-
Abnormalities of Tone and Movement in ChildrenRosalina Q. De
Sagun, M.D.Maria Antonia Aurora M. Valencia,M.D.Department of
PediatricsDepartment of Neurology and Psychiatry
-
Developmental DelayDelayed acquistion of milestones expected for
chronological age.Important to distinguish from Progressive
Neurological Disorders which manifests as LOSS of previously
acquired skills.Delays can involve any developmental parameter/s:
Motor, Language, Psychosocial
-
Localization of WeaknessUMNLMN
-
Upper Motor NeuronLower Motor NeuronBulkMinimal AtrophyProfound
AtrophyToneIncreased ; spasticDecreased; flaccidDTRsHyperreflexia
Decreased/ AbsentFasciculationsAbsentPresent/
AbsentBabinskiPresentAbsentSensory DeficitMay be presentMay be
present
-
Clinical Clues 1. Central nervous system Upper motor neuron
(spasticity, hyperreflexia); may beaccompanied by cerebral
manifestations(seizures, cognition, language and sensory
problems)2. Peripheral nervous system Lower motor neuron(decreased
to absent reflexes, flaccid)
-
Disorders of the motor system may be: 1. Acute - stroke/vascular
metabolic disorders infection seizures 2. Chronic - cerebral palsy
(static) congenital CNS lesion degenerative disorders
(progressive)
-
CEREBRAL PALSYRefers 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 (CP) Impairment in movement and posture leading
to functional deficits and the inability to perform activities of
daily living
Caused by a broad group of developmental, genetic, metabolic,
ischemic, infectious and other etiologies that produce a common
neurologic phenotype
- Epidemiology and EtiologyMost common and costly form of chronic
motor disability with a prevalence of 2/1000
-
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 CEREBRAL PALSY
-
Motor Disorders in CP Three main criteria in classification:
1. Type of motor disorder 2. Topographical distribution
3. Gross motor function
-
Types of Cerebral Palsy and the Major Causes
PhysiologicTopographicEtiologicFunctionalSpasticAthetoidRigidAtaxicTremorAtonicMixedUnclassifiedMonoplegiaParaplegiaHemiplegiaTriplegiaQuadriplegiaDiplegiaDouble
hemiplegiaPrenatal infection, metabolic, anoxia, toxic, genetic,
infarctionPerinatal anoxiaPostnatal toxins, trauma, infectionClass
I no limitation of activityClass II slight to moderate
limitationClass III moderate to great limitationClass IV no useful
physical activity
-
Motor SyndromeNeuropathologyMajor CausesSpastic
diplegiaPeriventricular
leukomalaciaPrematurityIschemiaInfectionEndocrine /metabolic/
geneticSpastic QuadriplegiaPVL/ Multicystic encephalomalacia,
MalformationsSame as aboveHemiplegiaStroke in utero or
neonatalThrombophilic disorders InfectionGeneticHemorrahgic
InfarctionExtrapyramidal/ AthetoidPathology in the basal ganglia,
putamen, globus pallidus,
thalamusAsphyxiaKernicterusMitochondrialGenetic/Metabolic
-
Clinical Manifestations of CPMovement disorders Spasticity
Athetosis Dystonia Rigidity Ataxia Mixed motor problems2.
Associated with a spectrum of developmental diasabilities: Mental
retardation, epilepsy, hearing and visual problems, speech,
cognitive and behavioral
-
Hypotonic Cerebral PalsyPhysiologic Classification
-
Physiologic classification
Hypotonia
-
Hypotonic Cerebral PalsyPhysiologic Classification
-
Spastic DiplegicCerebral PalsyPhysiologic Classification
-
Spastic Cerebral Palsy
-
Spastic Quadriplegic Cerebral Palsy
-
Spastic DiplegicCerebral Palsy
-
Spastic QuadriplegiaMost severe form because of involvement of
ALL four limbs and the HIGH association of mental retardation and
seizures.Feeding problems are common due to supranuclear bulbar
palsies.
-
AthetoidCerebral Palsy
-
Athetoid CPLess common than the spastic typeThis is the type
most likely associated with birth asphyxiaHypotonic infants who do
not have UMN signs but later on develop increased variable tone
(rigidity) and dystonia and other dyskinesiasIntellect is preserved
in many cases
-
AthetoidCerebral Palsy
-
AtaxicCerebral Palsy
-
Diagnosis Cerebral Palsy is a clinical diagnosis
Neuroimaging will document the extent of the structural
pathology, aid in diagnosis and prognostication and rule out
treatable causes and slowly progressive neurological disorders.
