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Case 6 / NBSS Bunga Diela

Cerebral Palsy and Mental Retardation 130110120114

Embryology of Cerebral Hemisphere


3rd week
Continue to elevate and Begin in cervical region
Slipper-shaped plate of thickened Elevation of the approach to each other
ectoderm (neural plate) lateral edges
Proceeds in cephalic and
fuse caudal directions

neural folds
neural tubes Open ends of the neural tube
Prosencephalon (forebrain)
4thweek
Dilations Cranial and caudal neuropores
Mesencephalon (midbrain) (primary
brain
vesicles)
Rhombencephalon (hindbrain)

Cervical flexure (at junction of the


hindbrain and the spinal cord)
flexures
Cephalic flexure (in midbrain region)

5thweek

 Cerebral hemisphere as bilateral evaginations of the lateral wall of the prosencephalon


 With further expansion, the hemispheres cover the lateral aspect of the diencephalon,
mesencephalon, and cephalic portion of the metencephalon

7thweek

 Continuous growth of the cerebral hemispheres in anterior, dorsal, and inferior


directions results in the formation of frontal, temporal, and occipital lobes.
 Area between the frontal and temporal lobes becomes depressed and is known as the
insula

9thweek

 During the final part of fetal life, the surface of the cerebral hemispheres grows so
rapidly that a great many convolutions (gyri) separated by fissures and sulci appear on
its surface
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Cerebral Palsy and Mental Retardation 130110120114

Anatomy of Cerebral Hemisphere


Main Sulci
 The central sulcus is of great importance because :
 the gyrus that lies anterior to it contains the motor cells
 posterior to it lies the general sensory cortex that receives sensory information from the opposite side of the body
 The lateral sulcus is a deep cleft found mainly on the inferior and lateral surfaces of the cerebral hemisphere.
 Consists of a short stem that arises on the inferior surface and on reaching lateral surface
 Stem divides into three rami: the anterior horizontal ramus, the anterior ascending ramus, the posterior ramus.
 An area of cortex called the insula lies at the bottom of the deep lateral sulcus and cannot be seen from the surface unless the lips
of the sulcus are separated
 The parieto-occipital sulcus begins on the superior medial margin of the hemisphere about 2 inches (5 cm) anterior to the occipital pole,
passes downward and anteriorly on the medial surface to meet the calcarine sulcus
 The calcarine sulcus is found on the medial surface of the hemisphere. It commences under the posterior end of the corpus callosum
and arches upward and backward to reach the occipital pole, where it stops.

Lobes of cerebral hemisphere


Superolateral Surface of the Hemisphere
 The frontal lobe occupies the area anterior to the central sulcus
and superior to the lateral sulcus.
o The precentral sulcus runs parallel to the central sulcus, and the precentralgyrus lies between them
 The parietal lobe occupies the area posterior to the central sulcus and superior to
the lateral sulcus; it extends posteriorly as far as the parieto-occipital sulcus.
o The postcentral sulcus runs parallel to the central sulcus, and the
postcentralgyrus lies between them.
 The temporal lobe occupies the area inferior to the lateral sulcus.
o The superior and middle temporal sulci run parallel to the posterior ramus
of the lateral sulcus and divide the temporal lobe into the superior,
middle,and inferior temporal gyri
 The occipital lobe occupies the small area behind the parieto-occipital sulcus
Medial and Inferior Surfaces of the Hemisphere
 The corpus callosum, which is the largest commissure of the brain
 The cingulate gyrus
 The paracentral lobule
 The precuneus
 The cuneus
 The collateral sulcus.
 Anterior to the lingual gyrus is the parahippocampalgyrus
 The medial occipitotemporalgyrus
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Cerebral Palsy and Mental Retardation 130110120114

Internal Structure of the Cerebral Hemispheres


 Lateral Ventricle
 Basal Nuclei
The corpus striatum is situated lateral to the thalamus. It is almost completely divided by a band of nerve fibers, the internal
capsule,into the caudate nucleus and the lentiform nucleus.
o The caudate nucleus,large C-shaped mass of gray matter that is closely related to the lateral ventricle, lies lateral to the thalamus.
o The lateral surface of the nucleus is related to the internal capsule, which separates it from the lentiform nucleus
o The lentiform nucleus is a wedge-shaped mass of gray matter whose broad convex base is directed laterally and its blade medially
o The claustrum, in turn, separates the external capsule from the subcortical white matter of the insula
The amygdaloid nucleus is situated in the temporal lobe close to the uncus, considered as a part of limbic system
The claustrum is a thin sheet of gray matter that is separated from the lateral surface of the lentiform nucleus by the external capsule
White matter of cerebral hemisphere
 Commisure fibers
Connect corresponding regions of the two hemispheres: the corpus callosum, the anterior commissure, the posterior commissure, the
fornix, and the habenular commissure
 Association fibers
Connect various cortical regions within the same hemisphere and may be divided into short and long groups.
 The short association fibers lie beneath the cortex and connect adjacent gyri
 The long association fibers are collected into named bundles that can be dissected in a formalin-hardened brain.
 Projection Fibers
Afferent and efferent nerve fibers passing to and from the brainstem to the entire cerebral cortex must travel between large nuclear
masses of gray matter within the cerebral hemisphere. At the upper part of the brainstem, these fibers form a compact band known as
the internal capsule,which is flanked medially by the caudate nucleus and the thalamus and laterally by the lentiform nucleus
 Septum pellucidum
is a thin vertical sheet of nervous tissue consisting of white and gray matter covered on either side by ependyma. It stretches between
the fornix and the corpus callosum. It is essentially a double membrane with a closed, slitlike cavity between the membranes
 The telachoroidea
is a two-layered fold of pia mater. It is situated between the fornix superiorly and the roof of the third ventricle and the upper surfaces
of the two thalami inferiorly.

Histology of Cerebral Hemisphere


Nerve cells of Cerebral cortex
1. Pyramidal cells (giant pyramidal cells : betz cells)
2. Stellate cells (granule cells) : small size, polygonal in shape, short axon,
terminates on nearby neurons
3. Fusiform cells : long axis vertical to the surface and are considerated
mainly in the deepest cortical layers. Inferior dendrite branches within
the same cellular layer, while the superficial layer dendrite ascends
toward the surface of the cortex and branches in the superficial layers.
4. Horizontal cells of Cajar : small, fusiform, horizontally oriented cells in
the most superfiscial layers of the cortex
5. Cells of Martinotti : small, multipolar cells that are present throughout
the levels of the cortex, where it ends in more superfiscial layer
Nerve fibers of Cerebral Cortex
1. The radial fibers. Run at right angles to the cortical surface.
2. The tangential fibers. Run parallel to the cortical surface.
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Cerebral Palsy and Mental Retardation 130110120114

Layers of cerebral cortex


1. Molecular layer (plexiform layer) consist of tangentially oriented nerve fibers.
2. External granular layer. Contains large numbers of small pyramidal cells and stellate cells.
3. External pyramidal layer. Composed of pyramidal cells, whose cell body size increases from the superficial to the deeper borders of layer.
4. Internal granular layer. Composed of closely packed stellate cells. There is a high concentration of horizontally arranged fibers known
collectively as the external band of Baillarger.
5. Ganglionic layer (internal pyramidal layer). Contains very large and medium-size pyramidal cells. Scattered among the pyramidal cells are
stellate cells and cells of Martinotti.
6. Multiform layer (layer of polymorphic cells). Majority of the cells are fusiform, many of the cells are modified pyramidal cells, whose cell
bodies are triangular or ovoid.

