MedElevator Войти в исследования
← Поиск публикаций

DOI 10.1007/s00702-026-03200-5

Медицинская библиотека · Journal of neural transmission (Vienna, Austria : 1996) · 2026

Creutzfeldt-Jakob disease in the Philippines: diagnostic and management challenges from the first multicenter registry.

Ayo Samuel T, Saranza Gerard, Mabulay Maria Kathleen Angela, Untalan Liamuel, Bilocura Florenz, Dizon Zenaida, Oliva Philip Manuel, Ong Jed Noel, Ng Arlene, Mata-Raciles Cecille, Macabaya Arlene, Lim Bryan, Sage-Nolido Anna Marie, Serrano Grace Kathleen, Surdilla Arturo, Pasco Paul, Kathleen Maria

СтатьяПеревод в очереди

Аннотация

Creutzfeldt-Jakob disease (CJD) is an exceedingly rare, fatal, and rapidly progressive neurodegenerative disorder caused by misfolded prion proteins. In the Philippines, CJD is underrecognized and underreported due to limited access to diagnostic modalities and the absence of a national registry. This multicenter case registry aimed to characterize the clinical, demographic, and diagnostic features of Filipino patients with CJD; determine the distribution of subtypes; describe presenting symptoms and disease progression; assess diagnostic modalities; estimate diagnostic delays and survival time; and identify unique regional features. A retrospective multicenter case series was conducted involving patients from the Philippines diagnosed with probable or suspected CJD from January 2000 to June 2025. Data were collated from hospitals and neurology clinics, and included demographics, clinical presentation, neuroimaging, EEG, CSF biomarkers, PRNP gene analysis, management, and outcomes. Descriptive statistics were used to summarize clinical features and outcomes. Nine CJD cases were identified (mean age: 67 years; 5 males), distributed across Luzon, Visayas, and Mindanao. Myoclonus (77%) and rapidly progressive dementia (55%) were the most frequent symptoms. Diagnostic workup commonly included MRI (cortical ribboning, caudate-putamen hyperintensity), EEG (periodic sharp wave complexes), and limited CSF biomarker testing (RT-QuIC, 14-3-3, tau). Eight cases were classified as probable CJD; average survival was 5.5 months. Stroke and other neurological disorders were common initial misdiagnoses. This registry highlights the rarity, varied presentations, and diagnostic challenges of CJD in a resource-limited setting. It underscores the urgent need for systematic surveillance, clinician education, and improved access to definitive diagnostic testing.

Для цитирования: Ayo Samuel T, Saranza Gerard, Mabulay Maria Kathleen Angela, Untalan Liamuel, Bilocura Florenz, Dizon Zenaida, Oliva Philip Manuel, Ong Jed Noel, Ng Arlene, Mata-Raciles Cecille, Macabaya Arlene, Lim Bryan, Sage-Nolido Anna Marie, Serrano Grace Kathleen, Surdilla Arturo, Pasco Paul, Kathleen Maria. Creutzfeldt-Jakob disease in the Philippines: diagnostic and management challenges from the first multicenter registry.. Journal of neural transmission (Vienna, Austria : 1996). 2026. DOI: 10.1007/s00702-026-03200-5.
Подготовка и перевод публикации
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…

История · было → стало

Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.

Тип публикации указан по данным источника. Уровень доказательности автоматически не присваивается.