PERIPHERAL NERVE DISORDER

Dr. Roslaini

Departement of Neurology
Al ISLAM Hospital
Bandung

The distribution of peripheral nerve disorder includes :

Spinal nerve anterior Horn med spinal

Distal peripheral nerve

Cranial nerve
Otonomic nerve

Peripheral nerve spread out in all of body

disorder

Characteristic lower motor neuron

Paralysis
depend

location

degree

fiber nerve

Onset

Pathology reaction of peripheral nerve

Neuropathy
It is condition where perifer nerve gets dysfunction and distruction.
Clinical manifestation of lower motor neuron paralysis included :
Sensory
Negative phenomenon :
Hypestesia, the loss of sense, pain, temperature and deep
sense
Positive phenomenon :
paresthesia
hiperalgesia
incomportable, burning sensation
Clinical symptom :
Distal gloves stocking hypesthesia
Dermatomel

Neuropathy ( contd )

Motoric
Weakness from light to severe especially distal
Loss of tendon reflex
Autonomic
Example :
Cool on limb
Postural syncope
Orthostatic hypotension
Hyperhidrosis

Principal Neuropathic Syndrome

I. Syndrome of acute motor paralysis with variable
disturbances of sensory and autonomic function
Guillain Barre Syndrome ( GBS )
II. Syndrome of sub acute sensorimotor paralysis
A. Symmetrical polyneuropathies
Vitamin deficiency
B. Unusual sensory neuropathies
Diabetic neuropathies
C. Polyradiculopathy

Principal Neuropathic Syndrome

III. Syndrome of chronic sensorimotor polyneuropathy
A. Less chronic, acquired forms
CIDP
B. Syndrome of more chronic polyneuropathy
Inherited polyneuropathy
C. Inherited polyneuropathy of mixed sensorimotor
types
IV. Neuropathy associated with mitochondrial diseases
V. Syndrome of recurrent or relapsing polyneuropathy
VI. Syndrome of mononeuropathy or plexopathy

Contd

Approach
Peripheral nerve disorder
LMN

Polyneuropathies

Guillain Barre Syndrome

Vitamin deficiency
amyloidosis
metabolic

Mononeuropathies

Acute
Subacute
chronic

Trauma
Pressure: CTS
Leprosy

Acute idiopathic polyneuropathies

( Guillain Barre Syndrome )
Etiology : - unknown
- post viral infection
- self immunity desease
Pathology :
Demyelinitation segmental
Severe axonal degeneration

Clinical symptoms

Non spesific viral infection

Acute paralysis progresif bilateral symetric
Autonomic disorder
Cranial nerve disorder
Sensoric abnormality : gloves and stocking
hypesthesia

Diagnostic test :
Lumbal puncture : elevated cerebrospinal fluid protein
NCV - EMG
Criteria to make a diagnosis of GBS :
Rapid onset
lead to frank paralysis : Leg and arms
Sometimes respiratory muscle
Accompanied by sensoric abnormalities
Loss of deep tendon reflex / decrease
Elevated cerebrospinal fluid protein, and normal cell
Absence of other causes of peripheral neuropathy

Prognosis :
75 85 % spontaneus recovery
10 17 % recover with disability
8 % recurrent
5 % died
Therapy :
Self limiting
Corticosteroid
Immunoglobulin
Plasmapharesis

Diabetic Neuropathy
Complications of diabetes mellitus
Insidens 30 % - 70 % of DM patient

Definition :
A disorder somatic or autonomic perifer
nerve. Cause only by diabetes mellitus can
happen clinically or sub clinic without other
causes

Pathogenesa
Vascular
Metabolik
Other
Clinical features
Diabetic opthalmoplegia
Acute mononeuropathy
Rapidly evening painfull, asimetric, predominantly
motoric
Multiple neuropathy
Asimetric proximal motor weakness
Distal symetric, primarily sensory polineuropathy
Autonomic neuropathy
Painfull thoraco abdoment radiculopathy

