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Презентация на тему Development of CNS in embrio. Clinical evaluation of abnormalities

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Anatomo-physiological peculiaritiesof CNS in children and their clinical importance
Background of the lectureDevelopment of CNS in embrio. Clinical evaluation of abnormalities.Features Anatomo-physiological peculiaritiesof CNS in children and their clinical importance The central nervous system appears at the beginning of the 3rd week Its lateral edges soon become elevated to form the neural folds. With Neural tube defects account for the most congenital anomalies of the CNS Neural tube defects (NTDs) spina bifida occulta meningocele myelomeningoceleencephalocele anencephal Neural tube defects (NTDs)can be diagnosed prenatally by ultrasound, and by determination Neural tube defects (NTDs)Meningocele (Meningoencephalocele) is herniation of meninges and brain(medulla) through lumbar meningomyelocele in a 3-day-infant Neural tube defects (NTDs) The sloping forehead and small head circumference are evident, although progressive ventricular In embryo at its cephalic end of the neural tube the brain Hemispheres of the brain are developed from the first brain bladder. Errors HydranencephalyMagnetic resonance imaging (MRI) showsthe brain stem andspinal cord with some remnants CSF (cerebral spinal fluid)CSF flow results from the pressure gradient that exists CSFHydrocephalus resulting from CSF accumulation inside the brain is called internal hydrocephalus. The cranial computerized tomogram (CT) of the infant`s brain with congenital virus-associated CSF is absorbed primarily by the arachnoid villi through tight junctions of External hydrocephalus in the newborn with in utero infection of the brain (MRI) Features of CNS in fetus and newbornThe brain development is characterizing by Features of CNS in fetus and newborn (continue)There is not clear differentiation Features of CNS in fetus and newborn (continue)The blood-brain barrier (BBB) of Features of CNS in fetus and newbornCentral and peripheral neurons form myelin Features of CNS in fetus and newbornThe features of the brain vascular Neurological examination Neurologic evaluation of the child. Complaints&History. Seizures (convulsion) are involuntary, violent contraction Opisthotonus in a brain-injured infant. This is the tonic seizure. Objective neurological examinationof the child should include 4 main diagnostic aspects:1. A well child is conscious, alert and responsive Level of consciousness (LOC) Level of consciousness (LOC) Lethargy or pathological sleepy (somnolence) is possible to Level of consciousness (LOC)This is a child with meningitis. The child is Stages of comaStupor: The stuporous patient arouses from sleep only after painful Mental development Head size Enlarged head? AF&PF A fontanel bulging is a reliable indicator of increased ICP, but vigorous Cranial nerves Oculomotor (3-rd) nerve paresis: ptosis (impossibility to lift an upper eyelid) and Facial nerve palsyFacial nerve palsy Facial nerve palsy. Notice the loss of the nasolabial fold and the A newborn with right facial palsy choking Unilateral (right-side) hypoglossal (12th) nerve paresis. Tongue deviation. Motor examination Abnormal gaits The spastic gait Circumduction gait Cerebellar ataxia waddling gait clumsy, tentative gait Movement disorders Paralysis (palsy) – the absence of any voluntary movements Paresis A newborn with brachial right sided paralysis (palsy). The arm hangs limp Movement disordersDystonia - slow twisting movements of limbs or trunk (alternation of Muscles   Examination includes assessment ofmuscles’ development: wasting, pseudohypertrophy Tone: hypotonia, hypertonia Strength: increase, decrease Posterior aspect of the legs of a father and his 6-year-old son HypotoniaOn ventral suspension, the baby assumes the position of a rag doll. Main semiotics of CNS disorders. Meningitis. Examination for neck rigidity in older childMeningeal irritation Brudzinski’s signMeningeal irritation Kernig’s signMeningeal irritation LPThe lumbar punction confirms the meningitis Normal Values for Cerebrospinal Fluid (CSF) CSF finding in bacterial meningitisICP  - increasedWhite blood cell count, μL MeningizmIf the analysis of a cerebrospinal fluid finds inflammatory changes, the child
Слайды презентации

Слайд 2

Anatomo-physiological peculiarities
of CNS in children and their clinical

Anatomo-physiological peculiaritiesof CNS in children and their clinical importance


importance


Слайд 3 The central nervous system appears at the beginning

The central nervous system appears at the beginning of the 3rd

of the 3rd week as a slipper-shaped plate of

thickened ectoderm, the neural plate.


