Peripheral Neuropathies

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Peripheral Neuropathies

  1. 1. Peripheral neuropathies Dr. Osman Sadig Bukhari
  2. 2. <ul><li>Peripheral nerve diseases </li></ul><ul><li>1- Mononeuropathies </li></ul><ul><li>2- Multiple mononeuropathies </li></ul><ul><li>(mononeuritis multiplex) </li></ul><ul><li>3- Polyneuropathies </li></ul>
  3. 3. <ul><li>Neuropathy = pathological processes damaging a nerve or nerves. The mechanisms </li></ul><ul><li>of damage may be:- </li></ul><ul><li>1- Demyelination e.g. GB, diphth, HSMN </li></ul><ul><li>2- Axonal degeneration : e.g. Toxic neuropathies </li></ul><ul><li>3- Compression : Causes segmental demyelinatio </li></ul><ul><li>e.g. Entrapment N. </li></ul><ul><li>4- Vasculopathy (infarction): Causes distal Wallerian degenerat e.g. DM, collagen diseases e.g. PAN. </li></ul><ul><li>5- Infiltration : e.g. leprosy, malig, sarcoidosis. </li></ul>
  4. 4. <ul><li>** With axonal degen nerve conduction velocity </li></ul><ul><li>is normal or slightly reduced & EMG shows </li></ul><ul><li>evidence of muscle denervation. </li></ul><ul><li>** With demyelination conduction velocity may </li></ul><ul><li>be slowed considerably or blocked in severe </li></ul><ul><li>cases without accompanying muscle denerv. </li></ul><ul><li>**- Mononeuropathy e.g. compression </li></ul><ul><li>- Multiple mononeuropathies e.g. vasculopath </li></ul><ul><li>infiltr, radiation </li></ul><ul><li>- Polyneuropathies e.g. hereditary, metab </li></ul><ul><li>toxic, GB, non metastatic manif of malig </li></ul>
  5. 5. <ul><li>Poly neuropathies </li></ul><ul><li>Causes </li></ul><ul><li>1- Inherited neuropathies : </li></ul><ul><li>- Charcot - Marie- Tooth disease </li></ul><ul><li>- Dejerine- Sottas disease </li></ul><ul><li>- Refsum,s disease </li></ul><ul><li>- Acute intermittent porphyria </li></ul><ul><li>- Friedreich,s ataxia </li></ul>
  6. 6. <ul><li>2- Metab & endocrine: </li></ul><ul><li>- DM </li></ul><ul><li>- Uraemia </li></ul><ul><li>- Chronic liver failure </li></ul><ul><li>- Hypothyroidism </li></ul><ul><li>- Acromegaly </li></ul><ul><li>- Amyloidosis </li></ul><ul><li>- Para & cryoproteinaemia </li></ul>
  7. 7. <ul><li>3- Toxic neuropathies : </li></ul><ul><li>- Alcohol </li></ul><ul><li>- Drugs ( INH, phenytoin, vincristine, ) </li></ul><ul><li>- Heavy metals (lead, arsenic, Hg) </li></ul><ul><li>- Organic solvents (acryl amide, organophos) </li></ul>
  8. 8. <ul><li>4- Infective: </li></ul><ul><li>- Leprosy </li></ul><ul><li>- Diphtheria </li></ul><ul><li>- HIV </li></ul><ul><li>- H. zoster </li></ul>
  9. 13. <ul><li>5- Inflammatory : </li></ul><ul><li>- Guillian – Barre’ syndrome. </li></ul><ul><li>- Chronic demyelinating polyneuropathy </li></ul><ul><li>- Idiopathic chronic sensorimotor neuropathy </li></ul><ul><li>- Connective tissue diseases </li></ul><ul><li>- Sarcoidosis </li></ul><ul><li>- Lyme borreliosis </li></ul>
  10. 14. <ul><li>6- Vitamin deficiency : </li></ul><ul><li>- B12 - B1 - B6 - Folate </li></ul><ul><li>- Nicotinic acid - Vit E </li></ul>
  11. 15. <ul><li>7- Neuropathy associated with malignant disease </li></ul><ul><li>8- Neuropathy associated wz critically ill pts </li></ul>
  12. 16. <ul><li>Modalities of polyneuropathies </li></ul><ul><li>- Sensory </li></ul><ul><li>- Motor </li></ul><ul><li>- Mixed </li></ul><ul><li>- Autonomic </li></ul>
