NEUROLOGICAL AND

MUSCLE WEAKNESS
I MADE BUDDY SETIAWAN
Cerebral Palsy
Mercer Rang (1990)
CP : is a result of demage of the developing brain that
produce disorder of movement and posture that is permanent
but “not unchanging”

H. Kerr Graham, P. Selber (2003)

CP : is a progressive lesion of the immature brain producing
a disorder of movement and posture as a result of
progressive musculoskeletal pathology.
Incidence
• Occurs in 1-5 in 1000 live births
• More common in advanced countries
• Advanced perinatal care increases survival of brain-damaged children
• Care only slightly reduces incidence of CP
• More common in socioeconomically disadvantaged
Etiology

oVarious causes and risk factors

o35% not known
o3 groups : prenatal, perinatal, postnatal

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 Prenatal Perinatal Postnatal
Congenital infections Prematurity Infections
meningitis
Torches : Low birth weight Encephalitis
Toxoplasmosis
Rubela Complicated delivery Head trauma
Cytomegalovirus (dystocia)
Herpes asphysia Cerebrovascular accident
Syphylis cerebral trauma cyanotic cong. heart
disease
AIDS Infections sickle cell anaemia
menigitis vascular malformation
Abuse drugs: herpes
tobacco Cerebral anoxia
alcohol Hyperbillirubinaemia near drowning
marijuana Blood incomptabilities aspiration asphyxia
cocaine Other hemolytic disorder cardiac arrest
drugs seizures
Hypoglycaemia
Cerebral malformation Malnutrition

Obstetric complication
pre eclampsia/eclampsia
abruptio placentae
placenta praevia

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Classification
3 Principle domains :
1. Movement
2. Anatomy
3. Function

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Abnormal Movement
o Spasticity (60 – 80)%
o Dyskinesia (10 – 25)%
o Mixed (10 – 25)%
o Ataxia (2 – 5)%
o Hipotonia (2 – 5)%

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Spasticity :
o velocity-dependent hyperactivity of stretch reflexes
o deep tendon reflexes increase
o clonus
o Pathologic reflexes

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Anatomical (Topography)
1. Unilateral
 monoplegia
 hemiplegia

2. Bilateral
 diplegia
 triplegia
 tetraplegia

Hemiplegia, diplegia, tetraplegia : 35%

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Function Classification :
1. community ambulator
2. household ambulator
3. exercise ambulator
4. non-ambulator

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Functional
o GMFCS

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Pathology

1. Nervous system

1. Musculoskeletal

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Nervous System by MRI
• white matter damage (prematurity) 43%
• basal ganglia damage 13%
• cortex/subcortical damage 9%
• CNS malformation 9%
• focal infarcts 7%
• others 7%
• normal MRI 12%

• OBSTETRICAL damage only 20%

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A. Periventricular Leucomalacia (P.V.L) or hemorrhage
in Premature infant 50%
in Full term (10 – 20)% } CP. diplegia
B. Thrombosis a. cerebralis media 30% CP. hemiplegia

o Prolonged hypoxia (hypoxia-ischemic encephalopathy : HIE)

c. o Infection (bacteria, viral) : TORCHES
o Brain malfuction
o Wide haemorrhage
o Corpus callosum dysgenesis and neural migration
Total body involvement

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Associate distrubances
o mental retardation and difficult to walk 40%
o epilepsy 30%
o Complex movement 20%
o Visual 16%
o malnutrition 15%
o hydrocephalus 14%

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Musculoskeletal pathology

CP : - Static encephalopathy
- with progressive musculoskeletal pathology

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Pathogenesis
1. Weakness
Upper motor neuron lesion causes :
• Loss of voluntary movement
• Weakness
• Easy fatigability
Pathogenesis
2. Spasticity
• Feature of all lesions of pyramidal system
• Abnormally increased contraction of a muscle related to excessive activity of disin
hibited spinal neurones
• Mediated via stretch reflex
• Tendon reflexes hypertonic
• Clonus may appear
• Posture characteristic because some neurones more active than others
• Attempts to change position lead to resistance which quickly yields clasp-knife ph
enomenon
Pathogenesis
3. Contracture
• Nature of muscle contracture : shortening of muscle-tendon unit due to f
ailure to keep pace with growth of bones
• Muscle adds sarcomeres at musculo-tendinous junction in response to c
onstant stretch
• In normal children, walking & movement provide all stretch needed
• When muscles spastic, this mechanism cannot occur
Pathogenesis
4. Deformity
• From unopposed muscle contracture
• Hip dislocation because of :
• Persistent hip adduction leads to valgus of femoral neck
• Persistent hip flexion leads to anteversion of femoral neck
• Results in acetabular dysplasia, hip subluxation & dislocation
Assesment : History
• Look for causes
Abnormal birth history, prematurity, neonatal nursery
• Normal development milestones
Head control, sitting independently, crawling, standing, walking
• Communication
Cognitive function, eye-sight
• Activity daily living
• Mobility
• Walking
Assesment : Examination
• Communication
• Muscles
• Spasticity vs contracture
• Psoas
• Hamstrings
• Adductor
• Quadriceps
• Triceps surae
• Posterior or anterior tibial muscles
• Peroneus
Assesment : Examination
• Neurology
• Gross weakness
• Clasp-knife phenomenon
• Primitive reflexes : Moro reflex, Labyrinthine reflex, Parachute reflex
• Upper limbs
• General conditions
• Look at resting point and contractures
• Asses joint stability
• Control
Treatment
Priorities :
1.Communication
2.Activities of daily living
3.Mobility in enviroment
4.Walking
Treatment
Objectivies :
1.Maintain straight spine and level pelvis
2.Maintain located, mobile, painless hips
3.Maintain plantigrade feet
4.Provide appropriate adaptive equipment, ex. Wheelchairs
5.Recognize and treat medical problems
Management acording to age, motor involvement, assosiate
d handicap, social condition

