Case series of probable Creutzfeldt- Jacob Disease admitted in a tertiary hospital in Metro Manila
- Author:
Myleene F. Erola-Fuentes
1
;
Jo Ann R. Soliven
1
Author Information
- Publication Type:Journal Article
- MeSH: Creutzfeldt-Jakob Syndrome; Neurodegenerative Diseases
- From: Philippine Journal of Neurology 2024;27(1):38-48
- CountryPhilippines
- Language:English
-
Abstract:
Background:Creutzfeldt-Jakob disease is a rapidly progressive, fatal, transmissible neurodegenerative
disorder caused by a prion protein. It is characterized by cognitive decline, motor dysfunction,
and eventually, death. It occurs globally with 1 case per one million population/year. And It is
still considered rare in countries like the Philippines due to challenges in its diagnosis and the
under recognition of its clinical features. As of now, the local prevalence or incidence of this
disease in our country remains unknown, as only a single case report has been documented. As
of now, the local prevalence or incidence of this disease in our country remains unknown, as
only a single case report has been documented.
Objective:To report a series of patients with probable sporadic CJD from a tertiary hospital in the Philippines.
Materials and Methods:Patients with rapidly developing dementia fulfilling the diagnostic criteria for sCJD were included. All were investigated in detail to find out any possible treatable cause, including electroencephalography (EEG), magnetic resonance imaging (MRI) of the brain, and cerebrospinal fluid analysis.
Results:A total of 3 patients with probable sCJD were diagnosed using the European diagnostic criterion from January 2022 to April 2023. The clinical features are consistent with other reported series. All 3 patients had the classical EEG findings, typical MRI features, and positive for 14-3-3 assay, and one was positive for RT-QuIC. Two patients died within 13 months from the disease onset.
Conclusion:This is the first reported case series of probable sCJD in the Philippines from a tertiary hospital in Metro Manila. Like in our patients, this disease should be considered in individuals with rapidly progressive dementia associated with myoclonus, neuropsychiatric symptoms, akinetic mutism, visual abnormality, and ataxia with signs of pyramidal and extra-pyramidal dysfunction. Although a definitive diagnosis must be histopathological, there are ancillary tests that are currently available that allow us to make a probable diagnosis of sCJD possible. Our study raises question about the prevalence of this disease in the Philippines which needs more validated studies from other parts of the country. - Full text:2024062912175305313pjn 4.pdf
