PARAPLEGIA, QUADRIPLEGIA

AND MOTOR NEURON DISEASE

 OVERALL OUTLINE
(not following the slide order)
1. Anatomy:
 motor n sensory pathway
 spinal cord
 spinal column

2. Paraparesis
 Spastic
- spinal cord compression
- multiple sclerosis
- motor neuron disease + transverse myelitis
- subacute combined degeneration of the cord
- Syringomyelia
 Flaccid

3. Quadriparesis
APPROACH TO PATIENT WITH
PARAPARESIS AND
QUADRIPARESIS
(SPINAL CORD DISORDERS)
SITI NURFARHANIE BINTI SAMSUDIN
012012100203
OUTLINE
• ANATOMY OF SPINAL CORD
• ASCENDING PATHWAY
- Spinothalamic Tract
- Posterior Column Tract

• DESCENDING PATHWAY
- Corticospinal Tract
• DEFINITION OF PARAPLEGIA AND QUADRIPLEGIA
• FLACCID PARAPLEGIA
ANATOMY

NERVOUS
SYSTEM

Somatosensor Motor
y system system
(ascending (descendi
tract) ng tract)
SOMATOSENSORY SYSTEM

Consist of 3 major sensory tracts:-

Spinocerebellar tract:-

Posterior columns - Proprioception about

tract:-
Propioception, fine
touch, pressure and
vibration Spinothalamic tract:-
the position of skeletal
muscle, tendons and
joint sent to cerebellum

- Anterior spinothalamic for

crude touch and pressure
sensation

- Lateral spinothalamic for pain

and temperature
All those tracts
involve chains of
neurons:

•First order neuron

– Cell body in the
dorsal or cranial
root ganglion
•Second order neuron
– An interneuron
with the cell body
in the spinal cord
•Third order neuron
– Transmit
information from
the thalamus to the
cerebral cortex
Nucleus
gracilis:
T6 and below

Nucleus
cuneatus: from
levels at T6
and above
Descending Tract (Motor System)
• Corticospinal • Subconscious
tract tract
- Corticobulbar tract - Vestibulospinal tract
- Lateral Corticospinal - Tectospinal tract
tract - Rubrospinal tract
- Anterior - Reticulospinal tract
Corticospinal tract

Fx --> Subconscious
Fx --> Conscious regulation of balance,
control of skeletal muscle tone, eye, hand,
muscles and upper limb
position
 SUBCONSCIOUS TRACTS

• Vestibulospinal tract
•Send information from the inner ear to
monitor position of the head

• Tectospinal tract
• Send information to the head, neck, and
upper limbs in response to bright and
sudden movements and loud noises

• Reticulospinal tracts
•Send information to cause eye movements
and activate respiratory muscles

• Rubrospinal tracts
•Send information to the flexor and extensor
muscles
Fine touch
 DEFINITIONS

Paresis : Weakness of body

Plegia/paralysis : Totally lost of voluntary
movement
Monoparesis : muscle weakness in one limb
Hemiparesis : muscle weakness in one side of the
body
Paraparesis : muscle weakness in both legs
Quadriparesis : muscle weakness in all four limb
WHAT IS PARAPLEGIA???
• Complete loss of motor funtion of lower half of
the body including both legs, with or without
sensory loss.
• HANDS and ARMS are not affected
• Involve thoracic and lumbrosacral segment.
FLACCID PARALYSIS
• Decrease tone
and contractility
along with
weakness.
• It is a lower
motor neuron
disease.
Differential diagnosis of Acute Flaccid Paralysis

SYSTEMIC DISEASE:
1. Acute porphyrias
2. Critical illness
neuropathy
3. Acute myopathy in
ICU patient

Reference :

http://jpcc.in/userfiles/2015
/0203-jpcc-jul-sep-
2015/JPCC0203049.html
Clinical Features
Features of lower motor neuron lesion:
• Weakness
• Wasting
• Hypotonia
• Reduced or absent reflexes
• Fasciculation
INVESTIGATION
• Routine blood investigation: FBC, Renal profile, etc.

