( BETA EDITION)

With
Prof. Dr Mohammed Abo El-Asrar

Edited By
El-Azhar Medical students 2012

 
 


" "
: 5

: : : :

 41

NEU RO
written
1- Floppy baby (generalized hypotonia ) .

2- MR.
MR
3- Convulsions
clinical
1. C.P.
2. Hydrocephalus
3. Duchenne myopathy
oral
Skull XX-ray

Intro ducti on

clinical pictures

Clinically :
neurology comment
1. Motor system.
2. Sensory manifestations.
3. Autonomic manifestations.
4. higher functions as MR or convulsions.
1 - Motor system
A Muscle power
Neuro anatomy :
-
UMN orders LMN

 cranial nerve or anterior horn cells

Axons .... target muscle

Any muscle fibers .* muscle
5 fibers fiber
AHC
 So, 5 AHCs give 5 ms fibers
) ( order
then to 5 fibers
5 AHCs
fiber
* 5 neuron fiber ms fiber
4 ms fibers
80 % ....
* 60 % )weakness fibers (
- Loss function fibers paralysis


muscle power
Weakness or
?? 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 occ
occur
ur with flexion of hip also extensor with extensors

C - Abductor & adductors
adductors abductors
.
3- Trunk.
A - Flexors
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 6 cm
Origin Insertion biceps 8 cm
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
Hyper tonia Level of the lesion ... Pyramidal tract
contract impulses stretch nerve
Lower Upper nerve
Lower
Lost tone Lower motor neuron lesion
tone Nerve
Lost hypo tonia tone Lower motor neuron lesion Nerve lesion
(
C - Deep Tendon Reflex
: tendons hummer
sudden passive stretch  sudden massive activation of AHCs  sudden massive contraction of all muscle
comment
fibers
deep tendon reflexes
Hyper refle upper motor neuron lesion
D - Muscle state
Size of the muscle 
lower motor Pseudo hypertrophy
hypertrophy

vascular flow energy vascular supply muscle vascular flow ... waste product
vascular supply activity Muscle neuro vascular connections muscle vascularity
nerve supply
vascular flow
arterial flow V.D.
signals Nerve
vascular supply Lower motor neuron vascular supply
so early ) ( wasting Muscle degeneration Upper motor neuron lesion disuse atrophy just so late Upper motor neuron lesion
E - Muscle fasciculation
isolated fiber contraction fasciculation contraction fibers ..... fibers
fasciculation : tongue Muscle  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
unilateral

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








So , Distal more than proximal

So,
So, Proximal more than
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- Senso ry system
superficial sensation deep sensation



in posterior root ganglion
1st order neuron dendrite skin ) Pathway (
3 - Autonomic manifestations
Pulse rate.

bradycardia tachycardia
Heart


Blood pressure
heart

If   sympathetic.

If   parasympathetic.


sphincteric control :
Sympathetic  retention.
Parasympathetic  incontinence.
4- Higher functions
development
LMNL
lower motor neuron lesion
lesions lower motor neuron
muscle AHCs lower motor neuron
1. AHCs (or cranial nuclei).
2. Peripheral nerve (cranial nerve).
3. Myo-neural junction.
4. Muscle.
lower motor neuron lesion
criteria of lower motor neuron lesion
Criteria o f 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- EMG
CP ) Upper motor neuron lesions Lower motor neuron lesions
( cerebral palsy
neuropahtic or myopathic
signals fibers waves
If myopathic
Muscle
If neuropathic.
AHCs Nerve 

Investigation
2- Nerve conduction velocity for neuropathic.
neuropathic.
myopathy

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

.
.

Hypotonia & neuromuscular disease
Disease affecting AHCs


Poliomyelitis.
Infection

Spinal muscle atrophy.

Amytrophic lateral sclerosis  rare.

Spinal m uscle atrop hy
Type one = Werding Hoffman disease
....

