BACKGROUND: Some pediatric patients with inflammatory demyelinating central nervous system disorders cannot be classified under any of the established disease entities, making their treatment and prognosis difficult. OBJECTIVE: The objective of this study is to characterize a subgroup of pediatric patients with recurrent demyelinating central nervous system disorders. METHODS: This study includes a case series of pediatric patients with monophasic or recurrent acute disseminated encephalomyelitis (ADEM) who later presented with either monophasic or recurrent optic neuritis (ON). RESULTS: We describe seven patients with a median follow-up of six years (five females, two males) who presented at a median age of 6 years (range 4-8 years) with monophasic (n = 4) or recurrent ADEM (two to four attacks) followed by monophasic (n = 3) or recurrent ON (two to nine attacks). Cranial magnetic resonance imaging (MRI) was typical for ADEM (n = 6) with complete or almost complete resolution of lesions on follow-up. Cerebrospinal (CSF) studies at the time of ADEM showed a pleocytosis in six patients and were negative for oligoclonal bands (OCBs) in all. In all patients high titers for serum anti-MOG antibodies were detected. CONCLUSION: ADEM followed by ON is a rare but distinct clinical phenotype among pediatric patients. Further studies are needed to allow recommendations on treatment or prognosis.
BACKGROUND: Some pediatric patients with inflammatory demyelinating central nervous system disorders cannot be classified under any of the established disease entities, making their treatment and prognosis difficult. OBJECTIVE: The objective of this study is to characterize a subgroup of pediatric patients with recurrent demyelinating central nervous system disorders. METHODS: This study includes a case series of pediatric patients with monophasic or recurrent acute disseminated encephalomyelitis (ADEM) who later presented with either monophasic or recurrent optic neuritis (ON). RESULTS: We describe seven patients with a median follow-up of six years (five females, two males) who presented at a median age of 6 years (range 4-8 years) with monophasic (n = 4) or recurrent ADEM (two to four attacks) followed by monophasic (n = 3) or recurrent ON (two to nine attacks). Cranial magnetic resonance imaging (MRI) was typical for ADEM (n = 6) with complete or almost complete resolution of lesions on follow-up. Cerebrospinal (CSF) studies at the time of ADEM showed a pleocytosis in six patients and were negative for oligoclonal bands (OCBs) in all. In all patients high titers for serum anti-MOG antibodies were detected. CONCLUSION: ADEM followed by ON is a rare but distinct clinical phenotype among pediatric patients. Further studies are needed to allow recommendations on treatment or prognosis.
Authors: Yael Hacohen; Yu Yi Wong; Christian Lechner; Maciej Jurynczyk; Sukhvir Wright; Bahadir Konuskan; Judith Kalser; Anne Lise Poulat; Helene Maurey; Esther Ganelin-Cohen; Evangeline Wassmer; Chery Hemingway; Rob Forsyth; Eva Maria Hennes; M Isabel Leite; Olga Ciccarelli; Banu Anlar; Rogier Hintzen; Romain Marignier; Jacqueline Palace; Matthias Baumann; Kevin Rostásy; Rinze Neuteboom; Kumaran Deiva; Ming Lim Journal: JAMA Neurol Date: 2018-04-01 Impact factor: 18.302
Authors: Cristina Fernandez-Carbonell; David Vargas-Lowy; Alexander Musallam; Brian Healy; Katherine McLaughlin; Kai W Wucherpfennig; Tanuja Chitnis Journal: Mult Scler Date: 2015-06-03 Impact factor: 6.312
Authors: Matthias Baumann; Astrid Grams; Tanja Djurdjevic; Eva-Maria Wendel; Christian Lechner; Bettina Behring; Astrid Blaschek; Katharina Diepold; Astrid Eisenkölbl; Joel Fluss; Michael Karenfort; Johannes Koch; Bahadir Konuşkan; Steffen Leiz; Andreas Merkenschlager; Daniela Pohl; Mareike Schimmel; Charlotte Thiels; Barbara Kornek; Kathrin Schanda; Markus Reindl; Kevin Rostásy Journal: J Neurol Date: 2018-02-08 Impact factor: 4.849