2. neuropathies

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2. neuropathies

  1. 1. Neuropathies Dr. RS Mehta, BPKIHS 1
  2. 2. What is Neuropathy • Neuropathy is a disorder resulting from injury to the peripheral nerves – the motor, sensory and autonomic nerves. • The main function of the peripheral nervous system is to connect the central nervous system (CNS) to the limbs and organs. • Unlike the CNS, the PNS is not protected by the bone of spine and skull, or by the blood brain barrier, leaving it exposed to toxins and mechanical injuries. 2 Dr. RS Mehta, BPKIHS
  3. 3. Review Basic Concept of Nerves Dr. RS Mehta, BPKIHS 3
  4. 4. Synapses Dr. RS Mehta, BPKIHS 4 Figure 11.17
  5. 5. Reflex Arc Spinal cord (in cross-section) Stimulus 2 Sensory neuron 3 Integration center 1 Receptor 4 Motor neuron Skin Interneuron 5 Effector Dr. RS Mehta, BPKIHS Figure 13.14 5
  6. 6. Structure of a Nerve Dr. RS Mehta, BPKIHS Figure 13.3b 6
  7. 7. Regeneration of Nerve Fibers Dr. RS Mehta, BPKIHS 7 Figure 13.4
  8. 8. Cranial Nerve X: Vagus Dr. RS Mehta, BPKIHS 8 Figure X from Table 13.2
  9. 9. Spinal Nerves Dr. RS Mehta, BPKIHS Figure 9 13.6
  10. 10. Brachial Plexus Dr. RS Mehta, BPKIHS Figure 13.9a 10
  11. 11. After the PNS is damaged! • The deterioration of the peripheral nerves disrupts the body’s ability to communicate with its muscles, organs and tissues. • Neuropathy is like the body’s wiring system going haywire, causing unusual or unpleasant irritations including tingling, burning, itchiness, dizziness and many many more! • If ignored, as this is done often these symptoms can lead to numbness at one extreme to unremitting pain at the other. • It can come and go, slowly progress over many years or become severe and debilitating. 11 Dr. RS Mehta, BPKIHS
  12. 12. Neuropathy – a rare illness ??? • Often it is misdiagnosed or thought of merely as a side effect of another disease such as diabetes or cancer or even kidney failure. • It can occur at any age however it is more common among older adults. 12 Dr. RS Mehta, BPKIHS
  13. 13. Neuropathy= pathological processes damaging a nerve or nerves. The mechanisms of damage may be:1. Demyelination e.g. GB Syndrome 2. Axonal degeneration: e.g. Toxic neuropathies 3. Compression: Causes segmental demyelination e.g. Entrapment N. 4. Vasculopathy (infarction): e.g. DM 5. Infiltration: e.g. leprosy, sarcoidosis. Dr. RS Mehta, BPKIHS 13
  14. 14. Neuropathies may affect just one nerve,- a condition called mononeuropathy, or several nerves – called polyneuropathy. The “peripheral nervous system” is part of the nervous system that includes nerves in the face, arms, legs, torso, and some nerves in the skull. In fact, all the nerves which are not located in the central nervous system (which includes the brain and the spinal cord) are peripheral nerves. Dr. RS Mehta, BPKIHS 14
  15. 15. There are four types of neuropathy: 1. Autonomic Neuropathy damage to the nerves that regulate the body functions that a person doesn’t control, including the nerves that regulate heart rate, blood pressure, perspiration and digestion. 2. Peripheral Neuropathy damage to the peripheral nervous system, which transmits information from the brain and spinal cord to every other part of the body. 3. Mononeuritis damage to a single nerve or nerve group, which results in loss of movement, sensation, or other function of that nerve. 4. Mononeuritis Multiplex damage to at least two separate nerve areas. Dr. RS Mehta, BPKIHS 15
