Jill Tichy April 1, 2009

1. Jill Tichy April 1, 2009

3. Clinical Manifestations Rapidly evolving (hours to days) Areflexic motor paralysis: �Rubbery legs� Sensory loss ? Proprioception Usually upward progressing Lower cranial nerves: OP dysphagia Pain: �Deep aching pain� Transient bladder dysfunction

4. Other Clinical Manifestations Autonomic Dysfunction: Wide fluctuations in BP, Orthostatic Hypotension, and Cardiac Arrhythmias SIADH

5. Antecedent Events 70% of cases occur 1-3 weeks after infectious process 20-30% of all cases are associated with Campylobacter jejuni (summer outbreaks in China among kids and young adults) Also HHV, CMV or EBV Mycoplasma pneumoniae Recent Immunizations Lymphoma (Hodgkin�s) HIV SLE

6. Molecular Mimicry Immune responses to non-self antigens Misdirect to host nerve tissue through resemblance Neural targets are gangliosides, particularly at the Nodes of Ranvier

7. Molecular Mimicry

8. Diagnosis Rapid development of muscle paralysis, areflexia, absence of fever CSF: Elevated protein level without pleocytosis EMG and Nerve conduction show slowing CSF and EMG are used to verify symptoms but may not be abnormal until a week after onset

9. Required Diagnostic Criteria Progressive, relatively symmetrical weakness of two or more limbs Areflexia Course < 4 weeks Exclusion of other causes

10. Supportive Diagnostic Criteria Symmetric Weakness accompanied by numbness and/or tingling Mild Sensory Involvement Facial Nerve or other cranial nerve involvement Absence of fever Typical CSF findings Evidence of demyelination from EMG

11. Differential Diagnosis Acute Myelopathies Botulism Diptheria Lyme disease with polyradiculitis Porphyria Vasculitis Neuropathy Poliomyelitis CMV polyradiculitis Critical Illness Neuropathy Myasthenia Gravis Poisonings with Organophosphates or arsenic Paresis from West Nile Virus Spinal Astrocytoma Motor Neuron Disease

12. Treatment Supportive Management: Telemetry, Blood pressure Rx, DVT prophylaxis, Vital Capacity/NIF High dose IVIg or PLEX: Two weeks after the first motor symptom immunotherapy is no longer effective Meta-anaylsis of randomized clinical trials indicate Rx reduces the need for mechanical ventilation by 50% and increases the likelihood of full recovery at one year from 55% to 68% Steroids have not shown to be effective

13. Prognosis Most of the time recovery starts after the 4th week from onset of disease 80% complete recovery within months to a year; Areflexia usually persists 5-10% have relapse ? CIDP Mortality rate is <5% in optimal settings Death results usually from pulmonary complications

14. Notable Sufferers Andy Griffith 1983 FDR; 2003 in a peer review study less likely poliomyelitis William �the refrigerator� Perry; 2008

15. References Harrison�s Principles of Internal Medicine, 17th ed. Sabatine. Pocket Medicine. Wiki Uptodate