First symptom in sporadic Creutzfeldt–Jakob disease

Rabinovici, Gil D.; Wang, P. N.; Xiong, Chengjie; Cook, L.; Pravdin, M.; Davis, J.; DeArmond, Stephen J.; Barbaro, Nicholas M.; Martindale, Jennifer L.; Miller, Bruce L.; Geschwind, Michael D.

doi:10.1212/01.wnl.0000196440.00297.67

Cited by 130 publications

(110 citation statements)

References 4 publications

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“…One patient with sporadic CJD had pure aphasia for 12 months, 11 and 2 other patients had aphasia for a few months prior to onset of other symptoms. 12,13 In a prior study, 14 we found that 6% of patients with sporadic CJD had a language problem as their first symptom. A few cases of aphasia in genetic prion disease are reported in the Japanese literature, 15 but none met Mesulam's criteria.…”

Section: Commentmentioning

confidence: 89%

Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

Johnson¹,

Dunkelberger²,

Henry³

et al. 2012

JAMA Neurol

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Section: Commentmentioning

confidence: 89%

Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

Johnson¹,

Dunkelberger²,

Henry³

et al. 2012

JAMA Neurol

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“…These criteria also do not include features that are often early signs of the illness, such as behavioral changes or aphasia. [12] We identified the first symptom in 114 sCJD subjects referred to our center and found the most common were cognitive (39% of patients), followed by cerebellar (21%), behavioral (20%), constitutional (20%), sensory (11%), motor (9%) and visual (7%). Three of these categories -behavioral, constitutional and sensory symptoms (e.g., headache, malaise, vertigo, etc….)…”

Section: Degenerative Dementias Prion Diseasesmentioning

confidence: 96%

Rapidly Progressive Dementia

2007

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Rapidly progressive dementias (RPDs) are neurological conditions that develop subacutely over weeks to months, or rarely acutely over days. In contrast to most dementing conditions that take years to progress to death, RPD can be quickly fatal. It is critical to evaluate the RPD patient without delay, usually in a hospital setting, as they may have a treatable condition. In this review, we discuss a differential diagnostic approach to RPD, emphasizing neurodegenerative, toxic/metabolic, infectious, autoimmune, neoplastic, and other conditions to consider.

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“…The initial presentation of sCJD is quite variable, and the two most recognized cardinal features of sCJD are rapidly progressing dementia and myoclonus (8,9). A previous cohort study of 114 patients with sCJD revealed that 20% of patients may present with 'behavioral symptoms', typically agitation, irritability and depression (10). However, reports of early psychiatric manifestations as an initial or sole symptom of sCJD are relatively rare.…”

Section: Discussionmentioning

confidence: 99%

Clinical and MRI evaluation of anxiety as the first symptom of sporadic Creutzfeldt-Jakob disease: A case report

Liu

Zhong

et al. 2016

Experimental and Therapeutic Medicine

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Abstract. Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, transmissible prion disease of the brain, characterized by prominent neurological symptoms and progressive dementia. Early psychiatric manifestations as an initial or sole symptom of sCJD are relatively rare. The current report describes an elderly female patient with sCJD demonstrating anxiety as an initial symptom. The present patient was initially diagnosed with adjustment disorder and anxiety; however, the rapid deterioration of the patient's cognitive and neurological functioning led to a diagnosis of sCJD. Diffusion-weighted magnetic resonance images of the brain supported a diagnosis of sCJD, and it was posited that the abnormalities of gray matter that were observed within the bilateral cingulate cortex may be the pathophysiological basis of the anxiety associated with this case of sCJD. The patient eventually succumbed to inhalational bronchopneumonia 5 months after anxiety onset. The present case report emphasizes the importance of secondary causes of anxiety symptoms in elderly patients, and indicates that brain DWI is a non-invasive and useful examination in the early diagnosis of sCJD.

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First symptom in sporadic Creutzfeldt–Jakob disease

Cited by 130 publications

References 4 publications

Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

Rapidly Progressive Dementia

Clinical and MRI evaluation of anxiety as the first symptom of sporadic Creutzfeldt-Jakob disease: A case report

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