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Page 1: Neuropathy complete2

Neuropathy

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Anatomy

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-Anterior horn cell

-Motor neuron

-Ventral root

-Dorsal root ganglion

-Dorsal root

-Plexus

-Peripheral nerve

-Axon

-Myelin sheath

-Neuromuscular junction

-Muscle

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Thoracic vertebra

Lower lumbar vertebra

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Nerve Anatomy

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Motor SystemMovement commands are generated by the brain and transmitted to muscles through the spinal cord and nerves.

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Sensory System

Many types of sensors receive information and send it to the brain through the nerves

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Basic Nerve Elements Neuron Synapse Motor Unit Endplate (Neuromuscular Junction) Motor and Sensory Nerves

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Neuron The neuron (nerve cell) is the basic unit of the nervous system.

A neuron consists of a cell body, axon (nerve fiber), etc. Electric impulses are generated by the neuron and travel on the

axon. The impulse travels faster where the axon is covered by myelin.

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Synapse

Synapse

The synapse is the neuron to neuron junction. Signal processing is done at the synapse.

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Motor Unit

Muscle fiber

DendritesAxon

MyelinMuscle fiber

Nucleus Soma Endplate

The motor unit consists of an anterior horn cell, its axon, and all the muscle fibers innervated by that axon and its branches.

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Endplate (Neuromuscular Junction)

When impulses reach the endplate, the muscle fibers contract.

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Motor and Sensory Nerves

A motor neuron connects to muscle fibers (other cells). A sensory neuron has a sensor, but it is part of the same cell.

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Conduction Velocity There are several types of nerves. A nerve consists of many fibers (axons). The velocity of myelinated (covered with myelin)

nerve fiber is higher than non-myelinated fiber. Conduction velocity is higher in thicker nerve fiber

than thinner nerve fiber. Each fiber has its own velocity, different than other

fibers. The higher the temperature, the higher the velocity.

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Nerve Types, Diameter and Conduction Velocity

The thicker the nerve, the higher the conduction velocity.

Sensory receptorProprioceptor

of skeletal muscle

Pain, temp.

Pain, temp, itch

Mechanoreceptors of

skin

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Comparison of Conduction Velocities

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Definition Peripheral neuropathy :

โรคที่��เกิดขึ้� นกิ�บระบบประสาที่ส�วนปลาย ซึ่��งได�แกิ�neuronal cell, axon และ myelin sheath ที่��หุ้��มaxon

Neuronopathy : ม�ความผิดปกิติหุ้ร#อพยาธิที่��ติ�วเซึ่ลล'ที่(าใหุ้�ม�อากิารที่าง

motor หุ้ร#อ sensory อย�างใดอย�างหุ้น��งRadiculopathy : ม�พยาธิสภาพที่�� root ม�กิจะเป,น 1-2 root ส�วนใหุ้ญ่�

เกิดจากิกิารกิดที่�บขึ้อง herniated disc หุ้ร#อosteophyte

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Definition (ติ�อ)Polyradiculopathy : ม�พยาธิสภาพที่�� root หุ้ลายๆ เส�น ม�กิเกิดจากิภาวะ inflammation

Polyradiculoneuropathy : ม�พยาธิสภาพที่� งที่�� root หุ้ลายๆ เส�น และ

peripheral nerve Plexopathy :

ม� พยาธิสภาพเกิดที่�� brachial หุ้ร#อ lumbosacral plexus

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Polyneuropathy : พยาธิสภาพขึ้อง peripheral nerve หุ้ลายๆ เส�น

ม�กิ symmetrical และ อากิารจะเร�มที่��เที่�าและขึ้า กิ�อนจะลามมาถึ�งม#อและแขึ้นMononeuropathy : พยาธิสภาพเกิดที่�� nerve เส�นใดเส�นหุ้น��ง ม�กิเกิด

จากิ entrapment หุ้ร#อ local trauma ที่�� nerve น� นMononeurities multiplex : พยาธิสภาพเกิดที่�� nerve แติ�ละเส�นที่��แขึ้นขึ้า หุ้ร#อจากิ

แขึ้นหุ้ร#อขึ้าไปย�งแขึ้น หุ้ร#อขึ้าอ�กิด�านหุ้ น��งหุ้ร#อด�านเด�ยวกิ�นล�กิษณะเป,น

patchy ม�กิพบในรายที่�� เป,นจากิ vascular ค#อ vasa nervorum ม�พยาธิ

สภาพ

Definition (ติ�อ)

