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With Prof. Dr Mohammed Abo El-Asrar
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41
NEURO NEURO written 1- Floppy baby (generalized (generalized hypotonia ) . 11 2MR. 2- MR
33- Convulsions clinical 1. 11 . C.P. 2. 2. Hydrocephalus 3. myopathy 3. Duchenne myopathy oral Skull X X--ray -ray
clinical pictures Introduction Introducti o n
Clinically :
comment
neurology
1. Motor system. 2. Sensory manifestations. 3. Autonomic manifestations. 4. higher functions as MR or convulsions. 11 - Motor system A – Muscle power : Neuro anatomy
cranial nerve or anterior horn cells LMN orders UMN .... target muscle Axons . fibers Any muscle AHC fiber 5 fibers * muscle So , 5 5 AHCs give 5 5 ms ms fibers ( order ) then to 5 fibers 5 AHCs fiber 4 ms fibers ms fiber fiber * neuron 5 .... 80 % ( fibers )weakness 60 % * paralysis fibers function Loss -
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muscle power
orr paralysis paralysis ?? Weakness o
History muscle 1- upper limb : A. A . Small muscle of the hands… A ... ( )
B. B B . Elbow…
extensiors
flexiors
C. C . Shoulder… C
flexors extensors adductors 2- Lower limb… A - Small muscle of the foot
normal muscle power Motor only motor and sensory sensory
B - Knee Knee extensors Flexion of knee occur occ ur with flexion of hip h ip also extensor with extensors
C - Abductor & adductors… abductors adductors .
3- Trunk. A - Flexors Flexor ss
B - Extensors
... extensors trunk B - Muscle tone
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tone origin
muscles .. Insertion Origin muscle stretched Insertion stimulation AHCs spinal cord signals spindle cells contract muscle hypotonia or atonia hyper tonia tone Paralysis and weakness
) tone ....Tone Tone 6 cm biceps Insertion 8 cm biceps Origin Insertion biceps stretch Origin insertion Origin is stretched <<<<
Impulses ( Muscle fibers
) muscle fibers
stretched dorsal root ganglion Axon .... spinal cord dorsal root ganglion T shaped Posterior horn cells dorsal root ganglion <<<< dorsal root ganglion impulses fibers nerve fibers Anterior horn cells <<< Anterior horn cells posterior horn cells <<<< contract ...Is contracting disuse atrophy tone Pyramidal
Inhibition
tone
pyramidal
( (:
) tone biceps ( ) biceps .... tone biceps tone <<< tone Pyramidal atrophy lost Inhibition pyramidal tract .... Inhibition tone pyramidal tract Lost ...... �
hyper tonia ↑↑↑ Level of the lesion ... Pyramidal tract contract impulses stretch nerve nerve Lower Upper tone
Hyper tonia
Lower
tone
Nerve tone tone Lower motor neuron lesion Nerve lesion ( Lost
L Lost
hypo tonia
Lower motor neuron lesion
C - Deep Tendon Reflex : tendons hummer sudden passive stretch sudden massive activation of AHCs sudden massive contraction of all muscle
fibers deep tendon reflexes Hyper refle upper motor neuron lesion
comment
D - Muscle state Size of the muscle
vascular flow energy vascular supply muscle vascular flow ... waste product activity Muscle vascular supply neuro vascular connections vascularity nerve supply muscl e vascular flow arterial flow V.D. signals Nerve Lower motor neuron vascular supply vascular supply so early ( ) wasting Muscle degeneration Upper motor neuron lesion disuse atrophy just so la te Upper motor neuron lesion
lower motor
Pseudo hy hypertrophy pertrophy
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
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weakness or paralysis muscle power .... ) ( Muscle fasciculation
muscle state
deep tendon reflex
tone
Distribution
1unilateral… … 11 - Bilateral or unilateral
bilateral 22- Symmetrical or asymmetrical …
extensor + extensor (...