(0 – 3) years : - physiotherapy
(4 – 6) years : - physiotherapy
- surgery with appropriate indicatio
n
(7 – 8) years : - schooling, psychosocial development
- some surgery
more 18 years : - orientation to work
- residence
- marriage (independent)

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Duchene Muscular Dystrhopy
INTRODUCTION
• Duchenne muscular dystrophy (DMD), included in a group of inflam
matory inherited disorders with progressive degeneration & weaknes
s of skeletal muscle without apparent cause in the peripheral CNS
CLASSIFICATION
SEX LINKED
• Duchenne
• Becker
• Emery-Dreifuss
AUTOSOMAL RECESSIVE
• Limb-girdle
• Infantile fascioscapulohumeral
AUTOSOMAL DOMINANT
• Fascioscapulohumeral
• Distal
• Ocular
• Oculopharyngeal
DEFINITION
• Progressive disease
• X-linked inheritence, affected boys >> with sex chrom
osome disorders
• Gene defect at locus p21
• Characteristic: X chromosome fails to produce dystrop
hin for Ca transport
• Unsuspected till the child start walking
Clinical Features
Genotype of DMD
• Females carry the DMD gene on the X
chromosome.
• Females are carriers and have a 50% chance
of transmitting the disease in each
pregnancy.
• Sons who inherit the mutation will have the
disease.
• Daughters that inherit the mutation will be
carriers.
• The DMD gene is located on the Xp 21 band
of the X chromosome.
• Mutations which affect the DMD gene.
• 96% are frameshift mutations
• 30% are new mutations
• 10-20% of new mutations occur in the
gametocyte (sex cell, will be pass on to the
next generation).
• The most common mutation are repeats of
the CAG nucleotides.
 DMD Pedigree

The X chromosome carrying the disease-causing mutation can be tracked through the family. Note: Shaded squa
res = affected males: dots in circles = carrier females.
EPIDEMIOLOGY
• Affecting 1:3500 male infants
• Start at age 3-5 y.o with symmetric proximal greater than distal weakn
ess in the arms & legs
• By 6-9 y.o develop spesific Gower’s sign
• By 10-12 y.o fail to walk
PATHOLOGY
• Abnormality in gene that produce dystrophin.
• Absence or reduction of dystrophin  destabilization of muscle cells
membrane  allows Creatinine Phospo Kinase (CPK) to leak into seru
m with progressive loss of muscle & replacement by fibro – fatty tissu
e.
Symptoms of DMD
• Delayed developmental milestones

• Loss of motor skills

• Characteristic gait

• Calf hypertrophy

• Clumsiness/frequent falls
More Symptoms of DMD
• Muscle weakness

• Difficulty climbing stairs or hills

• Difficulty rising (Gower’s sign)

• Difficulty walking/running
SPESIFIC SIGN
• Gower’s sign : the child method of rising from the flo
or by climbing up his legs
• Due to weakness of gluteus maximus and thigh muscl
e
• By 10 y.o : unable to walk
• By 20 y.o : cardiac or respiratory failure  death
DIAGNOSTIC
• Test for increased serum CPK : increased 200-400 times
• Characteristic on EMG
• Muscle biopsy for histochemical abnormalities : fibro – fatty tissue, re
placing muscle cells
• Decrased dystrophin ( immunohistochemical )
 Immunolabeling of Muscle Biopsy Sections

Dystrophin antibod
y staining of muscle
cells

Normal Control 4 year old boy with DMD – No detectable

dystrophin
Differential Diagnosis
• Becker’s MD
• Congenital myopathies
• Inflammtory myopathies
• Spinal muscular atrohpies (SMA)
 Treatment
• Prednisone therapy
• Initial dose : 0.75 mg/kg/day
• Weekly dose : 5-10 mg/kg
• Non-surgical treatment of contracture
• Night splints
• Daytime passive stretch
• Surgical treatment of contracture
• Early contracture release
• Archilles tenotomy
• Posterior tibial transfer
• Scoliosis-back bracing
Poliomyelitis
• A disease caused by viral destruction of the anterior horn cells in the s
pinal cord and brain stem motor nuclei
• hallmark is motor weakness with normal sensation
• Virus is a member of the enterovirus subgroup of picornaviridae famil
y
• 3 serotypes
• PV1
• PV2
• PV3
Transmision
• Fecal - oral route
• Oral - oral
Clinical Features
• Incubation 6-20 days
• Flaccid paralysis
• Weaknes or paralysis and reduced muscle tone