PATTERNS OF NERVE INJURY AND

NEUROPATHIES
.

PNS THE MOTOR UNIT

The functions of the neuromuscular system depend on the motor units, each consisting
of:

• (1) a lower motor neuron in the anterior horn of the spinal cord or cranial nerve
motor nucleus in the brain stem,

• (2) the axon of that neuron,

• (3) muscle fibers it innervates.

NORMAL PERIPHERAL NERVE

• The principal structural component of peripheral nerve is the
nerve fiber (an axon with its Schwann cells and myelin
sheath)

• A nerve consists of numerous fibers that are grouped into

fascicles by connective tissue sheaths.

• Myelinated and unmyelinated nerve fibers are intermingled

within the fascicle.

NORMAL PERIPHERAL NERVE

• Peripheral nervous system axons are myelinated in segments
(internodes) separated by nodes of Ranvier.

• A single Schwann cell supplies the myelin sheath for each

internode.
NORMAL PERIPHERAL NERVE
• The three major connective tissue components of peripheral
nerve are
• Epineurium, encloses the entire nerve

• Perineurium, encloses each fascicle

• Endoneurium, encloses individual nerve fibers.

BARRIERS
• Perineurial barrier

• Blood nerve barrier

• Nerve CSF barrier

RESPONSE OF PERIPHERAL NERVE TO

INJURY
• Diseases affecting Schwann cells: Segmental
demyelination

• Diseases affecting neurons & it’s axons: Axonal

degeneration regeneration and reinnervation of
muscle.
RESPONSE OF PERIPHERAL NERVE TO
INJURY
1. Segmental demyelination:

 when there is dysfunction of the Schwann cell (as in hereditary motor and sensory
neuropathy)

 or damage to the myelin sheath (e.g., in Guillain-Barré syndrome);

• There is no primary abnormality of the axon.

• The disintegrating myelin is engulfed by Schwann cells and macrophages.

RESPONSE OF PERIPHERAL NERVE TO

INJURY
• The denuded axon provides a stimulus for remyelination.

• A population of precursor cells within the endoneurium has the

capacity to replace injured Schwann cells. These cells proliferate and
encircle the axon and, in time, remyelinate the denuded portion.

• Newly formed myelinated internodes are shorter than normal. The new
myelin sheath is also thin in proportion to the diameter of the axon.

RESPONSE OF PERIPHERAL NERVE TO

INJURY
. Axonal degeneration and muscle atrophy:
2

• Primary destruction of the axon, with secondary disintegration of its myelin

sheath.
• Focal injury such as trauma or ischemia or
• Generalized abnormality affecting the neuron cell body (neuronopathy) or its
axon (axonopathy).
WALLERIAN DEGENERATION

In case of traumatic transection of a nerve, the distal portion of the fiber

undergoes Wallerian degeneration .
• Within a day the axon begins to break down,
• The affected Schwann cells begin to catabolize myelin and engulf axon
fragments, forming small oval compartments (myelin ovoids).
• Macrophages participate in the phagocytosis of axonal and
myelin-derived debris.
• The stump of the proximal portion of the severed nerve shows
degenerative changes involving only the most distal two or three
internodes and then undergoes regenerative activity.

WALLERIAN DEGENERATION
• The muscle fibers within the affected motor unit lose their
neural input and undergo denervation atrophy.

• Breakdown of myosin and actin, with a decrease in cell size,

but cells remain viable.

• The atrophic fibers are smaller than normal and have a

roughly triangular (“angulated”) shape.
NEUROPATHIES
• NEUROPATHIES
– Inflammatory
– Infectious
– HereditaryHMSN-II,
(HMSN-I
HMSN-III)

– Acquired Toxic/Metabolic
– Traumatic
– Neoplasms

NEUROPATHY, INFLAMMATORY
• Guillain-Barré
 – Preceded by “influenza”-like illness

– NO actual specific etiologic agent isolated, autoimmune disease to myelin

gangliosides most likely

– Inflammation of a peripheral nerve

– DEMYELINATION

– “ASCENDING” paralysis

NEUROPATHY, Infectious
• Leprosy
• Diphtheria
• V/Z (Varicella-Zoster)

NEUROPATHY, Hereditary
(defective myelination)
(Hereditary Motor and Sensory Neuropathy)

• HMSN-I (Charcot-Marie-Tooth)
• HMSN-II (Like CMT of the neurons)

• HMSN-III (Palpable Nerves) (aka, Dejerine-Sottas)

 NEUROPATHY, TOXIC/METABOLIC
• Diabetes Mellitus

• Vitamin Deficiencies (many Bs, E)

• Heavy Metals, Pb, As, etc.

• Organic Compounds

• CHEMO

NEUROPATHY, Traumatic
• Laceration

• Avulsion

• Carpal Tunnel

• Traumatic (amputation) “Neuroma”

• “Saturday Night” Palsy (radial n.)

• Morton “Neuroma”
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