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  • 1. APPROACH TO PERIPHERAL NEUROPATHY PROF.RUCKMANI REDDY’S UNIT M1
  • 2.
    • HUMAN NS-----CNS
    • -----PNS
    • EACH NEURON has
    • 3 parts
    • SOMA
    • DENDRITES
    • AXON
  • 3. What forms peripheral nerve?
  • 4.
    • 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
  • 5. Role of nerve fibres in peripheralneuropathy
  • 6. HISTORY
    • Onset –acute/subacute/c/c
    • Part of body involved
    • Sequence of involvement
    • Symptoms -----sensory
    • -----motor
    • -----autonomic
  • 7. 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 affected-AI,CIDP,antimyelinasso GP polyneuropathy
  • 8.
    • 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 – FREDERICH’S ATAXIA
    • --Purely sensory polyNP
  • 9. Motor symptoms SPASMS DIIFFICULTY WITH FOOD INTAKE TREMOR WASTING OF MUSCLE MYOKYMIA DECREASED MANUAL DEXTERITY FASCICULATIONS WEAKNESS MUSCLE CRAMPS
  • 10. Autonomic symptoms ANOREXIA,NAUSEA,VOMITING-GASTROPARESIS BLADDER,BOWEL,SEXUAL DYSFUNCTION HEAT INTOLERANCE SWEATING-INCREASED/DECREASED FAINTING SPELLS ORTHOSTATIC LIGHT HEADEDNESS
  • 11.
    • PANDYSAUTONOMIA-
    • Amyloidosis
    • Hereditary smallfibrePN
    • DRUG H/O-cisplatin,nitrofurantoin,pyridoxinetoxicity
    • FAMILY H/O-Repeated injury,c/c subcut&osteomyelitis infection
    • PAST H/O
    • recent infections
    • viral diseases
  • 12. GE-SKIN,HAIR,NAILS BULLOUS LESIONS VARIEGATE PORPHYRIA HYPERPIGMENTATION OSTEOSCLEROTIC MYELOMA-POEMS SKIN HYPERPIGMENTATION LEPROSY ANGIOKERATOMAS FABRY’S DISEASE PURPURA CRYOGLOBULINEMIA PURPURA,LIVEDORETICULARIS VASCULITIS
  • 13. CURLED HAIR GIANT AXONAL NEURPATHY ALOPECIA THALLIUM POISONING MEE’S LINES ARSENIC/THALLIUM INTOXICATION ICTHYOSIS REFSUM’S DISEASE
  • 14. NERVE THICKENING in NPthy
    • LEPROSY
    • DIABETES
    • AMYLOIDOSIS
    • NEUROFIBROMATOSIS
    • REFSUM’S DISEASE
    • DEJERINE SOTTAS DISEASE
    • ROUSSY LEVY SYNDROME
  • 15. DEFORMITY in NPthy
    • Foot,hand,spine
    • Talipes equinus
    • Claw foot
    • Pes cavus
    • Kyphoscoliosis
    • Burns
    • Pressure sores
  • 16. Cranial nerves
    • Neuropathy with facial nerve damage
    • GBS
    • C/C inflammatory polyradiculonuropathy
    • Lyme diasease
    • Sarcoidosis
    • HIV-1infection
    • Gelsolin familial amyloid neuropathy
    • Tangier’s disease
  • 17.
    • 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
  • 18.
    • Ataxia similar to cerebellar,but no nystagmus,dysarthria
    • SENSATIONS-
    • pin prick
    • temperature
    • vibration
    • pressure
    • position sense
    • Proprioception assessment
  • 19. Pattern of sensory loss Sensory loss of trunk,scalp,face d/t simultaneous damage of proximal&distal part of sensory nerve SENSORY GANGLIONOPATHY ESCUTCHEON pattern of sensory loss over abdomen and thorax SEVERE AXONAL NEUROPATHY Symmetrical,distal,legs>arms Can even progress to face POLY NEUROPATHY
  • 20. NERVE FIBRE AFFECTED? Poor balance Diminished or absent reflexes tingling Decreased joint position sense Pin & needle sensation Decreased vibration sense numbness SIGNS SYMPTOMS LARGE FIBRE NEUROPATHY
  • 21. Causes of large fibre/ataxic NP
    • SJOGREN’S SYNDROME
    • VIT B12 NEUROPATHY
    • CISPLATIN
    • PYRIDOXINE NEUROTOXICITY
    • FRIEDEREICH’S ATAXIA
  • 22. SMALL FIBRE NEUROPATHY Diminished temperature sensation Tight band like pressure Insensitive to heat and cold Decreased pinprick sensation PAIN-burning,shock like,stabbing,prickling,shooting,lancinating Allodynia
  • 23. Causes of small fibre neuropathy (painful NP&dissociated sensory loss)
    • Heriditary sensory neuropathy
    • Lepromatous leprosy
    • Diabetes mellitus
    • Amyloidosis(early familial&primary)
    • Tangier disease
    • Fabry’s disease-pain predomonates
    • Dysautonomia-riley-day syndrome
    • HIV & antiretroviral therapy neuropathy
  • 24. SMALL & LARGE FIBRE NEUROPATHY
    • Global sensory loss
    • Carcinomatous sensory neuropathy
    • Hereditary sensory neuropathy
    • Diabetic sensory neuropathy
    • Vacor intoxication
    • Xanthomatous neuropathy of primary biliary cirrhosis
  • 25. Motor predominant neuropathy
    • Immune neuropathies
    • Heriditary motor sensory neuropathies
    • Acute intermittent porphyria
    • Diphtheritic neuropathy
    • Lead neuropathy
    • Brachial neuritis
    • Diabetic lumbosacralplexus neuropathy
  • 26. Autonomic
    • Acute-pandysautonomia
    • -botulism
    • -porphyria
    • -GBS
    • -Amiodarone
    • -vincristine
    • Chronic-amyloid,diabetes,sjogren’s,HSN 1&3,chagas,paraneoplastic
  • 27. Distribution of neuropathy
    • ?MONONEUROPATHY
    • Focal involvement of a single nerve
    • Weakness & sensory loss in the territory of a single peripheral nerve
    • Pain along the pathway of the nerve
    neoplasms Vascular lesions entrapment compression Direct trauma
  • 28. ?MONONEUROPATHY MULTIPEX
    • Random pattern of nerve involvement
    • In distribution of separate nerves,asymmetric
    • May/may not be painful
    • Not length dependent
    • Isolated reflex loss
    • CAUSES —inflammatory-leprosy,sarcoid
    • Vascular-Diabetes
    • Pressure,Trauma,Infiltration
    • Vasculitis-PAN,SLE,RA,scleroderma
    • Immune-vaccination
  • 29. ?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
  • 30. CAUSES
    • Diabetes mellitus
    • Alcohol
    • Vit B12 deficiency
    • HIV
    • Although more than
    • one nerve involved
    • one will be prominant
  • 31. DDs of distal symmetricNP
    • Lumbosacral polyradiculopathy/stenosis
    • Myelopathy-structural
    • -nonstructural
    • Vascular insufficiency-exercise related cramps,aching pain>numbness
    • Orthopedics –stress #,plantar fascitis
  • 32.