)
33- Proximal more than distal
So , Distal more than proximal
–
–
So, than han distal So , Proximal more t (
)
proximal or distal
Upper limb lower limb distal ... proximal distal distal more aff ected than proximal comment distal Proximal distal Upper motor neuron lesion ( 2- Sensory system
deep sensation superficial sensation dendrite skin in posterior root ganglion 1st order neuron ) Pathway ( 3 - Autonomic manifestations
Pulse rate.
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bradycardia tachycardia
Heart
Blood pressure heart If sympathetic.
.
If parasympathetic. parasympathetic.
sphincteric control : Sympathetic retention. Parasympathetic incontinence. 4functions 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.
criteria of lower motor neuron lesion
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 fasciculation
AHCs ....
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facial palsy
.... Muscle junction distal Proximal ... distribution Muscle Investigations where is the lesion Investigations 1- EMG 11
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
2ic. 2- Nerve conduction velocity for neuropath nneuropathic. europath myopathy
Muscl e e ... superficial nerve . AHCs Nerve Normal peripheral neuropathy Impaired .
Hypotonia Hypotonia & neuromuscular disease Disease affecting AHCs
Poliomyelitis. Infection
Amytrophic lateral sclerosis rare. Spinal muscle atrophy.
Spinal muscle atrophy Type o ne = Werding Hoffman disease
....
- Autosomal recessive disease
female = male Affected persons homozygous carrier ( ) <<< So ,- positive family con sanguinity + +ve family history C/P
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5 nerve fibers to muscle ( give ) 5 neurons ) apoptosis & mitosis ( no mitosis and no apoptosis .... brain cells apoptosis of neuronal cells gene ... neuronal cells Programmed cell death Lower motor neuron : .. AHCs 1- decrease fetal Movement ( intrauterine ) : 16
multi para ( multi para )
.. ) 18
(
-
2- weakness then paralysis with hypotonia and hyporeflexia : weakness then paralysis with hypotonia and hyporeflexia diagnostic
neurons
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 fasciculat ion
neurotransmitter as it is a degenerative disease fasciculation irritation ( polio ) | | No MR or convulsions .... Normal sensation ☺ Cranial ne nerve nuclei staring look ( ) Investigations •
EMG neuropathic.
•
Nerve conduction velocity normal.
•
CPK normal
muscle
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Treatment Supportive only.
42 – 41 41 1
Post infectious poly neuropathy Guillian Barre syndrome Infection
Guillian Barre syndrome ) ( .. )) .. ( Introduction peripheral nerv e e motor and sensory
conduction of nerve impulse Nerve myelin nerve fibers
... jumping ( ) .. nerve impulse rapid transmission ... Schwann cells ... sheath Node depolarization node of Ranvier spinal cord sensory :
.. motor -
intrauterine
.. neuronal cells neck support myelination Neuronal cells -
... brain Neck muscle ( 3 ) neck support ... ( 4-5 ) spinal cord trunk muscles .... Innervation ( 9 ) ... arm thigh proxi mal muscles ... forearm hand foot more distal myelination
) ... virus measles , mumps, chicken pox, ( viral infection ..... myelin sheath antibodies ... auto -immunity coxachie virus .. nervous system
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-
:
( ) nerve impulse muscles ( AHCs or Cranial nuclei ) demyelination So , lower motor neuron lesion So, C/P
1- motor manifestations : as Weakness or paralysis. paralysis. , Hypotonia & hypo reflexia. reflexia.
muscle wasting . bilateral and symmetrical . distal Proximal ) signals .. spinal cord Motor signal .. ( ( signals ) So, distribution distal distal more than proximal proximal ........
2- sensory affection : As it is peripheral nerve so, so, sensory manifestations manifestations spinal cord sensation
pathway signal spinal cord spinal cord So, sensory loss distribution glove glove and stocking hypot hypothesia hesia. hesia 3- Autonomic affection : degeneration autonomic
If both sympathetic and para sympathetic degeneration 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
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S.C. & brain nervous system demyelination Guillian
( Encephalo-myelo-radiculopathy Encephalo-myelo-r adiculopathy 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
d)
.... Nerve
CSF examination Cyto-albuminous dissociation. Cyto cells …… Albuminous albumin. CNS auto immune
diagnostic protein CSF inflammatory cells 1. 11 . 2. 2. 3. 3.
