UPPER MOTOR NEURON HEMIPLEGIA-PARAPLEGIA - PowerPoint Presentation

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UPPER

MOTOR NEURON HEMIPLEGIA-PARAPLEGIA

DR NASIR FAROOQ BUTTASSISTANT PROFESSORDEPARTMENT OF MEDICINE KING EDWARD MEDICAL UNIVERSITYMAYO HOSPITAL LAHORE

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UPPER MOTOR NEURONS

(

UMN) start from cerebral cortex, passes through internal capsule,

corticospinal

tracts, and ends at cranial nerve nuclei in brain stem or anterior horn cells of spinal cord at different levels

. The axons of upper motor neurons are contained within the pyramidal system, which is composed of the

corticospinal

(pyramidal) and

corticobulbar

tracts.

LOWER MOTOR NEURONS

(LMN) start from nuclei of cranial nerves or anterior horn cells of spinal cord, extend through cranial /spinal nerves and ends at motor end plate of concerned muscle.

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.

.

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UPPER MOTOR

NEURONS LESION (UMNL)

.

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Pyramidal Drift Of An Upper Limb

Normally

, the outstretched upper limbs are held symmetrically, when the eyes are closed. With a pyramidal lesion, when both upper limbs are held outstretched, palms uppermost, the affected limb drifts downwards and medially. The

forearm tends to pronate and the fingers flex

slightly. This sign is often first to emerge, sometimes before weakness and/or reflex changes become apparent.

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Pyramidal Drift Of An Upper Limb

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Weakness And Loss Of Skilled Movement

A unilateral pyramidal lesion

above the

decussation

in the medulla causes

weakness of the opposite limbs

.

When

acute and complete

, this weakness will be immediate and total, a hemiplegia, e.g. following an

internal

capsule infarct

.

With

slowly progressive

lesions (e.g. a hemisphere

glioma

) a characteristic pattern of weakness emerges – a hemiparesis.

There is also

loss of skilled movement

– fine finger and toe control

diminishes

.

Slide8

Changes In Tone And Tendon Reflexes

An

acute

lesion of one pyramidal tract (e.g. internal capsule stroke) causes

initially

FLACCID

paralysis with loss

of

tendon reflexes.

Increase in tone follows, usually within several days due to loss of inhibitory effects of the

corticospinal

pathways and an increase in spinal reflex

activity.

SPASTICITY

is

characterized by sudden changing resistance to passive movement – the clasp-knife effect.

Relevant

TENDON REFLEXES become exaggerated

;

CLONUS

may

emerge.

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Changes In Superficial Reflexes

Abdominal (and cremasteric) reflexes are abolished on the side affected.The Babinski sign.

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Patterns of UMN disorders

There are three main patterns

:

HEMIPARESIS

means weakness of the limbs on one side; it is usually caused by a lesion in the brain and occasionally in the cord.

PARAPARESIS

means weakness of both lower limbs and usually diagnostic of a cord lesion; bilateral brain lesions occasionally cause

paraparesis

.

TETRAPARESIS

(syn.

quadriparesis

) means weakness of four limbs.

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Extrapyramidal system

The extrapyramidal system is a general term for

BASAL GANGLIA

motor

systems, i.e. corpus striatum (caudate nucleus +

globus

pallidus

+ putamen),

subthalamic

nucleus,

substantia

nigra

and parts of the thalamus. In basal ganglia/ extrapyramidal disorders, two features (either or both) become apparent, in limbs and axial muscles

:

Reduction in speed (

bradykinesia

, meaning slow movement) or

akinesia

(no movement), with muscle

rigidity

Involuntary

movements

(e.g. tremor, chorea,

hemiballismus

,

athetosis

, dystonia).

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Extrapyramidal system

Extrapyramidal

disorders are classified broadly

into:

Akinetic

-rigid

S

yndromes

where

poverty of movement

predominates

D

yskinesias

where there are involuntary

movements.

The

most common extrapyramidal disorder is

Parkinson’s disease.

