Diseases of Spine

and
Spinal Cord

dr. Mimi Lotisna,Sp.S

AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
DEFINITION
Amyotrophic Lateral Sclerosis
neurodegenertive disease
progressive degeneration of
motor neuron.
ALS known as Lou Gehrigs
disease. Lou Gehrig baseball
player he quit because of
difficulty in throwing ball and
running.
EPIDEMIOLOGY
ALS is the most common form of
motor neuron disease.
It affected 1-2/100.000 people per
year
5 10% familial
>> 40-60 years old
Male > female
Every race & ethnic
ETIOLOGY
Not yet known
Proposed etiology:
Familial : 5-10% (dismutation of SOD1
enzyme, mutation of gene FUS)
Sporadic :90% (>> found in West Papua,
West Pacific, Kii Peninsula in Japan)
Developing theory: oxydative stress,
mitochondrial dysfunction, glutamate
toxicity, slow virus, autoimmune.
RISK FACTORS
Exposure to neurotoxins or heavy
metal
Immunology system abnormality
DNA defect
PATOPHYSIOLOGY
Motor neuron as a connector from
the nerve system to the voluntary
muscle all over the body.
Motor neuron can be found in the
brain, brainstem, & spinal cord.
Message from the motor neuron in
the brain brought to the motor
neuron in the spinal cord, & then to
the muscles.
PATOPHYSIOLOGY
In ALS degeneration or necrosis
of the motor neuron, UMN & LMN
signs, no signal brought to the
muscle.
Muscles became atrophy &
fasciculation.
Voluntary control and movement of
muscles dissapear.
SYMPTOMS & SIGNS
Initial symptoms: weakness &/or muscle
atrophy, followed by fasciculation,
paresthesia, spasticity of the affected
muscle.
75% of cases affect extremities: difficulty
in walking or running, frequent falls or
imbalance, writing, button/unbuttoning
difficulty. Sometimes weakness only found
in one extremity
25% bulbar onset: difficulty in swallowing
(dysphagia), dysarthria.
CLINICAL
MANIFESTATION
Progressive weakness
Spasticity, hyperreflexes,vomiting reflex
Pathologic Reflex (+)
Dysphagia
Dysarhtria
LMN signs: muscle atrophy & fasciculation
15-45% patient experienced pseudobulbar
palsy
In later case, weakness spread to other
extremities movement difficulty.
DIAGNOSIS
Anamnesis
Physical examination
EMG (Electromyography)
NCV (Nerve Conduction Velocity)
MRI (for Differential diagnosis)
Differential Diagnosis
Spinal cord tumor
Multiple sclerosis
Syringomyelia
Cervical spondylosis
 Treatments
There is no known cure
for ALS.
The first drug treatment
for the disease is a
medicine called riluzole.
The goal of the
treatment is to control
the symptoms and
maintaining an optimal
quality of life.
Treatment is based on
individual therapy and
the continual adaptation
of medications.
PROGNOSIS
Incurable disease
Death in 3 5 year after onset,
because of respiratory arrest.
10% of ALS patient can survive up to
10 years or more.
COMPLETE SPINAL
TRANSECTION
DEFINITION
Spinal cord injury: dissection of the
spinal cord (partially or complete)
affect 3 functions in the spinal cord:
motoric, sensoric & autonomic
nerve system.
ETIOLOGY
Primary:
Traumatic :
- Vertebral dyslocation
- Vertebral fracture
- Gun shot injury
Non traumatic :
- Infection
- Tumor or malignancy
Secondary:
spinal cord vascular injury rupture of artery,
thrombosis, hypoperfusion due to shock.
Patophysiology
Mechanical trauma traction & compression of
the spinal cord.
Direct Sp.Cord compression by bone fragments,
discs, & ligaments disturb nerve system (central
& peripheral)
Vascular disturbance ischemic.
Rupture of axons & cell membrane of the neuron
Micro hemorrhage (occur in central gray matter)
meluas dalam beberapa jam.
Massive oedema occur in minutes oedema will
fill all the space in the spinal canal secondary
ischemic
Loss of autoregulation & spinal shock cause
systemic hypotention & worsen the ischemic.
Patophysiology
Secondary injury: contain toxic ischemic-
metabolytes & electrolyte changes.
Spinal shock: due to hypoperfusion in the gray
matter through the white matter action
potential changes along the axons.
Excess Glutamate release over stimulation of
the neuron free radicals disturbed normal
neuron. Exitotoxic mechanism demyelination.
Alpha-amino-3-hydroxy-5-methyl-4-isoxazole
propionic acid (AMPA) glutamate-receptor play
important role in oligodendrocyte cell death.
Can develop to syringomyelia.
Clinical Manifestations
On acute phase: classic manifestation is complete spinal cord
transection.