Physiology of Cerebral Hemisphere

A. Frontal Lobe
Area Location Function
Precentral Area
primary motor area (Brodmann Posterior region produces isolated movements on the opposite side of the body as
area 4) well as contraction of muscle groups concerned with the performance
of a specific movement., carry out the individual movements of
different parts of the body
premotor area/ secondary motor Anterior region produces muscular movements similar to those obtained by
(Brodmann area 6 and parts of stimulation of the primary motor area; however, stronger stimulation
areas 8, 44, and 45) is necessary to produce the same degree of movement, store
programs of motor activity assembled as the result of past experience
Supplementary motor area In the medial frontal gyrus on the medial
surface of hemisphere and anterior to the
paracentral lobule
Frontal eye field(Broadmann Extends forward from the fascial area of Conjugate movements of eyes, esp toward opposite side
areas 6, 8, and 9) precentralgyrus into middle frontal gyrus
Motor Speech areas of Broca between anterior and ascending rami and Brings about the formation of words by its connections with
(Brodmann areas 44 and 45). ascending the ascending and posterior rami of the adjacent primary motor areas
the lateral fissure
Prefrontal cortex (Brodmann Includes the greater parts of the superior, concerned with the makeup of the individual's personality
areas 9, 10, 11, and 12) middle, and inferior frontal gyri; the orbital
gyri; most of the medial frontal gyrus; and
the anterior half of the cingulate gyrus
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Cerebral Palsy and Mental Retardation 130110120114

B. Parietal Lobe
Area Location Function
Primary Somesthetic Area/Primary somatic Postcentralgyrus receive projection fibers from the ventral posterior lateral
sensory cortex S1 (Broadmann areas 1, 2, 3) and ventral posterior medial nuclei of the thalamus
Secondary somesthetic area/secondary somatic Superior lip of the posterior limb Neurons respond particularly to transient cutaneous stimuli,
sensory cortex S2 of the lateral fissure continue from primary somesthetic area
Somesthetic association area (Broadmann 5 Posterior of primary Receive and integrate different sensory modalities, recognize
and 7) somatosensory area things without seeing

C. Occipital Lobe
Area Location Function
Primary visual area (Brodmann area 17) Walls of the posterior part of the Receive visual impulse
calcarine sulcus
Secondary visual area/ visual association Surrounds the primary visual area Relate the visual information received by the primary visual
(Broadmann area 18 and 19) on the medial and lateral surfaces area to past visual experiences, enabling the individual to
recognize and appreciate what he or she is seeing
Occipital eye fields to be reflex and associated movements of eye when it is
following an object
D. Temporal Lobe
Area Location Function
Primary auditory area (Brodmann area 41 Inferior wall of lateral sulcus Reception of sound
and 42)
Secondary auditory area/ auditory association Posterior to the primary auditory Receives impulses from the primary auditory area and from
(Brodmann area 22) area in the lateral sulcus and in the thalamus
the superior temporal gyrus
Sensory speech area of Wernicke (Brodmann Left dominant hemisphere mainly permits the understanding of the written and spoken
area 22, 39, 40) in the superior temporal gyrus language and enables a person to read a sentence,
understand it, and say it out loud

E. Other Cortical Area


Area Location Function
Primary gustatory area Lower end of the postcentralgyrus in the superior wall of the Taste area
(Brodmann area 43) lateral sulcus and in the adjoining area of the insula.
Secondary olfactory area Smell area
(Brodmann area 28)
Vestibular area Near the part of the postcentralgyrus/ opposite the auditory Sensations of the face
area in the superior temporal gyrus
Insula Buried within the lateral sulcus and forms its floor Planning or coordinating the articulatory
movements necessary for speech

CEREBELLUM
ANATOMY
 The cerebellum is situated in the posterior cranial fossa and is covered superiorly by the tentorium cerebella
(duramater, separates the cerebrum with cerebellum)
 It is the largest part of the hindbrain and lies posterior to the fourth ventricle, the pons, and the medulla
oblongata.
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Cerebral Palsy and Mental Retardation 130110120114

 The cerebellum is ovoid in shape and constricted in its median part. It consists of two cerebellar hemispheres
joined by a narrow median vermis. The cerebellum is connected to the posterior aspect of the brainstem by
three symmetrical bundles of nerve fibers called the superior, middle, and inferior cerebellar peduncles.

The cerebellum is divided into three main lobes:


 The anterior lobe may be seen on the superior surface of the cerebellum and is separated from the
middle lobe by a wide V-shaped fissure called the primary fissure.
 The middle lobe (sometimes called the posterior lobe), which is the largest part of the cerebellum, is
situated between the primary and uvulonodular fissures.
 The flocculonodular lobe is situated posterior to the uvulonodular fissure.
 A deep horizontal fissure that is found along the margin of the cerebellum separates the superior from the
inferior surfaces; it is of no morphologic or functional significance.
 The cerebellum also divided in sagital subdivision: consist of
Vermis in the middle of cerebellum divided it into right and left hemisphere.

HISTOLOGY
The cerebellum is composed of an outer covering of gray matter called the cortex and inner white matter.
Embedded in the white matter of each hemisphere are three masses of gray matter forming the intracerebellar
nuclei.
Cerebellar Cortex
The cerebellar cortex can be regarded as a large sheet with folds lying in the coronal or transverse plane (folium).
The gray matter of the cortex throughout its extent has a uniform structure. It may be divided into three layers:
(1) an external layer, the molecular layer;
The molecular layer contains two types of neurons: the outer stellate cell and the inner basket cell. Neuroglial
cells are found between these structures.
(2) a middle layer, the Purkinje cell layer;
The Purkinje cells are large Golgi type I neurons. They are flask shaped and are arranged in a single layer in a plane
transverse to the folium, the dendrites of these cells are seen to pass into the molecular layer, where they undergo
profuse branching. The primary and secondary branches are smooth, and subsequent branches are covered by
short, thick dendritic spines. It has been shown that the spines form synaptic contacts with the parallel fibers
derived from the granule cell axons.
At the base of the Purkinje cell, the axon arises and passes through the granular layer to enter the white matter.
On entering the white matter, the axon acquires a myelin sheath, and it terminates by synapsing with cells of one
of the intracerebellar nuclei. Collateral branches of the Purkinje axon make synaptic contacts with the dendrites of
basket and stellate cells of the granular layer in the same area or in distant folia. A few of the Purkinje cell axons
pass directly to end in the vestibular nuclei of the brainstem.
(3) an internal layer, the granular layer.
The granular layer is packed with small cells with densely staining nuclei and scanty cytoplasm called granular cell.
Each cell gives rise to four or five dendrites, which make clawlike endings. The axon of each granule cell passes into
the molecular layer. These fibers, known as parallel fibers, run at right angles to the dendritic processes of the
Purkinje cells. Most of the parallel fibers make synaptic contacts with the spinous processes of the dendrites of the
Purkinje cells. Neuroglial cells are found throughout this layer. Scattered throughout the granular layer are Golgi
cells. Their dendrites ramify in the molecular layer, and their axons terminate by splitting up into branches that
synapse with the dendrites of the granular cells.
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Cerebral Palsy and Mental Retardation 130110120114

Intracerebellar Nuclei
Four masses of gray matter are embedded in the white matter of the cerebellum on each side of the midline. From
lateral to medial, these nuclei are
(1) The dentate nucleus is the largest of the cerebellar nuclei. It has the shape of a crumpled bag with the
opening facing medially. The interior of the bag is filled with white matter made up of efferent fibers that
leave the nucleus through the opening to form a large part of the superior cerebellar peduncle.
(2) The emboliform nucleus is ovoid and is situated medial to the dentate nucleus, partially covering its hilus.
(3) The globose nucleus consists of one or more rounded cell groups that lie medial to the emboliform
nucleus.
(4) The fastigial nucleus lies near the midline in the vermis and close to the roof of the fourth ventricle; it is
larger than the globose nucleus.
The intracerebellar nuclei are composed of large, multipolar neurons with simple branching dendrites. The axons
form the cerebellar outflow in the superior and inferior cerebellar peduncles.
White Matter
The white matter is made up of three groups of fibers:
(1) The intrinsic fibers do not leave the cerebellum but connect different regions of the organ.
(2) The afferent fibers(greater part) They enter the cerebellar cortex mainly through the inferior and middle
cerebellar peduncles.
(3) The efferent fibers. The great majority of the Purkinje cell axons pass to and synapse with the
intracerebellarnuclei. The axons of the neurons then leave the cerebellum. A few Purkinje cell axons in the
flocculonodular lobe and in parts of the vermis bypass the cerebellar nuclei and leave the cerebellum
without synapsing.
Fibers from the dentate, emboliform, and globose nuclei leave the cerebellum through the superior cerebellar
peduncle. Fibers from the fastigial nucleus leave through the inferior cerebellar peduncle.
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Cerebral Palsy and Mental Retardation 130110120114

Cerebellar Peduncles
The cerebellum is linked to other
parts of the central nervous system by
numerous efferent and afferent fibers
that are grouped together on each
side into three large bundles, or
peduncles. The superior cerebellar
peduncles connect the cerebellum to
the midbrain, the middle cerebellar
peduncles connect the cerebellum to
the pons, and the inferior cerebellar
peduncles connect the cerebellum to
the medulla oblongata.

