Acute fulminant demyelinating disease: a descriptive study of 60 patients.
BACKGROUND
Acute demyelinating encephalomyelitis (ADEM) is characterized by a severe inflammatory attack, frequently secondary to infectious events or vaccinations. To date, no clear criteria exist for ADEM, and the risk of subsequent evolution to multiple sclerosis (MS) remains unknown.


OBJECTIVE
To evaluate the risk of evolution to MS after a first episode of ADEM.


METHODS
Observational, retrospective case study.


METHODS
Thirteen French MS centers. Patients We retrospectively studied 60 patients with ADEM who were older than 15 years with no history suggestive of an inflammatory event who presented to MS centers from January 1, 1995, through December 31, 2005. We excluded 6 patients with multiphasic ADEM because this is a rare condition and somewhat difficult to classify. After a mean follow-up of 3.1 years (range, 1-10 years), the remaining 54 patients were then classified into 2 groups: monophasic ADEM (ADEM group) (n = 35) and clinically definite MS (MS group) (n = 19).


METHODS
Clinical, laboratory, magnetic resonance imaging, and follow-up data were evaluated for each group.


RESULTS
Patients in the ADEM group more frequently had atypical symptoms of MS (26 of 35 [74%]) than patients with MS (8 of 19 [42%]) (P = .02). Oligoclonal bands were more frequently observed in the MS group (16 of 19 [84%]) than in the ADEM group (7 of 35 [20%]) (P <.001). Patients in the ADEM group more frequently had gray matter involvement (21 of 35 [60%]) than those in the MS group (2 of 19 [11%]) (P <.001). On the basis of these results, we consider that the presence of any 2 of the following 3 criteria could be used to differentiate patients with ADEM from those with MS in our cohort: atypical clinical symptoms for MS, absence of oligoclonal bands, and gray matter involvement. On this basis, 29 of the 35 patients in the ADEM group (83%) and 18 of the 19 patients in the MS group (95%) were classified in the appropriate category.


CONCLUSIONS
Our study found some differences concerning the risk of evolution to clinically definite MS after a first demyelinating episode suggestive of ADEM. These findings led us to propose criteria that should now be tested in a larger, prospective cohort study.
de Seze
Jérôme
J
Departement de Neurologie, Hôpital Civil, 1 Place de l'Hôpital, BP 426, 67091 Strasbourg CEDEX, France. jerome.de.seze@chru-strasbourg.fr
Debouverie
Marc
M
Zephir
Hélène
H
Lebrun
Christine
C
Blanc
Frédéric
F
Bourg
Véronique
V
Wiertlewski
Sandrine
S
Pittion
Sophie
S
Laplaud
David
D
Le Page
Emmanuelle
E
Deschamps
Romain
R
Cabre
Philippe
P
Pelletier
Jean
J
Malikova
Irina
I
Clavelou
Pierre
P
Jaillon
Valérie
V
Defer
Gilles
G
Labauge
Pierre
P
Gout
Olivier
O
Boulay
Clotilde
C
Edan
Gilles
G
Vermersch
Patrick
P
D000328:Adult; D001921:Brain / Q000473:pathology; D015331:Cohort Studies; D016208:Databases, Factual; D003711:Demyelinating Diseases / Q000134:cerebrospinal fluid / Q000145:classification / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000473:pathology; D011379:Prognosis; D012189:Retrospective Studies; D013116:Spinal Cord / Q000473:pathology