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Case Scenario

An

83-year-old right-handed woman was eating dinner when she suddenly slumped to the right and her speech became slurred and

nonfluent

. Her vascular risk factors were hypertension and dyslipidemia, and the only other past medical history of interest was long-term hypothyroidism and a curative mastectomy for breast cancer. The patient was on two antihypertensive medications and a statin, and was not receiving any antiplatelet medication. Her husband immediately called the emergency medical services.

Slide16

Case Scenario

On Examination Pulse is 84/min (regular), BP: 180/105 mm Hg.

Angle of mouth is deviated towards left (UMN type Right 7

th

CN)

Tone is decreased in right upper and lower limb,

Power is 2/5 in right

right

upper and lower

limb,

Reflexex

are absent in right

right

upper and lower

limb.

Right Planter is

upgoing

.

Tone, power, reflexes are normal on left side with

downgoing

planter

What is your Diagnosis????

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STROKE

This patient has had STROKE causing Right sided uncrossed Hemiplegia with UMN type Facial Nerve Palsy

which can be caused by a vascular event such as thrombosis, embolism or hemorrhage or by

neaplasm

of brain (

aetiology

). The patient is limited by hemiplegia (functional status)

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Stroke

DEFINITION

: Stroke

is

defined

as a syndrome of

rapid onset

of cerebral deficit (usually focal) lasting >24

h or

leading to death, with no cause apparent

other than

a vascular

one.

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Stroke

Ischaemic

stroke/infarction (80%)

Thrombotic

Cardio-embolic

Large

artery stenosis

Small

vessel disease

Hypoperfusion

Haemorrhagic

stroke

(17

%)

Intracerebral

hemorrhage

(12

%)

Subarachnoid hemorrhage

(5

%)

Other

(3%),

e.g. arterial dissection, venous

sinus thrombosis

,

vasculitis

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Types of Stroke

Depending upon the rate of developing and reversibility, stroke can be of following types:

Transient Ischemic Attack (TIA):

means a brief episode of neurological dysfunction due to temporary focal cerebral or retinal

ischemia

without infarction, e.g. a weak limb, aphasia or loss of vision, usually lasting seconds or minutes with complete recovery

.

Stroke-in-Evolution

:

If neurological deficit increases by step wise progression or there is slow uninterrupted progression, it is called stroke-in-evolution. It may take 1-2 weeks to complete

Complete Stroke

: It means complete hemiplegia

RIND (Reversible

Ischemic Neurological

Deficit):

It is a term that is not widely used anymore.

It

means a stroke that the patient was able to fully recover from within a few weeks.

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Types of Stroke

Depending upon the blood supply of affected area of brain, stroke can be of following types:

ANTERIOR CIRCULATION STROKE:Carotid systemPOSTERIOR CIRCULATION STROKE:Vertebrobasilar system

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Risk Factor For Ischemic Stroke

Hypertension

Diabetes

Smoking

Lifestyle

Alcohol

High cholesterol

Atrial fibrillation

Obesity

Severe carotid

stenosis

Sleep

apnoea

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Rarer Causes Of Stroke

Hyperviscosity

states.

Thrombocythaemia

,

Polycythaemia

Thrombophilia

Anti-

cardiolipin

and lupus anticoagulant antibodies

oral contraceptive.

Migraine

Vasculitis

Systemic lupus

erythematosus

(SLE),

Polyarteritis

Giant

cell arteritis

Granulomatous

CNS

angiitis

:

Slide28

Rarer Causes Of Stroke

Amyloidosis

.