PHYSIOLOGY
The cerebellum plays a very important role in the control of posture and voluntary movements. Also as a
coordinator of precise movements by continually comparing the output of the motor area of the cerebral cortex
with the proprioceptive information received from the site of muscle action; it is then able to bring about the
necessary adjustments by influencing the activity of the lower motor neurons. This is accomplished by controlling
the timing and sequence of firing of the alpha and gamma motor neurons. It is also believed that the cerebellum
can send back information to the motor cerebral cortex to inhibit the agonist muscles and stimulate the antagonist
muscles, thus limiting the extent of voluntary movement. Each cerebellar hemisphere controls muscular
movements on the same side of the body. Important in balancing, cognitive, and language.
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Cerebral Palsy and Mental Retardation 130110120114

Anatomy and Physiology of Brain Stem


- Brainstem is part of brain between spinal cord and
diencephalon
- It is made up of medulla oblongata, pons, and midbrain
occupies the posterior cranial fossa of the skull

Medulla Oblongata

- Continuous with the superior part of the spinal cord and forms the inferior part of the brain stem
- Begins at the foramen magnum and extends to the inferior border of the pons
- Medulla’s white matter contains all sensory (ascending) tracts and motor (descending) tracts that extend between the spinal cord and
other parts of the brain.
- Some of the white matter forms bulges on the anterior aspect of the medulla. These protrusions, called the pyramids, are formed by the
large corticospinal tracts that pass from the cerebrum to the spinal cord. Just superior to the junction of the medulla with the spinal
cord, 90% of the axons in the left pyramid cross to the right side, and 90% of the axons in the right pyramid cross to the left side.
This crossing is called the decussation of pyramids
- Just lateral to each pyramid is an oval-shaped swelling called an olive).
- Within the olive is the inferior olivary nucleus, which receives input from the cerebral cortex, red nucleus of the midbrain, and spinal
cord. Neurons of the inferior olivary nucleus extend their axons into the cerebellum, where they regulate the activity of cerebellar
neurons. By influencing cerebellar neuron activity, the inferior olivary nucleus provides instructions that the cerebellum uses to make
adjustments to muscle activity as you learn new motor skills.
- The medulla also contains several nuclei. Some of these nuclei control vital body functions.
o The cardiovascular center regulates the rate and force of the heartbeat and the diameter of blood vessels
o The medullary rhythmicity area of the respiratory center adjusts the basic rhythm of breathing normal breathing rhythm
o The vomiting center of the medulla causes vomiting
o The deglutition center of the medulla promotes swallowing
o Sneezing involves spasmodic contraction of breathing muscles
o Coughing involves a long drawn and deep inhalation
o Hiccupping is caused by spasmodic contractions of the diaphragm
- Nuclei associated with sensations of touch, pressure, vibration, and conscious proprioception are located in the posterior part of the
medulla. These nuclei are the right and left gracile nucleus and cuneate nucleus.
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Cerebral Palsy and Mental Retardation 130110120114

- Ascending sensory axons of the gracile fasciculus and the cuneate fasciculus, which are two tracts in the posterior columns of the spinal
cord, form synapses in these nuclei. Postsynaptic neurons then relay the sensory information to the thalamus on the opposite side of the
brain. The axons ascend to the thalamus in a band of white matter called the medial lemniscus, which extends through the medulla,
pons, and midbrain. The tracts of the posterior columns and the axons of the medial leminiscus are collectively known as the posterior
column–medial lemniscus pathway.
- The medulla also contains nuclei that are components of sensory pathways: gustation (taste), audition (hearing), and equilibrium
(balance).
o The gustatory nucleus of the medulla is part of the gustatory pathway from the tongue to the brain; it receives gustatory input from
the taste buds of the tongue
o The cochlear nuclei of the medulla are part of the auditory pathway from the inner ear to the brain; they receive auditory input
from the cochlea of the inner ear
o The vestibular nuclei of the medulla and pons are components of the equilibrium pathway from the inner ear to the brain; they
receive sensory information associated with equilibrium from proprioceptors in the vestibular apparatus of the inner ear.
- Finally, the medulla contains nuclei associated with five pairs of cranial nerves: vestibulocochlear (VIII) nerves, glossopharyngeal (IX)
nerves, vagus (X) nerves, accessory (XI) nerves (cranial portion), and hypoglossal (XII) nerves.
Pons
- Lies directly superior to the medulla and anterior to the cerebellum
- The pons is a bridge that connects different parts of the brain with one
another. These connections are provided by bundles of axons.
- Like the medulla, the pons consists of nuclei, sensory tracts, and motor
tracts.
o Signals for voluntary movements from motor areas of the cerebral cortex
are relayed through several pontine nuclei into the cerebellum.
o Along with the medulla, the pons contains vestibular nuclei that are
components of the equilibrium pathway from the inner ear to the brain.
o Other nuclei in the pons are the pneumotaxic area and the apneustic
area of the respiratory center. Together with the medullary rhythmicity
area, the pneumotaxic and apneustic areas help control breathing.
- The pons also contains nuclei associated with the following four pairs of
cranial nerves: trigeminal (V) nerves, abducens (VI) nerves, facial (VII) nerves,
and vestibulocochlear (VIII) nerves.

Midbrain
- The midbrain extends from the pons to the diencephalon
- The aqueduct of the midbrain (cerebral aqueduct) passes
through the midbrain, connecting the third ventricle above with
the fourth ventricle
- Like the medulla and the pons, the midbrain contains both
nuclei and tracts.
- The anterior part of the midbrain contains paired bundles of
axons known as the cerebral peduncles. The cerebral peduncles
consist of axons of the corticospinal, corticopontine, and
corticobulbar tracts, which conduct nerve impulses from motor
areas in the cerebral cortex to the spinal cord, pons, and
medulla, respectively.
- The posterior part of the midbrain, called the tectum, contains
four rounded elevations
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- The two superior elevations, nuclei known as the superior colliculi,


serve as reflex centers for certain visual activities. Through neural
circuits from the retina of the eye to the superior colliculi to the
extrinsic eye muscles, visual stimuli elicit eye movements for tracking
moving images (such as a moving car) and scanning stationary
images (as you are doing to read this sentence). The superior
colliculi are also responsible for reflexes that govern movements of
the head, eyes, and trunk in response to visual stimuli.
- The two inferior elevations, the inferior colliculi, are part of the
auditory pathway, relaying impulses from the receptors for hearing
in the inner ear to the brain. These two nuclei are also reflex
centers for the startle reflex, sudden movements of the head, eyes,
and trunk that occur when you are surprised by a loud noise
- The midbrain contains several other nuclei:
o Left and right substantianigra, which large and darkly pigmented
o Left and right red nuclei, which look reddish due to their rich
blood supply and an iron-containing pigment in neuronal cell bodies
- Still other nuclei in the midbrain are associated with two pairs of cranial nerves: oculomotor (III) nerves and trochlear (IV) nerves
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CHILD DEVELOPMENT AND BEHAVIOUR


Definition
Development is the process of growth and differentiation in all aspect of child’s life.
Behavior is all of a person’s total activity, especially that which is externally observable.

