Literature DB >> 32219420

An unusual presentation of sporadic Creutzfeldt-Jakob disease.

Hannah Podger1, Anil Ipe1.   

Abstract

Sporadic Creutzfeld-Jakob disease (CJD) is a rare neurodegenerative disorder. It is uniformly fatal. Clinical signs include myoclonus, visual disturbances, cerebellar ataxia, akinetic mutism and pyramidal/extrapyramidal signs in addition to a rapidly progressive dementia. Premortem diagnosis is challenging due to the rarity of the condition and the subsequent low index of suspicion held for it. On literature review, isolated language impairment as the first neurological symptom occurs in only about 1% of patients with sporadic CJD (El Tawil et al. (2017, Acta Neurol Scand, 135: 316-23)). We present this patient's case, marked for the unusual presentation and the rapidity of decline, to emphasise the need for awareness of CJD as an important differential diagnosis on stroke units. In our case, magnetic resonance imaging findings drove the suspicion of sporadic CJD as the diagnosis. This seems to correlate with other case reports recognising CJD presenting with progressive aphasic disorders (Terrin et al. (2017, Neurol Sci, 38: 1535-7); Mandell et al. (1989, Neurology, 39: 55-8); Martory et al. (2012, Eur Neurol, 67: 360-2)).
© The Author(s) 2020. Published by Oxford University Press on behalf of the British Geriatrics Society. All rights reserved. For permissions, please email: journals.permissions@oup.com.

Entities:  

Keywords:  Creutzfeld-Jakob disease (CJD); dysphasia; neurodegenerative; older people

Mesh:

Year:  2020        PMID: 32219420     DOI: 10.1093/ageing/afaa013

Source DB:  PubMed          Journal:  Age Ageing        ISSN: 0002-0729            Impact factor:   10.668


  1 in total

Review 1.  Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships.

Authors:  Yacov Balash; Amos D Korczyn; Nadejda Khmelev; Anda Eilam; Meital Adi; Ronit Gilad
Journal:  Front Neurol       Date:  2021-02-25       Impact factor: 4.003

  1 in total

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