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Approach to Peripheral Neuropathy

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Approach to Peripheral Neuropathy

  1. 1. Approach to Peripheral Nerve Disease Dr N Anand
  2. 2. •History –Questions to be asked •Examination-What are typical Signs that implicate Peripheral Nerve from other causes •Electrodiagnostic Studies •Diagnosis and Treatment
  3. 3. Symptoms Early features Distal numbness and tingling Distal neuropathic pain Gait imbalance Toe weakness Latter features Progression of numbness and tingling to proximal body parts Prominent neuropathic pain Tripping easily Worsening of gait frequent falls Complaints
  4. 4. Motor Symptoms Positive Muscle Cramps,Fasciculations,Myokymia(or Tremors) Negative Weakness, Atrophy, Walking Difficulty Difficulty in turning keys in locks, unfasten button and opening bottles and jars Sensory Symptoms Positive Paresthesia, Band-like sensation on feet or trunk,Stumbling, Tingling Pain- Prickling, Searing, Burning,Pins and Needles Neuropathic Pain Allodynia, Hyperalgesia Negative Numbness, Lack of feeling/Loss of sensation, Walking on cotton wool
  5. 5. Autonomic Symptoms • Anhidrosis • Orthostatic Hypotension • Intolerance to light • Lack of tear and saliva • Sexual impotence • Bladder and Bowel dysfunction • Gastroparesis • Heat Intolerance
  6. 6. Temporal evolution?
  7. 7. Onset, Duration and Evolution of symptoms Acute(days to 4 weeks) GBS,Vasculitis,Radiclupathies,Toxic neuropathies,Acute intermittent Porphyria) Subacute (4-8 weeks) Chronic(>8 weeks) Most Neuropathies (Diabetes,CRF,CIDP,Paraneoplastic, Hereditary motor sensory neuropathies) Course Monophasic Progressive Relapsing CIDP,Porphyria,Toxic,HIV/AIDS,
  8. 8. Relevant History
  9. 9. Clues to diagnosis • Viral illnesses • lifestyle, and work and occupational exposure • Alcohol abuse, vitamin deficiencies, and dietary habits • Use of over-the-counter drugs, Vitamin B6 and zinc consumption • Gastric bypass surgery(Copper Deficiency) • Medications prescribed in the past • HIV infection • Diabetes
  10. 10. • Previous Diseases- amyloidosis, a history of thyroid disease, chronic renal and liver disease, • Malignancy, previous treatment with chemotherapeutic agents • Connective tissue disorders • recreational use of substances, and exposure to heavy metals, industrial agents, herbicides, and pesticides • foreign travel(leprosy) • A detailed family history for presence of hammer toes, high arches, weak ankles, gait abnormalities or “muscular dystrophy,” suggesting a longstanding or hereditary neuropathy. • possibility of a tick bite (Lyme disease) • it is not uncommon to find more than one in the same patient, such as diabetes, alcohol abuse, and vitamin b 12 deficiency
  11. 11. Drugs causing Neuropathies Axonal Demyelinating Vincristine Paclitaxel Nitrous oxide Colchicine (Probenecid, Col- Probenecid) Isoniazid Hydralazine Metronidazole Pyridoxine Didanosine Lithium Alfa interferon Dapsone Phenytoin Cimetidine Disulfiram Chloroquine Ethambutol Amitriptyline Amiodarone Chloroquine and Hydroxychloroquine
  12. 12. Examination
