Sporadic Creutzfeldt–Jakob Disease

Author:

Zhelyazkova S.12,Nachev S.3,Kalev O.4,Tournev I.125

Affiliation:

1. Clinic of Nervous Diseases, University Hospital “Alexandrovska” , Medical University – Sofia , Bulgaria

2. Department of Neurology , Medical University – Sofia , Bulgaria

3. Department of Neurosurgery , Laboratory of Neuropathology , Medical University Hospital “Sv. Ivan Rilski” – Sofia , Bulgaria

4. Institute of Pathology, Neuromed Campus , Kepler University Hospital, Johannes Kepler University – Linz , Austria

5. Department of Cognitive Science and Psychology , New Bulgarian University – Sofia , Bulgaria

Abstract

Abstract Sporadic Creutzfeldt–Jakob disease is a rare and fatal human prion disease characterized by a rapidly progressive dementia, myoclonus, cerebellar, pyramidal, extrapyramidal, visual, and psychiatric symptoms. These findings are all non-specific and making diagnosis is often difficult at the symptoms onset, especially in case of atypical clinical and radiological presentation. This case report describes a woman in her 60s, who presented with rapid cognitive decline, confusion, ataxia and electroencephalographic changes compatible with nonconvulsive status epilepticus. Her symptoms progressively worsened and she died 8 weeks after the onset. The two cerebrospinal fluid analyses were normal and no 14-3-3 protein was detected. The brain MRls revealed areas of cortical restricted diffusion involving the right frontal and parietal lobe. The electroencephalographic findings of continuous periodic generalized bi-triphasic complexes, typical for sporadic Creutzfeldt–Jakob disease, were detected 1 month after the onset. Sporadic Creutzfeldt–Jakob disease was neuropathologically confirmed. Although sporadic Creutzfeldt–Jakob disease is a rare neurodegenerative disease, it should be considered in the differential diagnosis of all cases with unexplained and rapid cognitive decline and confusion, along with ataxia, pyramidal/ extrapyramidal signs, myoclonus and dysphagia.

Publisher

Walter de Gruyter GmbH

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