AI Article Synopsis
- Creutzfeldt-Jakob disease (CJD) is a severe brain disorder characterized by rapid dementia and specific abnormalities seen in MRI scans, but distinguishing it from other conditions requires thorough testing and history.
- A 41-year-old male experienced fast-developing symptoms like forgetfulness and inability to walk or speak, ultimately leading to a diagnosis of probable sporadic CJD after MRI and EEG evaluations matched expected patterns.
- The case emphasizes the importance of considering CJD in patients with swift cognitive decline, as misdiagnosis can have serious implications, and the patient's condition worsened significantly despite supportive care.
Article Abstract
Background: Creutzfeldt-Jakob disease (CJD), is a deadly degenerative condition of the central nervous system marked by rapidly progressive dementia. Magnetic resonance imaging (MRI) abnormalities in the cerebral cortex, basal ganglia, thalamus, and cerebellum could indicate severe acute diseases caused by a variety of factors. Although their MRI patterns may resemble those of CJD, clinical history, additional MRI findings, and laboratory testing are all necessary to provide a reliable difference. Here, we report a misdiagnosed case of probable VV1 subtype of sporadic CJD (sCJD) in which follow-up MRI supported the diagnosis.
Case Presentation: A 41-year-old male patient attended the Neuropsychiatry Department with rapidly progressive dementia, akinetic mutism, and difficulty walking and speaking. His problem began with forgetfulness, disorganized behavior, and disorganized speech 7 months earlier which progressed rapidly and was accompanied by aphasia, apraxia, agnosia, and akinetic mutism in the last 2 months. On neurologic examination, hypertonia, hyperreflexia, frontal ataxia, bradykinesia, gait apraxia, and aphasia were noted. Based on clinical features and rapid symptoms progression the likely diagnosis of CJD was suspected. MRI and electroencephalography (EEG) were advised. MRI revealed features of diffuse cortical injury of both cerebral hemispheres also involving bilateral corpus striatum with evidence of cerebral volume loss. EEG showed lateralized periodic theta slow waves on the right side. According to the CDC's diagnostic criteria for CJD, the diagnosis of probable sCJD was established. Supportive care and symptomatic treatment are provided for the patient. After a 1-month follow up the patient's condition deteriorated significantly. The time-lapse from the first reported symptom to death was about 13 months.
Conclusion: The need of addressing CJD in patients presenting with rapidly progressive dementia is highlighted in this case report. In the early stages of the disease, interpretation of MRI results might cause diagnostic difficulties; therefore, follow-up MRI is critical in obtaining the correct diagnosis.
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|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10354985 | PMC |
| http://dx.doi.org/10.1186/s12883-023-03318-z | DOI Listing |
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