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Case Reports
. 2016 Sep 28;4(11):1045-1048.
doi: 10.1002/ccr3.666. eCollection 2016 Nov.

Recurrent transient global amnesia as presenting symptoms of CADASIL

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Case Reports

Recurrent transient global amnesia as presenting symptoms of CADASIL

Luca Pradotto et al. Clin Case Rep. .

Abstract

Despite transient global amnesia is considered unusual in Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and causal relation is still unclear, this report suggests to consider CADASIL in those patients with recurrent transient global amnesia, especially when MRI shows multifocal hyperintensities affecting the cerebral white matter or when it is followed by cognitive decline.

Keywords: CADASIL; NOTCH3 gene mutation; cognitive impairment; dementia; leukoaraiosis; microbleeds; transient global amnesia.

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Figures

Figure 1
Figure 1
Brain MRI findings of the proband. The FLAIR images (A–K) demonstrated the multifocal damage of the white matter involving the brainstem (A), the temporal poles (A, E, I), the left external capsule (F), the periventricular regions (E, J, K), the subcortical regions apparently sparing the U‐fibers (B–K). The gradient‐echo image (L) showed one microbleed in the right basal ganglia region.
Figure 2
Figure 2
NOTCH3 gene analysis. The forward sequence of the exon 24 disclosed the heterozygous G→T substitution at the second position of the codon 1298 (TGC→TTC). The affected nucleotides were indicated by the N letter.

References

    1. Dichgans, M. , Mayer M., Uttner D. P., Bruning R., Muller‐Hocker J., Rungger G., et al. 1998. The phenotypic spectrum of CADASIL: clinical findings in 102 cases. Ann. Neurol. 44:731–739. - PubMed
    1. Pradotto, L. , Orsi L., Daniele D., Caroppo P., Lauro D., Milesi A., et al. 2012. A new Notch3 mutation presenting as primary intracerebral haemorrhage. J. Neurol. Sci. 315:143–145. - PubMed
    1. Hereda, P. , and Friedland R. P.. 1997. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: study of two American families with predominant dementia. J. Neurol. Sci. 146:27–33. - PubMed
    1. Pradotto, L. , Azan G., Doriguzzi C., Valentini C., and Mauro A.. 2008. Sporadic vascular dementia as clinical presentation of a new missense mutation within exon 7 of NOTCH3 gene. J. Neurol. Sci. 271:207–210. - PubMed
    1. Chabriat, H. , and Bousser M. G.. 2007. Neuropsychiatric manifestations in CADASIL. Dialogues Clin. Neurosci. 9:199–208. - PMC - PubMed

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