• Plain X-ray of spine:

-look for paraspinal mass, collapse vertebrae,
osteophyte formation, or reduced intervertebral
space.

• Lumbar puncture and CSF analysis:

-look for increase of protein in CSF and to exlude ddx
meningitis.
• CT brain:
- To exclude other causes of weakness such as
infarction, hemorrhage, degenerative and
neoplasm.

• Spinal CT scan:
- Narrow disc space and look for compression with
contrast.

• MRI brain:
-To exclude other causes of weakness such as
infarction, hemorrhage, degenerative and neoplasm.

• MRI spine:
- Details view of cord compression
SPASTIC PARAPLEGIA

SITI NUR BAITI BINTI SHAIK

KHAMARUDIN
012013100196
 OUTLINE
Introduction
Definition of spastic paraplegia
Causes of spastic paraplegia

Causes in details
 Spinal cord compression
 Motor neuron disease
 Transverse myelitis
 SPASTIC PARAPLEGIA
Definition

Weakness or paralysis of
the muscle with increased
muscle tone

 a feature of upper motor

neuron lesion
CAUSES OF SPASTIC PARAPLEGIA
Spinal cord Intrinsic (non-compressive)
compression causes

Vertebral Congenital

Meninges Infective/inflammatory

Spinal cord Vascular

Neoplastic

Metabolic

Degenerative
 SPINAL CORD
COMPRESSION
 SPINAL CORD COMPRESSION
• Common neurological emergency.
• Space-occupying lesion within the spinal canal
damaging nerve tissue by:
– Directly: pressure
– Indirectly: blood supply interference

Oedema from venous obstruction

Impairs neuronal function, ischaemia

Necrosis of spinal cord

Is it reversible?
 early stages are reversible
 but severely damaged neurons do not recover
 CAUSES OF SPINAL CORD COMPRESSION

within the dural sac but not

within spinal cord
Davidson’s 22nd
Edition
pg: 1220
within the spinal cord
 CLINICAL FEATURES

• Onset of
symptoms is slow
(weeks).
• Can be acute due
to trauma or
metastases.
Davidson’s 22nd
Edition
pg: 1220
• The signs vary
according to spinal
cord compression
level & structures
involved
• May be tenderness
to spinal percussion,
associated with local
kyphosis

Davidson’s 22nd
Edition
pg: 1221
The Brown-Séquard
syndrome
– Damage is confined to
one side of cord
(hemisection of the
cord)
– probably the result of a
compressive or
destructive lesion of the
spinal cord.

250 Cases in Clinical Medicine,

4th Edition (Baliga)
Pg 330
INVESTIGATIONS
Davidson’s 22nd Edition
pg: 1221

MRI Define the extent of compression & associated

soft tissue abnormalities

Plain X-rays Bony destruction

Soft tissue abnormalities

Chest X-ray Evidence of systemic disease

Myelography + CSF analysis Complete spinal block = normal cell count with
increased protein

Needle biopsy Tissue diagnosis of secondary tumour

MANAGEMENT
i. Benign tumours  surgically excised
ii. Extradural compression secondary to
malignancy
 Radiotherapy – initiated within 24 hours
of symptoms
iii. Tuberculosis  may require surgery followed
by anti-TB chemotherapy
iv. Vertebral lesion  specialized neurosurgical
treatment
INTRINSIC DISEASES OF THE SPINAL
CORD
 INTRINSIC DISEASES OF THE SPINAL
CORD
• Interference of spinal cord function by
disorders of non-compressive involvement of
spinal cord itself.
• Signs and symptoms generally similar to
extrinsic causes:
– Urinary symptoms earlier in intrinsic causes than
in compressive causes
 Congenital