Hyperhomocysteinaemia

Neurosyphilis

Mitochondrial

disease

Drugs

Sympathomimetic drugs

cocaine

Vasoconstrictors

neuroleptics

CADASIL (cerebral dominant

arteriopathy

with subcortical

infarcts and

leucoencephalopathy

)

Slide29

Clinical Features of Stroke

SYMPTOMS:

Weakness of one half of body

Onset – Sudden, can be gradual

Headache, vomiting, fits

Unconciousness

Past H/O TIAs

H/O

smooking

, HTN, DM or IHD

Family H/O Stroke

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Clinical Features of Stroke

SIGNS:

PULSE

: Irregular (At. Fib), Slow (Raised ICP)

BP

: High

Unconciouness

Pupils

: Small or Dilated

EYES:

Devited

towards on side (conjugate gaze)

Cranial Nerves

may be involved

Dysphasia, Dysarthria

Initially limbs may be

flaccid

with absent reflexes (Spinal Shock). This may persist for days, weeks or months and then Classical UMNL signs develop

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Clinical Features of Stroke

SIGNS:

Hemiplegic

weakness

of

upper

limb

affects

the

shoulder

abductor,

elbow

extensors

,

wrist

and

finger

extensors

and

small

musles

of

hand

Hemiplegic

weakness

of

lower

limb

affects

hip

flexors

,

knee

flexors

and

dorsiflexors

and

evertors

of

the

foot

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Clinical Features of Stroke

SIGNS:

Signs

of

meningial

irritation

(SOMI) –

Subarachnoid

Hemorrhages

Fundoscopy

:

Diabetic

or

Hypertensive

Retinopathy

Carotid

Pulsation

:

weak

,

bruit

CVS

Exam

:

Murmur

eg

Mitral

stenosis

Homonymous

hemianopia

and

sensory

inattention

Speech

defects

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Amaurosis

fugax

R

efers

to a transient loss of vision in one or both eyes

due to embolism in retinal artery.

This is a feature of TIA in carotid circulation

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ISCHEMIC STROKE

Cerebral Thrombosis:

Occurs when patient is asleep

Atherosclerotic narrowing of blood vessel

Middle cerebral art is commonly involved

Patient usually remain conscious

HTN is a major risk factor

Clinical Features:

Pt

can not move one half of body

Dysphasia

Uncrossed hemiplegia

High BP

Carotid Bruit

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ISCHEMIC STROKE

Cerebral Embolism:

Sudden onset

Past history of TIAs

Source of Embolism

Carotid artery atheroma (weak carotid

pulse,bruit

)

Atrial Fibrillation or other

arrythmias

Recent Myocardial Infarction

Valvular

Heart Disease

Infective Endocarditis

Clinical Features:

Sudden loss of power in one half of body

Conciouness

may be lost

Dysphasia

Uncrossed hemiplegia

Irregular Pulse

Signs of

valvular

heart disease

Carotid Bruit

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HEMORRHAGIC STROKE

Primary

Intracerebral

Hemorrhage:

H/O HTN

Internal capsule is most common site. Other sites: subcortical white matter, pons, and cerebellum.

O

ccurs when

pt

is

engaged in some activity

Causes: HTN

,

Saccular

Aneurysm,

AV

Malformation, Bleeding Disorders

Clinical Features:

Sudden loss of power in one half of body

Headache, vomiting and loss of

Conciouness

Uncrossed hemiplegia

Fundoscopy

: Papilledema

Slide40

HEMORRHAGIC STROKE

Subarachnoid Hemorrhage:

Primary:

Bleeding occurs directly into subarachnoid space.

Causes

:

Saccular

Aneurysm, AV Malformation, Bleeding

Disorders,

Vasculitis

Clinical Features:

Sudden onset of severe Headache with or without vomiting

SOMI are present

Focal neurological signs

Fundoscopy

:

Subhyloid

hemorrhage, Papilledema

Secondary

:

Extension

of

intracerebral

hemorrhage

into

subarachnoid

space

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SPACE OCCUPYING LESION

Lesion which occupy the cranial cavity and displace or

destroy existing structures

Causes

:

Tumour

, Abscess, Hematoma,

Tuberculomas

Symptoms:

H/O gradual onset of weakness developing over weeks to months.

Headache and vomiting

There may be H/O trauma (Hematoma)

H/O waxing and waning of

concious

level (Subdural Hematoma)

H/O fever and ear discharge suggest an abscess.

