Clinical Presentation of Perinatal CNS Lesions

Stages of progression and structure of the recovery period

The chronological progression of the disease directly depends on the infant's gestational age at birth. Two basic stages are distinguished:

• Acute period: spans from birth to 3 months.
• Recovery period: in full-term infants, it runs from 4 to 12 months, whereas in premature infants it naturally extends up to 18–24 months.

During the recovery period, the clinical picture consists of several clusters of disorders. First and foremost is a delay in psycho-speech and motor development. These are joined by neurological deficits (convulsive syndrome, motor disorders) and regulatory disorders, including vegeto-visceral dysfunction and liquor-vascular dystonia. Parents most frequently complain of sleep problems and deviations in the infant's habitual behavior.

Physical examination and motor sphere

During a physical examination, the physician must simultaneously assess the somatic, motor, and psycho-emotional status. The severity of motor delay is divided into moderate (skill formation delayed by 1–4 months) and critical (delay of more than 4 months). The latter signals a high risk of permanent neurological disability.

Special tests are used to detail tone abnormalities, for example, the traction test (pulling up by the arms from a supine position):

• With a hypertonus pattern, pathological tension is visualized (often of the posterior calf muscle group).
• With a hypotonus pattern, the physiological grouping is absent: the limbs are extended, and the head is thrown back.

Focal deficit may manifest as paresis. For example, facial nerve paresis (paresis nervi facialis) is expressed by a smoothed nasolabial fold, lagophthalmos, and facial asymmetry, which becomes markedly prominent during crying. Limb paresis is identified by flaccidity or an unnatural posture compared to the healthy side, which maintains physiological flexion.

Comprehensive assessment of psychomotor development

The analysis of psychomotor status is based on the evaluation of three interrelated spheres:

1. Motor sphere: includes "gross" motor skills (head control, rolling over, walking) and "fine" motor skills (grasping toys, hand-eye coordination).
2. Cognitive sphere: intellectual-mnemonic processes (recognizing faces, reaction to novelty) and pre-speech/speech development (cooing, babbling).
3. Emotional-behavioral sphere: spectrum of emotions (alerting reaction, smiling) and behavior (social contact, sleep and appetite rhythms).

Liquor-vascular disorders vs. true hypertension

Liquor-vascular dystonia syndrome was previously mistakenly called hypertensive-hydrocephalic syndrome. However, true intracranial hypertension occurs in only 3–5% of cases.

Ventricular enlargement (ventriculomegaly) is more often passive (vacuolization) in nature. Due to the death of cerebral parenchyma, the ventricles mechanically fill the empty space (ex vacuo). Such a condition does not require dehydration therapy. On MRI, enlargement of the lateral ventricles due to cortico-subcortical atrophy is clearly visualized.

Criteria for true hypertensive syndrome include bulging of the anterior fontanelle, suture diastasis, persistent "projectile" vomiting, exophthalmos, and Graefe's sign.

Since an infant cannot describe a headache, physicians rely on markers of vascular disorders: obsessive head rubbing against the pillow, tugging at the ears, and autonomic dissociation (hot head with cold feet, skin mottling).

Structure of outcomes in the residual period

Outcomes of a past perinatal lesion depend on the severity of structural changes and are divided into four groups:

• Functional disorders (~45.5%): speech delays (dysarthria, dyslexia), motor clumsiness without paresis, emotional instability (tendency to tantrums).
• Favorable outcome (~30%): complete clinical recovery.
• Severe organic outcome (~25%): formation of permanent deficits. The dominance of motor disorders leads to cerebral palsy (spastic diplegia, hemiplegia, etc.), while mental disorders lead to intellectual disability. Generalized epilepsy and hydrocephalus are also possible.
• Fatal outcome: recorded in less than 0.5% of cases.

Class and exam questions

What else to study on this topic

• Clinical characteristics of various forms of cerebral palsy (spastic diplegia, ataxic form)
• Instrumental visualization: neurosonography, MRI, and Doppler ultrasound of cerebral vessels
• Specific speech disorders as an outcome of perinatal CNS lesions: dyslexia, dysarthria, general speech underdevelopment
• Assessment of somatic status aggravating the course of a perinatal lesion

Other topics in «Chapter 2. Neonatology»

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