    • ?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
  • 33.
    • ?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
  • 34.
    • ?PLEXOPATHY
    • Asymmetric
    • Painful onset
    • Multiple nerves in a single limb
    • Rapid onset of weakness,atrophy
    • Isolated reflex loss
  • 35. ?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
  • 36. ANATOMIC PATTERN?
  • 37. Proprioceptive weakness Distal & proximal weakness Distal weakness Vibration&proprioception>pain&temp Vibration&proprioception>pain&temp Pain&temp affected>vib,proprioception ataxia,paraesthesia Paraesthesia&weakness Dysesthesia&distal weakness rapid Acute/subacute Slow evolution Non length dependent UE,LE,face Proximal=distal Distal>proximal Length dependent NEURONAL DEMYELINATING AXONAL
  • 38. Poor recovery Rapid recovery Slow recovery Axonal degeneration,no regeneration Demyelination&remyelination Axonal degeneration&regeneration Sensoryamplitudeaffected. radial>sural Velocity>amplitude Amplitude affected>velocity areflexia areflexia Distal areflexia NEURONAL DEMYELINATING AXONAL
  • 39. Diabetes mellitus Diabetes mellitus MMN CMT pyridoxine CIDP HIV cisplatin diphtheria metabolic Sjogren’s GBS Toxic NEURONAL DEMYELINATING AXONAL
  • 40. 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
  • 41.
    • 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
  • 42.
    • 3.C/C sensorimotor polyneuropathy syndrome
    ACQUIRED GENETIC Metachromaticleucodystrophy amyloidosis Tangier’s disease paraproteinemia A beta lipoproteinemia myeloma Refsum’s disease carcinoma Portugeseamyloidosis/ andrade’s disease uremia Hereditary sensory NP Diabetes mellitus Dejerine sottas disease leprosy Peronealmuscle atrophy/CMT
  • 43.
    • RECURRENT POLYNEUROPATHY
    • Relapsing CIDP
    • Porphyria
    • Refsum’s disease
    • HNPP
    • GBS
    • Beriberi
    • Toxic neuropathy
  • 44.
    • SENSORY ATAXIC NEUROPATHY
    • Sensory NP(polyganglionopathy)
    • Paraneoplastic sensory NP=sjogren’s
    • =idiopathic
    • Toxic=cisplatin
    • =vit B6 excess
    • Demyelinating polyradiculopathy=MGUS
    • =Millerfisher
  • 45. HOW TO DISTINGUISH VARIOUS LMN LESIONS?
  • 46. Nl/dec normal absent dec/Nl DTR + absent Usually+ absent Sensoryloss absent Usually neg Can occur severe wasting absent absent Maybe+ve marked fasciculation Prox except SMA Extraoccular,bulbar distal distal Distribution of weakness ----- More in evening ------ ----- Fatigue,diurnalweakness variation Myopathy NMJ Neuropathy AHC
  • 47. 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
  • 48.
    • URINE
    • BJ protein
    • Porphyr ia
    • 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
  • 49.
    • 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
  • 50. NERVE BIOPSY-indications sural,sup peroneal&sup radialN METACHROMATIC LEUKODYSTROPY Paraprotein neuropathy KRABBE’S CIDPolyradiculoneuropathy LEPROSY CMT 1&3 SARCOIDOSIS Infantile neuroaxonal dystrophy AMYLOIDOSIS GIANT AXONAL NPthy VASCULITIS
  • 51.
    • 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
  • 52. H/O & EXAMINATION Mononeuropathy EDx Axonal/demyeln? Any systemic disorder Entrapment/compression
  • 53. MNPthy multiplex axonal Demyeln +focal condcn block Vasculitis/multifocal Nerve biopsy CIDP Paraprotein,HIV,lyme
  • 54. polyNPthy axonal Sub a/c c/c Toxins/systemic disease family h/o,genetics
  • 55. demyelination Uniform slowing,C/C Nonuniform slowing,condnblock paraprotein Family h/o,genetics c/c or suba/c-CIDP a/c-GBS
  • 56.
    • THANK YOU