Cerebral Palsy Treatment & Management

The management of patients with cerebral palsy must be individualized based on the child's clinical presentation and requires a multidisciplinary approach (see Consultations). Rehabilitation is a "comprehensive intervention strategy designed to facilitate adaptation to and participation in an increasing number and variety of settings in a particular society and culture."

Neurologists and rehabilitation medicine specialists (physiatrists) play significant roles in the management of antispasticity medications. The physician's responsibility is to closely supervise and manage the multiple medical complications associated with cerebral palsy (see Complications under Prognosis).

Parents frequently inquire about and seek complementary and alternative therapies; however, more research is needed. A randomized, controlled trial to determine whether cranial osteopathy affects the general health and wellbeing of children with cerebral palsy found no evidence that cranial osteopathy leads to sustained improvement in motor function, pain, sleep, or quality of life in children aged 5–12 years.^[29]

In addition, seizure disorders are common in persons with cerebral palsy. Thus, the clinician should be comfortable with the management of anticonvulsant medications (see Antiepileptic Drugs).

Numerous medications, although often used off label for age and indication, may relieve the movement difficulties associated with cerebral palsy. These drugs target spasticity, dystonia, myoclonus, chorea, and athetosis. For example, baclofen, administered either orally or intrathecally, is often used for treating spasticity in these patients.

Botulinum toxin with or without casting

Botulinum toxin (Botox) type A may reduce spasticity for 3-6 months and should be considered for children with cerebral palsy with spasticity in the lower extremities (gastrocnemius, in particular).^{[30, 31, 32, 33, 34]} This therapy can allow for improved range of motion, reduced deformity, improved response to occupational and physical therapy, and delay in the need for surgical management of spasticity. Casting, with or without botulinum toxin type A, may be an additional option for children with an equinus deformity, although the evidence is still somewhat conflicting.^[35]

The established total body dose of botulinum toxin is limited to 12 U/kg, to a maximum of 400 U per visit. (Many practices, however, have been safely using 20 U/kg, to a maximum of 600 U). Each small muscle receives 1-2 U/kg, and large muscles, 4-6 U/kg. The interval between doses should be at least 4 months in order to help prevent antibody formation, which could make subsequent botulinum toxin procedures less effective. Note that large muscles may not respond to this limiting dose, or quite often, patients need several muscles done at each visit.

Phenol intramuscular neurolysis

Historically, phenol intramuscular neurolysis has been considered another medication option. This agent can be used for some large muscles or when several muscles are treated, but phenol therapy is more difficult to administer than other agents. Because phenol is administered using a nerve stimulator, this treatment is more painful, and anesthesia is often used when the therapy is performed. In addition, phenol can, in certain nerves, cause unpleasant sensory dysesthesias, therefore, its use is often limited to nerves with only motor innervation, such as the musculocutaneous (for decreasing arm flexion) and the obturator (for decreasing hip adduction). Phenol treatment is also used for hamstring motor point blocks (for knee flexion).

Antiparkinsonian, anticonvulsant, antidopaminergic, and antidepressant agents

Although antiparkinsonian drugs (eg, anticholinergic and dopaminergic drugs) and antispasticity agents (eg, baclofen) have primarily been used in the management of dystonia, anticonvulsants, antidopaminergic drugs, and antidepressants have also been tried.

Anticonvulsants (including benzodiazepines such as diazepam, valproic acid, and barbiturates) have been useful in the management of myoclonus. Chorea and athetosis are often difficult to manage, although benzodiazepines, neuroleptics, and antiparkinsonian drugs (eg, levodopa) have been tried. Benzodiazepines and baclofen are commonly used to manage spasticity.

