Approach to Peripheral Neuropathy

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Approach to Peripheral Neuropathy

  1. 1. APPROACH TO PERIPHERAL NEUROPATHY PROF.RUCKMANI REDDY’S UNIT M1
  2. 2. <ul><li>HUMAN NS-----CNS </li></ul><ul><li>-----PNS </li></ul><ul><li>EACH NEURON has </li></ul><ul><li>3 parts </li></ul><ul><li>SOMA </li></ul><ul><li>DENDRITES </li></ul><ul><li>AXON </li></ul>
  3. 3. What forms peripheral nerve?
  4. 4. <ul><li>1.DORSAL NERVE ROOT </li></ul><ul><li>2.VENTRAL NERVE ROOT </li></ul><ul><li>3.DORSAL ROOT GANGLIA </li></ul><ul><li>4.CRANIAL NERVES EXCEPT 1 $ 2 </li></ul><ul><li>5.BRACHIAL & LUMBOSACRALPLEXUS </li></ul><ul><li>6.OTHER sensory,motor,autonomic nerves </li></ul>
  5. 5. Role of nerve fibres in peripheralneuropathy
  6. 6. HISTORY <ul><li>Onset –acute/subacute/c/c </li></ul><ul><li>Part of body involved </li></ul><ul><li>Sequence of involvement </li></ul><ul><li>Symptoms -----sensory </li></ul><ul><li>-----motor </li></ul><ul><li>-----autonomic </li></ul>
  7. 7. Sensory symptoms <ul><li>Pain -pricking,burning type </li></ul><ul><li>Allodynia </li></ul><ul><li>Hyperalgesia </li></ul><ul><li>Neuropathic pain </li></ul><ul><li>Anaesthesia </li></ul><ul><li>Tremors-action tremor-in a phase of PNP </li></ul><ul><li>-slow tremor with clumsiness </li></ul><ul><li>large fibres affected-AI,CIDP,antimyelinasso GP polyneuropathy </li></ul>
  8. 8. <ul><li>Paraesthesia </li></ul><ul><li>marked in hands & feet </li></ul><ul><li>objectory sensoryloss is lacking </li></ul><ul><li>Spontaneous parasthesia </li></ul><ul><li>-in acquired causes-60% </li></ul><ul><li>-in inherited causes-17% </li></ul><ul><li>Painful parasthesia –DM </li></ul><ul><li>--ALCOHOLIC NPY </li></ul><ul><li>--AMYLOID </li></ul><ul><li>Why pain?loss of large touch pressure fibres </li></ul><ul><li>Exception – FREDERICH’S ATAXIA </li></ul><ul><li>--Purely sensory polyNP </li></ul>
  9. 9. Motor symptoms SPASMS DIIFFICULTY WITH FOOD INTAKE TREMOR WASTING OF MUSCLE MYOKYMIA DECREASED MANUAL DEXTERITY FASCICULATIONS WEAKNESS MUSCLE CRAMPS
  10. 10. Autonomic symptoms ANOREXIA,NAUSEA,VOMITING-GASTROPARESIS BLADDER,BOWEL,SEXUAL DYSFUNCTION HEAT INTOLERANCE SWEATING-INCREASED/DECREASED FAINTING SPELLS ORTHOSTATIC LIGHT HEADEDNESS
  11. 11. <ul><li>PANDYSAUTONOMIA- </li></ul><ul><li>Amyloidosis </li></ul><ul><li>Hereditary smallfibrePN </li></ul><ul><li>DRUG H/O-cisplatin,nitrofurantoin,pyridoxinetoxicity </li></ul><ul><li>FAMILY H/O-Repeated injury,c/c subcut&osteomyelitis infection </li></ul><ul><li>PAST H/O </li></ul><ul><li>recent infections </li></ul><ul><li>viral diseases </li></ul>
  12. 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. 13. CURLED HAIR GIANT AXONAL NEURPATHY ALOPECIA THALLIUM POISONING MEE’S LINES ARSENIC/THALLIUM INTOXICATION ICTHYOSIS REFSUM’S DISEASE
  14. 14. NERVE THICKENING in NPthy <ul><li>LEPROSY </li></ul><ul><li>DIABETES </li></ul><ul><li>AMYLOIDOSIS </li></ul><ul><li>NEUROFIBROMATOSIS </li></ul><ul><li>REFSUM’S DISEASE </li></ul><ul><li>DEJERINE SOTTAS DISEASE </li></ul><ul><li>ROUSSY LEVY SYNDROME </li></ul>
