( BETA EDITION)

With
Prof. Dr Mohammed Abo El-Asrar

Edited By
El-Azhar Medical students 2012





" "
:

: : : :

41

NEURO
written
. ) 1- Floppy baby (generalized hypotonia
. 2- MR
3- Convulsions
clinical
1. C.P.

2. Hydrocephalus
3. Duchenne myopathy
oral
Skull X-ray

Introduction
clinical pictures

Clinically :
neurology comment
Motor system.
Sensory manifestations.
Autonomic manifestations.
higher functions as MR or convulsions.

1.
2.
3.
4.

1- Motor system
A Muscle power
Neuro anatomy :
UMN orders LMN cranial nerve or anterior horn cells Axons .... target muscle
Any muscle fibers .* muscle fibers 5 fiber AHC
So, 5 AHCs give 5 ms fibers
) ( order AHCs then to 5 fibers 5
fiber
* 5 neuron fiber ms fiber ms 4
fibers % 80 ....
* % 60 ) weakness fibers
(
Loss function fibers paralysis
muscle power
?? Weakness or paralysis

History
muscle
1- upper limb :
A. Small muscle of the hands
) ( ...

B. Elbow
flexiors extensiors
C. Shoulder
flexors
extensors
adductors
2- Lower limb
A - Small muscle of the foot


normal muscle power


Motor only
sensory motor and sensory
B - Knee
Flexion of knee occur with flexion of hip also extensor with extensors

C - Abductor & adductors
adductors abductors
.
3- Trunk.
A - Flexors

B - Extensors
trunk ... extensors
B - Muscle tone
tone
muscle Origin .. Insertion muscles origin Insertion stretched
spindle cells signals spinal cord AHCs stimulation muscle contract
tone hyper tonia hypotonia or atonia Paralysis and weakness)


.... Tone tone
Tone
biceps cm 6
Origin Insertion biceps cm 8
biceps Origin Insertion stretch
Origin insertion
is stretched
>>>>
stretched ) muscle fibers ( Muscle fibers Impulses
dorsal root ganglion
dorsal root ganglion .... spinal cord Axon
T shaped
fibers impulses >>>> dorsal root ganglion dorsal root ganglion
Posterior horn cells
>>>> posterior horn cells >>> Anterior horn cells Anterior horn cells
nerve fibers
contract
... Is contracting
disuse atrophy
tone
Pyramidal

pyramidal tone Inhibition
) ( (:
biceps ) ( biceps tone

 biceps tone ....

Pyramidal >>> tone tone
atrophy

pyramidal tract tone .... Inhibition pyramidal tract Inhibition
...... Lost
lost
tone
hyper tonia
Pyramidal tract ... Level of the lesion Hyper tonia
nerve stretch impulses contract
nerve Upper Lower
Lower
Lower motor neuron lesion tone Lost
Nerve tone
Nerve lesion Lower motor neuron lesion tone hypo tonia
Lost
(
C - Deep Tendon Reflex
hummer tendons :
sudden passive stretch sudden massive activation of AHCs sudden massive contraction of all
muscle fibers comment
deep tendon reflexes
upper motor neuron lesion Hyper refle
D - Muscle state
Size of the muscle
Pseudo hypertrophy lower motor
muscle vascular supply energy vascular flow ... waste product vascular flow
Muscle activity vascular supply neuro vascular connections nerve supply vascularity muscle
Nerve signals .V.D arterial flow vascular flow
vascular supply Lower motor neuron vascular supply
degeneration Muscle ) wasting ( so early Upper motor neuron lesion Upper motor neuron lesion just so late disuse atrophy
E - Muscle fasciculation
fibers contraction fasciculation isolated fibercontraction
fibers .....
fasciculation Muscle tongue : purely muscular organ covered by mucosa ( transparent ) and no submucosa or fat as other muscles


) .... muscle power weakness or paralysis
tone deep tendon reflex muscle state Muscle
( fasciculation
Distribution

1- Bilateral or unilateral
bilateral
2- Symmetrical or asymmetrical
) (...
+ extensor extensor
3- Proximal more than distal







So , Distal more than proximal

So, Proximal more than distal
) (
proximal or distal
Upper limb
lower limb
...
distal
distal proximal
comment distal more affected than proximal

Upper motor neuron lesion distal Proximal
distal
(
2- Sensory system
superficial sensation deep sensation

1st order neuron in posterior root ganglion dendrite skin ) Pathway (
3- Autonomic manifestations
Pulse rate.
tachycardia bradycardia
Heart
Blood pressure
heart

.
If sympathetic.
.
If parasympathetic.
sphincteric control :
Sympathetic retention.
Parasympathetic incontinence.
4- Higher functions
development
LMNL
lower motor neuron lesion
lower motor neuron lesions

muscle AHCs lower motor neuron

AHCs (or cranial nuclei).
Peripheral nerve (cranial nerve).
Myo-neural junction.
Muscle.
lower motor neuron lesion
criteria of lower motor neuron lesion
Criteria of LMNL
Motor system
Muscle power weakness or paralysis.
Muscle tone hypotonia
tone circle
Deep tendon reflex hypo reflexia.
Muscle state muscle wasting
LMNL
motor sensory Peripheral nerve sensory
So,
1- AHCs No sensory loss.
2- Myo-neural junction No sensory loss.
3- Muscle No sensory loss.
sensation
peripheral nerve
AHCs fasciculation
Nerve facial palsy
AHCs .... fasciculation
.... Muscle junction
distal Proximal ... distribution Muscle
Investigations where is the lesion
Investigations
1.
2.
3.
4.

