRT activity. These patients also all have hyperuricemia because purines cannot be salvaged. 

Spastic cerebral palsy with compulsive biting of hands and lips... 

Berweck S, Heinen F. Use of botulinum toxin in pediatric spasticity (cerebral palsy). Mov Disord. 2004 (Suppl 8)19 S162-S167. 

Wasiak J, Hoare B, Wallen M. Botulinum toxin A as an adjunct to treatment in the management of the upper limb in children with spastic cerebral palsy. Cochrane Database Syst Rev. 2004 CD003469. 

Mathew A, Mathew MC. Bedtime diazepam enhances well being in children with spastic cerebral palsy. Pediatr Rehabil 2005 8(l) 63-6. 

Denhoff E, Feldman S, Smith MG, Litchman H, Holden W. Treatment of spastic cerebral-palsied children with sodium dantrolene. Dev Med Child Neurol 1975 17(6) 736-42. 

Chemoneurectomy with aqueous phenol injection in 116 selected patients with spastic cerebral palsy, in whom 246 peripheral nerves were blocked, caused complications in 11 patients (28). Five patients, in whom the posterior tibial nerve was blocked, developed paresthesia one had complete loss of sensation, which recovered spontaneously after a couple of days and three had pain at the site of injection or in the distribution of the injected nerve, lasting for a few days to a month. In another study there was a 3% complication rate in 98 blocks (29), while adverse effects occurred in nine of 150 blocks, with muscle weakness in eight cases and painful paresthesia in one (30). 

In zooo, the prevalence of cerebral palsy in metropolitan Adanta was 3.1 per 1000 8-year-olds. Of 10-year-old children, 23 of every 10,000 had cerebral palsy. Eighty-one percent of these children had spastic cerebral palsy. Seventy-five percent had one or more other disabilities (epilepsy, mental retardation, hearing loss, or vision impairment).7 Like many neurological and neuropsychiatric disorders, a diagnosis of cerebral palsy is a description of symptoms, not an identification of causes. 

Simon SR, Deutsch SD, Nuzzo RM, Mansour MJ, Jackson JL, Koskinen M, Rosenthal RK. Genu recurvatum in spastic cerebral palsy. J of Bone and Joint Surgery 1978 7 882-894... 

In a prospective study of intrathecal baclofen in 17 children with spastic cerebral palsy, there were 80 adverse events eight of which were serious but not life-threatening [38 ]. 

Hoving MA, van Raak EP, Spincemaille GH, van Kranen-Mastenbroek VH, van Kleef M, Gorier JW, Vies JS Dutch Study Group on Child Spasticity. Safety and one-year efficacy of intrathecal baclofen therapy in children with intractable spastic cerebral palsy. Eur J Paediatr Neurol 2009 13(3) 247-56. 

Botulinum exotoxin impedes release of neurotransmitter vesicles from cholinergic terminals at neuromuscular junctions. Botulinum exotoxin is ingested with food or, in infants, synthesized in situ by anaerobic bacteria that colonize the gut. A characteristic feature of botulinum paralysis is that the maximal force of muscle contraction increases when motor nerve electrical stimulation is repeated at low frequency, a phenomenon attributable to the recruitment of additional cholinergic vesicles with repetitive depolarization of neuromuscular presynaptic terminals. Local administration of Clostridium botulinum exotoxin is now in vogue for its cosmetic effects and is used for relief of spasticity in dystonia and cerebral palsy [21]. 

Dantrolene is a drug that causes spastic muscle contraction. Unlike other muscle relaxants, it has a direct effect on the contractile mechanism by interfering in the process of calcium ion release from the sarcoplasmic reticulum. This results in a lack of coordination in the mechanism of excitation—contraction of skeletal muscle, which has a greater effect on fast muscle fibers than on slow muscle fibers. Dantrolene is used for controlling the onset of clinical spasticity resulting from serious clinical cases such as wounds, paralysis, cerebral palsy, and disseminated sclerosis. Synonyms of this drug are dantrium and danlen. 

Intrathecal-25, 50, or 100 meg appears to be beneficial in children for reducing spasticity in cerebral palsy. 

Spasticity Cyclobenzaprine is not effective in the treatment of spasticity associated with cerebral or spinal cord disease, or in children with cerebral palsy. 

