OVERVIEW What is the range of the

peripheral nervous system

(PNS)?
• The PNS includes all neural structures
lying outside the pial membrane of the
spinal cord and brainstem with the
exception of the optic nerves and olfactory
bulbs, which are special extensions of the
brain.
• ★Ⅲ ～Ⅻ Cranial nerve
• 31 pairs of spinal nerves
 Epineurium EP
Dura Mater (DM)
Endoneurium
(EN)
Subarachnoid
Angle (SA ）
Perineurium (P)
Arachnoid (A)
Anterior horn
Root Sheath
(RS)
Pia Mater (PM)
8 cervical,
12 thoracic,
5 lumbar,
5 sacral,
and 1
coccygeal
• myelinated fibers : axons are coated with
short segments of myelin of variable
length (250 to 1000 um), each of which is
enveloped by a Schwann cell and its
membrane, in charge and movements and
sensations.
• Unmyelinated : terminate in sympathetic or
parasympathetic ganglia.
 Unmyelinated

myelinated
 Etiology of PND
• Inflammatory: Guillain-Barre syndrome, collagen vascular
disease (eg, rheumatoid arthritis, sarcoidosis, Sjögren
syndrome)
• Nutritional: Diabetes mellitus, VB1/B12 deficiency
• Toxins: cis-platinum, alcohol, porphyric, arsenical, and
uremic
• Genetic disorders: Charcot-Marie-Tooth disease
• Traumatic
• Paraneoplastic
Pathology of Peripheral Nerve
Disease

Neuronal
degeneration
axoplasmic transport
• Wallerian degeneration is the process
of anterograde degeneration of the
axons and their accompanying myelin
sheaths due to a proximal axonal or
neuronal cell body insult.
• Wallerian degeneration might be
described as "dying forward," a
process in which the nerve
degenerates from the point of axonal
damage outward.
• The axon is affected from the distal-most
site to the proximal, with dissolution of
myelin that occurs in parallel with the
axonal change.
• One possible explanation for this process is
that the primary damage is to the neuronal
perikaryon, which fails in its function of
synthesizing proteins and delivering them to
the distal parts of the axon.
• “dying back”
• The myelin sheath is the most
susceptible element of the nerve
fiber,focal degeneration of the myelin
sheath with sparing of the axon is
called segmental demyelination.
• The myelin can regenerate better than
axon.
Neuronal degenetration
• The anterior horn cell is affected
by a disease process (motor
neuron disease), and in the latter,
the sensory ganglion cell
(ganglionopathy) is destroyed.
Degeneration of the respective
nerve fibers follows.
 SYMPTOMATOLOGY OF
PERIPHERAL NERVE DISEASE
• Impairment of Motor Function:
weakness, muscular atrophy
• Tendon Reflexes diminution or loss
• Sensory Loss
• Paresthesias, Pain, and
Dysesthesias: Pins and needles,"
stabbing, tingling, prickling, electrical.
Perversion of sensation,
hypersensitivity
“glove-and-stocking” distribution
Radicular pain
• Sensory Ataxia and Tremor
• Deformity and Trophic Changes Charcot
joint
• Autonomic Dysfunction lack of sweat, tears,
and saliva; sexual impotence; weak bowel
and bladder sphincters with urinary retention
or overflow incontinence; and weakness and
dilatation of the esophagus and colon.
• Fasciculations, Cramps, and Spasms
Charcot joint
Muscular atrophy
Fasciculation
 ‘rest and digest’
‘fight or flight’
 Guillain-Barre Syndrome
A condition in which the body's immune system attacks the nerve
s. It can cause weakness, numbness or paralysis. Weakness and
tingling in the hands and feet are usually the first symptoms. Thes
e sensations can quickly spread and may lead to paralysis.
•Incidence 0.4 to 1.7 cases per 100,000 persons per year
• age range 8 months to 81 years
The pathophysiology of GBS can be delineated
into two pivotal stages: initiation by an
immunological trigger and immune‐mediated
disruption of axons and/or myelin.
Based on electrophysiology, GBS has been
traditionally divided into two forms: acute
inflammatory demyelinating
polyradiculoneuropathy (AIDP) and acute motor
axonal neuropathy (AMAN).
 Clinical Vignette