1- EMG
) Upper motor neuron lesions Lower motor neuron lesions
( cerebral palsy CP
neuropahtic or myopathic signals fibers waves
If myopathic
Muscle
If neuropathic.
AHCs Nerve

Investigation
2- Nerve conduction velocity for neuropathic.
myopathy

Muscle ... superficial nerve
AHCs Nerve Normal
.1
Impaired peripheral neuropathy
.2

Hypotonia & neuromuscular disease
Disease affecting AHCs
Poliomyelitis.
Infection
Spinal muscle atrophy.

Amytrophic lateral sclerosis rare.

Spinal muscl e atrophy
Type one = Werding Hoffman disease
....

- Autosomal recessive disease
female = male
Affected persons homozygous
carrier ( >>> )
So,- positive family consanguinity + +ve family history
C/P

5 nerve fibers to muscle ( give ) neurons 5 ( ) apoptosis & mitosis
brain cells .... no mitosis and no apoptosis
apoptosis of neuronal cells gene ... neuronal cells Programmed cell death
Lower motor neuron
: .. AHCs
1- decrease fetal Movement ( intrauterine ) :
multi para ( multi para ) 16
.. ) 18 (
2- weakness then paralysis with hypotonia and hyporeflexia :
weakness then paralysis with hypotonia and hyporeflexia neurons diagnostic tongue fasciculation - Distribution : bilateral, symmetrical and ( proximal and distal )
3- Cranial nerves affection :
: bulbar cranial nerves ... Cranial nerve
Weak suckling and swallowing *
Repeated aspiration and chocking *
When cry >>> nasal tone of voice *
Hoarseness of voice *
| |
diagnostic tongue fasciculation
neurotransmitter as it is a degenerative disease
fasciculation irritation ( polio )
| |
No MR or convulsions .... Normal sensation
J
Cranial nerve nuclei
staring look L
( )
Investigations
EMG neuropathic.
Nerve conduction velocity normal.
CPK normal
muscle
Treatment
Supportive only.
8


41 42

Post infectious poly neuropathy

Guillian Barre syndrome
Infection
) Guillian Barre syndrome
.. ) ..
(
(

Introduction
motor and sensory peripheral nerve
Nerve conduction of nerve impulse
nerve impulse .. ) ( ... jumping nerve fibers
... myelin sheath ... Schwann cells rapid transmission
node of Ranvier depolarization Node


motor .. sensory spinal cord :
neuronal cells .. intrauterine
neck support
Neuronal cells myelination

neck support ) 3 ( Neck muscle ... brain Innervation .... trunk muscles spinal cord ( 5-4 ( ...

proximal muscles thigh ... arm ) 9 ( more distal foot hand ... forearm
myelination

viral infection virus ... ) ( measles , mumps, chicken pox, coxachie virus ... auto-immunity antibodies ..... myelin sheath

..

nervous system
nerve impulse ) (
:
demyelination ) ( AHCs or Cranial nuclei muscles
So, lower motor neuron lesion
So, C/P
1- motor manifestations :
as Weakness or paralysis. , Hypotonia & hypo reflexia.
muscle wasting .
bilateral and symmetrical .
Proximal distal
signals )
Motor signal .. spinal cord
( ..
) ( signals
9

So, distribution distal more than proximal

........
2- sensory affection :
As it is peripheral nerve so, sensory manifestations
spinal cord sensation pathway
signal spinal cord
spinal cord
So, sensory loss distribution glove and stocking hypothesia.
3- Autonomic affection :
degeneration autonomic
If both sympathetic and para sympathetic degeneration
upper hand
So,
If demyelination of para sympathetic sympathetic pulse, Bl. Pressure, retention of
urine and stool.
If sympathetic para sympathetic ()

4- ascending march :
demyelination may bulbar cranial nerves upper limbs trunk Lower limb ( So, ascending march ( landrys

5- tender cough muscles :
tender cough muscles
6- then , if extensive type may bulbar affection
Also, other cranial nerves may be affected
7- if more severe Encephalo-myelo-radiculopathy cranialis
S.C. & brain nervous system demyelination Guillian
(Encephalo-myelo-radiculopathy cranialis )
radiculopathy roots - .myelo S.C - encephalop Brain Cranialis cranial ns 8- higher functions :No MR or Convulsion
Investigations
a)
b)
c)
d)

EMG neuropathic pattern.

Nerve conduction velocity impaired So, peripheral neuropathy .
May nerve biopsy
rarely indicated .... Nerve
CSF examination Cyto-albuminous dissociation.
Cyto cells Albuminous albumin.
CNS auto immune diagnostic inflammatory cells protein CSF 1.
If meningitis neutrophils & protein.
2.
If viral encephalitis lymphocytes & protein.
3.
If auto immune protein ( as inflammation ) + no inflammatory cells
auto immune Inflammation .. Infection
Treatment

1st line of treatment IV immunoglobulin
( )
steroid and plasmapheresis
ABs
10

Physiotherapy.

