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Clinical Aspects of Peripheral Nerve and Muscle Disease

Clinical Aspects of Peripheral Nerve and Muscle Disease. Roy Weller Clinical Neurosciences University of Southampton School of Medicine. Normal Nerves. Anterior Horn Cell Dorsal root ganglion cell Motor Peripheral Nerve Sensory Peripheral Nerve Neuromuscular Junction Sensory ending

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Clinical Aspects of Peripheral Nerve and Muscle Disease

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  1. Clinical Aspects of Peripheral Nerve and Muscle Disease Roy Weller Clinical Neurosciences University of Southampton School of Medicine

  2. Normal Nerves • Anterior Horn Cell • Dorsal root ganglion cell • Motor Peripheral Nerve • Sensory Peripheral Nerve • Neuromuscular Junction • Sensory ending • Muscle Sensory Motor

  3. Peripheral Neuropathy Mononeuropathy Mononeuropathy Multiplex Polyneuropathy Distal distribution of Sensory and motor signs and symptoms

  4. Peripheral Neuropathy Sensory: Motor: Paraesthesia (tingling, pins & needles, prickling, burning…) Numbness (Hypoaesthesia) Abnormal degrees and types of sensation Weakness Wasting Muscle twitching (fasciculations)

  5. Peripheral NeuropathyInvestigations • Blood tests: Part of the general investigation • CSF: • Protein raised in inflammatory conditions • Radiology (CXR, MR Imaging) • Nerve Conduction Studies + EMG • Axonal degeneration Vs Demyelinating

  6. MRI of cervical Nerve root tumours

  7. MRI of cervical Nerve root tumour

  8. Peripheral NeuropathyInvestigations • Nerve Biopsy • Axonal degeneration • Segmental Demyelination • Inflammatory changes • Others (Amyloid, paraprotein,..)

  9. Axonal Degeneration and Regeneration

  10. Segmental Degeneration Autoimmune Segmental Degeneration

  11. Clinical Approach to Peripheral Neuropathy • Which systems are involved? • What is the distribution of weakness? • What is the nature of the sensory involvement? • What is the temporal evolution? Acute, Subacute, Chronic, relapsing,.. • Is there evidence of heredity?

  12. Where is the pathology? Dermatomes

  13. Clinical Evaluation of Compression Neuropathy

  14. Toxic Neuropathies

  15. Nerve Biopsy • When to biopsy a nerve? • Neuropathy is severe, actively worsening • Essential for diagnosis • Complications • 10-15% • Localised Sensory loss • Wound infection, dehiscence, neuroma formation • Unpleasant dysaesthesiae, neuropathic pain

  16. Acquired Vasculitis* Sarcoidosis* Amyloidosis* CIDP IgM Paraproteinaemic N Leprosy Tumour infiltration* *Nerve Biopsy often essential for Dx Hereditary CMT types 1A,1B &3 HNPP (tomaculous neuropathy) Amyloidosis Giant Axonal Neuropathy Metachromatic Leukodystrophy Polyglucosan body N* Refsum’s disease Peripheral Neuropathies in Which a Nerve Biopsy May Be Useful

  17. Muscle disease

  18. Diagnosis of Muscle Disease Clinical Pathology Genetics

  19. Myofibils EM of muscle fiber Sarcolemma Basal lamina or Basement membrane Satellite cell Nucleus Macrophages Perivascular Lymphocytes Regenerating myoblast

  20. Hereditary: Muscular dystrophies Myotonias Channelopathies Congenital myopathies Metabolic myopathies Mitochondrial myopathies Acquired: Inflammatory myopathies Endocrine myopathies Myopathies associated with other systemic illness Drug-induced myopathies Toxic myopathies Clinical Classification of Myopathies: Primary diseases of Muscle

  21. Myopathy • Features Supporting Diagnosis: • Distribution proximal • Muscle Bulk preserved or enlarged • Reflexes Parallel muscle strength

  22. Most myopathies have a proximal distribution of weakness and pain. Distribution of weakness can aid diagnosis in muscular dystrophies: a] Duchenne and Becker; b] Emery Dreifuss; c] limb-girdle; d] fascioscapulohumeral; e] distal; f] oculpharyngeal

  23. Myopathy • Features against diagnosis: • Distal weakness • Fasciculations • Tremor • Sensory signs (or symptoms) • Pathological fatigue • Early absence of reflexes

  24. Muscle biopsy • Which Muscle? • Moderately involved, but avoid muscle with severe weakness • Best Specific Muscles: Deltoid, Biceps, Quadriceps • Avoid: Muscle sampled by EMG or sites of recent trauma

  25. Muscle Disease: Pathology & Genetics 2nd Edition Edited by Hans H. Goebel, Caroline A. Sewry and Roy O. Weller Publisher, International Society of Neuropathology & John Wiley & Sons Limited 2013

  26. Myofibils EM of muscle fiber Sarcolemma Basal lamina or Basement membrane Satellite cell Nucleus Macrophages Perivascular Lymphocytes Regenerating myoblast

  27. 6 Myofibils 7 8 EM of muscle fiber 4 Sarcolemma 2 1 9 Basal lamina or Basement membrane 10 3 14 11 Satellite cell Nucleus 5/12/13 Macrophages Perivascular Lymphocytes 14/15/16 Regenerating myoblast 17

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