  13. 17. <ul><li>C/F of Polyneuropathies </li></ul><ul><li>1- Sensory dysfn: numbness, paraesthesiae, </li></ul><ul><li>hyperaesthesia & pain starting distally and </li></ul><ul><li>ascending proximally in gloves & stockings </li></ul><ul><li>with impaired perception of pain, touch, temp </li></ul><ul><li>vibration & position sense. </li></ul><ul><li>2- Motor dysfn : flaccid weakness most marked </li></ul><ul><li>distally. </li></ul><ul><li>3- tendon reflexes : depressed or absent. </li></ul>
  14. 18. <ul><li>4- Autonomic neuropathy : orthostatic hypot </li></ul><ul><li>dysphagia, gastro paresis, gustatory sweating </li></ul><ul><li>( facial sweating wz anhydrosis of the feet), </li></ul><ul><li>noct. diarrhoea, urine retention wz overflow </li></ul><ul><li>incontinence, failure of erection, resting tachycardia, fixed HR, C/R arrest, dependant </li></ul><ul><li>oedema wz cold feet, small non reactive pupils </li></ul><ul><li>- Causes : - DM - GB </li></ul><ul><li>- Acute intermittent porphyria </li></ul><ul><li>- Amyloidosis - Drugs </li></ul>
  15. 19. <ul><li>Investigations : guided by sympt & signs </li></ul><ul><li>The cause of polyneuropathy is suggested by </li></ul><ul><li>the history including the onset, FH, PMH, DH, SH </li></ul><ul><li>and za predominant clinical manifestations . </li></ul><ul><li>- CBC & ESR </li></ul><ul><li>- Renal profile & liver biochemistry </li></ul><ul><li>- Blood glucose & thyroid fns. </li></ul><ul><li>- ANF & RF </li></ul><ul><li>- Plasma electrophoresis </li></ul><ul><li>- Urinary levels of heavy metals. </li></ul><ul><li>- CSF </li></ul><ul><li>- CXR </li></ul>
  16. 20. <ul><li>- Serum lipids, lipo proteins, cry proteins </li></ul><ul><li>- Vitamins assay </li></ul><ul><li>- Urinary porphyrins </li></ul><ul><li>- Genetics </li></ul><ul><li>- Search for malig e.g. radiology, immaging, </li></ul><ul><li>PSA, stools for occult blood, endoscopy, </li></ul><ul><li>mamography - Nerve conduction studies </li></ul><ul><li>- EMG </li></ul><ul><li>- Nerve biopsy. </li></ul>
  17. 21. <ul><li>Inherited neuropathies </li></ul><ul><li>1- Charcot Marie Tooth syndrome </li></ul><ul><li>- Mainly AD </li></ul><ul><li>- presents in early adult life wz gait disturba </li></ul><ul><li>or foot deformities. Slow nerve degeneratio </li></ul><ul><li>lead to polyneuropathy wz distal weakness </li></ul><ul><li>and wasting (inverted champagne bottle), </li></ul><ul><li>variable sensory loss, absent kn & An jerks, </li></ul><ul><li>high steppage gait due to foot drop </li></ul><ul><li>- Pes cavus & claw feet +/- optic atrophy & deafness </li></ul><ul><li>- Dis arrested in middle life. Normal life span. </li></ul>
  18. 22. <ul><li>2- Dejerine Sottas disease </li></ul><ul><li>- AR or sporadic </li></ul><ul><li>- Progressive motor & sensory polyn with </li></ul><ul><li>weakness, ataxia, sensory loss & depressed </li></ul><ul><li>or absent reflexes </li></ul><ul><li>- palpable nerves wz segmental demyelination </li></ul><ul><li>- High CSF proteins due to obstruction by </li></ul><ul><li>nerve roots </li></ul>
  19. 23. <ul><li>3- Refsum’s disease </li></ul><ul><li>- AD </li></ul><ul><li>- Due to disturbance of phytanic acid metab. </li></ul><ul><li>- Pigmentary retinal degeneration with </li></ul><ul><li>progressive sensorimotor polyn & cerebellar </li></ul><ul><li>signs. Auditory dysfn, cardiomyopathy and </li></ul><ul><li>cutaneous manifestations </li></ul><ul><li>- Marked reduction of conduction velocity </li></ul><ul><li>- TR by restriction of phytanic acid and its </li></ul><ul><li>precursors. </li></ul>