Upper Cervical lesion:

Respiratory insufficiency
Tetraplegia & areflexia
Anesthesia below the afected segment
Neurogenic shock (hypothermia and hypotension, without
tachycardia)
Rectal & bladder spinchter tone
Urinary & alvi retention
Horner syndrome (ipsilateral ptosis, miosis, anhidrosis)
Clinical Manifestations
Injury on the lower cervical & upper thoracal:
Same as upper cervical injury, without respiratory
muscles involvement.
Tetraparesis/Paraparesis UMN type
Autonomic system disturbance
Injury on the lower thoracal & lumbosacral
segment :
Paraparesis UMN
Alvi & urinary retention
Clinical Examination
Tendon Reflexes & superficial
reflexes: must be evaluated! to
identify the level of the lesion.
Loss of abdominal muscle contraction
lesion on Th 9-11 spinal cord.
Loss of cremaster reflex lesion on Th
12-L1 spinal cord.
RADIOLOGICAL
EXAMINATION
Plain Vertebral X-Ray (AP
& lateral posistion,
oblique position for
cervical vertebra)
CT Scan
Myelography
CT-Myelography
MRI
MANAGEMENT
Pre hospital management
Vertebra immobilisation spine
board or cervical collar until
vertebral fracture is excluded
through radiologic exam.

Goal: life saving

Airway, Breathing dan Circulation
A,B,C
MANAGEMENT
ON hospital management
1.Prevention of aspiration jaw thrust, and
intubation if necessary
Upper spinal cord segment lesion (mid cervical)
Function of diaphragm (N.phrenicus: C3-5),
Monitoring of vital capacity.
2. Managing secondary effect due to the spinal cord
injury R/ Steroid: Methylprednisolone iv,
within 8 hours of onset motoric & sensoric
improvement.
MANAGEMENT
3. Spinal shock
Due to autonomic dysfunction
Need of fluid replacement to regain
normotension.
Decrease perfusion to vital organ, e.g.
Kidney renal failure (urine < 30
ml/hour)
-Agonist (Phenylephrine) increase
peripheral vascular resistence
Dopamine 2 5 g/kg/min.
MANAGEMENT
4. Acute Respiratory Failure
Supportive management O2
100%.
Intubation if acute respiratory
failure develop, decrease of
consciousness (GCS <9), increased
respiratory rate with hypoxia, PCO2
> 50, vital capacity < 10 ml/kg.
MANAGEMENT
5. Nutrition & fluid management
Fluid intake to maintain plasma
volume & kidney function
Parenteral fluid/nutrition
administration.
Examination of peristaltic function.

6. Surgical management
 PROGNOSIS
Complete spinal cord injury: chance
of recovery < 5%.
Incomplete spinal cord injury
better prognosis compare to
tetraplegia with loss of sensation.
 HEMISECTION OF SPINAL CORD OR
BROWN SEQUARD SYNDROME:
CAUSES:
Fractures
Tumors
accidents
FEATURES: 3 components:
1) above the level of hemisection.
2) at the level of hemisection.
3) below the level of hemisection.
 This syndrome was named by Brown
Sequard as:
1. MOTOR LOSS IPSILATERALLY
2. PROPRIOCEPTIVE IPSILATERALLY
3. EXTEROCEPTIVE CONTRALATERALLY