Management by spasticity type

A multidisciplinary panel conducted a systematic evaluation of published evidence of efficacy and safety of pharmacologic treatments for childhood spasticity due to cerebral palsy.^[36] The panel members consisted of the Quality Standards Subcommittee of the American Academy of Neurology (AAN), and the Practice Committee of the Child Neurology Society.^[36]

Localized or segmental spasticity

For localized or segmental spasticity, results of the panel found botulinum toxin type A is effective treatment in the upper and lower extremities; however, conflicting evidence exists regarding functional improvement.^[36] Botulinum toxin type A was found to be generally safe in children with cerebral palsy; however, the US Food and Drug Administration (FDA) investigated isolated cases of generalized weakness resulting in poor outcomes and issued a black box warning, as follows:

"Effects of all botulinum toxin products may spread from the area of injection to produce symptoms consistent with botulinum toxin effects. These symptoms may include asthenia, generalized muscle weakness, diplopia, blurred vision, ptosis, dysphagia, dysphonia, dysarthria, urinary incontinence, and breathing difficulties. These symptoms have been reported hours to weeks after injection. Swallowing and breathing difficulties can be life threatening, and death have been reported. The risk of symptoms is probably greatest in children treated for spasticity, but symptoms can also occur in adults treated for spasticity and other conditions, particularly in those patients who have underlying conditions that would predispose them to these symptoms. In unapproved uses, including spasticity in children and adults, and in approved indications, cases of spread of effect have been reported at doses comparable to those used to treat cervical dystonia and at lower doses."

Generalized spasticity

For generalized spasticity, the panel listed diazepam as probably effective and tizanidine as possibly effective, although insufficient data exist regarding motor function and side-effect profile.^[36] The panel recommends diazepam for short-term use, and tizanidine may also be considered as a treatment option. Data were insufficient for use of dantrolene, oral baclofen, and intrathecal baclofen, and toxicity was frequently reported.^[36]

This section will briefly discuss the following:

• Intrathecal baclofen pump insertion
• Selective dorsal rhizotomy
• Stereotactic basal ganglia
• Orthopedic surgical intervention

Intrathecal baclofen pump insertion

Intrathecal insertion of a baclofen pump to treat spasticity and/or dystonia is useful in the patient with diffuse spasticity or dystonia^[37] ; the baclofen pump is most useful in helping to decrease spasticity in the lower extremities and trunk, but it can also reduce spasticity in the upper extremities and improve speech. The pump is placed in the anterior abdominal wall and connects to a catheter inserted in the subarachnoid space overlying the conus of the spinal cord. Intrathecal baclofen can allow more local presynaptic inhibition of I-a sensory afferents and has fewer adverse effects than oral baclofen.

The degree of improvement in the upper extremities is increased with higher placement of the pump catheter. The dose can be adjusted by the physician with an external handheld programmer, with different doses administered during the day and evening, depending on the patient's needs. The patient will need monthly appointments to refill the pump with intrathecal baclofen. The monthly refills are performed in the physician's office, with a single percutaneous needlestick used to access the pump's refill port.

Selective dorsal rhizotomy

Another neurosurgical treatment is that of selective dorsal rhizotomy, which may be beneficial in both the short term^[38] and long term^[39] to treat velocity-dependent spasticity. This procedure includes a laminectomy and then surgical ablation of 70-90% of the dorsal or sensory nerve roots. By cutting I-a sensory fibers, selective dorsal rhizotomy decreases spasticity by decreasing reflexive motoneuron activation, which is thought to result from the lack of descending fiber input.

Gait analysis has revealed improved range of motion at the knee and hip, with improved stride length following selective dorsal rhizotomy. Patients must be selected carefully for this procedure, because the weakness produced may decrease the level of functional independence. Underlying weakness is uncovered with the decrease in spasticity. Some patients also depend on some of their spasticity to stand or walk.

This surgery has come to be performed less frequently since the advent of the baclofen pump. Because of the laminectomies, some of the earlier surgeries had complications of more severe lumbar lordosis several years after surgery. Most surgeons are currently doing smaller laminectomies of only 1-2 levels.

Stereotactic basal ganglia

Although data are limited in this population, stereotactic basal ganglia surgery may improve rigidity, choreoathetosis, and tremor.

Orthopedic surgical intervention

Scoliosis and hip dislocation are the most common conditions requiring surgery. Tendon lengthening or transfer can decrease spastic muscle imbalance and deforming forces, and osteotomy can realign limbs, including the femoral neck, tibia, and calcaneus.

Additionally, reconstructive surgery to the upper extremities can restore muscle balance, release contractures, and stabilize joints to improve placement of the hand in space, as well as voluntary grasp, release, and pinch functions.

