Motor Disturbance in Children.ppt
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Pediatric Motor disturbance
About Children who trip and About Children who trip and stumble stumble . . .
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Common Patterns
Toe walking
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Common Patterns
Toe Walking
Clumsiness, Stumbling and Tripping
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Common Patterns
Hypotonia
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Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Hypotonia
Imbalance
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Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Imbalance
Hypotonia
Peculiar walking patterns
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Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Imbalance
Hypotonia
Peculiar movements
Weakness
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Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Imbalance
Peculiar movements
Weakness
Deformities
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Common Patterns
Clumsiness, Stumbling and Tripping
Toe walking
Imbalance
Peculiar movements
Weakness
Deformities
Very Peculiar Signs
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Motor DevelopmentMotor Development
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NewbornNewborn
5 months5 months
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7 months7 months
10-11 mo10-11 mo
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Newborn
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6 weeks
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3 months(12 weeks)
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5 months5 months
6 months6 months
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5 months
6 months
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7 months7 months
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4 months5 months
6 months7 months
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4 months
6 months
5 months
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Verticalization
7 mo7 mo 8 mo8 mo 9 mo9 mo 10 mo10 mo 11 mo11 mo
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7 months7 months
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8 months8 months
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9th months
Verticalization – stepping forward – upright posture, first loading predominantly the medial edges of the feet
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9 – 10 months 9 – 10 months 10-11 months10-11 months
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PHENOMENOLOGY
Gross motor clumsiness
Fine motor clumsiness
Abnormal movements
Abnormal posture and gait
Muscular weakness
Hypotonia or Spasticity
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LOCALIZATIONCENTRAL MOTOR NEURONCORTEXCORTICO-SPINAL TRACTS
PERIPHERAL Motor NeuronANTERIOR-HORN CellsMotor Roots and Peripheral NervesNeuro-Muscular JunctionMuscles
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ETIOLOGYStatic Dysfunction cerebral palsyTraumaAcute Infection , ToxicityCVA
Progressive ConditionMetabolic, GeneticAutoimmineChronic Infection
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Hypotonia
CENTRAL
PERIPHERAL
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CEREBRAL HYPOTONIA
Encephalopathy present !
Change in Alertness and Activity
Seizures *Cognitive Delay
Signs of Upper Motor Neuron Disease:
Reflexes Increased
Pathologic reflexes
Persistence of Primitive Reflexes
Motor Delay
Functional Asymmetry
Progression to Spasticity
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PERIPHERAL HYPOTONIA Normal Brain Functions Signs of Lower Motor Neuron Disease:Reflexes Depressed (Myopathy) of Absent Signs of Denervation of Muscles: Amyotrophy,
Fasciculations Low Tone in Extremities No Spasticity Pathologic reflexes Absent Decreased Movements and Postural Reactions NO Functional Asymmetry Electrophysiologic Findings Fibrillation Potentials Abnormal Nerve Conduction
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CEREBRAL SYNDROMES
Benign Hypotonia of Infancy (Common, Self-limited)
Hypotonia of the Prematurity
Hypoxic Ischemic Encephalopathy
Encephalopathic Syndromes
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GENETIC SYNDROMES
Down’s Syndrome 21 translocation, deletion, mosaicism
Prader-Willi Syndrome 5 (q11-13) deletion “HHHH”: -Hypotonia,
-Hypogonadism, -Hypomentia; Hypo HyperphagiaSmall Hands and FeetMicrocephaly, Short StatureCongenital Hip Dislocation
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Work Up: Imaging studies
Nerve Conduction Studies and EMG
EEG
Laboratory Studies: CPK; SGOT/SGPT Screening Tests; Amino Acids and Organic Acids; Long Chain Fatty Acids; Lactate and Pyruvate; Chromosomal DNA testing
Muscle and/or Nerve Biopsy
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5 (ms)
Right Tibial Motor
O
P
TR
Ankle 100.0mA
O
P
T
R Poplit 100.0mA
200 (µV) 5 (ms)
Right Peroneal Motor
O
P
TR
Ankle 100.0mA
O
P
T
R Fib Hed 100.0mA
Amp: 0.53 mV [>3.0] Vel: 43.75 m/s [>41.0]
Amp: 0.18 mV [>2.5] Vel: 43.0 m/s [>40]
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1000 (µV) 20 (ms)
EMG - Left Tib Ant
200 (µV) 10 (ms)
EMG - Left Gastrocnem
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Mental Deficiency (improved after change in custodial caretakers)
Dysmorphic Features
Hypotonia, H/o clubbed feet
Posture and Gait Abnormality
Distal Legs Wasting:
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Work Up:
Brain Imaging
Cervical Imaging (r/o syrinx)
EMG and Nerve Conduction Studies:
rule out congenital neuropathy, muscular dystrophy, myopathy.
