Cerebral palsy

Dantrolene is the mainstay of MH treatment. It has long been available for the treatment of muscle spasm in cerebral palsy and similar diseases. It is a hydantoin derivative that was first synthesized in 1967, and reported to be effective in the treatment of porcine MH in 1975. Also in 1975, dantrolene was shown to be more effective than procainamide in the treatment of human MH, which until that time was the drug of choice. However, the intravenous preparation was not made available until November 1979. It significantly lowered mortality. The half-life of dantrolene is estimated to be 6-8 hr. Dantrolene s primary mode of action is the reduction in calcium release by the sarcoplasmic reticulum. Dantrolene also exerts a primary antiarrhythmic effect by increasing atrial and ventricular refractory periods. Side effects of dentrolene include hepatotoxicity, muscle weakness, ataxia, blurred vision, slurred speech, nausea, and vomiting. Dantrolene is not contraindicated in pregnancy, but it does cross into breast milk and its effect on the neonate is unknown. 

There are reports of the benefits of botulinum toxin in the treatment of cerebral palsy in children. The toxin, produced by Clostridium botulinum, is a powerful and deadly poison, but is also an effective muscle relaxant. It is not licensed for use as such in the UK but is undergoing clinical trials. Current evidence suggests that repeat injections are necessary some 4-6 months after the first. 

CP Chest pain cerebral palsy EF Ejection fraction... 

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]. 

Dystonia due to identifiable structural or biochemical abnormalities ( secondary dystonia) often occurs weeks or months after strokes or other focal lesions, which commonly involve the basal ganglia, but may also involve the thalamus or cerebellum. Dystonia is also seen in children with cerebral palsy and in patients with abnormalities of dopaminergic transmission. For instance, dystonia may develop in the context of Parkinson s disease, either as an early parkinsonian sign, or in response to dopaminergic drugs. A particularly interesting inherited disease results in a combination of dystonia and parkinsonian features at a young age, which responds dramatically to treatment with low-dose levodopa ( dopamine-responsive dystonia ). 

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... 

For obvious reasons, neurodevelopmental illnesses usually appear at birth or in early childhood. It is during these early years that the connections that form brain circuitry are established. Some of the best-known examples of neurodevelopmental illnesses include cerebral palsy, phenylketonuria, and the autistic disorders. 

An epidemic of intoxication from ingestion of fish contaminated with methyl mercury occurred in the Minamata district in Japan, and, as a result, methyl mercury intoxication is often referred to as Minamata disease." Infants born to mothers with exposure to large amounts of methyl mercury had microen-cephaly, mental retardation, and cerebral palsy with convulsions. In an incidence in Iraq, ingestion of wheat products contaminated with methyl mercury fungicide by pregnant women caused similar symptoms of neurological damage and mental retardation. The fetus is... 

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. 

Toxicity/symptoms nervous system, developmental effects include cerebral palsy-like symptoms with involvement of the visual, sensory, and auditory systems, tingling around lips and mouth, tingling in fingers and toes, vision and hearing loss... 

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. 

And I loved her to bits and I tried everything. I just couldn t understand how she couldn t get involved with her own two weans you know. Especially with Conor the way he is [with cerebral palsy]. 

Uniabeied Uses Treafmenf of bladder spasms, cerebral palsy, infracfable hiccups or pain, Flunfingfon s chorea, frigeminal neuralgia... 

Contraindications Skelefal muscle spasm due fo cerebral palsy, Parkinson disease, rheumafic disorders, CVA, cough, infracfable hiccups, neuropaf hie pain... 

Diazepam 2 mg po 11-year-old with cerebral palsy, mental retardation,posthamstring release With concomitant morphine at 0.1 mg/kg q2h Richtsmeier et al., 1992... 

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. 

Peak blood levels occur within 15 minutes after smoking. The effects last for approximately 4 hours, although it may take more than 24 hours for an individual to return to a normal state. The drugs are stored in fatty tissue and released slowly. PCP has a long half-life ranging from many hours to days, and the PCP glucuronide metabolite can be found in urine for several days or weeks. PCP is found in breast milk. The half-life of ketamine is three to four hours, and metabolites of ketamine are excreted in urine. PCP and ketamine cross the placental barrier, and infants of chronic abusers have been born with cerebral palsy, facial deformities, and behavioral abnormalities. 

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. 

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