Oral - For the control of clinical spasticity resulting from upper motor neuron disorders such as spinal cord injury, stroke, cerebral palsy, or multiple sclerosis. It is of particular benefit to the patient whose functional rehabilitation has been retarded by the sequelae of spasticity. Such patients must have presumably reversible spasticity where relief of spasticity will aid in restoring residual function. 

Benzodiazepines have the capacity to depress polysynaptic reflexes and have been shown to decrease decerebrate rigidity in cats and spasticity in patients with cerebral palsy. What is not clear is whether they can, in humans, relax voluntary muscles in doses that do not cause considerable central nervous system depression. Nevertheless, benzodiazepines, such as diazepam, are often prescribed for patients who have muscle spasms and pain as a result of injury. In these circumstances, the sedative and anxiolytic properties of the drug also may promote relaxation and relieve tension associated with the condition. 

It is an orally active muscle relaxant and is used in treating the spasticity and muscle rigidity of spinal cord injuries, cerebral palsy, and other related neurologic disorders. 

It is indicated as hypnotic, in anxiety, tension, muscle spasm, psychosomatic and behaviour disorders, dysmenorrhoea, cerebral palsy, upper motor neuron spasticity, sedative for surgical procedures, labour, tetanus, eclampsia and epilepsy. 

It is indicated in spasticity due to neurological disorders e.g., multiple sclerosis, chronic myelopathy, degenerative diseases of the spinal cord, cerebrovascular accidents and cerebral palsy painful muscle spasm associated with static and functional disorders of the spine (cervical and lumbar syndromes) painful muscle spasm following surgery e.g., for herniated intervertebral disc or for osteoarthritis of the hip. 

The therapeutic use of botulinum toxin for ophthalmic purposes and for local muscle spasm was mentioned in Chapter 6. Local facial injections of botulinum toxin are widely used for the short-term treatment (1-3 months per treatment) of wrinkles associated with aging around the eyes and mouth. Local injection of botulinum toxin has also become a useful treatment for generalized spastic disorders (eg, cerebral palsy). Most clinical studies to date have involved administration in one or two limbs, and the benefits appear to persist for weeks to several months after a single treatment. Most studies have used type A botulinum toxin, but type is also available. 

Baclofen GABAb agonist, facilitates spinal inhibition of motor neurons Pre- and postsynaptic inhibition of motor output Severe spasticity due to cerebral palsy, mulitple sclerosis, stroke Oral, intrathecal Toxicities Sedation, weakness... 

Ronan S, Gold JT Nonoperative management of spasticity in children. Childs Nerv Syst 2007 23 943 [PMID 17646995] Verrotti A et al Pharmacotherapy of spasticity in children with cerebral palsy. Pediatr Neurol 2006 34 1. [PMID 16376270] Ward AB Spasticity treatment with botulinum toxins. J Neural Transm 2008 115 607. [PMID 18389166]... 

Uses. Diazepam is used in patients with spasticity resulting from cord lesions and is sometimes effective in patients with cerebral palsy. 

Botulinum toxin injection has been documented as a means to control severe spasticity in various clinical situations. This intervention, for example, can help remove spastic dominance in certain patients so that volitional motor function can be facilitated. For example, judicious administration of botulinum toxin can result in improved gait and other functional activities in selected patients with cerebral palsy, stroke, or traumatic brain injury.7,36,49,78 Even if voluntary motor function is not improved dramatically, reducing spasticity in severely affected muscles may produce other musculoskeletal benefits. For example, injection of botulinum toxin can reduce spasticity so that muscles can be stretched or casted more effectively, thus helping to prevent joint contractures and decreasing the need for surgical procedures such as heel-cord lengthening and adductor release.12,98... 

Botulinum toxin A complexed with haemagglutinin is currently employed medicinally to counter involuntary facial muscle spasms, e.g. around the eye. Very small (nanogram) amounts are injected locally and result in the destruction of the acetylcholine release mechanism at the neuromuscular junction. Since new nerve junctions will gradually be formed over two months or so, the result is not permanent, and the treatment will need to be repeated. It has also been found useful in easing muscle spasticity in children with cerebral palsy. 

Benzodiazepines are used to treat insomnia and for the acute treatment of epileptic seizures, convulsive disorders, and spastic disorders such as cerebral palsy,... 

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