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Neuritis : อ�กิเสบขึ้องเส�นประสาที่Plexitis : อ�กิเสบขึ้อง brachial หุ้ร#อ

lumbosacral plexusDemylinating neuropathy : พยาธิสภาพเกิดที่�� myelin sheath กิ�อนAxonal neuropathy : พยาธิสภาพเกิดขึ้� นที่�� axon กิ�อน

Definition (ติ�อ)

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Polyneuropathy

Poly(radiculopathy)

Neuronopathy

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Autonomic neuropathy : ม�พยาธิสภาพขึ้องเส�นประสาที่อ�ติโนม�ติ ที่� ง sympathetic และ

parasympathetic พบร�วมกิ�บ diabetic neuropathy, Guillain Barre syndrome และalcoholic polyneuropathy ได� หุ้ร#อม�พยาธิเฉพาะในระบบ

ประสาที่อ�ติโนม�ติกิ4ได� (dysautonomia) Pure motor neuropathy

(predominonthy motor polyneuropathy):

ม�พยาธิสภาพจะอย5�ที่�� motor nerve เป,นส�วนใหุ้ญ่� sensory จะม�น�อยหุ้ร#อไม�ม�เลย จะแยกิจากิพวกิที่��ม�พยาธิสภาพที่�� anterior

horn cell (motor neuronopathy) ได�ยากิ

Definition (ต่�อ)

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Definition (ติ�อ)Pure sensory neuropathy (predominantly

sensory polyneuropathy) : ม�พยาธิสภาพจะอย5�ที่�� sensory nerve เป,นส�วนใหุ้ญ่�

-1. Large afferent fiber – impaired vibration, propioception areflexia, sensory ataxia

- 2. Small afferent fiber – numbness, hypoesthesia to pin prick and temperature, painful burning, dysesthesia

- 3. Pansensory-- 1+2 อาจเป,นจากิ sensory neuronopathy

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Symptoms of neuropathy

Pain - burning, short jabs , tight or band like pressure, painful

hypersensitivity of non noxious stimuli

Paresthesia “pain and needle” sensationsSensory loss “ชา” “no sensation” “like block of

wood”Weakness - distal symmetrical weakness

- proximal symmetrical weakness – GBS - unilateral limb weakness

- brachial plexus- lumbosacral plexus

Unstable balance - sensory loss - weakness in the legs

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CPK level

Electromyography

Duchenne’s muscular dystrophy

Polymyositis Rhabdomyolysis

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NCS and Peripheral Neuropathy

NormalMCV SCV

Demyelination

Axonal degeneration

Prolonged latency, duration, and low amplitude

Low amplitude, but no delay of latency

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Neuronopathy

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Poliomyelitis

Anterior horn cell disease

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Poliomyelitis

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Motor Neuron Disease

Werdnig-Hoffman disease

Kugelberg-Welander disease

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A 55 year-old male presents with 2- year history of progressive weakness in both hands. He denies any numbness or abnormal sensation.

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Tongue Fasciculation

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Cardinal Features of Amyotrophic Lateral Sclerosis (ALS)

•Mixture of UMN & LMN signs

•No sensory deficit

•Progressive course

•No sphincter muscle or ocular muscle involvement

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Dorsal root ganglia lesion

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Herpes zoster of thoracic dermatome

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Neuropathy from History and physical exam

Mononeuropathy Mononeuropathy multiplex Polyneuropathy

axonal demyelinatingEntrapmentDMSubclinical polyneuropathy

VasculitisDM(rare)

HNPPMMNCIDP(rare)

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Neuropathy from History and physical exam

Mononeuropathy Mononeuropathy multiplex Polyneuropathy

axonal demyelinating

DMToxicMetabolicNutritional deficiencyParaprotein emiaCAidiopathic

Hereditary

subacute chronicacute

GBS(axonal)Porphyria

acute subacute chronic

GBSDiphtheria

CIDPParaproteinemia

Hereditary

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Axonopathy

Demyelination

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Polyneuropathy: Axonal type

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-Metabolic

-Toxic or drug

-Nutritional deficiency

Polyneuropathy caused by

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Mees’ line in arsenic

poisoning

Arsenic

Thallium Lead

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Basophilic stippling in Lead poisoning

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Classification of Diabetic neuropathies