Combined use of a continuous-infusion device and oral analgesics has been shown to be more effective than oral medications alone in reducing pain intensity in children with cerebral palsy undergoing lower extremity orthopedic procedures.^[40]

Given that prenatal factors greatly outnumber perinatal and postnatal factors in the origin of cerebral palsy and that prenatal factors are difficult to isolate from perinatal and postnatal factors as a cause for this disorder, determining causality due to intrapartum asphyxia or medical neglect is difficult.

Medicolegal issues were outlined extensively in a 1997 review article by Perlman.^[41] Obstetricians are at risk of malpractice claims because of the association of cerebral palsy with birth asphyxia, even though most cerebral palsy cases are thought to be caused by prenatal insult.

To determine the presence of medical negligence related to birth asphyxia, the following must be documented^[41] :

• An adverse outcome occurred (eg, cerebral palsy as a consequence of intrapartum asphyxia).
• The standard of care was breached during labor or delivery, directly causing the asphyxia.
• An alternative medical strategy more likely than not would have altered the outcome in a positive fashion.

To ascribe the cause of cerebral palsy to intrapartum asphyxia, the following must not be present: (1) clinical evidence indicating any potential antenatal injury, (2) neuroimaging evidence of antenatal cerebral injury, (3) clinical evidence of severe perinatal asphyxia, and (4) evidence of other causes of neonatal encephalopathy.

As previously mentioned, a multidisciplinary team approach is needed in the management of patients with cerebral palsy. Among the specialists who should be consulted are physiatrists; orthopedic surgeons; neurologists and neurosurgeons; geneticists; gastroenterologist, nutritionist, and a feeding and swallowing team; pulmonologists; learning disability team; and other specialists.

Physiatrist

A rehabilitation medicine specialist (physiatrist) should be consulted for the evaluation and management of the rehabilitation program. This specialist can help with many aspects of care, including, but not limited to, those relating to spasticity management, therapies, modalities, bracing, sialorrhea, and insomnia. Physiatrists may also administer intramuscular botulinum toxin type A.

Orthopedic surgeons

An orthopedic surgeon may be needed to help correct any structural deformities and should be consulted for the surgical management of hip dislocation, scoliosis, and spasticity (eg, tenotomy, a tendon-lengthening procedure). Orthopedic surgeons may also administer intramuscular botulinum toxin type A.

Neurologists and neurosurgeons

A neurologist may help with differential diagnosis and with ruling out other neurologic disorders. Consultation with a neurologist may also be helpful in the treatment of patients with seizures.

A neurosurgeon should be consulted for identifying and treating hydrocephalus, a tethered spinal cord, or spasticity. Neurosurgeons perform the dorsal rhizotomy procedure.

Geneticists

A specialist in genetics may help with the differential diagnosis and with ruling out other disorders. For example, a geneticist should be consulted to evaluate for an underlying genetic syndrome, particularly in the setting of dysmorphic features, multiple organ abnormalities, or a family history of a similar neurologic syndrome.

Gastroenterologist, nutritionist, and feeding/swallowing team

The gastroenterologist, nutritionist, and a feeding and swallowing team provide management of feeding and swallowing difficulties and gastroesophageal reflux and assess nutritional status.

A gastroenterologist may help with reflux and constipation and may aid in coordinating feedings to regulate weight gain or loss, if needed. A G-tube or J-tube also may be needed to help augment nutrition.

A periodic nutrition consultation is important to make sure that the child does not suffer from growth failure or nutritional deficiencies.

Pulmonologists

A pulmonologist should be consulted for the management of chronic pulmonary disease due to bronchopulmonary dysplasia and frequent or recurrent aspiration.

Learning disability team

A multidisciplinary learning disability team specializing in children with special needs should be consulted to identify specific learning disabilities, monitor cognitive progression, and guide services through early intervention and school. The child should be evaluated by a communication enhancement center to guide speech and language treatment and the use of communicative devices.

Other specialists

Consultation with an ophthalmologist may be indicated for follow-up of any patient experiencing visual deficits, and an audiologist may help to screen for hearing deficits. In addition, regular dental visits are important. An endocrinologist is occasionally needed for precocious puberty or treatment of osteoporosis.

Multidisciplinary cerebral palsy clinics can allow for the frequent, comprehensive follow-up of children with this disorder while decreasing the need for patient travel. Close neurologic follow-up is required for patients with cerebral palsy.