1. The Child with Motor Weakness Neurology Module Pediatrics II

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

3. The Child with a Motor Weakness  C.R.,2 yrs. Old, F, referred to neuropdevelopmental clinic because of delay in walking  Born by CS, term, BW 1.5 kg, had respiratory distress and resuscitated  Frerquent Cough a & fever, 2 hospitalizations.

4.  Described as quiet, passive  Able to hold head up and steady at 6 months, rolled over at 8 months; sat alone 1 yr.  Able to take steps at 1 ½ yrs.  Vocalized at 6-8 months  Socialized with mother at 6 months and other siblings at 1 ½ yrs.

5. P.E / N.E. Temp= 37.3’C; PR=95/min; RR=31/min; HC=48 cm; Wt = 9.2 kg; Ht=75 cm. Temp= 37.3’C; PR=95/min; RR=31/min; HC=48 cm; Wt = 9.2 kg; Ht=75 cm. No skin lesions, no dysmorphisms Awake, alert, responds to sounds and simple commands Follows moving objects in all directions, Pupils 2 mm equally reactive to light

6. Neuro exam  No facial asymmetry; with drooling  Moves all extrwemities, the left more than the right  Spastic Rt UE and Rt LE; left extremities=normal tone.  Deep tendon reflexes: Rt ++++; Lt ++  BaBINSKI + Rt.

7. Salient Points  2 yr. old F  Asphyxia in the newborn period  Delayed motor development  Delayed language development  Rt hemiparesis  Hyperreflexia (Rt)  Babinski (Rt)000

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

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

10. QUESTION #2: Where is the lesion?  C.R appears to have an upper motor lesion specifically the cerebral hemispheres. specifically the cerebral hemispheres.

11. Spasticity is the opposite with hyperextension of the limbs as in this patient

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

13. 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.

14.  Clinical manifestations: 1. Delay in development – i.e. poor head control, delays in gross motor or fine motor development 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 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 3. Associated developmental disabilities – mental retardation, epilepsy, visual, hearing, speech and behavioral abnormalities CEREBRAL PALSY

15. Types of Cerebral Palsy and the Major Causes PhysiologicTopographicEtiologicFunctional SpasticAthetoidRigidAtaxicTremorAtonicMixedUnclassifiedMonoplegiaParaplegiaHemiplegiaTriplegiaQuadriplegiaDiplegiaDouble hemiplegia hemiplegiaPrenatal (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

16. Topographic Classification Diplegia HemiplegiaQuadriplegia More Affected Less Affected

17. Hypotonic Cerebral Palsy Physiologic Classification

18. Spastic Diplegic Cerebral Palsy Physiologic Classification

19. 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 Neuroimaging – CT/MRI Metabolic screening Metabolic screening Chromosomal study Chromosomal study

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

21. Management  Multidisciplinary 1.Pediatrician 2.Neurologist 3.Rehabilitation specialists 4.Physical and occupational therapists 5.Developmental psychologists 6.Education specialists 7.Orthopedic surgeons 8.Social workers

22. Thank you!