If meningitis neutrophils neutrophils & protein. protein.
If viral encephalitis lymphocyt lymphocytes protein. lymphocyt es & protein.
If auto immune protein protein ( as inflammation ) + no inflammatory cells cells auto immune Inflammation .. Infection Treatment
1st st IV immunoglobulin 11 line of treatment IV (
)
steroid steroid and plasmapheresis
ABs
Physiotherapy.
3 3 age of onset 3 auto immune N.B. Any cranial nerve affection here here is bilateral. Myoneural Junction affection Causes - Myasthenia gravis. - Organo phosphorous compound. - Tick-born paralysis. - Botulism. (
):
Duchenne myopathy
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Introduction
ATP
Muscle :
Skeletal muscle - Heart Heart - Brain cells cells ATP
glycolysis ATP .. ATP glycolys is mitochondria generator 3 muscle kidney creati nine creatine .. generator generator endoplasmic reticulum creatinine creatine
endoplasmic Muscle cells creatine phosphokinase cytoplasmic enzyme dystrophin carrier protein sacrolemmal membrane retic ulum
-
Duchenne & as
X X- chromosome
short arm - region Z -
band X X- linked recessive gene
it is x linked So , common in males Duchenne
3 New mutation, turner or ?????? C/P C/P
CPK abnormal dystrophin ... ATP creatinine creatine 3 degeneration Muscle fibers ATP skeletal muscle So , progressive course . fibers .. fibers motorr neuron lesion criteria criteria : lower moto
Weakness or paralysis Hypotonia & hypo reflexia What about muscle state????
( fibrous and fatty tissue ) degeneration muscle Muscle ( fibrous and and fatty tissue ) Muscle wasting
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fibro fibro--fatty -fatty tissue Pseudo -hype rtrophy ...
-
Excessive fibro -fatty tissue deposition
as delt deltoi oid, d, tric tricep epss and and glut gluteu euss Medi Medius us , culf culfff musc muscle less
.. quadriceps ..
-
some muscles never to be affected as upper fibers of trapezius, t rapezius, sternomastoid sternomastoid muscle, clavicular head of pectoralis major muscle, small muscle of the hand, sphincter of the bladder
theories Mitochondria
ATP ATP
- No autonomic or sensory affection. Distribution Bilateral and symmetrical + proximal > distal distal
ATP signs distal proximal Signs 1- +ve slipping sign :
shoulder
adductors positive slipping sign ... .... adductors
2- exaggerated lumbar lordosis :
..
anterior
..
back . ... ... extensors ... Lordosis ... lumbar vertebrae exaggerated lumbar lordosis
-
extensors
.... -
3- waddling gait :
... ...
... -
gluteal muscles muscles of the back .. ... ... gluteus muscle waddling gait 4- Winging of the scapula : ( medial border ) winging of the the scapula
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-
rhomboidus muscle
serratus anterior medial border ... medial serratus anterior scapula Medial b border order pushing border 55 - Pes cavus high high arched foot 5 fibrosis deformity 66 - Positive Gower sign 6 back muscles tone )) ( ☺
Complications Complications heart failure failur e e
.... heart myopathy brain cells -
Heart
So, may: MR 20 - 30 % of cases. - Epilepsy
( Irritate )
Causes of death - H.F. - UTI due to hypercalcemia hy percalcemia >>> 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
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-
- Physiotherapy + supportive treatment. - Recently myeloblast myeloblast transplantation. - Under trial gene gene therapy therapy.. UMNL 3 neurons 1- Motor area 4
And 2 accessory neurons 2- Basal0 0 ganglia 3- Cerebellum 1- Functions ss of motor area 4 11 Function 111 - Initiation of voluntary movement. LMN
Area 4
Weakness or paralysis ...
