Acute flaccid paralysis

lecture of 51 slide

1. Acute Flaccid Paralysis ( GBS and Poliomyelitis ) By Dr . Magdy Shafik Ramadan Senior Pediatric and Neonatology consultant M.S, Diploma, Ph.D of Pediatrics

2. Introduction Acute flaccid paralysis (AFP) is defined as onset of paralysis ( <4 weeks ) in a child (age < 15 years) for which no obvious cause has been found; or, a paralysis in a person of any age in whom polio is suspected. • The Paralysis is associated with loss of muscle tone and loss of eflexes . • Reporting of all AFP cases less than 15 years of age is mandatory in India. • All AFP cases should be investigated within 48 hours.

3. Anatomical localization and Etiolgical agents in Acute Flaccid Paralysis Etiology Anatomical site Poliovirus Anterior Horn Cell HSV, CMV, rabies Dorsal root ganglia Acute transverse myelitis Spinal Cord GBS,, Vit b12 deficiency, diphtheria, rabies, Heavy metals, Hypokalemia Peripheral nerves Myasthenia crisis, organophosphorus poisoning, botulism Neuromuscular junctionNeuromuscular junction Polymyositis , SLE, Lyme diseas , toxoplasmosis Muscle

4. Clinical assessment Etiology Onset of Paralysis GBS, traumatic neuritis, transverse myelitis , myasthenia crisis, viral myositis Within hours to few days Polio, Japanese B Encephalitis, Tick bite paralysis Within 2-3 weeks Lyme disease, Post diphtheritic polyneuropathy Weeks to months Rabies Months to years

5. Disease Progression of paralysis Traumatic neuropathy Maximum Deficit at onset Poliomyelitis, Japanese B encephalitis Hours to few days GBS, Varicella zoster, Tick bite paralysis Up to 2-4 weeks Lyme disease and polymyositis Few weeks to months

6. Disease Topography Poliomyelitis, Japanese B encephalitis, Traumatic neuropathy Asymetric involvement GBS, Transverse myelitis , rabies, botulism Symmetric involvement

7. Disease Clinical Feature Neuropathy secondary to neurotropic viruses (Rabies, varicella zoster) GBS Transverse myelitis Lyme disease Traumatic neuropathy Sensory features DiseaGBG V ses of AHC, peripheral nerves and in spinal shock phase of cord diseases DTR- Areflexia Transverse myelitis Bladder Bowel involvement Polio and non polio enteroviruses , other neurotropic viruses and occasionaly with transverse myelitis , viral myositis Fever at onset of paralysis

8. Investigations • Serum Potassium • MRI Spine with contrast • NCS, NST and EMG • CSF Examination • Serum Creatinine phosphokinase • Urine for porphobilinogen • Lyme’s serology

9. Guillain-Barre Syndrome

10. Introduction .. It is an acute inflammatory demyelinating Polyradiculo neuropathy • It is an acute diffuse post-infective disease causing generalized paralysis and areflexia . The average age of incidence is 4-8 years, and rare below 2 years

11. Clinical Features A progressive ascending, symmetrical paralysis coming on over hours, days to a few weeks is the hallmark of GBS. Pain and refusal to walk are the presenting symptoms. Cranial nerve involvement in 50% cases. Autonomic manifestations seen in one third patients

12. Motor weakness: – Begins in lower limbs and progressively involves trunk, upper limbs, diaphragm, respiratory, pharyngeal and laryngeal muscles. – May also involve lower cranial nerves – Proximal muscles involved more in limbs. – Progression of paralysis for 4-10 days. – Areflexia

13. Consciousness is normal. Cranial Nerves: – 7th nerve bilateral involvement is frequently seen Sensory symptoms – No objective sensory loss. – There may be distal impairment of vibration and position sense.

14. Lab Features CSF : Normal pressure • Xanthochromic appearance • Raised CSF protein 1-10 g/l (100-1000mg/dl) ALBUMINOCYTOLOGICAL DISSOSCIATION: Cell count normal, no pleocytosis – Proteins : elevated, twice the normal limit

15. Nerve conduction velocity studies. Electromyography: Nerve Biopsy Serum Creatinine kinase may be normal to elevated

16. Treatment Supportive care : Ventilation, physiotherpary IVIG : Indications: Acute GBS of less than 2 weeks • Inability to walk unaided • Bulbar weakness. Dose : 0.4mg/kg/day for 5 days

17. Plasmapharesis Dose : 200-250 ml/kg/bodyweight of plasma is removed on alternate days in 4-6 sittings for 8- 12 days. Complications: CVS complication • Dysautonomia • Hepatitis and AIDS

18. Poliomyelitis

19. Introduction Greek poliós = "grey", myelós = marrow, and the suffix - itis = inflammation First described by British physician Micheal Underwood in 1799 referring to it as "debility of the lower extremities. Epidemiology: Poliovirus: belongs to “ Picorna ” viruses which are small RNA containing viruses. Non Enveloped.

