Peripheral Peripheral neuropathiesneuropathiesDr. Osman Sadig Dr. Osman Sadig

BukhariBukhari

Peripheral nerve diseasesPeripheral nerve diseases 11 - -MononeuropathiesMononeuropathies

22 - -Multiple mononeuropathiesMultiple mononeuropathies

( ( mononeuritis multiplexmononeuritis multiplex))

33 - -PolyneuropathiesPolyneuropathies

NeuropathyNeuropathy= pathological processes = pathological processes damaging a nerve or nerves. The mechanisms damaging a nerve or nerves. The mechanisms

of damage may beof damage may be-:-:

11 - -Demyelination Demyelination e.g. GB, diphth, HSMNe.g. GB, diphth, HSMN

22 - -Axonal degenerationAxonal degeneration: e.g. Toxic neuropathies: e.g. Toxic neuropathies

33 - -CompressionCompression: Causes segmental : Causes segmental demyelinatiodemyelinatio

e.g. Entrapment Ne.g. Entrapment N..

44 - -VasculopathyVasculopathy )infarction(: Causes distal )infarction(: Causes distal Wallerian degenerat e.g. DM, collagen Wallerian degenerat e.g. DM, collagen diseases e.g. PAN diseases e.g. PAN . .

55-- InfiltrationInfiltration: e.g. leprosy, malig, sarcoidosis: e.g. leprosy, malig, sarcoidosis . .

** ** With axonal degen nerve conduction velocityWith axonal degen nerve conduction velocity

is normal or slightly reduced & EMG showsis normal or slightly reduced & EMG shows

evidence of muscle denervationevidence of muscle denervation..

** ** With demyelination conduction velocity mayWith demyelination conduction velocity may

be slowed considerably or blocked in severebe slowed considerably or blocked in severe

cases without accompanying muscle denervcases without accompanying muscle denerv..

-** -** MononeuropathyMononeuropathy e.g. compression e.g. compression

- - Multiple mononeuropathiesMultiple mononeuropathies e.g. vasculopath e.g. vasculopath

infiltr, radiationinfiltr, radiation

- - PolyneuropathiesPolyneuropathies e.g. hereditary, metab e.g. hereditary, metab

toxic, GB, non metastatic manif of maligtoxic, GB, non metastatic manif of malig

Poly neuropathiesPoly neuropathies CausesCauses

11 - -Inherited neuropathiesInherited neuropathies:: - - Charcot - Marie- Tooth diseaseCharcot - Marie- Tooth disease

- - Dejerine- Sottas diseaseDejerine- Sottas disease

- - Refsum,s diseaseRefsum,s disease

- - Acute intermittent porphyriaAcute intermittent porphyria

- - Friedreich,s ataxiaFriedreich,s ataxia

22 - -Metab & endocrineMetab & endocrine:: - - DMDM

- - UraemiaUraemia

- - Chronic liver failureChronic liver failure

- - HypothyroidismHypothyroidism

- - AcromegalyAcromegaly

- - AmyloidosisAmyloidosis

- - Para & cryoproteinaemiaPara & cryoproteinaemia

33 - -Toxic neuropathiesToxic neuropathies:: - - AlcoholAlcohol

- - Drugs ) INH, phenytoin, vincristine, (Drugs ) INH, phenytoin, vincristine, (

- - Heavy metals )lead, arsenic, Hg(Heavy metals )lead, arsenic, Hg(

- - Organic solvents )acryl amide, Organic solvents )acryl amide, organophos(organophos(

44 - -InfectiveInfective:: - - LeprosyLeprosy

- - DiphtheriaDiphtheria

- - HIVHIV

- - H. zosterH. zoster

55 - -InflammatoryInflammatory:: - - Guillian – Barre’ syndromeGuillian – Barre’ syndrome..

- - Chronic demyelinating Chronic demyelinating polyneuropathypolyneuropathy

- - Idiopathic chronic sensorimotor Idiopathic chronic sensorimotor neuropathyneuropathy

- - Connective tissue diseasesConnective tissue diseases

- - SarcoidosisSarcoidosis

- - Lyme borreliosisLyme borreliosis

66 - -Vitamin deficiencyVitamin deficiency::

- - B12 B12 - B1 - B1 - B6 - B6 - Folate - Folate

- - Nicotinic acid Nicotinic acid - Vit E - Vit E

77 - -Neuropathy associated with Neuropathy associated with malignant diseasemalignant disease

88 - -Neuropathy associated wz critically Neuropathy associated wz critically ill ptsill pts

Modalities of Modalities of polyneuropathiespolyneuropathies

- -SensorySensory

- - MotorMotor

- - MixedMixed

- - AutonomicAutonomic

C/F ofC/F of PolyneuropathiesPolyneuropathies11 - -Sensory dysfn:Sensory dysfn: numbness, paraesthesiae numbness, paraesthesiae,,

hyperaesthesia & pain starting distally andhyperaesthesia & pain starting distally and ascending proximally in gloves & stockingsascending proximally in gloves & stockings

with impaired perception of pain, touch, tempwith impaired perception of pain, touch, temp vibration & position sensevibration & position sense..