  15. 15. DEFORMITY in NPthy <ul><li>Foot,hand,spine </li></ul><ul><li>Talipes equinus </li></ul><ul><li>Claw foot </li></ul><ul><li>Pes cavus </li></ul><ul><li>Kyphoscoliosis </li></ul><ul><li>Burns </li></ul><ul><li>Pressure sores </li></ul>
  16. 16. Cranial nerves <ul><li>Neuropathy with facial nerve damage </li></ul><ul><li>GBS </li></ul><ul><li>C/C inflammatory polyradiculonuropathy </li></ul><ul><li>Lyme diasease </li></ul><ul><li>Sarcoidosis </li></ul><ul><li>HIV-1infection </li></ul><ul><li>Gelsolin familial amyloid neuropathy </li></ul><ul><li>Tangier’s disease </li></ul>
  17. 17. <ul><li>Look for-muscle wasting </li></ul><ul><li>Strength of muscle </li></ul><ul><li>REFLEXES –decreased/lost </li></ul><ul><li>--sign of PNP except in spinal shock </li></ul><ul><li>Incoordination of movements </li></ul><ul><li>Gait abnormality-flinging/slapping gait </li></ul><ul><li>ATAXIA CAUSE? proprioceptive deafferentiation </li></ul><ul><li>spinocerebellar dysfunction </li></ul><ul><li>ATAXIA WITHOUT WEAKNESS -tabesdorsalis </li></ul><ul><li>DDs-DM,miller fisher variant,sensoryNPthy </li></ul>
  18. 18. <ul><li>Ataxia similar to cerebellar,but no nystagmus,dysarthria </li></ul><ul><li>SENSATIONS- </li></ul><ul><li>pin prick </li></ul><ul><li>temperature </li></ul><ul><li>vibration </li></ul><ul><li>pressure </li></ul><ul><li>position sense </li></ul><ul><li>Proprioception assessment </li></ul>
  19. 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. 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. 21. Causes of large fibre/ataxic NP <ul><li>SJOGREN’S SYNDROME </li></ul><ul><li>VIT B12 NEUROPATHY </li></ul><ul><li>CISPLATIN </li></ul><ul><li>PYRIDOXINE NEUROTOXICITY </li></ul><ul><li>FRIEDEREICH’S ATAXIA </li></ul>
  22. 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. 23. Causes of small fibre neuropathy (painful NP&dissociated sensory loss) <ul><li>Heriditary sensory neuropathy </li></ul><ul><li>Lepromatous leprosy </li></ul><ul><li>Diabetes mellitus </li></ul><ul><li>Amyloidosis(early familial&primary) </li></ul><ul><li>Tangier disease </li></ul><ul><li>Fabry’s disease-pain predomonates </li></ul><ul><li>Dysautonomia-riley-day syndrome </li></ul><ul><li>HIV & antiretroviral therapy neuropathy </li></ul>
  24. 24. SMALL & LARGE FIBRE NEUROPATHY <ul><li>Global sensory loss </li></ul><ul><li>Carcinomatous sensory neuropathy </li></ul><ul><li>Hereditary sensory neuropathy </li></ul><ul><li>Diabetic sensory neuropathy </li></ul><ul><li>Vacor intoxication </li></ul><ul><li>Xanthomatous neuropathy of primary biliary cirrhosis </li></ul>
  25. 25. Motor predominant neuropathy <ul><li>Immune neuropathies </li></ul><ul><li>Heriditary motor sensory neuropathies </li></ul><ul><li>Acute intermittent porphyria </li></ul><ul><li>Diphtheritic neuropathy </li></ul><ul><li>Lead neuropathy </li></ul><ul><li>Brachial neuritis </li></ul><ul><li>Diabetic lumbosacralplexus neuropathy </li></ul>
  26. 26. Autonomic <ul><li>Acute-pandysautonomia </li></ul><ul><li>-botulism </li></ul><ul><li>-porphyria </li></ul><ul><li>-GBS </li></ul><ul><li>-Amiodarone </li></ul><ul><li>-vincristine </li></ul><ul><li>Chronic-amyloid,diabetes,sjogren’s,HSN 1&3,chagas,paraneoplastic </li></ul>
