Encephalopathy due to anti-GFAP antibodies in a patient with rheumatoid arthritis

Bueno, Marvin; Acero, Sofia; Zuluaga, Paola

doi:10.1016/j.medcle.2023.03.034

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Subtle hyperdensity of the right lentiform nucleus (arrow). In the patient's context, it may be related to a chorea hyperglycaemia basal ganglia syndrome-related abnormality." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Joaquín Valle Alonso, Leandro Noblia Gamba, Esther Montoro Jorquera" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Joaquín" "apellidos" => "Valle Alonso" ] 1 => array:2 [ "nombre" => "Leandro" "apellidos" => "Noblia Gamba" ] 2 => array:2 [ "nombre" => "Esther" "apellidos" => "Montoro Jorquera" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0025775323002270" "doi" => "10.1016/j.medcli.2023.03.034" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775323002270?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020623003686?idApp=UINPBA00004N" "url" => "/23870206/0000016100000007/v2_202311091450/S2387020623003686/v2_202311091450/en/main.assets" ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "Letter to the Editor" "titulo" => "Encephalopathy due to anti-GFAP antibodies in a patient with rheumatoid arthritis" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "317" "paginaFinal" => "318" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Marvin Bueno, Sofia Acero, Paola Zuluaga" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Marvin" "apellidos" => "Bueno" ] 1 => array:2 [ "nombre" => "Sofia" "apellidos" => "Acero" ] 2 => array:4 [ "nombre" => "Paola" "apellidos" => "Zuluaga" "email" => array:1 [ 0 => "ypzuluaga.germanstrias@gencat.cat" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "University Hospital Germans Trias i Pujol, Barcelona, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Encefalopatía por anticuerpos anti-GFAP en paciente con artritis reumatoide" ] ] "textoCompleto" => "Glial fibrillary acidic protein (GFAP) autoimmune astrocytopathy is a form of autoimmune encephalitis recently described in 2016, based on the presence of IgG against GFAP present in the cytoplasm of astrocytes.<a class="elsevierStyleCrossRef" href="#bib0025">1</a>We present the case of 67 years old women diagnosed with seronegative rheumatoid arthritis, who developed non-paraneoplastic autoimmune encephalitis due to anti-GFAP antibodies in cerebrospinal fluid (CSF). Her medical history included a history of seronegative rheumatoid arthritis, for which she received several immunosuppressive drugs, such as methotrexate and adalimumab. She was currently under treatment with leflunomide and methylprednisolone at a dose of 4mg/day. The patient went to the emergency department due to progressive functional decline, generalized weakness, difficulty walking, behavioral changes and impaired speech in the last month. Physical examination revealed motor weakness in the lower limbs with hyporeflexia and preserved sensitivity and in the blood test hypokalemia of 1.7mmol/L and hyponatremia of 124mmol/L. During the hospitalization, an electroencephalogram was performed showing mildly slowed activity without epileptiform alterations. The computed tomography (CT) cranial scan and the magnetic resonance imaging (MRI) of the brain were normal and a MRI of the spinal cord ruled out the presence of signs of myelopathy or focal lesions. An immunological study included antibodies related to autoimmune encephalitis, onconeuronal antibodies and anti-neuronal surface antibodies (anti-NMDA receptor; AMPA; GABAa; GABAb; mGluR2; mGluR5; DPPX; IgLONS5; Neurexin; LGI1 and CASPR2), all of them were negative. The CSF study revealed lymphocytic pleocytosis, higher concentration of proteins (2.5g/L) and negative viral panel (Epstein Barr Virus, Varicella Zoster Virus, Cytomegalovirus, Herpes Simplex Virus 6, Enterovirus and Parechovirus). Finally, anti-GFAP antibodies by immunohistochemistry were isolated in CSF, confirmed by cellular assay. No anti-GFAP antibodies in serum were obtained, as well as no other types of onconeuronal or neuronal anti-surface antibodies in CSF. Treatment with corticotherapy at a dose of 1mg/kg/day was started, with progressive improvement and resolution of the neurological clinic in two months after the treatment. Subsequently, a Positron Emission Tomography – CT scan was performed, which ruled out underlying neoplasms.Autoimmune astrocytopathy due to anti-GFAP antibodies is a meningoencephalomyelitis characterized by acute or subacute symptoms of meningitis (headache), encephalitis (delirium, behavioral alterations) and/or myelitis (loss of strength or alterations in sensitivity).