Symmetric 1. Distal, primarily sensory

polyneuropathy a. Mainly large fibers affected b. Mixed (a)

c. Mainly small fibers affected (a)

2. Autonomic neuropathy 3. Chronically evolving proximal motor

neuropathy (a,b)

Asymmetric 1. Acute or subacute proximal motor

neuropathy (a,b)

2. Cranial mononeuropathy (b)

3. Truncal neuropathy (a,b)

4. Entrapment neuropathy in the limbsa Often painful.b Recovery, partial or complete, is likely.

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Diabetic Radiculopat

hy

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Amyloid neuropat

hy

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Polyneuropathy :

Demyelinating type

weakness

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- Guillain Barre Syndrome Most common type --- Acute inflammatory demyelinating

polyneuropathy (AIDP)

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- Diagnostic Criteria for Guillain Barre Syndrome

REQUIRED

1 2. Progressive weakness of or more limbs due to neuropathy 2. Areflexia

3. <4Disease course weeks - llllll lllllllll lllllllllll llllllllll lllllllll llllll llll ll lllll llllll lllll4. [..,( ,, ) ,(, ),, ,,

ome]

SUPPORTIVE

1. Relatively symmetric weakness 2. Mild sensory involvement 3. Facial nerve or other cranial nerve involvement 4. Absence of fever

lllllllllll llllllll ll lllllll llllll5. (,) llllllllllllllllll llllllll ll lllllllllllll6.

a Excluding M. Fisher and other variant syndromes.

SOURCE: Modified from AK Asbury, DR Cornblath: Ann Neurol 27:S21, 1990

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Scattered distribution of sensory loss in

Multiple Mononeuropat

hy

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Multiple mononeuropathy Can found in: Classic PANChurg-Strauss diseaseWegener’s granulomatosisOverlap syndromeVasculitis associated with connective

tissue diseaseSjóģren syndromeLyme diseaseLeprosyDiabetes mellitus

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Multiple mononeuropat

hy with vasculitis

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Leprous neuritis (infectious cause of multiple mononeuropathy)

Mycobacterium leprae – coolest tissue in the

body

Tuberculoid (high-resistance) leprosy – single patch

of hypoesthesia or anesthetic skin in any location

Lepromatous (low resistance) leprosy – numerous

bacilli, wide spread skin thickening, cutaneous

anesthesia, anhydrosis sparing axilla, groin and

skin beneath the scalp hair

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Leprosy (Hansen’s disease)

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Neuropathies with HIV infection

Seroconversion - Guillain Barre syndrome

- Chronic inflammatory demyelinating polyneuropathy (CIDP)

Symptomatic stage - mononeuritis multiplex axonal type

subacute or chronic Late symptomatic stage - distal symmetrical sensory polyneuropathy, most

common neuropathy frequently coexists with symptomatic encephalopathy and myelopathy - toxic polyneuropathy - subacute asymmetrical polyneuropathy of cauda

equina, caused by cytomegalovirus

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Advanced stage of diffuse sensory

neuronopathy

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Hereditary Motor-Sensory type I

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Hereditary Motor-Sensory type I

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Hereditary Motor-

Sensory type III

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Hereditary Sensory Neuropat

hy

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Peripheral neuropathy by clinical course

Acute onset (within day) Guillain-Barré syndromeAcute intermittent porphyriaCritical illness polyneuropathyThallium toxicity

Subacute onset (weeks to months)

Toxins or medicationsNutritional deficiencyMetabolic abnormalityParaneoplastic syndromeCIDP

Chronic course (years) Hereditary motor and sensory neuropathy (HMSN)Inherited sensory neuropathyCIDP

Relapsing/remitting course

Guillain-Barré syndromeCIDPHIV/AIDSToxin (intermittent exposure)Porphyria

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Radiculopathy

Cervical

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Cervical radiculopathy

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Lumbar disc herniation

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Clinical features of herniated L4 and L5

nucleus pulposus

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Sensory impairment related to level of

spinal cord

injury

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Normal Dermatomes

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Dermatome1. No C1 dermatome2. C4 and T2 dermatome are contiguous on trunk3. Thumb, middle finger, and fifth digits are

innervated by C6, C7, and C8, respectively4. Nipple is at T4 level5. Umbilicus is at T10 level6. Lumbar and sacral dermatome are contiguous

in the posterior axial line of leg

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Segment pointer musclesRoot Muscle Primary function

