Brain, Vol. 123, No. 12, 2407-2422,
December 2000
© 2000 Oxford University Press
Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children
1 Departments of Neurology, 2 Radiology and 3 Histopathology, Great Ormond Street Hospital for Children and 4 Institute of Child Health, University College London, London, UK
Correspondence to:
Dr Russell Dale, Department of Neurology, Great Ormond Street Hospital for Children, London WC1 3JH, UK E-mail: russ{at}russdale.fsnet.co.uk
Forty-eight children with disseminated demyelination of the CNS, 28 with acute disseminated encephalomyelitis (ADEM), seven with multiphasic disseminated encephalomyelitis (MDEM) and 13 with multiple sclerosis were studied for a mean follow-up period of 5.64 years. The presentation findings of the ADEM/MDEM group were compared with those of the multiple sclerosis group. The following findings were more commonly seen in ADEM/MDEM presentation compared with the multiple sclerosis presentations: predemyelinating infectious disease (74 versus 38%, P < 0.05); polysymptomatic presentation (91 versus 38%, P < 0.002); pyramidal signs (71 versus 23%, P < 0.01); encephalopathy (69 versus 15%, P < 0.002); and bilateral optic neuritis (23 versus 8%, not significant). Seizures occurred only in the ADEM/MDEM group (17 versus 0%, not significant). Unilateral optic neuritis occurred only in the multiple sclerosis patients (23 versus 0%, P < 0.01). There were no differences in the frequencies of transverse myelitis, brainstem involvement, cerebellar signs and sensory disturbance between the two groups. ADEM/MDEM patients were more likely to have blood leucocytosis (64 versus 22%, P < 0.05), CSF lymphocytosis (64 versus 42%, not significant) and CSF protein elevation (60 versus 33%, not significant). Patients presenting with multiple sclerosis were more likely to have intrathecal synthesis of oligoclonal bands on presentation (64 versus 29%, not significant). MRI showed that subcortical white matter lesions were almost universal in both groups, though periventricular lesions were more common in multiple sclerosis (92 versus 44%, P < 0.01). By contrast, in ADEM/MDEM there was absolute and relative periventricular sparing in 56 and 78% of patients, respectively. Follow-up MRI revealed complete or partial lesion resolution in 90% and no new lesions in the ADEM/MDEM group. All of the multiple sclerosis patients had new lesions on repeat MRI (five during relapse and six during asymptomatic convalescent phases). The outcome in the ADEM patients was mixed; 57% of patients made a complete recovery. The mean follow-up for the 35 ADEM/MDEM patients was 5.78 years (range 1.0–15.4 years). Eight of the 13 multiple sclerosis patients relapsed within the first year; 11 had a relapsing–remitting course, one a primary progressive course and one a secondary progressive course. These differences in the presentation of ADEM/MDEM compared with multiple sclerosis may help in the prognosis given to families regarding the possibility of later development of multiple sclerosis.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  E Portaccio, B Goretti, S Lori, V Zipoli, S Centorrino, A Ghezzi, F Patti, V Bianchi, G Comi, M Trojano, et al. The brief neuropsychological battery for children: a screening tool for cognitive impairment in childhood and juvenile multiple sclerosis Multiple Sclerosis, May 1, 2009; 15(5): 620 - 626. [Abstract] [PDF]
|
|
|
|
 |
|
 E. M. Yiu, A. J. Kornberg, M. M. Ryan, L. T. Coleman, and M. T. Mackay Acute Transverse Myelitis and Acute Disseminated Encephalomyelitis in Childhood: Spectrum or Separate Entities? J Child Neurol, March 1, 2009; 24(3): 287 - 296. [Abstract] [PDF]
|
|
|
|
|
|
M Atzori, P. Battistella, P Perini, M Calabrese, M Fontanin, A. Laverda, A Suppiej, P Drigo, P Grossi, L Rinaldi, et al. Clinical and diagnostic aspects of multiple sclerosis and acute monophasic encephalomyelitis in pediatric patients: a single centre prospective study Multiple Sclerosis, March 1, 2009; 15(3): 363 - 370. [Abstract] [PDF]
|
|
|
|
 |
|
 M. P. Amato, B. Goretti, A. Ghezzi, S. Lori, V. Zipoli, E. Portaccio, L. Moiola, M. Falautano, M. F. De Caro, M. Lopez, et al. Cognitive and psychosocial features of childhood and juvenile MS Neurology, May 13, 2008; 70(20): 1891 - 1897. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. de Seze Differential Diagnosis Between Acute Disseminated Encephalomyelitis and Multiple Sclerosis During the First Episode--Reply Arch Neurol, May 1, 2008; 65(5): 677 - 677. [Full Text] [PDF]