22 - -Motor dysfnMotor dysfn: flaccid weakness most marked: flaccid weakness most marked distallydistally..

33 - -tendon reflexestendon reflexes: depressed or absent: depressed or absent..

44 - -AutonomicAutonomic neuropathyneuropathy: orthostatic hypot: orthostatic hypot

dysphagia, gastro paresis, gustatory sweatingdysphagia, gastro paresis, gustatory sweating

( ( facial sweating wz anhydrosis of the feetfacial sweating wz anhydrosis of the feet,),)

noct. diarrhoea, urine retention wz overflownoct. diarrhoea, urine retention wz overflow

incontinence, failure of erection, resting incontinence, failure of erection, resting tachycardia, fixed HR, C/R arrest, dependanttachycardia, fixed HR, C/R arrest, dependant

oedema wz cold feet, small non reactive pupilsoedema wz cold feet, small non reactive pupils

- -CausesCauses: - DM : - DM - GB - GB

- - Acute intermittent porphyriaAcute intermittent porphyria

- - Amyloidosis Amyloidosis - Drugs - Drugs

InvestigationsInvestigations: guided by sympt & : guided by sympt & signssigns

The cause of polyneuropathy is suggested byThe cause of polyneuropathy is suggested by the the historyhistory including the onset, FH, PMH, DH, including the onset, FH, PMH, DH,

SHSH and za predominant and za predominant clinical manifestationsclinical manifestations..

- - CBC & ESRCBC & ESR - - Renal profile & liver biochemistryRenal profile & liver biochemistry

- - Blood glucose & thyroid fnsBlood glucose & thyroid fns.. - - ANF & RFANF & RF

- - Plasma electrophoresisPlasma electrophoresis - - Urinary levels of heavy metalsUrinary levels of heavy metals..

- - CSFCSF - - CXRCXR

- - Serum lipids, lipo proteins, cry proteinsSerum lipids, lipo proteins, cry proteins

- - Vitamins assayVitamins assay

- - Urinary porphyrinsUrinary porphyrins

- - GeneticsGenetics

- - Search for malig e.g. radiology, Search for malig e.g. radiology, immagingimmaging,,

PSA, stools for occult blood, endoscopyPSA, stools for occult blood, endoscopy,,

mamography mamography - Nerve conduction studies - Nerve conduction studies

- - EMGEMG

- - Nerve biopsyNerve biopsy..

Inherited neuropathiesInherited neuropathies 11 - -Charcot Marie Tooth syndromeCharcot Marie Tooth syndrome

- - Mainly ADMainly AD - - presents in early adult life wz gait disturbapresents in early adult life wz gait disturba or foot deformities. Slow nerve degeneratioor foot deformities. Slow nerve degeneratio lead to polyneuropathy wz distal weaknesslead to polyneuropathy wz distal weakness

and wasting )inverted champagne bottle(and wasting )inverted champagne bottle(,, variable sensory loss, absent kn & An jerksvariable sensory loss, absent kn & An jerks,,

high steppage gait due to foot drophigh steppage gait due to foot drop - - Pes cavus & claw feet +/- optic atrophy & Pes cavus & claw feet +/- optic atrophy &

deafness deafness - - Dis arrested in middle life. Normal life spanDis arrested in middle life. Normal life span..

22 - -Dejerine Sottas diseaseDejerine Sottas disease - - AR or sporadicAR or sporadic

- - Progressive motor & sensory polyn Progressive motor & sensory polyn withwith

weakness, ataxia, sensory loss & weakness, ataxia, sensory loss & depresseddepressed

or absent reflexesor absent reflexes

- - palpable nerves wz segmental palpable nerves wz segmental demyelinationdemyelination

- - High CSF proteins due to obstruction High CSF proteins due to obstruction byby

nerve rootsnerve roots

33 - -Refsum’s diseaseRefsum’s disease - - ADAD

- - Due to disturbance of phytanic acid Due to disturbance of phytanic acid metabmetab..

- - Pigmentary retinal degeneration withPigmentary retinal degeneration with

progressive sensorimotor polyn & progressive sensorimotor polyn & cerebellarcerebellar

signs. Auditory dysfn, cardiomyopathy signs. Auditory dysfn, cardiomyopathy andand

cutaneous manifestationscutaneous manifestations

- - Marked reduction of conduction velocityMarked reduction of conduction velocity

- - TR by restriction of phytanic acid and itsTR by restriction of phytanic acid and its

precursorsprecursors..