  13. 13. Examination • Abnormal Sensation especially Distal • Weakness(typically Distal,but may be proximal/both) • Normal Muscle Tone-No Spasticity • Absent Tendon Reflexes • Abnormal Gait • Is there a evidence of UMN involvement- Consider combined system degeneration with neuropathy-Vitamin B 12 deficiency,copper deficiency,HIV,Severe Hepatic Disease,adrenomyeloneuropathy)
  14. 14. Signs Early Signs Distal sensory loss to cold, pinprick, and/or vibration Reduced or lost ankle reflex Romberg sign Impaired tandem walking Toe extensor weakness Latter features Distal loss of cold, pinprick, vibration, and joint position sense Areflexia at ankles and knees Footdrop Inability to toe-and-heel walk
  15. 15. Cranial Nerve Involvement
  16. 16. Cranial Nerve Involvement • External ophthalmoplegia - Miller Fisher syndrome • Trigeminal sensory loss - Sjogren's syndrome • Lower cranial nerve palsy with gynecomastia-Kennedy's syndrome • Facial nerve palsy –GBS, Leprosy, Lyme Disease, Sarcoidosis, HIV
  17. 17. Distribution-Focal or multifocal
  18. 18. • Mononeuropathy, the neurological deficit follows the distribution of a single nerve. Eg- foot drop due to a common fibular (peroneal) nerve lesion(sensory loss is restricted to the lower two-thirds of thelateral leg and dorsum of the foot) Causes-Leprosy,Entrapment neuropathy,trauma • Multiple mononeuropathies (mononeuropathy multiplex), the neurological findings should point to simultaneous or sequential damage to two or more noncontiguous peripheral Nerves. Eg- Diabetes,Vasculitis
  19. 19. Causes of Multiple Mononeuropathies Axonal injury Demyelination Vasculitis (systemic, nonsystemic) Diabetes mellitus Sarcoidosis Hansen disease (leprosy) Human immunodeficiency virus 1 infection Multifocal motor neuropathy Multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) Multiple compression neuropathies (hypothyroidism, diabetes) Hereditary neuropathy with liability to pressure palsies
  20. 20. Motor or Sensory Symmetric or Asymmetric Proximal or Distal
  21. 21. Distribution of Motor and Sensory Involvement • Predominant Motor Guillain-Barré syndrome Chronic inflammatory demyelinating polyradiculoneuropathy Neuropathy with osteosclerotic myeloma Diabetic lumbar radiculoplexopathy(Amyotrophy) Hereditary motor sensory neuropathies (Charcot-Marie-Tooth disease) Porphyria Lead intoxication Multifocal motor neuropathy Paraneoplastic Acute motor axonal neuropathy
  22. 22. Asymmetric Weakness Without Sensory Loss • -a motor neuronopathy such as motor neuron disease or multifocal motor neuropathy. Symmetric Weakness without sensory loss • Proximal and Distal- Spinal Muscular Atrophy • Distal-Hereditary Motor Neuropathy Symmetric with predominant Motor(Both Proximal and Distal) • AIDP • CIDP
  23. 23. Predominant Sensory Loss • Leprosy • Drugs(Vincristine,NFT,INH) • Diabetes Mellitus • Amyloidosis • Alcohol • Vitamin B12 • Sjogrens Syndrome
  24. 24. Sensory Involvement Symmetric sensory Loss(with/without distal weakness) • Diabetes, carcinoma, Sjögren syndrome, dysproteinemia, acquired immunodeficiency syndrome (AIDS), Vitamin B 12 deficiency, celiac disease • Inherited and idiopathic sensory neuropathies(CSPN) • Intoxications-Cisplatin, thalidomide, or pyridoxine.
