Clinical time course of pediatric acute disseminated encephalomyelitis

Abstract

The detailed clinical time course in acute disseminated encephalomyelitis (ADEM) from initial symptoms, through exacerbation, to remission has not been widely reported. Hence, this study aimed to investigate the clinical time course of pediatric ADEM. This was a multicenter retrospective study based on registry data from medical chart reviews. The study included children who met the international consensus diagnostic criteria for ADEM. The patients comprised 18 boys and 6 girls, with a mean age of 5.5±3.3 years at onset. From onset, the time until peak neurological symptoms, time until initial improvement, and time until full recovery was 3.1±3.7 days, 6.0±4.5 days, and 26±34 days, respectively. Twenty-three (96%) patients were treated with high-dose methylprednisolone (mPSL) with a mean duration of 4.1±4.0 days from onset. The condition of 15 patients (65%) improved within 3 days of high-dose mPSL initiation, whereas, that of four patients began to improve after >5 days of high-dose mPSL initiation. Only one patient (4%) did not achieve full recovery despite treatment with high-dose mPSL, intravenous immunoglobulin, and plasma exchange. This study presents the detailed clinical time course in pediatric ADEM in Japan. Progression of neurologic deficits typically lasts a few days, with initial improvement in 1 week leading to full recovery within 1 month. Day 140 Day 122 Day 42 Day Case Worsening period Nadir period Improving period Initiation of high-dose mPSL Initiation of IVIg 1 11 21 31

Clinical and Neuroimaging Characteristics of Pediatric Acute Disseminating Encephalomyelitis With and Without Antibodies to Myelin Oligodendrocyte Glycoprotein

“…A higher median mRS was recorded in the ADEM without MOG-abs group before immunotherapy; however, no significant difference existed between the groups during the last follow-up. Furthermore, the median time from immunotherapy to an improvement in clinical symptoms was 7 (range: 3–30) days and 8 (range: 4–30) days in the ADEM with MOG-abs and without MOG-abs groups, respectively, which is consistent with the findings of a previous study ( 27 ). This finding suggests that the progression of neurologic deficits in pediatric ADEM typically only lasts a few days, with the initial improvement occurring within 1 week, leading to a full recovery within 1 month.…”

“…According to the updated recommended treatment approaches for MOGAD in children, first-line immunotherapy normally consists of intravenous corticosteroids, IVIG, and PE in isolation or combination ( 26 ). The prognosis of ADEM is typically favorable, and the complete recovery rate has been reported as between 57 and 92% in several pediatric cohorts of ADEM ( 27 ). Patients with MOG-abs and a monophasic disease course often have good clinical recovery and lesion resolution in the context of declining anti-MOG-abs MOG-abs ( 5 ).…”

Fulminant Acute Disseminated Encephalomyelitis: A Remarkable Outcome with Cyclophosphamide

“…[8][9][10][11] In 2007 the International Pediatric Multiple Sclerosis Society Group (IPMSSG) published consensus definitions for demyelinating disorders of childhood, including ADEM, 12 which was updated in 2013. 13 The clinical presentation has reportedly a latent period between anteceding viral infection and onset of symptoms of approximately 12 days, 14,15 during which prodromal symptoms can include fever, malaise, headache, nausea, and vomiting progressing to encephalopathy and coma. 1,2,7 The mean time to maximum symptoms is around 4 to 5 days.…”

Acute disseminated encephalomyelitis in children and adults: A focused review emphasizing new developments

“…12,14 The latency period between infection and onset of typical symptoms averages approximately 2 weeks but ranges from 0 to 60 days. 12,15 Whether infections occurring >60 days prior to presentation are causally or coincidentally associated with ADEM remains controversial.…”