  27. 27. Distribution of neuropathy <ul><li>?MONONEUROPATHY </li></ul><ul><li>Focal involvement of a single nerve </li></ul><ul><li>Weakness & sensory loss in the territory of a single peripheral nerve </li></ul><ul><li>Pain along the pathway of the nerve </li></ul>neoplasms Vascular lesions entrapment compression Direct trauma
  28. 28. ?MONONEUROPATHY MULTIPEX <ul><li>Random pattern of nerve involvement </li></ul><ul><li>In distribution of separate nerves,asymmetric </li></ul><ul><li>May/may not be painful </li></ul><ul><li>Not length dependent </li></ul><ul><li>Isolated reflex loss </li></ul><ul><li>CAUSES —inflammatory-leprosy,sarcoid </li></ul><ul><li>Vascular-Diabetes </li></ul><ul><li>Pressure,Trauma,Infiltration </li></ul><ul><li>Vasculitis-PAN,SLE,RA,scleroderma </li></ul><ul><li>Immune-vaccination </li></ul>
  29. 29. ?POLYNEUROPATHY <ul><li>MC type –Distal symmetric polyneurpathy </li></ul><ul><li>Burning sensation,tingling,numbness </li></ul><ul><li>Length dependent pattern </li></ul><ul><li>Starts in feet,distal stocking glove pattern </li></ul><ul><li>Fairly symmetric </li></ul><ul><li>Symmetrically decreased reflexes </li></ul><ul><li>Sensory>motor </li></ul>
  30. 30. CAUSES <ul><li>Diabetes mellitus </li></ul><ul><li>Alcohol </li></ul><ul><li>Vit B12 deficiency </li></ul><ul><li>HIV </li></ul><ul><li>Although more than </li></ul><ul><li>one nerve involved </li></ul><ul><li>one will be prominant </li></ul>
  31. 31. DDs of distal symmetricNP <ul><li>Lumbosacral polyradiculopathy/stenosis </li></ul><ul><li>Myelopathy-structural </li></ul><ul><li>-nonstructural </li></ul><ul><li>Vascular insufficiency-exercise related cramps,aching pain>numbness </li></ul><ul><li>Orthopedics –stress #,plantar fascitis </li></ul>
  32. 32. <ul><li>?POLYRADICULOPATHY </li></ul><ul><li>Disease of multiple peripheral nerve roots </li></ul><ul><li>Asymmetric with erratic distribution-proximal in one,distal in another </li></ul><ul><li>Pain is a common feature </li></ul><ul><li>?MONORADICULOPATHY </li></ul><ul><li>Root disease by disease of spinal column </li></ul><ul><li>Changes in distribution of spinal nerve root </li></ul>
  33. 33. <ul><li>?SENSORY NEURONOPATHY </li></ul><ul><li>Ganglion cells predominantly affected </li></ul><ul><li>Both proximal & distal involvement </li></ul><ul><li>Sensory ataxia is common </li></ul><ul><li>No weakness </li></ul><ul><li>But awkward movement d/t sensory disturbances </li></ul><ul><li>?MOTOR NEURONOPATHY </li></ul><ul><li>Disorder of ant horn cells </li></ul><ul><li>Weakness,fasciculation,atrophy </li></ul><ul><li>Not properly a process of peripheral NP </li></ul>
  34. 34. <ul><li>?PLEXOPATHY </li></ul><ul><li>Asymmetric </li></ul><ul><li>Painful onset </li></ul><ul><li>Multiple nerves in a single limb </li></ul><ul><li>Rapid onset of weakness,atrophy </li></ul><ul><li>Isolated reflex loss </li></ul>
  35. 35. ?polyneuritis cranialis <ul><li>a/c idiopathic polyneuritis </li></ul><ul><li>Peripheral nerve+cranial nerve involvement </li></ul><ul><li>Self limiting painful ophthalmoplegia </li></ul><ul><li>CAUSES-TB meningitis </li></ul><ul><li>osteomyelitis skull </li></ul><ul><li>otitis media </li></ul><ul><li>syphilitic meningitis </li></ul><ul><li>sarcoidosis </li></ul><ul><li>carcinomatous meningitis </li></ul>
  36. 36. ANATOMIC PATTERN?
