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• Area postrema syndrome: another feature of anti-GFAP encephalomyelitis
  Jonathan Ciron, Damien Biotti, Thierry Tchoumi, Marie Benaiteau, Nicolas Maubeuge and Romain Marignier
  Published on: 9 April 2019

• Published on: 9 April 2019

  Area postrema syndrome: another feature of anti-GFAP encephalomyelitis

  • Jonathan Ciron, Neurologist University Hospital of Toulouse, France
  • Other Contributors:
    • Damien Biotti, Neurologist
    • Thierry Tchoumi, Neurologist
    • Marie Benaiteau, Neurologist
    • Nicolas Maubeuge, Neurologist
    • Romain Marignier, Neurologist

  Dear Editor,

  We read with great interest the recent paper of Sechi et al. that describes 13 patients with anti-GFAP related myelitis and compares them with 41 patients with anti-AQP4 related myelitis.1 To date, very little data is available about anti-GFAP related disorders.2-3 Sechi et al. highlight some differences between the two entities to help clinicians differentiate them.1 One of these clinical differences relates to area postrema syndrome (APS). Indeed, it is well known that APS is a classical feature of neuromyelitis optica spectrum disorders, particularly among anti-AQP4 positive patients.4 Sechi et al. report this syndrome as a prodromal event in 20% of anti-AQP4 related myelitis. Conversely, the authors do not report any case of APS preceding or accompanying myelitis related to anti-GFAP.1 Given these data, APS could be an indicator for ruling out anti-GFAP encephalomyelitis, particularly useful for centers that do not yet have access to biological testing for anti-GFAP Abs.
  However, we report the case of a 41-year-old woman who in April 2016 developed intractable nausea and vomiting lasting for five weeks and leading to 35 kilograms in weight loss. An extensive search for a digestive disease was negative, and no neurological explorations were performed. One month following the resolution of digestive symptoms, she developed mental confusion, diplopia, dysarthria, dizziness, bilateral blurred vision (with optic disc edema) and paraparesis. Brain...

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  Dear Editor,

  We read with great interest the recent paper of Sechi et al. that describes 13 patients with anti-GFAP related myelitis and compares them with 41 patients with anti-AQP4 related myelitis.1 To date, very little data is available about anti-GFAP related disorders.2-3 Sechi et al. highlight some differences between the two entities to help clinicians differentiate them.1 One of these clinical differences relates to area postrema syndrome (APS). Indeed, it is well known that APS is a classical feature of neuromyelitis optica spectrum disorders, particularly among anti-AQP4 positive patients.4 Sechi et al. report this syndrome as a prodromal event in 20% of anti-AQP4 related myelitis. Conversely, the authors do not report any case of APS preceding or accompanying myelitis related to anti-GFAP.1 Given these data, APS could be an indicator for ruling out anti-GFAP encephalomyelitis, particularly useful for centers that do not yet have access to biological testing for anti-GFAP Abs.
  However, we report the case of a 41-year-old woman who in April 2016 developed intractable nausea and vomiting lasting for five weeks and leading to 35 kilograms in weight loss. An extensive search for a digestive disease was negative, and no neurological explorations were performed. One month following the resolution of digestive symptoms, she developed mental confusion, diplopia, dysarthria, dizziness, bilateral blurred vision (with optic disc edema) and paraparesis. Brain MRI was highly suggestive of anti-GFAP encephalomyelitis, notably with the presence of diffuse T2 abnormalities in periventricular white matter, with linear perivascular enhancement oriented radially to the ventricles.Spinal cord MRI revealed longitudinally extensive T2 hyperintensity (extended to the brainstem, with an upper limit involving the area postrema), with ill-defined margins, as described by Sechi et al.1. Anti-AQP4 and anti-MOG IgG were negative in the serum. Anti-GFAP IgG were detected in the serum (by cell-based assay and tissue-based assay). Lumbar puncture was performed before the patient was referred to our department, and was not repeated given the dramatic clinical improvement rapidly achieved with intravenous steroids. Among the 13 patients reported by Sechi et al., anti-GFAP IgG were detected only in the serum for two patients for which no CSF was available, like for our patient.
  The patient gave written informed consent for information and images to be published
  Our case underlines that the clinical spectrum of anti-GFAP encephalomyelitis is not fully described and understood yet and that clinical features of this syndrome may overlap with those of neuromyelitis optica spectrum disorders (related to anti-AQP4 IgG, to anti-MOG IgG or negative for both antibodies). As such, combining clinical data with radiological and biological data remains essential in order to properly classify patients with such rare central nervous system inflammatory disorders.

  References
  1 Sechi E, Morris PP, McKeon A et al. Glial fibrillary acidic protein IgG related myelitis: characterisation and comparison with aquaporin-4-IgG myelitis. J Neurol Neurosurg Neuropsychiatry 2018 (in press).
  2 Fang B, McKeon A, Hinson SR et al. Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol 2016;73(11):1297-1307.
  3 Flanagan EP, Hinson SR, Lennon VA et al. Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients. Ann Neurol 2017;81:298-309.
  4 Wingerchuk DM, Banwell B, Bennett JL et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 2015;85:177-189.

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  Conflict of Interest:
  None declared.

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