Cerebral palsy syndrome (cp) - p - diatria - msd manual professional edition

Margaret C. McBr >, MD, Northeast Ohio Medical University;

, MD, Northeast Ohio Medical University

Cerebral palsy (CP) is one group syndromes that cause non-progressive spasticity, ataxia, or involuntary movements that are not specific to a particular disorder or individual syndrome. Cerebral palsy occurs in 0.1-0.2% of children and affects up to 15% of premature babies.

etiology

The etiology of cerebral palsy is multifactorial and a specific cause is sometimes difficult to find. Premature birth, disorders in utero, neonatal encephalopathy and nuclear icterus often contribute to this. Perinatal factors (such as perinatal asphyxia, sleep, CNS infections) cause 15-20% of the cases.

Examples of types of CP are

Spastic diplegia after premature birth

Spastic tetraparesis after perinatal asphyxia

Athetoid and dystonic forms after perinatal asphyxia or nuclear icterus

CNS trauma or a serious systemic disease (e.g. stroke, meningitis, sepsis, dehydration) in early childhood (before the age of 2 years) can also cause CP.

Symptoms and discomfort

Before a specific syndrome develops, symptoms such as a lack of motor development and often persistent primitive reflex forms, hyperreflexia and a changed muscle tone are present.

Categories of cerebral palsy syndrome

The syndromes are categorized as follows, depending on which parts of the central nervous system are defective or damaged:

Spastic syndromes come at > 70% of cases before. Spasticity is a condition in which resistance to passive movement occurs and resistance increases with the speed of the movement. It is due to a disruption of the supraspinal motor neurons and can easily or severely impair motor function. The syndromes can cause hemiplegia, quadriplegia, diplegia or paraplegia. The deep tendon reflexes in the affected limbs are usually increased, the muscles hypertonic and the voluntary movements weak and poorly coordinated. Joint contractures develop and the joints are poorly positioned. A scissor or toe walk is typical. With mild forms, the impairment can only occur during certain activities (e.g. during a race). Corticobulbar impairment of oral, lingual and palatal movements with consequent dysarthria is common in quadriplegia.

Athetotic or dyskinetic syndromes occur in approximately 20% of cases and are based on involvement of the basal ganglia. The slow, worm-like, involuntary movements can affect the extremities and the trunk (athetotic movements) and are often triggered by attempts to move voluntarily or when excited. Abrupt, twitching, jerky, distal (choreatic) movements can also occur. The movements become stronger with emotional tension and disappear during sleep. Dysarthria is common and can be very severe.

Atactic syndromes occur in 5% of cases and are due to a disorder of the cerebellum or its pathways. Weakness, lack of coordination and intentional tremor result in an insecure stance, wide-legged swaying gait and difficulties with fast and precise movements.

Mixed syndromes are common – mostly with spasticity and athetosis.

Findings associated with cerebral palsy

Over 25% of patients, mostly those with spasticity, have other manifestations. Strabismus or other visual disturbances can occur. Children with atherosis due to a nuclear icterus often have deafness and an upward palsy.

Many children with spastic hemiplegia or paraplegia have normal intelligence; Children with spastic quadriplegia or mixed syndrome usually have a severe intellectual disability.

diagnosis

Sometimes tests to rule out hereditary metabolic or neurological disorders

If CP is suspected, it is important to identify the underlying disease. The medical history may indicate a cause. A cranial magnetic resonance tomography is carried out, which in most cases also shows abnormalities.

Cerebral palsy can rarely be confirmed in early childhood, and a specific syndrome usually cannot be defined before the age of 2. High-risk children (e.g. with asphyxia, stroke, periventricular abnormalities in cranial ultrasound in premature babies, jaundice, meningitis, neonatal seizures, hypertension, Hypotension or reflex suppression) should be checked closely.

differential diagnosis

Cerebral palsy should be distinguished from progressive hereditary neurological disorder and disorders that require surgical or other specific neurological treatment.

Atactic forms are particularly difficult to distinguish. In many children with persistent ataxia, a progressive cerebellar degenerative disease is ultimately identified as the cause.

