- Last edited on February 4, 2022
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geri:dementia:creutzfeldt-jakob-disease-cjd [on June 2, 2019] |
geri:dementia:creutzfeldt-jakob-disease-cjd [on April 25, 2020] |
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====== Creutzfeldt-Jakob Disease (CJD) ====== | ====== Creutzfeldt-Jakob Disease (CJD) ====== | ||
+ | {{INLINETOC}} | ||
===== Primer ===== | ===== Primer ===== | ||
**Creutzfeldt-Jakob Disease** (CJD) is a rare, fatal, progressive neurodegenerative disorder. It affects about 1 in every 1 million people per year worldwide. CJD has a rapid course and usually appears in late life. There is a very short survival time, death usually occurs within one year of symptom onset. | **Creutzfeldt-Jakob Disease** (CJD) is a rare, fatal, progressive neurodegenerative disorder. It affects about 1 in every 1 million people per year worldwide. CJD has a rapid course and usually appears in late life. There is a very short survival time, death usually occurs within one year of symptom onset. | ||
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===== Investigations ===== | ===== Investigations ===== | ||
==== Neuroimaging ==== | ==== Neuroimaging ==== | ||
- | Prion disease is diagnosed with least one of the characteristic biomarker features: cortical ribboning or lesions on [[neurology:investigations:neuroimaging:mri|magnetic resonance imaging]] with DWI (diffusion-weighted imaging)[([[https://www.ncbi.nlm.nih.gov/pubmed/26238299|Abdulmassih, R., & Min, Z. (2016). An ominous radiographic feature: cortical ribbon sign. Internal and emergency medicine, 11(2), 281-283.]])] or FLAIR (fluid-attenuated inversion recovery). | + | Prion disease is diagnosed with least one of the characteristic biomarker features: cortical ribboning or lesions on [[neurology:mri|magnetic resonance imaging]] with DWI (diffusion-weighted imaging)[([[https://www.ncbi.nlm.nih.gov/pubmed/26238299|Abdulmassih, R., & Min, Z. (2016). An ominous radiographic feature: cortical ribbon sign. Internal and emergency medicine, 11(2), 281-283.]])] or FLAIR (fluid-attenuated inversion recovery). |
==== Cerebrospinal Fluid ==== | ==== Cerebrospinal Fluid ==== | ||
- | Tau or 14-3-3 protein in [[neurology:investigations:lp|cerebrospinal fluid]] can be used to discriminate CJD from other neurodegenerative diseases.[([[https://www.ncbi.nlm.nih.gov/pubmed/16633900|Satoh, K., Shirabe, S., Eguchi, H., Tsujino, A., Eguchi, K., Satoh, A., ... & Matsuo, H. (2006). 14-3-3 protein, total tau and phosphorylated tau in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease and neurodegenerative disease in Japan. Cellular and molecular neurobiology, 26(1), 45-52.]])] | + | Tau or 14-3-3 protein in [[neurology:lumbar-puncture-lp|cerebrospinal fluid]] can be used to discriminate CJD from other neurodegenerative diseases.[([[https://www.ncbi.nlm.nih.gov/pubmed/16633900|Satoh, K., Shirabe, S., Eguchi, H., Tsujino, A., Eguchi, K., Satoh, A., ... & Matsuo, H. (2006). 14-3-3 protein, total tau and phosphorylated tau in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease and neurodegenerative disease in Japan. Cellular and molecular neurobiology, 26(1), 45-52.]])] |
==== EEG === | ==== EEG === | ||
- | Characteristic triphasic waves can be seen on [[neurology:investigations:eeg|electroencephalogram]]. | + | Characteristic triphasic waves can be seen on [[neurology:eeg|electroencephalogram]]. |