  20. 24. <ul><li>4- Acute intermittent porphyria </li></ul><ul><li>- Mainly motor, proximal & UL > LL </li></ul><ul><li>- Sensory manifestations may occur </li></ul><ul><li>- Axonal in type </li></ul><ul><li>-High CHO diet & conc. dextrose is helpful and </li></ul><ul><li>propranolol for tachycardia & HT. </li></ul>
  21. 25. <ul><li>Neuropathies associated with metab </li></ul><ul><li>and endocrine disorders </li></ul><ul><li>1- Diabetes mellitus (occur singly or in combin) </li></ul><ul><li>- Symmetric sensory or mixed polyn </li></ul><ul><li>- Asymmetric motor radiculopathy </li></ul><ul><li>( diabetic amyotrophy) </li></ul><ul><li>- Mononeuritis or mononeuritis multiplex </li></ul><ul><li>- Autonomic </li></ul>
  22. 26. <ul><li>2- Uraemia </li></ul><ul><li>- Progressive sensorimotor polyn, LL > UL </li></ul><ul><li>- Improves wz successful RT & to a lesser </li></ul><ul><li>by chronic HD </li></ul>
  23. 27. <ul><li>Toxic neuropathies </li></ul><ul><li>- Alcoholic polyn </li></ul><ul><li>- Distal sensorimotor polyn frequently accomp </li></ul><ul><li>by painful cramps, muscle tenderness and </li></ul><ul><li>painful paraesthesia, often marked in za legs </li></ul><ul><li>- Autonomic </li></ul><ul><li>- May respond to B1 </li></ul><ul><li>- Recurs or progress wz alcohol intake </li></ul><ul><li>- Similar distal sensorimotor polyn occurs in </li></ul><ul><li>beri beri (thiamine def) </li></ul>
  24. 28. <ul><li>Vitamin deficiency </li></ul><ul><li>- Def states occur in malnutrition </li></ul><ul><li>- preventable </li></ul><ul><li>- potentially reversible if treated early </li></ul><ul><li>1- B12 def </li></ul><ul><li>- Distal sensory polyn, sp proprioception </li></ul><ul><li>- Absent ankle jerk </li></ul><ul><li>- Extensor planter </li></ul><ul><li>- Optic neuropathy </li></ul><ul><li>- Intellectual dysfn </li></ul>
  25. 29. <ul><li>2- Thiamine def ( beri beri) </li></ul><ul><li>- polyn </li></ul><ul><li>- cardiac failure </li></ul><ul><li>Werneckes Korsakoff psychosis (nystagmus, </li></ul><ul><li>ophthalmoplegia, ataxia, amnesia, confusion </li></ul><ul><li>coma </li></ul><ul><li>- Parental B1 for TR </li></ul><ul><li>3- Pyridoxine def (B6) </li></ul><ul><li>- Mainly sensory </li></ul><ul><li>- More common in slow acetylaters on INH. </li></ul><ul><li>- 10mg per day for TR </li></ul>
  26. 30. <ul><li>Infective neuropathies </li></ul><ul><li>1- Leprosy </li></ul><ul><li>- In TL leads to hypo pigmented anaethetic </li></ul><ul><li>patches & involves peripheral nerves of </li></ul><ul><li>predeliction which are thickened </li></ul><ul><li>- In LL leads to gloves & stockings sensory </li></ul><ul><li>loss </li></ul><ul><li>- Multiple mononeuropathy </li></ul><ul><li>2- Diphtheric neuropathy (demyelinating) </li></ul><ul><li>- Palatal palsy I 1-2W </li></ul><ul><li>- Loss of accomodation in2-4W </li></ul><ul><li>- polyn in 4-6W </li></ul>
  27. 31. <ul><li>3- AIDS neuropathy </li></ul><ul><li>- Chronic symmetric sensorimotor polyn </li></ul><ul><li>- progressive polyradiculopathy or </li></ul><ul><li>radiculomyelopathy ? CMV </li></ul><ul><li>- Seropositive Pts may also develop </li></ul><ul><li>demyeelinating polyradiculopathy and </li></ul><ul><li>mononeuritis multiplex, </li></ul>
