Ultrasound assessment of diaphragmatic function in patients with amyotrophic lateral sclerosis.

Background And Objective: Evaluation of diaphragm function in Amyotrophic Lateral Sclerosis (ALS) is critical in determining when to commence non-invasive mechanical ventilation (NIV). Currently, forced vital capacity (FVC) and sniff nasal inspiratory pressure (SNIP) are volitional measures for this evaluation, but require collaboration and are poorly specific. The primary aim of this study was to assess whether diaphragmatic thickness measured by ultrasound (US) correlates with lung function impairment in ALS patients.

Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy.

Very few studies examined trend over time of the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) and factors influencing it; previous studies, then, included only patients attending tertiary ALS Centres. We studied ALSFRS-R decline, factors influencing this trend and survival in a population-based setting. From 2009 onwards, a prospective registry records all incident ALS cases among residents in Emilia Romagna (population: 4.

A further Rasch study confirms that ALSFRS-R does not conform to fundamental measurement requirements.

Our objective was to verify and expand previous evidence of psychometric inadequacies in the ALSFRS-R, in a different sample of subjects suffering from ALS. Since 2009, a prospective registry records all incident cases of ALS in Emilia Romagna Region, Italy (4.4 million inhabitants) referred to its 17 neurological departments.

Epidemiology of amyotrophic lateral sclerosis in Emilia Romagna Region (Italy): A population based study.

Our objective was to describe incidence and clinical features of ALS from a prospective population-based study in Emilia Romagna Region (ERR). From 2009 onwards, a prospective registry recorded all incident cases of ALS among residents in the ERR (population, 4.4 million inhabitants), involving 17 neurological departments.

Early-onset dementia with prolonged occipital seizures: an atypical case of Kufs disease.

Objective: Kufs disease is the adult-onset form of neuronal ceroid lipofuscinosis (NCL). Its two clinical phenotypes are type A (progressive myoclonus epilepsy with dementia) and type B (behavioral abnormalities and dementia, associated with pyramidal and extrapyramidal signs).

Methods: We describe the clinical evolution of an atypical case characterized by progressive dementia and focal occipital seizures.

Systolic and diastolic functions in elderly patients with and without heart failure.

Background: A considerable number of patients with heart failure (HF) have a normal left ventricular ejection fraction (LVEF). In these subjects, HF has usually been related to diastolic heart failure (DHF), still a frequently overlooked clinical entity.

Methods: This study reports the clinical, instrumental, and conventional echocardiographic evaluation of 159 consecutive, hospitalized elderly patients, 87 admitted with HF and 72 admitted for other reasons without overt HF.