- Autosomal recessive disease
 female = male
 Affected persons  homozygous

carrier ( <<< )

So,- positive family consanguinity + +ve family history

C/P


5 neurons ) give (
5 nerve fibers to muscle ) apoptosis & mitosis (
no mitosis and no apoptosis .... brain cells
neuronal cells ... gene apoptosis of 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 :
neurons
weakness then paralysis with hypotonia and hyporeflexia tongue fasciculation diagnostic) - Distribution : bilateral, symmetrical and ( proximal and distal
3- Cranial nerves affection :

Cranial nerve bulbar cranial nerves ... :
* Weak suckling and swallowing
* Repeated aspiration and chocking
* When cry >>> nasal tone of voice
* Hoarseness of voice
| |
tongue fasciculation diagnostic
 as it is a degenerative disease neurotransmitter
) polio ( irritation fasciculation
| |
sensation .... Normal No MR or convulsions

Cranial nerve nuclei

staring look
) (
Investigations

muscle

10

EMG  neuropathic.

Nerve conduction velocity  normal.

CPK  normal

Treatment
Supportive only.
5
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 ) 4-5 ( ...

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

11

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

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

12

S.C. & brain nervous system demyelination Guillian

(Encephalo-myelo-radiculopathy cranialis)
roots
radiculopathy - S.C.
myelo - Brain

encephalop -

cranial ns
Cranialis 8- higher functions : No MR or Convulsion

Investigations
a)

EMG  neuropathic pattern.

b)

Nerve conduction velocity  impaired So, peripheral neuropathy .

c)

May nerve biopsy

rarely indicated
.... Nerve

d)

CSF examination  Cyto-albuminous dissociation.

Cyto  cells Albuminous albumin.
CNS auto immune diagnostic inflammatory cells protein
CSF 1.
2.
3.

If meningitis   neutrophils &  protein.

If viral encephalitis   lymphocytes
lymphocytes &  protein.

If auto immune   protein ( as inflammation ) + no inflammatory cells

auto immune
Inflammation .. Infection
Treatment

1st line of treatment  IV immunoglobulin

( )

 steroid and plasmapheresis

ABs

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

13


Introduction

Muscle ATP
:
Skeletal muscle - Heart - Brain cells
ATP
generator
mitochondria glycolysis .. ATP ATP glycolysis 3


muscle
generator .. creatine creatinine kidney generator endoplasmic reticulum
creatine creatinine
cytoplasmic enzyme creatine phosphokinase Muscle cells endoplasmic
reticulum sacrolemmal membrane carrier protein dystrophin
Duchenne
X- linked recessive gene band - region Z - short arm X- chromosome

& as

it is x linked So, common in males

Duchenne
3
?????? New mutation, turner or
C/P

dystrophin abnormal CPK creatine creatinine ATP ...
3
skeletal muscle
 ATP Muscle fibers degeneration
.. fibers
So, progressive course.
fibers
lower motor
motor neuron lesion criteria :

Weakness or paralysis
Hypotonia & hypo reflexia
????What about muscle state
muscle degeneration ) ( fibrous and fatty tissue
) ( fibrous and fatty tissue Muscle

Muscle wasting

14

 fibro
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 (

15


medial border serratus anterior rhomboidus muscle ...
Medial border scapula serratus anterior medial
pushing border border
5- Pes cavus  high arched foot
deformity fibrosis
 6- Positive Gower sign
back muscles
) tone
(

Complications
Heart myopathy .... heart
heart failure brain cellsSo, may: MR  20 - 30 % of cases.
 - Epilepsy
) Irritate (

Causes of death
- H.F.
- UTI  due to hypercalcemia >>> so, repeated renal stones

Muscles osteoblast bone Ca & Ph
as in rickets
Investigations
 EMG  myopathic cytoplasm.
  Enzymes
... degenerated muscle  aldolase  SGOT  CPK
 Muscle biopsy  fibro-fatty tissue.
 Urine analysis   creatine &  creatinine.
 ECG & ECHO  cardic affection.
 EEG & IQ  for MR & convulsions.
  Gene study
Duchenne prenatal diagnosis .
 As the mother is a carrier  slight  in CPK.
Treatment

16

- Physiotherapy + supportive treatment.

