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:BackgroundDural arteriovenous fistula (dAVF) rarely presents with a syndrome of reversible parkinsonism and rapidly progressive dementia, which has been described in 19 patients to date. However, its presenting features, pathophysiology, and response to treatment have not been reviewed.MethodsWe report the clinical course and treatment of two novel patients with this syndrome.ResultsDespite severe presentations, both patients recovered fully after fistula obliteration.ConclusionsAtypical parkinsonism with early cognitive changes and other focal neurological signs, along with features such as pulsatile tinnitus or worsening headache, should raise the possibility of dAVF. When treated, this syndrome is nearly always reversible, highlighting the importance of early diagnosis.
| S-EPMC6174482 | biostudies-literature
Project description:Neuro-Behçet's disease (NBD) is subcategorized into parenchymal-NBD (P-NBD) and non-parenchymal-NBD types. Recently, P-NBD has been further subdivided into acute P-NBD (A-P-NBD) and chronic progressive P-NBD (CP-P-NBD). Although an increasing number of studies have reported the various clinical features of A-P-NBD and CP-P-NBD over the last two decades, there was a considerable inconsistency. Two investigators systematically searched four electrical databases to detect studies that provided sufficient data to assess the specific characteristics of A-P-NBD and CP-P-NBD. All meta-analysis was carried out by employing the random-model generic inverse variance method. We included 11 reports consisted of 184 A-P-NBD patients and 114 CP-P-NBD patients. While fever (42% for A-P-NBD, 5% for CP-P-NBD, p?<?0.001, I2?=?93%) was more frequently observed in A-P-NBD cases; sphincter disturbances (9%, 34%, P?=?0.005, I2?=?87%), ataxia (16%, 57%, P?<?0.001, I2?=?92%), dementia (7%, 61%, P?<?0.001, I2?=?97%), confusion (5%, 18%, P?=?0.04, I2?=?76%), brain stem atrophy on MRI (4%, 75%, P?<?0.001, I2?=?98%), and abnormal MRI findings in cerebellum (7%, 54%, P?=?0.02, I2?=?81%) were more common in CP-P-NBD. Cerebrospinal fluid cell count (94/mm3, 11/mm3, P?=?0.009, I2?=?85%) was higher in A-P-NBD cases. We demonstrated that A-P-NBD and CP-P-NBD had clearly different clinical features and believe that these data will help future studies investigating P-NBD.
| S-EPMC5579041 | biostudies-literature
Project description:Dementia developed in a patient with widespread neurologic manifestations; she died within 5 months. Pathologic findings showed granulomatous inflammation with caseation necrosis, foreign body-type giant cells, and proliferative endarteritis with vascular occlusions. Broad-range polymerase chain reaction identified Mycobacterium neoaurum as the possible pathogen. Central nervous system infection by M. neoaurum may result in rapidly progressive dementia.
| S-EPMC3323241 | biostudies-literature
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.
| S-EPMC5773088 | biostudies-literature
Project description:Background: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. Clinical case: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. Conclusion:RT-QuIC offers higher sensitivity than currently prevalent diagnostic methods and appears most promising for CJD diagnosis.
| S-EPMC6362841 | biostudies-literature
Project description:IntroductionWhipple's disease (WD) is a very rare systemic disease caused by the gram-positive bacillus Tropherymawhippleii 1st described in the year 1907. It is a disease with multisystem involvement and high degree of suspicion is needed for diagnosis. However the classical (OMM)oculomasticatory (OFMM)oculofacial-skeletal myorhythmia clubbed with dementia, head ache and other neurologic features should deserve an attempt to confirm whenever possible and therapeutic trial as it is one of the treatable dementias. Males are more affected and probable route of infection is oral though clustering of cases is not reported so far.Case report63 year old hypertensive patient presented with abdominal pain, weight loss, dementia, ataxia, extrapyramidal features, falls, up gaze palsy,oculomastigatory skeletal myorhythmia,skin of the face showing nodules which were pigmented and itchy fallowing HAJ pilgrimage.. Investigations for immune mediated,vasculitic,paraneoplastic, sarcoid were noncontributory. Duodenal biopsy showed nonspecific changes. MRI was consistent with changes reported in Whipples. Patient responded to treatment of Whipples disease.Discussion and conclusionOur patient presented with the typical and unique oculomastigatory myorhythmia clubbed with systemic features of whipplesdisese and showed response to treatment. Limitation of our report we could not do PCR due to lack of availability. This case is being reported for its rarity and to create awareness regarding the typical eye movements.
| S-EPMC5968653 | biostudies-literature
Project description:Behçet's disease (BD) is an inflammatory multisystem disease with unknown etiology, and consists of a TRIAD comprising recurrent oral ulcers, genital ulcers, and uveitis. In some cases, the disease affects the central nervous system, called Neuro-Behçet Disease (NBD). Few cases of NBD simulating a brain tumor have been previously reported.Here, we describe the case of a 46-year-old male patient with a previous diagnosis of brain tumor who was later diagnosed for BD.This case highlights the importance of differential diagnosis of lesions with tumoral features. Checking for the possibility of NBD may help avoiding biopsy in these types of cases.
| S-EPMC5473079 | biostudies-other
Project description:Background:Subacute sclerosing panencephalitis (SSPE) is a disease of childhood and adolescence, but can affect adults. Rapidly progressive cognitive decline, seizures including myoclonic jerks, spasticity, ataxia, visual disturbances, and incontinence are typical manifestations. Case report:A 62-year-old woman who presented with rapidly progressive dementia and myoclonus was diagnosed with SSPE. There was resolution of the movement disorder with clonazepam and valproic acid treatment and some amelioration of cognitive decline after 3 months of therapy with interferon alfa and isoprinosine. Discussion:With the recent rise in measles cases worldwide, any increased incidence of SSPE would require vigilance for early interventions.
| S-EPMC6777289 | biostudies-literature
Project description:Behçet's disease is a rare, systemic variable vessel vasculitis mostly seen in patients from the Middle East, Northern Africa and Central Asia. Neuro-Behçet disease (NBD) is often diagnosed in patients with known Behçet's disease who present with neurological symptoms and radiological features of central nervous system involvement. There are very few cases with neuro-Behçet reported from Sub-Saharan Africa in the literature. We report a case of severe parenchymal neuro-Behçet with pseudo-tumoral brainstem lesions in a young male patient of South African origin.
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