NEUROPATHIES, MYOPATHIES

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MYOPATHIES
NEUROPATHIES
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NEUROPATHIES, MYOPATHIES

• NEUROPATHIES (7)
• Inflammatory
• Infectious
• Hereditary (HMSN-I)
• HMSN-II, HMSN-III
• Acquired Toxic/Metabolic
• Traumatic
• Neoplasms

• MYOPATHIES (9)
• Denervation
• Dystrophies
• Ion Channel
• Congenital
• Genetic Metabolic
• Inflammatory
• Toxic
• NeuroMuscular Junction
• Neoplasms

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GENERAL Reactions

• NERVE
• DEMYELINATION (segmental)
• AXONAL DEGENERATION
• NERVE REGENERATION
• REINNERVATION

• MUSCLE FIBER
• NECROSIS
• VACUOLIZATION
• REGENERATION
• ATROPHY
• HYPERTROPHY

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HYPERTROPHY, ATROPHY
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NEUROPATHIES, MYOPATHIES

• NEUROPATHIES (7)
• Inflammatory
• Infectious
• Hereditary (HMSN-I)
• HMSN-II, HMSN-III
• Acquired Toxic/Metabolic
• Traumatic
• Neoplasms

• MYOPATHIES (9)
• Denervation
• Dystrophies
• Ion Channel
• Congenital
• Genetic Metabolic
• Inflammatory
• Toxic
• NeuroMuscular Junction
• Neoplasms

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NEUROPATHY, Inflammatory

• Guillain-Barré
• Preceded by influenza-like illness
• NO actual specific etiologic agent isolated,
  autoimmume disease to myelin gangliosides most
  likely
• Inflammation of a peripheral nerve
• DEMYELINATION
• ASCENDING paralysis

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Guillain-Barré, (AIDP), Acute Inflammatory
Demyelinating Polyneuropathy
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NEUROPATHY, Infectious

• Leprosy
• Diphtheria
• V/Z (Varicella-Zoster)

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N E U R O T O X I N
C. DIPHTHERIAE
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Z O S T E R
POSTHERPETIC NEURALGIA
ZOSTER in DRG
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NEUROPATHY, Hereditary(defective
myelination)(Hereditary Motor and Sensory
Neuropathy)

• HMSN-I (Charcot-Marie-Tooth)
• HMSN-II (Like CMT of the neurons)
• HMSN-III (Palpable Nerves) (aka, Dejerine-Sottas)

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PES CAVUM(S), in CMT
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NEUROPATHY, Toxic/Metabolic

• Symmetric, Asymmetric
• Sensory, Sensorimotor
• Somatic, Autonomic
• Focal, Multifocal

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NEUROPATHY, Toxic/Metabolic

• Diabetes Mellitus
• Vitamin Deficiencies (many Bs, E)
• Heavy Metals, Pb, As, etc.
• Organic Compounds
• CHEMO

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DEMYELINATION
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NEUROPATHY, Traumatic

• Laceration
• Avulsion
• Carpal Tunnel
• Traumatic (amputation) Neuroma
• Saturday Night Palsy
• Morton Neuroma

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TRAUMATIC NEUROMA Regenerating Axons and Glia
(Schwann Cells), but with no direction
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MEDIAL Plantar Nerve
3rd COMMON digital branch
MORTONS NEUROMA Traumatic Compression FgtM Inte
rdigital Intermetatarsal
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NEUROPATHY, Neoplastic

• Benign Schwannoma
• Malignant Malignant Schwannoma

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Antoni A Palisaded Antoni B NON-Palisaded
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QUIZ

• Why are Schwannomas the ONLY tumors of peripheral
  nerve?

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MYOPATHIES
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NEUROPATHIES, MYOPATHIES

• NEUROPATHIES (7)
• Inflammatory
• Infectious
• Hereditary (HMSN-I)
• HMSN-II, HMSN-III
• Acquired Toxic/Metabolic
• Traumatic
• Neoplasms

• MYOPATHIES (9)
• Denervation (SMA)
• Dystrophies
• Ion Channel
• Congenital
• Genetic Metabolic
• Inflammatory
• Toxic
• NeuroMuscular Junction
• Neoplasms

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MYOPATHY, Denervation
MUSCLE FIBERS CANNOT SURVIVE UNLESS THEY ARE
INNERVATED
PERIPHERAL NERVE PATHOLOGY ANTERIOR HORN CELL
PATHOLOGY
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SPINAL MUSCULAR ATROPHY

• Childhood diseases
• Chromosome 5 that harbors the survival motor
  neuron gene (SMN1)
• Anterior Horn Cells
• Often PAN-fascicular
• More peripheral muscles

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MYOPATHY, Dystrophic

• Jerrys kids, no DYSTROPHIN
• DUCHENNE (DMD), x-linked
• BECKER (BMD) (less common, less severe, same
  chromosome)
• Many others also, all of which have complex
  genetic patterns which have all been precisely
  defined
• MYOTONIA is a common feature

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DMD
NORMAL
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MYOPATHY, Ion ChannelChannelopathies

• MYOTONIA/HYPOTONIC PARALYSIS
• FAMILIAL, (genetic) DISEASES
• TRIGGERED BY
• Exercise
• Cold
• Carb Intake
• Classified by K, ?K, ?K
• MALIGNANT HYPERTHERMIA can be triggered off by
  anesthetic halogenated inhalation agents in some
  of these patients!!!

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MYOPATHY, CongenitalFloppy Babies

• HYPOTONIC
• FAMILIAL, (genetic) DISEASES
• MANY TYPES, in most of which the precise genetic
  defects have been identified

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MYOPATHY, Metabolic(genetic also)

• LIPID
• Mitoch. Enz. Def.? LIPID ACCUMULATION
• MITOCHONDRIAL
• PARKING LOT mitochondria

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PARKING LOT MITOCH.
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MYOPATHY, Inflammatory

• DERMATOMYOSITIS
• POLYMYOSITIS
• INCLUSION BODY MYOSITIS
• ALL HAVE UNCLEAR ETIOLOGIES

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DERMATOMYOSITIS (often peri-vascular)
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POLYMYOSITIS, usually endo-myseal
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INCLUSION BODY MYOSITIS, rimmed vacuole
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MYOPATHY, Toxic

• THYROTOXICOSIS
• ETHANOL
• DRUGS (steroids, chloroquine)
• DRUGS (MANY MANY others)

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MYOPATHY, NeuroMuscular Junction

• Myasthenia Gravis
• Associated with thymomas
• Thymectomies often useful Rx
• AUTOIMMUNE DISEASE, CLEARLY
• Abs to ANTI-CHOLINESTERASE RECEPTORS
• Anticholinesterase test is very diagnostic
• YOUNG WOMEN WITH EYE MUSCLE
• Ptosis ?
• Diplopia?
• General Weakness
• Lambert-Eaton Syndrome (paraneoplastic)

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MYOPATHY, Neoplastic

• Benign
• Rhabdomyoma
• Malignant
• Rhabdomyosarcoma