Farry, dr.

NEUROMUSCULAR DISEASE

ANATOMY OF PERIPHERAL NERVE

INTRODUCTION
Neuromuscular disease
inherited

acquired

neurophysiol
ogy
Influence
regeneration

PATHOPHYSIOLOGY
MYELIN SHEATH
(DEMYELINATION
)
INJURED
NERVE
AXON OF
NERVE CELL
(AXOTOMY)

IMPAIR
NEURAL
FUNCTION

AXOTOMY

Nerve is
injured

Wallerian
Degenera
tion

regeneratio
n

Wallerian Degeneration
Function

Clear axonal &

myelin debris
Create
hospitable
environment

Degenerative process
includes
Granular
disintegration of
axonal cytoskeleton

Axoplasmic
calcium

Decrease produce of
neurotransmitter

Production of protein,eg: tubulin,

actin, GPs

24
hours
Schwann cells begin to
divide
Forming Band of
Bunger
Myelin debris is
removed

72
hours
Accumulation of
Macrophage
f/
Phagocytes of myelin
Express 1a antigen
Produce IL-1
Stimulate nerve GF

RECOVERY FROM AXONAL

NEURAL INJURY

Myelin thickness
Increase later
smaller than
preinjury
Conduction velocity

Axonal cytoskeleton sprout from nodes

of Ranvier
Growth cone develops from tips
axon
Schwann cells & basal
lamina

Enter distal
segment

12mm/day

Axonal
Regeneration occur

Augmented by
guidance, eg
- Artery
- Vein
- Muscle
- Collagen
- Slicone
- etc

Limited by scar w/
- Obstructive
- Decrease axon to
distal
- Delay elongation
- Misdirect axon

DEMYELINATION
Progressive
demyelination
Chronic Nerve
Compression
Carpal tunnel
Syndrome

No axonal
degeneration

Slowing nerve conduction

velocity
Slow macrophage
recruitment
Schwann Cell
turnover

INJURY OF NERVE TISSUE

Seddon (1943) classification

Sunderland (1951) classification

Treatment of Nerve Injury

Type I & II : no surgical treatment
Type III : incomplete
regeneration
excessive scar tissue
surgery is required
Type IV & V : poor prognosis
poor recovery
potential

NERVE CONDUCTION VELOCITY

STUDIES
Assess large myelinated
nerve fibers
Complementary to
EMG
- determine physical
integrity
- excitability thresholds
- dysfunction

Median, ulnar,
radial
Peroneal,
posterior tibial,
plantar, femoral,
sural

1.Size of response (mV for motor &

V for sensory)
2.Latency of response (msec)
3.Conduction velocity (m/s)

MOTOR NERVE CONDUCTION VELOCITY STUDIES

Evaluate response by supramaximal

stimulation
Summation of activity from
muscle fibers
Recorded in
orthodromic
Median, ulnar, peroneal,
tibial

MCVS

measures severity of
diffuse/focal disorders of
peripheral nerves
Identify and localize
compression, ischaemia, or other
focal lesions

Focal lesion:
Conduction block w/ neurapraxia
Conduction velocity at affected
area
Wallerian degeneration
Neural regeneration

SENSORY NERVE CONDUCTION

Identify whether the defect
Preganglionic
Postganglionic
Ganglionic refers to dorsal root
ganglion
(primary sensory neuron)

Postganglionic
eg: brachial plexopathies
Continuity nerve-dorsal root is
affected
Integrity challenged
Abnormal sensory conduction
velocity

Preganglionic
eg: Radiculopathy
Sensory symptoms (+)
Alteration of sensory nerve conduction
studies (-)

ELECTROMYOGRAPHY
Records and analyzes electrical
activity in motor units of the muscle
when the muscle contracts/muscle
membranes are perturbed
Forms:
1. Intramuscular needle EMG
2. Single fiber EMG
3. Surface EMG

Function

Diferentiate neurogenic vs
myogenic disorder

Follow disease progression

STAGES IN EMG
1.

2.

3.

4.

