Presentation on nNeromilitis Optica

1. Case Presentation
DR. TAHMINA ZAFAR

2. How we will proceed 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:
• A 36 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 present illness:
• 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 has hx of laparoscopic cholecystectomy in
2015.
• No hx of blood transfusion
• No hx of any trauma/accident

7. Family Hx:
• No Family hx of
– DM
– HTN
– IHD
• No hx of similar illness in any other family
member

8. Musculoskeletal hx:
• No previous hx of joint pains,
swellings or rheumatic disease.

9. Gynecological Hx
• Age of 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:
• She is 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
• Central nervous 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 lower limbs
– 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).

17. 2017
Cervico-
Dorsal spine
with and
without
contrast
Showing
hyperintense
lesion (Blue
arrow)(T2):

18. 2017 MRI
brain and
dorso-
cervical
spine with
iv contrast

19. • MRI
Brain
done in
2017
• (T2 and
T1):

20. 2017 MRI
images
(Sagittal):

21. 2017 MRI
images
(Coronal
With and
without
contrast):

22. 2017Cevi
c-dorsal
spine
(T2):

23. MRI
Cervico-
dorsal
spine
with
contrast.
(2021)
Showing
atrophic
changes:

24. MRI
Cervico-
dorsal
spine
with
contrast.
(2021)
Showing
atrophic
changes:

25. 2021

26. 2021

27. 2021

28. Diffrential diagnosis
• Transverse myelitis
• MS
• NMO spectrum disorder or Devic’s
disease

29. Activity:
• Can it be 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 main pathological 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 usually present 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 is usually 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.

34. Diagnosis:

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.