  25. 25. Asymmetric Sensory Loss with Distal Weakness Involvement of Multiple Nerves - Mulitifocal CIDP, Vasculitis, Cryglobulinemia, Amyloidoisis,Sarcoid Infectious (leprosy, Lyme, hepatitis B, C, or E, HIV, CMV) Tumor infiltration Hereditary Neuropathy with liability to pressure palsies Involvement of single Nerves/Region Compressive mononeuropathy, plexopathy, or radiculopathy
  26. 26. Course of sensory and motor dysfunction in Polyneuropathies • Distal gradient sensory loss from the toes to proximal legs. This stocking- and-glove pattern is characteristic of axonal neuropathy(length dependent). By the time sensory disturbance reaches the shin, dysesthesias develop in fingers. • They are relatively symmetric. • Strength is lost in a similar pattern to the sensory loss but usually arises later in the evolution of the illness. • Usually affects the extensor muscles of the toes more than the flexor muscles. • In early motor involvement, the examiner may not find objective weakness, and the patient’s only symptom may be the inability to walk on his heels or slapping of feet suggestive of early ankle dorsiflexion weakness. • Unsteadiness of gait may be out of proportion to the muscle weakness because of proprioceptive loss
  27. 27. • As the disease advances, patients will often develop a foot drop from weakness of the anterior tibial muscles. • As the motor involvement progresses, patients develop hand weakness. This often manifests as atrophy of the intrinsic hand muscles and weakness of spreading and opposition of the fingers. • Reflexes are diminished or lost progressing from distal to -proximal. Ankle reflexes are lost first, followed by the knee, brachioradialis, and lastly, the biceps brachii and triceps. • In early neuropathy, ankle and knee reflexes are lost even with preserved muscle bulk and strength.
  28. 28. Axonal Neuropathy Demyelinating Neuropathy Usually Gradual and insidious Onset Usually Acute or subacute Large and long long axons are affected early, hence initially lower extremeties are affected Diffuse process. Starts in lower limbs.But not always distal Stocking-glove sensory motor loss results in symmetrical distal clinical signs in legs and arms Generalized Weakness and mild sensory loss. Distal involvement Proximal and distal involvement Ankle jerk lost early and proximal tendon reflexes preserved All reflexes are lost early Muscle wasting Common Relatively absent CSF Proteins normal CSF Proteins elevated(since nerve roots are involved Slow Recovery Rapid Recovery Residual deformity Common Residual deformity less common Normal Conduction normal or slightly lowered Nerve Conduction is slowed
  29. 29. Modalities of sensation loss • Loss of sensation in peripheral neuropathies often involves all sensory modalities. • The impairment may be restricted to selective sensory modalities which correlates the type of sensory loss with the diameter size of affected afferent fibers • Pain and temperature sensation are mediated by unmyelinated and small myelinated Aδ fibers,whereas vibratory sense, proprioception, and the afferent limb of the tendon reflex are subserved by large myelinated Aα and Aβ fibers. • Light touch is mediated by both large and small myelinated fibers. • Autonomic Functions are also small unmyelinated fibres
  30. 30. Type of Fiber Involved
  31. 31. Small Fiber Neuroathy Diminished pain and temperature sensation predominate alongwith spontaneous burning pain, painful dysesthesias, and autonomic dysfunction with preservation of tendon reflexes, balance, and motor strength. • Diabetes mellitus and impaired glucose tolerance • Amyloid neuropathy (early familial and primary) • Alcoholic Polyneuropathy • HIV-associated distal sensory neuropathy • Hereditary sensory and autonomic neuropathies • Fabry disease • Tangier disease • Sjögren (sicca) syndrome • Cryptogenic small-fiber neuropathy
  32. 32. Large-fiber sensory loss • Sensory ataxia • Loss of joint position and vibration sense • Areflexia • Romberg Positive • Affected patients will note imbalance, especially in the dark • Striking sensory ataxia, together with pseudoathetosis or asymmetrical truncal or facial sensory loss, directs attention to a primary disorder of sensory neurons or poly ganglionopathies • A dramatic loss of proprioception with vibration loss and normal strength points to a sensory neuronopathy/ganglionopathy. The loss is asymmetric or affects the arms more than the legs, this pattern suggests a non-length- dependent process.
