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Case Report: Three Case Reports of Rapidly Progressive Dementias and Narrative Review
Carlos Andrés Clavijo1,2, Ana María Portilla Buenaventura1, Galo Santiago Benavides Albornoz1
1Department of Neurology, Neurology Laboratory, Institute of Neuroscience, Pontificia Javeriana Cali, Cali, Colombia.
Abstract:
Rapidly progressive dementia (RPD) is a heterogeneous group of diseases characterized by cognitive impairment and other neurological disorders developed in a short span of fewer than 2 years. Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nevertheless, they significantly compromise many patients' quality of life. Here, we drive 3 clinical cases that evolve as RPD with different etiologies.
Case 1:
70-year-old woman presented to the emergency with neuropsychiatric syndrome for 18 days. The researchers identified inflammatory cerebrospinal fluid (CSF), protein 14-3-3-positive T-tau protein, MRI: T2 and FLAIR hyperintensities in bilateral caudate nuclei with diffusion restriction, EEG shows a generalized periodic pattern with triphasic wave morphology.
Case 2:
29-year-old man with cognitive impairment and faciobrachial dystonia seizure. The diagnosis was confirmed by achieving elevated antibodies against voltage-gated potassium channels.
Case 3:
A 49-year-old woman with encephalopathy and myoclonic seizures; EEG and MRI showed subtle changes. The patient also had a normal CSF but a positive CBA serologic NMDA-R antibody test. We described fundamental aspects of RPD to allow made differential diagnoses in patients with cognitive impairment and encephalopathy. Establishing an early and accurate diagnosis can benefit patients with RPD etiologies that are treatable and even reversible, decreasing in morbidity and mortality.
Insights
Rapidly progressive dementia (RPD) presents diverse causes and symptoms. Early diagnosis of RPD is crucial for managing treatable conditions and improving patient outcomes.
Area of Science:
- Neurology
- Neuroscience
Background:
- Rapidly progressive dementia (RPD) is a group of neurological disorders characterized by rapid cognitive decline.
- RPD is often misdiagnosed due to its infrequent nature and limited medical understanding.
- RPD significantly impacts patients' quality of life.
Observation:
- Case 1: A 70-year-old woman presented with neuropsychiatric symptoms, inflammatory cerebrospinal fluid (CSF), and specific MRI/EEG findings.
- Case 2: A 29-year-old man experienced cognitive impairment and seizures, diagnosed via elevated voltage-gated potassium channel antibodies.
- Case 3: A 49-year-old woman showed encephalopathy and seizures, with a positive NMDA-R antibody test despite normal CSF.
Findings:
- The cases illustrate varied etiologies of RPD, including inflammatory and autoimmune conditions.
- Diagnostic tools such as CSF analysis, antibody testing, MRI, and EEG are vital for identifying RPD causes.
- Subtle or normal findings in initial tests do not exclude RPD, necessitating further investigation.
Implications:
- Accurate and timely diagnosis of RPD is essential for effective treatment.
- Identifying treatable RPD etiologies can lead to reversible conditions, reducing patient morbidity and mortality.
- Understanding RPD's diverse presentations aids clinicians in differential diagnosis and patient management.
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