- Recently  myeloblast transplantation.
- Under trial  gene therapy.
therapy.
UMN L
3 neurons
1- Motor area 4
And 2 accessory neurons
2- Basal0
0 ganglia 3- Cerebellum
1- Functions
Functions of motor area 4
1- Initiation of voluntary movement.
LMN Area 4
Weakness or paralysis ...

Distribution
Area 4
. limb
Mono plegia
.
Hemi plegia
. L.L.
Cortical para plegia
: 2 limbs
Quadri plegia
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
left more than left  double hemiplegia
k
43 42

Proximal or distal muscles Upper motor neuron lesion

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

area 4 distal area 4 & 6 proximal
proximal muscles motor area 6
complete loss of function of distal muscles ... area 4
But proximal muscles Area 6
So, in area 4 affection  distal > proximal

17

 Area 4
2- Suppression of muscle tone & deep tendons reflexes .
suppression .. anti gravity muscles) anti gravity pro gravity
anatomical position ( Pro gravity
) ...
flexors ... Lower limb
.. flexors extensions .. (
Upper limb
Extensors
Pro gravity

)a

Flexors
anti gravity

gravity

)b

Lower limb
Extensors
anti gravity

Flexors
pro gravity

more suppression adductors abductors Area 4  tone &  deep tendon reflexes
suppression
But anti gravity > pro gravity
hemi plegia ..
Extensors > flexors
knee
  flexion extension
) flexion upper limb
(
adductors abductors shock stage
Area 4 tone adductors special gait adductors lower limbs trunk
So, circumduction gait
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 3-4

18


3-4 Area 4 maturation
suppression to pathological reflexes
neonatal reflexes
: 2&1
1- Moro reflex :

..

3-4
) (
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 o f 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

19

So, there is hypertonia (clasp knife, spasticity)

antigravity > pro gravity
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.
maturation ) area 4 ) ( Babniski sign is positive NB
(
cerebellum .. area 4
2- C erebellum
functions 3 parts physiologically
2-

Archi cerebellum :
anatomically

Motor area 4

Inner ear
Effect of lesion

.. Mechanism
archi cerebellum drunken gait ..
2- Paleo cerebellum :
animals anatomically

spinospino-cerebellar  ventral lateral spinal cord .. muscle tone

tracts
Effect of lesion
Hypo tonia
tone .. paleo cerebellum
deep tendon reflexes Muscle tone stimulation cerebellum

( tone )
tone
hypotonia and hypo reflexia ...
weakness or paralysis ... lower motor neuron lesion

20

3- Neo-cerebellum :
Functions & effects of lesion
In human
paleo neo area 4 .. ...
neo cerebellum

..
1- estimation of distance :
... estimation of distance area 4 either  dysmeteria
 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 - - nystagmus3- break function
function  ability to stop mov. At any level of it.


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

paleo paleo- cerebellum

21

 ... 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,
Dysmetria, dys diadia-dokinesia , kinetic tremors, nystagmus, rebound +
gait disorder as drunken gait deviation gait zigzag gait
Upper motor neuron
3- Basal ganglia
3 functions
1- Emotions associated with movement.

associated movement
A - Swinging of the hands

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

exaggerated emotions

) ( basal ganglia


.... ....
) basal ganglia (
C associated movements with speech :
associated movement ..
Effect of lesion
basal ganglia disturbed emotions associated movement chorea 2- Suppression of basal activities of motor area 4.
basal ganglia

22


Motor area 4 )( .. 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 ....
twisting movement in the trunk
dystonia

