1. A 13 years old girl with Acute Flaccid Paralysis F.Ahmadabadi Child Neurologist

2. Problems List 1.Acute flaccid paraplegia 2.Urinary retention & incontinence 3.Sensory level(L3) 4.Parestesia & low back pain 5.Vesicourethral reflux

3. Differential Diagnosis of Acute Flaccid Paralysis(AFP) Cerebral Anterior horn Myelopathy Neuropathy Neuromuscular junction Myopathy

4. Differential Diagnosis of Acute Flaccid Paralysis(AFP) Brainstem disorders – Stroke – Encephalitis Poliomyelitis – Poliovirus – other neuropathic viruses Acute Myelopathy – SOL SOL – Acute transverse myelitis Acute transverse myelitis – Ischemic attack – Truama – Infective myelitis (HTLV1-HIV-West nill-Arbo virus)

5. Spinal cord Tumors Primary Astrocytoma Epandymoma Secondary <5 years Neuroblastoma >5 years Ewing Sarcoma NOTE Motor deficit is seen in 86% of cases and back pain occurs in 63%.

6. Differential Diagnosis … Cont… Peripheral neuropathies – GBS – Post Rabies vaccination – Diphtheritic – Heavy metals – Toxins(Glue-Herbicides) – Drugs (INH- Vincristine- Nitrofurantoin - Zidovudine) – Porphyria – Vasculitis (PN-MNM)JRA

7. Differential Diagnosis … Cont… Neuromuscular transmission – Myasthenia gravis – Botulism – Tick paralysis Disorders of muscle – Hypokalemia – Hypophosphatemia – Inflammatory Myopathy – Acute rhabdomyolysis – Trichinosis – Periodic paralysis

8. Spinal cord syndromes

9. Approach to spinal cord syndromes

10. Transverse Myelitis An acute demyelinating disorder of the spine Incidance:1-4 /million annually Peak 2 nd and 4 th decades TM +Optic neuritis= Devic disease MS is usually suspect Max deficit within 2 days DTR  Decreased or Increased Outcome: 50% complete recovery 40% incomplete &10% don’t recover MRI  swelling at the level of myelitis-Gadolinium enhancement CSF: Pleocytosis-increased IgG

11. Criteria's for Transverse Myelitis Bilateral(may be asymmetric) Motor &Autonomic dysfunction of spinal cord Sensory level Max deficit 4 h -21 days R/O radiation-Compressive-Vascular and neoplastic etiologies CSF changes

12. Symptoms interfering with daily function Inadequate response Clinical observation & Repeat Imaging IV Methyl prednisolone 20-30mg/Kg/day for 3-5 days Life-threatening demyelination NOYes Incomplete improvement Marked clinical improvement Rehabilitation PLEX 5-8 Exchanges Over 10 days IVIg 2 G/Kg over 2-5 days Oral prednisone taper starting at 1mg/kg/day and tapering over 21 days

13. Common pitfalls in AFP 1.“His motor strength was ‘grossly intact’ when I saw him. I even documented that on my chart.” Ambiguous documentation 2. “Her main complaint was the scalp wound she sustained after she fell. She didn’t even tell me she was weak.” Falling or gait instability are frequent signs of weakness. 3. “Her only complaint was tingling, and I often have trouble interpreting hyporeflexia on my physical examination. How was I supposed to know she would windup on a ventilator the next day?” in a patient complaining of paresthesias and/or weakness,consider an acute polyneuropathy.

14. 4. “His pulse ox was 99%, so I admitted him to a non monitored bed and told the neurologist he could see him in the morning.” Pulse oximetry may reveal nothing about respiratory insufficiency until it’s too late—get PFTs. 5. “The radiologist on call said an emergent MRI of the spine was not warranted.” A patient identified to have an epidural compression syndrome or signs of a significant myelopathy needs steroids and a stat MRI. 6. “That woman had so much psychosocial stuff going on,her weakness had to be psychosomatic.

15. 7.“ The patient told me his vision was blurred. Lots of patients complain of blurry vision. How was I supposed to know he meant he was experiencing double vision?” Do a detailed examination of the extraocular muscles and specifically ask the patient about double vision. 8. “The parents said that their infant was lethargic and not feeding well, but the kid was afebrile. I ruled out sepsis and dehydration. What was the problem?” A good history is necessary in all cases of acute weakness. In this case, the child had infantile botulism.

16. Management 1.History Taking & Ph Ex 2.EMG-NCV 3.Electrolytes 4.CPK-LDH 5.S/E (Report to Health Care Service) 6.MRI 7.CSF/A 8.Rheumatology Consult

17. About our case… Transverse Myelitis Vascular syndromes Rheumatologic disorders