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Acute Peripheral Neurologic disorder Praween Lolekha, MD Thammasat University
Outline ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Neural Control of Breathing ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Neural Control of Breathing ,[object Object],[object Object],[object Object]
Neural Control of Breathing ,[object Object],[object Object],[object Object],[object Object],[object Object]
Neural Control of Breathing ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Neural Control of Breathing ,[object Object],[object Object],[object Object],[object Object]
Neural Control of Breathing ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Neural Control of Breathing Mechanoreceptors -Respiratory muscle -Lungs: Pulmonary stretch receptor Chemoreceptors -Carotid bodies -Aortic bodies Medullary respiratory Center (RC center) CN IX,X CN X -Phrenic n (C3-5) - Intercostal n (T2-T12) Larygopharyngeal m. Bronchoconstrictors CN IX
Symptoms ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Signs ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Respiratory Investigations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Respiratory Investigations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Neurological Disorders and respiratory failure ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
Peripheral neurology and respiratory failure ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Guillain-Barré syndrome
Guillain- Barré  syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Epidemiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Guillain- Barré  syndrome AIDP  = Acute   inflammatory demyelinating polyneuropathy AMAN  = Acute motor axonal neuropathy AMSAN = Acute motor sensory axonal neuropathy MFS  = Miller Fisher syndrome   (Ophthalmoparesis, ataxia, areflexia) GBS AIDP AMAN MFS AIDP with 2 degeneration AMSAN
Etiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical Features ,[object Object],[object Object],[object Object]
Motor symptoms ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Sensory symptoms ,[object Object],[object Object],[object Object],[object Object]
Autonomic dysfunctions ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Diagnostic criteria ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Electrophysiologic study ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],“ Evidence of demyelination”
CSF analysis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Treatment ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Treatment
Plasma Exchange ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Plasma Exchange
Immunoglobulin ,[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Immunoglobulin
Prognosis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],Prognosis
Myasthenia gravis (MG) ,[object Object],[object Object]
Epidemiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Pathophysiology
Modified Osserman Classification  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Quantitative MG Score
Clinical features ,[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical features ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical features ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
“ Myasthenic snarl”
Associated disorder ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Diagnosis of MG ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Repetitive Nerve Stimulation “ Decrement response”
Precipitating factors ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Treatment   ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Acethycholineaterase inhibitors ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Acethycholineaterase inhibitors ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
MG crisis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Diagnosis of MG Evaluate for Thymectomy Anticholinesterase Crisis Generalized Occular Low risk If unsatifactory MRI Anticholinesterase Intensive Care Respiratory Infection High risk Improve Thymectomy Prednisolone Immunosuppressive Plasmapheresis / IVIg Not improve
Prognosis   ,[object Object],[object Object],[object Object],[object Object]
MG crisis vs Cholinergic crisis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Lambert-Eaton Myasthenic Syndrome   (LEMS) ,[object Object],[object Object],[object Object],[object Object],[object Object]
Pathophysiology ,[object Object]
Clinical Features ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Diagnostic methods ,[object Object],[object Object],[object Object],[object Object]
Repetitive Nerve Stimulation Posttetanic facilitation - incremental response >200%
Treatment ,[object Object],[object Object],[object Object],[object Object],[object Object]
 
Botulism ,[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Botulism
C. botulinum ,[object Object],[object Object],[object Object],[object Object],[object Object]
Pathophysiology
Clinical presentations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Autonomic dysfunction ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Diagnostic methods ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Treatment ,[object Object],[object Object],[object Object],[object Object],[object Object]
Vincent van Gogh
Porphyria ,[object Object],[object Object],[object Object],[object Object]
Pathway of Haem Synthesis
Acute intermittent prophyria ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical features ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Precipitating factors ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Laboratory diagnosis ,[object Object],[object Object]
Management ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Neurotoxic marine poisoning ,[object Object],[object Object],[object Object],[object Object],[object Object]
 
