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Grasso, Irene (2015) Psychological and neuropsychological advances in Motor neuron diseases: Amyotrophic lateral sclerosis and X-linked spinal-bulbar muscular atrophy. [Tesi di dottorato]

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Abstract (inglese)

Motor neuron disease (MND) is a heterogeneous family of neurodegenerative diseases, rare, characterized by an early involvement of motor neurons and can be hereditary or sporadic, including Amyotrophic lateral sclerosis (ALS) and X-linked spinal-bulbar muscular atrophy (SBMA).
Three studies will be briefly presented below, with the aim of developing an understanding of psychological and neuropsychological aspects in these diseases.
In Chapter 3 is presented a retrospective study with the aim to verify whether the presence of cognitive impairment in ALS patients (according to the classification proposed by Phukan et al., 2012) may have an impact on the degree of disease progression in terms of motor decline. Up to date, in literature the debate about the influence of cognitive impairment on survival in ALS patients is still ongoing and results are conflicting. In summary, after 6 or 12 months follow up we observed the same degree of disease progression both in patients with presence or absence of cognitive impairment.
In Chapter 4 it is presented a pilot study with the aim to investigate the applicability and efficacy of a hypnotic treatment on the physical and psychological well-being perceived by ALS patients, considering also the indirect effect on the well-being on caregivers. ALS implies an inevitable and devastating psychological impact and this study represent, even if at a preliminary level, the first application of a protocol of psychological intervention with evidence of efficacy in ALS patients.
In Chapter 6, it is presented a study with the aim to investigate the neuropsychological and psychological profile in patients with SBMA, poorly characterized in the recent literature. In summary, in contrast with the previous literature, executive functioning seems to be apparently preserved in patients with SBMA; nevertheless patients seem to have a specific dysfunction on the cognitive component of the Theory of Mind (ToM), while the affective component seems to be preserved.

Abstract (italiano)

La Malattia del motoneurone (MND) è una famiglia eterogenea di malattie neurodegenerative, rare, caratterizzate da un precoce coinvolgimento dei motoneuroni e possono essere ereditarie o sporadiche, e includono tra le altre la Sclerosi laterale amiotrofica (SLA) e l'Atrofia muscolare spinobulbare (SBMA). Sono stati condotti tre studi, brevemente presentati di seguito, con l'obiettivo di sviluppare la comprensione degli aspetti psicologici e neuropsicologici nelle suddette malattie.
Nel Capitolo 3 viene presentato uno studio retrospettivo con l'obiettivo di verificare se la presenza di deficit cognitivo in pazienti affetti da SLA (in accordo con la classificazione proposta da Phukan et al., 2012) possa avere un impatto sul grado di progressione di malattia in termini di declino motorio, ambito nel quale i dati presenti in letteratura sono contrastanti. In sintesi, a distanza di 6 o 12 mesi dal baseline si osserva lo stesso grado di progressione di malattia sia in presenza sia in assenza del deficit cognitivo.
Nel Capitolo 4 viene presentato uno studio pilota con l'obiettivo di indagare l'applicabilità e l'efficacia di un trattamento ipnotico sul benessere psicologico e fisico percepito dai pazienti, valutando inoltre l'effetto indiretto sul benessere dei familiari. Nonostante la SLA implichi un inevitabile e devastante impatto psicologico il presente studio rappresenterebbe, anche se a un livello preliminare, la prima applicazione di un protocollo di intervento psicologico con prove di efficacia in pazienti affetti da SLA.
Nel Capitolo 6 viene presentato uno studio con l'obiettivo di indagare il profilo neuropsicologico e psicologico nei pazienti affetti da SBMA, ad oggi poco investigato con riferimento alla recente letteratura. In sintesi, a differenza degli studi presenti in letteratura, il funzionamento esecutivo sembra essere apparentemente preservato nei pazienti affetti da SBMA; ciononostante i pazienti sembrano avere una specifica disfunzione a carico della componente cognitiva della Teoria della Mente (ToM), mentre risulta preservata la componente affettiva.

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Tipo di EPrint:Tesi di dottorato
Relatore:Sorarù, Gianni
Dottorato (corsi e scuole):Ciclo 27 > scuole 27 > SCIENZE MEDICHE, CLINICHE E SPERIMENTALI > NEUROSCIENZE
Data di deposito della tesi:07 Luglio 2015
Anno di Pubblicazione:07 Luglio 2015
Parole chiave (italiano / inglese):malattia del motoneurone, sclerosi laterale amiotrofica, malattia di kennedy, atrofia muscolare spinobulbare, progressione di malattia, funzioni esecutive, intervento psicologico, teoria della mente / motor neuron disease, amyotrophic lateral sclerosis, Kennedy’s disease, spinal and bulbar muscular atrophy, disease progression, executive functions, psychological intervention, theory of mind
Settori scientifico-disciplinari MIUR:Area 06 - Scienze mediche > MED/26 Neurologia
Struttura di riferimento:Dipartimenti > Dipartimento di Scienze Cardiologiche, Toraciche e Vascolari
Codice ID:8848
Depositato il:22 Ago 2016 11:26
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Chapter 1 - References Cerca con Google

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Chapter 2 - References Cerca con Google

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Chapter 5 - References Cerca con Google

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Chapter 6 - References Cerca con Google

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