Basic Principle of Child Development and Behavior


1. Development proceeds from the head downward.
2. Development proceeds from the center of the body outward.
3. Development depends on maturation and learning.
4. Development proceeds from the simple (concrete) to the more complex
5. Growth and development is a continuous process.
6. Growth and development proceed from the general to specific
7. There are individual rates of growth and development.

Development itu bersifat continuous: sepanjang umur anak, perkembangan selalu berjalan, tidak pernah terputus.
Dan development juga sangat berkaitan dengan central nervous system, sehingga impaired brain pasti akan
memberikan efek pada delayed development and behavior.

Influencing Factors of Growth And Development


1. Biological factors:
Something that comes from inside of the body, for example genetic, postpartum illness.
2. Psychological influences
Attachment: Tendency to have a dependency
Contingency: Responses to nonverbal gesture
3. Social factors
Family system and ecologist model

Prenatal Factors.
- Maternal depression, during pregnancy and in the postpartum period, undermines the mother-child
relationship, and may threaten the infant's cognitive and emotional development.
- Conflict with the father during pregnancy may diminish the mother's ability to become absorbed with her infant.

Peripartum and Postpartum Influences.


- The continuous presence during labor of a woman trained to offer friendly support and encouragement results
in shorter labor, fewer obstetric complications, and reduced postpartum hospital stays.
- Skin-to-skin contact between mothers and infants immediately after birth may correlate with an increased rate
and longer duration of breast-feeding.
- Most new parents value even a brief period of uninterrupted time in which to get to know their new infants, and
increased mother-infant contact over the first days of life may improve long-term mother-child interactions.
Nonetheless, early separation, although predictably very stressful, does not inevitably impair a mother's ability
to bond with her infant. Early discharge home from the maternity ward may undermine bonding, particularly
when a new mother is required to resume full responsibility for a busy household.

Period of Child Development and Behavior


1. Prenatal period
2. First year of life period
3. Second year of life period
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1. Prenatal Period
* Milestones of Prenatal Development
Week Developmental Events
1 Fertilization and implantation; beginning of embryonic period (embryo consists of blastocyst)
2 Endoderm and ectoderm appear (bilaminar embryo)
3 Mesoderm appears (trilaminar embryo); somites begin to form; Heart tubes have begun to pump
4 Neural folds fuse; folding of embryo into human-like shape; arm and leg buds appear; CRL 4-5 mm
5 Lens placodes, primitive mouth, digital rays on hands
6 Primitive nose, philtrum, primary palate; CRL 21–23 mm
7 Eyelids begin
8 Ovaries and testes distinguishable
9 Fetal period begins – Start to changes in body proportion*; CRL 5 cm; weight 9 g
10 External genitals distinguishable; The face is recognizably human; Lung development proceeds
20 Usual lower limit of viability; primitive alveoli have formed and surfactant production has begun; weight
460 g; length 19 cm
25 Third trimester begins; weight 900 g; length 25 cm
28 Eyes open; fetus turns head down; weight 1,300 g
38 Term
* Changes in body proportion from the fetal to adulthood

a. Neurologic Development

3rd Week : Neural plate appears on Neural Tube Central Nervous System (CNS)
infolding
the ectodermal surface
of the trilaminar embryo Neural Crest Peripheral Nervous System (PNS)

Neuroectoderm differentiates to neurons, astrocytes, oligodendrocytes, and ependymal cells


Mesoderm differentiates to microglial cells

5th week :
- The three main subdivisions of forebrain, midbrain, and hindbrain are evident.
- The dorsal and ventral horns of the spinal cord have begun to form.
- Myelinization begins and continues throughout the 1st 2 yr of life.
th
8 week :
- The gross structure of the nervous system has been established*.
- The growth of axons and dendrites and the elaboration of synaptic connections continue at a rapid pace,
making the CNS vulnerable to teratogenic or hypoxic influences throughout gestation.
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Cerebral Palsy and Mental Retardation 130110120114

b. Behavioral Development.
- 8th week : Muscle contraction first appear, soon followed by lateral flexion movements.
- 13–14th week : Breathing and swallowing motions appear and tactile stimulation elicits graceful movements.
- 17th week : The grasp reflex appears at (and is well developed by 27th week).
- 26th week : Eye opening.
- 2nd trimester : The full range of neonatal movements can be observed.
- 3rd trimester : fetuses respond to external stimuli with heart rate elevation and body movements.

*Fetal behavior
- Fetal behavior is affected by maternal medications and diet, and may be the mother's diurnal rhythms.
- Reactivity to auditory (vibroacoustic) and visual (bright light) stimuli vary depending on their behavioral
state (characterized as quiet sleep, active sleep, and awake).
- Fetal movement increases in response to a sudden auditory tone, but decreases after several repetitions
(habituation – as a form of learning). This ability is diminished in neurologically impaired fetuses.

* Illustration of the sensitive or critical periods in prenatal development (in respons to teratogens)

2. The First Year


* Patterns of Behavior During the First Year of Life
Neonatal Period (1st 4 Wk)
Prone: Lies in flexed attitude; turns head from side to side; head sags on ventral suspension
Supine: Generally flexed and a little stiff
Visual: May fixate face or light in line of vision; “doll's-eye” movement of eyes on turning of the body
Reflex: Moro response active; stepping and placing reflexes; grasp reflex active
Social: Visual preference for human face
At 4 Wk
Prone: Legs more extended; holds chin up; turns head; head lifted momentarily to plane of body on ventral
suspension
Supine: Tonic neck posture predominates; supple and relaxed; head lags on pull to sitting position
Visual: Watches person; follows moving object
Social: Body movements in cadence with voice of other in social contact; beginning to smile
Case 6 / NBSS Bunga Diela
Cerebral Palsy and Mental Retardation 130110120114

At 8 Wk
Prone: Raises head slightly farther; head sustained in plane of body on ventral suspension
Supine: Tonic neck posture predominates; head lags on pull to sitting position
Visual: Follows moving object 180 degrees
Social: Smiles on social contact; listens to voice and coos
At 12 Wk
Prone: Lifts head and chest, arms extended; head above plane of body on ventral suspension
Supine: Tonic neck posture predominates; reaches toward and misses objects; waves at toy
Sitting: Head lag partially compensated on pull to sitting position; early head control with bobbing motion;
back rounded
Reflex: Typical moro response hasn’t persisted; make defensive movement or selective withdrawal reaction
Social: Sustained social contact; listens to music; says “aah, ngah”
At 16 Wk
Prone: Lifts head and chest, head in approximately vertical axis; legs extended
Supine: Symmetric posture predominates, hands in midline; reaches and grasps objects and brings them
to mouth
Sitting: No head lag on pull to sitting position; head steady, tipped forward; enjoys sitting with full truncal
support
Standing: When held erect, pushes with feet
Adaptive: Sees pellet, but makes no move to it
Social: Laughs out loud; may show displeasure if social contact is broken; excited at sight of food
At 28 Wk
Prone: Rolls over; pivots; crawls or creep-crawls (Knobloch)
Supine: Lifts head; rolls over; squirming movements
Sitting: Sits briefly, with support of pelvis; leans forward on hands; back rounded
Standing: May support most of weight; bounces actively
Adaptive: Reaches out for and grasps large object; transfers objects from hand to hand; grasp uses radial
palm; rakes at pellet
Language: Polysyllabic vowel sounds formed
Social: Prefers mother; babbles; enjoys mirror; responds to changes in emotional content of social
contact
At 40 Wk
Sitting: Sits up alone and indefinitely without support, back straight
Standing: Pulls to standing position; “cruises” or walks holding on to furniture
Motor: Creeps or crawls
Adaptive: Grasps objects with thumb and forefinger; pokes at things with forefinger; picks up pellet with
assisted pincer movement; uncovers hidden toy; attempts to retrieve dropped object; releases
object grasped by other person
Language: Repetitive consonant sounds (mama, dada)
Social: Responds to sound of name; plays peek-a-boo or pat-a-cake; waves bye-bye
Case 6 / NBSS Bunga Diela
Cerebral Palsy and Mental Retardation 130110120114

