AQUAPORIN-4 (AQP-4) IMMUNOGLOBULIN G SEROPOSITIVE NEUROMYELITIS OPTICA: A REVIEW AND CASE REPORT

Overview

Journal Ann Ib Postgrad Med

Specialty General Medicine

Date 2024 May 6

PMID 38706629

Authors

C Nwaze

Y Eghwrudjakpor

N Chinedu-Anunaso

Affiliations

Soon will be listed here.

Abstract

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a relatively new terminology composed to encompass patients with neuromyelitis optica (NMO) and related immunological conditions. The diagnosis of this condition requires a seropositive aquaporin-4 immunoglobulin G (AQP-4 IgG), the presence of at least one core clinical characteristic and the exclusion of alternative diagnoses. Very few cases have been reported in sub-Saharan Africa.

Objective: The aim of this article is to report a classical case of NMOSD with AQP-4 IgG seropositivity and normal brain, cervical and thoracic MRI findings.

Result: We report a 25-year-old Nigerian woman who presented with recurrent and alternating weakness, pain and numbness of all limbs, associated with episodic painful left-sided tonic spasms and urinary incontinence. She had earlier had symptoms of recurrent, episodic and alternating loss of vision in both eyes, associated with ocular pain.Examination findings revealed an intact mental status, no cranial nerve deficit and no focal limb weakness. Right-sided deep tendon reflexes were exaggerated. Vital signs were within normal limits. Brain MRI, Cervical spine MRI and Thoracic spine MRI all revealed normal findings. Serum aquaporin-4 IgG assay returned positive with a titer of 1:32.She was commenced on high dose steroids and there was gradual improvement of symptoms.

Conclusion: These findings confirmed the diagnosis of neuromyelitis optica spectrum disorder, and satisfies the diagnostic criteria published in 2015 by the International Panel for NMO Diagnosis (IPND).

References

Houzen H, Niino M, Hirotani M, Fukazawa T, Kikuchi S, Tanaka K . Increased prevalence, incidence, and female predominance of multiple sclerosis in northern Japan. J Neurol Sci. 2012; 323(1-2):117-22. DOI: 10.1016/j.jns.2012.08.032. View

Waters P, McKeon A, Leite M, Rajasekharan S, Lennon V, Villalobos A . Serologic diagnosis of NMO: a multicenter comparison of aquaporin-4-IgG assays. Neurology. 2012; 78(9):665-71. PMC: 3286228. DOI: 10.1212/WNL.0b013e318248dec1. View

Wingerchuk D, Hogancamp W, OBrien P, Weinshenker B . The clinical course of neuromyelitis optica (Devic's syndrome). Neurology. 1999; 53(5):1107-14. DOI: 10.1212/wnl.53.5.1107. View

Bennis A, El Otmani H, Benkirane N, Harrizi I, El Moutawakil B, Rafai M . Clinical course of neuromyelitis optica spectrum disorder in a moroccan cohort. Mult Scler Relat Disord. 2019; 30:141-148. DOI: 10.1016/j.msard.2019.02.012. View

Marignier R, Bernard-Valnet R, Giraudon P, Collongues N, Papeix C, Zephir H . Aquaporin-4 antibody-negative neuromyelitis optica: distinct assay sensitivity-dependent entity. Neurology. 2013; 80(24):2194-200. DOI: 10.1212/WNL.0b013e318296e917. View

Peschl P, Bradl M, Hoftberger R, Berger T, Reindl M . Myelin Oligodendrocyte Glycoprotein: Deciphering a Target in Inflammatory Demyelinating Diseases. Front Immunol. 2017; 8:529. PMC: 5420591. DOI: 10.3389/fimmu.2017.00529. View

Palace J, Leite M, Nairne A, Vincent A . Interferon Beta treatment in neuromyelitis optica: increase in relapses and aquaporin 4 antibody titers. Arch Neurol. 2010; 67(8):1016-7. DOI: 10.1001/archneurol.2010.188. View

Wingerchuk D, Lennon V, Lucchinetti C, Pittock S, Weinshenker B . The spectrum of neuromyelitis optica. Lancet Neurol. 2007; 6(9):805-15. DOI: 10.1016/S1474-4422(07)70216-8. View

Verkman A . Aquaporins in clinical medicine. Annu Rev Med. 2012; 63:303-16. PMC: 3319404. DOI: 10.1146/annurev-med-043010-193843. View

10.

Wingerchuk D, Lennon V, Pittock S, Lucchinetti C, Weinshenker B . Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006; 66(10):1485-9. DOI: 10.1212/01.wnl.0000216139.44259.74. View

11.

Papp V, Magyari M, Aktas O, Berger T, Broadley S, Cabre P . Worldwide Incidence and Prevalence of Neuromyelitis Optica: A Systematic Review. Neurology. 2020; 96(2):59-77. PMC: 7905781. DOI: 10.1212/WNL.0000000000011153. View

12.

Watanabe S, Nakashima I, Misu T, Miyazawa I, Shiga Y, Fujihara K . Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica. Mult Scler. 2007; 13(1):128-32. DOI: 10.1177/1352458506071174. View

13.

Jacob A, Panicker J, Lythgoe D, Elsone L, Mutch K, Wilson M . The epidemiology of neuromyelitis optica amongst adults in the Merseyside county of United Kingdom. J Neurol. 2013; 260(8):2134-7. DOI: 10.1007/s00415-013-6926-y. View

14.

Pittock S, Berthele A, Fujihara K, Kim H, Levy M, Palace J . Eculizumab in Aquaporin-4-Positive Neuromyelitis Optica Spectrum Disorder. N Engl J Med. 2019; 381(7):614-625. DOI: 10.1056/NEJMoa1900866. View

15.

Assadeck H, Toudou Daouda M, Omar E, Mamadou Z, Djibo F, Maiga D . Inflammatory demyelinating diseases of the central nervous system in Niger. Rev Neurol (Paris). 2018; 175(4):261-268. DOI: 10.1016/j.neurol.2018.05.003. View

16.

De Seze J, Kremer L, Collongues N . Neuromyelitis optica spectrum disorder (NMOSD): A new concept. Rev Neurol (Paris). 2016; 172(4-5):256-62. DOI: 10.1016/j.neurol.2016.03.003. View

17.

Etemadifar M, Dashti M, Vosoughi R, Abtahi S, Ramagopalan S, Nasr Z . An epidemiological study of neuromyelitis optica in Isfahan. Mult Scler. 2014; 20(14):1920-2. DOI: 10.1177/1352458514537699. View

18.

Zuliani L, Lopez de Munain A, Ruiz Martinez J, Olascoaga J, Graus F, Saiz A . [NMO-IgG antibodies in neuromyelitis optica: a report of 2 cases]. Neurologia. 2006; 21(6):314-7. View

19.

Osuntokun B . The pattern of neurological illness in tropical Africa. Experience at Ibadan, Nigeria. J Neurol Sci. 1971; 12(4):417-42. DOI: 10.1016/0022-510x(71)90110-9. View

20.

Mealy M, Wingerchuk D, Greenberg B, Levy M . Epidemiology of neuromyelitis optica in the United States: a multicenter analysis. Arch Neurol. 2012; 69(9):1176-80. DOI: 10.1001/archneurol.2012.314. View