  16. 16. A number of conditions can lead to damage of the autonomic nerves and these possible causes are similar to those for peripheral neuropathies. The most common cause is diabetes. Other causes include: – – – – – – – Alcoholism Amyloidosis- abnormal protein buildup in organs Autoimmune diseases Tumors Multiple system atrophy Surgical or traumatic injury to nerves Certain medications, (including chemotherapy drugs and anticholinergics) – Parkinson’s disease and HIV/AIDS Dr. RS Mehta, BPKIHS 16
  17. 17. More than 100 types of peripheral neuropathy have been identified, each with its own characteristic set of symptoms, pattern of development, and prognosis. Dr. RS Mehta, BPKIHS 17
  18. 18. Peripheral Neuropathies Dr. RS Mehta, BPKIHS 18
  19. 19. Peripheral nerve diseases: 1- Mononeuropathies 2- Multiple mononeuropathies (mononeuritis multiplex) 3- Polyneuropathies Dr. RS Mehta, BPKIHS 19
  20. 20. Mononeuropathies Dr. RS Mehta, BPKIHS 20
  21. 21. Mononeuropathies 1- Acute : sustained pressure e.g. tourniquet 2- Chronic: entrapment Causes according to site of compression 1- Carpal tunnel 2- Cubital tunnel Median N Ulnar N Radial N 4- Inguinal ligament Lateral cutaneous of thigh 5- Neck of fibula Common peroneal N 6- Flexor retinaculum (tarsal tunnel) Post tibial 3- Spiral groove of humerus Dr. RS Mehta, BPKIHS 21
  22. 22. Brachial Plexus: Distribution of Nerves Dr. RS Mehta, BPKIHS Figure 22 13.9c
  23. 23. Lumbar Plexus Dr. RS Mehta, BPKIHS 23 Figure 13.10
  24. 24. Sacral Plexus Dr. RS Mehta, BPKIHS Figure 24 13.11
  25. 25. Mononeuritis multiplex Causes 123456789- leprosy (commonest) DM vasculitis sarcoidosis amyloidosis malignancy neurofibromatosis HIV infection Idiopathic multifocal motor neuropathy Dr. RS Mehta, BPKIHS 25
  26. 26. Poly neuropathies Causes: 1- Metab & endocrine: DM Uraemia Chronic liver failure Hypothyroidism Acromegaly Amyloidosis Dr. RS Mehta, BPKIHS 26
  27. 27. 2- Toxic neuropathies: - Alcohol Drugs ( INH, phenytoin, vincristine) Heavy metals (lead, arsenic, Hg) Organic solvents (acryl amide, organophos) Dr. RS Mehta, BPKIHS 27
  28. 28. 3- Infective: - Leprosy Diphtheria HIV H. zoster Dr. RS Mehta, BPKIHS 28
  29. 29. Dr. RS Mehta, BPKIHS
  30. 30. Dr. RS Mehta, BPKIHS
  31. 31. Dr. RS Mehta, BPKIHS
  32. 32. Dr. RS Mehta, BPKIHS
  33. 33. 4.Inflammatory: - Guillian – Barre’ syndrome. Chronic demyelinating polyneuropathy Idiopathic chronic sensorimotor neuropathy Connective tissue diseases Sarcoidosis Dr. RS Mehta, BPKIHS 33
  34. 34. 5- Vitamin deficiency: - B12 - B1 - B6 - Folate - Nicotinic acid - Vit E Dr. RS Mehta, BPKIHS 34
  35. 35. 6- Neuropathy associated with malignant disease 7- Neuropathy associated with critically ill pts Dr. RS Mehta, BPKIHS 35
  36. 36. Modalities of polyneuropathies: - Sensory - Motor - Mixed - Autonomic Dr. RS Mehta, BPKIHS 36
  37. 37. C/F of Polyneuropathies 1- Sensory dysfn: numbness, paraesthesiae, hyperaesthesia & pain starting distally and ascending proximally in gloves & stockings with impaired perception of pain, touch, temp vibration & position sense. 2- Motor dysfn: flaccid weakness most marked distally. 3- tendon reflexes: depressed or absent. Dr. RS Mehta, BPKIHS 37
  38. 38. 4- Autonomic neuropathy: gastro paresis, gustatory sweating, noct. diarrhoea, over flow incontinence, failure of erection, resting tachycardia Causes: - DM - GB - Acute intermittent porphyria - Amyloidosis - Drugs Dr. RS Mehta, BPKIHS 38