3 3 age of onset
3 auto immune
N.B. Any cranial nerve affection here is bilateral.
Myoneural Junction affection

Causes
Myasthenia gravis.
Organo phosphorous compound.
Tick-born paralysis.
Botulism.
) : )
Duchenne myopathy

Introduction

ATP Muscle
:
Skeletal muscle - Heart - Brain cells
ATP
ATP .. ATP glycolysis mitochondria generator 3 glycolysis
muscle
kidney creatinine creatine .. generator endoplasmic reticulum generator
creatinine creatine
Muscle cells creatine phosphokinase cytoplasmic enzyme
carrier protein sacrolemmal membrane endoplasmic reticulum
dystrophin
Duchenne
X- short arm - region Z - band X- linked recessive gene
chromosome & as it is x linked So, common in males
Duchenne
3
New mutation, turner or ??????

C/P

creatinine creatine CPK abnormal dystrophin
3 ... ATP
Muscle fibers ATP skeletal muscle
degeneration
. So, progressive course fibers ..
fibers
lower motor neuron lesion criteria :
Weakness or paralysis
Hypotonia & hypo reflexia
What about muscle state????
( fibrous and fatty tissue ) degeneration muscle
Muscle ( fibrous and fatty tissue )
11

 Muscle wasting
fibro-fatty tissue ... Pseudo-hypertrophy
Excessive fibro-fatty tissue deposition

quadriceps .. as deltoid, triceps and gluteus Medius , culff muscles ..

-
some muscles never to be affected
as upper fibers of trapezius, sternomastoid muscle, clavicular head of pectoralis major muscle, small
muscle of the hand, sphincter of the bladder

theories
ATP ATP
Mitochondria
- No autonomic or sensory affection.
Distribution
Bilateral and symmetrical + proximal > distal
ATP
proximal distal signs
Signs
1- +ve slipping sign :
adductors shoulder
adductors .... positive slipping sign ...
2- exaggerated lumbar lordosis :
.. anterior ..
extensors back .... extensors ...
... .
... lumbar vertebrae ... Lordosis exaggerated lumbar lordosis3- waddling gait :
... ... ...
.. muscles of the back gluteal muscles
gluteus muscle ... ... waddling gait
4- Winging of the scapula :
winging of the scapula ) medial border (
medial border serratus anterior ... rhomboidus muscle
pushing Medial border scapula serratus anterior medial border
5- Pes cavus high arched foot
deformity fibrosis
6- Positive Gower sign
back muscles

12

tone )

( J
Complications
.... heart myopathy Heart heart failure
brain cells So, may: MR 20 - 30 % of cases.
- Epilepsy
( Irritate )
Causes of death
- H.F.
- UTI due to hypercalcemia >>> so, repeated renal stones

Ca & Ph bone osteoblast Muscles
as in rickets ,,,,
Investigations
EMG myopathic cytoplasm.
Enzymes
CPK SGOT aldolase ... degenerated muscle
Muscle biopsy fibro-fatty tissue.
Urine analysis creatine & creatinine.
ECG & ECHO cardic affection.
EEG & IQ for MR & convulsions.
Gene study
. prenatal diagnosis Duchenne
As the mother is a carrier slight in CPK.
Treatment
- Physiotherapy + supportive treatment.
- Recently myeloblast transplantation.
- Under trial gene therapy.
UMNL
neurons 3
1- Motor area 4
And 2 accessory neurons
2- Basal0 ganglia 3- Cerebellum
Functions of motor area 4 -1
1- Initiation of voluntary movement.
Area 4
Weakness or paralysis ... LMN
Distribution
Area 4
. limb Mono plegia
. Hemi plegia
. .L.L Cortical para plegia
: limbs 2 Quadri plegia

13

1- May upper limb more than lower limb

bi plegia
2- Diplegia lower limb more than upper limb
3- Right more than left Or left more than left double hemiplegia

42 43

Upper motor neuron lesion muscles Proximal or

distal
shock stage

as small muscles of the hand & foot ...loss of all fine movement
crude movement

motor area 6 proximal muscles area 4 & 6 distal area 4

area 4 complete loss of function of distal muscles ...

Area 6 But proximal muscles
So, in area 4 affection distal > proximal
Area 4
2- Suppression of muscle tone & deep tendons reflexes .
suppression .. anti gravitymuscles
) anti gravity pro gravity
anatomical position ( Pro gravity
) ...
flexors ... Lower limb
.. flexors extensions ..
(
Upper limb
)a
Extensors Pro gravity
)b
Flexors anti gravity
Lower limb

Extensors anti gravity

Flexors pro gravity

more suppression adductors abductors Area 4 suppression tone & deep tendon reflexes
But anti gravity > pro gravity
hemi plegia ..
knee
Extensors > flexors
J flexion extension
) flexion upper limb

(
Area 4 tone adductors abductors shock stage special gait adductors lower limbs
trunk So, circumduction gait

14

 para plegia scissoring gait

hyper tonia initiation of movement area 4 resistance .. clasp knife spasticity
3- Suppression to pathological reflexes
reflexs Normally

As Neonatal reflexes
reflexes 4-3

4-3 Area 4 maturation
suppression to pathological reflexes
neonatal reflexes
: 2&1
1- Moro reflex :
..
4-3 ) ( sub conscious level Area 4

) .. (
Area 4
.2- Grasp reflex :

reflex 4-3 ) ( .. .. Area 4
3- +ve babiniski sign :
scratching of lateral side of the sole of the foot planter flexion
positive Babiniski sign
... planter dorsi flexion

