Neuropsychological features of adult form of Alexander disease

Published:April 23, 2019DOI:https://doi.org/10.1016/j.jns.2019.04.030

      Highlights

      • Varying cognitive abnormalities are common in adult-onset Alexander disease.
      • Routine cognitive screening tests are typically normal.
      • Extensive neuropsychological assessment needed to detect the cognitive impairment.
      • Patients can have reasoning, attention/executive, and memory retrieval deficits.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic and Personal

      Subscribe:

      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Yoshida T.
        • Sasaki M.
        • Yoshida M.
        • et al.
        Nationwide survey of Alexander disease in Japan and proposed new guidelines for diagnosis.
        J. Neurol. 2011; 258: 1998-2008https://doi.org/10.1007/s00415-011-6056-3
        • Li R.
        • Johnson A.B.
        • Salomons G.
        • et al.
        Glial fibrillary acidic protein mutations in infantile, juvenile, and adult forms of Alexander disease.
        Ann. Neurol. 2005; 57: 310-326https://doi.org/10.1002/ana.20406
        • Pareyson D.
        • Fancellu R.
        • Mariotti C.
        • et al.
        Adult-onset Alexander disease: a series of eleven unrelated cases with review of the literature.
        Brain. 2008; 131: 2321-2331https://doi.org/10.1093/brain/awn178
        • Garcia-Reitboeck P.
        • MacKinnon A.D.
        • McEntagart M.
        • et al.
        Prominent cognitive decline and behavioural disturbance in late-onset Alexander disease.
        J. Neurol. Sci. 2015; 357: 319-321https://doi.org/10.1016/j.jns.2015.07.03
        • Filley C.M.
        Cognitive dysfunction and dementia.
        in: The Behavioral Neurology of White Matter. Oxford University Press, Oxford2001: 201-218
        • Ohtani T.
        • Nestor P.G.
        • Bouix S.
        • et al.
        Exploring the neural substrates of attentional control and human intelligence: diffusion tensor imaging of prefrontal white matter tractography in healthy cognition.
        Neuroscience. 2017; 341: 52-60https://doi.org/10.1016/j.neuroscience.2016.11.002
        • O'Sullivan M.
        • Jones D.K.
        • Summers P.E.
        • Morris R.G.
        • Williams S.C.
        • Markus H.S.
        Evidence for cortical "disconnection" as a mechanism of age-related cognitive decline.
        Neurology. 2001; 57: 632-638
        • Mesulam M.M.
        Attentional networks, confusional states, and neglect syndromes.
        in: Mesulam M.M. Principles of behavioral and cognitive neurology. Oxford University Press, New York2000: 174-256
        • Swardfager W.
        • Cogo-Moreira H.
        • Masellis M.
        • et al.
        The effect of white matter hyperintensities on verbal memory: mediation by temporal lobe atrophy.
        Neurology. 2018; 90: e673-e682https://doi.org/10.1212/WNL.0000000000004983
        • Markowitsch H.J.
        Which brain regions are critically involved in the retrieval of old episodic memory?.
        Brain Res. Rev. 1995; 21: 117-127https://doi.org/10.1016/0165-0173(95)00007-0