Signs:

In addition to motor deficit, following signs may be present:

Bradycardia

Fundoscopy

……Papilledema

Investigations:

CT scan (with contrast), MRI

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Localization of Lesion

UNCROSSED HEMIPLEGIA

:

Cranial nerves involvement and hemiplegia are on the same side

Lesion is above brainstem on the side opposite to the hemiplegia

CROSSED

HEMIPLEGIA

:

Cranial nerves

are involved on one side

and hemiplegia

is

on the

other

side

Lesion is

in

brainstem on the side

of cranial nerve involvement.

HEMIPLEGIA WITHOUT CRANIAL NERVES INVOLVEMENT:

Lesion is in spinal cord above C5 on the side of paralysis.

Slide43

Localization of Lesion

UNCROSSED HEMIPLEGIA

:

Lesion can be in Motor cortex, Corona

Radiata

or Internal Capsule

Motor cortex:

Specific manifestation due to lesion of various lobes:

Parietal Lobe

Temporal Lobe

Occipital Lobe

Frontal Lobe

Slide44

Localization of Lesion

PARIETAL LOBE

:

Sensory cortex (

Precentral

Gyrus

)

demage

only affects special sensory functions

Loss of cortical sensations:

Two point discrimination

Astereogonosis

(inability to identify common objects by palpation with one hand while senses are intact)

Homonymous lower

quadrantanopia

Apraxia

(inability to perform a learned act in the absence of significant motor weakness.

Additional Features of Dominant Hemisphere

Gerstmann’s

synd

: Inability to calculate, write, distinguish between right and left, to recognize different fingers.

Alexia (inability to read), Bimanual

astereognosis

,

Sensory dysphasia

Slide45

Localization of Lesion

PARIETAL LOBE

:

Additional

Features

of Non-

Dominant

Hemisphere

Sensory inattention (when both side are touched separately patient can recognize them, but when they are touched simultaneously, side opposite to affected parietal lobe is ignored)

Indifference to illness

Anosognosia

(

pt

is unaware of paralysis)

Autopagnosia

(

pt

ignores paralyzed body)

Spatial disorientation (

Pt

is unable to find his way around)

Slide46

Localization of Lesion

TEMPORAL LOBE

:

Auditory or Olfactory hallucination

Auditory or Visual hallucination

Homonymous upper

quadrantanopia

of opposite side

Deja

vu phenomenon (feeling of undue familiarity with surroundings)

Automatism (lip smacking, swallowing, walking aimlessly or unconscious performance of highly skilled activity like driving a car)

OCCIPITAL LOBE

:

Visual

hallucination

Homonymous hemianopia of opposite side

Slide47

Localization of Lesion

PRE-FRONTAL LOBE :

Lack of initiative and concentration

Lack of concern of consequences of any action

Micturition

disturbance

Grasp Reflex is +

ve

PRECENTRAL GYRUS (FRONTAL LOBE):

Weakness of

of

opposite half of the body

Jacksonian

fits

Expressive, motor or non-fluent dysphasia if lesion is in the dominant hemisphere.

Slide48

Localization of Lesion

UNCROSSED HEMIPLEGIA

:

CORONA RADIATA:

Although Weakness is more marked in one limb, whole half is involved to some extent

INTERNAL CAPSULE:

Motor and sensory

fibere

are very closely packed…so lesion cause

Dense complete hemiplegia

Facial nerve palsy of same side

Cortical sensory loss and hemianopia may occur

Slide49

Localization of Lesion

CROSSED HEMIPLEGIA

:

Midbrain:

3

rd

/4

th

CN are involved on one side and hemiplegia of opposite side

Pons:

5

th

, 6

th

, 7

th

and 8

th

CN nuclei are present in pons.

One or more of these CNs are paralyzed on one side and hemiplegia is on the other side.

Pupils are pinpoint but reactive to light

Hyperpyrexia may be present

Medulla Oblongata:

9

th

,

10

th

,

11

th

and

12

th

CN nuclei are present in

medulla.

