Mortalidade por esclerose lateral amiotrófica no município de São Paulo de 2002 a 2006 [PDF]
OBJECTIVE: To describe the mortality rates of amyotrophic lateral sclerosis (ALS) in the city of São Paulo as a function of demographics, year, and region. METHOD: This was a retrospective descriptive study.
Conde, Mônica Tilli Reis Pessoa +6 more
core +2 more sources
This study resulted in agendas to improve oral health care, research, and education, advocating for a shift in ALS oral health care: from neglect to proactive integration. ABSTRACT Background and Objective Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that impairs motor function, including oral musculature, complicating
Merel C. Verhoeff +2 more
wiley +1 more source
Predicting disease progression in progressive supranuclear palsy in multicenter clinical trials [PDF]
INTRODUCTION: Clinical and MRI measurements can track disease progression in PSP, but many have not been extensively evaluated in multicenter clinical trials.
AL-108-231 Investigators, . +12 more
core +1 more source
Diagnostic Utility of the ATG9A Ratio in AP‐4–Associated Hereditary Spastic Paraplegia
ABSTRACT Adaptor protein complex 4–associated hereditary spastic paraplegia (AP‐4‐HSP), a childhood‐onset neurogenetic disorder and frequent mimic of cerebral palsy, is caused by biallelic variants in the adaptor protein complex 4 (AP‐4) subunit genes (AP4B1 [for SPG47], AP4M1 [for SPG50], AP4E1 [for SPG51], and AP4S1 [for SPG52]).
Habibah A. P. Agianda +12 more
wiley +1 more source
The clinical spectrum of sporadic and familial forms of frontotemporal dementia [PDF]
The term frontotemporal dementia (FTD) describes a clinically, genetically and pathologically diverse group of neurodegenerative disorders. Symptoms of FTD can present in individuals in their twenties through to their nineties, but the mean age at onset ...
Rohrer, JD, Woollacott, IO
core +1 more source
Abstract INTRODUCTION The olfactory system is an early target in Alzheimer's disease (AD), yet regional glial pathology interactions remain poorly defined. We examined how glial activation and pathological burden differ between the olfactory cortex (OC) and olfactory bulb (OB) across disease stages.
Da Hae Jung +4 more
wiley +1 more source
Olfactory decline tracks central‐to‐peripheral spread of tau pathology in Alzheimer's disease
Abstract INTRODUCTION Olfactory decline often precedes cognitive symptoms in Alzheimer's disease (AD) and is linked to tau pathology. Yet, whether tau aggregates start in peripheral olfactory structures or spread from central regions remains debated. METHODS We analyzed 34 post mortem human olfactory bulb (OB) and 2 neuroepithelium (ONE) samples across
Kamar Bouchoucha +13 more
wiley +1 more source
Decision-making and referral processes for patients with motor neurone disease: a qualitative study of GP experiences and evaluation of a new decision-support tool [PDF]
Background The diagnosis of motor neurone disease (MND) is known to be challenging and there may be delay in patients receiving a correct diagnosis. This study investigated the referral process for patients who had been diagnosed with MND, and whether a
Susan Baxter +17 more
core +3 more sources
INCIDENCE OF AMYOTROPHIC LATERAL SCLEROSIS IN SICILY: A POPULATION BASED STUDY. [PDF]
Our objective was to investigate incidence of amyotrophic lateral sclerosis (ALS) in Sicily, southern Italy, by means of a population based study. We included people with ALS resident in fi ve Sicilian provinces, whose onset occurred in the two-year ...
ARIDON, Paolo +8 more
core +1 more source
Hereditary spastic paraplegia: Identification of an SPG3A gene mutation in a Chinese family [PDF]
Hereditary spastic paraplegias are a heterogeneous group of chronic central motor system disorders, characterised by progressive lower limb spasticity. Hereditary spastic paraplegia is clinically classified into pure and complicated forms, by the absence
Chan, AYW +4 more
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