Contributions
Abstract: P337
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - Diagnosis and differential diagnosis
Acute demyelinating syndromes (ADS) are frequently associated with anti-MOG antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are wrongly considered as multiple sclerosis (MS). Here, we describe three children with progressive cognitive and motor impairment, epilepsy and behavior disorders. The brain MRI showed diffused bilateral white matter injuries including optic nerves, periventricular regions, corpus callosum, cerebellum and spinal cord. Cerebrospinal fluid (CSF) analysis showed high levels of proteins in two cases and meningitis with a majority of lymphocytes in two cases. There were no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Due to their atypical presentation, brain biopsies were performed in two children and showed white matter lesions with no argument for histiocytosis nor for tumor. Steroids were ineffective, clinical and radiological improvement and stabilization were obtained after active immunotherapy associating Mitoxantrone in two patients and Natalizumab in one of them. After nine years of follow up, all three children have cognitive impairment. A retrospective analysis for anti-MOG antibodies in these children at onset of disease was positive, and two children turned seronegative after treatment and during follow-up. Leucopathy-like ADS with anti-MOG-antibodies display distinct phenotypes and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.
Key words: Acute Demyelinating Syndromes (ADS), leucopathy-like, anti-MOG antibodies, Multiple Sclerosis (MS), immunotherapy
Disclosure: No conflict of interest
Abstract: P337
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - Diagnosis and differential diagnosis
Acute demyelinating syndromes (ADS) are frequently associated with anti-MOG antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are wrongly considered as multiple sclerosis (MS). Here, we describe three children with progressive cognitive and motor impairment, epilepsy and behavior disorders. The brain MRI showed diffused bilateral white matter injuries including optic nerves, periventricular regions, corpus callosum, cerebellum and spinal cord. Cerebrospinal fluid (CSF) analysis showed high levels of proteins in two cases and meningitis with a majority of lymphocytes in two cases. There were no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Due to their atypical presentation, brain biopsies were performed in two children and showed white matter lesions with no argument for histiocytosis nor for tumor. Steroids were ineffective, clinical and radiological improvement and stabilization were obtained after active immunotherapy associating Mitoxantrone in two patients and Natalizumab in one of them. After nine years of follow up, all three children have cognitive impairment. A retrospective analysis for anti-MOG antibodies in these children at onset of disease was positive, and two children turned seronegative after treatment and during follow-up. Leucopathy-like ADS with anti-MOG-antibodies display distinct phenotypes and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.
Key words: Acute Demyelinating Syndromes (ADS), leucopathy-like, anti-MOG antibodies, Multiple Sclerosis (MS), immunotherapy
Disclosure: No conflict of interest