One or more of these CNs are paralyzed on one side and hemiplegia is on the other side.

Slide50

Localization of Lesion

SPINAL CORD.

Brown

-

Sequard

syndrome

(BSS)

is a rare neurological condition characterized by a lesion in the spinal cord which results in weakness or paralysis (

hemiparaplegia

) on one side of the body and a loss of sensation (

hemianesthesia

) on the opposite

side.

Causes:

Trauma

Non-traumatic: Tumor

(primary or

metastatic), Multiple sclerosis, Disk herniation,

Epidural

hematoma, Vertebral

artery

dissection, Transverse myelitis,

etc

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MIDDLE CEREBRAL ARTERY

Supplies lateral surface of

frontal,frontal

,

perital

and temporal lobes

Most of the motor and sensory cortex

Ecxept

the part on medial side and control lower limb, internal capsule

Total Occlusion

:

Uncrossed hemiplegia

Hemianesthesia

Global Dysphasia

Homonymous hemianopia

Apraxia,

Agnosia

Partial occlusion

:

Any of the above features.

Lower limb is either not or minimally involved

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ANTERIOR CEREBRAL ARTERY

Supplies MEDIAL surface of hemisphere

M

otor and sensory cortex on medial side which control lower limb

Soon its origin it gives a penetrating branch (

Heubner’s

artery) which supply internal capsule (containing fibers of upper limb and face)

Occlusion at the origin

:

Features are similar to total occlusion of MCB

Occlusion of

Heubner’s

artery

:

Weakness of upper limb and face

Occlusion

after the origin of

Heubner’s

artery

:

Weakness of

lower limb

Loss of cortical sensory function in lower limb

Slide55

POSTERIOR

CEREBRAL ARTERY

Branch of Basilar artery

Supplies occipital lobe which include visual cortex

Occlusion

:

Visual field loss

Homonymous

hemianopia

Visual

agnosia

Disorders

of reading

Disorders of color vision

Memory impairment

Motor dysfunction

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LATERAL MEDULLARY SYNDROME

Also called posterior

inferior cerebellar artery (PICA) thrombosis and

Wallenberg’s syndrome

)

A

common

example of brainstem infarction presenting as acute vertigo with cerebellar

and other

signs. It follows

T

hromboembolism

in the PICA or its branches, vertebral artery thromboembolism or dissection.

Features

depend on the precise structures damaged.

MEDIAL MEDULLARY SYNDROME

C

haracterized

by a set of clinical features resulting from occlusion of the anterior spinal artery. This results in the infarction of medial part of the medulla oblongata.

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Investigations - STROKE

The purpose of investigations in stroke is:

To

confirm the clinical diagnosis

and distinguish between

hemorrhagic

and thromboembolic infarction;

To look for

underlying causes

and to direct therapy;

To

exclude other causes

, e.g.

tumour

.

Sources of embolus should be sought

Slide62

Investigations of Stroke

Brain

Computed

Tomography (CT) Scan

Magnetic resonance imaging (MRI

)

Computed Tomography Arteriogram and Magnetic Resonance

Arteriogram:

Aneurysm, AV

malformation

Carotid

Ultrasound

ECG and

Echocardiography

Lumber Puncture:

Subarachnoid

Hemorrhage

Slide63

Investigations - STROKE

Routine bloods (for ESR,

polycythaemia

,

infection,

vasculitis

, thrombophilia, syphilitic serology, clotting studies, autoantibodies)

Blood Sugar Level

Fasting

Lipid profile

Chest X-ray

ECG and Echocardiography

Carotid Doppler studies

Lumber

Puncture:

Subarachnoid

Hemorrhage

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Investigations - STROKE

Imaging in Acute Stroke.

Non-contrast CT:

will

demonstrate

haemorrhage

immediately but cerebral infarction is often not detected or only subtle changes are seen initially.

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CASE SCENARIO

A 40 year old male presented in OPD with C/O gradually progressive weakness of both legs for the last one month. He is also C/O backache. There is no H/O Trauma.

