1. Acute flaccid paralysis PRESENTED BY DR. ABDURAHMAN AL SLAYED

2. Definition A medical emergency clinical syndrome characterized by rapid onset (usually hours to days), progressive weakness of at least one, usually two or more limbs. There may be bulbar and respiratory involvement. If not managed appropriately, paralysis can progress to respiratory failure and death.

6. NEUROANATOMICAL CORRELATE OF DIFFERENTIAL DIAGNOSIS 1.Muscle. 2.Neuromuscular Junction. 3.Nerve Roots (acute polyradiculopathies) and plexus lesions. 4.Anterior Horn Cell. 5.Spinal Cord.

7. NEUROANATOMICAL CORRELATE OF DIFFERENTIAL DIAGNOSIS 1. Muscle: 1.Inflammation : inflammatory myopathy (polymyositis ) 2.Infection : Viral myositis 3.Periodic paralyses (hypokalemic, hyperkalemia) 4.Channelopathy : Hypokalemic periodic paralysis 5.Dyselectrolytemia : Hypokalemia

8. NEUROANATOMICAL CORRELATE OF DIFFERENTIAL DIAGNOSIS 2. Neuromuscular Junction : 1.Immune mediated: myasthenia Gravis. 2.Drugs, toxins : Organophosphates, snake venom, Botulism. 3. Dyselectrolytemia : Hypermagnesemia.

9. NEUROANATOMICAL CORRELATE OF DIFFERENTIAL DIAGNOSIS 3. Roots/nerves : 1. Immune mediated : Gillian Barre syndrome 2. Toxin : Post diphtheritic, porphyria, arsenic 3. Viral : Rabies 4. Trauma :Injection related sciatic neuritis

10. NEUROANATOMICAL CORRELATE OF DIFFERENTIAL DIAGNOSIS 4. Anterior Horn Cell 1.Viral : Poliomyelitis, vaccine associated poliomyelitis, Enteroviral myelitis, Japanese encephalitis Poliomyelitis. 2. Vascular: Anterior spinal artery infarction 5. Spinal Cord 1.Compressive: Traumatic spinal injury, epidural abscess, hematoma, discitis. 2.Inflammatory : Transverse myelitis.

11. Diagnosis History Examination Investigations.

12. Clues In History and Examination Fever at onset Polio Or Enterovirus Myelitis. Transverse Myelitis. Myositis, Epidural Abscess. Trauma: head/neck Trivial trauma may lead to spinal compression in patients with cervical vertebral instability (Patients with Downs syndrome, congenital cervicovertebral anomalies or juvenile idiopathic arthritis) Exposure Toxins: lead, arsenic Snake envenomation Dog bite: Rabies

13. Clues In History and Examination Preceding infectious prodrome/vaccination Guillain Barre syndrome or transverse Myelitis. Sore throat, neck swelling, diphtheretic polyneuropathy (non/partly immunized) Precipitating factors Diarrhea: Hypokalemia, enteroviral Myelitis. Exertion or post parandial: Hypokalemic periodic paralysis I.M injection: Polio, traumatic sciatic neuritis Sensory loss/level Compressive myelopathy, transverse myelitis

14. Clues In History and Examination Early bowel/bladder involvement Compressive myelopathy, transverse myelitis Constipation in <1 y Botulism (H/o honey exposure) Prominent autonomic signs/symptoms Guillain Barre syndrome, Rabies, acute myelopathy

15. Clues In History and Examination Ascending weakness Guillain Barre syndrome, Rabies, Varicella zoster virus, ascending myelitis Descending weakness Diphtheria, Botulism Prominent and early ptosis Myasthenia Gravis, Botulism

16. Clues In History and Examination Facial weakness Guillain Barre syndrome, Myasthenia Gravis, Botulism Fluctuating symptoms, fatigability Myasthenia Gravis Muscle tenderness Myositis, inflammatory myopathy, (myalgias may be severe in Guillain Barre syndrome)

