PERIPHERAL NERVE DISORDER

Nani Kurniani

Faculty of Medicine Padjadjaran University / Dr. Hasan Sadikin General 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

Contd

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

Approach
Peripheral nerve disorder LMN

Polyneuropathies

Mononeuropathies

Guillain Barre Syndrome Vitamin deficiency amyloidosis metabolic

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 L4 5 L5 L5 S1 S1

Muscles affected

Quadriceps

Peroneals; anterior tibial; extensor hallucis longus

Great toe, dorsum of foot

Gluteus maximus; gastrocnemius; plantar flexors of toes

Lateral foot, small toe

Area of pain and sensory loss

Anterior thigh, medial shin

Reflex affected
Straight leg raising

Knee jerk
May not increase pain

Posterior tibial
Aggravates root pain

Ankle jerk
Aggravates root pain

Common root syndromes of invertebral disc disease

Disc space Root affected Muscles affected

C4 5 C5

C5 6 C6

C6 7 C7 Triceps; wrist extensors

C7 T1 C8 Intrinsic hand muscles Index, fourth, fifth fingers Triceps

Deltoid; biceps

Area of pain and sensory loss Reflex affected

Shoulder, anterior arm, radial forearm Biceps

Thumb, middle fingers

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

Nani Kurniani

Faculty of Medicine Padjadjaran University / Dr. Hasan Sadikin General 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