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#1013 Approaching Neuropathies

#1013 Approaching Neuropathies. January 18 to 21 Steven M. Nash, MD Assistant Professor of Clinical Neurology Department of Neurology The Ohio University Medical Center Isabelle Periquet, MD Assistant Professor of Neurology Department of Neurology The Ohio University Medical Center.

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#1013 Approaching Neuropathies

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  1. #1013 Approaching Neuropathies January 18 to 21 Steven M. Nash, MD Assistant Professor of Clinical Neurology Department of Neurology The Ohio University Medical Center Isabelle Periquet, MD Assistant Professor of Neurology Department of Neurology The Ohio University Medical Center

  2. Steven M. Nash, MD Assistant Professor of Clinical Neurology Department of Neurology The Ohio University Medical Center 1

  3. Profile Mr. Winkleman • 67 year old male Symptoms • Falling down frequently • Unexplained weight loss • Low back, hip, and leg pain • Tingling in right side of trunk • Right foot drop 2

  4. Profile Mr. Winkleman Evaluation • Weakness in multiple muscle groups - Asymmetrical from side to side • Less severe sensory loss • EMG showed active, asymmetrical sensory motor polyneuropathy • Sural nerve biopsy revealed vasculitic neuropathy Diagnosis: Vasculitic neuropathy 2A

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  6. Key Points • Neuropathies may present in many different ways • Neuropathy is a result of some other pathology • Treatment requires identification and removal of the underlying cause • Work-up includes a careful history and physical exam, blood work, and EMG 4

  7. Neuropathies • Mononeuropathy (including radiculopathy, plexopathy) • Multiple mononeuropathies • Neuronopathy • Axonal polyneuropathy • Demyelinating polyneuropathy 5

  8. Peripheral Motor Neurons • Cell bodies located in anterior horn of spinal cord or in brainstem nuclei • Axons myelinated • Axons terminate on skeletal muscle fibers 6

  9. Peripheral Motor Neurons • Cell bodies in dorsal root ganglia (pseudounipolar) • Both myelinated (proprioception) and unmyelinated (pain/temperature) axons • Terminate in sensory receptors 7

  10. PeripheralAutonomic Neurons • Cell bodies (second order neurons) in spinal cord nuclei. Axons terminate on third order neurons in autonomic ganglia • Axons are unmyelinated (slow) • Axons of third order neurons terminate in glands and smooth muscle 8

  11. Symptoms of Neuropathy • Numbness • Imbalance • Burning, stinging pain dysesthesia) • Insomnia • Depression • Weakness 9

  12. Signs of Neuropathy • Loss of position and vibration sensitivity • Pain and temperature loss • Romberg sign • Weakness and loss of reflexes • Trophic changes of skin, hair loss, decrease / increase of sweating 10

  13. Common Causesof Neuropathy • Diabetes • Alcohol abuse 11

  14. Presentations ofDiabetic Neuropathy • Mononeuropathy (including cranial nerves, lumbosacral plexus) • Multiple mononeuropathies • Distal sensorimotor polyneuropathy 12

  15. Alcoholic Neuropathies • Direct toxic effect • Secondary nutritional effects (vitamin deficiencies) 13

  16. Uncommon Causesof Neuropathy • Nutritional (vitamin deficiencies) • Guillain-Barre syndrome • Toxic (drugs, hexacarbons, heavy metals) • Hereditary • Rheumatologic disease • Amyloid 14

  17. Other Uncommon Causes • Paraneoplastic (Anti-Hu) • Infection • Systemic disease (uremia, hypothyroid, etc) • Tumors (Especially in neurofibromatosis, type1) 15

  18. Guillain-Barre Syndrome • “Ascending paralysis”, loss of reflexes • Elevated CSF protein, normal cell count • Slowing on nerve conduction studies • Diagnosis often requires high index of suspicion • Most recover with prompt care • Plasmapheresis / IVIg both speed recovery 16

  19. Neuropathy Due toVasculitis • May be isolated to peripheral nerves • Sometimes associated with rheumatologic diseases • Multiple mononeuropathies • Requires nerve biopsy for definite diagnosis • Treated with corticosteroids; cyclophosphamide often required 17

  20. Blood Work in Work-upof Neuropathy Patients • Glucose, BUN, creatinine, liver enzymes, TSH, ESR, hemoglobin A1C, serum protein electrophoresis • ANA, RF, ANCA in selected patients • Gene testing in selected patients • Antibody testing in selected patients 18

  21. Antibody Testingin Polyneuropathies • Anti-Hu useful in sensory neuronopathies • Anti-GM1 only for motor neuropathies in the absence of conduction block on NCS • Anti-MAG, anti-sulfatide not helpful for diagnosis or treatment 19