A 47-year-old man reported a 10-day history of progressive distal and proximal

weakness and paresthesias in his arms and legs. He did not report bowel or
bladder dysfunction, dysarthria, dysphagia, or dyspnea. He remembered a mild
and transient upper respiratory infection 2 weeks before onset of his
neuropathic symptoms but otherwise had been well. His medical, family, and
social history were unremarkable. He was not taking medications. Vital signs
were normal without orthostatic hypotension or tachycardia. Forced vital
capacity and negative inspiratory force were normal. Neurologic examination
demonstrated mild facial and symmetric primarily distal weakness in the lower
and upper extremities. The patient was areflexic, and his toes were flexor
to plantar stimulation. Vibration and joint position sensation were abnormal at
the toes and ankles but normal at the fingers. Pinprick, temperature, and
light touch sensation were normal, with no spinal cord “sensory level.” The
patient displayed mild dysmetria with heel-to-shin testing but performed
well on
finger-to-nose testing. His gait was characterized by weak-ness, with bilateral
foot drop, and he was unsteady. The Romberg test was abnormal.
Cerebrospinal fluid (CSF) examination results demonstrated increased protein
of 107 mg/dL and only 3 WBCs. EMG disclosed multifocal signs of demyelination.
This clinical and laboratory set of findings was highly suggestive of GBS. The
significant degree of weakness made him a good candidate for
immunomodulatory therapy.
Plasmapheresis (PE) was begun on the second hospital day, with IVIG held
in reserve. His autonomic and respiratory statuses were monitored closely.
Except for mild intermittent tachycardia, the patient remained free of
dysautonomia or respiratory compromise. By day 9 of hospitalization, the
patient was walking without assistance. He was transferred to a rehabilitation unit
after completing the PE. At follow-up 4 weeks later, he was asymptomatic.
 Symptomatology
• Antecedent events ： up to 4 weeks before
upper respiratory tract infections/ gastroenteritis
vaccinations/ ganglioside administration/ surgery
• Symmetrical weakness ,usually the lower extremities before
the upper ; the trunk, intercostal, neck, and cranial muscles
may be affected later.
★5 percent of patients to total motor paralysis with
respiratory failure
More than half of the patients complain of pain and
an aching discomfort in the muscles, mainly those
of the hips, thighs, and back
• Reduced and then absent tendon reflexes
are consistent findings.
• Sensory loss occurs to a variable
degree ,deep sensibility (touch-pressure-
vibration) tends to be more affected than
superficial (pain-temperature).
• cranial nerve palsies, Facial diplegia ★ ,
• Disturbances of autonomic function (sinus
tachycardia and less often bradycardia,
facial flushing, fluctuating hypertension
and hypotension, loss of sweating, or
episodic profuse diaphoresis)
 Variants of the Guillain-Barre
Syndrome
• 1.Fisher syndrome ★
• A syndrome comprising virtual or complete
ophthalmoplegia with ataxia and areflexia that probably
represents a variant of GBS was described by Fisher.
• 2. Acute Axonal Form of Guillain-Barre Syndrome
• widespread and severe axonal degeneration
• rapid evolution of polyneuropathy and very slow and
poor recovery.
 Laboratory Findings

• CSF examination
• protein-cell dissociation ★
• cell<10/mm3
• increase in CSF protein, a reflection of
the widespread inflammatory disease of
the nerve roots
•
• EMG
Prolonged distal latencies (reflecting distal
conduction block) and prolonged or absent
F-responses (indicating involvement of
proximal parts of nerves and roots) are
other important diagnostic findings, all
reflecting focal demyelination.

During a needle EMG, a needle electrode inserted directly

into a muscle records the electrical activity in that muscle.
A nerve conduction study, another part of an EMG,
uses electrode stickers applied to the skin (surface
electrodes) to measure the speed and strength of
signals traveling between two or more points.
 Pathogenesis and Etiology
• HIV, EBV or CMV, bacterial (particularly C.
jejuni) infections, induce such an
autoimmune response
• cell-mediated immunologic reaction
directed at peripheral nerve
• Autoantibodies detected in patients with
GBS, anti-GQ1b ,anti-GM1
 Differential Diagnosis
• acute spinal cord disease, marked by
sensorimotor paralysis below a defined
level and a marked sphincteric disturbance.
• Poliomyelitis, marked by fever,
meningoencephalitic symptoms, early
pleocytosis in the spinal fluid, and purely
motor and usually asymmetrical areflexic
paralysis.
 Treatment
• General medical aspects
• In severe cases, respiratory assistance
and careful nursing are paramount
• management of cardiovascular autonomic
instability
• Prevention of electrolyte imbalance
• pulmonary embolism in patients who are
bedbound
 Specific treatment ★
• plasma exchange