20. Human is the only reservoir. It multiplies in the intestine and spreads via faeco -oral route. The maximum excretion of virus occurs just before the onset of paralysis and during the first 2 weeks after the onset of paralysis. However, virus is intermittently excreated for upto 2 months after infection. Three antigenically distinct serotypes . Type1, 2 and 3

21. Polio virus can remain active for several days at room temperature and can be stored indefinitely at -20 degree C. Most frequent cause of epidemic polio is Type 1 followed by type 3 . The primary site of replication is small intestine and regional lymph nodes. Poliovirus accesses the CNS via peripheral nerves and primarily infects motor neuron cells in the spinal cord (the anterior horn cells) and the medulla oblongata.

22. Infants born to mothers with few antibodies are protected for a few weeks.

23. Clinical manifestations

24. ACTIVE IMMUNIZATION  Oral (Sabin) Polio Vaccine: 1961 by Albert Sabin, Live attenuated vaccine. • Provide both humoral and Mucosal (Gut) immunity. Mucosal intestinal immunity prevent infection with wild polio virus.

25. Intestinal immunity is the main reason why mass campaigns with OPV can rapidly stop person to person transmission of wild polio virus. Immunity probably lifelong. Shed in stool for up to 6 weeks following vaccination.

26. Risks associated with use of OPV Vaccine Associated Paralytic Poliomyelitis: Those cases of AFP, which have residual weakness 60 days after onset of paralysis and from whose stool samples, vaccine related poliovirus is isolated. In some recipient of OPV, there is genetic change ( <1%), in the VP1 gene of vaccine virus.

27. This minor change turns the vaccine virus virulent.  Causes paralysis in recipient (recipient VAPP) or among unimmunized close contacts (contact VAPP)  1 case of VAPP occurs after 2.3 million first doses, and after 12 million subsequent doses.

28. Vaccine derived Poliovirus: There is a greater genetic change (1-15%) in the VP1 gene of the vaccine virus. It has potential for human infection and paralysis. Types of VDPV : 1) cVDPV (Circulating vaccine derived polio virus ) : A cVDPV is associated with sustained person to person transmission and is circulating in the community under conditions of low population immunity, with evidence of causing 2 or more paralytic cases.

29. 2) iVDPV ( Immununo deficiency –related vaccine –derived polio virus) reported in immunodeficient patients who have prolonged infections after exposure to OPV. 3) aVDPV (ambiguous vaccine derived polio virus) currently have unclassified source (i.e., a single isolate from a healthy or non- immunodeficient person; environmental isolates without an associated AFP case).

30.  Inactivated (Salk) vaccine: Developed in 1955 by Dr Jonas Salk. • Consists of inactivated polio strains of all three types. • Excellent humoral immunity. • Gives Mucosal protection but not mucosal immunity. • No risk of VAPP or VDPV . • 0.5 ml IM or SC or a fractional dose of 0.1ml Intradermal .

31.  National Immunization Schedule: – bOPV at birth,6-10-14 weeks followed by booster at 15- 18month. – IPV at 14 week (0.5ml) – In some states Fractional doses of IPV at 6 and 14 weeks instead of IM dose. The IAP Schedule : ‘ Sequential IPV-OPV schedule’ – bOPV at birth – IPV at 6-10-14 weeks – bOPV at 6 and 9 month – IPV at 15-18 month – bOPV at 5 year

32. Pulse Polio Immunization (PPI) Program Following the Global Polio Eradication Initiative of WHO in 1988, GOI conducted the first round of PPI consisting of 2 immunization days 6 wks apart on 9th dec 1995 and 20th jan . 1996

33. Strategy adopted: The basic strategy for eradicating polio consisted of: a) Immunizing every child below 1 year with at least 3 doses of OPV. b) National Immunization Days during which every child below 5 years gets 2 additional doses of OPV on 2 days separated by 4 to 6 weeks. c)Surveillance of AFP to identify all reservoirs of wild poliovirus transmission.

