Contributions
Abstract: EP1314
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - MS Variants
Background: Anti-MOG (myelin oligodendrocyte glycoprotein) antibodies (Abs) have been recently related to aquaporin-4 (AQP4) Ab negative neuromyelitis optica spectrum disorder (NMOSD) cases. Treatment of MOG Ab-associated disease is poorly standardized: several drugs have been employed, with variable results, in small case series. Very few adult patients with MOG Ab-associated disease have been treated with long-term intravenous immunoglobulins (IVIg), with different schedules. Here we report a case of relapsing MOG Ab-associated disease treated with an IVIg empirical therapeutic strategy.
Case report: A 50-year-old Caucasian male was admitted to hospital in 2009, with symptoms and signs of severe acute transverse myelitis (urinary retention, paraparesis and legs dysesthesias). Expanded disability status scale (EDSS) score was 6.0. Moreover, he reported three previous episodes of dizziness and diplopia, suggestive of demyelinating attacks. Brain and spinal cord MRI showed multiple demyelinating lesions. Cerebrospinal fluid analysis revealed no oligoclonal bands (OCBs). Many rheumatologic, infectious and autoimmune diseases were excluded by biochemical tests, including anti-AQP4 Abs. After steroid pulse therapy, he had good recovery (EDSS 2.0) and a diagnosis of multiple sclerosis (MS) was made. A subsequent MRI showed disappearance of all the lesions. He was treated with interferon-beta 1a, then with fingolimod, and finally with rituximab. All these treatments were ineffective: he experienced several spinal and brainstem relapses, with residual disability. In July 2016 an empirical therapy with IVIg was started at the dosage of 2 gr/kg in five days (first cycle), then 0,8 gr/kg in two days every three weeks. Calling into question the diagnosis of MS, we performed another lumbar puncture (OCBs still negative), serum anti-AQP4 test (negative) and anti-MOG test (positive). After the diagnosis of MOG Ab-associated disease, we decided to continue with IVIg therapy, considering the initial good results. During a 22-month follow-up, the patient experienced only one mild relapse (annualized relapse rate decreased from 3.0 to 0.57), while EDSS remained stable (4.0). Two consecutive brain and spinal MRI revealed no new lesions.
Conclusions: Our patient, with an aggressive and atypical MOG Ab-associated disease, showed a very good response to long-term IVIg treatment. IVIg therapy should be considered in all the cases of aggressive MOG Ab-associated disease.
Disclosure: Elena Tsantes served on scientific advisory boards for Roche and has received funding for travel from Biogen, Merck Serono and Sanofi Genzyme.
Erica Curti served on scientific advisory boards for Merck Serono and has received funding for travel from Biogen, Merck Serono,Novartis, Sanofi Genzyme, and Roche.
Ernesto Siena has received funding for travel from Biogen, Almirall and Sanofi Genzyme.
Franco Granella served on scientific advisory boards for Biogen, Merck Serono, Novartis, and Sanofi Genzyme. He received funding for travel and speaker honoraria from Biogen Idec, Merck Serono, and Sanofi Genzyme. His institution received research grants from Biogen and Sanofi Genzyme.
Abstract: EP1314
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - MS Variants
Background: Anti-MOG (myelin oligodendrocyte glycoprotein) antibodies (Abs) have been recently related to aquaporin-4 (AQP4) Ab negative neuromyelitis optica spectrum disorder (NMOSD) cases. Treatment of MOG Ab-associated disease is poorly standardized: several drugs have been employed, with variable results, in small case series. Very few adult patients with MOG Ab-associated disease have been treated with long-term intravenous immunoglobulins (IVIg), with different schedules. Here we report a case of relapsing MOG Ab-associated disease treated with an IVIg empirical therapeutic strategy.
Case report: A 50-year-old Caucasian male was admitted to hospital in 2009, with symptoms and signs of severe acute transverse myelitis (urinary retention, paraparesis and legs dysesthesias). Expanded disability status scale (EDSS) score was 6.0. Moreover, he reported three previous episodes of dizziness and diplopia, suggestive of demyelinating attacks. Brain and spinal cord MRI showed multiple demyelinating lesions. Cerebrospinal fluid analysis revealed no oligoclonal bands (OCBs). Many rheumatologic, infectious and autoimmune diseases were excluded by biochemical tests, including anti-AQP4 Abs. After steroid pulse therapy, he had good recovery (EDSS 2.0) and a diagnosis of multiple sclerosis (MS) was made. A subsequent MRI showed disappearance of all the lesions. He was treated with interferon-beta 1a, then with fingolimod, and finally with rituximab. All these treatments were ineffective: he experienced several spinal and brainstem relapses, with residual disability. In July 2016 an empirical therapy with IVIg was started at the dosage of 2 gr/kg in five days (first cycle), then 0,8 gr/kg in two days every three weeks. Calling into question the diagnosis of MS, we performed another lumbar puncture (OCBs still negative), serum anti-AQP4 test (negative) and anti-MOG test (positive). After the diagnosis of MOG Ab-associated disease, we decided to continue with IVIg therapy, considering the initial good results. During a 22-month follow-up, the patient experienced only one mild relapse (annualized relapse rate decreased from 3.0 to 0.57), while EDSS remained stable (4.0). Two consecutive brain and spinal MRI revealed no new lesions.
Conclusions: Our patient, with an aggressive and atypical MOG Ab-associated disease, showed a very good response to long-term IVIg treatment. IVIg therapy should be considered in all the cases of aggressive MOG Ab-associated disease.
Disclosure: Elena Tsantes served on scientific advisory boards for Roche and has received funding for travel from Biogen, Merck Serono and Sanofi Genzyme.
Erica Curti served on scientific advisory boards for Merck Serono and has received funding for travel from Biogen, Merck Serono,Novartis, Sanofi Genzyme, and Roche.
Ernesto Siena has received funding for travel from Biogen, Almirall and Sanofi Genzyme.
Franco Granella served on scientific advisory boards for Biogen, Merck Serono, Novartis, and Sanofi Genzyme. He received funding for travel and speaker honoraria from Biogen Idec, Merck Serono, and Sanofi Genzyme. His institution received research grants from Biogen and Sanofi Genzyme.