<a class="elsevierStyleCrossRef" href="#bib0030">2</a> In our case, the patient presented with a subacute course of the disease with the characteristic clinical manifestations. Severe hypokalemia has been previously described although it is an unusual finding in this entity.<a class="elsevierStyleCrossRef" href="#bib0035">3</a> The disease occurs mostly in women, and in 20% of cases, it coexists with other autoimmune syndromes.<a class="elsevierStyleCrossRef" href="#bib0040">4</a> There are few cases in the literature describing coexistence with rheumatoid arthritis, and the effect of chronic immunotherapy, either with glucocorticoids or with other immunomodulators, remains unknown. There is limited information regarding their pathogenesis but is associated with neoplastic diseases, before and after the diagnosis and has been associated with an infiltration of glial tissue by macrophages and CD8+ T lymphocytes.<a class="elsevierStyleCrossRef" href="#bib0025">1</a> The characteristic MRI feature is brain linear perivascular radial gadolinium enhancement in the white matter perpendicular to the ventricle.<a class="elsevierStyleCrossRefs" href="#bib0030">2,4</a> In our case, the brain MRI did not show any finding suggestive of encephalitis. In the CSF, study is observed positivity for anti-GFAP antibodies by immunohistochemistry, which is confirmed by cellular assay. Despite of the described findings, there are no defined diagnostic criteria for this entity.<a class="elsevierStyleCrossRef" href="#bib0030">2</a> Treatment of the acute phase of anti-GFAP astropathy includes high-dose corticosteroid therapy, intravenous immunoglobulins and plasma exchange. Characteristically it presents a good response to corticosteroids.<a class="elsevierStyleCrossRef" href="#bib0040">4</a>FundingScholarships: Economy and Competitiveness, Institute of Health Carlos III, Spain (RD21/0009/0004, Programa Juan Rodes JR20/00016, gran number PI20/00883) and the Agency for Management of University and Research Grants, Government of Catalonia (grant number 2021SGR00945)." 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Aksamit" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1001/jamaneurol.2016.2549" "Revista" => array:6 [ "tituloSerie" => "JAMA Neurol" "fecha" => "2016" "volumen" => "73" "paginaInicial" => "1297" "paginaFinal" => "1307" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27618707" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0030" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Autoimmune glial fibrillary acidic protein astrocytopathy: a review of the literature" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "F. Shan" 1 => "Y. Long" 2 => "W. Qiu" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3389/fimmu.2018.02802" "Revista" => array:5 [ "tituloSerie" => "Front Immunol" "fecha" => "2018" "volumen" => "9" "paginaInicial" => "2802" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/30568655" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0035" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Uncommon manifestations of a rare disease: a case of autoimmune GFAP astrocytopathy" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "H. Di" 1 => "Y. Yin" 2 => "R. Chen" 3 => "Y. Zhang" 4 => "J. Ni" 5 => "X. Zeng" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1186/s12883-021-02070-6" "Revista" => array:5 [ "tituloSerie" => "BMC Neurol" "fecha" => "2021" "volumen" => "21" "paginaInicial" => "37" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/33504323" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0040" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Autoimmune glial fibrillary acidic protein astrocytopathy" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "A. Kunchok" 1 => "A. Zekeridou" 2 => "A. McKeon" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1097/WCO.0000000000000676" "Revista" => array:6 [ "tituloSerie" => "Curr. Opin. Neurol." 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Vol. 161. Issue 7.

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