Distribution Distribution
Area 4 . limb Mono plegia . Hemi plegia . L. L . Cortical para para plegia L L .L . L. L : 2 limbs Quadri plegia 111 - May upper limb more than lower limb limb bi plegia 2Diplegia lower limb more than upper limb 2- Diplegia 3ll eft more than left double double hemiplegia hemiplegia 3- Right more than left Or left
43 – 42
Proximal or distal muscles
Upper motor neuron lesion shock stage st age
loss of all fine movement ... as small muscles of the hand & foot
crude movement
area 4 distal area 4 & 6 6 proximal 6 pr oximal 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
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Area 4 22- Suppression of muscle tone & deep tendons reflexes . anti gravity muscles
aa) ) a ) b) ) b b )
.. suppression pro gravity gravity anti gravity ) ( Pro gravity anatomical position ... ) ... Lower limb flexors ( .. extensions .. flexors Upper limb Extensors Pro gravity Flexors anti grav gravity ity Lower limb Extensors anti gravity Flexors pro gravity abductors adductors more suppression -
tone tone & deep deep tendon reflexes suppression Area 4 But anti gravity > pro gravity .. hemi plegia Extensors > flexors knee extension
flexion ☺
upper limb flexion (
)
shock stage abductors adductors add uctors tone Area 4 trunk lower limbs adductors special gait So , circumduction gait
scissoring gait par a plegia
initiation of movement
clasp knife spasticity
-
hyper tonia .. resi stance area 4
33- Suppression to pathological reflexes reflexes
reflexs Normally As Neonatal reflexes reflexes
33-4 -4
reflexes
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maturation Area 4 33-4 -4 suppression to pathological reflexes neonatal reflexes : 2&1 1- Moro reflex :
Area
.. Area 4 sub conscious level ( ) 33-4 -4 ( .. ) 4 . 2- Grasp reflex :
Area 4 ..
..( ) 4 -3 reflex
3- +ve babiniski sign :
scratching of lateral side of the sole of the foot positive Babiniski sign dorsi flexion ... planter planter flexion
4- Clonus :
Muscle sustained stretch Motor area 4 muscle spindle tendon reflex suppression ( ) continuous contraction 5- adductor reflex :
medial border of popliteal fossa lower limb adductors ... adductors suppression area 4 .. reflex normally reflex reflex adductor tendon
: Criteria of motor area 4 lesions 1- Loss of 11 st initiation of movement ) 11 function function ( initiation So, weakness or paralysis which: mono plegia, hemi plegia, para plegia p legia or quadri plegia( bi plegia, di plegia, double hemi plegia)… distal > proximal 22- Loss of suppression of the tone
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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. 33- 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- Cerebellum functions 3 parts 22-
physiologically
Archi cerebellum : anatomically
Motor area 4 Inner ear
lesion Effect of lesion
.. Mechanism archi cerebellum cerebellum drunken gait ..
2- Paleo cerebellum :
spinospino -cerebellar -cerebellar ventral lateral spinal cord .. muscle tone tracts lesion Effect of lesion Hypo tonia tone .. paleo cerebellum deep tendon reflexes Muscle tone stimulation cerebellum animals anatomically
tone ) tone hypotonia and hypo reflexia ... weakness or paralysis ... lower motor neuron lesion (
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3- Neo-cerebellum Neo-cerebellum : Functions & effects of lesion lesion In human
paleo area 4 neo -
... .. neo cerebellum ☺ ..
111 - estimation of distance :
area 4 ... estimation of distance eith er dysmeteria hypermetria hypermetria or hypometria or parametria 22- Smooth mov. : single movement
.. called decomposition of mov . area 4 dys -diadokokinesia supination and pronation antagonist muscle suppression cerebellum kinetic tremors tremors which is coarse - - nystagmus object fixation 3function ion ability to stop mov. At any level of it. 3- break funct ) ( no a bility to break rebound signs 44- equilibrium : deep sensation
postural paleocerebellum deep sensation Neopaleo -cerebellum -cerebellum Neo - cerebellum paleopaleo - cerebellum ��
deviation gait ( )
Neo ...