44 - -Acute intermittent porphyriaAcute intermittent porphyria - - Mainly motor, proximal & UL > LLMainly motor, proximal & UL > LL

- - Sensory manifestations may occurSensory manifestations may occur

- - Axonal in typeAxonal in type

- - High CHO diet & conc. dextrose is High CHO diet & conc. dextrose is helpful andhelpful and

propranolol for tachycardia & HTpropranolol for tachycardia & HT..

Neuropathies associated with Neuropathies associated with metabmetab

and endocrine disordersand endocrine disorders

11 - -Diabetes mellitusDiabetes mellitus )occur singly or in )occur singly or in combin(combin(

- - Symmetric sensory or mixed polynSymmetric sensory or mixed polyn

- - Asymmetric motor radiculopathyAsymmetric motor radiculopathy

( ( diabetic amyotrophydiabetic amyotrophy))

- - Mononeuritis or mononeuritis Mononeuritis or mononeuritis multiplexmultiplex

- - AutonomicAutonomic

22 - -UraemiaUraemia - - Progressive sensorimotor polyn, LL > Progressive sensorimotor polyn, LL >

ULUL

- - Improves wz successful RT & to a Improves wz successful RT & to a lesserlesser

by chronic HDby chronic HD

Toxic neuropathiesToxic neuropathies

- - Alcoholic polynAlcoholic polyn - - Distal sensorimotor polyn frequently Distal sensorimotor polyn frequently

accompaccomp

by painful cramps, muscle tenderness andby painful cramps, muscle tenderness and

painful paraesthesia, often marked in za painful paraesthesia, often marked in za legslegs

- - AutonomicAutonomic

- - May respond to B1May respond to B1

- - Recurs or progress wz alcohol intakeRecurs or progress wz alcohol intake

- - Similar distal sensorimotor polyn occurs Similar distal sensorimotor polyn occurs inin

beri beri )thiamine def(beri beri )thiamine def(

Vitamin deficiencyVitamin deficiency

- - Def states occur in malnutritionDef states occur in malnutrition

- - preventablepreventable

- - potentially reversible if treated earlypotentially reversible if treated early

11 - -B12 defB12 def - - Distal sensory polyn, sp Distal sensory polyn, sp

proprioceptionproprioception

- - Absent ankle jerkAbsent ankle jerk

- - Extensor planterExtensor planter

- - Optic neuropathyOptic neuropathy

- - Intellectual dysfnIntellectual dysfn

22 - -Thiamine defThiamine def ) beri beri( ) beri beri( - - polynpolyn

- - cardiac failurecardiac failure

Werneckes Korsakoff psychosis Werneckes Korsakoff psychosis )nystagmus)nystagmus,,

ophthalmoplegia, ataxia, amnesia, ophthalmoplegia, ataxia, amnesia, confusionconfusion

comacoma

- - Parental B1 for TRParental B1 for TR

33 - -Pyridoxine defPyridoxine def )B6( )B6( - - Mainly sensoryMainly sensory

- - More common in slow acetylaters on INHMore common in slow acetylaters on INH..

- - 10mg per day for TR10mg per day for TR

Infective neuropathiesInfective neuropathies 11 - -LeprosyLeprosy

- - In TL leads to hypo pigmented anaetheticIn TL leads to hypo pigmented anaethetic patches & involves peripheral nerves ofpatches & involves peripheral nerves of

predeliction which are thickenedpredeliction which are thickened - - In LL leads to gloves & stockings sensoryIn LL leads to gloves & stockings sensory

lossloss - - Multiple mononeuropathyMultiple mononeuropathy

22 - -Diphtheric neuropathyDiphtheric neuropathy )demyelinating( )demyelinating(

- - Palatal palsy I 1-2WPalatal palsy I 1-2W - - Loss of accomodation in2-4WLoss of accomodation in2-4W

- - polyn in 4-6Wpolyn in 4-6W

33 - -AIDS neuropathyAIDS neuropathy - - Chronic symmetric sensorimotor polynChronic symmetric sensorimotor polyn

- - progressive polyradiculopathy orprogressive polyradiculopathy or

radiculomyelopathy ? CMVradiculomyelopathy ? CMV

- - Seropositive Pts may also developSeropositive Pts may also develop

demyeelinating polyradiculopathy anddemyeelinating polyradiculopathy and

mononeuritis multiplexmononeuritis multiplex,,

Inflammatory polyneuropathiesInflammatory polyneuropathies 11 - -Acute post infective polynAcute post infective polyn

- - 1-4/521-4/52 following resp tract infectionfollowing resp tract infection,, campylobacter jejuni infection of za gutcampylobacter jejuni infection of za gut

( ( in 25% of cases, more severe & in 25% of cases, more severe & residualresidual

deficit(, surgery & immunizationdeficit(, surgery & immunization - - Demyelination of the spinal roots & Demyelination of the spinal roots &

periph N has probably immunological periph N has probably immunological basisbasis..