4- Clonus :
sustained stretch Muscle
Motor area 4 suppression tendon reflex muscle spindle
) continuous contraction (
5- adductor reflex :
adductors lower limb medial border of popliteal fossa adductor
tendon
normally reflex .. area 4 suppression
... adductors reflex
:
Criteria of motor area 4 lesions
) 1- Loss of 1st function ( initiation of movement
So, weakness or paralysis which:
mono plegia, hemi plegia, para plegia or quadri plegia( bi plegia, di plegia, double hemi plegia) distal
> proximal
2- Loss of suppression of the tone
)So, there is hypertonia (clasp knife, spasticity
antigravity > pro gravity

15

upper limb flexors > extensors

lower limb extensors > flexors
adductors > abductors
so,
If unilateral lesion circumduction gait.
If bilateral lesion scissoring gait.
Exaggerated deep tendon reflexes.
3- Pathological reflexes :
) Persistent neonatal reflexes as ( moro and grasp
Positive Babniski sign.
Clonus
Adductor reflex positive.
NB Babniski sign is positive ) ( ) area 4
maturation )
.. area 4 cerebellum
2- Cerebellum
physiologically parts 3 functions
2Archi cerebellum :
anatomically
Motor area 4

Inner ear
Effect of lesion
Mechanism ..
.. drunken gait archi
cerebellum
2- Paleo cerebellum :
anatomically animals
.. muscle tone spinal cord lateral ventral
spino-cerebellar tracts
Effect of lesion
.. paleo cerebellum tone Hypo tonia
cerebellum stimulation Muscle tone deep tendon reflexes

) tone (
tone
hypotonia and hypo reflexia ...
lower motor neuron lesion ... weakness or paralysis
3- Neo-cerebellum :
Functions & effects of lesion
In human
paleo neo area 4 .. ...
J neo cerebellum

..
1- estimation of distance :

16

 ... estimation of distance area 4

dysmeteria either
or parametria or hypometria hypermetria
2- Smooth mov. :
.. single movement
area 4 called decomposition of
.mov

supination and pronation dys-diadokokinesia

cerebellum suppression antagonist muscle tremors which is coarse kinetic tremors
fixation object - - nystagmus
3- break function ability to stop mov. At any level of it.



)

(
rebound no ability to break
signs
4- equilibrium :
deep sensation
Neo- cerebellum deep sensation paleo-cerebellum postural

paleo- cerebellum
... Neo
) ( deviation gait
drunken gait
drunken gait deviation gait drunken gait Neo- cerebellum ... zigzag gait
cerebellar lesion
1- Hypo tonia, hypo reflexia without muscle weakness or paralysis.
2- Dysmetria, dys dia-dokinesia , kinetic tremors, nystagmus, rebound +
gait disorder as drunken gait deviation gait zigzag gait
Upper motor neuron
3- Basal ganglia
,,,, functions 3
1- Emotions associated with movement.

associated movement
17

A - Swinging of the hands

) )
B also, Emotion - associated movement as Facial expression

exaggerated emotions

) ( basal ganglia

.... ....

) basal ganglia (
C associated movements with speech :
associated movement ..
Effect of lesion

basal ganglia disturbed emotions associatedmovement

chorea
2- Suppression of basal activities of motor area 4.
basal ganglia

Motor area 4 ) .. (J basal ganglia area

... 4
.. !!!
basal ganglia
Effect of lesion
basal ganglia activities abnormal movement

chorea ... area 4 ..
) ...( cortex ) (

excitation sleep
asthetosis ... abnormal movement
Twisting movement in extremities ....
dystonia twisting movement in the trunk

3- Effect of basal ganglia on tone
Mediators basal ganglia

stimulation. Acetyl choline

Dopamine suppression of tone.
basal ganglia control tone
or
Effect of lesion
If lesion acetyl choline hypo tonia and hypo reflexia as chorea.
If lesion dopamine hyper tonia and areflexia
motor area 4 rigidity lead pipe cog wheel
lead pipe resistance

18

 resistance cog wheel

tone areflexia
reflex flexors Knee agonist and antagonist tone

Rigidity more in flexors and adductors short steppage & S parkinsonism

shuffling gait
4- Coarse static tremors

Cerebral palsy ( CP)
Definition
Central motor neuron deficit
central neuron
Motor area 4, Cerebellum or Basal ganglia (BG)
brain growth Post natal natal ante natal defect manifested early in neonatal period
central motor neuron
1- Ante natal, natal or post natal
2- Manifested early
Etiology
1- Ante natal
motor neurons Infection STORCH infections -1
rash fever
drugs or irradiation teratogen -2
Hypoxia intrauterine -3
circulation placenta
- or mother pressure placental insufficiency
Toxiemia of pregnancy

brain abnormal metabolites inborn error of metabolism -4
by exclusion congenital anomalies in fetus brain -5
by exclusion 2- Natal
A Delayed delivary
( ) true uterine contraction contraction ... 12 6 brain hypoxia hypoxia .. placenta
cells cerebral anoxia
cause hypoxic ischemic encephalopathy
B - Birth injury
intra .. gift forceps 6 cranial hemorrhage
3- Post-natal

19

neonatal period
A infection :
infection ... as meningitis or encephalitis
B - Bilirubin encephalopathy
neonatal jaundice

..
exchange transfusion ..
bilirubin encephalopathy
C May neonatal hypoxia pneumonia
D - Metabolic disorders
E - Intra cranial hemorrhage
Clinical pictures
CP
1- Spastic type most common 75 %

