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NMO Presentattion.ppt
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- 2. How we willproceed with this presentation: Learning objectives Introduction and Case Scenario Activity Related To The Case • Introduction • Pathophysiology • Presentation • Investigations • Diagnosis Neuromyelitis Optica Closing Activity Take Home Message • To be able to identify signs and symptoms • To be able to make a differential diagnosis • To reach a final diagnosis
- 3. Case Scenario: • A36 y/o Female, resident of Chungi, Lahore presented to us with symptoms of: – Bi-lateral upperlimb numbnes 12 Days – Fecal incontinence 10 Days
- 4. Hx Of presentillness: • A normotensive, normoglycemic woman who was in her usual state of health presented to us with – History of bilateral upper-limb numbness for 12 days which was: • Gradual and progressive in nature • Involving both upper limbs at the same time – History of fecal incontinence for 10 days – No association of: • Fever • Sore Throat, • Chestpain, • Cough • Dysuria, • Burining Micturition • Previous Hakim Medication – Urinary incontinence cannot be commented upon as she was bed ridden for 2 years and foley's in-situ which will be explained in past medical illness.
- 5. Past Medical Hx •In 2015 she presented with hx of bi-lateral lower limb weakness and was diagnosed as Multiple Sclerosis • She was treated but not fully recovered, and discharged on medication • In 2017 she again presented with worsening of previous symptoms and urinary incontinence but was discharged again without complete recovery • According to the patient in 2020 her condition got worsened but she did not consult any doctor because of the Corona epidemic
- 6. • She hashx of laparoscopic cholecystectomy in 2015. • No hx of blood transfusion • No hx of any trauma/accident
- 7. Family Hx: • NoFamily hx of – DM – HTN – IHD • No hx of similar illness in any other family member
- 8. Musculoskeletal hx: • Noprevious hx of joint pains, swellings or rheumatic disease.
- 9. Gynecological Hx • Ageof menarche was at 16 with regular menstruation cycles, with no hx of menorrhagia or oligomenorrhea • She has 4 children, all alive and healthy, all by normal SVD • No hx of OCPs
- 10. Personal hx: • Sheis a housewife with 4 children, all alive and healthy • She has no history of smoking or any drug abuse • She lives in a 10 Marla house, 4 bathrooms, in a combined family with total 12 people living there. • Good Ventilation
- 11. General Physical Examination: •An ill looking female with average height and built lying comfortably on bed with iv line on right hand and folleys insitu – Vitally stable with – Bp120/70 – Pulse 77/min – Temp 98.6f • There is no pallor jaundice cyanosis raised jvp clubbing edema feet palpable lymphnodes kilonychea
- 12. Systemic examination • Centralnervous system examination • HMF • Oriented with time place and person • Memory intact recent and past – Speeh normal – Cranial nerves all intact
- 13. Motor system lower limbs •Bulk tone normal • Bilateral power 3/5 • No fasciculations »Upper limbs Tone bulk normal • Power 5/5 no fasciculations • Reflexes • Upper limbs • Normal( bicep tricep and brachioradialus) • +2
- 14. • Reflexes lowerlimbs – All absent • Planters – Bilateral downgoing • Sensory system – Sensory level at t7 to t10 • Spine and skull – Normal • Gait – Cant b assessed
- 15. Other systems • Respiratory •Cardiac • Git • Musculoskeletal – No positive findings
- 16. Investigations • CBC, RFTS,LFTS, BSR, PT/INR/APTT, TFTS, VIT B12 levels, RA FACTOR, ANA, VDRL: – WITHIN NORMAL RANGES • HEPATITIS B and C – NEGATIVE • CT BRAIN PLAIN – NO SIGNIFICANT FINDINGS • MRI BRAIN WITH CONTRAST – NO SIGNIFICANT FINDINGS • MRI CERVICODORSAL SPINE – SHOWING HYPERINTENSE LESION(2017), And cord atrophy at the level of C3-6 (2021).
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- 18. 2017 MRI brain and dorso- cervical spinewith iv contrast
- 19. • MRI Brain done in 2017 •(T2 and T1):
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- 28. Diffrential diagnosis • Transversemyelitis • MS • NMO spectrum disorder or Devic’s disease
- 29. Activity: • Can itbe multiple sclerosis? – Give your answers – Give at least two reasons supporting your answer • Can it be transverse myelitis? – Give answer – Supporting evidence or reason in two points at least
- 30. Neuromyelitis Optica: • Introduction: •Neuromyelitis optica (NMO), also known as Devic’s disease, is an immune-mediated demyelinative disorder of the central nervous system (CNS). • Originally, it was considered a variant of multiple sclerosis (MS). • Only in 2004, with the discovery of specific IgG antibodies directed against aquaporin 4 (AQP4), considered as pathognomic for NMO, did it become possible to classify this disorder as a separate entity. • In 2015, Wingerchuk et al. published the international consensus on diagnostic criteria for NMOSD • . According to these, NMOSD includes: – classical NMO (optic neuritis—ON + longitudinal extensive transverse myelitis—LETM) – isolated ON or LETM – ON and/or LETM associated with autoimmune systemic diseases – ON and LETM accompanied by symptoms of brainstem, diencephalon or cerebral involvement
- 31. Pathophysiology: • The mainpathological mechanism involved in the background to NMO is associated with autoreactive anti-AQP4 IgG antibodies • Possible mechanisms include inappropriate recognition of this protein by T-cells or impairment of early B-cell tolerance checkpoints
- 32. Presentation: • Patients usuallypresent with hx of bi lateral limb numbness and decreasing visual acuity. • Although symptoms may vary between various types of NMOSD variants. • It follows a relapsing course with incomplete recovery and each episode worsening the previous symptoms. • Differentiating NMO from MS is particularly relevant, because disease-modifying therapies, effectively used in MS, appear to have weak or even adverse effects upon NMO course.
- 33. Investigations: • MRI isusually the first investigation of choice. • The presence of AQP4 antibodies in serum confirms the diagnosis. • For other variants of NMOSD, different diagnostic criteria exist that can be used to distinguish them from other auto-immune disorders given in the next slide.
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- 35. Ending activity: Group A: •1. • 2. Group B: • 1. • 2.
- 36. • MRI findings •Antiaquaporin-4 antibodies positive in serum • MOG serum antibody status – CSF analysis: » Raised protein levels » Absence of oligoclonal bands Confirming the Diagnosis:
- 37. Treatment Options: • Methylprednisolone •Plasma exchange • Intravenous immunoglobulin • Mycophenolate mofetil • Azathioprine • Methotrexate • Cyclophosphamide • Rituximab
- 38. Take Home Message: •NMOSD is a distinct group of auto immune disorders. • The presence of AQP4 is pathognomic for diagnosis of NMO. • It is a relapsing condition with incomplete recovery and each episode worsening the previous injury. • NMOSD Should be differentiated from MS because the treatment modalities may worsen the condition. • Early diagnosis and treatment leads to better outcomes and decrease in morbidity.