Слайд 4
Its lateral edges soon become elevated to form

Its lateral edges soon become elevated to form the neural folds.

the neural folds. With further development, the neural folds

become more elevated, approach each other in the midline, and finally fuse, thus forming the neural tube.

Слайд 5 Neural tube defects account for the most congenital

Neural tube defects account for the most congenital anomalies of the

anomalies of the CNS and result from the failure

of the neural tube to close spontaneously between the 3rd and 4th wk of in utero development. Neural tube defects (NTDs) involve the meninges, vertebrae, muscles, and skin.

Слайд 6 Neural tube defects (NTDs)
spina bifida occulta
meningocele

Neural tube defects (NTDs) spina bifida occulta meningocele myelomeningoceleencephalocele anencephal


myelomeningocele
encephalocele
anencephal


Слайд 7 Neural tube defects (NTDs)
can be diagnosed prenatally by

Neural tube defects (NTDs)can be diagnosed prenatally by ultrasound, and by

ultrasound, and by determination of ά-fetoprotein (AFP) levels in

maternal serum and amniotic fluid. The cranium or vertebra can be visualized since 12 weeks of gestation, and defects can be detected.
Recent evidence indicates that folic acid (folate) reduces the incidence of NTDs in certain populations.

Слайд 8 Neural tube defects (NTDs)
Meningocele (Meningoencephalocele) is herniation of

Neural tube defects (NTDs)Meningocele (Meningoencephalocele) is herniation of meninges and brain(medulla)

meninges and brain(medulla) through a defect in the skull

or vertebra split producing a fluid-filled sac in the occipital or lumbar region.

Слайд 9 lumbar meningomyelocele in a 3-day-infant
Neural tube

lumbar meningomyelocele in a 3-day-infant Neural tube defects (NTDs)

defects (NTDs)


Слайд 10 The sloping forehead and small head circumference
are

The sloping forehead and small head circumference are evident, although progressive

evident, although progressive ventricular
enlargement often subsequently occurs in

such children.

Occipital meningoencephalocele


Слайд 11 In embryo at its cephalic end of the

In embryo at its cephalic end of the neural tube the

neural tube the brain bladders are forming from which

all parts of the brain are originated within approximately 2-3 months of in utero development, including neural parts of ear, eye and sense of smell.

Слайд 12 Hemispheres of the brain are developed from the

Hemispheres of the brain are developed from the first brain bladder.

first brain bladder. Errors of embryogenesis, connected with an

action of a teratogen (the factor inducing abnormalities) can lead to severe pathology of the fetus and newborn, for example, microcephaly and anencephaly. The cerebral hemispheres and cerebellum are usually absent, and only a residue of the brain stem can be identified when anencephaly presents.

Слайд 13 Hydranencephaly
Magnetic resonance
imaging (MRI) shows
the brain stem and
spinal

HydranencephalyMagnetic resonance imaging (MRI) showsthe brain stem andspinal cord with some

cord
with some remnants
of the cerebellum
and the

cerebral
cortex.
The remainder volume
of cranium
is filled with CSF

Слайд 14 CSF (cerebral spinal fluid)
CSF flow results from the

CSF (cerebral spinal fluid)CSF flow results from the pressure gradient that

pressure gradient that exists between the ventricular system and

venous channels. The intraventricular pressure is twice higher than the pressure in the superior sagittal sinus.


Слайд 15 CSF
Hydrocephalus resulting from CSF accumulation inside the brain

CSFHydrocephalus resulting from CSF accumulation inside the brain is called internal hydrocephalus.

is called internal hydrocephalus.


Слайд 16 The cranial computerized tomogram (CT) of the infant`s

The cranial computerized tomogram (CT) of the infant`s brain with congenital

brain with congenital virus-associated encephalopathy
Cerebral atrophy with enlarged

ventricles and widened sulsi (internal hydrocephalus).

Слайд 17 CSF is absorbed primarily by the arachnoid villi

CSF is absorbed primarily by the arachnoid villi through tight junctions

through tight junctions of their endothelium by the pressure

forces.
Hydrocephalus resulting from malfunction of the arachnoid villi is called nonobstructive or communicating hydrocephalus.