|
|
|
|
|
|
S. Mesaros, M. A. Rocca, M. Absinta, A. Ghezzi, N. Milani, L. Moiola, P. Veggiotti, G. Comi, and M. Filippi Evidence of thalamic gray matter loss in pediatric multiple sclerosis Neurology, March 25, 2008; 70(13_Part_2): 1107 - 1112. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. de Seze, M. Debouverie, H. Zephir, C. Lebrun, F. Blanc, V. Bourg, S. Wiertlewski, S. Pittion, D. Laplaud, E. Le Page, et al. Acute Fulminant Demyelinating Disease: A Descriptive Study of 60 Patients Arch Neurol, October 1, 2007; 64(10): 1426 - 1432. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. B. Krupp, B. Banwell, S. Tenembaum, and for the International Pediatric MS Study Group Consensus definitions proposed for pediatric multiple sclerosis and related disorders Neurology, April 17, 2007; 68(16_suppl_2): S7 - S12. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. S. Hahn, D. Pohl, M. Rensel, S. Rao, and for the International Pediatric MS Study Group Differential diagnosis and evaluation in pediatric multiple sclerosis Neurology, April 17, 2007; 68(16_suppl_2): S13 - S22. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Tenembaum, T. Chitnis, J. Ness, J. S. Hahn, and for the International Pediatric MS Study Group Acute disseminated encephalomyelitis Neurology, April 17, 2007; 68(16_suppl_2): S23 - S36. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. M. Ness, D. Chabas, A. D. Sadovnick, D. Pohl, B. Banwell, B. Weinstock-Guttman, and for the International Pediatric MS Study Group Clinical features of children and adolescents with multiple sclerosis Neurology, April 17, 2007; 68(16_suppl_2): S37 - S45. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. S. MacAllister, J. R. Boyd, N. J. Holland, M. C. Milazzo, L. B. Krupp, and for the International Pediatric MS Study Group The psychosocial consequences of pediatric multiple sclerosis Neurology, April 17, 2007; 68(16_suppl_2): S66 - S69. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C-H Lin, J-S Jeng, S-T Hsieh, P-K Yip, and R-M Wu Acute disseminated encephalomyelitis: a follow-up study in Taiwan J. Neurol. Neurosurg. Psychiatry, February 1, 2007; 78(2): 162 - 167. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. A. Sobel Anti-Viral T-Cell Immunity + Anti-CNS Autoantibody = A Model for Human Acute Disseminated Encephalomyelitis or Multiple Sclerosis Relapse? Am. J. Pathol., February 1, 2007; 170(2): 436 - 438. [Full Text] [PDF]
|
|
|
|
 |
|
 S Macleod and R E Appleton Neurological disorders presenting mainly in adolescence Arch. Dis. Child., February 1, 2007; 92(2): 170 - 175. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. D. Singhi, M. Ray, S. Singhi, and Narendra Kumar Khandelwal Acute Disseminated Encephalomyelitis in North Indian Children: Clinical Profile and Follow-Up J Child Neurol, October 1, 2006; 21(10): 851 - 857. [Abstract] [PDF]
|
|
|
|
|
|
M Demirkiran, A ozeren, and H Bozdemir Letter to the Editor Multiple Sclerosis, August 1, 2006; 12(4): 519 - 520. [PDF]
|
|
|
|
|
|
E. Marchioni, S. Ravaglia, G. Piccolo, M. Furione, E. Zardini, D. Franciotta, E. Alfonsi, L. Minoli, A. Romani, A. Todeschini, et al. Postinfectious inflammatory disorders: Subgroups based on prospective follow-up Neurology, October 11, 2005; 65(7): 1057 - 1065. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. S. Khurana, J. J. Melvin, S. V. Kothare, I. Valencia, H. H. Hardison, S. Yum, E. N. Faerber, and A. Legido Acute Disseminated Encephalomyelitis in Children: Discordant Neurologic and Neuroimaging Abnormalities and Response to Plasmapheresis Pediatrics, August 1, 2005; 116(2): 431 - 436. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Natarajan and R. Weinstein Therapeutic Apheresis in Neurology Critical Care J Intensive Care Med, July 1, 2005; 20(4): 212 - 225. [Abstract] [PDF]
|
|
|
|
|
|
R C Dale and J A Branson Acute disseminated encephalomyelitis or multiple sclerosis: can the initial presentation help in establishing a correct diagnosis? Arch. Dis. Child., June 1, 2005; 90(6): 636 - 639. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. S. MacAllister, A. L. Belman, M. Milazzo, D. M. Weisbrot, C. Christodoulou, W. F. Scherl, T. E. Preston, C. Cianciulli, and L. B. Krupp Cognitive functioning in children and adolescents with multiple sclerosis Neurology, April 26, 2005; 64(8): 1422 - 1425. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. RamachandranNair, P. Manoj, M. Rafeequ, and A.S. Girija Childhood Acute Disseminated Encephalomyelitis Presenting as Foix-Chavany-Marie Syndrome J Child Neurol, February 1, 2005; 20(2): 163 - 165. [Abstract] [PDF]