At 52 Wk (1 Yr)
Motor: Walks with one hand held (48 wk); rises independently, takes several steps (Knobloch)
Adaptive: Picks up pellet with unassisted pincer movement of forefinger and thumb; releases object to other
person on request or gesture
Language: A few words besides “mama,” “dada”
Social: Plays simple ball game; makes postural adjustment to dressing

3. The Second Year


* Patterns of Behavior During the Second Year of Life
15 Mo
Motor: Walks alone; crawls up stairs
Adaptive: Makes tower of 3 cubes; makes a line with crayon; inserts pellet in bottle
Language: Jargon; follows simple commands; may name a familiar object (ball)
Social: Indicates some desires or needs by pointing; hugs parents
18 Mo
Motor: Runs stiffly; sits on small chair; walks up stairs with one hand held; explores drawers and
wastebaskets
Adaptive: Makes a tower of 4 cubes; imitates scribbling; imitates vertical stroke; dumps pellet from bottle
Language: 10 words (average); names pictures; identifies one or more parts of body
Social: Feeds self; seeks help when in trouble; may complain when wet or soiled; kisses parent with
pucker
24 Mo
Motor: Runs well, walks up and down stairs, one step at a time; opens doors; climbs on furniture; jumps
Adaptive: Tower of 7 cubes (6 at 21 mo); circular scribbling; imitates horizontal stroke; folds paper once
imitatively
Language: Puts 3 words together (subject, verb, object)
Social: Handles spoon well; tells immediate experiences; helps to undress; listens to stories with pictures
Case 6 / NBSS Bunga Diela
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SUMMARY: Developmental Milestones in the First Two Year of Life


Average Age of
Milestone Developmental Implications
Attainment (mo)
Gross Motor
Head steady in sitting 2.0 Allows more visual interaction
Pull to sit, no head lag 3.0 Muscle tone
Hands together in midline 3.0 Self-discovery
Asymmetric tonic neck reflex gone 4.0 Child can inspect hands in midline
Sits without support 6.0 Increasing exploration
Rolls back to stomach 6.5 Truncal flexion, risk of falls
Walks alone 12.0 Exploration, control of proximity to parents
Runs 16.0 Supervision more difficult
Fine Motor
Grasps rattle 3.5 Object use
Reaches for objects 4.0 Visuomotor coordination
Palmar grasp gone 4.0 Voluntary release
Transfers object hand to hand 5.5 Comparison of objects
Thumb-finger grasp 8.0 Able to explore small objects
Turns pages of book 12.0 Increasing autonomy during book time
Scribbles 13.0 Visuomotor coordination
Builds tower of two cubes 15.0 Uses objects in combination
Builds tower of six cubes 22.0 Uses visual, gross, and fine motor coordination
Communication and Language
Smiles in response to face, voice 1.5 Child more active social participant
Monosyllabic babble 6.0 Experimentation with sound, tactile sense
Inhibits to “no” 7.0 Response to tone (nonverbal)
Follows one-step command with gesture 7.0 Nonverbal communication
Follows one-step command without gesture 10.0 Verbal receptive language (e.g., “Give it to me”)
Speaks first real word 12.0 Beginning of labeling
Speaks 4–6 words 15.0 Acquisition of object and personal names
Speaks 10–15 words 18.0 Acquisition of object and personal names
Speaks two-word sentences (e.g., “Mommy shoe”) Beginning grammaticization, corresponds with 50+
19.0
word vocabulary
Cognitive
Stares momentarily at spot where object Lack of object permanence (out of sight, out of mind)
2.0
disappeared (e.g., yarn ball dropped)
Stares at own hand 4.0 Self-discovery, cause and effect
Bangs two cubes 8.0 Active comparison of objects
Uncovers toy (after seeing it hidden) 8.0 Object permanence
Egocentric pretend play (e.g., pretends to drink Beginning symbolic thought
12.0
from cup)
Uses stick to reach toy 17.0 Able to link actions to solve problems
Pretend play with doll (gives doll bottle) 17.0 Symbolic thought
Case 6 / NBSS Bunga Diela
Cerebral Palsy and Mental Retardation 130110120114

Aspect or Domain of Child Development

A. Psychoanalytic
The psychosexual stages of development by Sigmund Freud
Freud outlined a path that children travel as they progress from autoerotic sexual activity to reproductive activity. He believed,
children move fromautoeroticism to reproductive sexuality and develop their adult personality
1. Oral stage
The earliest stage of development in which the infant’s needs, percepptions, and modes of expression are centered in
the oral zone (mouth, tongue, lips, an others). Occurs during the first 18 months of life.
Character traits : provides a basis in character forcapacities to give and receive from others without excessive
dependence or envy and a capacity to rely on others with sense of trust
2. Anal stage
The stage that is prompted by maturation of neuromuscular control over spinchter, particularly the anal spinchter.
Thus permitting more voluntary control over retention or expulsion of feces. This period extends from 1 year until 3
years of age.
Character traits : provides the basis for the development of personal autonomy, independet, initiative without guilt,
self-determining behavior without shame or doubt.
3. Urethral stage
The stage is envisioned as a transitional stage between the anal and phallic stages.
Character traits : provides a sense of pride and self-competence derived from performance, budding gender identity
and subsequent identification
4. Phallic stage
This stage begins during the third year until the end of fifth year. The phase that is characterized by primary focus of
sexual interests, stimulation, and excitement in the genital area
Character traits : provides the foundations for an emerging sense of sexual identity, sense of curiosity without
embarrasment,
5. Latency stage
The stage of relative quiescence or inactivity of the sexual drive during the period until pubescence (from about 5-6
years until 11-13 years)
Character traits : provides the child to have a capacity for mastery of objects and concepts that allows autonomous
function with a sense of initiative withoyt running the risk of failure or defeat a sense of inferiority.
6. Genital stage
This stage extends from the onset of puberty from ages 11-13 until the person reaches young adulthood.
Character traits : provides fully mature personality with a capacity for full and a self integrity and consistent sense of
identity

Psychological development and crisis theory by Erik Erikson


There are eight stages of the life cycle that represent point along a continuum of development in which physical, cognitive,
instinctual, and sexual changes combine to trigger an interal crisis whose resolution results in either psychosocial regression or
growth and the development of spesific virtue.
stage Associated virtue Psychological crisis
1 Birth-18 months Hope Trust vs. mistrust
2 18 months-3 years Will Autonomy vs. Shame and doubt
3 3 years-5 years Purpose Initiative vs. Guilt
4 5 years-13 years Competence Industry vs. Inferiority
5 13 years-21 years Fidelity Identity vs. Role confusion
6 21 years-40 years Love Intimacy vs. Isolation
7 40 years-60 years Care Generativity vs. Stagnation
8 60 years-death wisdom Integrity vs. despair
Case 6 / NBSS Bunga Diela
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B. COGNITIVE
Cognition is a group of mental processes that include attention, learning, memory, producing and
understanding language, reasoning, problem solving, and decision making. Innate cognitive ability
also known as intelligence, define as aggregate or global capacity of an individual to act purposely,
think rationally, and deal effectively with his/her enviroment. Global means that intelligence is
characterized as individual’s behaviour as a whole, and aggregate means that intelligence is
composed of a number of independent and qualitatively distinguishable cognitive abilities.