Слайд 18 External hydrocephalus in the newborn with in utero

External hydrocephalus in the newborn with in utero infection of the brain (MRI)

infection of the brain (MRI)


Слайд 19 Features of CNS in fetus and newborn
The brain

Features of CNS in fetus and newbornThe brain development is characterizing

development is characterizing by gradual formation and maturation of

brain structures from ontologically "old" to "young“. Note the line: the spinal cord, brain stem, subcortical formations, cerebellum and at last the cortex are making mature.
First months of life there is some functional minority of regulating activity of the cortex in favour to the subcortical formations with domination of thalamopallidal and striopallidal areas.
• The child’s brain contains more protein than the brain of the adult. Cerebral proteins make the tissues of brain hydrophilic and bent them to cellular edema.

Слайд 20 Features of CNS in fetus and newborn (continue)
There

Features of CNS in fetus and newborn (continue)There is not clear

is not clear differentiation of the brain’s layers (grey

and white substances are indistinctly differentiated among themselves).
The gyri and sulci of the cortex are not deep that reduces the absolute and relative area of the child's cortex in comparison to adult.

Слайд 21 Features of CNS in fetus and newborn (continue)
The

Features of CNS in fetus and newborn (continue)The blood-brain barrier (BBB)

blood-brain barrier (BBB) of the fetus and newborn
is

normally indiscriminately permeable, allowing protein and other large and small molecules to pass freely between the cerebral vessels and the brain.
becomes mature only to the ending of the neonatal period

Слайд 22 Features of CNS in fetus and newborn
Central and

Features of CNS in fetus and newbornCentral and peripheral neurons form

peripheral neurons form myelin coating gradually. Myelinization finally finishes

only after the 3-rd year of life.
Due to undeveloped myelinization in children long time the cortex physiology will be characterizing to be bent to generalization of irritation and difficulties of neuronal braking.


Слайд 23 Features of CNS in fetus and newborn
The features

Features of CNS in fetus and newbornThe features of the brain

of the brain vascular system of fetus when anastomoses

develop insufficiently make the brain of premature newborn easily vulnerable to hypoxia, mechanical, and thrombotic damages. This can promote for cerebral ischemia and hypoxia with form of cerebral palsy.

Слайд 24
Neurological examination

Neurological examination

Слайд 25 Neurologic evaluation of the child. Complaints&History.
Seizures (convulsion)

Neurologic evaluation of the child. Complaints&History. Seizures (convulsion) are involuntary, violent

are involuntary, violent contraction of muscles. Seizures may be:

tonic or clonic,
focal or generalized.
Tonic seizures are characterized by increased tone or rigidity.
Clonic seizures consist of rhythmic muscle contraction and relaxation, when stereotypic, wide movements observe in extremities and other parts of a body.


Слайд 26 Opisthotonus in a brain-injured infant. This is the

Opisthotonus in a brain-injured infant. This is the tonic seizure.

tonic seizure.


Слайд 27 Objective neurological examination
of the child should include 4

Objective neurological examinationof the child should include 4 main diagnostic aspects:1.

main diagnostic aspects:
1. Level of consciousness (LOC)
2.

Mentality
3. Head examination
4. Evaluation of motor system

Слайд 28
A well child is conscious, alert and responsive

A well child is conscious, alert and responsive Level of consciousness (LOC)


Level of consciousness (LOC)


Слайд 29 Level of consciousness (LOC)
Lethargy or pathological sleepy (somnolence)

Level of consciousness (LOC) Lethargy or pathological sleepy (somnolence) is possible

is possible to determine as an unusual sleep of

the patient.
Confusion. The responses of confused patients demonstrate a failure to comprehend their surroundings. The patient is unable to estimate direction or location, is apt to be disoriented in time and may misidentify people.
Cоmа is absence of consciousness.

Слайд 30 Level of consciousness (LOC)
This is a child with

Level of consciousness (LOC)This is a child with meningitis. The child

meningitis. The child is somnolent and can not arouse.

Note the face of a gray color.

Слайд 31 Stages of coma

Stupor: The stuporous patient arouses from

Stages of comaStupor: The stuporous patient arouses from sleep only after

sleep only after painful stimuli. Verbal responses are slow

or even absent. The patient lapses into an unresponsive state when the stimulus ceases.
Light coma: the patient has response to painful stimulus.
Deep cоmа: there is no response to painful stimulus.
Terminal coma: coma with a muscular relaxation and apnea.

Слайд 32 Mental development

Mental development

Слайд 34 Head size

Head size

Слайд 35
Enlarged head?

Enlarged head?

