Parkinsonism and Rapidly Progressive Dementia Secondary to Neuro-Behcet.
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ABSTRACT:
SUBMITTER: Rubio-Hernandez M
PROVIDER: S-EPMC7525201 | biostudies-literature | 2020 Sep
REPOSITORIES: biostudies-literature
Publications
Parkinsonism and Rapidly Progressive Dementia Secondary to Neuro-Behçet.
Rubio-Hernandez Moises M Rodriguez-Leyva Ildefonso I Alvarado-Gutierrez Alma A Gallegos Ríos Carlos C Abud Mendoza Carlos C Abad-Guangorena Edgar E
Movement disorders clinical practice 20200929 Suppl 3
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Project description:Background and purposeRapidly progressive dementia (RPD) is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. This study investigated the profile of patients with rapidly progressive dementia at first presentation.MethodsRetrospective case analysis was done in 187 patients with rapidly progressive dementia who presented to the Postgraduate Institute of Medical Education and Research, Chandigarh, India from January 2008 to August 2016. Patients were divided into three groups: (1) Reversible (treatable) secondary dementia group, (2) Prion dementia group (sporadic Creutzfeldt-Jakob disease), (3) Non-prion Neurodegenerative and vascular dementias (primary neurodegenerative and vascular dementia). Cases presenting with delirium secondary to metabolic, drug induced or septic causes and those with signs of meningitis were excluded.ResultsSecondary reversible causes formed the most common cause for RPD with immune mediated encephalitides, neoplastic and infectious disorders as the leading causes. The patients in this series had an younger onset of RPD. Infections presenting with RPD accounted for the most common cause in our series (39%) with SSPE (41%) as the leading cause followed by neurosyphilis (17.9%) and progressive multifocal leukoencephalopathy (15.3%). Immune mediated dementias formed the second most common (18.1%) etiologic cause for RPD. The neurodegenerative dementias were third common cause for RPD in our series. Neoplastic disorders and immune mediated presented early (< 6 months) while neurodegenerative disorders presented later (> 6 months).ConclusionsRapidly progressive dementia is an emergency in cognitive neurology with potentially treatable or reversible causes that should be sought for diligently.
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Project description:ObjectiveTo investigate brain MRI abnormalities in a cohort of patients with rapidly progressive dementia (RPD) with and without a diagnosis of Creutzfeldt-Jakob disease (CJD).MethodsOne hundred and seven patients with diagnosis of prion disease (60 with definite sCJD, 33 with probable sCJD and 14 with genetic prion disease) and 40 non-prion related RPD patients (npRPD) underwent brain MRI including DWI and FLAIR. MRIs were evaluated with a semiquantitative rating score, which separately considered abnormal signal extent and intensity in 22 brain regions. Clinical findings at onset, disease duration, cerebrospinal-fluid 14-3-3 and t-tau protein levels, and EEG data were recorded.ResultsAmong patients with definite/probable diagnosis of CJD or genetic prion disease, 2/107 had normal DWI-MRI: in one patient a 2-months follow-up DWI-MRI showed CJD-related changes while the other had autopsy-proven CJD despite no DWI abnormalities 282 days after clinical onset. CJD-related cortical changes were detected in all lobes and involvement of thalamus was common. In the npRPD groups, 6/40 patients showed DWI alterations that clustered in three different patterns: (1) minimal/doubtful signal alterations (limbic encephalitis, dementia with Lewy bodies); (2) clearly suggestive of alternative diagnoses (status epilepticus, Wernicke or metabolic encephalopathy); (3) highly suggestive of CJD (mitochondrial disease), though cortical swelling let exclude CJD.ConclusionsIn the diagnostic work-up of RPD, negative/doubtful DWI makes CJD diagnosis rather unlikely, while specific DWI patterns help differentiating CJD from alternative diagnoses. The pulvinar sign is not exclusive of the variant form.
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Project description:Developing methods for accurately diagnosing prion diseases has been a challenge in the search for successful diagnosis and treatment of rapidly progressive dementia. prion diseases are rare. However, they should be considered in the differential diagnosis. Despite their rarity, several other conditions are often misdiagnosed as prion diseases. Most Alzheimer's (AD) and Lewy Body Disease (LBD) patients also meet Creutzfeldt-Jakob Disease (CJD) criteria. The similarities in symptomology and pathology between these two patient groups complicates diagnosis and can compromise patient care. Prevalent methods for the diagnosis of CJD lack the heightened sensitivity to conclusively detect CJD. Of all currently available methods, real-time quaking induced conversion (RT-QuIC) analysis provides the highest sensitivity necessary to allow for an accurate diagnosis and yields early, quantitative results. A 75-year-old woman with rapidly progressing dementia, for which CJD could not be ruled out, appeared for care at a neurological center. Laboratory test results, Magnetic Resonance Imaging (MRI), Cerebrospinal Fluid (CSF) studies, Positron Emission Tomography (PET), and an Electroencephalogram (EEG) proved inadequate to confirm CJD. In addition to AD, LBD, or CJD, other potential, yet improbable, pathologies could have caused the patient's symptoms. The patient's diagnosis ultimately was limited to either LBD or prion disease. Spongiform encephalogy was confirmed by a brain biopsy, and further testing confirmed sporadic CJD. RT-QuIC offers higher sensitivity than currently prevalent diagnostic methods and appears most promising for CJD diagnosis.
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