Diagnostic test
Blood glucose level test
Nerve conduction study : NCV
Therapy
Maintenance of the blood glucose level
Microangiopathy antiplatelet agregasi ASA
Symptomatic theraphy for neuropathic pain
Psikotherapy, physiotherapy

Nutritional polineuropathy
Etiology :
Deficiency vitamin in daily food
Tight diet
Malabsorption
Post operatif GIT
Alcoholic
Patology anatomy feature : axonal degeneration

Clinical features

Sensoric :
Paresthesia, hyperalgeria, hyperaesthesia
Motoric : Paralysis
Fisiologi reflex decrease
Sistemic disorder : delirium anemi, etc

Diagnostic test
Serum level of vitamin B1, B6, B12, Folic acid
Electroneuromyography : NCS Nerve Conduction Study
NCV
Therapy
Vitamin suplement administration vitamin B1, B6, B12

Mononeuropathy
Radial nerve
Arises from the C. 5 8
Motor nerve innervated : the chief extensor of the
fore arm, wirst, and finger
Cause by external pressure in axilla and fracture
humerus
Pressure against some hard surface especially in
sleep : saturday night palsy

Radial Nerve

Median nerve
Arises from the C. 5 6 7 8 th I
Its fairly common as a result of compression of the
nerve by transverse carpal segment ( Carpal
tunnel syndrome / CTS )
The symptoms : severe pain often weakly patient
from sleep, usually in the thumb and index finger
Athrophy M. Thenar
Trauma axilla area ape hand

Median Nerve

Ulnar nerve
Arises from the C7 8 th I
It is frequently injured by gunshot, wound in
olecranon or head radius bone, most compressed
at elbow, leprosy, diabetic neuropathy
Clawn hand its on the ring finger

Ulnar Nerve

Sciatic nerve
Derived from the L 4 5 S1
Its commonly by fracture of pelvis or femur, wound of
the buttock and thigh.

In fossa poplitea the nerves devides :

Common perineal nerve
Tibial nerve

Sciatic nerve

Common perineal nerve

Most frequent cause of injury, pressure,
obstetrical, prolonged lying the ill patient.

Common Peroneal
Nerve

Tibial nerve
Lession in tibial nerve are uncommon
Caused by trauma, and compression in tarsal tunnel

The brachial plexus

Formation of brachial plexus :
Upper : from C5 6
Middle : from C7 8
Lower : from C8 Th. 1
Causes the lession brachial plexus :
Cut
Gunshot
Wound
Direct trauma
Tumor or aneurysm
Traction in delivery at birth

The brachial plexus

Site of the lession

Upper brachial syndrome ( Erb Duchene
Syndrome )
Middle brachial syndrome
Lower brachial syndrome ( Klumpke deyerine
syndrome

Common root syndromes of invertebral disc disease

Disc space
Root affected

L3 4

L4 5

L5 S1

L4

L5

S1

Muscles affected

Quadriceps

Peroneals;
anterior tibial;
extensor
hallucis
longus

Gluteus
maximus;
gastrocnemius;
plantar flexors
of toes

Area of pain and

sensory loss

Anterior thigh,
medial shin

Great toe,
dorsum of
foot

Lateral foot,
small toe

Reflex affected

Knee jerk

Posterior
tibial

Ankle jerk

Straight leg
raising

May not
increase pain

Aggravates
root pain

Aggravates
root pain

Common root syndromes of invertebral disc disease

Disc space
Root affected

C4 5

C5 6

C6 7

C7 T1

C5

C6

C7

C8

Muscles
affected

Deltoid; biceps

Triceps; wrist
extensors

Intrinsic
hand
muscles

Area of pain
and sensory
loss

Shoulder, anterior
arm, radial
forearm

Thumb, middle
fingers

Index,
fourth, fifth
fingers

Reflex affected

Biceps

Triceps

Triceps

Myopathy
Diseases concerning with sceletal muscles
Symptoms of skeletal muscle disease :
Falique
Weakness
Atrophy
Muscle tic
Cramps
Painfull

