Glial fibrillary acidic protein IgG related myelitis: characterisation and comparison with aquaporin-4-IgG myelitis

Sechi, Elia; Morris, Pearse; McKeon, Andrew; Pittock, Sean J.; Hinson, Shannon R.; Weinshenker, Brian G.; Aksamit, Allen J.; Krecke, Karl N.; Kaufmann, Timothy J.; Jolliffe, Evan; Zalewski, Nicholas L.; Ζεκερίδου, Αναστασία; Wingerchuk, Dean M.; Jitprapaikulsan, Jiraporn; Flanagan, Eoin P.

doi:10.1136/jnnp-2018-318004

Cited by 54 publications

(63 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This recent entity is defined as a distinct autoimmune astrocytopathy, responsible for a corticosteroid-responsive meningo-encephalomyelitis. Nevertheless, in the recent publication of 13 patients with LETM associated to GFAP-antibodies [30], all have extraspinal symptoms simultaneously or preceding myelitis onset, on the contrary to our patients. Such as recent identification of GFAP antibodies, other antibodies could be identified in the future and help to break down this heterogeneous entity of seronegative LETM.…”

Section: Limitationscontrasting

confidence: 85%

Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies

Maillart

Durand‐Dubief²,

Louapre

et al. 2020

J Neuroinflammation

View full text Add to dashboard Cite

Background: Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for any diagnosis, despite a large work-up including AQP4-Ab and MOG-Ab. Data about natural history, disability outcome, and treatment are limited in this group of patients. We aimed to (1) describe clinical, biological, and radiological features of double seronegative LETM patients; (2) assess the clinical course and identify prognostic factors; and (3) assess the risk of recurrence, according to maintenance immunosuppressive therapy. Methods: Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS. Results: Fifty-three patients (median age 38 years (range 16-80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1-8.5), associated to a median EDSS at last follow-up of 4.0 (0-8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%). Conclusions:A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition.

show abstract

Section: Limitationscontrasting

confidence: 85%

Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies

Maillart

Durand‐Dubief²,

Louapre

et al. 2020

J Neuroinflammation

View full text Add to dashboard Cite

show abstract

“…However, meningoencephalitis and meningoencephalomyelitis were the predominant phenotypes in previous reports, with isolated myelitis being rare. 4,5,7 In our case, this patient met the diagnosis of autoimmune GFAP astrocytopathy rather than NMOSD. Specifically, GFAP antibody was detected in CSF, which is considered a specific biomarker for autoimmune GFAP astrocytopathy.…”

Section: Discussionmentioning

confidence: 54%

Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord

Li¹,

Han

Zhao

et al. 2020

Ther Adv Neurol Disord

View full text Add to dashboard Cite

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy has been considered a novel central nervous system autoimmune disease characterized by relapse and responsiveness to corticosteroid with a specific GFAP-Immunoglobulin G (IgG) being noted in cerebrospinal fluid. We report the case of a 21-year-old girl presenting with dysuria and weariness, who subsequently developed blurry vision, slight dysphagia, slurred speech, and sensory abnormality. GFAP-IgG was detected in her cerebrospinal fluid. Magnetic resonance imaging using both T2-weighted and contrast-enhanced T1-weighted images revealed a rare finding of lesions distributed mainly in the entire spinal cord rather than typical brain lesions. After treating with corticosteroids, her clinical symptoms were alleviated, and the spinal cord lesion enhancement was reduced. Our observations extend the clinical spectrum of autoimmune GFAP astrocytopathy. We suggest that rare distributed lesions in the entire spinal cord in patients with autoimmune GFAP astrocytopathy cannot be ignored by neurologists. The identification of potential atypical lesions broadens the understanding of autoimmune GFAP astrocytopathy.

show abstract

“…Clinicians should have a low threshold for checking aquaporin-4-IgG via cell-based assay (CBA), particularly if lesions are longitudinally extensive with patchy or ring-like contrast enhancement. 3 MOG-IgG myelitis (evaluated via serum CBA) is a consideration with conus involvement and faint/ no contrast enhancement. 4 Seronegative NMOSD is rare and an alternative etiology is often discovered when investigated thoroughly.…”

Section: Sectionmentioning

confidence: 99%

“…4 Seronegative NMOSD is rare and an alternative etiology is often discovered when investigated thoroughly. 3,4 Neurosarcoidosis is characterized by avid gadolinium enhancement (typically dorsal subpial predominant) and inflammatory CSF, making this diagnosis unlikely. 1 Isolated paraneoplastic myelopathies are rare, typically presenting as tractopathy with progressive course.…”

Section: Sectionmentioning

confidence: 99%

Clinical Reasoning: A 70-year-old man with rapid stepwise paraparesis and sensory loss

2020

View full text Add to dashboard Cite

A 70-year-old man with a history of cardioembolic stroke, hypertension, hyperlipidemia, peripheral vascular disease, and recently diagnosed myelodysplastic syndrome presented to an outside facility with acute sensory loss in the left buttock and lower extremity without weakness. Spine MRI demonstrated nonenhancing T2-hyperintense lesions in the upper thoracic cord and conus medullaris. MRI brain showed areas of nonspecific T2-hyperintensity. CSF revealed 20 total nucleated cells (TNC)/μL; 11,000 red blood cells (RBC)/μL; normal glucose, protein, and immunoglobulin G (IgG) index; 0 unique CSF oligoclonal bands; negative Gram stain, herpes simplex virus (HSV) 1/2 PCR, and Borrelia PCR; cytology was normal. Pleocytosis was attributed to traumatic lumbar puncture (utilizing correction of 1 TNC/μL per 500 RBC/μL), and history was inconsistent with HSV or subarachnoid hemorrhage as a cause of elevated RBCs. Serum aquaporin-4-IgG was negative. Mild anemia and thrombocytopenia were apparent. He was discharged with a provisional diagnosis of demyelinating disease. Three weeks later, he developed acute paraparesis with bladder and bowel dysfunction within hours. Repeat MRI at the outside institution showed enlargement of the nonenhancing lesions in the upper thoracic cord, now longitudinally extensive (≥3 vertebral body segments), and the lower thoracic cord extending into the conus (figure, A and E). Given concern for progressive demyelination due to motor decline and expanding lesions, IV methylprednisolone was initiated. Despite treatment, his paraparesis worsened and he was transferred to our facility. Collateral history revealed 2 months of fatigue attributed to myelodysplastic syndrome, but no preceding infections, travel, or rashes. The patient was afebrile and hemodynamically stable. Neurologic examination demonstrated asymmetric flaccid paraparesis with plegia of the left lower extremity, saddle anesthesia, and loss of vibratory and pain sensation to the midthigh bilaterally with no truncal sensory level. He was areflexic in the lower extremities with mute plantar responses. Questions for consideration: 1. What is the best localization of the clinical presentation? 2. What diagnoses should be considered?

show abstract

Glial fibrillary acidic protein IgG related myelitis: characterisation and comparison with aquaporin-4-IgG myelitis

Cited by 54 publications

References 5 publications

Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies

Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies

Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord

Clinical Reasoning: A 70-year-old man with rapid stepwise paraparesis and sensory loss

Contact Info

Product

Resources

About