AI Article Synopsis
- A 66-year-old man exhibited symptoms of sporadic Creutzfeldt-Jakob disease (sCJD) alongside positive IgLON5 antibodies, demonstrating unusual clinical and imaging characteristics.
- MRI findings, EEG abnormalities, and the presence of 14-3-3 protein in CSF supported the diagnosis, while matching IgLON5 antibodies were found in both serum and CSF.
- The potential link between IgLON5 autoimmunity and CJD is noteworthy, suggesting a possible interaction that could exacerbate neurodegeneration, although further investigation is needed to clarify this relationship.
Article Abstract
Objective: We present the case of a patient with clinical and imaging features of sporadic Creutzfeldt-Jakob disease (sCJD) and positive IgLON5 antibodies (Abs) in the serum and CSF.
Case Report: A 66-year-old Chinese man presented to the hospital with a stroke-like episode, followed by rapidly progressive cognitive decline, mutism, and parkinsonism. The MRI results showed a cortical ribboning sign in diffusion-weighted MRI, periodic triphasic waves with a slow background in EEG, and positive protein 14-3-3 in CSF. There were matching IgLON5 Abs in the serum and CSF. A literature review showed positive autoimmune encephalitis Abs or autoimmune inflammatory disease between 0.5 and 8.6% among patients with clinical suspicion of CJD, most commonly anti-voltage-gated potassium channel (VGKC) complex and anti-N-methyl-D-aspartate receptor (NMDAR) Abs; however, IgLON5 autoimmunity in CJD has been rarely reported. This is an intriguing association as both conditions have been associated with brain deposits of phosphorylated tau protein.
Conclusion: IgLON5 Abs may be observed in patients with a diagnosis of CJD; it is unknown whether a synergistic effect of IgLON5 Abs with CJD exists, increasing neurodegenerative changes.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10989518 | PMC |
| http://dx.doi.org/10.3389/fneur.2024.1367361 | DOI Listing |
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Article Synopsis
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- MRI findings, EEG abnormalities, and the presence of 14-3-3 protein in CSF supported the diagnosis, while matching IgLON5 antibodies were found in both serum and CSF.
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