Слайд 37
A fontanel bulging is a reliable indicator of

A fontanel bulging is a reliable indicator of increased ICP, but

increased ICP, but vigorous crying can cause a protuberant

fontanel in a normal infant.

ICP-intracranial pressure


Слайд 38 Cranial nerves

Cranial nerves

Слайд 40 Oculomotor (3-rd) nerve paresis: ptosis (impossibility to lift

Oculomotor (3-rd) nerve paresis: ptosis (impossibility to lift an upper eyelid)

an upper eyelid) and removal of an eyeball laterally

(temporally).

Слайд 42 Facial nerve palsy
Facial nerve palsy

Facial nerve palsyFacial nerve palsy

Слайд 43 Facial nerve palsy. Notice the loss of the

Facial nerve palsy. Notice the loss of the nasolabial fold and

nasolabial fold and the mouth deviated to the left

when he smiles.

Слайд 44 A newborn with right facial palsy

A newborn with right facial palsy

Слайд 45
choking

choking

Слайд 47 Unilateral (right-side) hypoglossal (12th) nerve paresis. Tongue deviation.

Unilateral (right-side) hypoglossal (12th) nerve paresis. Tongue deviation.

Слайд 48 Motor examination

Motor examination

Слайд 50 Abnormal gaits
The spastic gait
Circumduction gait
Cerebellar

Abnormal gaits The spastic gait Circumduction gait Cerebellar ataxia waddling gait clumsy, tentative gait

ataxia
waddling gait
clumsy, tentative gait


Слайд 51 Movement disorders
Paralysis (palsy) – the absence of

Movement disorders Paralysis (palsy) – the absence of any voluntary movements

any voluntary movements
Paresis is incomplete paralysis
Ataxia - gross

uncoordination that may become worse with the eyes closed
Athetosis - slow, writhing, wormlike, constant, grossly uncoordinated movements that increase on voluntary activity and decrease on relaxation

Слайд 52 A newborn with brachial right sided paralysis (palsy).

A newborn with brachial right sided paralysis (palsy). The arm hangs

The arm hangs limp alongside the body and internally

rotated, and the wrist is pronated hand (hangs limp downwards).

Слайд 53 Movement disorders
Dystonia - slow twisting movements of limbs

Movement disordersDystonia - slow twisting movements of limbs or trunk (alternation

or trunk (alternation of a hypotonia with rigidity, formation

of elaborate postures)
Tics - involuntary, compulsive, stereotyped movements of an associated group of muscles (can be suppressed by strong-willed effort).
Tremors - constant small very fast involuntary movements.

Слайд 54 Muscles
Examination includes assessment of
muscles’ development: wasting,

Muscles  Examination includes assessment ofmuscles’ development: wasting, pseudohypertrophy Tone: hypotonia, hypertonia Strength: increase, decrease

pseudohypertrophy
Tone: hypotonia, hypertonia
Strength: increase, decrease


Слайд 55
Posterior aspect of the legs of a father

Posterior aspect of the legs of a father and his 6-year-old

and his 6-year-old son with a rare autosomal dominant

muscular dystrophy. Hypertrophy of the calves resembles Duchenne muscular dystrophy

Слайд 56 Hypotonia
On ventral suspension, the baby assumes the position

HypotoniaOn ventral suspension, the baby assumes the position of a rag

of a rag doll.
When pulled up from the

supine to the sitting position, the head of the baby lags.

Слайд 57 Main semiotics of CNS disorders. Meningitis.

Main semiotics of CNS disorders. Meningitis.

Слайд 59 Examination for neck rigidity in older child
Meningeal irritation

Examination for neck rigidity in older childMeningeal irritation

Слайд 60 Brudzinski’s sign
Meningeal irritation

Brudzinski’s signMeningeal irritation

Слайд 61 Kernig’s sign
Meningeal irritation

Kernig’s signMeningeal irritation

Слайд 62 LP


The lumbar punction confirms the meningitis

LPThe lumbar punction confirms the meningitis

Слайд 63 Normal Values for Cerebrospinal Fluid (CSF)

Normal Values for Cerebrospinal Fluid (CSF)



Слайд 64 CSF finding in bacterial meningitis
ICP - increased
White

CSF finding in bacterial meningitisICP - increasedWhite blood cell count, μL

blood cell count, μL - 100 –

10000
Cell type - neutrophiles 100%
Protein content - ≥ 40 mg/dl (0.4 g/l)
Glucose - ≤ 40 mg/dl (≤ 50% blood glucose)
Culture - positive

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