DEVELOPMENT OF COGNITIVE STAGE


Cognitive develops in human lifetime :
 Sensorimotor stage (develop in infancy period)
Intelligence is present; motor activity but no symbols; knowledge is developing yet limited;
knowledge is based on experiences/ interactions; mobility allows child to learn new things; some
language skills are developed at the end of this stage
 Pre-operational stage (develop in toddler – early childhood period)
Symbols or language skills are
present; memory and
imagination are developed;
nonreversible and nonlogical
thinking; egocentric thinking
predominates
 Concrete operational
stage (elementary – early
adolescemce period)
Logical and systematic form of
intelligence; manipulation of
symbols related to concrete
objects; operational thinking
predominates nonreversible
and egocentric thinking
 Formal operational stage
(adolescence–adulthood)
Logical use of symbols related to abstract concepts; egocentric thinking comes back early in this
stage; formal thinking is uncommon
Cortex Area For Cognitive
PREFRONTAL AREA has its own function from intelligence :
 Control behavior ventral part of frontal lobe (part of limbic association cortex)
 Completing goals
 Concept of working memory
Neurotransmitter In Cognitive Ability
 cholinergic agonists  Enhance cognitive ability
 cholinergic antagonist (reduce effect of acetylcholine)  Impair cognitive ability
Case 6 / NBSS Bunga Diela
Cerebral Palsy and Mental Retardation 130110120114

SCREENING AND MONITORING CHILD GROWTH AND DEVELOPMENT


GROWTH ASSESSMENT
- Is an essential component of pediatric health surveillance because almost any problems within the physiologic,
interpersonal, and social domains can adversely affect growth
- The most powerful tool in growth assessment is the growth chart
- Other indices of growth:
 Body proportions: can be assessed by measuring the lower body segment and the upper body segment
 Skeletal maturation: Bone age correlates well with stage of pubertal development and can be helpful in
predicting adult height in early or late maturing adolescents
 Dental development: Includes mineralization, eruption, and exfoliation
GROWTH MONITORING
- Basis of comprehensive child health care
- Includes the regular measurement of weight (and sometimes length) of the child
- Weighing starts at birth and continues until the child is 5 y.o. Ideally the child weight must be taken once a
month until the age of 2 years and then thrice a month until the child is 5 y.o
- Weight is also checked against the growth chart to see if weight falls within the acceptable range for child’s age
- If the child is underweight, food supplements are available at clinics or the child is referred to secondary or
tertiary level hospital

DEVELOPMENTAL ASSESSMENT
- Includes early identification of problems through screening and surveillance, and more definitive assessment
including both standardized and nonstandardized measures
- The goal of developmental assessment is not only to generate a diagnosis, but equally important to analyze the
pattern of strengths and weakness in the child, family, and available developmental, educational, and social
support systems in order to direct treatment
DEVELOPMENTAL SURVEILLANCE
- Surveillance has been put forward as an antidote to the shortcomings of developmental screening
- A with physical growth, a series of observations made over time provides much more information rather than an
assessment at single time point, allowing for estimation of developmental rate
- Prediction is more accurate when it makes use of sources of information, includes medical and social histories
- Concept of developmental surveillance: a process that includes regular elicitation of the developmental history,
attention to parental concerns, careful development observations, and promotion of development
DEVELOPMENTAL SCREENING
- Refers to the administration of brief, standardized and validated instruments that have been researched for
their sensitivity in the detection of children with probable problems and their specificity in determining when
children probably do not have problems
- Standards for screening test accuracy require 70-80% sensitivity and specificity
- There are many screening and assessment instruments that can completed by family, other caregiver or
professional doctor
- Doesn’t give a diagnosis, but identifies areas in which a child’s development differs from same-age norms
- Screening of all children regardless of concern is called universal screening
- Quality screening principles: * Screening provides useful information about a child’s development
* Screening involves the use of a standardized tool and is an ongoing process
* Screening takes into account a child’s and family’s individual circumstances
* Screening is culturally sensitive
* Screening requires follow up
Case 6 / NBSS Bunga Diela
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SUMMARY: Child Growth and Development Screening and Monitoring Tools

Instrument Age Range Time Notes


(min)
Denver-II 0–6 yr 30–45 Better sensitivity for language delays than older version
Early Screening 3–6 yr 15–20 A quick, multidomain screen with good sensitivity and
Inventory specificity compared with McCarthy Scales (a well-accepted
test)
ELM 0–3 yr 5–10 Well-normed, quick screen for expressive, receptive, and visual
language; very useful in infancy; does not assess other
domains
CAT/CLAMS 0–3 yr 10–20 CLAMS alone gives quick language quotient; CAT/CLAMS
correlates well with Bayley (traditional gold standard)
Ages and Stages 4–48 mo 15–20 Series of self-administered questionnaires; multiple domains;
Questionnaires (ASQ) good sensitivity and specificity; designed for ages that fall
between usual pediatric schedule (e.g., 4, 8, 16 months)
PEDS 0–8 yr <5 Well-documented psychometrics; can be self- administered by
parents
PEER, PEEX, and 4–15 yr 45 Rich source of observations and analysis of school- related
PEERAMID (different functions, weak on validation
tests)
Adapted from Blackman JA: Developmental screening: Infants, toddlers, and preschoolers. In Levine MD, Carey WB,
Crocker AC (editors): Developmental-Behavioral Pediatrics. Philadelphia, WB Saunders, 1992, pp 617–623.
Case 6 / NBSS Bunga Diela
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CEREBRAL PALSY
Definition
A group of disorders of the development of movement and posture causing activity limitations that are
attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The
motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition,
communication, perception, and/or behavior and/or a seizure disorder.
Epidemiology
- Cerebral palsy is the leading cause of childhood disability affecting function and development.
- In developed countries, the estimated prevalence of cerebral palsy is 2-2.5 cases per 1000 live births.
- In the developing countries, the estimated prevalence are 1.5-5.6 cases per 1000 live births.
- All races are affected. But the risk may increased in lower socioeconomic status and male sex.
- This initiating event occurs between prenatal development and age 3 years. Children are usually not
diagnosed until after age 1 year, when children fail to meet developmental milestones.
- 30-50% of patients with cerebral palsy have mental retardation, depending on the type.
Classification (according to the topographic predominance: resting tone and what limbs are involved)
Type Clinical manifestation Cause
Spastic cerebral palsy Increased muscle tone Results from motor neuron
(80%) Prolonged primitive reflexes involvement and injury to the cerebral
Exaggerated deep tendon reflexes cortex in one or both hemispheres
Rigidity of the extremities, scoliosis and
contractures
Extrapyramidal or Difficulty in fine motor coordination and Injury to the basal ganglia or
Dyskinetic cerebral palsy purposeful movements extrapyramidal tracts
(10-15%) Jerky, uncontrolled and abrupt
movements
Ataxic cerebral palsy Gait disturbance Damage at cerebellum
(<5%) Instability
Hypotonia at birth but stiffness of the
trunk muscles develops by late infancy
* Many patients have characteristics of both spastic and extra-pyramidal cerebral palsy

Etiology
- Brain injury or abnormal brain development:
* Cerebral injury before the 20th week of gestation can result in a neuronal migration deficit
* Cerebral injury between the 26th and 34th weeks can result in periventricular leukomalacia
* Cerebral injury between the 34th and 40th weeks can result in focal or multifocal cerebral injury:
- The physical stress on premature infants and the immaturity of the brain and cerebral vasculature
* Results in spastic diplegia due to the tendency for hypoperfusion to the periventricular white matter,
which can cause germinal matrix hemorrhages or periventricular leukomalacia that carry fibers
responsible for the motor control and muscle tone of the legs.
- Periventricular leukomalacia:
* Asymmetric injury to the periventricular white matter can result in one side of the body being more
affected than the other, characterized as an asymmetric spastic diplegia.
- Periventricular hemorrhage – intraventricular hemorrhage
- Term cerebral vascular and hypoperfusion injuries
* Vascular injuries in the middle cerebral artery, resulting in a spastic hemiplegic cerebral palsy
* Brain hypoperfusion, resulting in spastic quadriplegic cerebral palsy
* The basal ganglia also can be affected, resulting in extrapyramidal or dyskinetic cerebral palsy
Case 6 / NBSS Bunga Diela
Cerebral Palsy and Mental Retardation 130110120114