3- Effect of basal ganglia on tone
Mediators basal ganglia

 stimulation.  Acetyl choline

Dopamine  suppression of tone.
basal control tone
basal ganglia
  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 lead pipe
rigidity cog wheel

lead pipe resistance

cog wheel resistance
areflexia tone
tone agonist and antagonist Knee flexors reflex
- Rigidity more in flexors and adductors
parkinsonism S
& shuffling gait
short steppage
4- Coarse static tremors

) Cerebral palsy ( CP
Definition

23

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
drugs or irradiation teratogen -2
Hypoxia intrauterine -3
circulation placenta
or  mother pressure
pressure placental insufficiency  Toxiemia of pregnancy

brain abnormal metabolites
metabolites
inborn error of metabolism -4
by exclusion congenital anomalies in fetus brain -5
by exclusion 2- Natal
A Delayed delivary
) (  true uterine contraction .. placenta contraction ... 12 6 cerebral anoxia
brain cells hypoxia

hypoxia

cause hypoxic ischemic encephalopathy

B - Birth injury
intra cranial .. gift forceps 6 hemorrhage
3- PostPost-natal

24

neonatal period
A infection :
as meningitis or encephalitis ... infection
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

25

3- Extra pyramidal type CP

basal ganglia

1- Emotional & associated movement disturbance
2- Abnormal movement ( chorea, asthetosis, dystonia ..etc)
3- tone

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

mentality IQ ..
... audiometry
.. ... " "
.... ... ..
......
Treatment

26

- 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 ventricle Lateral ventricle Lateral ventricle choroid plexus CSF foramina 2 lateral foramina brain 4th ventricle aquiduct of sylvius
brain sub arachinoid space CSF of Lushka
sub foramen of magendi spinal cord sub arachinoid arachinoid around spinal cord
SSS sub arachinoid space brain SPC
CSF CSF absorption arachinoid villi  superior sagittal sinus
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
A Non obstructive
obstructive (communicating) Hydrocephalus:
1- Excess secretion or production of CSF
... 200 CC absorption 200 CC
200 CC absorption 300 CC
2- Decrease absorption.
200 100 CC 200 CC
B - Obstruction.

A - Non obstruct ive ( commun icating ) hydrocephalus
1- excess secretion
choroid plexus 1- congestion choroids plexus:

27

 mucosa congestion Mucous membrane congestion

excess secretion of mucous
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
2- pseudo tumor cerebri :
CSF brain tumors manifestations of  ICT
ICT
Causes of pseudo tumor cerebri
1- Hyper or hypo vitaminosis A.
2- Hyper vitaminosis D.
3- Pseudo hypo para thyroidism 
End organ resistance .... receptors 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

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

A Achondroplasia  shallow post. Cranial fossa :
posterior cranial fossa cerebellum cerebellum posterior cranial fossa cerebellum shallow fossa curve sub arachinoid space space
Post fossa

28

  CSF Achondro plasia shallow post fossa hydrocephalic changes large skull achondroplasia
large skull
B Arnold chiari malformations  (meningeocele or Meningo-myelocele) and Hydrocephalus

spinal foramen magnum )brain (
spinal cord CSF
Brain cerebellum Downward displacement of the brain
sub arachinoid space brain foramen magnum foramen of magendi CSF spinal canal CSF ) S.C .. .. foramen magnum (
.... sub arachinoid space CSFpressure
spinal canal
intra uterine period embryo vertebral body ) (
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
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
2- May not anatomical
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 lateral sinus thrombosis