Degenerative Infective
Inflammatory

AETIOLOGY

Metabolic Vascular

Neoplastic
(A) CONGENITAL
Clinical features
• LMN features, deformity and legs sensory loss
• Impaired sphincter function
Diastematomyelia • Hairy patch or pit over low back
(spina bifida) Investigation
MRI spine  splitting of spinal cord
Management
Surgery
Clinical features
• Adult onset
• Slowly progressive UMN features, legs > arms
Hereditary Spastic • Little or no sensory loss
Paraplegia
Investigation
(autosomal
MRI  thinning of corpus callosum
dominant)
Genetic testing
Management
Baclofen (improve spasticity), rehab, genetic counselling
(B) VASCULAR
Clinical features
• Acute paraplegia
• Loss of spinothalamic sensation
Anterior Spinal • Sparing of posterior column sensation below lesion
Artery Infarct Investigation
MRI spine  hyperintense lesion = ischaemia)
Management
Physiotherapy & rehabilitation
Clinical features
• UMN, LMN features, sphincter disturbance
• Not localised to AVM sites
Spinal arteriovenous
Investigation
malformation
• MRI spine  swelling of cord, enlarged arteries & veins
(Spinal AVM)
• Spinal angiography
Management
Therapeutic: Endovascular embolization during angiography
(C) NEOPLASTIC
Clinical features
• Weakness and sensory loss
• Pain
• UMN features below lesion
• LMN features in conus
• Impaired sphincter function
Glioma, Investigation
ependymoma • Neuroimaging (CT head, MRI)
Management
• Gliomas may recur
• Partial excision (debulking) may be useful
• Radiotherapy and chemotherapy

(Davidson’s 22nd Edition, pg: 1215)

MOTOR
NEURON
DISEASE
 MOTOR NEURON DISEASE
A neurodegenerative condition, caused by:

Loss of upper and lower motor neurons in spinal cord, cranial

nerve nuclei and motor cortex

Causes:
 Abnormalities in superoxide dismutase (SOD1) gene  20%
 Expanded repeat sequence in C9ORF72 gene on chromosome
9
 Most common form is amyotrophic sclerosis (ALS)

Average age of onset: 65 years

 10% before 45
• Amyotrophic sclerosis (ALS) is characterized by
combination of upper and lower motor
neuron signs, rarely:
– pure lower motor neuron (progressive muscular
atrophy)
– Upper motor neuron (progressive lateral sclerosis)
 Davidson’s 22nd Edition
CLINICAL FEATURES Pg: 1200

• Presents focally, either:

 limb onset  foot drop
 bulbar symptoms
• Respiratory onset is rare
but it is a common
terminal event.
• Upon examination:
 combination of upper
and lower motor
neuron signs (e.g., brisk
reflexes in wasted
fasciculating muscles)
without sensory
involvement
  cognitive impairment is under-recognized in
MND:
 50% have executive impairment (cognitive, emotional
and behavioural difficulties)
 10% will develop frontotemporal dementia (FTD)
• 10% will develop ALS within few years of dementia onset

MND is progressive = up to 50% die within 2 years of

diagnosis
Davidson’s 22nd Edition
Pg: 1200
 Kumar and Clark’s Clinical Medicine, 7th Edition, pg: 1166
AMYOTROPHIC PROGRESSIVE BULBAR PRIMARY LATERAL
PROGRESSIVE
LATERAL SCLEROSIS & PSEUDOBULBAR SCLEROSIS
MUSCULAR ATROPHY
(ALS) PALSY (least common)
Course Course Involvement Involvement
• Progressive spastic • Wasting begins in • Lower cranial nerve Confined to UMN
tetraparesis/ hand small muscles, nuclei & supranuclei
paraparesis with may start connections Course
added LMN signs & unilaterally but may Progressive
fasciculation follow on opposite Features tetraparesis with
side Bulbar and terminal
Lateral sclerosis = pseudobulbar palsy: pseudobulbar palsy
disease of lateral Features • Dysarthria
corticospinal treacts • Widespread wasting • Dysphagia
& weakness • Nasal regurgitation
Amyotrophy = muscle • Fasciculation of fluid
wasting/atrophy • Loss of tendon • Chocking
reflex Mixed UMN and LMN
• Late onset sphincter signs in lower cranial
disturbance nerves
• E.g. wasted
fibrillating tongue
with spastic weak
palate
INVESTIGATION
Exclusion of treatable causes, example:
• immune-mediated multifocal motor neuropathy with
conduction block
• cervical myeloradiculopathy