  28. 32. <ul><li>Inflammatory polyneuropathies </li></ul><ul><li>1- Acute post infective polyn </li></ul><ul><li>- 1-4/52 following resp tract infection, </li></ul><ul><li>campylobacter jejuni infection of za gut </li></ul><ul><li>( in 25% of cases, more severe & residual </li></ul><ul><li>deficit), surgery & immunization </li></ul><ul><li>- Demyelination of the spinal roots & periph N has probably immunological basis. </li></ul><ul><li>- Patient presents wz distal weakness and </li></ul><ul><li>numbness ascending over days to involve </li></ul><ul><li>the face, resp muscles & bulbar muscles. </li></ul><ul><li>- Patient may C/O back pain at za onset. </li></ul>
  29. 33. <ul><li>- Clinically there is muscle weakness, areflexia </li></ul><ul><li>and variable sensory loss. Sphincters are </li></ul><ul><li>intact & there is no sensory level. </li></ul><ul><li>- Rapid deterioration wz resp failure may </li></ul><ul><li>occur. </li></ul><ul><li>- Ophthalmoplegia, ataxia & areflexia can be </li></ul><ul><li>a presentation ( Miller Fissure) </li></ul><ul><li>- CSF shows protein/ cell dissociation that </li></ul><ul><li>may take 2-3 W to develop. </li></ul><ul><li>- Nerve conduction velocity is slowed. </li></ul><ul><li>- Investigations to search for a cause e.g CMV </li></ul><ul><li>mycoplasma, campyl (CXR, stool culture and serology) </li></ul>
  30. 34. <ul><li>- DD include diphtheria, pophyria, lead N, </li></ul><ul><li>( pry motor N), botulism, polio & pry muscle disease </li></ul><ul><li>- Treatment: </li></ul><ul><li>- supportive ( ABC, nursing, physioth) </li></ul><ul><li>- mechanical ventilation if resp paralysis </li></ul><ul><li>occur. Monitor resp wz vital capacity. </li></ul><ul><li>- Plasmapheresis & IV Ig if given early </li></ul><ul><li>- Use of C/S is controversial. </li></ul><ul><li>- complete recovery occur in 80% in 3-6 M </li></ul><ul><li>- mortality 4% & 3% relapse. </li></ul><ul><li>- remainder left wz disability. </li></ul>
  31. 35. <ul><li>Predominantly motor neuropathy </li></ul><ul><li>1- GB </li></ul><ul><li>2- Ca neuropath </li></ul><ul><li>3- Charcot Marie tooth disease (peroneal muscular atrophy </li></ul><ul><li>4- lead poisoning </li></ul>
  32. 36. <ul><li>Management of neuropathies </li></ul><ul><li>1- In 1/3 treatable cause: </li></ul><ul><li>- toxins & offending drugs removed </li></ul><ul><li>- Deficiencies & metab abn corrected </li></ul><ul><li>- inflammatory causes by immunosuppression </li></ul><ul><li>2- In 1/3 there is identifiable cause but no </li></ul><ul><li>TR as in hereditary </li></ul><ul><li>3- In 1/3 no specific cause </li></ul><ul><li>-- Physiotherapy & occupational therapy </li></ul>
  33. 37. <ul><li>Mononeuropathies </li></ul><ul><li>1- Acute : sustained pressure e.g. tourniquet </li></ul><ul><li>2- Chronic: entrapment </li></ul><ul><li>Causes according to site of compression </li></ul><ul><li>1- Carpal tunnel Median N </li></ul><ul><li>2- Cubital tunnel Ulnar N </li></ul><ul><li>3- Spiral groove of humerus Radial N </li></ul><ul><li>4- Inguinal ligament Lateral cutaneous of thigh </li></ul><ul><li>5- Neck of fibula Common peroneal N </li></ul><ul><li>6- Flexor retinaculum (tarsal tunnel) Post tibial </li></ul>
  34. 40. <ul><li>Mononeuritis multiplex </li></ul><ul><li>Causes </li></ul><ul><li>1- leprosy (commonest) </li></ul><ul><li>2- DM </li></ul><ul><li>3- vasculitis </li></ul><ul><li>4- sarcoidosis </li></ul><ul><li>5- amyloidosis </li></ul><ul><li>6- malignancy </li></ul><ul><li>7- neurofibromatosis </li></ul><ul><li>8- HIV infection </li></ul><ul><li>9- Idiopathic multifocal motor neuropathy </li></ul>

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