  33. 33. Small Fiber Neuropathy Large Fiber Neuropathy Loss of Pain and Temperature Loss of touch,vibration and position sense-Sensory ataxia Preservation of touch,vibration and pressure Preservation of Pain and Temperature Relative Preservation of Reflexes and motor function Reflexes lost early and motor functions impaired Spontaneous pain and Autonomic Dysfunction No Such phenomenon Electrophysiologically silent Quantitative sensory testing and skin biopsy are used Impaired nerve conduction velocity
  34. 34. Sensory Ataxic Neuropathies Sensory neuropathies (polyganglionopathies) • Paraneoplastic sensory neuronopathy (malignant inflamma- • tory sensory polyganglionopathy): • Sjögren syndrome • Idiopathic • Toxic (cisplatin and analogs, vitamin B 6 excess) Chronic immune sensory polyradiculopathy Demyelinating polyradiculoneuropathies: Guillain-Barré syndrome (Miller Fisher variant) Immunoglobulin M monoclonal gammopathy of undetermined significance Tabes dorsalis produces severe ataxia with damage to the sensory nerve fibers at the root entry zone of the dorsal roots.
  35. 35. Autonomic Involvement
  36. 36. Neuropathies with Autonomic Nervous System Involvement Acute Guillain-Barré syndrome Porphyria Toxic: vincristine, Vacor (rodenticide) Acute Pandysautonomic neuropathy(idiopathic,paraneoplastic) Chronic Diabetes mellitus Amyloid neuropathy (familial and primary) HIV virus–related autonomic neuropathy Paraneoplastic sensory neuropathy Hereditary sensory and autonomic neuropathy
  37. 37. Other Findings
  38. 38. Nerve Thickening Palpation of peripheral nerves for thickening Hypertrophy of a single nerve trunk suggests either • a neoplastic process (e.g., neurofibroma, schwannoma, malignant nerve sheath tumor) • localized perineurial hypertrophic neuropathy. Generalized or multifocal nerve hypertrophy is found in a limited number of peripheral nerve disorders including leprosy(radial and greater auricular), Neurofibromatosis, Charcot-Marie-Tooth (CMT) disease types 1 and 3, (ulnar,peroneal nerve), acromegaly, Refsum disease, and rarely CIDP
  39. 39. Deformities and Trophic Changes Hereditary • Pes cavus and hammer toes in CMT disease • Overriding toes and ichthyosis in Refsum disease Chronic Childhood polyneuropathies leads to deformities • Talipes Equinus,Claw Foot,Kyphoscoliosis Anesthetic and immobile limb show tight and shinny skin,thickened subcutaneous tissue,curved nails and diminished hair growth. Distal Trophic Ulcers ,Thinning of Bone,Tinning of phallanges, pathologic fractures or neuropathic arthropathy in long standing neuropathies
  40. 40. Neuropathies with Skin, Nail, or Hair Manifestations Vasculitis Purpura, livedo reticularis Arsenic or thallium intoxication Mees lines Thallium poisoning Alopecia Cryoglobulinemia Purpura Leprosy Skin hypopigmentation Osteosclerotic myeloma (POEMS syndrome) Sskin hyperpigmentation or hypertrichosis Fabry disease Angiokeratomas Giant axonal neuropathy Curled hair
  41. 41. Investigations
  42. 42. Electrodiagnosis Confirms diagnosis of neuropathy Helps in differentiating • Neuropathy vs myopathy • Root/Plexus vs Distal Nerve trunk involvement • UMN vs LMN weakness • Axonal vs Demyelinating Nature, activity and prognosis Anatomy(which nerves are involved) Characterization of disorder of neuromuscular junction Identification of chronic partial denervation,fasciculations and myotonia especially in muscles of normal bulk and strength
  43. 43. Evaluation of peripheral neuropathy,following electrodiagnosis tests are performed •Nerve conduction study of sensory and motor nerves •Late responses (F response and H reflex) •Needle electromyography (EMG)