= Motor area 4 lesion
1- weakness or paralysis
- mono, hemi, para, quadri plegia ( bi, di , double )
- Distal > proximal
2- Hyper tonia clasp knife
more in anti gravity & in adductors scissoring
3- Exaggerated deep tendon reflexes
4- Pathological reflexes:
- grasp, moro ( neonatal reflexes)
- Positive Babiniski sign
- Clonus, adductor reflexes
2- Ataxic type
Hypo tonia cerebellum
.Dysmetria, dysdiadokinesia .others .etc
Abnormal gaits
3- Extra pyramidal type CP
basal ganglia

1- Emotional & associated movement disturbance
2- Abnormal movement ( chorea, asthetosis, dystonia ..etc)
3- tone
J
4- Atonic type
lesion .... Unexplained type exaggerated deep tendon reflex sever hypotonia
sever MR

20

5- Mixed type.
Criteria of CP
CP
1- upper motor neuron lesion
2- normal sensation
3- Must be antenatal, natal or postnatal
) )
other associated manifestations as MR, deafness(especially extrapyramidal type), fits -4
NB CP is Non fatal, Non curable & Non progressive disease
Degrees of CP
Motor
Class one no motor dysfunction.
motor dysfunction severity
Class two, three and four (mild , moderate and severe ) .
Investigations

mentality IQ ..

... audiometry
.. ... " "
..
.... ...
......
Treatment
- Only supportive treatment as:
Physiotherapy, auditory aids .etc.
44 43

: .. ..
CSF circulation
NB ventricles in brain 2 lateral ventricles , 3rd ventricle and 4th ventricle ( cerebellum )
3rd Lateral ventricle Lateral ventricle choroid plexus CSF brain 4th ventricle aquiduct of sylvius ventricle
sub arachinoid space CSF foramina of Lushka lateral foramina 2
brain
foramen of magendi spinal cord sub arachinoid sub arachinoid around spinal cord
sub arachinoid space brain SPC CSF arachinoid villi superior sagittal sinus SSS
CSF absorption
right side of the heart superior vena cava IJV
:
Hydrocephalus
CSF hydrocephalus
?? either Inside or outside the brain
Definition
So, Hydrocephalus is defined as CSF inside the brain or inside and outside brain .
causes

21

A Non obstructive (communicating) Hydrocephalus:

1- Excess secretion or production of CSF
... CC 200 absorption CC 200
CC 200 absorption CC 300
2- Decrease absorption.
200 CC 100 CC 200
B - Obstruction.

A- Non obstructive ( communicating ) hydrocephalus
1- excess secretion
choroid plexus 1- congestion choroids plexus:
congestion Mucous membrane congestion
excess secretion of mucous mucosa
congestion
1- CNs infection
-as meningitis or encephalitis or any inflammatory reaction choroid congestion CSF ICT
brain edema
brain inflammatory edema ICT meningitis
CSF secretion choroid plexus congestion
2- may sterile inflammation called meningism (meningeal irritation)
but no organism ( ) inflammatory reaction toxin
: pseudo tumor cerebri -2
brain tumors manifestations of ICT
ICT CSF
Causes of pseudo tumor cerebri
1- Hyper or hypo vitaminosis A.
2- Hyper vitaminosis D.
3- Pseudo hypo para thyroidism
receptors .... End organ resistance para thormone hormone
4- Expired tetracycline (out dated tetracycline).
over dose dose
5- Adolescent obese female.
CSF
3- May tumor as papilloma from choroid plexus
2- absorption

sub- arachinoid superior sagital sinus absorption SSS
1- may Anatomical defect
absorption SSS CSF sub arachinoid space
. CSF

A Achondroplasia shallow post. Cranial fossa :

22

 posterior cranial fossa cerebellum cerebellum posterior cranial fossa space sub arachinoid space curve fossa
shallow cerebellum Post fossa
CSF Achondro plasia shallow post fossa hydrocephalic changes large skull
achondroplasia
large skull
B Arnold chiari malformations (meningeocele or Meningo-myelocele) and Hydrocephalus

spinal cord foramen magnum ) brain ( CSF
Brain cerebellum Downward displacement ofthe brain

sub arachinoid space brain foramen magnum foramen of magendi CSF spinal canal CSF ) S.C .. .. foramen magnum (
.... sub arachinoid space CSF pressure
spinal canal
intra uterine period embryo vertebral body ) J (
anterior .. body Post arch neural tube
post arch of the vertebrae spinal foramen SC
pressure posterior arch .. vertebrae vertebrae ) body ( .. Spina
pifida
pressure CSF herniation Cause cystic swelling called meningeocele .
spinal cord Meningo-myelocele CSF CSF secretion ) spinal canal ( SC back pressure 4th ventricle Pressure
3rd ventricle
hydrocephalus
Arnold Chiari malformation hydrocephalus J
2- May not anatomical defect.
sub arachinoid space pathway ) CSF (

1- Sub arachinoid hemorrhage and hematoma
2- Sub arachinoid adhesion
meningitis fibrosis3- Abnormal metabolites as in muco-poly saccharidosis
3- Sinus thrombosis in SSS or in lateral sinus.
thrombosis .. absorption lateral sinus Pressure SSS
Auditic hydrocephalus cholesteatoma cochlear disease lateralsinus thrombosis

secretion absorption ) Non obstructive ( communicating

hydrocephalus

Pathway CSF Patent

23

B- Obstructive ( Non- communicating ) hydrocephalus

: obstruction
1- congenital stenosis or atresia
a - Foramen of Monoro.
Congenital stenosis or atresia ( )
b - Congenital at foramen of magendi and lushka
Congenital Dandy Walker malformation
4th ventricle Pressure magendi or Lushka Dandy Walker Occipital area cerebellum
malformation
hydrocephalus with marked occipital enlargement
c - Congenital at aquiduct of Sylivus.
zigzag shape aquiduct of Sylivus obstruction forking
Aquiduct of Sylivus vein of Galen Partial obstruction ...
2- Acquired causes
As space occupying lesion as tumor, abscess, hemorrhage and hematoma foramen