  39. 39. Investigations: guided by sympt & signs The cause of polyneuropathy is suggested by the history including the onset, FH, PMH, DH, and predominant clinical manifestations. - CBC & ESR - Renal profile & liver biochemistry - Blood glucose & thyroid fns. - Plasma electrophoresis - Urinary levels of heavy metals. - CSF - CXR Dr. RS Mehta, BPKIHS 39
  40. 40. - Serum lipids, lipo proteins, cry proteins - Vitamins assay - Genetics - Search for cause e.g. radiology, immaging, stools for occult blood, endoscopy, mamography - Nerve conduction studies - EMG - Nerve biopsy. Dr. RS Mehta, BPKIHS 40
  41. 41. Tests to evaluate neuropathy • Electrodiagnositic Tests 1. Nerve Conduction Studies - evaluates how the nerves transmit electrical stimuli. 2. Electromyography – (EMG) measures the electrical activity of muscles in response to nerve stimulation. • Skin Biopsy – Small Fiber Neuropathy cannot be diagnosed with EMG and nerve conduction studies that only measure the large fibers. 41 Dr. RS Mehta, BPKIHS
  42. 42. Other Investigations:  Diabetes  Autoimmunity  Infections  Nutritional Deficiencies  Toxins  Hereditary Conditions  Certain Cancer and Cancer Treatments 42 Dr. RS Mehta, BPKIHS
  43. 43. Neuropathies associated with metab and endocrine disorders Diabetes mellitus (occur singly or in combin) - Symmetric sensory or mixed polyn - Asymmetric motor radiculopathy ( diabetic amyotrophy) - Mononeuritis or mononeuritis multiplex - Autonomic Dr. RS Mehta, BPKIHS 43
  44. 44. Toxic neuropathies - Alcoholic polyn - Distal sensorimotor polyn frequently accomp by painful cramps, muscle tenderness and painful paraesthesia in legs - Autonomic - May respond to B1 - Recurs or progress with alcohol intake - Similar distal sensorimotor polyn occurs in beri beri (thiamine def) Dr. RS Mehta, BPKIHS 44
  45. 45. Vitamin deficiency - Def states occur in malnutrition - preventable - potentially reversible if treated early 1- B12 def - Distal sensory polyn Absent ankle jerk Extensor planter Optic neuropathy Intellectual dysfn Dr. RS Mehta, BPKIHS 45
  46. 46. 2- Thiamine def ( beri beri) - polyn - cardiac failure - nystagmus, ophthalmoplegia, ataxia, amnesia, confusion, coma - Parental B1 for Rx 3- Pyridoxine def (B6) - Mainly sensory - More common in slow acetylaters on INH. - 10mg per day Dr. RS Mehta, BPKIHS 46
  47. 47. Infective neuropathies 1- Leprosy - peripheral nerves are thickened - In LL leads to gloves & stockings sensory loss - Multiple mononeuropathy 2- Diphtheric neuropathy (demyelinating) - Loss of accomodation in 2-4 W - polyn in 4-6 W Dr. RS Mehta, BPKIHS 47
  48. 48. 3- AIDS neuropathy - Chronic symmetric sensorimotor polyn - progressive polyradiculopathy or radiculomyelopathy ? CMV - Seropositive Pts may also develop demyeelinating polyradiculopathy and mononeuritis multiplex Dr. RS Mehta, BPKIHS 48
  49. 49. Inflammatory polyneuropathies 1- Acute post infective polyn - 1-4/52 following resp tract infection, ( in 25% of cases, more severe & residual deficit), surgery & immunization - Demyelination of the spinal roots & periph N has probably immunological basis. - Patient presents with distal weakness and numbness ascending over days to involve the face, resp muscles & bulbar muscles. - Patient may C/O back pain. Dr. RS Mehta, BPKIHS 49