There is H/O Numbness

and

paresthesias

in

legs. He is unable to control his urine.

Slide72

CASE SCENARIO

On Examination Pulse is 84/min (regular), BP: 180/105 mm Hg.

Bulk is normal in both legs

Tone

is

increased

in

both legs,

Power is

3/5

in right

leg

and

2/5 in left leg.

Knee and Ankle

Reflexex

are

Brisk

bilaterally

.

B

oth Planters are

upgoing

. Ankle Clonus is present on both sides.

Upper limbs are normal.

Tenderness at back of chest.

What is your Diagnosis????

Slide73

This patient has SPASTIC PARAPLEGIA (Lesion) at T10 spinal level due to Cord compression (

aetiology

). It is complicated by bladder involvement (functional status)

Slide74

SPASTIC PARAPLEGIA

HISTORY:

Ask about onset, duration and course of symptoms

Back pain: whether localized

Ask about radicular pain

Numbness and

parasthesia

particularly below the level of lesion

Weakness: whether gradual or sudden

Sphincter control and bladder sensation

Functional status: wheelchair transfers, walking aids, orthotic shoes and whether house has been modified for the patient’s disability

Take a family history (hereditary spastic paraplegia)

Take a history of birth anoxia (cerebral palsy)

History of urinary infections, pressure sores and deep venous

thromboses

.

Slide75

SPASTIC PARAPLEGIA

EXAMINATION:

Increased tone in both lower

limbs,

Hyperreflexia

, Ankle

clonus

Weakness in both lower

limbs

Then

proceed to:

Check

the sensory level

Examine

the spine (spinal tenderness or deformity

)

Tell the examiner that you would like to do the

following:

Check sacral sensation

Examine

the hands to rule out involvement of upper limbs

Check

for cerebellar signs (multiple sclerosis,

Friedreich’s

ataxia).

Slide76

SPASTIC PARAPLEGIA

EXAMINATION:

Try to

localize the level of lesion

using the following

:

UMNL signs in

lower

limb and LMNL signs in upper

lims

:

lesion of

C5–T1.

UMNL signs in lower limb and

Wasting of small muscles of hand:

lesion of

C8–T1

).

Spasticity of the lower limb alone:

lesion of thoracic cord (T2–L1).

Flaccid

Paraplegia:

Lesion

below L1

Slide77

SPASTIC PARAPLEGIA

Paraplegia means UMN type weakness of both lower limbs.

CAUSES:

Spinal Cord Compression

Transverse Mellitus

Subacute

combined

degenration

of spinal cord

Friedreich’s

Ataxia

Amyotrophic Lateral Sclerosis (MND)

Spinal cord infarction

Hereditary

Spaatic

parapresis

Tabes

dorsalis

(

Neurosyphilis

)

Syringomyelia

Intracranial Lesions

Parasagital

Meningioma

Thrombosis of Sagittal Sinus

Slide78

SPINAL CORD COMPRESSION

CAUSES

A. EXTRADURAL:

Vertebral Traumatic Fracture

Myeloma

Tuberculosis of Spine

Metastases (breast, prostate, lungs)

Traumatic and degenerative lesions of disc

B. EXTRAMEDULLARY

:

Meningioma

AV Malformation

C. INTRAMEDULLARY

:

Spinal Cord Tumors

Slide79

SPINAL CORD COMPRESSION

Symtoms

:

H/O Backache,

agg

revated

by

coughing, sneezing and straining

Pain in the distribution of nerve root

H/O Trauma

Weakness is usually gradually progressive except in trauma when it is of sudden onset.

Numbness and

paresthesia

in lower limbs

Micturation

-urgency or

hesistancy

Signs:

Local examination of the vertebral column may reveal deformity or tenderness

Sensory loss with definite upper limit

Slide80

SPINAL CORD COMPRESSION

Investigations:

X- Rays of vertebral column may show reduce disc space, bony

errosions

,

osteolytic

or

osteosclerotic

lesions, vertebral collapse or fracture.

