Cerebral palsy

Morphological substrate and neuroimaging

The etiology of the disease is diverse, and the clinical picture is dictated by neuromorphology. Diagnosis is based on clinical evaluation, but magnetic resonance imaging (MRI) is used to objectify the lesions.

The following basic radiological patterns are distinguished:

• Cystic periventricular leukomalacia (PVL): symmetric cystic cavities in the white matter around the lateral ventricles. This is a classic marker of prior hypoxic-ischemic damage to the immature brain.
• Post-ischemic hemiatrophy: a total reduction in the volume of one hemisphere with local dilatation of the ventricles and subarachnoid spaces (a direct outcome of severe ischemia).
• Other changes: gross ventriculomegaly, cystic transformation of tissues, and cortico-subcortical atrophy.

ICD-10 classification

Official systematization is built around the leading clinical syndrome:

1. Spastic forms (G80.0, G80.1, G80.2): the leading symptom is hypertonus. The topy of paresis determines the subtype: hemiparetic (arm and leg on one side), double hemiparetic, and diplegic (symmetric lesion). Lower diplegia with involvement of the legs is known as Little's disease.
2. Extrapyramidal / hyperkinetic form (G80.3): most often develops as an outcome of hemolytic disease of the newborn (HDN) due to the toxic effect of unconjugated bilirubin on the basal ganglia. It manifests as involuntary movements that disrupt breathing and swallowing.
3. Cerebellar / ataxic form (G80.4): muscular hypotonia, impaired statics, and coordination of movements dominate.
4. Mixed forms: combine symptoms of different lesions and have an extremely unfavorable prognosis.

Clinical features and complications

An obligate (mandatory) sign of the disease is a disturbance in the motor sphere. A facultative (optional) sign is changes in intellect.

The symptom complex is formed due to muscle tone disorders (spasticity or hypotonia), hyperkinesia, and seizures (epileptic syndrome). A crucial pathogenetic factor is the failure of primitive neonatal reflexes (spinal automatisms) to fade in a timely manner, which physiologically blocks the development of normal voluntary movements. Clinically, this is visualized as abnormal postures and aberrant reactions (for example, head lag during the traction test).

Markers of a severe clinical course include the presence of intrauterine infections, clinical-morphological dissociation (discrepancy between clinical presentation and MRI data), and treatment resistance, indicating exhaustion of brain reserves or ongoing cellular apoptosis.

Over time, trophic and orthopedic complications progress:

• Gross irreversible muscle atrophy.
• Persistent joint contractures that limit the range of motion.

Structure of cognitive impairments

Despite the primary nature of the motor deficit, concomitant higher cortical disorders are extremely common:

• In 80–88% of patients: decreased concentration, spatial perception disorders, and pronounced speech disorders.
• In 60–75% of patients: impaired perception of one's own body schema, memory decline, and disorders of kinesthetic praxis and constructive activity.

Principles of correction and rehabilitation

Therapeutic measures are conceptually divided into three interrelated tasks:

1. Neurophysiological correction: normalization of the excitation-inhibition balance in the central nervous system, and stimulation of blood flow in the brain and muscles.
2. Orthopedic correction: combating contractures, muscle weakness, and dystrophy, and normalizing tone.
3. General somatic and psychological support: improving the quality of life.

To relieve severe spasticity, pharmacotherapy actively employs botulinum therapy (botulinum toxin type A preparations — Dysport, Botox). As part of surgical treatment, neurotomy is used — the partial transection of peripheral motor nerve fibers to persistently reduce hypertonus.

A mandatory component of rehabilitation is social and domestic adaptation: creating an ergonomic environment, adapting household items (tableware, light switches), installing ramps, and using specialized transport.

Class and exam questions

What else to study on this topic

• Mechanisms of cystic periventricular leukomalacia (PVL) formation
• Pathogenesis of bilirubin-induced toxic damage to the basal ganglia
• Features of performing and interpreting the neurological traction test
• The role of cellular apoptosis in the development of treatment resistance
• Clinical and morphological dissociation: causes of discrepancy between the MRI picture and neurological status

Other topics in «Chapter 2. Neonatology»

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