Peripheral Neuropathy

Department of Neurology,
General hospital
Ningxia Medical University
Haining Li
 Peripheral Neuropathy
 Outline
 Guillain-Barre Syndrome
 Idiopathic facial palsy
 Trigeminal neuralgia
 OUTLINE
 Anatomy
 Pathological Processes
 Clinical classifation
 Symptoms
 Investigation
 Anatomy
Peripheral nerves are made up of numerous axons
bound together by three types of connective
tissue—endoneurium, perineurium, epineurium.
Peripheral nerve trunks contain myelinated and
unmyelinated fibres.
 Crinial nerves
 Spinal nerves
 Pathological Processes
 Wallerian degeneration: disintegration of
axons and myelin distal to the site of injury.
 Axonal degeneration: distal dying of axons
and loss of myelin.
 Neuronal degeneration: death of neuron body
 Segmental demyelination
Normal Wallerian Axonal Neuronal Segmental
degeneration degeneration degeneration demyelination
 Symptoms
 Sensory disturbance
 Motor deficits
 Tendon reflexes
 Autonomic disturbances
 Others
 Investigation
 Nerve conduction velocity(NCV)
 Electromyography(EMG)
 Nerve biopsy
 Blood tests
 CSF examination
Trigeminal Neuralgia
 Etiology and Pathology
 A facial pain syndrome of unknown cause.
 Demyelination
 Clinical Features
 It develops in middle to late life.
 It consists of severe paroxysms electric-
shock-like pain, usually in the V3 and V2
division of the trigeminal nerve, lasting for
several seconds or minutes each time.
 Involvement of V1 division or bilateral
disease occurs in less than 5% of cases.
 Clinical Features
 Occurrence during sleep is rare.
 Painfree intervals may last for minutes to
weeks, but long-term spontaneous remission is
rare.
 Sensory stimulation of trigger zones about the
cheek, nose, or mouth by touch, cold, wind,
talking, or chewing can precipitate the pain.
 Clinical Features

 Course could be periodic

 Physical examination is normal
Diagnosis and Differential Diagnosis
 Diagnosis
depending on the clinical features
 Differential Diagnosis
 Secondary trigeminal neuralgia
 Tooth ache
 Glosspharyngeal neuralgia
 Treatment
 Drug is preferential
AEDs: carbamazapine 0.1 bid po
phenitoin, clonazepam
 Baclofen
 VitaminB12
 Surgery
Idiopathic Facial Palsy
(Bell’s palsy)
 Idiopathic Facial Palsy
(Bell’s palsy)
 Anatomy
 Etiology and pathogenesis
 Clinical features
 Diagnosis and differential diagnosis
 Treatment
 Etiology and Pathology

 Etiology: unknown
 Pathology: edema, demyelination, axonal
degeneration
 Clinical features

 Onset
□ Occurs in any age, usually unilateral.
□ Paralysis: Progresses over 3 to 72 hours
□ Pain (50%): Near mastoid process
□ Excess tearing (33%)
□ Other: Hyperacusis; Dysgeusia
 Clinical features
 Signs
□ Facial weakness
Upper & Lower
Unilateral
Degree: Partial (30%); Complete (70%)
□ Stapedius dysfunction (33%): Hyperacusis
□ Lacrimation: mildly affected in some patients
□ Taste: No clinically significant changes in most
patients.
□ There should be no sensory loss in the face.
□ No abnormalities beyond the territory of the facial
nerve.
□ Hunt syndrome: caused by reactivation of herpes zoster
in the geniculate ganglion, consists of a severe facial palsy
associated with a vesicular eruption in the external
auditory canal and sometimes in the pharynx and other
parts of the cranial integument; often the eighth cranial
nerve is affected as well.
 Clinical features
 Prognosis better
□ Incomplete paralysis