drunken gait
drunken gait drunken gait deviation gait zigzag gait ... Neo - cerebellum cerebella cerebellarr lesion
1- Hypo tonia, hypo hy po reflexia without muscle weakness 11 weakness or paralysis. 2dia--dokinesia 2- Dysmetria, Dysme tria, dys dia -dokinesia , kinetic tremors, nystagmus, rebound + gait disorder as drunken gait gait – deviation gait gait – zigzag gait gait Upper motor neuron 3- Basal ganglia 3 functions
111 - Emotions associated with movement.
associated movement
A - Swinging of the hands (
)
B – also, Emotion - associated mo movement vement vement as Facial expression
exaggerated emotions emotion s ( basal ganglia ) ) .... .... ( basal ganglia ) )
C – associated movements with speech :
.. associated movement
Effect of lesion associated movement
disturbed emotions basal ganglia chorea -
24.. 2- Suppression of basal activities of motor area 4 basal ganglia
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... area 4 basal ganglia .. ( ☺ ) Motor area 4 .. !!! basal ganglia
lesion Effect of lesion abnormal movement )
activities basal ganglia
.. area 4 ... chorea ( ) ...( cortex sleep slee p excitation
... abnormal movement asthetosis Twisting movement in extremities ....
twisting movement in the trunk dystonia
3tone 3- Effect of basal ganglia on tone basal ganglia
Mediators Acetyl choline stimulation. stimulation. Dopamine suppression suppression of tone. tone control basal ba sal ganglia or or lesion Effect of lesion
If lesion acetyl choline hypo tonia and hypo reflexia as chorea. If lesion dopamine hyper tonia and areflexia lead pipe rigidity
motor area 4 resistance lead pipe resistance cog wheel
cog wheel
tone areflexia reflex flexors Knee agonist and antagonist tone
- Rigidity more in flexors and adductors & shuffling gait short steppage 4tremors 4- Coarse static tremors
S
Cerebral palsy ( CP) Definition
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parkinsonism
Central motor neuron deficit deficit
central neuron
Motor area area 4, Cerebellum Cerebellum or Basal Basal ganglia (BG) brain growth Post natal
ante natal defect
natal
-
manifested early in neonatal period central motor neuron 1- Ante natal, natal or post natal 2- Manifested early Etiology
1- Ante natal natal 11 motor neurons
rash
Infection STORCH infections -1 fever drugs rugs or irradiation teratogen -2 d d Hypoxia intrauterine
-3
circulation placenta or mother mother pressure placental placental insufficiency insufficiency Toxiemia of pregnancy brain abnormal metaboli metabolites tes inborn error of metabolism tes by exclusion -
-4
congenital anomalies in fetus brain by exclusion 22- Natal
A – Delayed delivary delivary
( ) true uterine contraction .. placenta contraction ... 12 6 6 cerebral anoxia brain cells hypoxia hypoxia
cause hypoxic ischemic encephalopathy B - Birth injury intra cranial .. gift ii ntra gift forceps forceps
hemorrhage 3Post--natal natal 3- Post -natal
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6 6
-
-5
neonatal period A – infection : as meningitis or encephalitis ... infection B - Bilirubin encephalopathy neonatal jaundice
..
exchange transfusion bilirubin encephalopathy
C – May neonatal hypoxia hypoxia
..
pneumonia
D - Metabolic disorders disorders E - Intra cranial hemorrhage hemorrhage Clinical pictures CP
1- Spastic type most most common … 75 11 75 %
= Motor area 4 lesion 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 22- Ataxic type Hypo tonia cerebellum Dysmetria, dysdiadokinesia ….others .etc.
Abnormal gaits
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33- Extra pyramidal type CP basal ganglia
1- Emotional & associated movement disturbance 2- Abnormal movement ( chorea, asthetosis, dystonia …..etc) 3- tone
☺ 4type 4- Atonic type Unexplained Unexplained type .... lesion
exaggerated deep tendon reflex sever hypotonia sever MR 55 - Mixed type. 5 Criteria of CP
CP 111 - upper motor neuron lesion
22- normal sensation
3postnatal 3- Must be antenatal, natal or postnatal (
)
extra 4deafness( (especially especially extrapyramidal ), 4- other associated manifestations as MR, MR , deafness (( extra pyramidal type) type ) ), fits NB CP is Non fatal, Non curable curable & Non progressive disease Degrees Degree ss of CP Motor Class one no no motor dysfunction. motor dysfunction severity Class two, three and four (mild (mild , moderate and severe se vere ) . Investigations
mentality IQ .. ... audiometry .. ... ... " " .... ... ..