Needle introduction insertional

activity
Assessment abnormal
spontaneous activity in muscle
Resting position detect
fasciculations
Assessment volitional activity
Motor Unit Potential (MUP),
recruitment & interference
frequencies

EVALUATION OF
NEUROMUSCULAR DISEASE

ANTERIOR HORN CELL DISORDER

Poliomyelitis

Caused by polio virus

Infects through fecal oral route anterior
horn cells of spinal cord LMN type
paralysis
Phases:

Acute: initial infection diffuse and severe

paralysis
Subacute: anterior horn cell survival axon
sprouting muscle hypertrophy
Postpolio syndrome: 30-40 years after initial
infection increasing muscle weakness, muscle

Amyotrophic Lateral Sclerosis

(ALS)
Most common neurodegenerative
disease
Affects LMN in anterior horn spinal
cord & UMN in precentral gyrus
Sign & symptoms: weakness, muscle
cramps, bulbar problems with speech
and swallowing
UMN: spasticity, hyperreflexia
LMN: muscle atrophy, weakness

RADICULOPATHY

Root involvement of neuropathy

Sensory or motor disturbances
NCVS (N) preganglionic lesions
EMG myotomal patterns

BRACHIAL PLEXOPATHY

Traumatic, inflammatory, neoplastic,

radiation
Severe sensory and motor deficits
NCVS abnormal postganglionic
EMG widespread dennervation
Myokymia radiation-induced

PERIPHERAL NEUROPATHY

Eg: Carpal Tunnel Syndrome

Mononeuropathy/multiple
mononeuropathy/ polyneuropathy
Entrapment of nerve affects motor &
sensory
Axonal loss severe forms

Multiple mononeuropathy
Peripheral nerve vasculitis
Polyneuropathy
Axonal or demyelinating conditions
B12 deficiency
Hypothyroidism
Toxicity
Immune conditions

MYOPATHY

Inflammatory muscle
disease/muscular dystrophy
Require surgery
EMG short duration & small
amplitude polyphasic potential
Fibrillations (+) muscle fibre
necrosis/membrane defects
Muscle creatine phosphokinase

NERVE REPAIR
Objective :
Maximize axons regeneration
Maximize accuracy of reinnervation

Conditions:
Clean wound
Healthy tissue bed
Available surgeon
Surgical equipment & staff(+)
Stable patient

Primary repair
Sharp transection
Crush segment excised
Open # Explore nerve
Delayed primary
5-7 days
Avulsion/ crush injury zone of injury is unclear
May require nerve grafting
Secondary nerve repair
> 7 days after injury

Principle of nerve repair

Quantitative preop & postop clinical
assessment
Motor & sensory systems evaluation
pinch/grip; 2 point discrimination; pressure
stimulus; vibration threshold
Tension free
Tools:

Microscopes and loupes

Microsurgical instruments
Appropriate sutures

Techniques
Epineural suture
Mixed sensory and motor fascicle
and not possible to identify individual
groups fascicle
Identify proximal and distal nerve
ends in a bloodless field epineural
sleeves joined to oppose the neural
contents nerve ends are trimmed
with axons are aligned suture is
made using 8-0, 9-0, 10-0 suture with

Grouped fascicular repair

A particular fascicle is recognized as a
specific function
Higher magnification is needed
determine the alignment of fascicles
allow matching, trimming, and repair of
fascicular groups

Nerve Graft
Indications
Large nerve gaps (end to end nerve
repair cannot be achieved without
tension)
Very proximal injuries
Compromised tissue beds (radiated
tissue)
Large caliber donor nerve grafts

Nerve graft
Large fascicles
Little connective tissue
Large diameter
Large caliber axon
Little variability
Little branching
Accesible location

Sites for graft

Sural
Medial antebrachial cutaneus (anterior
branch)
Lateral antebrachial
Terminal branch of interosseus nerves

SYNTHETIC GRAFT

Cadaveric nerve allograft

Acellullar allograft
Synthetic axonal/nerve guidance channels

Made from:
Silicone, ECM, basal lamina (laminin &
fibronectin)
Schwann cell coated, brain derived GF,
insuline-like GF-1

THANK YOU