CEREBRAL PALSY

Salters Textbook of Disorders & Injuries of the Musculoskeletal System

Chapter 12: Neuromuscular disorders Sub-chapter: Disorders of the brain

Definition
Cerebral palsy encompasses the various types & degrees of the nonprogressive brain disorders that develop before, during, or relatively soon after birth. Manifest as disturbances of voluntary muscle balance & perception. Clinically apparent in early childhood persist throughout the life.

Incidence
Six CP for each 100.000 population per year

Etiology
Intrapartum cerebral anoxia Brain injury during traumatic delivery

Genetic Congenital cerebral malformation Prenatal intracranial hemorhage Premature birth Erythroblastosis fetalis (Rh incompatibility) Encephalitis Accidental or nonaccidental head injury

Pathogenesis & Pathology

The underlying lesion is irreparable & nonprogressive. Release phenomenon Manifestations of cerebral palsy depend on the extent of afflicted area: cerebral cortex, basal ganglia, or cerebellum.

Release Phenomenon
The lost of function in one neuron system of the brain results in the release of normal control over interdependent systems that, in turn, tend to overact.

 Three main types of cerebral palsy:

Spastic (65%) Athetoid (20%) Ataxic (5%) Other three additional types: tremor, rigidity, & atonia (make up the remaining 10%)

Clinical Features & Diagnosis

Tend to appear slowly over several months after birth. CP can be suspected failing to achieve normal milestones of motor development;
Turning over (5 months) Sitting up (7 months) Pulling up to standing position (10 months) Standing alone (14 months) Walking unaided (15 months)

Clinical Features & Diagnosis

Various degree of intellectual disability:
Serious intellectual disability / uneducable (40%) Moderate intellectual disability / below average (40%) Average or above (20%)

Severity of CP varies greatly:

Mildest forms: pt is capable of leading an almost normal life Severe forms: pt is almost completely incapacitated.

(1) Spastic Type

Paralysis of patterns of voluntary movement (rather than of individual muscles) Increased muscle tone (hypertonicity, spasticity, increased deep tendon reflexes, & clonus) Startle reflex a mass muscle spasm elicited by any sudden noise. The spastic limbs seem stiff & exhibit an increased stretch reflex (sudden contraction of a muscle when stretched)

(1) Spastic Type

Depending on the extent of lesion on cerebral cortex, the limb involvement can be divided into:
Monoplegia affecting one limb Hemiplegia upper & lower limb on the same side Diplegia / paraplegia both lower limbs Tetraplegia / quadriplegia / bilateral hemiplegia all four limbs

(1) Spastic Type

Some muscles are more spastic than others, and some are weaker than others muscle imbalance. Muscles that cross two joints (e.g. biceps, gastrocnemius) tend to be more spastic than those that cross only one joint.
Flexor muscle tend to out-pull extensor muscle Adductor tend to out-pull abductor Internal rotator tend to out-pull external rotator

Thus, the neurogenic deformities flexion, adduction, internal rotation.

(1) Spastic Type

Spastic gait is characteristically stiff, clumsy, & jerky when bilateral, it is producing scissors gait. Spastic paralysis of muscles of speech difficulty learning to speak clearly Spastic paralysis of muscles of swallowing difficulty of swallowing saliva drooling.

(2) Athetoid Type

Characteristic features: involuntary, uncontrollable movements (mobile spasms), in muscle group of the face & all four limbs. Producing twisting, writhing contortions in the limbs & meaningless grimaces in the face. Difficulty with speech & swallowing. The deep tendon reflexes & the plantar cutaneous reflexes are usually normal. The athetoid childs intelligence is usually normal.

(3) Ataxic type

Cerebellar ataxia Disturbed coordination of muscle groups & relative lack of equilibrium. The gait is unsteady & the child frequently appears about to fall. There is neither spasticity nor athetosis, and because the lesion is primarily cerebellar, intelligence is usually unaffected.

Prognosis
In general, the prognosis of children with cerebral palsy with respect to their intelligence & ability to walk (without treatment) can be stated as follows:
Hemiplegics 70% normal intelligence & all will walk Diplegics 60% have intelligence above 80 & 75% will walk Quadriplegics 90% have intellectual disability & only 25% will walk. Athetoid limited motor skills, problem with verbal communication. Mostly have normal intelligence, as do the ataxic children.

Prognosis
Approximately one third of all children with CP have so severe brain lesion that treatment is ineffective & institutional care is required. One sixth have lesion so mild that treatment is unnecessary. The remaining half can be helped by realistic treatment.

Treatment
Psychological considerations Therapeutic drugs Physical & occupational therapy Speech therapy Orthopaedic appliances Surgical manipulations Orthopaedic operations Neurosurgical operation (selective posterior rhizotomy) Rehabilitation

Therapeutic drugs
For very young children with spastic diplegia or quadriplegia & dynamic deformities of the lower limbs, but no structural contractures. Injection of Botulinum-A toxin into the myoneural junction of the most spastic muscles (usually calf, hamstrings, adductors). The beneficial effects last from 3-4 months, after which the injections can be repeated several times.

Orthopaedic Appliances
Removable splints are helpful in preventing deformity. During the early years, braces for the lower limbs are often necessary to enable a child to stand & walk with the help of crutches.

Orthopaedic Operations
Aims of surgery:
Prevention of musculoskeletal deformity Correction of existing musculoskeletal deformity Improvement of muscle balance Improvement of function

It is of value primarily in the spastic type but is not indicated until the child has at least developed kneeling balance. Operations can improve function, but they cannot make spastic limbs function normally.

Orthopaedic Operations
Types of operation
Tendon lengthening Tendon transfer Arthrodesis

Serious complication of the complex muscle imbalance in a child with severe CP (e.g. Progressive paralytic subluxation of the hip) muscle release often fail need to perform combined soft tissue releases, capsular repaire, & extensive osteotomies.