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Case Reports
. 2022 Nov 11;14(3):441-455.
doi: 10.1159/000525701. eCollection 2022 Sep-Dec.

Case Report: Three Case Reports of Rapidly Progressive Dementias and Narrative Review

Carlos Andrés Clavijo  1   2 , Ana María Portilla Buenaventura  1 , Galo Santiago Benavides Albornoz  1 , Juan José Muñoz Cabrera  1 , María Camila Murillo Reyes  1 , Alejandra Chauvez Gallego  1 , Carlos Alberto Hurtado González  3   4   5 , Sebastian Ospina Otalvaro  3   4 , Carlos Steven Marmolejo Escobar  3   4 , Karen Julieth Quebrada Mera  3   4 , Paola Andrea Gutiérrez Lenis  3   4 , Lina María Arango García  5 , Armando Lucumí  6
Affiliations
Case Reports

Case Report: Three Case Reports of Rapidly Progressive Dementias and Narrative Review

Carlos Andrés Clavijo et al. Case Rep Neurol. .

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.

Keywords: Creutzfeldt-Jakob disease; Encephalitis; NMDAR; Rapidly progressive dementia; Voltage-gated potassium channel complex.

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Conflict of interest statement

The authors declare that the research was conducted without any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Fig. 1
Fig. 1
Case 1: a Axial T2-weighted Fluid attenuated inversion recovery (FLAIR) image. It is showing slightly high signal intensity (red flag) in the caudate nucleus bilateral. b Axial MRI ADC (Apparent diffusion coefficient). Signal decrease in the bilateral caudate nucleus higher left.
Fig. 2
Fig. 2
Case 2: 12 h of EEG with LFF at 1 Hz and HFF at 70 Hz. It Illustrates continuous fast activity mixed with muscle artifact during and after faciobrachial dystonic seizure.
Fig. 3
Fig. 3
CASE 3: a 12 h of EEG with LFF at 1 Hz and HFF at 70 Hz. Evidence bilateral frontal intermittent slowing complex. b−e Hyperintense lesions on T2 and Fluid attenuated inversion recovery (FLAIR) MRI image compromised the basal ganglia right side with restricted diffusion, no lesion enhancing.
See this image and copyright information in PMC

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