C3 Diaphragm Respiration

C4 Diaphragm Respiration

C5 Deltoid Arm abduction

C5 Biceps Forearm flexion

C6 Brachioradialis Forearm flexion

C7 Triceps Forearm extension

L3 Quadriceps femoris Knee extension

L4 Quadriceps femoris Knee extension

L4 Tibialis anterior Foot dorsiflexion

L5 Extensor hallucis longus Great toe dorsiflexion

S1 Gastrocnemius Plantar flexion

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RadiculopathyCauses

Compressive : herniated disc, spondylosis, tumor

Infiltrative : tumor seeding, infection

Inflammatory : immune-mediated

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PlexopathyBrachial plexusLumbosacral plexus

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Brachial Plexus

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Trunk Root

DivisionCordNerve

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Brachial Plexus

Root : C5-C8, T1Trunk : Upper, middle, lowerDivision : 3 anterior and 3 posteriorCord : lateral, posterior, medialNerve : musculocutaneous,

median, axillary, radial, ulnar

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Long thoracic nerve to serratus anterior

Dorsal scapular n. to rhomboids

Thoracodorsal nerve to latissimus dorsi

Ulnar n.Radial n.

Axillary n.

Median n.

Musculocutaneous n.

Suprascapular n.

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Brachial plexus Symptoms and signs

Sensory : acute pain in shoulder and arm, deep and

nonlocalizedParesthesiaSensory loss, when existed, will be limited to outer

surface of arm and radial aspect of forearmMotor

Weakness of shoulder girdle, deltoid, spinati, biceps and triceps

Atrophy of affected muscle

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Brachial plexusN.ที่�แยกก�อนเข้�าplexus

Dorsal scapular nerve : to rhomboidLong thoracic nerve : to serratus

anterior

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Long thoracic nerve to serratus anterior

Dorsal scapular n. to rhomboids

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Anatomy Lateral cord

Posterior cord

Medial cord

musculocutaneous nervemedian nerve

axillary nerveradial nerve

median nerveulnar nerve

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Main sites of brachial plexus damage

1.upper trunk : ถู�กย�งหรื�อแที่งที่�คอ

C5,6 destroyed Numbness : lateral arm, forearm, hand Weakness : abduct, internal and external rotate

of shoulder, elbow flexion, radial wrist extension Bicep and supinator jerk absent Spare rhomboid and serratus anterior

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Main sites of brachial plexus damage

2.lower trunk :cervical rib syndrome, CA lung apex (Pancoast’s syndrome), metastatic disease in axillary gland

Pain : in shoulder at night time Tingling and numbness : medial arm,

forearm, little and ring finger Weakness : finger flexor and extensor,

intrinsic m. of hand

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Main sites of brachial plexus damage

3.radial lesion in axilla : crutch injury, stab wound, neoplastic disease in axillary gland

Weakness : radial nerve innervated muscle including triceps

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Main sites of brachial plexus damage

4. posterior cord : small caliber low-velocitiy bullet wound to plexus

Radial nerve palsy and axillary nerve lesion Weakness : elbow, wrist, finger extension,

second 90 of shoulder abduction(deltoid paralysed)

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Radiation damage to plexus

มั�กโดน C7 มัากสุ�ด เพรืาะเป็#น center of radiation field C8,T1 โดนน�อยกว่�า มั�กเก�ด - 1218 เด�อนหลั�งradiation Numbness and weakness of forearm and hand NO PAIN Symptom progress over a few weeks

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Causes of brachial plexopathy

TraumaTumor infiltrationInfection by viralImmune-mediatedDelayed effects of radiotherapy

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Traction birth injury

(Erb’s palsy)

Acute pain in back of shoulderPostmastectomy

and radiation

Brachial plexopathy

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Brachial plexus

Upper C5,6 or Erb-Duchenne type Lower C8, T1 or Dejerine-Klumpke type Total

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Klumpke’s palsy (injury

of lower brachial plexus

C7,C8,T1) and often Horner’s

syndrome

Erb’s palsy (injury

of upper brachial plexus C5,C6)

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Lumbosacral plexus

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Lumbosacral Plexus

Common peroneal n.

Tibial n.

Sciatic n.

