Transcript Document

Approach to a Child with
Neurodevelopmental
Disability
Prof. Shaul Harel
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Approach to a child with
neurodevelopmental
disability 2004
Lecture Plan
• Introduction
• Differential Diagnosis
• “Red Flags”
• “Take Home Message”
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Approach to a child with
neurodevelopmental
disability 2004
Principles of development
• A continuous process from conception to maturity
• Depends on maturation and myelination of nervous
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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
Approach to a child with
neurodevelopmental
disability 2004
Developmental disability
nosologic categories
• Global developmental delay
• Motor delay
• Cerebral palsy
• Developmental language disorder
• Primary sensory impairments
• Auditory
• Visual
• Autisic spectrum disorders
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Approach to a child with
neurodevelopmental
disability 2004
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
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Approach to a child with
neurodevelopmental
disability 2004
Psychomotor delay
• Special sensory deprivation (auditory,
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visual)
Static vs. progressive
Mental retardation
Environmental deprivation
“Late bloomer”
Approach to a child with
neurodevelopmental
disability 2004
Delayed maturation
“slow starters”
“late bloomers”
• Motor (Illingworth 1961, 1972; Hagberg 1969)
• Auditory (Illingworth 1972)
• Speech (Illingworth 1972)
• Visual (Illingworth 1961; Mellor & Fielder 1980;
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Harel 1983; Hoyt 1983; Cole 1984)
Sucking- swallowing reflex (Leroy-Malherbe 1994)
Social (Harel)
Approach to a child with
neurodevelopmental
disability 2004
“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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Approach to a child with
neurodevelopmental
disability 2004
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Approach to a child with
neurodevelopmental
disability 2004
Non- progressive lesions
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Examination:
History:
• Perinatal brain insult • UMN vs. LMN
• CNS malformation • LMN- central vs
• Infection
peripheral
• Trauma
• Poisoning
• Symptoms
• Mental, motor, senses,
speech, behavior,
Approach to a child with
convulsions
neurodevelopmental
disability 2004
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Approach to a child with
neurodevelopmental
disability 2004
Progressive CNS lesion
History:
• Symptoms
• Grey matter vs
white matter
• Age of onset
• Pattern and rate of
course
• Family Hx
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Examination:
• Primary CNS vs.
generalized disease
• Retinal or optic N.
involvement
• Peripheral NS involvement
• Special senses
• Head circumference
Approach to a child with
neurodevelopmental
disability 2004
Chromosomal study indications
• impression suggesting specific chromosome
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syndrome (dysmorphic features)
Two or more minor congenital
malformations
Failure to thrive, short stature
Mental retardation
Abnormal genitalia, sexual characteristics
Multiple early abortions
Approach to a child with
neurodevelopmental
disability 2004
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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)
Approach to a child with
Family Hx
neurodevelopmental
disability 2004
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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neurodevelopmental
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neurodevelopmental
disability 2004
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neurodevelopmental
disability 2004
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neurodevelopmental
disability 2004
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Approach to a child with
neurodevelopmental
disability 2004
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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neurodevelopmental
disability 2004
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neurodevelopmental
disability 2004
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neurodevelopmental
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Approach to a child with
neurodevelopmental
disability 2004
Red flags (3)
Infancy/ early childhood
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• 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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Approach to a child with
neurodevelopmental
disability 2004
Red flags (4)
Infancy/ early childhood
• Delayed appearance of postural reflexes and
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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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neurodevelopmental
disability 2004
Midline and
Neurocutaneus Lesions
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disability 2004
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neurodevelopmental
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neurodevelopmental
disability 2004
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” →
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dystonia → dyskinesia
Testing intelligence using conventional tests often
erroneous in CP:
• Associated motor problems, visual, auditory
Approach to a child with
and speech deficits
neurodevelopmental
disability 2004
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neurodevelopmental
disability 2004
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neurodevelopmental
disability 2004