Creutzfeldt–Jakob Disease Presenting With Dizziness and Gaze-Evoked Nystagmus

Choi, Yong‐Jin; Kang, Kyung Tae; Lee, Sae-Young; Kang, Seung‐Ho; Lee, Seung-Han; Kim, Byeong C.

doi:10.1097/md.0000000000002766

Cited by 7 publications

(4 citation statements)

References 9 publications

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“…Dizziness presentation is not frequent in sCJD, which was reported to be present as an initial symptom in 2.6% of CJD patients [18]. Studies also reported that the initial symptoms of CJD could mimic benign peripheral vestibulopathy, leading to a misdiagnosis or a delayed diagnosis [23][24][25][26]. The relatively high percentage of dizziness as an initial manifestation in Chinese sCJD patients may due to the lack of a thorough knowledge of early deficits of sCJD, and it is important to raise awareness of the clinical symptoms and presentations of this rare disease.…”

Section: Discussionmentioning

confidence: 99%

Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease

Yang

Kuang

Wang

et al. 2020

Prion

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Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, incurable, and fatal neurodegenerative disorder. The objective of this study was to describe the clinical features and survival time of Chinese sCJD patients, and to explore the associations between clinical data and survival. In this study, we analysed the clinical data of 21 sCJD patients in a tertiary care hospital and used all Chinese case material available from 152 patients with sCJD in literatures between 2008 and 2018. The mean age of onset of all 173 deceased patients was 61.44 year-olds (y), with the highest incidence in the population of 60 to 69 y. The most common manifestation at disease onset was progressive dementia. With the progression of the disease, the four main clinical symptoms and signs were developed, including myoclonus, visual or cerebella disturbance, pyramidal or extrapyramidal dysfunction, and akinetic mutism. Extrapyramidal symptoms were more frequently observed. The mean survival time was 7.34 months, and 82.10% of cases died within 1 year after disease onset. The follow-up showed that the survival time was longer and the myoclonus sign was more frequently presented in younger-onset sCJD patients. Patients with abnormalities only in cortical regions had a higher frequency of pyramidal dysfunction than patients having lesions in both cortex and basal ganglia. The findings of this study might provide some insight into the clinical characteristics of sCJD patients in China, but further studies could examine the presences of clinical features and survival time in patients with early age of onset in a prospective manner.

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Section: Discussionmentioning

confidence: 99%

Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease

Yang

Kuang

Wang

et al. 2020

Prion

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“…There are descriptions of early presentations of sCJDVV2(sCJD with homozygotic kodon 129 Valin/Valin) with rapidly progressive ataxia and oculomotor disturbances without prominent cognitive impairment [7]. There are also other descriptions of unusual clinical manifestations [8,9] of sCJD, but none of them reports such a clear presence of a triad of signs of ataxia, gaze paresis and dementia with confabulations seen in WKs [10]. The patient did not fulfil the current criteria for probable sCJD till the late stage of the disease when akinetic mutism was observed.…”

Section: Discussionmentioning

confidence: 99%

Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease

Bielewicz

Szczepańska-Szerej

Ogórek

et al. 2018

Prion

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We reported the case of a patient with Wernicke-Korsakoff syndrome (WKs) as an early clinical manifestation of sporadic Creutzfeld-Jakob disease (sCJD). The 66-year-old female complained of dizziness and imbalance which mostly occurred while walking. A neurological examination revealed a triad of symptoms characteristic for WKs such as gaze paresis, ataxia of limbs and trunk as well as memory disturbances with confabulations. The disturbances increased during the course of the disease, which led to the death of the patient four months after the appearance of the signs. The patient was finally diagnosed with sCJD disease. The most useful ancillary examination results supporting sCJD diagnosis were brain diffusion DWI MRI (diffusion weighted magnetic resonance imaging) and the presence of 14-3-3 protein in CSF (cerebrospinal fluid). Since that manifestation of sCJD is very unique other causes should be taken into consideration while making a final diagnosis.

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“…Ocular motor cerebellar signs and ataxia of gait/stance may precede classical features in sporadic Creutzfeldt-Jakob disease [57] and may reflect prominent features in immune-mediated anti-DPPX encephalitis [58]. Hereditary cerebellar neurodegeneration may initially present as (positional) DBN, as described for spinocerebellar ataxia type 6 [59] and X-linked adrenoleukodystrophy [60].…”

Section: Nystagmus In the Differential Diagnosismentioning

confidence: 98%

Nystagmus

Tarnutzer

Straumann

2018

Current Opinion in Neurology

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In clinical practice, a structured description of nystagmus including its three-dimensional beating direction, trigger factors and duration is of major importance. The differential diagnosis of downbeat nystagmus is broad and includes acute intoxications, neurodegenerative disorders and cerebrovascular causes amongst others. In patients with positional nystagmus, the distinction between frequent benign peripheral and rare but dangerous central causes is imperative.

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Creutzfeldt–Jakob Disease Presenting With Dizziness and Gaze-Evoked Nystagmus

Abstract: Supplemental Digital Content is available in the text

Cited by 7 publications

References 9 publications

Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease

Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease

Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease

Nystagmus

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