Peripheral Neuropathy George Pope Presenta3on of neuropathy and - - PowerPoint PPT Presentation

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Peripheral Neuropathy George Pope Presenta3on of neuropathy and - - PowerPoint PPT Presentation

Dec 23, 2022 138 likes •507 views

Peripheral Neuropathy George Pope Presenta3on of neuropathy and ae3ology Neurophys Acute Subacute Chronic Distal Symmet : Axonal Toxins Systemic dise. As subac. Porphyria Def. States

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SLIDE 1

Peripheral Neuropathy

George Pope

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SLIDE 2

Presenta3on of neuropathy and ae3ology

Neurophys Acute Subacute Chronic Distal Symmet: Axonal Toxins Systemic dise. As subac.

Porphyria Def. States HMSN 2 Toxins Cancer assoc Amyloid

Demylina-ng GBS CIDP HMSN 1 Diphtheria CIDP ‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐‐ Mul8focal + Axonal Vasculi3s Diabetes Leprosy mononeuri8s Sarcoid mul8plex Demylina-ng GBS CIDP CIDP

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SLIDE 3

Peripheral neuropathy

  • Classifica3on
  • Causes
  • Presenta3on
  • Inves3ga3ons
  • GBS
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SLIDE 4

Classifica8on

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SLIDE 5

Peripheral neuropathy Classifica3on 1

Axonal Demylina3ng Vascular Sensory/motor

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Axonal

  • Axon lost
  • Glove + stocking sens.loss
  • Muscle weak/wasted
  • Longest axons most affected
  • Absent reflexes
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SLIDE 7

Axonal neuropath causes

  • Diabetes
  • Hypothyroid
  • Uraemia
  • Rheumatoid
  • Lupus
  • Vascular neuropathies
  • Toxic
  • Vit.def
  • Neoplasms
  • Infec3on
  • HIV
  • HMSN
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SLIDE 8

Demylina8ng

  • Myelin Sheath lost
  • Sparing pain +temp
  • º was3ng/weakness
  • Nerve enlargment
  • Absent reflexes
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SLIDE 9

Classifica8on 2

  • Gene3c
  • Metabolic/endocrine
  • Nutri3onal def
  • Toxic
  • Drug induced
  • Conec3ve 3ssue

disease

  • Cancer related
  • Unknown (20%)
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SLIDE 10

Toxins/Drugs

distal sensorimotor symetrical peripheral neuropathy

  • Arsenic
  • Ethylene oxide
  • Lead (motor )
  • Mercury
  • Organophosphates
  • Thallium
  • Ethanol
  • Amiodorone
  • Cispla3num
  • Dapsone
  • Gold
  • Isoniaside
  • Metronidazole
  • Thalidomide
  • Vincris3ne
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SLIDE 11

Vitamin def

Painfull,sensory,distal symet. axonal neuropathy

  • Pernicious anaemia(B12)
  • Pellegra (niacin def.)
  • Pyridoxine def. (B6)
  • Thiamine def. (B1)
  • Folate def.
  • Vitamin E def.
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SLIDE 12

Diabetes

Most common in West

1) Distal Symmetrical 75 % Usually sensory +autonomic Painfull paraesthesia/numbness Neuropathic arthropathies 2)Asymmetrical prox. Neuropathy Diabe3c amyotrophy Lumbosacral radiculopathy 3)Autonomic 4)Neuropathies amd radiculopathies

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SLIDE 13

Diabetes

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SLIDE 14
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SLIDE 15
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Neoplasia

Symmetrical distal sensorimotor axonal neuropathy

  • 1% pts with cancer
  • Types

Nerve invasion‐‐lymphoma

  • Malig. Meningi3s

Paraneoplas3c‐‐symet.distal sensorimotor axonal neuropathy

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SLIDE 17

Infec3ve neuropathies

Leprosy Tuberculoid nerve thickening + mononeuri3s mul3plex Lepromatous Superficial sensory neuri3s/anaesthe3c depig skin Lyme Borrelia Burgdorferi

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SLIDE 18

Vasculi3c Neuropathies Wegner’s granulomatosis (cANCA) Rheumatoid Arthri3s (Rh factor) Sjogren’s syndrome (An3‐Ro an3‐SS‐B) Polyarteri3s Nodosa Dx Histol.+suppor3ve serology Rx Immunosuppress.

