APPROACH TO PERIPHERAL NEUROPATHY

PROF.RUCKMANI REDDYS UNIT M1

HUMAN NS-----CNS -----PNS EACH NEURON has 3 parts SOMA DENDRITES AXON

What forms peripheral nerve?

1.DORSAL NERVE ROOT 2.VENTRAL NERVE ROOT 3.DORSAL ROOT GANGLIA 4.CRANIAL NERVES EXCEPT 1 $ 2 5.BRACHIAL & LUMBOSACRALPLEXUS 6.OTHER sensory,motor,autonomic nerves

Role of nerve fibres in peripheralneuropathy

HISTORY
Onset acute/subacute/c/c Part of body involved Sequence of involvement Symptoms -----sensory -----motor -----autonomic

Sensory symptoms
Pain -pricking,burning type Allodynia Hyperalgesia Neuropathic pain Anaesthesia Tremors-action tremor-in a phase of PNP -slow tremor with clumsiness large fibres affectedAI,CIDP,antimyelinasso GP polyneuropathy

Paraesthesia marked in hands & feet objectory sensoryloss is lacking Spontaneous parasthesia -in acquired causes-60% -in inherited causes-17% Painful parasthesia DM --ALCOHOLIC NPY --AMYLOID Why pain?loss of large touch pressure fibres Exception FREDERICHS ATAXIA --Purely sensory polyNP

Motor symptoms
MUSCLE CRAMPS FASCICULATIONS WEAKNESS DECREASED MANUAL DEXTERITY
WASTING OF MUSCLE DIIFFICULTY WITH FOOD INTAKE

MYOKYMIA TREMOR

SPASMS

Autonomic symptoms
ORTHOSTATIC LIGHT HEADEDNESS FAINTING SPELLS SWEATING-INCREASED/DECREASED HEAT INTOLERANCE BLADDER,BOWEL,SEXUAL DYSFUNCTION ANOREXIA,NAUSEA,VOMITINGGASTROPARESIS

PANDYSAUTONOMIA Amyloidosis Hereditary smallfibrePN DRUG H/Ocisplatin,nitrofurantoin,pyridoxinetoxicity FAMILY H/O-Repeated injury,c/c subcut&osteomyelitis infection PAST H/O recent infections viral diseases

GE-SKIN,HAIR,NAILS
VASCULITIS
CRYOGLOBULINEMIA

PURPURA,LIVEDORETICUL ARIS PURPURA ANGIOKERATOMAS SKIN HYPERPIGMENTATION

HYPERPIGMENTATION BULLOUS LESIONS

FABRYS DISEASE LEPROSY

OSTEOSCLEROTIC MYELOMA-POEMS VARIEGATE PORPHYRIA

REFSUMS DISEASE ICTHYOSIS ARSENIC/THALLIUM MEES LINES INTOXICATION THALLIUM POISONING ALOPECIA

GIANT AXONAL NEURPATHY

CURLED HAIR

NERVE THICKENING in NPthy

LEPROSY DIABETES AMYLOIDOSIS NEUROFIBROMATOSIS REFSUMS DISEASE DEJERINE SOTTAS DISEASE ROUSSY LEVY SYNDROME

DEFORMITY in NPthy
Foot,hand,spine Talipes equinus Claw foot Pes cavus Kyphoscoliosis Burns Pressure sores

Cranial nerves
Neuropathy with facial nerve damage GBS C/C inflammatory polyradiculonuropathy Lyme diasease Sarcoidosis HIV-1infection Gelsolin familial amyloid neuropathy Tangiers disease

Look for-muscle wasting Strength of muscle REFLEXES decreased/lost --sign of PNP except in spinal shock Incoordination of movements Gait abnormality-flinging/slapping gait ATAXIA CAUSE? proprioceptive deafferentiation spinocerebellar dysfunction ATAXIA WITHOUT WEAKNESS -tabesdorsalis DDs-DM,miller fisher variant,sensoryNPthy

Ataxia similar to cerebellar,but no nystagmus,dysarthria SENSATIONS pin prick temperature vibration pressure position sense Proprioception assessment

Pattern of sensory loss

POLY NEUROPAT HY SEVERE AXONAL NEUROPAT HY SENSORY GANGLION OPATHY Symmetrical,distal,legs>arms Can even progress to face ESCUTCHEON pattern of sensory loss over abdomen and thorax

Sensory loss of trunk,scalp,face d/t simultaneous damage of proximal&distal part of sensory nerve

NERVE FIBRE AFFECTED?