  44. 44. Conduction block refers to a decline in the compound muscle action potential exceeding 20% on proximal stimulation compared to that on distal stimulation Demyelinating neuropathy - • Slowing of nerve conduction velocity, • Prolongation of terminal latency, • Temporal dispersion and • Conduction block are consistent Uniform demyelination favors inherited neuropathythy Findings with difference between nerves and segments of the same nerve are more in favor of acquired demyelination
  45. 45. Axonal neuropathy- • Mild slowing of nerve conduction due to a fall out of large-diameter axons, whereas the remaining axons may have normal nerve conduction. • Reduced CMAP amplitude • Fibrillations on EMG. Sensory nerve action potentials and sensory conduction velocities are reduced in both axonal and demyelinating neuropathies Routine sensory nerve conduction studies assess only large myelinated fibers. It is entirely normal in selective small fiber neuropathies. Quantitative sensory testing assessing cold and heat-pain thresholds, tests of sudomotor function, and skin biopsy with analysis of intraepidermal nerve fibre density may be helpful in confirming the unmyelinated nerve fibre abnormalities
  46. 46. F Waves • F waves are used for evaluating conduction problems in the proximal segments of nerves,plexus,nerve roots and spinal cord. A strong electrical stimulus (is applied to the skin surface above the distal portion of a nerve so that the impulse travels both distally (towards the muscle fiber) and proximally (back to the motor neurons of the spinal cord). • When the orthodromic stimulus reaches the muscle fiber, it elicits a strong M-response indicative of muscle contraction. • When the antidromic stimulus reaches the motor neuron cell bodies, a small portion of the motor neurons backfire and orthodromic wave travels back down the nerve towards the muscle. This reflected stimulus evokes small proportion of the muscle fibers causing a small, second CMAP called the F wave.
  47. 47. Laboratory Investigations Blood- • TC,DC,ESR,Urea,Electrolytes,LFT • Blood Sugar • Thyroid Function Tests • Serum Protein electrophoresis • Autoantibodies-ANA,Rhematoid Factor, Antigangliosisde antibodies, Antineuronal Antibodies • Vitamin B12 level and Folate Levels • DNA analysis-Chromosome 17 duplication(HMSN1 and HMS1A)
  48. 48. CSF Analysis- • GBS and CIDP,- elevated cerebral spinal fluid (CSF) protein with no pleocytosis • If cells are present,consider HIV infection, Lyme disease, sarcoidosis, or lymphomatous or leukemic infiltration of nerve roots
  49. 49. • Urine-Bence Jones Protein,Porphyrins • immunoelectrophoresis, or immunofixation -a monoclonal gammopathy in amyloidosis. • antineutrophil cytoplasmic antibodies (ANCA), cryoglobulins, hepatitis serology, • Western blot for Lyme disease, • HIV • Imaging-Xray chest for sarcoidosis and malignancy • Skeletal survey for for multiple myeloma • Screening for malignancy • Autonomic Function tests
  50. 50. Nerve Biopsy Primary indication - suspicion for amyloid neuropathy or vasculitis,Leprosy,Sarcoidosis and leukodystrophies The sural nerve is most commonly biopsied because it is a pure sensory nerve. Used to diagnose a small-fiber neuropathy where EMG/NCS are normal
  51. 51. Alcoholic Neuropathy • Alcoholic neuropathy is defined to be neuropathy following regular intake of more than 100 grams of ethanol daily for at least 10 years, with normal thiamine levels. • Insidious and slowly progressive. • Muscle weakness begins distally and spreads to more proximal muscles. • Gait difficulty, weakness, and muscle cramps are common. • Sensory loss and burning paresthesias starts distally. • Distal muscle wasting, loss of tendon reflexes, and sensory loss of all modalities in a stocking-glove distribution
  52. 52. • In advanced cases, sensory ataxia caused by loss of joint position sense may coexist with alcoholic cerebellar ataxia. • Autonomic dysfunction due to vagal nerve or sympathetic nerve involvement may be present. • EDX studies show an axonal sensorimotor polyneuropathy. Needle EMG shows active denervation with chronic reinnervation in distal muscles
