Paraneoplastic encephalomyeloradiculits with multiple autoantibodies against ITPR-1, GFAP and MOG: case report and literature review.
Autoantibody
Encephalitis
Encephalomyelitis
Encephalomyeloradiculits
GFAP
ITPR-1
MOG
Multiple antibodies
Paraneoplastic
Journal
Neurological research and practice
ISSN: 2524-3489
Titre abrégé: Neurol Res Pract
Pays: England
ID NLM: 101767802
Informations de publication
Date de publication:
11 Oct 2021
11 Oct 2021
Historique:
received:
31
05
2021
accepted:
23
07
2021
entrez:
12
10
2021
pubmed:
13
10
2021
medline:
13
10
2021
Statut:
epublish
Résumé
Recently, antibodies against the alpha isoform of the glial-fibrillary-acidic-protein (GFAPα) were identified in a small series of patients with encephalomyelitis. Coexisting autoantibodies (NMDA receptor, GAD65 antibodies) have been described in a few of these patients. We describe a patient with rapidly progressive encephalomyeloradiculitis and a combination of anti-ITPR1, anti-GFAP and anti-MOG antibodies. A 44-year old caucasian woman with a flu-like prodrome presented with meningism, progressive cerebellar signs and autonomic symptoms, areflexia, quadriplegia and respiratory insufficiency. MRI showed diffuse bilateral T2w-hyperintense brain lesions in the cortex, white matter, the corpus callosum as well as a longitudinal lesion of the medulla oblongata and the entire spinal cord. Anti-ITPR1, anti-GFAP and anti-MOG antibodies were detected in cerebrospinal fluid along with lymphocytic pleocytosis. Borderline tumor of the ovary was diagnosed. Thus, the disease of the patient was deemed to be paraneoplastic. The patient was treated by surgical removal of tumor, steroids, immunoglobulins, plasma exchange and rituximab. Four months after presentation, the patient was still tetraplegic, reacted with mimic expressions to pain or touch and could phonate solitary vowels. An extensive literature research was performed. Our case and the literature review illustrate that multiple glial and neuronal autoantibodies can co-occur, that points to a paraneoplastic etiology, above all ovarian teratoma or thymoma. Clinical manifestation can be a mixture of typically associated syndromes, e.g. ataxia associated with anti-ITPR1 antibodies, encephalomyelitis with anti-GFAPα antibodies and longitudinal extensive myelitis with anti-MOG antibodies.
Sections du résumé
BACKGROUND
BACKGROUND
Recently, antibodies against the alpha isoform of the glial-fibrillary-acidic-protein (GFAPα) were identified in a small series of patients with encephalomyelitis. Coexisting autoantibodies (NMDA receptor, GAD65 antibodies) have been described in a few of these patients. We describe a patient with rapidly progressive encephalomyeloradiculitis and a combination of anti-ITPR1, anti-GFAP and anti-MOG antibodies.
CASE PRESENTATION AND LITERATURE REVIEW
UNASSIGNED
A 44-year old caucasian woman with a flu-like prodrome presented with meningism, progressive cerebellar signs and autonomic symptoms, areflexia, quadriplegia and respiratory insufficiency. MRI showed diffuse bilateral T2w-hyperintense brain lesions in the cortex, white matter, the corpus callosum as well as a longitudinal lesion of the medulla oblongata and the entire spinal cord. Anti-ITPR1, anti-GFAP and anti-MOG antibodies were detected in cerebrospinal fluid along with lymphocytic pleocytosis. Borderline tumor of the ovary was diagnosed. Thus, the disease of the patient was deemed to be paraneoplastic. The patient was treated by surgical removal of tumor, steroids, immunoglobulins, plasma exchange and rituximab. Four months after presentation, the patient was still tetraplegic, reacted with mimic expressions to pain or touch and could phonate solitary vowels. An extensive literature research was performed.
CONCLUSION
CONCLUSIONS
Our case and the literature review illustrate that multiple glial and neuronal autoantibodies can co-occur, that points to a paraneoplastic etiology, above all ovarian teratoma or thymoma. Clinical manifestation can be a mixture of typically associated syndromes, e.g. ataxia associated with anti-ITPR1 antibodies, encephalomyelitis with anti-GFAPα antibodies and longitudinal extensive myelitis with anti-MOG antibodies.
Identifiants
pubmed: 34635185
doi: 10.1186/s42466-021-00145-w
pii: 10.1186/s42466-021-00145-w
pmc: PMC8504129
doi:
Types de publication
Journal Article
Langues
eng
Pagination
48Informations de copyright
© 2021. The Author(s).
Références
Clin Immunol. 2011 Mar;138(3):247-54
pubmed: 21169067
Neurol Neurochir Pol. 2017 Jan - Feb;51(1):86-91
pubmed: 27908616
Neurol Neuroimmunol Neuroinflamm. 2017 Dec 08;5(1):e418
pubmed: 29379822
Front Immunol. 2017 May 08;8:529
pubmed: 28533781
JAMA Neurol. 2016 Nov 1;73(11):1297-1307
pubmed: 27618707
J Neuroinflammation. 2016 Oct 24;13(1):278
pubmed: 27776522
Circulation. 2013 Sep 17;128(12):1286-97
pubmed: 23983250
Ann Neurol. 2017 Feb;81(2):298-309
pubmed: 28120349
J Neuroinflammation. 2015 Sep 17;12:166
pubmed: 26377085
Development. 1996 Mar;122(3):1029-39
pubmed: 8631248
J Neuroinflammation. 2016 Nov 1;13(1):281
pubmed: 27802825
J Neuroinflammation. 2014 Dec 11;11:206
pubmed: 25498830
J Neurol Neurosurg Psychiatry. 2020 Dec 28;:
pubmed: 33372052
Neuroimmunomodulation. 2017;24(2):113-119
pubmed: 28922662
Neurol Neuroimmunol Neuroinflamm. 2017 Feb 03;4(2):e326
pubmed: 28203616