|
|
|
|
 |
|
 Y. Mikaeloff, C. Adamsbaum, B. Husson, L. Vallee, G. Ponsot, C. Confavreux, M. Tardieu, S. Suissa, and the KIDMUS Study Group on Radiology MRI prognostic factors for relapse after acute CNS inflammatory demyelination in childhood Brain, September 1, 2004; 127(9): 1942 - 1947. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S Pradhan and V N Mishra A central demyelinating disease with atypical features Multiple Sclerosis, June 1, 2004; 10(3): 308 - 315. [Abstract] [PDF]
|
|
|
|
|
|
L Bennetto and N Scolding INFLAMMATORY/POST-INFECTIOUS ENCEPHALOMYELITIS J. Neurol. Neurosurg. Psychiatry, March 1, 2004; 75(90001): i22 - 28. [Full Text] [PDF]
|
|
|
|
|
|
E. A. Yeh, A. Collins, M. E. Cohen, P. K. Duffner, and H. Faden Detection of Coronavirus in the Central Nervous System of a Child With Acute Disseminated Encephalomyelitis Pediatrics, January 1, 2004; 113(1): e73 - 76. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M Stonehouse, G Gupte, E Wassmer, and W P Whitehouse Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians Arch. Dis. Child., February 1, 2003; 88(2): 122 - 124. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Mariotti, A.P. Batocchi, C. Colosimo, M. Lo Monaco, M. Caggiula, F. Colitto, P.A. Tonali, and F. Guzzetta Multiphasic demyelinating disease involving central and peripheral nervous system in a child Neurology, January 28, 2003; 60(2): 348 - 349. [Full Text] [PDF]
|
|
|
|
 |
|
 J. G. Millichap Outcome of Acute Disseminated Encephalomyelitis AAP Grand Rounds, January 1, 2003; 9(1): 7 - 8. [Full Text] [PDF]
|
|
|
|
 |
|
 R K Garg Acute disseminated encephalomyelitis Postgrad. Med. J., January 1, 2003; 79(927): 11 - 17. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Shahar, J. Andraus, D. Savitzki, G. Pilar, and N. Zelnik Outcome of Severe Encephalomyelitis in Children: Effect of High-Dose Methylprednisolone and Immunoglobulins J Child Neurol, November 1, 2002; 17(11): 810 - 814. [Abstract] [PDF]
|
|
|
|
|
|
S. Tenembaum, N. Chamoles, and N. Fejerman Acute disseminated encephalomyelitis: A long-term follow-up study of 84 pediatric patients Neurology, October 22, 2002; 59(8): 1224 - 1231. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. Watemberg, O. Vardi, D. Lev, C. Vinkler, and T. Lerman-Sagie Congenital Cytomegalovirus Infection Presenting as an Apparent Neurodegenerative Disorder Clinical Pediatrics, September 1, 2002; 41(7): 519 - 522. [PDF]
|
|
|
|
|
|
S. N. K. Murthy, H. S. Faden, M. E. Cohen, and R. Bakshi Acute Disseminated Encephalomyelitis in Children Pediatrics, August 1, 2002; 110(2): e21 - 21. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Kubota, T. Kanbayashi, Y. Tanabe, and Y. Kohno A Case of Acute Disseminated Encephalomyelitis Presenting Hypersomnia With Decreased Hypocretin Level in Cerebrospinal Fluid J Child Neurol, July 1, 2002; 17(7): 537 - 539. [Abstract] [PDF]
|
|
|
|
|
|
A Ghezzi, C Pozzilli, M Liguori, M G Marrosu, N Milani, C Milanese, I Simone, and M Zaffaroni Prospective study of multiple sclerosis with early onset Multiple Sclerosis, April 1, 2002; 8(2): 115 - 118. [Abstract] [PDF]
|
|
|
|
|
|
C. H. Wang and K. Walsh Multiple Ring-Enhancing Lesions in a Child With Relapsing Multiple Sclerosis J Child Neurol, January 1, 2002; 17(1): 69 - 72. [Abstract] [PDF]
|
|
|
|
|
|
S. Sacconi, L. Salviati, and E. Merelli Acute Disseminated Encephalomyelitis Associated With Hepatitis C Virus Infection Arch Neurol, October 1, 2001; 58(10): 1679 - 1681. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H.-P. Hartung ADEM: Distinct disease or part of the MS spectrum? Neurology, May 22, 2001; 56(10): 1257 - 1260. [Full Text] [PDF]
|
|
|
|
|
|
J.L. Hynson, A.J. Kornberg, L.T. Coleman, L. Shield, A.S. Harvey, and M.J. Kean Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children Neurology, May 22, 2001; 56(10): 1308 - 1312. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Acute Disseminated Encephalomyelitis vs. Multiple Sclerosis in Children Journal Watch Neurology, April 12, 2001; 2001(412): 7 - 7. [Full Text]
|
|