3- congeniatal infections STORCH
toxoplasmosis congenital infection gliosis fibrosisClinical picture
A - Before closure of sutures & fontanels

1- skull circumference
2- widely opened fontanel :
) ( 18 1.5 6 4.5 anterior fontanel

. rickets delayed closure called widely opened fontanel
birth diameter diameter

5 anterior fontanel

Enumerate causes and discuss how to diagnose one of them ??

delayed closure widely opened

Hydrocephalus, megalocephaly ...
3- still opened post. F.
. Posterior fontanel
4- widely separated sutures:
widely separated sutures
5- scalp:
dilated and visible scalp veins Stretched skin
6- craniotabes
. ) (
7- Direct percussion on skull bone
dullness resonant stony dullness
Mac Ewen's sign resonant
8- trans illumination :
anterior fontanel ( J ) torch

24

 ) ( differential diagnosis
megalocephaly .. opaque torch trans illumination
9- Walker malformation
10- Meningocele
11- manifestations of increase ICT :
manifestations ICT ) ( cry
anterior fontanel ICT So, late )
(
12- Eye :
upper part of the cornea is covered by the eye lid lower part of corneal not covered by lid !!
lower part of the cornea is Covered by lower lid Sunset appearance
...,

skull stretch occipito-frontalis eye brow

brain (Phospholipid (fat

optic nerve globe inferio medial surface brain
optic nerve globe

squint nucleus .S.R cortex
supra tentonrial ) ( compression SR IR
B - After the closure of the fontanel and sutures
1- Early manifested with ICT .
2- Skull is not significantly enlarged .
N.B. ICT projectile vomiting. & It is a good sign.
.... Good prognosis
NB hydrocephalus intra uterine period
Investigations
1- Skull X-ray .
hydrocephalus
2- CT or MRI
.. obstruction
.. lateral ventricle dilated foramen of Monoro
3- Positron emission topography ( PET ) .
.. MRI anatomy
4- Spectroscopic PET
anatomy functions
) 5- Ventricular pressure monitoring ( CSF
Progressive or stationary.
6- Serology of STORCH.
Treatment

mild or moderate Just medical treatment
1- Mediacal ttt :
lines 3
1- Is there is a cause treatment of the cause

25

2- secretion of CSF
salt restriction - Carbonic anhydrase inhibitor ( Diamox )
choroid plexus secretion
- Give cortisone in congestion
3- absorption of CSF
blood vessel osmotic pressure
Give mannitol osmotic pressure in SSS CSF absorption
2- Surgical ttt :
neurosurgery
1- Surgical removal of the cause
2- If choroid plexus , papilloma
diatheramy cryotherapy
3- If abscess or hematoma
Lateral ventricle shunt
shunt
a- Lt. atrium ( ventriculo-atrial shunt ) but may cause LSHF
b- to pleura :
ventriculo- pleural shunt pleura
inflammation and fibrosis ( pleura csf ) .. respiratory distress

c- to peritoneum :
peritonium ... ventriculo-peritoneal shunt

shunt
d- to cisterna magna :
cisterna magna ... Ventriculo-cisternal shunt
4- Obstruction of foramen of Monoro >>> third ventriclostomy
Complications of the shunt
1- Obstruction of the shunt.
) ( shunt
2- Infection meningeo-encephalitis

3- Brain dysfunction

4- Thrombus , arrhythmia ( on heart ) , Focal GN ( peritoneal sac )
5- Acute shunt failure
shunt

45 44

1- Macrocranium
percentile 95 2SD + skull circumference skull circumference 35

42 6
46 12
49 3
0.5 cm every year 7 3

26

 0.25 cm every year 12 7

Causes of macrocranium
1.
2.

size of bone itself .

Or intra cranial content .

1 - size of the bone .
medulla in between outer cortex and inner cortex skull flat bone
bone
a- Medulla :
Inner )Inner cortex outer cortex
.. medulla ( brain
skull circumference

chronic hemolytic anemia
Due to activity of bone marrow
b- cortex :
cortex .. cortex Protein part osteoid tissue
Mineral part
Mineral part Ca- Ph
excess osteoid tissue deposit suppression bone Ca
cortex
1- protien part
rickets .. bone Ca
primary hyperphosphatemia .. Ph
ph
ratio Serum Ca / serum Ph fixed number
stones .. .. ( 40 ) 45
Ph .. Ph
Ph Excess loss of Ca Ca in bone
protein part .. osteogenesis imperfecta
2- mineral part as in thickness of the bone
( osteopetrosis ( Marble bone disease osteoclast .. Ca Ph osteoblast .. osteoclast cortex ..
3- familial

2- intra cranial causes .
1- cerebrospinal fluid CSF . as in hydrocephalus .
2- Brain size .


a- increase number of cells
b- increase size of cells:
.. megalencephaly( )
1- MPS
(mucopoly saccharidosis (MPS
MPS

