ENFERMEDADES DESMIELINIZANTES DEL SISTEMA NERVIOSO CENTRAL, ACTUALIZACION EN DEFINICION Y DIAGNOSTICO QUE INCLUYE DIAGNOSTICO DIFERENCIAL

1. Neuromyelitis optica
spectrum disorder
(NMOSD)
MR ROGER MEZA MARTINEZ
NEUROLOGÍA

2. INTRODUCTION
• previously known as Devic disease or neuromyelitis optica
(NMO)
• Is an inflammatory disorder of the central nervous system.
• Immune-mediated demyelination and axonal damage
predominantly targeting optic nerves and the spinal cord.
UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

4. EPIDEMIOLOGY
• In adults 0,37 a 10 por 100.000
• Women is up to 10 times higher than in men
• Age is 32 to 41 years old (Comparatively the mean age of onset
of MS is 24 years)
UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

5. PATHOGENESIS
a) Neuropathology: involve multiple segments of the spinal cord and
optic nerves with astrocyte death, axonal loss, perivascular
lymphocytic infiltration, and associated vascular proliferation.
b) Autoimmune pathogenesis: mediated primarily by the humoral
immune system
c) Astrocytopathy: which causes immune-mediated inflammation and
secondary demyelination
d) AQP4 water channel protein: NMO-IgG target antigen is a water
channel protein that is abundant in the astrocytic processes of the
foot in the blood-brain barrier and is highly concentrated in the
gray matter of the spinal cord
UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

6. The target antigen: AQP4
Is expressed in:
• Optic nerve
• Spinal cord
• The periventricular areas, hypothalamus and
subpial regions,
• Brainstem and the area postrema

7. Neuromyelitis optica: State of the art Jorge Daza Barriga1 , Angélica Roncallo Del Portillo 2017

8. CLINICAL FEATURES
• Spectrum of symptoms
 acute attacks of bilateral or rapidly sequential optic neuritis.
 transverse mielitis with a typically recurring course
 Other symptoms: intractable nausea, vomiting, hiccups,
excessive daytime sleepiness, or narcolepsy
UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

9. Optic neuritis, WHAT IS IT?
a) Inflammation of the optic nerve
b) Individual attacks of optic neuritis in NMOSD may be similar to
isolated optic neuritis syndromes of MS although visual loss is
usually more severe
c) Involvement of the chiasm and optic tracts is characteristic.
d) which is simultaneously bilateral, affects the optic chiasm, causes
an altitudinal visual field defect, or causes severe residual visual
loss.
e) Papilla edema suggests a diagnosis of antibody-associated disease
against myelin oligodendrocyte glycoprotein (MOGAD)
UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

10. • VISUAL EVOKED POTENTIAL :They show increased latency and
decreased wave amplitude, observed in more than 65%
• Desaturation to the red color is altered in 88%.
• afferent pupillary defect is an extremely sensitive indicator
• pain related to eye movements

11. • OPTIC NERVES:
• SPINAL CORD
• DESSERT AREA
• BRAIN STEM
• DIENCEPHALON
• CEREBRAL CORTEX.
N.O. bilateral affecting the optic chiasm with severe vision loss.
Intractable nausea, hiccups and vomiting
Complete acute spinal cord syndrome
oculomotor dysfunctions, signs of the long tract and
ataxia
Narcolepsy, anorexia, hypothermia and hypersomnia

12. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

13. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

14. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

15. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

16. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

18. CRITERIOS DIAGNOSTICOS

19. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

20. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

21. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

22. UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

23. Optical coherence tomography:
• report a thinning of the nerve fiber layer of the retina, reflecting
more severe axonal aggression.
• Microcystic macular edema of the inner nuclear layer
• reduced ganglion cell thickness
UpToDate, Christopher C Glisson, DO, MS, FAAN, Francisco González-Scarano, MD, agosto 2022

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