Vial

INDIVIDUAL TEST 880

Aquaporin 4 (AQP4) Antibodies  (IgG)

Indication

Suspected Neuromyelitis Optica (NMO).

Method

Indirect immunofluorescens (IIF) in cells transfected with Aquaporin 4 (AQP4).

Answer

Results are given as positive or negative. A positive result is followed by a titre.

Interpretation

Antibodies against AQP4 in serum are a strong marker for neuromyelitis optica (NMO/NMOSD) and can be detected in about 75-80% of the cases.

It is recommended to use the test on serum. However, seronegative cases where anti-AQP4 antibodies have been detected in the cerebrospinal fluid (CSF) have been reported. According to guidelines the relevance of anti-AQP4 antibodies in CSF is unclear and the diagnostic criteria are based on analysis on serum.

References 

Sato DK et al. Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders. Neurology 82 (2014) 474-481. 

Majed M, Fryer JP, McKeon A, Lennon VA, Pittock SJ. Clinical utility of testing AQP4-IgG in CSF. Neurol Neuroimmunol Neuroinflamm June 2016 vol. 3 no. 3 e231. 

Höftberger R et al. Antibodies to MOG and AQP4 in adults with neuromyelitis optica and suspected limited forms of the disease. Mult Scler. 2015 Jun;21(7):866-74. 

Wingerchuck DM et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015 Jul 14;85(2): 177-89. 

Klawiter E.C. et al. NMO-IgG detected in CSF in seronegative neuromyelitis optica. Neurology 72 March 24, 2009 

Jarius S. et al Cerebrospinal fluid antibodies to aquaporin-4 in neuromyelitis optica and related disorders: frequency, origin, and diagnostic relevance. Journal of Neuroinflammation 2010, 7:52 

Lennon VA, Wingerchuk DM, Kryzer J, Pittock SJ, Lucchinetti CF, Fujihara K, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364:2106-12. 

Lycke J, Malmestrom C. Neuromyelitis optica – viktig differentialdiagnos till MS. Läkartidningen 2010; 107: 3212-3215 

Sellner J. et al. EFNS guidelines on diagnosis and management of neuromyelitis. European Journal of Neurology 2010, 17: 1019–1032. 

Weinshenker BG, Wingerchuk DM, Pittock SJ, Lucchinetti CF, Lennon VA. NMO-IgG: a specific biomarker for neuromyelitis optica. Dis Markers. 2006;22(4):197-206 

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Vial

ENSKILD ANALYS 880

Aquaporin 4 (AQP4) -antikroppar (IgG)

Indikation

Misstanke om Neuromyelitis Optica (NMO).

Metod

Indirekt immunofluorescens (IIF) med celler transfekterade med Aquaporin 4 (AQP4).

Svar

Resultatet anges som negativt eller positivt. Positivt resultat titreras (serum).

Tolkning

Antikroppar mot Aquaporin 4 är en stark markör för neuromyelitis optica (NMO/NMOSD) och kan påvisas i ca 75-80% av fallen.

Rekommendationen är att endast testa serum men det finns seronegativa fall beskrivna där antikroppar mot aquaporin 4 har detekterats i likvor. Enligt guidelines är dock betydelsen av dessa antikroppar i likvor oklar och diagnoskriterierna baseras på analys i serum.

Referenser

Sato DK et al. Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders. Neurology 82 (2014) 474-481.
Majed M, Fryer JP, McKeon A, Lennon VA, Pittock SJ. Clinical utility of testing AQP4-IgG in CSF. Neurol Neuroimmunol Neuroinflamm June 2016 vol. 3 no. 3 e231.
Höftberger R et al. Antibodies to MOG and AQP4 in adults with neuromyelitis optica and suspected limited forms of the disease. Mult Scler. 2015 Jun;21(7):866-74.
Wingerchuck DM et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015 Jul 14;85(2): 177-89.
Klawiter E.C. et al. NMO-IgG detected in CSF in seronegative neuromyelitis optica. Neurology 72 March 24, 2009
Jarius S. et al Cerebrospinal fluid antibodies to aquaporin-4 in neuromyelitis optica and related disorders: frequency, origin, and diagnostic relevance. Journal of Neuroinflammation 2010, 7:52
Lennon VA, Wingerchuk DM, Kryzer J, Pittock SJ, Lucchinetti CF, Fujihara K, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364:2106-12.
Lycke J, Malmestrom C. Neuromyelitis optica – viktig differentialdiagnos till MS. Läkartidningen 2010; 107: 3212-3215
Sellner J. et al. EFNS guidelines on diagnosis and management of neuromyelitis. European Journal of Neurology 2010, 17: 1019–1032.
Weinshenker BG, Wingerchuk DM, Pittock SJ, Lucchinetti CF, Lennon VA. NMO-IgG: a specific biomarker for neuromyelitis optica. Dis Markers. 2006;22(4):197-206

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