Blood tests Normal

Cardiac enzymes Mildly increase creatinine kinase

May illicit reduced amplitude of motor AP due to

Nerve conduction studies
axonal loss
Confirm typical features of widespread denervation
Electromyography
and re-innervation

DNA testing Genetic factors

MANAGEMENT
No cure for MND NIV support and feeding
by percutaneous
gastrostomy
Multidisciplinary action:
 Physiotherapists
 Speech therapist
 Occupational therapist
 Dieticians Rapid access benefits
 Ventillatory and feeding support patients in terminal stage
of MND
 Palliative care teams
TRANSVERSE MYELITIS
 An acute, usually monophasic, demyelinating disorder affecting
the spinal cord.

• Usually thought to be post-infection.

• Any age.
• Presentation:
– subacute paraparesis with a sensory level + severe pain in
the neck or back at the onset

INVESTIGATION
MRI Distinguish TM from external lesion affecting
spinal cord

CSF analysis Cellular pleocytosis often with polymorphs at

onset

CSF oligoclonal band screening Absent (unlike in multiple sclerosis, it is

present)
 MANAGEMENT UpToDate

High-dose intravenous corticosteroids

• standard of care and first-line therapy in acute idiopathic TM.
• effective in acute inflammatory central nervous system diseases like TM, such as multiple
sclerosis.
• helps to reduce swelling of spinal cord
• methylprednisolone (1000 mg daily) or dexamethasone (200 mg daily) for three to five
days
TREATMENT
Plasma exchange (PLEX)
• ifIVno
high-dose methyl-prednisolone
improvement (Davidson’s)
occurs with corticosteroid therapy.
• preferred regimen is five treatments, each with exchanges of 1.1 to 1.5 plasma volumes,
every other day for 10 days.
OUTCOME

Static Develop
deficit MS

Recover with
no relapse

Davidson’s 22nd Edition

Pg: 1193
REFERENCE
SPASTIC PARAPLEGIA
QUADRIPLEGIA
SITI NURDAYANA BINTI MOHD
SUHAIMI
 OUTLINE
• Syringomylia
• Subacute combined degeneration of spinal
cord
• Multiple sclerosis
• Approach to quadriplegia
SYRINGOMYELIA
 SYRINGOMYELIA &
SYRINGOBULBIA

• Fluid filled cavity within • Fluid filled cavity

spinal cord within the brainstem
 SYRINGOMYELIA
• a disorder in which a cyst, called a syrinx,
forms around the central canal of the cervical
cord.
• expands and elongates over time, it
compresses and damages part of your spinal
cord from its center outward.
 SYRINGOMYELIA

• usually appears in the 3rd or 4th decade of

life, with a mean age of onset of 30 years
• Men > female
• Symptoms may be static for years, but then
worsen fast (slow but progressive)
- eg: on coughing/sneezing
(may increase pressure extension,eg into braistem (syringobulbia)
SYRINGOMYELIA

Causes? (blocked csf ciculation)