  37. 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. 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. 39. Diabetes mellitus Diabetes mellitus MMN CMT pyridoxine CIDP HIV cisplatin diphtheria metabolic Sjogren’s GBS Toxic NEURONAL DEMYELINATING AXONAL
  40. 40. COURSE OF DISEASE <ul><li>1.syndrome of a/c ascending motor paralysis </li></ul><ul><li>a.acute idiopathic polyneuritis </li></ul><ul><li>b.IMN with polyneuritis </li></ul><ul><li>c.diphtheria </li></ul><ul><li>d.hepatitis with polyneuritis </li></ul><ul><li>e.porphyria </li></ul><ul><li>f.TOCP poisoning </li></ul><ul><li>g.paraneoplastic </li></ul><ul><li>h.post vaccinial </li></ul>
  41. 41. <ul><li>2.syndome of subacute sensorymotor NP </li></ul><ul><li>A.Deficiency=alcoholic beriberi </li></ul><ul><li>pellagra </li></ul><ul><li>vit B12 </li></ul><ul><li>B.Toxins=arsenic,lead,Hg,Pb </li></ul><ul><li>C.Drugs=nitrofurantoin,INH </li></ul><ul><li>dapsone,disulfiram </li></ul><ul><li>clioquinol </li></ul><ul><li>D.Uremic </li></ul><ul><li>E.DM,PAN,sarcoidosis </li></ul><ul><li>A,B,C,D====SYMMETRIC </li></ul>
  42. 42. <ul><li>3.C/C sensorimotor polyneuropathy syndrome </li></ul>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. 43. <ul><li>RECURRENT POLYNEUROPATHY </li></ul><ul><li>Relapsing CIDP </li></ul><ul><li>Porphyria </li></ul><ul><li>Refsum’s disease </li></ul><ul><li>HNPP </li></ul><ul><li>GBS </li></ul><ul><li>Beriberi </li></ul><ul><li>Toxic neuropathy </li></ul>
  44. 44. <ul><li>SENSORY ATAXIC NEUROPATHY </li></ul><ul><li>Sensory NP(polyganglionopathy) </li></ul><ul><li>Paraneoplastic sensory NP=sjogren’s </li></ul><ul><li>=idiopathic </li></ul><ul><li>Toxic=cisplatin </li></ul><ul><li>=vit B6 excess </li></ul><ul><li>Demyelinating polyradiculopathy=MGUS </li></ul><ul><li>=Millerfisher </li></ul>
  45. 45. HOW TO DISTINGUISH VARIOUS LMN LESIONS?
  46. 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. 47. INVESTIGATIONS <ul><li>BLOOD </li></ul><ul><li>TC,DC,ESR </li></ul><ul><li>Urea,electrolytes,LFT </li></ul><ul><li>RBS,HbA1C </li></ul><ul><li>Serum protein electrophoresis </li></ul><ul><li>Auto Ab=ANA,Antiganglioside,Antineuronal </li></ul><ul><li>Vit B 12 level </li></ul><ul><li>DNA analysis=chr 17 duplication-HMSN1&1A </li></ul><ul><li>=chr 17 deletion -HLPP </li></ul>
  48. 48. <ul><li>URINE </li></ul><ul><li>BJ protein </li></ul><ul><li>Porphyr ia </li></ul><ul><li>Heavy metals </li></ul><ul><li>CSF ANALYSIS </li></ul><ul><li>NERVE CONDUCTION STUDY </li></ul><ul><li>Variation in axonal,demyelinating neuropathy </li></ul><ul><li>Conduction block-CIDP,GBS,MMN </li></ul><ul><li>EMG -muscle denervation changes </li></ul><ul><li>Sensory threshold </li></ul><ul><li>Thermal & vibration threshold </li></ul>
  49. 49. <ul><li>IMAGES </li></ul><ul><li>CXR-sarcoidosis,malignancy </li></ul><ul><li>Skeletal survey-multiple myeloma </li></ul><ul><li>Screening for malignancy </li></ul><ul><li>AUTONOMIC FUNCTION TESTS </li></ul><ul><li>Diagnostic tests imp in </li></ul><ul><li>Asymmetric,motor predominant,rapid onset,demyelinating neuropathy </li></ul>
  50. 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. 51. <ul><li>ETIOLOGICAL CLASSIFICATION </li></ul><ul><li>1. metabolic -DM,amyloidosis,porphyria </li></ul><ul><li>2. infections -leprosy,HIV,CMV,syphilis, </li></ul><ul><li>diphtheria,lymedisease </li></ul><ul><li>3. immune - GBS,CIDN,MMN </li></ul><ul><li>4. hereditary -CMT </li></ul><ul><li>5 .Toxic -drugs,alcohol,heavymetals </li></ul><ul><li>6. vasculitis -PAN,CSS,cryoglobulinemia </li></ul><ul><li>7. paraneoplastic -lung </li></ul><ul><li>8. nutritional - B1,B6,B12 </li></ul>
  52. 52. H/O & EXAMINATION Mononeuropathy EDx Axonal/demyeln? Any systemic disorder Entrapment/compression
  53. 53. MNPthy multiplex axonal Demyeln +focal condcn block Vasculitis/multifocal Nerve biopsy CIDP Paraprotein,HIV,lyme
  54. 54. polyNPthy axonal Sub a/c c/c Toxins/systemic disease family h/o,genetics
  55. 55. demyelination Uniform slowing,C/C Nonuniform slowing,condnblock paraprotein Family h/o,genetics c/c or suba/c-CIDP a/c-GBS
  56. 56. <ul><li>THANK YOU </li></ul>

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