This patient care meeting discusses a 12-year-old girl admitted with inability to walk or sit for 2 months and generalized weakness. She has a history of similar symptoms in 2020 treated with steroids and plasma exchange, with improvement. Examination shows increased tone and weakness in lower limbs. Investigations indicate a urinary tract infection and positive aquaporin antibodies. MRI shows myelitis and optic nerve involvement. She is diagnosed with a relapse of neuromyelitis optica spectrum disorder. Treatment options like immunosuppressants and novel therapies are discussed to suppress attacks and reduce relapse risk.
2. Disclosure Statement
I declare:
I do not have any financial relationship with proprietary entities
producing, marketing, re-selling or distributing healthcare goods or
services consumed or used on patients in the past 24 months.
3. History
• Presenting Complaint :
12 Year old girl was electively admitted via clinic with complaints of
- Inability to walk and sit for 2 months
- Generalized Weakness for 2 months
4. • HOPC :
• Father claims that before she started experiencing urine
incontinence problems in December 2022, she was healthy
and regularly attended school. After that, she had trouble
walking and needed assistance. Gradually, it got worse,
and she started having trouble using the walker to lift her
feet. They visited the neurology clinic with similar worries,
and admission was suggested.
5. • Past Medical History :
- Admitted in AKUH in 2020 with similar complaint where underwent 5 doses of
methylprednisolone and 5 cycles of Plasmapheresis which improved her weakness
and was discharged on Azathioprine and Prednisolone .
- History of UTI in 2020 for which she was treated
- Had issues of enuresis in 2022 for which she was started on Desmopressin after
workup , which she stopped herself
• Past Surgical History :
- None
• Birth History :
Normal
6. Examination :
• Vitals :
HR = 90 beats/min
R/R = 24 breaths/min
B.P = 125/86
SaO2= 99 RA
• Anthropometric Measures :
Length
Weight : 33 Kg
On Examination:
Head to Toe: A thin Lean Girl , lying on bed well oriented . No
obvious rash
Systemic Examination:
CNS: GCS 15/15
Upper Limb: Lower Limb :
Tone Increased Bilateral Limb Increase Tone
Bilaterally
Power : 3/5 RUL ; 2/5 LUL Power : 0/5 in both
lower Limbs
Reflexes : 3+ Reflexes :3 +
Clonus +ve
Cranial Nerves : Intact
Eye Examination : Only Counts Fingers ; Cant read words
CVS: S1 +S2+0
CHEST: Bilateral equal air entry
ABDOMEN: soft and non tender ; Bladder palpable
9. Investigation Cont
CSF D/R
CSF Glucose 82
CSF Chloride 125
CSF Protein 26
Appearance Clear
CSF RBC 0.000
CSF TLC 0.001
Oligoclonal
Bands :
MATCHING OLIGOCLONAL BANDS PRESENT IN BOTH CEREBROSPINAL FLUID (CSF) AND
SERUM INDICATIVE OF A SYSTEMIC (NON INTRATHECAL) IMMUNE REACTION
Aquaporin Antibody :
Positive
10. Radiology :
• MRI Brain + Spine with Contrast:
Thinning of the optic nerves is noted bilaterally, more marked on the left . Subtle
enhancement of optic nerves is noted canalicular and prechiasmatic parts.
T2 and FLAIR hyperintense signals are noted in bilateral optic nerves, optic chiasm,
bilateral
medial temporal lobes, periaqueductal region , tectal plate as well as the anterior fornix
without enhancement in these regions on postcontrast images.
Patchy enhancement and oedema are noted in the upper cervical cord.
Swollen cervical cord with diffuse abnormal T2 hyperintense signals is redemonstrated ,
extending from obex to T1 level with patchy enhancement. This is showing interval
increase in the swelling of cord with worsening of signals
Prominent central spinal canal is noted in the dorsal cord, likely because of cranial cord
inflammation.
Above findings are secondary to demyelinating disease, likely diffuse myelitis with interval
worsening of disease process.
11. Investigation Continued
• Visual Evoked Potential : Normal
• Visual Examination : Bilateral Pale Optic Disc ; Visual Acuity
couldn’t be commented because patient was bed ridden .
13. Point of Discussion
• Treatment Options for Neuromyelitis Optica Spectrum Disorder
• Role of Immunosuppressants in Preventing Relapse
• Novel Therapies
14. NMOSD
• Neuromyelitis optica spectrum disorders (NMOSD; previously
known as Devic disease or neuromyelitis optica [NMO]) are
inflammatory disorders of the central nervous system
characterized by severe, immune-mediated demyelination and
axonal damage predominantly targeting optic nerves and the
spinal cord.
21. Learning Points
• Patient presenting with lower limb weakness and eye
involvement always consider NMOSD
• Ensure counselling patients to continue immunosuppressive
therapy after active disease has resolved to prevent relapse
• Physiotherapy to be ensured always to reduce spasticity
• Provide Psychological Support to Patient