...... Treatment
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- 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 cerebellum ) NB
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 CSF inside the brain or inside and outside brain . So, Hydrocephalus is defined as CSF causes A – Non obstructive ob ob structive (communicating) Hydrocephalus: 111 - Excess secretion or production of CSF
... 200 CC absorption 200 CC 200 C C C absorption 300 CC 22- Decrease absorption. 200 B - Obstruction.
100 CC 200 CC
A A - Non obstructive ( communicating ) hydrocephalus hydrocephalus 1- excess secretion 11
choroid plexus 1- congestion choroids plexus:
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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 --as 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 cerebri 1- Hyper or hypo vitaminosis A. 2- Hyper vitaminosis D. 3thy roidism 3- Pseudo hypo para thyroidism End organ resistance .... receptors para thormone hormone 44- Expired tetracycline (out dated tetracycline). t etracycline). over dose
dose
55 - Adolescent obese o bese female. 5
CSF
3- May tumor as papilloma from choroid plexus 2absorption 2- absorption
SSS subsub - arachinoid superior sagital sinus absorption
-
1- may Anatomical defect . CSF absorption absorpt ion SSS
CSF sub arachinoid space
A – Achondroplasia shallow shallow post. Cranial fossa :
posterior cranial fossa cerebellum cerebellum posterior cranial fossa cerebellum shallow fossa curve sub arachinoid space space Post fossa fossa
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CSF achondroplasia large skull hydrocephalic hydrocephalic changes post fossa large skull B – Arnold chiari malformations (meningeocele or Meningo-myelocele) and Hydrocephalus ( )brain foramen magnum spinal spina l cord CSF Downward displacement of the brain cerebellum Brain foramen magnum brain sub arachinoid space CSF spinal canal CSF foramen of magendi ( .. foramen magnum .. S. S . C .C ) S C pressure CSF sub arachinoid space .... spinal canal vertebral body embryo intra uterine period ( ☺ ) Post arch .. body anterior post arch of neural tube SC spinal foramen the vertebrae vertebrae .. posterior arch pressure Spina pifida .. ( body ) vertebrae vertebrae herniation CSF pressure Cause Cause cystic called meningeocele meningeocele . c ystic swelling called Meningo -myelocele spinal cord ( SC ) spinal canal secretion CSF CSF 3rd 3rd ventricle Pressure 4th 4th ventricle back pressure hydrocephalus ☺ hydrocephalus Arnold Chiari malformation shallow Achondro plasia
2anatomical omical defect. 2- May not anat ( ) CSF
pathway sub arachinoid space
1- Sub arachinoid hemorrhage and hematoma 2- Sub arachinoid adhesion fibrosis meningitis
-
3- Abnormal metabolites as in muco-poly saccharidosis 3- Sinus thrombosis in SSS or o r in lateral sinus. SSS Pressure lateral sinus .. absorption lateral sinus thrombosis cochlear disease
cholesteatoma
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thrombosis Auditic hydrocephalus -
Non obstructive ( communicating )) hydrocephalus absorption absorption
secretion secretion
Patent
CSF
Pathway
B- Obstructive ( Non- communicating ) hydrocephalus :
obstruction 1- congenital stenosis or atresia at resia
a - Foramen of Monoro.