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SLIDE 19

Inves3ga3ons

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SLIDE 20

Basic Inves3ga3ons

  • ESR
  • FBC
  • Ca
  • Crea3nine
  • Fas3ng glucose
  • Plasma electroph.
  • B12
  • Urinalysis
  • CXR
  • Autoan3.
  • TFT’s
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SLIDE 21

Inves3ga3ons

  • NCS
  • LP
  • Toxins/Vitamin levels
  • HIV
  • Nerve bx.
  • ANS
  • Gene3c
  • ACE/ Urinary Ca
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SLIDE 22

Nerve conduc3on studies

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SLIDE 23

GBS

Demylina3ng neuropathy

  • 1859 Landry described

10 cases of ascending paralysis and sensory changes

  • WW1

Guillain,Barre,Strohl described above + areflexia

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SLIDE 24

Demylina3ng Neuropathies GBS/CIDP GBS

Autoimmune process characterized by progressive weakness and mild sensory changes. All ages. 2 per 100,000 per yr. 70% antecedent infec3on Campylobacterjejuni CMV EBV

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SLIDE 25

GBS

  • Predom. motor polyradiculopathy

Limbs + cranial nerves/mild sensory s+s Reflexes lost with 3me ANS ‐‐arrhythmias/Bp ↓↑/ urinary / bowel progression (average 5 – 10 days)

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SLIDE 26

Inves8ga8ons

  • CSF

‐ protein 200 – 600 mg/l ‐ cells: few or none

  • MRI

‐ cranial nerve enhancement may occur

  • Serum an3bodies CIDP

‐ IgG vs ganglioside GQ1b

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SLIDE 27

Prognosis

  • 5 % mortality
  • 20 % con3nued disability at 1 yr
  • 3 % recurrance
  • Axonal =slower + less complete recovery
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SLIDE 28

CIDP

  • GBS > 6 MTS
  • Rx steroids / Plasma exchange / Ig’s
  • May be assoc with monoclonal gammopathy
  • f unknown significance
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Miller‐Fisher Syndrome

Variant of acute inflammatory demyelina3ng polyneuropathy Epidemiology

  • Onset: mean 40 years (13 – 78 yrs)
  • Clinical prodrome: usually respiratory
  • 1% of Guillain‐Barre Syndrome
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Clinical Features

  • Onset

‐ diplopia (80%)

‐ myalgia & paresthesias ‐ ver3go & ataxia

  • Eye

‐ external ophthalmoplegia (100%)

‐ pupillary dysfunc3on (42%) ‐ ptosis (58%)

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SLIDE 31
  • Ataxia (100%)
  • Areflexia (100%)
  • Sensory

‐ distal & facial parasthesias (24%)

  • Weakness (20%)
  • Other cranial nerve disorders

‐ oropharyngeal weakness (26%) ‐ facial weakness (32%)

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SLIDE 32
  • Progression

‐ over days to weeks ‐ may progress to generalized weakness ‐ recovery over 2 weeks to 2 months ‐ many pa3ents with no residual defects long term.

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SLIDE 33

Treatment

Indica3ons for rapid treatment:

  • VC < 1 Lt
  • Respiratory dysfunc3on
  • Bulbar disorders
  • Inability to walk without assistance
  • ?milder weakness
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SLIDE 34

Plasma exchange vs. IV Immunoglobulins

  • verall no difference in efficacy

Immunoglobulins

↓ side‐effects ↓ cost / easy access/admin

Use Children Pts with ‐ diarrhoea prodrome ‐ infec3ous disorders (HIV) ‐ autonomic instability ‐ poor venous access

Plasma exchange

? Fewer late relapses No allergic reac3ons

Use Adults with good venous access History of S/Es with Igs Pregnancy Conges3ve cardiac failure Renal insufficiency

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SLIDE 35
  • Rx. Complica3ons
  • Pneumonia
  • Complica3ons of ven3la3on
  • Pulmonary embolism
  • Corneal exposure
  • Dysphagia
  • Cardiac arrhythmias
  • Malnutri3on
  • Speech Rx ect.
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SLIDE 36

References

Asbury AK: Diagnos3c considera3ons in Guillain‐Barre syndrome. Ann Neurol 1981; 9 Suppl: 1‐5. Berlit P, Rakicky J: The Miller Fisher syndrome. Review of the literature. J Clin Neuroophthalmol 1992 Mar; 12(1): 57‐63. Chiba A, Kusunoki S, Obata H: Serum an3‐GQ1b IgG an3body is associated with

  • phthalmoplegia in Miller Fisher syndrome and Guillain‐Barre syndrome:

clinical and immunohistochemical studies. Neurology 1993 Oct; 43(10): 1911‐7. Dwyer JM: Manipula3ng the immune system with immune globulin. N Engl J Med 1992 Jan 9; 326(2): 107‐16. Fisher M: An unusual variant of acute idiopathic polyneuri3s (syndrome of

  • phthalmoplegia, ataxia and areflexia). N Engl J Med 1956; 255: 57.

Plasma Exchange/Sandoglobulin Guillain‐Barre Syndrome Trial Group: Randomized trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain‐Barre syndrome. Lancet 1997 Jan 25; 349(9047): 225‐30.