27

CSF absorption .. sub arachinoid space mucopolysaccharides

size of the brain .. brain cells ..
2- Achondroplasia
achondroplesia


.Shallow posterior fossa partial obstruction of sub arachinoid space by cerebellum

.May number or size of the brain cells
3- Hydranencephaly
- No cerebral hemisphere but 2 sacs filled with CSF
Caesarean
brain stem
4- Sub dural effusion or hemorrhage
hydrocephalus
Micro cranium -2

skull circumference
( 46 ) 40

micro cranium
3rd percentile SD 2-
causes
1- microcephaly:

size of the brain

brain

Micro cephaly
( cereberal palsy ) CP

( post natal + natal + ante natal )
+
3
1- Familial
small brain with normal function
2- Chromosomal disordes Down .....
3- Fetal alcohol syndrome
.. ..
Clinical pictures of microcephaly
- Small skull .
- No manifestations of ICP .
- Early, fontanel is open .
CP
2- early closure of sutures:
cranio- sentosis cranio-stenosis sutures one or more cranio stenosis
cranio sentosis pre mature closure of one suture
line of sutures brain growth
growth
sutures skull bone So, coronal and lambdoid sutures ant-post enlargement of the skull
Sagittal and Metopic sutures laterally

28

 cranio stenosis
C/P of craniostenosis
1- abnormal shape of skull:
sutures
1- sagittal
sagittal suture
Called Dolecocephaly
2- coronal
coronal .. ) Called Brachy cephaly ( bilateral coronal suture
)3- lambdoid or coronal (unlat.
coronal suture ) ( Unilateral lambdoid suture .. ) (
Called Plagio cephaly
coronal anterior plagio cephaly
lambdoid posterior plagio cephaly
4- metopic
metopic suture Pointed
Called trigono cephaly
Closure of sutures in lateral part only but central still opened -5
acrocephaly
Closure of sutures in lateral part only but central still opened

6- closure of all sutures

Pointed head Oxy cephaly
2- increasde ICT
sutures brain .. manifestation of ICT
.. high pitched cry
+ vomiting convulsion
Investigations of craniostenosis
Skull X-ray abnormal shape + silver beaten appearance .
Treatment of craniostenosis
sutures
Craniotomy open
.1
Cranioectomy
.2
Mental Retardation
Definition
Impairment of intelligence .
.. ,
-1 memory
..
-2 mode of thinking


) MR (
-3 adaptive social behavior




-4

29

Etiology

) CP ( cerebral palsy

Enviromental causes most ommon cause of MR
, ) (


Socially dissociated

curable
Post immunization as DPT
) encephalopathy (P 6 .. MR degree encephalopathy
Chromosomal
CP
How to prevent CP ???? MR
Good antenatal care .
Drugs .
.
Irradiation .
. ultra sound
STORCH infection .
fever skin rash
Previous history of chromosomal abnormality
chorionic villus biopsy
early abortion
Natal causes prevent causes of obstructive labor .

1-

231-

2-

3- Post natal care of baby as early detection of hypothyroidism & other in born errors .
Investigations
1- To reach the etiology
IQ
MR IQ 70
:
1- mild
IQ 50-70 Mild
educable ) (

IQ 4-3
.
2- moderate
IQ 35- 50 moderate Imbecile
.. Just trainable
severe -3
IQ 20 - 35 sever idiot
..
profound -4

30

2- IQ

 profound IQ 0- 20

Just MR ..
ttt
As CP but no need to physiotherapy .
Floppy baby
3 written ..
severe generalized hypotonia
3
floppy
floppy

floppy
supine
.. 3 .. examiner
called head lag

1- Head lag
drop of the head backwards due to hypotonia of the neck muscles
2- curved
neck back extension examiner
back muscles .. extension trunk

+ Curved back


3- Dangle hands and legs
4- frog leg position
.. both lower limbs flexion adduction ..
..
5- Drop of head ant. + dangle of the lower limbs
vertical suspension
generalized hypotonia
1.
Central as :
Chromosomal down , trisomy 13 ( not trisomy 13 of mongolism ) .
CP atonic ataxic .
Other syndromes : As Lowe's syndrome or Pradder Willi .
2.
Peripheral causes .
lower motor neuron lesion
AHCs extensive polio, Werding Hoffmann, Meningo-myelocele .
Peripheral nerves causes of peripheral neuropathy .
Myoneural junction .
Muscle causes of myopathies
1.
2.
3.
4.
5.
6.
7.

???? How to approach diagnosis

Abnormal features in chromosomal So, central not peripheral .
Convulsions central .
If MR central .
If deep reflexes normal or exaggerated So, central
if hypo peripheral .
Muscle wasting peripheral .
Fasciculation peripheral .
Loss of consciousness central .

31

1.
2.

1.
2.
3.
1.
2.

central causes
Abnormal features chromosomal .
Course
if progressive ( other syndromes ).
If stationary CP atonic exaggerated tendon reflexes .
Or Ataxic .
Causes of increased ICT
3
Cranio stenosis .
Pseudo tumor cerebri .
Space occupying lesion .

