title: Transient magnetic resonance diffusion abnormalities in West syndrome: the radiological expression of non-convulsive status epilepticus?
abstract: The purpose of this study was to report patients with pharmacoresistant West syndrome of unknown cause whose magnetic resonance imaging (MRI) with diffusion weighted imaging (DWI) showed a transient decrease of diffusion in subcortical structures. Of 20 patients investigated over a 2-year period, three males and three females constitute the present series. They had daily clusters of infantile spasms with hypsarrhythmia for 4 to 24 months before the first investigation. Four were severely hypotonic. All aetiological studies involving intermediary metabolism, peroxysomes, mitochondria, and neurotransmitters in cerebrospinal fluid were negative. Patients underwent DWI when first examined at the mean age of 13 months, and on follow-up examination 6 to 18 months later. Diffusion was decreased in the pallidi and posterior brainstem. It was also decreased for five patients in thalami and for three in dentate nuclei. Repeat MRI, performed when spasms were still present but hypsarrhythmia had ceased, did not show the same abnormalities. Because of recruitment bias, this series probably overestimates the true incidence of such DWI abnormalities. The eventuality of toxic lesions, including some inborn error of metabolism or drug toxicity, is considered unlikely although it could not be excluded. The contribution of the epileptic encephalopathy itself appears the most likely course.
authors.affiliation: Department of Neuropaediatrics, APHP, Necker-Enfants Malades Hospital, Paris, France. isabelle.desguerre@nck.aphp.fr
authors.forename: Isabelle
authors.initials: I
authors.lastname: Desguerre
authors.forename: Ixtasu
authors.initials: I
authors.lastname: Marti
authors.forename: Vassili
authors.initials: V
authors.lastname: Valayannopoulos
authors.forename: Nadia
authors.initials: N
authors.lastname: Bahi-Buisson
authors.forename: Olivier
authors.initials: O
authors.lastname: Dulac
authors.forename: Perrine
authors.initials: P
authors.lastname: Plouin
authors.forename: Pascale
authors.initials: P
authors.lastname: Delonlay
authors.forename: Lucie
authors.initials: L
authors.lastname: Hertz-Pannier
authors.forename: Nathalie
authors.initials: N
authors.lastname: Boddaert
mesh_terms: D001479:Basal Ganglia / Q000000981:diagnostic imaging; D001921:Brain / Q000000981:diagnostic imaging* / Q000473:pathology; D002529:Cerebellar Nuclei / Q000000981:diagnostic imaging; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008636:Mesencephalon / Q000000981:diagnostic imaging; D011859:Radiography; D013036:Spasms, Infantile / Q000000981:diagnostic imaging* / Q000473:pathology*; D013226:Status Epilepticus / Q000000981:diagnostic imaging* / Q000473:pathology*; D013788:Thalamus / Q000000981:diagnostic imaging