□ Early improvement

□ Slow progression

□ Younger age
 Clinical features

 Prognosis better
□ Normal taste
□ Electrodiagnostic tests normal
Diagnosis and differential diagnosis
 Diagnosis: acute onset with peripheral facial
palsy.
 Differential diagnosis:
 GBS
 Treatment
 Corticosteroids: prednisone 60mg qd po
dexamethason
 VitaminB1,B12
 Baclofen
 Rehabilitation
 Physiotherapy
Guillain-barré Syndrome
GBS is a kind of autoimmune disease
characterized by demyelination of peripheral
nerves and nerve roots. Inflammation of
lymphocytes and macrophages around small
vessels is the pathological feature.
Clinical classification
 AIDP
 AMAN
 AMSAN
 Fisher syndrome ( gait ataxia, areflexia,
and external ophthalmoplegia)
 Unclassifiable GBS
 Epidemiology

 Incidence: 0.6 to 1.9/100,000/year

 Male: Female = 1.25: 1
 Peak ages: 16~25 years old
45~60 years old
 Etiology and pathogenesis
 The precise cause is unclear.
 GBS often follows minor infective illness,
inoculations or surgical procedures.
Clinical and epidemiologic evidence
suggest an association with preceding
Campylobacter Jejuni(CJ) infection.
 The pathogenesis resembles EAN
Molecular mimicry
 Clinical features
 GBS Prodrome
It often follows 1-4 weeks after a respiratory
infection or diarrhea.
Campylobacter jejuni(CJ) has been particularly
implicated as a cause of the diarrhea.
 Clinical features

 Weakness: Most often symptomatic in legs

Distribution: Proximal + Distal; Symmetric
Severity: Quadriplegia in 30%;
Bedbound another 30%
Respiratory failure.
 Clinical features
 Sensory: ususlly less marked than motor
symptoms.
Paraesthesias: Initial symptom in 50%;
Eventually occur in 70% to 90%
Pain
Loss: with classic glove-and-stocking pattern
of sensory loss, but rarely occurs.
 Clinical features

 Cranial nerve: Ⅶ, Ⅸ,Ⅹ

facial weakness is present in 50% of cases.
 Autonomic dysfunction
tachycardia, cardiac irregularitis, labile blood
presure, disturbed sweating and so on.
 Monophase course
 Investigations
 CSF: a characteristic abnormality, with increased
protein concentration but a normal cell count.
 Eletrophysiologic studies
 marked slowing of motor and sensory condu-
ction velocity,
 evidence of denervation and axonal loss.
 F wave reflex is delayed or absent.
 Sural nerve biopsy: demyelination
Diagnostic criteria for GBS(1)

Acute or subacute progressive weakness of

more than one limb.
Distal areflexia with proximal areflexia
or hyporeflexia.
Progression for up to 4 weaks.
Relatively symmetric deficits.
Mild sensory involvement.
 Diagnostic criteria for GBS(2)
Cranial nerve(especiallyⅦ)involvement.
Recovery beginning within 4 weeks after
progression stops.
Autonomic dysfunction.
Increased CSF protein after 1 week.
CSF white blood cell count10/l.
Nerve conduction slowing and F wave reflex
delayed or absent.
 Treatment
 Assisting respiration: Patients who are seve -
rely affected are best managed in ICU where
facilities are available for monitoring and assis-
ted respiration if necessary. Sometimes
antibiotic is necessary for preventing
respiratory tract’s infection.
FVC<15ml/kg, PO2<70mmHg
 Symptomatic therapy: The aim is to prevent
such complications as respiratory failure or
vascular collapse.
 Treatment
 Etiological therapy:
Plasma exchange ( plasmapheresis)
Intravenous immunoglobulin:
0.4g /kg /d for 5 days
Corticosteroids: it has not been successful
in acute GBS and can bring about adverse
outcome.
 Rehabilitation
 Prognosis
 The disorder is self-limiting,and improvement
occurs over the weeks or months following
onset. About 70-75% of patients recover
completely, 25% are left with mild neurologic
deficits, and 5% die, usually as a result of
respiratory failure.
 The prognosis is poorer when there is
evidence of preceding CJ infection.
 Chronic inflammatory
demyelinating polyneuropathy
（CIDP）
Epidemiology
 Occurs at any age, peak time is 40-60 years old
adults.
 Clinical Manifestations

Chronic, relapsing inflammatory

polyradiculoneuropathy.
Weakness and areflexia are characteristic signs.
Course is more than 2 months.
Treatment
 Steroids
 IVIg
Thank you