MRI

Routine Investigations

Slide81

TRANVERSE MYELITIS

Inflamation

of spinal cord

Can present with paraplegia or quadriplegia

Respiratory paralysis can

occur

Causes:

Unknown 60 % (

viral

infections or

post-infectious??)

Remaining 40 % associated

with autoimmune disorders such as multiple sclerosis,

neuromyelitis

optica

, systemic lupus erythematous,

Sjogren’s

syndrome

and

sarcoidosis

Slide82

TRANVERSE MYELITIS

4 classical

Symptoms:

Backache/discomfort is first symptoms

Rapidly progressive weakness of lower limbs

Sensory symptoms such as numbness or tingling

Sphincter disturbances (Bladder/bowel) are

common

Signs

:

UMN signs in lower limbs

Sensory

Level

Investigations

:

Lumber Puncture with CSF Exam Lymphocytosis

X-Ray and MRI to rule out Cord compression

Slide83

Subacute

Combined Degeneration Of Spinal Cord

Due to

Vit

B12 Deficiency

Degeneration of Posterior columns and pyramidal tract

Peripheral Neuropathy

Symptoms:

Symptoms of anemia

Gradual progressive weakness of legs

Numbness, tingling and

hyperesthesias

Signs:

Pallor

Paraplegia, Ankle Jerk Absent, Up going planters.

Sense of position and vibration are lost

Slide84

Subacute

Combined Degeneration Of Spinal Cord

Investigations:

Peripheral Blood Film:

Macrocytosis

(MCV is raised),

Hypersegmented

Neutrophils

Pancytopenia

Mild unconjugated

hyperbilirubinemia

(du to ineffective

erythropoisis

.

Serum B12 is low. Elevated LDH,

Bonemarrow

biopsy:

Megaloblasts

Schilling Test

Causes of

Vit

B12 Deficiency:

Nutritional

def

,, Pernicious anemia, Bacterial overgrowth and blind loop syndrome, Diverticulosis,

Diphyllobothrium

latum

infestation, diseases of terminal ileum (

Crohn’s

disease,

ileocecal

TB)

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Friedreich’s

Ataxia

Familial Autosomal Recessive

Slowly progressive degeneration of

P

osterior

columns,

P

yramidal

tracts,

S

pinocerebellar

tracts and

P

eripheral

nerves

Age of onset: 8 – 16 years

Slowly progressive weakness of legs and unsteadiness on walking

UMN type weakness of legs

Absent Ankle jerk

Planters

up going

Sense of position and vibration are lost

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Friedreich’s

Ataxia

Sensory Ataxia, Romberg’s sign may be +

ve

Cerebellar Signs (

nystagmus

, scanning speech, intension tremors) may be present

Associated features:

Pes

cavus

(high arched foot)

Kyphoscoliosis

Spina

Bifida

Hypertrophic Obstructive

Cardiomuopathy

(HOCM)

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Pes

Cavus

Causes:Hereditary Motor and Sensory Neuropathy Type 1 (Charcot-Marie-Tooth disease) Friedreich's Ataxia

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Causes Of Absent

Ankel

Jerk And Up Going Planters

:

Friedreich's

ataxia

Subacute

combined degeneration of the spinal cord

Motor

neurone

disease

Syphilis

(

taboparesis

)

Combined

conus

medullaris

and

cauda

equina

lesions

Combination

of common problems e.g. diabetic peripheral neuropathy and cervical

spondylosis

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CASE SCENARIO

A

65

year old

male

presented in OPD with complains of

slowly

progressive weakness of legs

with numbness

, tingling and

hyperesthesias

. He is a strict vegetarian. On

examination

he has

spastic

parapresis

with absent ankle jerk and

upgoing

planters. Sense of position and vibration are

lost.

His hemoglobin

is 6

gm

/dl.

What

is most likely diagnosis?

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CASE SCENARIO

Answer:

Subacute

Combined Degeneration Of Spinal Cord

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