  22. Electromyography / NerveConduction Studies • Used to evaluate function of the large, myelinated peripheral nerve fibers • All patients with clinical evidence of polyneuropathy should be studied to determine distribution, type (axonal vs. demyelinating), severity, and activity 20

  23. Conclusions • Neuropathies may present in many different ways • Neuropathy is a result of some other pathology • Treatment requires identification and removal of underlying cause • Work-up includes a careful history and physical exam, blood work, and EMG 21

  24. Summary Mr. Winkleman Treatment • IV corticosteroids • Tapering dose of oral Prednisone • Six months of oral Cytoxan • Physical therapy • Not back to baseline, but improving • Re-gained some weight Prognosis: Good 22

  25. Isabelle Periquet, MD Director, Peripheral Neuropathy Center Assistant Professor of Neurology Department of Neurology The Ohio University Medical Center 23

  26. Profile Mrs. Blanton • 57 year old female Symptoms • 8 year history of burning foot pain • Tingling sensations 24

  27. Profile Mrs. Blanton Evaluation • Strength preserved • Reflexes preserved • Sensory testing: - Normal vibration, position and light touch sensation - Diminished pin sensation • Normal EMG Diagnosis: ? 24 A

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  29. Evaluation • Is this a neuropathy or is it something else? • How can I confirm a diagnosis of neuropathy? • What laboratory tests are needed to evaluate for a cause? • How do I treat this patient? 26

  30. Painful Sensory NeuropathyProspective Evaluation Using Skin Biopsy • 140 consecutively referred patients • Inclusion criteria - Pain in the extremities as a primary complaint - No significant weakness - No identified cause 27

  31. Evaluation: EMG / NCS 114 Patients EMG / NCS Abnormal Normal 60/114 (53%) 54/114 (47%) QST AUTO Skin Biopsy 28

  32. Evaluation: QST • Computerized method of determining vibration threshold (large fiber function) and temperature threshold (small fiber function) • QST was abnormal in 72% of patients with normal EMGs 29

  33. Evaluation: Autonomic Testing • Battery of tests evaluating sweat function (QSART), heart rate and blood pressure responses to deep breathing, valsalva and tilt • QSART was abnormal in 59% of patients with normal EMG’s 30

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  35. Evaluation: Blood Studies • Routine blood studies - CBC, lytes, ESR, BUN, Cr, Ca++, LFTs, TSH, HgbA1C, B12 (MMA, HC), chol, TG • Immune / infectious blood studies - HIV, FTA, ANA, ENA, RF, IEP with IF - Nerve antibodies (GM1, MAG, SGPG, Hu, sulfatide) • DNA - PMP22 mutations, FAP (met 30) 32

  36. Differential Diagnosis • Large Fiber Sensory Neuropathy - Hereditary 5 - Connective tissue disease 3 (Sjogren’s, MCTD) - Monoclonal gammopathy 2 - Amyloidosis 2 - Cancer (CML) 1 - Vasculitis (non-systemic) 1 - Ganglionitis 1 - Old GBS 1 - Drug-induced (Taxol) 1 - Creutzfeld-Jacob disease 1 - Idiopathic 42 33

  37. Differential Diagnosis • Small Fiber Sensory Neuropathy - Hereditary 1 - Monoclonal gammopathy 1 - PROMM 1 - Idiopathic 41 • Need also to consider - diabetes, AIDS, uremia, porphyria, Tangier Fabry 34

  38. Treatment • Non-Pharmacologic Measures - TENS - Immersion in cold / warm water - Application of creams (Lidocaine cream) - Massage - Dorsal column stimulation 35

  39. Treatment • Tricylic antidepressants (amitriptyline, nortriptyline, desipramine) • Anticonvulsants (carbamazepine, phenytoin, gabapentin, clonazepam, lamotrigine, topiramate) • Antiarrythmics (mexiletine, lidocaine drip) • SRIs (fluoxetine, paroxetine, ventrafaxine) • Opiates • Others (tramadol, baclofen, transdermal / intrathecal clonidine) 36

  40. Press: # (pound) + 71on your phone keypad tospeak with Dr. Periquet, and Dr. Nash Questions on this subject? Visit OMEN OnLine http://omen.med.ohio-state.edu 37

  41. NEXT WEEK #1014 Ulcer Disease Update January 25 to 28 Hagop S. Mekhjian, MD Professor of Internal Medicine Division of Digestive Diseases Medical Director, OSU Hospitals The Ohio University Medical Center E. Christopher Ellison, MD Zollinger Professor of Surgery and Interim Chair, Department of Surgery The Ohio University Medical Center

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