SYMPTOMS numbness Pin & needle sensation tingling Poor balance LARGE FIBRE NEUROPATHY SIGNS Decreased vibration sense Decreased joint position sense Diminished or absent reflexes

Causes of large fibre/ataxic NP

SJOGRENS SYNDROME VIT B12 NEUROPATHY CISPLATIN PYRIDOXINE NEUROTOXICITY FRIEDEREICHS ATAXIA

SMALL FIBRE NEUROPATHY

PAINburning,shock like,stabbing,pric kling,shooting,la ncinating Allodynia Tight band like pressure Insensitive to heat and cold Decreased pinprick sensation

Diminished temperature sensation

Causes of small fibre neuropathy (painful NP&dissociated sensory loss)

Heriditary sensory neuropathy Lepromatous leprosy Diabetes mellitus Amyloidosis(early familial&primary) Tangier disease Fabrys disease-pain predomonates Dysautonomia-riley-day syndrome HIV & antiretroviral therapy neuropathy

SMALL & LARGE FIBRE NEUROPATHY

Global sensory loss Carcinomatous sensory neuropathy Hereditary sensory neuropathy Diabetic sensory neuropathy Vacor intoxication Xanthomatous neuropathy of primary biliary cirrhosis

Motor predominant neuropathy

Immune neuropathies Heriditary motor sensory neuropathies Acute intermittent porphyria Diphtheritic neuropathy Lead neuropathy Brachial neuritis Diabetic lumbosacralplexus neuropathy

Autonomic
Acute-pandysautonomia -botulism -porphyria -GBS -Amiodarone -vincristine Chronic-amyloid,diabetes,sjogrens,HSN 1&3,chagas,paraneoplastic

Distribution of neuropathy

?MONONEUROPAT HY Focal involvement of a single nerve Weakness & sensory loss in the territory of a single peripheral nerve Pain along the pathway of the nerve

Direct trauma

compressio n

entrapment Vascular lesions

neoplasms

?MONONEUROPATHY MULTIPEX

Random pattern of nerve involvement In distribution of separate nerves,asymmetric May/may not be painful Not length dependent Isolated reflex loss CAUSESinflammatory-leprosy,sarcoid Vascular-Diabetes Pressure,Trauma,Infiltration Vasculitis-PAN,SLE,RA,scleroderma Immune-vaccination

?POLYNEUROPATHY
MC type Distal symmetric polyneurpathy Burning sensation,tingling,numbness Length dependent pattern Starts in feet,distal stocking glove pattern Fairly symmetric Symmetrically decreased reflexes Sensory>motor

CAUSES
Diabetes mellitus Alcohol Vit B12 deficiency HIV Although more than one nerve involved one will be prominant

DDs of distal symmetricNP

Lumbosacral polyradiculopathy/stenosis Myelopathy-structural -nonstructural Vascular insufficiency-exercise related cramps,aching pain>numbness Orthopedics stress #,plantar fascitis

?POLYRADICULOPATHY Disease of multiple peripheral nerve roots Asymmetric with erratic distribution-proximal in one,distal in another Pain is a common feature ?MONORADICULOPATHY Root disease by disease of spinal column Changes in distribution of spinal nerve root

?SENSORY NEURONOPATHY Ganglion cells predominantly affected Both proximal & distal involvement Sensory ataxia is common No weakness But awkward movement d/t sensory disturbances ?MOTOR NEURONOPATHY Disorder of ant horn cells Weakness,fasciculation,atrophy Not properly a process of peripheral NP

?PLEXOPATHY Asymmetric Painful onset Multiple nerves in a single limb Rapid onset of weakness,atrophy Isolated reflex loss

?polyneuritis cranialis
a/c idiopathic polyneuritis Peripheral nerve+cranial nerve involvement Self limiting painful ophthalmoplegia CAUSES-TB meningitis osteomyelitis skull otitis media syphilitic meningitis sarcoidosis carcinomatous meningitis

ANATOMIC PATTERN?