29

Non obstructive ( communicating ) hydrocephalus  absorption  secretion

Patent CSF Pathway

B- Obstructive ( N on- 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
Dandy Walker malformation Occipital area cerebellum
hydrocephalus with marked occipital enlargement

c - Congenital at aquiduct of Sylivus.
forking zigzag shape aquiduct of Sylivus obstruction
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 fibrosis

gliosis
congenital infection -

Clinical picture
A - Before closure of sutures & fontanels

1-    skull circumference
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

30


... Hydrocephalus, megalocephaly
3- still opened post. F.
Posterior fontanel .
4- widely separated sutures:
sutures:

sutures widely separated
5- scalp:
Stretched skin scalp veins dilated and visible
6- craniotabes
) ( .
7- Direct percussion on skull bone
stony dullness resonant dullness
'Mac Ewen
resonant Ewen's sign
8- trans illumination :
) torch ( anterior fontanel
) ( differential diagnosis megalocephaly

opaque

.. torch trans illumination

9- Walker malformation
1010- Meningocele
1111- manifestations of increase ICT :
manifestations  ICT ) (cry
So, ) (
anterior fontanel ICT So, late

1212- 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
...

occipito eye brow skull stretch occipito-frontalis

brain (fat) Phospholipid

optic nerve globe inferio medial surface brain optic nerve
globe

squint nucleus S.R. cortex tentonrial supra
) (compression SR IR

31

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
intra uterine period hydrocephalus NB
Investigations
1- Skull XX-ray .
hydrocephalus
2- CT or MRI 
obstruction ..
dilated foramen of Monoro .. lateral ventricle
3- Positron emission topography ( PET ) .
anatomy .. MRI
4- Spectroscopic PET 
functions anatomy
5- Ventricular pressure monitoring ( CSF )
Progressive or stationary.
6- Serology of STORCH.
Treatment

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

32

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 :
.. respiratory distress ventriculo- pleural shunt pleura
inflammation and fibrosis ( pleura csf )

c- to peritoneum :
ventriculo-peritoneal shunt... peritonium
shunt
d- to cisterna magna :
cisterna magna ... Ventriculo-cisternal shunt
4- Obstruction of foramen of Monoro >>> third ventriclostomy
Complications
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
k
45 44

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


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

33

Causes of macrocranium
1.

 size of bone itself .

2.

Or  intra cranial content .

1 -  size of the bone .

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

chronic hemolytic anemia
 Due to  activity of bone marrow
b-  cortex :
cortex .. cortex osteoid tissue
Protein part
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
(Marble bone disease ) osteopetrosis osteoclast .. Ca Ph osteoblast .. osteoclast cortex ..
3- familial

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

34

2-  Brain size .


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

skull circumference
( 46 ) 40

micro cranium
3rd percentile -2 SD
causes
1- microcephaly:
 size of the brain
brain

Micro cephaly
(cereberal palsy ) CP

35

( 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 
pre mature closure of one suture
cranio sentosis 
line of growth
sutures brain growth
sutures skull bone So, coronal and lambdoid sutures  ant-post enlargement of the skull
Sagittal and Metopic sutures  laterally
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.)
( Unilateral ) coronal suture ( ) .. lambdoid suture
Called Plagio cephaly
anterior plagio cephaly

coronal 
posterior plagio cephaly

lambdoid 

36

4- metopic
metopic suture
Pointed
trigono cephaly
Called
5- Closure of sutures in lateral part only but central still opened
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
Investigations of craniostenosis
Skull X-ray  abnormal shape + silver beaten appearance .
Treatment of craniostenosis
sutures
.
.

open
Craniotomy

Cranioectomy
Mental Retardation
Definition

Impairment of intelligence
intelligence .
..
-1 memory
..
-2 mode of thinking


) MR (
-3 adaptive social behavior




-4
Etiology

) CP ( cerebral palsy

37

1- Enviromental causes  most ommon cause of MR

) (

Socially dissociated


curable
2- Post immunization as DPT
) encephalopathy (P 6
.. MR degree encephalopathy
3- Chromosomal
CP
 How to prevent CP ????  MR
1- Good antenatal care .
 Drugs .
.
 Irradiation .
. ultra sound
 STORCH infection .
fever skin rash
  Previous history of chromosomal abnormality
chorionic villus biopsy
early abortion
2- Natal causes  prevent causes of obstructive labor .
3- Post natal care of baby as early detection of hypothyroidism & other in born errors .
Investigations
1- To reach the etiology
2- IQ