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Lumbosacral plexusMultiple spinal roots and peripheral

nerves ที่�ข้าRoots : T12-S4Nerves : iliohypogastric, ilioinguinal,

genitofemoral, lateral femoral cutaneous, femoral, obturator, superior gluteal, inferior gluteal, sciatic, posterior femoral cutaneous, pudendal nerves

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Lumbar plexopathy

Sacral plexopathy

Clinical manifestation

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Causes of lumbosacral plexopathyTumors : CA cervix, prostate, bladder,

colorectal, kidney, breast, testis, ovary, sarcoma, lymphoma

Compressed by aortic aneurysmRadiation plexopathyPlexitis : follow herpes zostorDiabetic amyotrophyTrauma (rare)As a manifest of mononeuropathy multiplex

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Common Mononeuropathies

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Axillary nerve

Deltoid

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Musculocutaneous nerve

Biceps

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Median nerve

Carpal tunnel syndrome

Anterior interosseous syndrome

Pronator syndrome

Ligament of Struthers

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Median nerveCarpal tunnel syndrome

ชาป็ลัายน�'ว่Nocturnal pain or paresthesiaThenar atrophy

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AtrophySensory loss

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Ulnar nerve

Lesion at condylar groove

Lesion at wrist and handGuyon’s canal

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Ulnar nerve At elbow : Condylar groove or in cubital

tunnel ชาน�'ว่ 4+5+มั�อด�านmedial Wasting hypothenar eminence and

web space of น�'ว่โป็(ง+ ช�' (1st dorsal interossei)

Claw hand

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Radial nerve

Saturday night palsy

Posterior interosseous syndrome

Cheiralgia parestheticaCheiralgia paresthetica

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Radial nerve Axillary lesion : weak triceps and radial innervated

m. Mid-upper arm lesion : ‘Saturday night palsy’

(spiral groove or intermuscular septum) : wrist drop, normal triceps, variable motor and sensory deficit

Posterior interosseous : weak extensor of thumb and other fingers, no sensory loss

Superficial radial n. : terminal cutaneous br.

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Radial nerve

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Wrist drop

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Lateral femoral cutaneous nerve of thigh (L2 and L3)

• Meralgia paresthetica

• Pure sensory

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Femoral nerve

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Femoral nerve (L2,3,4)

• Mix sensorimotor• Quadriceps femoris or knee extensor• Weakness of hip flexor in intraabdominal lesion• Sensory deficit over anteromedial aspect of thigh and perhaps leg• Absent or diminished knee jerk

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Obturator nerves

• L2,3,4

• Hip adductors

• vulnerable during obstetric and gynecological procedures

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Sciatic

Sciatic nerve

•L4-S3

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Sciatic nerve

Composed of 2 main nerves of leg : common peroneal and tibial nerve

Paralysis of all muscles below knee plus hamstrings and for high lesion, external rotators of thigh

Sensory loss below knee except anteromedial aspect of leg and foot

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Common peroneal nerve

• Foot-drop

• Paralysis of anterior and lateral compartment of leg

• Sensory loss over dorsum of foot and toes and anterolateral aspect of leg

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Peroneal nerve

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Tibial nerve

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Tibial nerve Medial division of sciatic nerve Lesions at ankle

Tarsal tunnel syndrome Pain and paresthesia in sole Paralysis of intrinsic muscles of foot Tenderness of Tinel’s sign at flexor retinaculum

Sural nerve compression syndrome Pure sensory Numbness on lateral aspect of foot

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Tarsal tunnel syndrome

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Facial nerve

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Facial nerve

Chorda tympani

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Bell’s palsy (idiopathic facial paralysis)

Clinical features :

postauricular pain (few days) lower motor neuron facial weakness impaired taste hyperacusis

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Bell’s palsy

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Facial nerve palsy Bell’s palsy : idiopathic, HSV 1 Ramsay Hunt syndrome : external ear pain with

presence of herpes zoster vesicles in auditory canal and pinna, VZV

Trauma : blunt impact to temporal bone Middle ear infection : otitis media (infrequent in ATB

era), mastoid pain persist after acute infection resolved Neoplasm : rarely compressed by CPA tumor but due to

surgery for tumor removal

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Bell’s palsy

Management Reassurance – not a stroke

Short course of prednisolone 60 mg/day Prognosis :

complete recovery 75% satisfactory 15% poor function 10%

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ผู้��ป็*ว่ยอย��ในลั�กษณะป็กต่�

ผู้��ป็*ว่ยหลั�บต่าแลัะย�งฟั0นเต่1มัที่�

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Guillain Barre syndrome

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The End