- - Patient presents wz distal weakness andPatient presents wz distal weakness and numbness ascending over days to involvenumbness ascending over days to involve

the face, resp muscles & bulbar musclesthe face, resp muscles & bulbar muscles.. - - Patient may C/O back pain at za onsetPatient may C/O back pain at za onset..

- - Clinically there is muscle weakness, Clinically there is muscle weakness, areflexiaareflexia

and variable sensory loss. Sphincters areand variable sensory loss. Sphincters are intact & there is no sensory levelintact & there is no sensory level..

- - Rapid deterioration wz resp failure mayRapid deterioration wz resp failure may occuroccur..

- - Ophthalmoplegia, ataxia & areflexia can beOphthalmoplegia, ataxia & areflexia can be a presentation ) Miller Fissure(a presentation ) Miller Fissure(

- - CSF shows protein/ cell dissociation thatCSF shows protein/ cell dissociation that may take 2-3 W to developmay take 2-3 W to develop..

- - Nerve conduction velocity is slowedNerve conduction velocity is slowed.. - - Investigations to search for a cause e.g Investigations to search for a cause e.g

CMVCMV mycoplasma, campyl )CXR, stool culture mycoplasma, campyl )CXR, stool culture

and serology(and serology(

- - DD include diphtheria, pophyria, lead NDD include diphtheria, pophyria, lead N,, ( ( pry motor Npry motor N ,) ,)botulism, polio & pry botulism, polio & pry

muscle diseasemuscle disease - - TreatmentTreatment::

- - supportive ) ABC, nursing, physioth(supportive ) ABC, nursing, physioth( - - mechanical ventilation if resp mechanical ventilation if resp

paralysisparalysis occur. Monitor resp wz vital capacityoccur. Monitor resp wz vital capacity..

- - Plasmapheresis & IV Ig if given earlyPlasmapheresis & IV Ig if given early - - Use of C/S is controversialUse of C/S is controversial..

- - complete recovery occur in 80% in 3-complete recovery occur in 80% in 3-6 M6 M

- - mortality 4% & 3% relapsemortality 4% & 3% relapse.. - - remainder left wz disabilityremainder left wz disability..

Predominantly motor neuropathyPredominantly motor neuropathy 11 - -GBGB

22 - -Ca neuropathCa neuropath

33 - -Charcot Marie tooth disease )peroneal Charcot Marie tooth disease )peroneal muscular atrophy muscular atrophy

44 - -lead poisoninglead poisoning

Management of neuropathiesManagement of neuropathies 11 - -In 1/3 treatable causeIn 1/3 treatable cause::

- - toxins & offending drugs removedtoxins & offending drugs removed

- - Deficiencies & metab abn correctedDeficiencies & metab abn corrected

- - inflammatory causes by inflammatory causes by immunosuppressionimmunosuppression

22 - -In 1/3 there is identifiable cause but noIn 1/3 there is identifiable cause but no

TR as in hereditaryTR as in hereditary

33 - -In 1/3 no specific causeIn 1/3 no specific cause

-- --Physiotherapy & occupational therapyPhysiotherapy & occupational therapy

MononeuropathiesMononeuropathies 11 - -Acute : sustained pressure e.g. tourniquetAcute : sustained pressure e.g. tourniquet

22 - -Chronic: entrapmentChronic: entrapment

CausesCauses according to site of compression according to site of compression

11 - -Carpal tunnelCarpal tunnel Median N Median N

22 - -Cubital tunnelCubital tunnel Ulnar N Ulnar N

33 - -Spiral groove of humerusSpiral groove of humerus Radial N Radial N

44 - -Inguinal ligamentInguinal ligament Lateral cutaneous of Lateral cutaneous of thighthigh

55 - -Neck of fibulaNeck of fibula Common peroneal N Common peroneal N

66 - -Flexor retinaculumFlexor retinaculum )tarsal tunnel( Post )tarsal tunnel( Post tibialtibial

Mononeuritis Mononeuritis multiplexmultiplex CausesCauses

11 - -leprosy )commonest(leprosy )commonest( 22 - -DMDM

33 - -vasculitisvasculitis 44 - -sarcoidosissarcoidosis 55 - -amyloidosisamyloidosis 66 - -malignancymalignancy

77 - -neurofibromatosisneurofibromatosis 88 - -HIV infectionHIV infection

99 - -Idiopathic multifocal motor neuropathyIdiopathic multifocal motor neuropathy