- Arnold-Chiari malformation (most)
# cerebellum herniates through foramen magnum
- Other causes of syringomyelia include
spinal cord tumors (ependymoma/hemangioblastoma)
- d/t fluid secreted from neoplastic cell/h’
spinal cord injuries
damage caused by inflammation around your spinal
cord.(eg: transverse myelitis)
 Clinical feature
• Symptoms begins insidiously, gradual onset over month/years
• Upper limb pain exaggerated by exertion or cough
• Dissociated sensory loss: Spinothalamic sensory loss (pain and
temperature)…leads to painless upper limbs burns and trophic changes
(scars and nail dystrophy) ,dorsal column preserved
• Cervical fluid filled cavity (additional):
Loss of upper limbs reflexes
Hand and forearm muscle wasting
Spastic paraparesis
Neuropathic joints & ulcers
Brainstem signs (if syrinx extend into brainstem): tongue atrophy &
fasciculation, bulbar palsy, nystagmus, Horner’s syndrome, hearing loss
& impairment of facial sensation.
Investigation
• MRI:
• To know the size of the syrinx
• TRO any base-of-brain (chiari) malformations
Management
• Needs surgery:
• Decompression @ the foramen magnum
may be tried in Chiari malformations
• To promote free flow of CSF
• Prevent syrinx dilatation
• Reduces pain & progression
SUBACUTE COMBINED DEGENERATION OF
SPINAL CORD
• refers to degeneration of the posterior and
lateral columns of the spinal cord as a result
of vitamin B12 deficiency
-posterior column dysfunction decreases
vibratory sensation and proprioception (joint
sense).
-Lateral corticospinal tract dysfunction
produces spasticity + UMN sign

• Onset: insidious
Vitamin B12 Def:- causes

78
-posterior column dysfunction decreases
vibratory sensation and proprioception (joint
sense).
-Lateral corticospinal tract dysfunction
produces spasticity + UMN sign
 CLINICAL FEATURES OF
SUBACUTE COMBINED DEGENERATION
a. Numbness and tingling of fingers and toes
b. Distal sensory loss (proprioception, light touch & vibration
c. ataxia
d. Absent ankle reflex (LMN)
e. Absent knee reflex (LMN)
f. Positive Babinski sign (umn)
g. Optic atrophy and retinal hemorrhage may occur
h. Dementia

# spinothalamic tract still preserved

management
TREATMENT
Hydroxycobalamin 1000 mcg
IM for 6 doses 2-3 days apart
Followed by maintenance
therapy of 1000 mcg every 3
months for life

Without treatment, fatal within

5 years
MULTIPLE SCLEROSIS
 What is multiple sclerosis?
A chronic neurological disorder that affects the
central nervous system,

in which myelin is destroyed in the brain and spinal

cord (demyelinating d/o of cns)

causes scarring at multiple sites in the CNS.

3
 MULTIPLE SCLEROSIS

Most common disabling condition in young adults

Most common demyelinating disorder
Progresses to disability in majority of cases
Current theory favors immunologic pathogenesis

4
 MULTIPLE SCLEROSIS AFFECT:

>
The ratio is
increasing worse
now prognosis

Predominant age: 20-40

onset before puberty or after the age of 60 years
is rare.
Highly variable and unpredictable

MS is more
common in
temperate
regions, such as
northern
Europe and
North America,
but much rarer
in the tropics.

87
 MULTIPLE SCLEROSIS
• Cause- UNKNOWN
• But, there is a linked that:
ENVIRONMENT:
sunlight exposure, vitamin D deficiency exposure to Epstein
Barr Virus (EBV)-mechanism became unclear
GENETIC:
↑ risk in first degree relative. Twin.
The risk of developing MS is associated with certain class I
and class II alleles of the major histocompatibility complex
(MHC), particularly the HLA-DRB1 locus

• The most widely accepted theory is that MS begins

as an inflammatory autoimmune disorder
mediated by autoreactive lymphocytes.
• Later, the disease is dominated by microglial
activation and chronic neurodegeneration
CLASSIFICATION OF MULTIPLE SCLEROSIS

PRMS Progressive Relapsing MS

Steady decline since onset with super-imposed attacks.