Hypertensive encephalopathy .
CNS infection .
46 45

Transverse myelitis
- Mostly auto immune disease
DPT vaccine .. viral infection

inflammation of part of spinal cord mostly cervical or thoracic
auto immune So, acute .. segments

1.
At level of the lesions destructed AHCs LMNL
Level
2.
Below level of the lesion .
A- Motor
as pyramidal tract to the AHCs below So, UMNL
6 2 .. acute attack criteria

Weakness or paralysis
( Hyper tonia ( clasp knife
Exaggerated deep tendon reflexes
( Muscle state ( normal
Clonus , Babiniski

flaccid paralysis .. shock stage
B- sensation
lost below superficial sensation in lateral spino thalamic tract
deep sensation lost below level
C- disorder in sphincteric control
brain signals rectum bladder evacuation .. bladder and rectum spinal cord Local reflex
automatic bladder ( 5 ) baby So, disorder in sphincteric control
investigations
TM
spinal cord CT or MRI

radiological evidence in TM
Other focal lesions
32

1234-

1.

2.

- Also, do CT and MRI on brain to exclude auto immune encephalitis .

Treatment
- It is self limited disease in more than 50 % of cases .
- So, just symptomatic treatment .
- All immunotherapy under trial .
Seizures
Cerebral dysrrhythmia Abnormal signals of the brain
motor seizures convulsion
attack of seizures fits Types of convulsion

Tonic convulsion .
High arched back continuous contractions of all groups of muscles Clonic : repetitive contraction and relaxation .

Tonic - clonic .
..
N.B. Generalized tonic -clonic convulsion the most common type of convulsion in pediatrics.
Myoclonic repetitive contraction and relaxation in one group of muscles
extensors contractions flexors

Etiology :

1ry seziures.
idiopathic
a) Epilepsy
b) Febrile convulsion
fever
2ry seziures .

a) Congenital cerebral malformations
b) Inflammatory

brain abscess encephalitis meningitis
c) Tumors
metastasis in brain leukemia .. 1ry brain tumor as meningioma
d) Degenerative disease
.. apoptosis Neurons .. Unkown etiology
neurotransmitter

If early convulsion grey matter degenerative disease
If late convulsion white matter degenerative disease
e) Drugs analeptics amphteamine

f) Vascular causes
Infarction in brain
IC hemorrhage
Vasculitis ( as lupus encephalitis )
g) Trauma
h) Encephalopathies
Renal
Hepatic
33

i)

Hypoxia
CO2
CO
Lead toxicity
Metabolic disease
You must exclude metabolic disorders
hypoglycemia
-Tatany and hypernateremia dryness of the brain cells
vit. B6 Vit. B6 dependence

j) Toxicology
..
Classification of epilepsy

clonic myoclonic
- Clonic contraction of all muscles
flexors extensors
extension flexion

- Myoclonic one group So, flexion followed by extension

classification of epilepsy
1.

Generalized
Loss of consciousness .
Aura .
May tonic , clonic, tonic clonic , may myoclonic , may infantile spasm
2.
Partial .
a) Simple no loss of consciousness .
May motor
.. May sensory
.. psychic
psychic
NB. If psychic multi system complain not related to each other
adloscent female
) .. ..
(
So, psychic
May autonomic as abdominal epilepsy abdominal colic
then EEG by exclusion ..
May psychic
b) Complex partial
Loss of consciousness .
c) With 2ry generalization .
3.

Un calssified .

34

Febrile convulsion

Idiopathic causes
febrile convulsion febrile convulsions over diagnosis criteria
Criteria of febrile convulsion
1- Age 3 months to 5 years .
years 6 6months
2- Fever .
:
a- High grade fever 38
b- Rapidly rising
12 ..

40
c- Attack of convulsion occurs within 12 hours
( fever 12 )
3- Convulsion description .
a- Generalized tonic clonic

b- duration
febrile convulsion .. 15 ..

c- No post ictal loss of consciousness
d- Not repeated in the same disease
..

( ) fever ) ..
( febrile
4- No manifestations of any CNS infection .
5- source of fever
fever .. extracranial source of infection

1234-

epilepsy febrile convulsion

attack EEG
N.B. In febrile convulsion EEG is normal 2-3 weeks after the attak
Positive family history of epilepsy .
If attack less than 9 months .
Abnormal neurological signs .
Atypical presentation .
3 attack -Or one of attack is focal or not generalized
Treatment of febrile convulsion

1.
2.
3.
4.

fever .
Treatment of the cause .
May short term anti convulsants .
Avoid fever later .

35

Investigations of seziures
1. EEG .
attack
-Not a good positive and not a good negative
clinical
type 2. may need lumbar puncture
CNS infection
3. Ca & Mg level .
4. Glucose level .
5. May need fundus examination
ICT papilloedema
6. MRI or CT.
: history Indications
History of head trauma
History of malignancy
History of bleeding tendency
History of focal convulsions
Or focal neurological signs
7. If suspect toxins .

Treatmetn of any convulsion
1. Treatment of the cause .
2. Anti epileptic drugs .
Status epilepticus
Means that attack of convulsion persist > 30 mins.

Treatment
ABCD
A. Air way mouth gag .
mask of O2 .. secretion

Do Endotracheal intubation & even Mechanical ventilation
B. Blood pressure .
C. Circulation .
D. Drugs
( valium ( Diazpam

If over dose R.C. respiratory failure .
- Dose 0.1 - 0.3 mg / kg/ dose
- Slowly IV
.. .. 3 .. ..

Phenobarbione or phenotoyin immediately

very slowly IV loading dose 15-20 mg /kg / once 1/3
mg /kg /day 5 -3 Maintenence dose 12 General anasethsia neuro
enocrine 46 26

36