  53. 53. Diabetic Neuropathy • Distal symmetric sensory or sensorimotor polyneuropathy is the MC form • Autonomic neuropathy, • Mononeuropathies. • Diabetic polyradiculopathy is a syndrome characterized by severe disabling pain in the distribution of one or more nerve roots. It may be accompanied by motor weakness. • Involvement of the lumbar plexus or femoral nerve may cause severe pain in the thigh or hip and may be associated with muscle weakness in the hip flexors or extensors (diabetic amyotrophy). Usually self-limited and resolve over 6–12 months
  54. 54. Entrapment Neuropathies Mechanical distortion of the nerve fibers leads to focal demyelination or, in severe cases, to wallerian degeneration. Edx is diagnostic -short-segment conduction delay (i.e., focal slowing) or conduction block across the site of entrapment Common Sites • Median Nerve in Carpal tunnel - Paresthesia, pain, thenar atrophy • Ulnar nerve at the elbow - Clawing and sensory loss of 4th and 5th fingers • Lateral cutaneous nerve of thigh at the inguinal ligament - Sensory loss in lateral thigh • Radial in the spiral groove - Wrist drop, sensory loss • Peroneal Neuropathy - Foot drop, weak ankle eversion,sensory loss in dorsum of foot
  55. 55. Cryptogenic(idiopathic) Sensory and Sensimotor Polyneuropathy • Diagnosis of exclusion • Onset of CSPN is predominantly in the sixth and seventh decades • Both large- and small-fiber loss • Distal numbness, tingling, and often burning pain that begins in the feet and may eventually involve the fingers and hands. • Patients exhibit a distal sensory loss to pinprick, touch, and vibration in the toes and feet, and occasionally in the fingers.
  56. 56. Hereditary Neuropathies The inherited neuropathies can broadly be classified into two groups: • Those in which the neuropathy is the sole or primary part of the disease (e.g., Charcot-Marie-Tooth disease, CMT) • Those in which the neuropathy is part of a more generalized neurological or multisystem disorder. • Charcot-Marie-Tooth Disease is the most coomon type.
  57. 57. Classification of the Inherited Neuropathies Neuropathies in which the neuropathy is the sole or primary part of the disorder Charcot-marie-tooth disease (CMT) Hereditary neuropathy with liability to pressure palsies (HNPP) Hereditary sensory and autonomic neuropathies/hereditary sensory neuropathies (HSAN/HSN) Distal hereditary motor neuropathies (dHMN) Hereditary neuralgic amyotrophy (HNA) Neuropathies in which the neuropathy is part of a more widespread neurological or multisystem disorder Familial amyloid polyneuropathy (FAP) Disturbances of lipid metabolism (e.g., adrenoleukodystrophy) Porphyrias Disorders with defective DNA (e.g., ataxia telangiectasia) Neuropathies associated with mitochondrial diseases Neuropathies associated with hereditary ataxias
  58. 58. Charcot Marie Tooth Disease • MC type • CMT 1 -demyelinating neuropathy, • CMT 2 - the axonal neuropathy • 1st and 2nd decade • Slowly Progressive Distal Weakness • Foot deformity and weakness • Pes Cavus and Hammer toes • Ankle Jerk absent mostly and total areflexia in 50%
  59. 59. HIV 1. Distal symmetric polyneuropathy 2. Inflammatory demyelinating polyneuropathy (including both GBS and CIDP), 3. Multiple mononeuropathies (e.g., vasculitis, CMV-related), 4. Polyradiculopathy (usually CMV-related), 5. Autonomic neuropathy, 6. Sensory ganglionitis.
  60. 60. DSP is the most common form of peripheral neuropathy • Usually seen in patients with AIDS • It is characterized by numbness and painful paresthesias involving the distal extremities. AIDP and CIDP can occur as a complication of HIV infection.lymphocytic pleocytosis is evident in the CSF
  61. 61. Thank You

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