SPMS Secondary Progressive MS

Initial RRMS that suddenly begins to decline without
periods of remission and relapses.

PPMS Primary Progressive MS

Gradual progression of the disease from its
onset with no relapses or remissions

RRMS Relapsing/ Remitting MS

Unpredictable attacks which may or may not leave
permanent deficits followed by periods of remission
CLINICAL
FEATURES
 Diagnosis of MS
2 or more attacks affecting
different parts of the CNS
i.e. dissemination in time and
space and exclusion of other
possible causes
Macdonald criteria for the diagnosis
of multiple sclerosis
 Investigation
To further demonstrate the dissemination in time and space.
To exclude other D/D.
1. MRI brain and spinal cord: show the demyelination plaques.
2. VEP: delayed indicating optic neuritis.
3. Lumbar puncture and CSF analysis:
CSF may show a lymphocytic pleocytosis in the acute phase.

CSF electrophoresis >>>>oligoclonal bands between attacks.

in 90 % of cases (not specific)
28
TREATMENT
i. IV or oral steroids
ii. Acute relapse: IV
methylprednisolone 3mg for 3
days
iii. To prevent further relapse:
i. Beta-interferon SC/IM
injections every alternating
days OR
ii. Glatiramer acetate SC every
alternating days OR
iii. Alemtuzumab IV infusion for
2 courses (5 days, 3 days)…in
severe cases.
QUADREPLEGIA

95
OUTLINE
• Causes
• Clinical presentation according to the level of lesion
• Quadriplegia
Paralysis affecting all 4
limbs
Also called as tetraplegia

# lesion above T1
vertebra cause
quadriplegia
CAUSES
• Traumatic
- Motor vehicle accident
-Falls
-Sport injuries
-Gunshot and stabbing injury

• Non Traumatic

• Process affecting spinal cord or blood supply directly:

• Multiple sclerosis
• Transvere myelitis
• Syringomyelia
• Spinal cord infarction
• Spinal atriovenous malformation
• Compressive lession
• Spinal epidural abscess
• neoplasm
• metastatic
• discitis
• Innervation of Muscles
C5 arm abductors, external rotators, & extensors
C6 biceps, brachioradialis, & wrist extensors
C7 triceps, pronator teres, wrist flexors
&finger extensors
C8 finger flexors
T1 intrinsic mucsles of hand
Clinical presentation according to the level
of lesion

C1-C4 Quadriplegia
• C1 – C2 : may have functional phrenic nerves
• C3: impaired breathing, ventilator dependent
• C4: may be free from advanced respiratory
support but require functional equipment
need as C3
C5 Quadriplegia
Muscular weakness • Deltoid
• Supraspinatus
• Brachoradialis
Deep tendon reflexes • Biceps
affected • Supinator
Radicular • Neck
pain/paraesthesia • Top of shoulder
• Outer aspect of arm & forearm
Superficial sensory • Outer aspect of the forearm
deficit
How to localize the lesion to the 7th cervical root
level?
C7 Quadriplegia
Muscular • Triceps
weakness • Most of the muscles on
the dorsum of forearm
Deep tendon • Triceps jerk
reflexes affected
Radicular • Neck
pain/paraesthesia • Shoulder
• Arm
• Forearm to index &
middle fingers
Superficial sensory • Mostly middle & index
deficit finger
How to localize the lesion to the 8th cervical root
level?
C8 Quadriplegia
Muscular • Flexors of the forearm
weakness
Deep tendon • Finger jerk
reflexes affected
Radicular • Neck
pain/paraesthesia • Shoulder
• Arm
• Ring & little finger
Superficial sensory • Ring & little finger
deficit
 REFERENCES
• 250 CASES IN CLINICAL MEDICINE, 4TH ED, R.R BALIGA (PG 89)

• MEDSCAPE

• WWW.HOPKINSMEDICINE.ORG

• WWW.DISABLED-WORLD.COM
THANK YOU