IQ

MR IQ 70
:
1- mild
50-70
IQ
Mild
educable ) (

38

 IQ 3-4
.
2- moderate
35- 50
IQ
Imbecile
moderate
.. Just trainable
3- severe
20 - 35
IQ
idiot
sever
..
4- profound
0- 20
IQ

profound

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

floppy
supine
examiner .. 3 ..
 called head lag

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


3- Dangle hands and legs
4- frog leg position
.. adduction flexion .. both lower limbs
..
5- Drop of head ant. + dangle of the lower limbs
vertical suspension
generalized hypotonia

39

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
How to approach diagnosis ????

1.

Abnormal features in chromosomal  So, central not peripheral .

2.

Convulsions  central .

3.

If MR  central .

4.

If deep reflexes normal or exaggerated  So, central

if hypo  peripheral .

5.

Muscle wasting  peripheral .

6.

Fasciculation  peripheral .

7.

Loss of consciousness  central .

central causes

1.

Abnormal features  chromosomal .

2.

Course 
if progressive  ( other syndromes ).
If stationary  CP

 atonic  exaggerated tendon reflexes .

Or  Ataxic .
Causes of increased ICT
3
1. Cranio stenosis .
2. Pseudo tumor cerebri .
3. Space occupying lesion .

1.

Hypertensive encephalopathy .

2.

CNS infection .
46 45

T ransverse my elitis
- Mostly auto immune disease
DPT vaccine .. viral infection

40

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

1.

At level of the lesions  destructed AHCs  LMNL

Level

2.

Below level of the lesion .

A- Motor
So, UMNL
to the AHCs below
as pyramidal tract

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
- 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
-

41

Cerebral dysrrhythmia  Abnormal signals of the brain

motor seizures
convulsion
fits
attack of seizures Types of convulsion

1- Tonic convulsion .
High arched back continuous contractions of all groups of muscles 2- Clonic : repetitive contraction and relaxation .

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


Etiology :

1.

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

2.

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
neurotransmitter .. apoptosis Neurons .. Unkown etiology

 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 )

42

g) Trauma
h) Encephalopathies
 Renal
 Hepatic
 Hypoxia
 CO2
 CO

 Lead toxicity
i) 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

43

 .. psychic

psychic
NB. If psychic  multi system complain not related to each other
adloscent female
.. .. ) (

So, psychic
 May autonomic  as abdominal epilepsy  abdominal colic
.. by exclusion then EEG
  May psychic
b) Complex partial
 Loss of consciousness .
c) With 2ry generalization .
Un calssified .

3.

Febrile convulsion

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


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

b- duration

44

 febrile convulsion .. 15 ..
c- No post ictal loss of consciousness
d- Not repeated in the same disease
..
( febrile ( ) fever ) ..
4- No manifestations of any CNS infection .
5- source of fever
fever .. extracranial source of infection
epilepsy febrile convulsion
attack EEG
N.B. In febrile convulsion EEG is normal 2-3 weeks after the attak
1- Positive family history of epilepsy .
2- If attack less than 9 months .
3- Abnormal neurological signs .
4- Atypical presentation .
3 attack Or one of attack is  focal or not generalized Treatment of febrile convulsion
1.

 fever .

2.

Treatment of the cause .

3.

May short term anti convulsants .

4.

Avoid fever later .

Investigations of seziures

1. EEG .
attack
Not a good positive and not a good negativeclinical
type 2. may need lumbar puncture
CNS infection
3. Ca & Mg level .
4. Glucose level .
5. May need fundus examination
 ICT
papilloedema

45

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 ep ilepticus
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
( Diazpam ) valium

 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 1515-20 mg /kg / once
loading
loading dose 1/3
3- 5 mg /kg /day Maintenence dose 12 General anasethsia  neuro
enocrine 46 26

46