Athetosis, self-mutilation and hyperuricaemia in boys indicate Lesch-Nyhan syndrome.

Infantile spinal muscular atrophy, muscular dystrophies, and neuromuscular junction disorders associated with hypotension and hyporeflexia are usually absent from the signs of cerebral disease.

Adrenoleukodystrophy begins later in childhood, but other leukodystrophies also start earlier and can initially be mistaken for cerebral palsy.

Clarification of a cause

If the history and / or MRI of the skull does not identify a clear cause, laboratory tests should be performed to determine certain progressive memory disorders that affect the motor system (e.g. Tay-Sachs syndrome, metachromatic leukodystrophy, mucopolysaccharidoses) and metabolic disorders ( e.g. organic or amino acid metabolism disorders).

Other progressive disorders, such as infantile neuroaxonal dystrophy, may be suspected based on nerve conduction studies and electromyography. These and many other brain disorders that cause CP (and other manifestations) are increasingly being identified with genetic tests that are performed to test for a specific disease or to screen for many disorders (microarray and whole genome testing)..

forecast

Most children survive into adulthood. Children with severe sucking and swallowing restrictions who require gastrostomy nutrition have a lower life expectancy.

The aim is that the children have the greatest possible independence within the border their motor skills and the associated deficits. With appropriate organizational effort, many children, especially those with spastic paraplegia or hemiplegia, can lead an almost normal life.

treatment

Physiotherapy and occupational therapy

Support treatment, restriction therapy, drug therapy or surgical procedures for the treatment of spasticity

Injections with botulinum toxin

Physiotherapy and occupational therapy are initially used to stretch, strengthen and facilitate movement patterns and are continued later. Support treatment, restriction therapy and medication can be added.

Botulinum toxin can be injected into the muscles to avoid uneven stress on the joints and to prevent fixed contractures.

Baclofen, benzodiazepine (e.g. diazepam), Tizan >Baclofen (with a subcutaneous pump and catheter) is the most effective therapy for severe spasticity.

Orthopedic interventions (e.g. muscle tendon relaxation or transfer) can help to reduce restricted joint movements and joint misalignments. A selective dorsal rhizotomy performed in neurosurgery can help a few children if spasticity only affects the legs and cognitive skills are good.

If the intellectual limitations are not severe, children can attend regular school classes and participate in customized training programs and even competitions. Speech therapy and other forms of communication facilitation may be needed to improve interactions.

Some severely affected children can benefit from the practice of simple everyday activities that promote independence (e.g. washing, dressing, eating). This promotes their independence and self-respect and reduces the burden on the family and caregivers. Assistive devices can improve mobility and communication, they can help maintain mobility and make everyday life easier. Some children need lifelong monitoring and help to varying degrees.

Many children’s facilities set up transition programs for patients when they grow up and have less support to help them with their special needs.

For all chronically handicapped children, parents need constant help and advice to understand their child’s condition and opportunities and to deal with their own feelings of guilt, anger, denial and grief (children with chronic illnesses: family effects ). These children can only achieve their maximum possibilities with stable and sensitive parental care and with the help of public and private support (community workers, self-help groups, rehabilitation groups such as United Cerebral Palsy).

Important points

Cerebral palsy is a syndrome (not a specific disease) that includes nonprogressive spasticity, ataxia, and / or involuntary movement.

The etiology is often multifactorial and sometimes unclear, but includes pre- and perinatal factors that are associated with malformation or damage to the CNS (e.g. genetic diseases and those in the womb, premature birth, core icterus, perinatal asphyxia, stroke, CNS infections) are.

Mental retardation and other neurological manifestations (e.g. strabismus, deafness) are not part of the syndrome, but may be present depending on the cause.

The syndromes manifest before the age of 2 years; a later onset of similar symptoms indicates another neurological disorder.

An MRI of the skull is performed and, if necessary, tests for inherited metabolic and neurological disorders.

Treatment depends on the type and degree of disability, but physiotherapy and occupational therapy are usually used; some children benefit from bracing, botulinum toxin, benzodiazepines, other muscle relaxants, intrathecal baclofen, and / or surgery (e.g. muscle tendon distension or transfer, rarely dorsal rhizotomy).