(Plasmapheresis)
• to remove
harmful
antibodies from
people’s plasma.
intravenous immunoglobulin (IVIG), 0.4
g/kg per day for 5 consecutive days
 Prognosis
• 90% patients recover nearly completely
• residual disability in 10%
Bell's Palsy
• occurs at all ages and all times of the year
• increased incidence in pregnant women
and diabetic
 viral agent
• HSV type I in the geniculate ganglion
• Varicella zoster virus (VZV) , isolated from patients with
the Ramsay Hunt syndrome
 symptomatology
• acute onset,attain maximum paralysis in
48 h and practically all within 5 days
• Pain behind the ear may precede the
paralysis by a day or two
• Unilateral paralysis of muscles supplied
by the affected nerve
• Impairment of taste is present to some
degree
• Hyperacusis in the ipsilateral ear indicates
paralysis of the stapedius muscle
• The Ramsay Hunt syndrome★, due
presumably to herpes zoster of the
geniculate ganglion, consists of a facial
palsy associated with a vesicular eruption
in the external auditory canal, other parts
of the cranial integument, and mucous
membrane of the oropharynx.
 prognosis
• Fully 80 percent of patients recover within a month or two.

• A poor prognosis for complete recovery is suggested by

severe pain at onset and complete palsy when the patient
is first seen.
 Treatment
• prednisone 40 to 60 mg/day ★
• antiviral agents may be useful , acyclovir
• Physical therapy
• Protection of the eye during sleep
• massage of the weakened muscles
• Acupuncture
 Differential diagnosis
• Bilateral Facial Palsy is most often a manifestation of the
Guillain-Barré syndrome (GBS)
Supranuclear Forms of Facial Paralysis

• contralateral
unilateral
lower facial
paralysis only
because the
upper face is
bilaterally
innervated
Trigeminal Neuralgia
(Tic Douloureux)
• higher for women than for men in a ration of 3:2
• The mean age of onset is 52 to 58 years
 Etiology

• Unclear
• In some cases, the pain is caused by pressure from a
small vessel on the root entry zone of the nerve
 Symptomatoloy★
(idiopathic)
• paroxysms of intense, stabbing pain in the distribution of the
mandibular and maxillary divisions
• lightninglike momentary jabs of excruciating pain occur and
spontaneously abate.
• The pain seldom lasts more than a few seconds or a minute or
two
• The paroxysms recur frequently, both day and night, for several
weeks at a time.
• trigger zones ★: a characteristic feature is the initiation
of a jab or a series of jabs of pain by stimulation of certain
areas of the face, lips, or gums, as in shaving or brushing
the teeth, or by movement of these parts in chewing,
talking, or yawning.
 Laboratory test
• MRI may reveal neurovascular contact of the trigeminal
nerve root
 Symptomatic （ secondary ） trigeminal
neuralgia
• due to involvement of the fifth nerve by some other
disease:
• multiple sclerosis (may be bilateral), aneurysm of the
basilar artery,
• tumor (acoustic or trigeminal neuroma, meningioma,
epidermoid) in the cerebellopontine angle.
• A, Benign tumors along the extraaxial course of the trigeminal root (arrows).
• B, Demyelinating plaques along the intraaxial course of the trigeminal afferents
(arrows).
 Treatment
• Carbamazepine★, 400–1200 mg/d po
• phenytoin, 200–400 mg/d
• Lamotrigine 400 mg/d or baclofen 10 mg three times daily
for refractory cases
• Posterior fossa microvascular decompressive surgery has
been used in drug-resistant cases
 Questions
• 1. which of the following does not belong
to the peripheral nerve system?
• A facial nerve
• B olfactory nerve
• C sciatic nerve
• D trochlear nerve
• E phrenic nerve
• In a patient with trigeminal neuralgia,which of the
following description is not correct?
• A. the pain happens and stops abruptly.
• B. The touch of mouth,lip,or nose may induce the onset
of the pain.
• C. Usually unilateral.
• D. Usually last for 1-2 hours.
• E. Tic douloureux may occur
• Which is the common feather of Bell palsy?
• A. Isolated unilateral facial weakness
• B. Pain
• C. Hyperacusis (a sensitivity to sound)
• D. Ageusia （ loss of taste ）
• E. Isolated bilateral facial weakness
• Which is of little benefit in the AIDP?
• A. Plasma exchange
• B. Intravenous immunoglobulin
• C. Corticosteroids
• D. Respiratory care
• E. Physical therapy
• Which has less importance for the diagnosis of PND?
• A. medical history
• B. physical examination
• C. blood tests
• D. EMG
• E. MRI
• Which is not one of pathologic type of PND?
• A.neuronal degeneration
• B. Wallerian degeneration
• C. Lewis’s body
• D. segmental demyelination
• E. axonal degeneration
Definitions
• Fisher syndrome
• trigger zone
• Ramsay-Hunt syndrome