AXONAL Distal>proximal Length dependent Slow evolution Dysesthesia&dist al weakness Pain&temp affected>vib,pro prioception Distal weakness

DEMYELINATING NEURONAL Proximal=distal Non length dependent UE,LE,face rapid ataxia,paraesthes ia Vibration&proprio ception>pain&tem p Proprioceptive weakness

Acute/subacute Paraesthesia&wea kness Vibration&proprioc eption>pain&temp Distal & proximal weakness

AXONAL Distal areflexia

DEMYELINATING NEURONAL areflexia areflexia

Amplitude Velocity>amplitude Sensoryamplitude affected>velocit affected. y radial>sural Axonal Demyelination&re Axonal degeneration&r myelination degeneration,no egeneration regeneration Slow recovery Rapid recovery Poor recovery

AXONAL Toxic metabolic HIV Diabetes mellitus CMT

DEMYELINATING NEURONAL GBS diphtheria CIDP Diabetes mellitus MMN Sjogrens cisplatin pyridoxine

COURSE OF DISEASE
1.syndrome of a/c ascending motor paralysis a.acute idiopathic polyneuritis b.IMN with polyneuritis c.diphtheria d.hepatitis with polyneuritis e.porphyria f.TOCP poisoning g.paraneoplastic h.post vaccinial

2.syndome of subacute sensorymotor NP A.Deficiency=alcoholic beriberi pellagra vit B12 B.Toxins=arsenic,lead,Hg,Pb C.Drugs=nitrofurantoin,INH dapsone,disulfiram clioquinol D.Uremic E.DM,PAN,sarcoidosis A,B,C,D====SYMMETRIC

3.C/C sensorimotor polyneuropathy syndrome

GENETIC ACQUIRED Peronealmuscle atrophy/CMT leprosy

Dejerine sottas disease Hereditary sensory NP Portugeseamyloidosis/ andrades disease Refsums disease A beta lipoproteinemia Tangiers disease Metachromaticleucodystrophy Diabetes mellitus uremia carcinoma myeloma paraproteinemia amyloidosis

RECURRENT POLYNEUROPATHY Relapsing CIDP Porphyria Refsums disease HNPP GBS Beriberi Toxic neuropathy

SENSORY ATAXIC NEUROPATHY Sensory NP(polyganglionopathy) Paraneoplastic sensory NP=sjogrens =idiopathic Toxic=cisplatin =vit B6 excess Demyelinating polyradiculopathy=MGUS =Millerfisher

HOW TO DISTINGUISH VARIOUS LMN LESIONS?

AHC
Fatigue,diur ----nalweaknes s variation Distribution distal of weakness fasciculation marked wasting severe

Neuropat NMJ hy -----More in evening

distal

Myopat hy -----

Extraocc Prox ular,bulb except ar SMA Maybe+ve absent absent Can occur Usually neg Usually+ absent absent normal absent + Nl/dec

Sensoryloss absent DTR dec/Nl

INVESTIGATIONS
BLOOD TC,DC,ESR Urea,electrolytes,LFT RBS,HbA1C Serum protein electrophoresis Auto Ab=ANA,Antiganglioside,Antineuronal Vit B 12 level DNA analysis=chr 17 duplication-HMSN1&1A =chr 17 deletion -HLPP

URINE BJ protein Porphyria Heavy metals CSF ANALYSIS NERVE CONDUCTION STUDY Variation in axonal,demyelinating neuropathy Conduction block-CIDP,GBS,MMN EMG-muscle denervation changes Sensory threshold Thermal & vibration threshold

IMAGES CXR-sarcoidosis,malignancy Skeletal survey-multiple myeloma Screening for malignancy

AUTONOMIC FUNCTION TESTS

Diagnostic tests imp in Asymmetric,motor predominant,rapid onset,demyelinating neuropathy

NERVE BIOPSY-indications sural,sup peroneal&sup radialN

VASCULITIS AMYLOIDOSIS GIANT AXONAL NPthy Infantile neuroaxonal dystrophy CMT 1&3

SARCOIDOSIS LEPROSY
KRABBES

CIDPolyradiculoneuropathy
Paraprotein neuropathy

METACHROMATIC LEUKODYSTROPY

ETIOLOGICAL CLASSIFICATION 1.metabolic-DM,amyloidosis,porphyria 2.infections-leprosy,HIV,CMV,syphilis, diphtheria,lymedisease 3.immune- GBS,CIDN,MMN 4.hereditary-CMT 5.Toxic-drugs,alcohol,heavymetals 6.vasculitis-PAN,CSS,cryoglobulinemia 7.paraneoplastic-lung 8.nutritional- B1,B6,B12

H/O & EXAMINATION

Mononeuropathy EDx
Axonal/demyeln?

Entrapment/compression

Any systemic disorder

MNPthy multiplex

axonal

Demyeln +focal condcn block

Vasculitis/multifocal

CIDP

Nerve biopsy

Paraprotein,HIV,lyme

polyNPthy

axonal Sub a/c c/c

Toxins/systemic disease

family h/o,genetics

demyelination

Uniform slowing,C/C

Nonuniform slowing,condnblock

paraprotein

c/c or suba/c-CIDP

a/c-GBS

Family h/o,genetics

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