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Normal Cognitive DevelopmentChronic Presentation“Subcompensated” courseGower’s SignToe Walking“car accident”
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BILATERAL Toe Walking
Idiopathic Toe Walking (Achilles Shortening)
Spastic DiplegiaPeripheral Neuropathy (CMT)Myscular Dystrophy (Duchenne)Cutaneous calcinosis (in dermatomyositis)Spinal Cord Anomaly Juvenile MSDystoniaMannerism, as one of the autistic traits
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UNILATERAL Toe Walking
Dystonia
Spastic Hemiparesis
Local Conditions:Achilles Tendonitis, Calf HemangiomaLinear Scleroderma
Conversion reaction
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Work Up for Toe Walking
Gait Analysis
Measurement of Ankle Dorsiflexion
Brain Imaging
Thoraco-lumbar Spine Imaging
Nerve Conduction Studies, EMG
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AtaxiaAtaxia
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Ataxia Differential:
Brain Abnormalities: Leukodystrophies (Krabbe) Cerebellar Frontal
Posterior Columns
Spino-Cerebellar Ataxias
Ataxia-Telangiectasia
Neuropathies
Postinfectious (Varicella Cerebellitis)
Autoimmune (Miller-Fisher Variant of GBS)
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Ataxia Work Up:
Brain Imaging
Metabolic Studies
Genetic Studies
Spinal Tap
Nerve Conduction Studies
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MUSCLE
DISEASE
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Muscle Weakness
Gower’s Sign:
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Muscle Weakness
Gower’s Sign:
Paretic Gait:
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Muscle Weakness
Gower’s Sign
Reflex abnormalities
Tone Abnormalities:
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Muscle Weakness
Gower’s Sign
Reflex abnormalities
Tone Abnormalities
Muscle Deformities:
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Muscle Phenomena
Myotonia:
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Muscle Phenomena
Myotonia:
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Differential Muscle DiseaseAnterior Horn Disease (Spinal Muscular Atrophy)
Polyradiculoneuropathies Acute (Guillain-Barre) Chronic (CIDP)
Neuropathies
Myopathy
Muscular Dystrophies
Myasthenia
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Muscle Disease EvaluationHistory, presentation…
Clinical Appearance
Examination
Nerve Conduction Studies, EMG
Muscle / Nerve Biopsy
Genetic Testing (Duchenne, Myotonic Dystrophy, CMT, SCD..)
Labs: electrolytes, CPK, SGOT/SGPT; TFT; carnitine levels, antibodies analysis
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EMG, Nerve Conduction Data
Low amplitudes
Slowing of conduction velocities
Prolonged Latencies
Fibrillation potentials, positive waves
Decreased recruitment
Pathological patterns
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Myotonic DystrophyMyotonic Dystrophy
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Dystonia Differential:Degenerative Encephalopathies: Pelizaeus-Merzbacher Lesch-Nyhan Infantile seroid lipofuscinosis Niemann-Pick Mitochondrial diseases
Genetic Syndromes Idiopathic generalized dystonia Dystonia-Plus: DOPA-responsive
Metabolic Syndromes Biotinidase deficiency, Methylmalonic aciduria, Glutaric aciduria Pediatric neurotransmitters deficiencies
Bilirubin Encephalopathy Potentially Treatable
-Glucose transporter deficiency; Biotin Deficiency; DOPA-responsive, Homocystinuria; Wilson's D-se;
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