The Child with Motor Weakness Neurology Module Pediatrics II

Cerebral Palsy - Objectives At the end of this topic you should be able to: provide a clear definition of cerebral palsy; discuss aetiological and risk factors associated with cerebral palsy; explain the clinical features and associated impairments common to cerebral palsy; demonstrate an understanding of diagnostic methods; recognize the various methods of effectively managing cerebral palsy.

The Floppy Baby J.M., 17-month-old boy admitted because of cough Born preterm at 7 months age of gestation by a repeat Caesarean section; BW=1.3 kg; Apgar score not known to mother but resuscitation was reportedly done. He developed sepsis and stayed in the NICU for a month. Discharged with difficulty in feeding such that he would often cough while sucking.

Case: J.M., 17 months old He was never able to regard, hold head, roll over, use his hand purposively, babble or startle. He had one episode of febrile seizure.

Case: J.M., 17 months old Pertinent Physical Examination Findings: Wt=5.95 kg(<P5) Length=65.5cm(<p5) HC=42cm(<P5) awake but oblivious to surroundings, shrill cry, does not alert nor startle to sound, tongue midline, crackles over both lungs Head lag on traction maneuver, slips through on vertical suspension, truncal hypotonia, loops over on horizontal suspension Spastic limbs, limited movement of both lower extremities with +++ DTRs

Salient Points 17 months old, with developmental delay Preterm birth (+) Neonatal asphyxia (+) Infection (+) History of seizures Marked developmental delay Neurologic abnormalities - truncal hypotonia with spastic limbs, weakness of both lower extremities

QUESTION #1: Is there a neurologic disease? Yes, as evidenced by the abnormal neurologic examination.

QUESTION #2: Where is the lesion? The abnormalities in the tone (hypotonia) and movement (diplegia) point to the motor system. Weakness can be due to lesions in the : 1. Central nervous system – Upper motor neuron (spasticity, hyperreflexia); may be accompanied by cerebral manifestations (seizures, cognition, language and sensory problems) 2. Peripheral nervous system – Lower motor neuron (decreased to absent reflexes, flaccid)

QUESTION #2: Where is the lesion? J.M appears to have an upper motor lesion specifically the cerebral hemispheres.

QUESTION #3: What is the nature of the lesion? Disorders of the motor system may be: 1. Acute - strokes/vascular metabolic disorders infection 2. Chronic - cerebral palsy (static) congenital CNS lesion degenerative disorders (progressive)

CEREBRAL PALSY Refers to a group of disorders characterized by motor abnormalities (tone, posture or movement) which are neither progressive nor episodic. The brain lesions are static and result from disorders of early brain development, usually insults in the perinatal period. They are not progressive but the symptoms may change in time.

CEREBRAL PALSY Clinical manifestations: 1. Delay in development – i.e. poor head control, delays in gross motor or fine motor development 2. Motor deficit – depending on the area of the brain involved and usually the risk factors present 3. Associated developmental disabilities – mental retardation, epilepsy, visual, hearing, speech and behavioral abnormalities

Types of Cerebral Palsy and the Major Causes Physiologic Topographic Etiologic Functional Spastic Athetoid Rigid Ataxic Tremor Atonic Mixed Unclassified Monoplegia Paraplegia Hemiplegia Triplegia Quadriplegia Diplegia Double hemiplegia Prenatal (e.g., infection, metabolic, anoxia, toxic, genetic, infarction) Perinatal (e.g., anoxia) Postnatal (e.g. toxins, trauma, infection) Class I – no limitation of activity Class II – slight to moderate limitation Class III – moderate to great limitation Class IV – no useful physical activity

Topographic Classification Diplegia Hemiplegia Quadriplegia More Affected Less Affected

Physiologic Classification Hypotonic Cerebral Palsy

Physiologic Classification Spastic Diplegic Cerebral Palsy

Diagnosis 1. Thorough history, developmental assessment, physical and neurological examinations 2. Hearing and vision screening 3. EEG if with seizures 4. If no possible etiology or risk factors for CP, may do diagnostic tests as: Neuroimaging – CT/MRI Metabolic screening Chromosomal study

Differential Diagnosis 1. Motor delays from congenital structural lesions 2. Progressive disorders of the brain – white matter diseases 3. Muscle disorders- myopathies, dystrophies.

Management Multidisciplinary Pediatrician Neurologist Rehabilitation specialists Physical and occupational therapists Developmental psychologists Education specialists Orthopedic surgeons Social workers

Thank you!