17. Clues In History and Examination Muscle stretch reflexesAbsent: Guillain Barre syndrome, Polio, Diphtheria, spinal shock, at level of spinal cord damage Preserved : Myasthenia Gravis, periodic paralysis, Botulism Exaggerated: Below level of spinal lesion, Upper motor neuron lesion Spinal tenderness painful spine movement Spinal trauma, epidural abscess or other extradural compression Neck stiffnessPolio, enteroviral myelitis, Guillain Barre syndrome, transverse myelitis

18. PHYSICAL EXAMINATION Distribution and degree of weakness looking specially for extra ocular muscles, facial muscles and bulbar involvement Assess for fatigability

19. PHYSICAL EXAMINATION Sensory impairment: particular modality (vibration/ proprioception vs. pain / temperature) - is there a sensory level Reflexes: Are the deep tendon reflexes lost? (ie.areflexic), depressed, preserved, or brisk) Autonomic features (postural fall, abnormal sweating, pupillary response, ileus)

20. PHYSICAL EXAMINATION Skin: lines on nails with arsenic poisoning (Mee’s lines), ticks, photosensitivity, Gottron’s papules on extensor surfaces & heliotrope discoloration over eyelids (dermatomyositis), fang marks Spinal tenderness (with epidural abscess or hematoma, spinal tumour)

21. Investigations 1. MRI Spine: It is indicated when there is a suspicion of : spinal cord compression or transverse myelitis. More specifically, any child with history of neck or back trauma, rapid onset flaccid profound quadreparesis, early or persistent bladder or bowel involvement, sensory loss or sensory level on examination, spinal tenderness, neurocutaneous markers, or appearance of UMN signs on examination (e.g., up going plantars) should get an MRI of the spine

22. Investigations 2. CSF examination: This is helpful to narrow the diagnostic possibilities. A raised CSF cell count would be seen in patients with transverse myelitis, infective myelitis viz. polio or enteroviral myelitis, varicellas or herpes myelitis, rabies, etc. A raised CSF protein with normal cell count (albumin cytological dissociation) suggests Guillain Barre syndrome, post diphtheritic polyneuropathy or rarely may be seen in transverse myelitis. The CSF can be normal early in the course of these illnesses.

23. Investigations 3. Nerve Conduction studies and Electro Myography (EMG): These studies confirm the involvement of nerves and help in diagnosis of anterior horn cell diseases. These are particularly helpful to confirm Guillain Barre syndrome. The repetitive nerve stimulation test helps to diagnose myasthenia gravis and botulism. Rarely, these may aid the diagnosis of an inflammatory myopathy.

24. Investigations According to the suspected site of lesion and cause of paralysis. Neuroimaging (spinal cord) MRI indicated in all cases of myelopathy, suspected transverse myelitis X- ray spine: suspected atlantoaxial dislocation, vertebral tuberculosis. Electrophysiological testing (NCV & electromyography): Guillain Barre syndrome Lumbar puncture (CSF): Guillain Barre syndrome, suspicion of viral myelitis Biochemistry: Creatine Kinase, Potassium, Magnesium, Phosphate. ECG: Hypokalemia Urine for porpho-bilinogens in porphyria, toxins: arsenic

29. Management supportive care. supportive care. ABCs I.Ensure protection of airway and adequate ventilation (especially if there is respiratory muscle weakness, shallow/paradoxical respiration, dysphagia, weak gag),some children pick up the weakness early and need elective intubation, II.Check and support: BP and Heart Rate. III.Immobilize neck if history of neck/head trauma. IV.Send electrolytes and get an ECG- to look for hypokalemia.

30. Management S pecifi c therapy: S pecifi c therapy: 1.Guillain Barre syndrome: IVIG. 2.Transverse myelitis: IV methyl-prednisolone. 3.Compressive myelopathy: spinal immobilization, surgical intervention, steroids (acute traumatic myelopathy). 4.Dermatomyositis, Myasthenia Gravis: Immunomodulation. 5.Hypokalemia: Intravenous potassium correction.

31. Conclusions 1.AFP is a medical emergency. 2.Accurate and early diagnosis of the cause has important bearing on the management and prognosis. 3.The immediate priorities are to detect and manage respiratory, bulbar muscle weakness and rapidly exclude causes which are reversible like electrolytes disturbance or toxemia (snake bite). 1.Evaluation of spine by imaging may be needed urgently in patients with suggestive clinical features.

32. THANK YOU