Risk Factor
a. Prenatal: b. Perinatal:
- Maternal - Anasthesia during labor and delivery
* Metabolic disease - Mechanical trauma during delivery
* Nutritional deficiencies - Immaturity at birth
* Twin or multiple births - Metabolic disorders
* Blood incompabilities - Electrolyte disturbances
* Exposure to radiation c. Postnatal:
* Infection - Head trauma
- Prematurity - Infections
* Premature labor - Cerebrovascuar accidents
- Genetic factors - Toxicosis
* Absence of corpus callosum - Enviromental toxins
- Congenital Anomalies of the brain

Clinical Characteristic
TYPE OF CEREBRAL PALSY CLINICAL CHARACTERISTIC
Spastic hemiplegia (20-30%) - Cognitive impairment
Cerebral palsy affects 1 side of the body, - Arm generally affected more than leg
including an arm and a leg, with involvement of - Impaired sensation and position sense
upper extremity spasticity more than lower - Possible unilateral sensory deficits
extremity spasticity. - One-sided upper motor neuron deficit
- Visual-field deficits and strabismus
Spastic diplegia (30-40%) - Cognitive impairment.
Cerebral palsy affecting bilateral lower - Upper motor neuron findings in legs more than arms
extremities more than upper extremities - Hypotonia and extensor spasticity in lower extremities.
- Have a delay in developing gross motor skills.
- Spastic muscle imbalance
Spastic quadriplegia (10-15%) - Cognitive impairment and seizures
Cerebral palsy affecting all 4 extremities and the - Legs generally affected equally or more than arms All
trunk (full body). But categorized as double limbs affected, either full-body hypertonia or truncal
hemiplegic if arms more involved than legs hypotonia with extremity hypertonia
Dyskinetic cerebral palsy - Intelligence is normal.
Cerebral palsy with extrapyramidal signs - Arms more affected than legs
characterized by abnormal movements and - Extrapyramidal movement patterns, abnormal regulation
hypertonicity. of tone, abnormal postural control & coordination deficit
- A high incidence of sensorineural hearing loss.
- Hypotonia with movement disorder emerging at 1-3yo
- Deep tendon reflexes usually normal to slightly increased
- Some spasticity, gait difficulties, truncal instability
- Defects in postural control and motor dysfunction
Mixed cerebral palsy
Cerebral palsy with no single specific tonal
quality predominating; typically characterized by
a mixture of spastic and dyskinetic components
Hypotonic cerebral palsy (rare)
Cerebral palsy with truncal and extremity
hypotonia with hyperreflexia and persistent
primitive reflex.
Monoplegia (rare)
Involvement is noted in 1 limb, either an arm or
a leg.
Case 6 / NBSS Bunga Diela
Cerebral Palsy and Mental Retardation 130110120114

Diagnostic Tools (Neurological Examination and Radiology Findings)


Despite the fact that the lesion to the developing brain occurs before age 3 years, the diagnosis of
cerebral palsy may not be made until after that time.

NEUROLOGICAL EXAM:
1. General appearance, including posture and motor activities Suspect test results:
2. A speech and mini-mental status exam - lack of or diminished sociability
3. Cranial Nerve testing - no or little response to sounds
4. Motor System, including atrophy, tone, and strength - jerky body movements
5. Sensory System, including vibration and pin-prick - hyper-reflexia
6. Reflexes, including deep tendon and the plantar reflex - fisting of one or both hands
7. Coordination and gait tests - retention of the thumb in the fist

LABORATORY STUDIES:
No definitive laboratory studies to diagnose condition, only studies to rule out other symptom causes:
- Thyroid function studies: abnormal thyroid function may be related to abnormalities in muscle tone or
deep tendon reflexes or to movement disorders.
- Lactate and pyruvate levels: abnormalities may indicate an abnormality of energy metabolism
- Ammonia levels: elevated ammonia levels may indicate liver dysfunction or urea cycle defect.
- Organic and amino acids: Serum quantitative amino acid and urine quantitative organic acid values
may reveal inherited metabolic disorders.
- Chromosomal analysis (karyotype analysis and specific DNA testing: indicated to rule out a genetic
syndrome, if dysmorphic features or abnormalities of various organ systems are present.
- Cerebrospinal protein: levels may assist in determining asphyxia in the neonatal period; protein levels
can be elevated, as can the lactate-to-pyruvate ratio.

IMAGING STUDIES:
- Cranial ultrasonography: Can be performed in the early neonatal period to delineate clear-cut
structural abnormalities and show evidence of hemorrhage or hypoxic-ischemic injury.
- Computed tomography scanning of the brain: In infants, helps to identify congenital malformations,
intracranial hemorrhage, and periventricular leukomalacia or early craniosynostosis.
- Magnetic resonance imaging of the brain: defines cortical and white matter structures, and
abnormalities more clearly than does any other method; also allows for the determination of whether
appropriate myelination is present for a given age
- Electroencephalography: Important in the diagnosis of seizure disorders
- Electromyography and nerve conduction studies: Helpful when a muscle or nerve disorder is suspected

Differential Diagnosis
- Inherited Metabolic Disorders
- Metabolic Myopathies
- Metabolic Neuropathy
- Movement Disorders in Individuals with Developmental Disabilities
- Traumatic Peripheral Nerve Lesions
- Tumors of the Conus and Cauda Equina
- Vascular Malformations of the Spinal Cord
Case 6 / NBSS Bunga Diela
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Principal Management
THERAPY
- Physical Therapy
* Training in right position and alternative movement like walking and strengthening wheelchair.
- Occupational Therapy
* Increasing self-supporting by fine motoric skill.
* Helping children using adaptive tools like eating, sitting and going to bathroom.
- Speech Therapy
* Increasing communication, help them to communicate by verbal/non-verbal.
- Education
* Parents must be tell how to treat their children in daily live like giving food, carry, wearing clothes,
giving bath and playing to reducing muscle tonus effect.
MEDICINE
- Neuromuscular blocker agent, toxins: OnabotulinumtoxinA (BOTOX )
- Antiparkinsonian agents:
* Anticholinergic drugs: Trihexyphenidyl
* Dopaminergic drugs: Levodopa/carbidopa (Sinemet, Sinemet CR, Parcopa)
- Anticonvulsant Agents: Phenobarbital, Levetiracetam (Keppra), Oxcarbazepine (Trileptal), Valproic acid
(Depakote, Depakene, Depacon),
- α2 Adrenergic Agonist Agents: Tizanidine (Zanaflex)
- Muscle relaxants: Baclofen (Lioresal, Gablofen), Dantrolene (Dantrium, Revonto),
- Benzodiazepines: Diazepam (Valium, Diastat)
SURGERY
- Intrathecal baclofen pump insertion: To treat spasticity and/or dystonia
- Selective dorsal rhizotomy: To treat velocity-dependent spasticity
- Stereotactic basal ganglia: May improve rigidity, choreoathetosis, and tremor
- Orthopedic surgical intervention: To treat scoliosis, joint contractures or dislocation

Complication
- Skin complications: decubitus ulcers and sores
- Orthopedic complications: contractures, hip dislocation, scoliosis.
- Gastrointestinal and nutritional complications: swallowing difficulties, obesity, gastroesophageal
reflux, constipation
- Respiratory complications: aspiration pneumonia, lung disease/bronchopulmonary dysplasia, asthma.
- Neurologic complications: epilepsy, hearing loss, visual-field abnormalities, strabismus
- Cognitive/psychologic/behavioral complications: mental retardation, attention-deficit/hyperactivity
disorder, learning disabilities, impact on academic performance and self-esteem, depression, sensory
integration difficulties, progressive development disorder, autism .

Course and Prognosis


- With appropriate therapy, patients may be able to fully integrate academically and socially.
- The morbidity and mortality of cerebral palsy relate to the severity of this condition and concomitant
medical complications, such as respiratory and gastrointestinal difficulties.
- Cognitive impairment occurs more frequently in persons with cerebral palsy (30-50%) than in the
general population. And 75% of patients have learning disability (including mental retardation).
- 25% of children with cerebral palsy have mild involvement with minimal or no functional limitation in
ambulation, self-care, and other activities.
- Patients with severe forms of cerebral palsy may have a significantly reduced life span.
Case 6 / NBSS Bunga Diela
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MENTAL RETARDATION

Definition
A mental disorder characterized by significantly subaverage general intellectual functioning associated
with impairment in adaptive behavior and manifested in the developmental period.