  50. 50. - Treatment: - supportive ( ABC, nursing, physioth) - mechanical ventilation if resp paralysis occur. - Monitor resp with vital capacity. - Plasmapheresis & IV Ig if given early - Use of steriod is controversial. - complete recovery occur in 80% in 3-6 M - mortality 4% & 3% relapse. - remainder left with disability. Dr. RS Mehta, BPKIHS 50
  51. 51. Predominantly motor neuropathy 1- GB 2- Ca neuropath 3- Charcot Marie tooth disease (peroneal muscular atrophy 4- lead poisoning Dr. RS Mehta, BPKIHS 51
  52. 52. Management of neuropathies 1- In 1/3 treatable cause: - toxins & offending drugs removed - Deficiencies & metab abn corrected - inflammatory causes by immunosuppression 2- In 1/3 there is identifiable cause but no TR as in hereditary 3- In 1/3 no specific cause -- Physiotherapy & occupational therapy Dr. RS Mehta, BPKIHS 52
  53. 53. • Rx…………… • Drugs: Gabapentin, Amitriptyline etc. • In addition to these medications there are ointments and creams used by patients. • Oxycodone, Hydrocodone and as well as other opiates are often prescribed for neuropathy pain. • Patients have described getting relief from acupuncture, reflexology and others. Dr. RS Mehta, BPKIHS 53
  54. 54. Types of Neuropathy: Summary • • • • • • • • • • • Autonomic Neuropathy Cancer-Related Neuropathies Compressive Neuropathies Diabetic Neuropathy Drug-Induced and Toxic Neuropathy G.I. and Nutrition-Related Neuropathies Hereditary Neuropathies Immune-Mediated Neuropathie Infectious Diseases and Neuropathy Neuropathic Pain Chemotherapy Induced Neuropathy 54 Dr. RS Mehta, BPKIHS
  55. 55. • TENS is delivering tiny electrical impulses to specific nerve pathways through small electrodes placed on skin. TENS • This method is based on teaching a patient to control certain body responses that reduce pain. BIOFEEDBACK • During hypnosis a patient receives suggestions intended to decrease perception of pain. • It is proven that acupuncture can be an effective treatment for chronic pain, including the pain of neuropathy. ACUPUNCTURE • Designed to help reduce the muscle tension. They range from deepbreathing exercises to visualization, yoga and meditation. HYPNOSIS Dr. RS Mehta, BPKIHS RELAXATION TECHNIQUES 55
  56. 56. Thank you Dr. RS Mehta, BPKIHS 56
  57. 57. Dr. RS Mehta, BPKIHS 57
  58. 58. Simplified diagram of lumbosacral plexus. Contribution of L1 root is not shown. Lumbosacral trunk or cord is shown. Dr. RS Mehta, BPKIHS 58
  59. 59. Dr. RS Mehta, BPKIHS Origin, course, and distribution of sciatic nerve 59
  60. 60. Components • • • • • 12 pair of Cranial nerves 31 pair of Spinal nerves Sympathetic trunks Ganglia Splanchnic nerves Dr. RS Mehta, BPKIHS 60
  61. 61. What exactly is the deficit – Sensory • • • • • • Pain Touch Pressure Temperature Vibration Position •Spinothalamic •Posterior Column Dr. RS Mehta, BPKIHS 61
  62. 62. What exactly is the deficit – Motor Test: Abd D Minimi Ulnar nerve supplies: Claw Hand Hypothenar muscles Card Test 3,4 Lumbricals All interossi Book Test Adductor Pollicus Anatomy Based Tests Dr. RS Mehta, BPKIHS 62
  63. 63. What is the effect of the loss – wasting, residual paralysis, deformity, contractures, sores Dr. RS Mehta, BPKIHS Restoring: Dorsi-flexion of wrist in Erbs Palsy. Wrist extensors have been isolated and tested will now be powered by suturing Pronator Teres 63 insertion to

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