) Congenital stenosis or atresia ( b - Congenital at foramen fo ramen of magendi and lushka Congenital Dandy Walker malformation 4th Pressure magendi or Lushka 4th ventricle
Dandy dy Walker malformation Occipital area Dan 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 sp space oc occupying le lesion as as tu tumor, ab abscess, hemorrhage an and h heematoma
foramen
3- congeniatal infections STORCH toxoplasmosis
fibrosis
gliosis congenital infection -
Clinical picture A - Before closure of sutures & fontanels
1skull circumfere circumference nce 11 - skull nce 22- widely opened fontanel :
( ) 18 18 1.5 1.5 6 6 4.5 4.5 anterior fontanel . rickets delayed elayed closure called widely opened fontanel d d birth diameter diameter 5 5 anterior fontanel Enumerate causes causes and discuss t hem ?? ?? discuss how to diagnose one one of them
delayed closure widely opened
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H ydrocephalus, megalocephaly megalocephaly Hydrocephalus,
...
33- still opened post. F. . Posterior fontanel
4sutures:: 4- widely separated sutures widely separated sutures 55 - scalp: 5 dilated and visible scalp veins
Stretched skin
66 - craniotabes 6 .
(
)
77 7 - Direct percussion on skull bone dullness resonant
stony dullness Mac Ewen' Ewen 's '' s sign resonant
88 - trans illumination : 8
anterior fontanel ( ☺ ) torch ( ) opaque megalocephaly differential diagnosis trans illumination torch ..
99- Walker malformation 1010- Meningocele 11-- manifestations of increase ICT ICT : 11
ICT ICT manifestations ) So, So , late ICT anterior fontanel ( cry )
( 1212- Eye :
upper part of the cornea is covered by the eye lid !! lower part of corneal not covered by lid Sunset appearance
lower part of the cornea is Covered by lower lid -
... eye brow occipito occipito -frontalis -frontalis stretch skull ( fat ) Phospholipid brain optic nerve brain inferio medial surface globe optic nerve globe tentonrial supra cortex S. S .R R . nucleus squint S . R. R IR SR ( compression )
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B - After the closure of the th e 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 X X--ray 11 -ray .
hydrocephalus
22- CT or MRI
obstruction
dilated foramen of Monoro .. lateral ventricle ..
33- Positron emission topography ( PET ) .
.. MRI anatomy
44- Spectroscopic PET
functions
anatomy
55 5 - Ventricular pressure monitoring ( CSF ) Progressive or stationary. 66 - Serology of STORCH. 6 Treatment
Just medical treatment mild or moderate 1- Mediacal ttt : 11 3 lines 1- Is there is a cause treatment of the cause 2secretion of CSF 2- secretion CSF
salt restriction - Carbonic anhydrase inhibitor ( Diamox )
secretion choroid plexus Give cortisone in congestion --Give 3absorption of CSF 3- absorption
osmotic pressure blood vessel
Give mannitol osmotic pressure in SSS CSF absorption 2- Surgical ttt :
neurosurgery
1- Surgical removal of the cause
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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 ventriculo-peritoneal shunt ... peritonium shunt d- to cisterna magna : cisterna magna ... Ventriculo-cisternal Ventriculo-cisternal shunt 4- Obstruction Obstruction of foramen of Monoro >>> third ventriclostomy ventriclostomy Complications ss of the shunt shunt Complication 1- Obstruction of the shunt.
( ) shunt
2- Infection meningeo-encephalitis meningeo-encephalitis
3- Brain dysfunction
4- Thrombus , arrhythmia ( on heart ) , Focal GN ( peritoneal sac ) 5- Acute shunt failure
shunt
45 – 44
1- Macrocranium 95 percentile
skull circumference 35 42 6 46 12 49 3 0.5 cm every year 7 3 0.25 cm every year 12 7
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+ 2SD skull circumference
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 :
( brain (
Inner ) ) Inner cortex outer cortex
.. medulla 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 hy drocephalus .
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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 2
skull circumference circumference ) 40 ( 46 micro cranium 3rd percentile -2 SD causes 1- microcephaly:
size of the brain brain
Micro cephaly ( cereberal cereber al palsy ) CP (
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(
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 -Small skull . No manifestations of ICP . --No Early, fontanel is open . --Early, CP
2- early closure of sutures: cranio- sentosis
cranio-stenosis one or more
sutures cranio stenosis
pre mature closure of one suture cranio sentosis
line of growth sutures
brain growth
skull bone sutures 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 posterior plagio cephaly
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coronal lambdoid
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 manifestation of ICT
.. brain sutures .. high pitched cry convulsion vomiting + Investigations ons of craniostenosis Investigati
Skull X-ray abnormal shape + silver beaten appearance . Treatment of craniostenosis
sutures
open Craniotomy
.