-
DiagnosisThorough history to identify risk factors,
developmental assessment, physical and neurological
examinationsHearing and vision screeningEEG if with seizuresIf no
possible etiology or risk factors for CP, do diagnostic tests as:
Neuroimaging CT/MRI Metabolic screening, Chromosomal studies
-
Differential Diagnosis 1. Motor delays from congenital
structural malformations 2. Progressive disorders of the brain
white matter diseases 3. Muscle disorders- myopathies, dystrophies.
4. Anterior horn cell disease- spinal muscular atrophy (SMA)
-
DifferentialsDysmyelinating / demyelinating disorders - Abnormal
myelin formation as in some metabolic disorders - Abnormal myelin
secondary to brain insults from infection, trauma, autoimmune.
Clinically: - White matter involvement with progressive
neurologic deficits ( spasticity, hyperreflexia, hearing and vision
may be affected.)
-
2. Spinal Muscular atrophy - disorders of the anterior horn cell
- usually progressive - of 3 varieties depending on the age of
onset of symptoms Infantile type Werdnig-Hoffman disease
Intermediate Juvenile - Kugelberg Welander disease Present with LMN
signs: fasciculations, floppy, areflexic
-
DifferentialsMuscle - Congenital myopathyNerve disorders -
manifestations of lower motor disease, decreased reflexes, tone
(e.x. Hereditary Motor-Sensory Neuropathy)
-
C PEffective management requires 1. Understanding of the
pathophysiology of the disorder 2. Careful assessment of the
patients capabilities and limitations 3. Knowledge of available
treatment regimens, their applications and limitations
-
Management MultidisciplinaryPediatricianNeurologist- management
of seizures, botulinum toxin injections Rehabilitation specialists
Physical and occupational therapistsDevelopmental
psychologistsEducation specialistsOrthopedic surgeonsSocial
workers
-
Autism Spectrum DisorderAutism is a neurodevelopmental disorder
of unknown etiology but with a strong genetic basis.Behavioral
phenotype:Qualitative impairment in language/ communicationImpaired
Social interactions and reciprocityLack of Imaginative play
-
Signs and SymptomsNo pathognomonic symptom or behaviorMost will
present with:Impairment in joint attention ( use of eye contact and
pointing to share experiences with others) Normally develops at 18
monthsLack of protoimperative pointing ( to get an object of
desire)Lack of protodeclarative pointing ( to share an object of
interest/ naming)Lack of imaginative play
-
ManifestationsPoor eye contactVerbal abilities vary: non-verbal
to advanced speechBut speech may have odd prosody or intonation,
echolalia, pronoun reversal, non-sense rhymingIQ from MR to
superior functioningStereotypical/ ritualistic behaviorsMarked need
for sameness
-
DiagnosisDSM IV Criteria for AutismModified Checklist for Autism
in Toddlers (M-CHAT)Pervasive Developmental Disorder Screening Test
(PDDST)
-
TreatmentMultidisciplinaryDevelopmental pediatrician, Pediatric
Neurologist/ Child PsychiatristPsychologistOccupational/ Speech
therapistSpecial Education Teacher (if necessary)Medications
depending on co-morbid conditions
-
Attention Deficit Hyperativity Disorder (ADHD)Most common
neurobehavioral disorder in childhoodCharacterized by (DSM IV):(1)
Inattention(2) Poor impulse control(3) Motor overactivity /
restlessnessSymptoms should be present for more than 6 months in at
least 2 settings ans significantly affects social, academic or
occupational functioning.
-
EtiologyMultifactorialGenetic susceptobilityMaternal
complications during pregnanceMaternal smoking amnd alcohol intake
during pregnancyAbnormal brain structuresPsychosocial family
stressors exacerbate and/ or contribute to the symptoms.
-
PathogenesisSmaller prefrontal cortex and basal ganglia with
5-10% less blood flow in these areas.These areas are rich in
dopamine receptors led to the dopamine hypothesis disturbances in
dopamine system are related to the onset of ADHD
-
DiagnosisClinical interview and historyFulfills criteriaFamily
Hx of ADHDFamily discord, situational stressBehavior rating scales
( e.x. Conners rating scale), helps establish the magnitude and
pervasiveness of symptoms and can be used for monitoring
improvement with intervention
-
TreatmentPsychosocial treatmentsBehaviorally oriented
treatmentsMedications : Methylphenidate and Atomoxetine
-
PrognosisPersists throughout the life spanReduction in
hyperactive behavior with age Other symptoms: Impulsivity,
disorganization and inattention become prominent affecting
relationships and occupational functioningHigh risk for risk taking
behaviors ( substance abuse) and psychiatric dso.
-
Thank you!