An unusual presentation of sporadic Creutzfeldt-Jakob disease.

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)).

journal_name

Age Ageing

journal_title

Age and ageing

authors

Podger H,Ipe A

doi

10.1093/ageing/afaa013

subject

Has Abstract

pub_date

2020-04-27 00:00:00

pages

490-492

issue

3

eissn

0002-0729

issn

1468-2834

pii

5812707

journal_volume

49

pub_type

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