Epidemiology
- Prevalence 1-3% in the population
- Occurs more frequently in boys than in girls:
* 2:1 in mild mental retardation and 1.5:1 in severe mental retardation
* May be a consequence of X-linked disorders, the most prominent being fragile X syndrome.

Etiology
Biologic causes of mild mental retardation:
- Genetic syndromes with multiple minor congenital anomalies
- Perinatal insults
- Intrauterine exposure to drugs of abuse
- Familial clustering
Biologic cause of moderate and severe mental retardation:
- Chromosomal (e.g., Down syndrome) & other genetic syndromes (e.g., fragile X syndrome)
- Abnormalities of brain development (e.g., lissencephaly),
- Inborn errors of metabolism/neurodegenerative disorders (e.g., mucopolysaccharidoses).

Classification
Determined by the use of standardized tests of intelligence, and the term significantly subaverage is
defined as an IQ of approximately ≤70 or two standard deviations below the mean for the particular test.
a. Borderline Intellectual Functioning: IQ=67-85 (71-84, DSM-IV)
b. Mild MR: IQ=52-68, ICD9 (50-55 to 70, DSM-IV)
- May not be diagnoses until the affected children enter school.
- As they grow older, cognitive deficits such as poor ability to abstract and egocentric thinking may
distinguish them from others in their age.
- Communication deficits, poor self-esteem, relative lack of social spontaneity.
c. Moderate MR: IQ=36-51, ICD9 (35-40 to 50-55, DSM-IV)
- Diagnosed around the preschool period, at a younger age than mild MD.
- They will be able to achieve in school to about the elementary school level.
- Language development, achievement of activities of daily living, social and communication skills
development are often delayed.
- They are aware and frustrated of their deficits, and often alienated from their peers.
d. Severe MR : IQ=20-35, ICD-9 (20-25 to 35-40, DSM-IV)
- Diagnosed very early (in preschool years) because even the earliest motor milestones are delayed.
- Able to learn a few words and a few self-help skills, but will need protected environment as an adult.
- Speech is minimal, motor development is poor. Some language development may occur in the
school age years. By adolescence, if language is poor, nonverbal forms of communication may have
evolved, the inability to articulate needs fully may reinforce the physical means of communicating.
e. Profound MR - IQ=19 and below, ICD-9 (less than 20-25, DSM-IV)
- Affected children will generally need full care as adults, often in a nursing home environment.
Case 6 / NBSS Bunga Diela
Cerebral Palsy and Mental Retardation 130110120114

Clinical Characteristic
- Fine motor/adaptive delay
- Gross motor delay
- Language delay, including expressive language (speech) and receptive language (understanding).
Red flags include no mama/dada/babbling by 12 months, no 2-word phrases by age 2, and parents
reporting they are concerned that the child may be deaf.
- Cognitive delay, such as have difficulties with memory, problem-solving and logical reasoning. This
may be expressed early on with preacademic difficulties or difficulty following directions.
- Significant delays in activities, such as self-feeding, toileting, and dressing.
- Social delays, such as lack of interest in age-appropriate toys and delays in imaginative play and
reciprocal play with age-matched peers.
- Behavioral disturbances, such as have difficult temperaments, hyperactivity, disordered sleep, colic,
aggression, self-injury, defiance, inattention, hyperactivity, sleep disturbances, and stereotypic
behaviors.

Diagnostic Tools (Neurological Examination and Psychiatric Examination)


NEUROLOGICAL EXAMINATION:
- Observing the child's development at regular intervals is probably the most reliable approach.
- Skull X-rays are usually taken routinely, but are illuminating in only a relatively few conditions, such as
craniosynostosis, hydrocephalus, and other disorders that result in intracranial calcifications (e.g.,
toxoplasmosis, tuberous sclerosis, cerebral angiomatosis, and hypoparathyroidism).
- Computed tomography (CT) scans and magnetic resonance imaging (MRI) have become important
tools for uncovering CNS pathology associated with mental retardation. Occasionally, findings are of
internal hydrocephalus, cortical atrophy, or porencephaly in severely retarded, brain-damaged
children.
PSYCHOLOGICAL ASSESSMENT:
- Some report the correlation of abnormalities during infancy with later abnormal functioning as very
low, and others report it to be very high. The correlation rises in direct proportion to the age of the
child at the time of the developmental examination; however, copying geometric figures, the
Goodenough Draw-a-Person Test, the Kohs Block Test, and geometric puzzles all may be used as quick
screening tests of visual-motor coordination.
- These tests are also useful for mildly retarded children. In addition, a psychological evaluation should
assess perceptual, motor, linguistic, and cognitive abilities. Information about motivational, emotional,
and interpersonal factors is also important.

Principal Management
PHARMACOLOGY:
Medication may be helpful in treating associated behavioral and psychiatric disorders, including ADHD,
self-injurious behavior, aggression, anxiety, and depression.
- Antipsychotic Medication is effective for reducing aggressive and self-injurious behavior.
- Atypical Antipsychotic Agent are known to have a lower risk of causing extrapyramidal adverse effect.

NON-PHARMACOLOGY:
- Counseling
- Appropriate educational and behavioral interventions
- Structured classroom training in combination with behavioral management techniques
- Facilitated communication
Case 6 / NBSS Bunga Diela
Cerebral Palsy and Mental Retardation 130110120114

Course and Prognosis


- Mental retardation is not always a lifelong disorder. Children may meet criteria for mental retardation
at an early age but later evolve into a more specific developmental disorder (e.g., communication
disorder, autism, slow learner–borderline normal intelligence).
- As adults, many individuals with mild mental retardation are capable of gaining economic and social
independence with functional literacy. They may need periodic supervision, however, especially when
under social or economic stress. Most live successfully in the community, either independently or in
supervised settings.

Comorbidity
Pertaining to a disease or other pathological process that occurs simultaneously with another.
- The more severe the mental retardation, the higher risk for other mental disorders
- Neurological impairment increases as intellectual impairment increases
- Psychosocial features: they experience repeated failure and disappointment in not meeting their
parents’ and society’s expectations and in falling progressively behind their peers and even their
younger siblings.

BHP
CEREBRAL PALSY Life Expectancy
Most children with cerebral palsy can live long, happy, quality lives. Admittedly, their care may involve
more visits to health care practitioners, require therapy or medications, and perhaps surgery at some
point. They may be evaluated for special education assistance and require assistive technology devices
and services, but a child’s physical impairment, in general, will not limit his or her life span. However,
the severity level of the child’s condition, as well as improper management of his or her symptoms,
may put the child at risk for diminished life expectancy. Research indicates properly managing a child’s
health status may assist in optimizing life span.
MENTAL RETARDATION
- Informed consent preceding every course of action given to patient
- Breaking bad news to patient’s family regarding permanent mental retardation
- Multidisciplinary approach from both family and medical treatment

PHOP
CEREBRAL PALSY
- Parents, medical community, research community and government all play important roles in
preventing cerebral palsy
- Prevent CP by avoiding factors that lead to developmental problems during the pregnancy and labor.
- Patient with CP should be given a multidisciplinary approach with physical therapy and vocational
therapy to maximize extremities function and decrease contracture risk.
- Society education about the diseases.
MENTAL RETARDATION (prevention of mental retardation)
- Genetic: Prenatal screening for genetic defect and genetic counseling for inherited risk family
- Social: Government programs for the poor
- Toxic: Environmental programs in increasing public concern by reducing exposure of mercury or other
toxic substances associated with mental retardation
- Infection: Prevention for congenital rubella syndrome is one of the program which prevent mental
retardation later in life

CRP → see the epidemiology of each disease

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