34. d) Extensive house-to-house immunization mopping –up campaigns in the final stages where wild poliovirus transmission per sists . Key Points : Targeted all children upto 3 yrs later on WHO increased the age upto 5 yrs. PPI occurs in two rounds about 4-6 wks apart during low transmission season of polio , i.e. Between nov to feb .

35. These doses are extra dose which supplements and do not replace the doses received during immunization services. There is no minimum interval between PPI and scheduled OPV doses. On 26 May 2012, the World Health Assembly declared the completion of poliovirus eradication to be a “ programmatic emergency for global public health” and called for the development of comprehensive polio endgame strategy.

36. In response to this directive, the GPEI developed Polio Eradication & Endgame Strategic Plan (PEESP) 2013-2018. This strategy shall prevent circulating VDPV by augmenting the immunity induced by earlier doses of trivalent OPV. By 2019 there shall be complete cessation of OPV

37. AFP-SURVEILLANCE Nationwide AFP (acute flaccid paralysis) surveillance is the gold standard for detecting cases of poliomyelitis. Surveillance identifies new cases and detects importation of wild poliovirus .

38. The four steps of surveillance are: Finding and reporting children with AFP. 2. Transporting stool samples for analysis. 3. Isolating and identifying poliovirus in the laboratory. 4. Mapping the virus to determine the origin of the virus strain.

39. Finding and reporting children with acute flaccid paralysis (AFP) surveillance 1 -The first links in the surveillance chain are staff in all health facilities- from district health centres to large hospitals. 2 - They must promptly report every case of AFP in any child under 15 years of age. 3-The number of AFP cases reported each year is used as an indicators of a country’s ability to detect polio-even in countries where disease no longer occurs.

40. A country’s surveillance system needs to be sensitive enough to detect at least one case of AFP for every 100,000 children under 15- e ven in absence of polio.

41. Transporting stool samples for analysis 1-Two stool specimens should be collected at an interval of 24 to 48 hours apart and within 14 days of onset of paralysis. 2 -However, when AFP cases are seen late (i.e. greater than 2 weeks after paralysis onset ), stool specimen may be collected up to 60 days after onset of paralysis. 3 -The specimen should be placed in a clean container s uch as wide mouth plastic or glass bottle with screw on cap.

42. 4 -It need not be autoclaved , but should be cleaned. 5-At least ‘one thumb sized’ 8 gm of stool is required. 6-Stool sample should be adequate and in good condition accompanied by all details as required by laboratories.

43. Isolating Poliovirus If poliovirus is isolated the next step is to distinguish between wild and vaccine related . If wild polio virus is isolated then identify which of the two surviving types of wild virus is involved.

44. Mapping The Virus 1 - Once wild poliovirus has been identified further tests are carried out to determine where the strain may have originated. 2 -By determining the genetic makeup of virus , wild virus can be compared to others and classified into genetic families which cluster in defined geographic areas . 3- When polio has been pinpointed to a precise geographical area, it is possible to identify the source of impor tation of poliovirus- both long range and cross border

45. Environmental Surveillance Environmental surveillance involves testing sewage or other environmental samples for the presence of poliovirus. Environmental surveillance often confirms wild poliovirus infections in the absence of cases of paralysis. Systematic environmental sampling provides important supplementary surveillance data.

46. أصبحت مصر خالية من شلل الأطفال منذ عام 2004 . وقد أبلغ عن آخر حالة من مدينة أسيوط في أيار/ مايو 2004. وأبلغ عن آخر عينة بيئة بها فيروس بري أصلي في كانون الثاني/يناير 2005 . وفي نهاية عام 2008، تم اكتشاف فيروسين من الفيروسات البرية البيئية وافدة من جنوب السودان والهند على التوالي. ؟؟ أما أحدث حالة وافدة من شمال السودان، فقد اكتشفت في كانون الأول/ديسمبر عام 2010 من عينة مياه الصرف الصحي تم أخذها من مدينة أسوان .

47. ترصد شلل الأطفال الرخو الحاد بدأت مصر في إعداد تقارير الترصد منذ مطلع تسعينيات القرن العشرين. وفي عام 1996، تحولت مصر إلى تصنيف الحالات الفيروسية، ومنذ ذلك الحين، تم الحفاظ على مؤشرات ترصد شلل الأطفال الرخو الحاد في حدود معايير الإشهاد النموذجية للمنظمة.

48. Jonas Salk Albert Sabin George Guillain Jean Barre Thanks .