Cranioectomy Mental Retardation
.
Definition lligence . Impairment of intelligence inte
.. memory -11
..
mode of thinking -2
( ) MR adaptive social behavior -3
-4 Etiology
( cerebral palsy ) CP
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1- Enviromental causes most ommon cause of MR ( )
Socially dissociated curable
2- Post immunization as DPT ( P P ) encephalopathy encephalopathy degree .. MR
6 6
3- Chromosomal CP
MR MR ???? How to prevent CP 1- Good antenatal care .
Drugs . .
Irradiation . . ultra sound
STORCH infection .
Previous history of chromosomal abnormality chorionic villus biopsy early abortion skin rash
fever
2- Natal causes prevent causes of obstructive labor . 3- Post natal care of baby as early detection of hypothyroidism & other o ther in born errors . Investigations 1- To reach the etiology 2- IQ IQ 70 IQ
MR : 1- mild
Mild
50-70 IQ
educable
( )
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3-4 IQ . 2- moderate Imbecile moderate
35- 50 IQ
.. Just trainable
3- severe
idiot sever ..
20 - 35 IQ
4- profound
profound
Just MR
..
0- 20 IQ
ttt As CP but no need to physiotherapy . Floppy baby
3 ☺ written .. severe generalized hypotonia 3
floppy
floppy
floppy
.. examiner called head lag supine
.. 3
1- Head lag drop of the head backwards due to hypotonia of the neck muscles 2- curved nec k k 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 mongolism ) . CP atonic ataxic . Other syndromes syndromes : As Lowe's Lowe's syndrome or Pradder 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 ICT 3
1. Cranio stenosis . 2. Pseudo tumor cerebri . 3. Space occupying lesion .
1.
Hypertensive encephalopathy .
2.
CNS infection .
46 – 45
Transverse myelitis - Mostly auto immune disease DPT vaccine
.. viral infection
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inflammation of part of spinal cord mostly cervical or thoracic So , acute auto immune 1.
At level of the lesions destructed AHCs LMNL
2.
.. segments Level
Below level of the lesion . A- Motor
6 2 .. acute attack criteria
So, UMNL to the AHCs below as pyramidal tract
Hyper tonia ( clasp knife ) Exaggerated deep tendon reflexes Muscle state ( normal ) Clonus , Babiniski Weakness or paralysis
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 Local reflex spinal cord automatic bladder ( 5 ) baby So, disorder in sphincteric control investigations
TM CT or MRI spinal cord 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 . --It So, just symptomatic treatment . --So, - All immunotherapy under trial . Seizures
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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
encephalitis meningitis brain abscess 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 )
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g) Trauma h) Encephalopathies
i)
Renal Hepatic 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
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.. 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 . Febrile convulsion
Idiopathic causes
criteria
febrile convulsion
febrile convulsions over diagnosis
Criteria of febrile convulsion 1- Age 3 months to 5 years .
6 years 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
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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
febrile convulsion attack EEG
epilepsy
N.B. In febrile convulsion EEG is normal 2-3 weeks after the attak 1- Positive family history of epilepsy 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 generalizedgeneralized-
-
Treatment of febrile convulsion 1. 2. 3. 4.
fever . Treatment of the cause . May short term anti convulsants . Avoid fever later . Investigations of seziures
1. EEG .
attack Not a good positive and not a good negativeclinical
type 2. may need lumbar puncture p uncture CNS infection 3. Ca & Mg level . 4. Glucose level . 5. May need fundus examination
ICT papilloedema
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6. MRI or CT. : history Indications
History of head trauma t rauma 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 ( 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 15/kg 15 -20 -20 mg / /kg / once loading loadin g dose 1/3 35 mg / /kg 3- 5 /kg /day // day Maintenence dose 12 General anasethsia neuro enocrine 46 26
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