Ciguatera poisoning ,[object Object],[object Object],[object Object]
Ciguatera poisoning ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Management ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Puffer fish poisoning
Puffer fish poisoning ,[object Object],[object Object],[object Object],[object Object]
Puffer fish poisoning ,[object Object],15min- 24 hr Severe respiratory failure and hypoxia, hypotension, bradycardia, unconsciousness  Grade 4 15min-several hrs. Generalized flaccid paralysis, respiratory failure, aphonia, fixed or dilated pupils, patient is conscious. Grade 3 10-60 min Lingual and face numbness, early motor paralysis and incoordination. Slurred speech. Normal reflexs Grade 2 5-45 min Perioral numbness and paraesthesia, ± GI symptoms (N/V) Grade 1
Puffer fish poisoning ,[object Object],[object Object],[object Object],[object Object]
Management ,[object Object],[object Object],[object Object],[object Object],[object Object]
Organophosphate Poisoning ,[object Object],[object Object],[object Object]
Acetylcholine in PNS
Acute intoxication ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Seizure, coma, depression, agitation Brain CNS -Muscarinic/ nicotinic Fasciculation, weakness, paralysis Skeletal muscle NMJ -Nicotinic Miosis, Lacrimation Salivation Bronchorrhea, bronchospasm Bradyarrhythmia, prolong QT Diarrhea, emisis, increase motility Urinary incontinence Diaphoresis Increase catecholamines Eye Mouth Lungs Hearts GI tract GU tract Sweat glands Adrenal ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Clinical Effect Organ Receptor
Intermediate syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Organophosphate-induced delayed polyneuropathy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Management ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Neurological Respiratory Failure Level of consciousness History of Drugs, Toxic substance Normal   consciousness Poor, Unconsciousness Long tract signs  Brain stem signs -Brainstem lesion: infraction -Increase ICP -Brain herniation PNS Acute Chronic Polyneuropathy : GBS NMJ :  MG crisis Botulism Toxin : Porphyria Tetrodotoxin -Drugs -Metabolic -Infection -Other systems ALS Muscular dystrophy Myopathy Critical illness myopathy,neuropathy Others Other systems Yes No Airway: -COPD, asthma Alveoli: -Pneumonia CNS
- - Normal Normal Normal Normal Symmetric Proximal Proximal Chronic Chronic ,[object Object],[object Object],[object Object],- + + + Normal Normal Normal Normal Delay distal  Symmetric Proximal Proximal Dessending Proximal Gradual Gradual Acute Acute NMJ -MG  -LEMS -Botulism -Organophos phate + + + Normal Mild Mild Distal, perioral Symmetric Assending Distal Acute Acute Acute ,[object Object],[object Object],[object Object],[object Object],- Normal Asymmetric Chronic Ant. horn cell - ALS Autonomic DTR Sensory Motor Tone Onset
The END

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Acute Peripheral Neurologic disorder

  • 1. Acute Peripheral Neurologic disorder Praween Lolekha, MD Thammasat University
  • 2.
  • 3.
  • 4.
  • 5.
  • 6.
  • 7.
  • 8.
  • 9. Neural Control of Breathing Mechanoreceptors -Respiratory muscle -Lungs: Pulmonary stretch receptor Chemoreceptors -Carotid bodies -Aortic bodies Medullary respiratory Center (RC center) CN IX,X CN X -Phrenic n (C3-5) - Intercostal n (T2-T12) Larygopharyngeal m. Bronchoconstrictors CN IX
  • 10.
  • 11.
  • 12.
  • 13.
  • 14.
  • 15.  
  • 16.
  • 18.
  • 19.
  • 20. Guillain- Barré syndrome AIDP = Acute inflammatory demyelinating polyneuropathy AMAN = Acute motor axonal neuropathy AMSAN = Acute motor sensory axonal neuropathy MFS = Miller Fisher syndrome (Ophthalmoparesis, ataxia, areflexia) GBS AIDP AMAN MFS AIDP with 2 degeneration AMSAN
  • 21.
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  • 47.
  • 48. Repetitive Nerve Stimulation “ Decrement response”
  • 49.
  • 50.
  • 51.
  • 52.
  • 53.
  • 54. Diagnosis of MG Evaluate for Thymectomy Anticholinesterase Crisis Generalized Occular Low risk If unsatifactory MRI Anticholinesterase Intensive Care Respiratory Infection High risk Improve Thymectomy Prednisolone Immunosuppressive Plasmapheresis / IVIg Not improve
  • 55.
  • 56.
  • 57.
  • 58.
  • 59.
  • 60.
  • 61. Repetitive Nerve Stimulation Posttetanic facilitation - incremental response >200%
  • 62.
  • 63.  
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  • 65.
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  • 68.
  • 69.
  • 70.
  • 71.
  • 73.
  • 74. Pathway of Haem Synthesis
  • 75.
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  • 92.
  • 93.
  • 94.
  • 95.
  • 96.
  • 97. Neurological Respiratory Failure Level of consciousness History of Drugs, Toxic substance Normal consciousness Poor, Unconsciousness Long tract signs Brain stem signs -Brainstem lesion: infraction -Increase ICP -Brain herniation PNS Acute Chronic Polyneuropathy : GBS NMJ : MG crisis Botulism Toxin : Porphyria Tetrodotoxin -Drugs -Metabolic -Infection -Other systems ALS Muscular dystrophy Myopathy Critical illness myopathy,neuropathy Others Other systems Yes No Airway: -COPD, asthma Alveoli: -Pneumonia CNS
  • 98.