Approach to a Child with Neurodevelopmental Disability

1. Approach to a child with neurodevelopmental disability 2004 1 Approach to a Child with Neurodevelopmental Disability Prof. Shaul Harel

2. Approach to a child with neurodevelopmental disability 2004 2 Lecture Plan Introduction Differential Diagnosis �Red Flags� �Take Home Message�

3. Approach to a child with neurodevelopmental disability 2004 3 Principles of development A continuous process from conception to maturity Depends on maturation and myelination of nervous system The sequence is the same, the rate varies from child to child Primitive reflexes should be lost before the voluntary movement develops Cephalo � caudal direction of development Generalized mass activity? individual responses No child is mentally retarded if backward in a single field of development and normal in all others

4. Approach to a child with neurodevelopmental disability 2004 4 Developmental disabilitynosologic categories Global developmental delay Motor delay Cerebral palsy Developmental language disorder Primary sensory impairments Auditory Visual Autisic spectrum disorders

5. Approach to a child with neurodevelopmental disability 2004 5 Global developmental delay Significant delay in two or more developmental domains: Gross motor Fine motor Cognition Speech/language Personal/social (play, recreation) Activities of daily living skills

6. Approach to a child with neurodevelopmental disability 2004 6 Psychomotor delay Special sensory deprivation (auditory, visual) Static vs. progressive Mental retardation Environmental deprivation �Late bloomer�

7. Approach to a child with neurodevelopmental disability 2004 7 Delayed maturation�slow starters��late bloomers� Motor (Illingworth 1961, 1972; Hagberg 1969) Auditory (Illingworth 1972) Speech (Illingworth 1972) Visual (Illingworth 1961; Mellor & Fielder 1980; Harel 1983; Hoyt 1983; Cole 1984) Sucking- swallowing reflex (Leroy-Malherbe 1994) Social (Harel)

8. Approach to a child with neurodevelopmental disability 2004 8 �Late Bloomer" Normal Pre-perinatal history No evidence for congenital or genetic malformations /abnormalities Positive family history Lack of obvious neurological abnormalities Pattern of developmental dissociation Complete �Catch Up� ?

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10. Approach to a child with neurodevelopmental disability 2004 10 Non- progressive lesions History: Perinatal brain insult CNS malformation Infection Trauma Poisoning Symptoms Mental, motor, senses, speech, behavior, convulsions Examination: UMN vs. LMN LMN- central vs peripheral

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12. Approach to a child with neurodevelopmental disability 2004 12 Progressive CNS lesion History: Symptoms Grey matter vs white matter Age of onset Pattern and rate of course Family Hx Examination: Primary CNS vs. generalized disease Retinal or optic N. involvement Peripheral NS involvement Special senses Head circumference

13. Approach to a child with neurodevelopmental disability 2004 13 Chromosomal study - indications impression suggesting specific chromosome syndrome (dysmorphic features) Two or more minor congenital malformations Failure to thrive, short stature Mental retardation Abnormal genitalia, sexual characteristics Multiple early abortions

14. Approach to a child with neurodevelopmental disability 2004 14 Metabolic screening - indications Pcychomotor retardation Failure to thrive Cutaneous changes Skin color, rash, hair abnormalities Eye abnormalities Cataract, corneal clouding, retinal degeneration, optic atrophy, blindness Organomegaly Neurologic symptoms Behavioral abnormalities, irritability, apathy, somnolence, seizures Unusual odor Crisis deterioration (infection, nutrition, metabolic acidosis) Family Hx

15. Approach to a child with neurodevelopmental disability 2004 15 Red flags (1) � newborn State of arousal Lack of alertness Poor quality of sleep Abnormal cry Feeding problems, drooling Spontaneous motility Symmetry Tremor vs. seizures Abnormal tone Abnormal head size or shape

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24. Approach to a child with neurodevelopmental disability 2004 24 Red flags (2)Infancy/ early childhood Increased or reduced head circumference Lack of alertness, delayed social smile Poor head control (at 3-4 months) Persistent primitive reflexes (ATNR)

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34. Approach to a child with neurodevelopmental disability 2004 34 Red flags (3)Infancy/ early childhood Early asymmetry (handedness <12 months) Increased tone Constant fisting >3 months of age Scissoring Equinus position of feet, toe walking Early rolling over (<4 months) Extensor tone in supine position (window sign) Spastic hand approach and grasp Persistent and sustained clonus �Changing tone� baby

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40. Approach to a child with neurodevelopmental disability 2004 40 Red flags (4)Infancy/ early childhood Delayed appearance of postural reflexes and developmental milestones Increased associated tone and movements in one limb (paretic limb) Visual problems Roving eyes, no visual following, persistent squint Lack of social/Comunicative skills Lack of auditory response, delayed speech, avoiding eye contact, repetitive behavior, desire for sameness, social isolation, lack of imaginative play

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42. Approach to a child with neurodevelopmental disability 2004 42 Midline and Neurocutaneus Lesions

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50. Approach to a child with neurodevelopmental disability 2004 50 Essential to remember! Transient neuromotor abnormalities Axial hypotonia Hypertonic jittery baby Clonus Periodic repeated exams (R/O progression) Clinical pattern of CP evolves over time: hypotonia ? spasticity ? �changing tone� ? dystonia ? dyskinesia Testing intelligence using conventional tests often erroneous in CP: Associated motor problems, visual, auditory and speech deficits

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