Classification muscle diseases

The inflammatory myopathies
Polymyositis
The muscular dystrophies
Duchene muscular dystrophy
The metabolic and toxic myopathies
The congenital neuromuscular disorders
The hereditary myotonias and periodic paralysis
Disorders of muscle characterized by cramp,
spasm, pain and localized masses

Diagnosis of muscle disease

Clinical symptoms
Hereditary
Laboratory examination
Electromyography
Nerve conduction study
Muscle biopsy

Muscle distrophy

Duchene muscular dystrophy

Inherited muscle disease, degeneratif and progresif
Similar x linked disease
Atrophy shortage

Clinical manifestation
Mostly find on children under 3 years age
Usually boy
The story late to walk often fallen, cant run
difficult to stand up from the squad position
( Gowers sign ) finally paralysis

Physical diagnostic
Atrophy on proximal muscle calf muscle
Pseudo hypertrophy
Progresifity variety
Fatal cases respiratory muscle failure and
secondary infection

Diagnostic test
Increases the CPK level
EMG, classical myogen feature
Muscle biopsy

Therapy
No medication for disease
Physiotherapy
Genetic conseling

Polymyositis
Disorders of sceletal muscle
Onset acute / subacute
PA infiltration of muscle by lymphocyt
Etiology
Unknown idiopathic polymyositis / autoimmun
Collagen vascular disease
Infection
Drug
Systemic disease
Endocrin disease
Metabolic disease
Toxic

Clinical manifestation
No family history
Progressition is measured in week / month
Symptom may improve spontaniously
Dysphagia, weakness neck / flexor muscle
Arthralgia, myalgia

Diagnostic test
EMG ; myopathy feature

Therapy
Corticosteroid and immuno supresif drug
Immunoglobulin intravenous

Myasthenia Gravis
Autoimmun disease
Causes by transmission disorders on neuro muscular
junction
Acetyl cholin receptor ist attack by antibody
Related with thymus gland

Clinical features
Fluctuated weakness of certain voluntary muscle
Viral, other infection, emotional, can worst the
symptom
The symptom response to anticholin esterase drug
Sporadically to all ages
Tendon reflex normal
No sensory deficit

Classification
1. Ocular myasthenia : 20 % cases
2. A. Mild generalized myasthenia
- Slow progression
No crisis drug respons
B. Moderate generalized myasthenia
- Severe secletal and bulbar involment
- No crisis drug respon less than statisfactory
3. Acute fulminating myasthenia
- Rapid progression of severe symptom
- Respiratory crisis high mortality
4. Late severe mysthenia

Diagnostic test
Tensilon test or prostigmin test
Measurement of receptor antibody for acetylcholine
Repetitive stimulating test : progressif decrement
Single fiber electromyography
X ray, CT Scan, MRI, for thymoma gland
Differential diagnostic :
Eaton lambert syndrome
Follow lung carcinoma
Myasthenia gravis related with drug, toxin
Therapy :
Anticholine esterase drug prostigmin
Cortico steroid
Thymectomy
Plasma pharesis and immunosupressan
Immunoglobulin

BIOPSY

Dr Roslaini

DEPARTEMENT OF NEUROLOGY
AL ISLAM HOSPITAL
Bandung

Biopsy
Biopsy done if no other diagnostic test
Clinical test is not suffition for disease
Supporting the diagnostic when biochemistry
test is positive
Neurology diagnostic biopsy done on the
primary tissue of skin, sceletal muscle, nerve,
bone marrow, and brain.

Skin biopsy
Storage disease
Neuroaxonal dystrophy
Mythochondrial cytopathies

Sceletal muscle biopsy

On the normal and abnormal muscle area
Primary muscle disease
Denervation disease
Systemic disease
Mitochondria disease
Nerve biopsy
Diagnostic chronic neuropathy
Inherrited neuropathy
Sural nerve is common

Bone marrow biopsy

Nieman pick disease
Rectum biopsy
Autonomic nerve disease
Amyloid disease
Brain biopsy
An infasive test / high risk procedure
No other diagnostic test
Scar biopsy can be epileptic focus
Its done for Alzheimer disease, levy body disease, brain
tumor, infection