MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob diseaseReportar como inadecuado

MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease - Descarga este documento en PDF. Documentación en PDF para descargar gratis. Disponible también para leer online.

Journal of Neurology

, Volume 256, Issue 3, pp 355–363

First Online: 23 January 2009Received: 09 September 2007Revised: 29 April 2008Accepted: 04 June 2008


ObjectiveIatrogenic Creutzfeldt-Jakob disease iCJD is mainly associated with dura mater DM grafts and administration of human growth hormones hGH. Data on disease course in DM-CJD are limited. We describe the clinical and diagnostic findings in this patient group with special emphasis on MRI signal alterations.

MethodsTen DM-CJD patients were studied for their clinical symptoms and diagnostic findings. The MRIs were evaluated for signal increase of the cortical and subcortical structures.

ResultsDM-CJD patients had a median incubation time of 18 years and median disease duration of 7 months. The majority of patients were MM homozygous at codon 129 of the prion protein gene PRNP and presented with gait ataxia and psychiatric symptoms. No correlation between the graft site and the initial disease course was found. The MRI showed cortical and basal ganglia signal increase each in eight out of ten patients and thalamic hyperintensity in five out of ten cases. Of interest, patients with thalamic signal increase were homozygous for methionine.

ConclusionThe MRI findings in DM-CJD largely resemble those seen in sporadic CJD, as the cortex and basal ganglia are mainly affected.

Key wordsCJD MRI dementia cerebellar disorder CNS infection  Download to read the full article text



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