La degenerazione colpisce i neuroni presenti nella corteccia cerebrale denominati motoneuroni, il cui compito è quello di trasportare le informazioni sul movimento dal cervello ai muscoli.
E’ anche identificata con il nome di Morbo di Lou Gehrig, per il fatto che questi, un famoso giocatore di baseball statunitense, è stato il primo cui è stata diagnosticata la malattia.
I sintomi della sclerosi laterale amiotrofica (SLA) si presentano dapprima in maniera molto blanda, e prevalentemente localizzati agli arti, con una certa difficoltà ad eseguire movimenti anche semplici, e piccoli disturbi come fiacchezza muscolare, rigidità, crampi.
Se la progressione della malattia colpisce dapprincipio gli arti inferiori il malato avrà sempre più problemi a camminare, e progressivamente perderà la capacità di stare in piedi, se invece viene colpito il braccio un segnale d’allarme è l’incapacità di svolgere movimenti anche relativamente semplici come scrivere o tenere in mano un oggetto.
In un quarto dei casi il debutto del male invece di colpire gli arti colpisce i muscoli del viso, in particolare quelli dedicati alla deglutizione alla masticazione ed al parlare, cosicchè diventa non solo difficile articolare le parole per esprimersi ma anche mangiare.
L’aggravamento del male comporta l’incapacità di svolgere qualsiasi movimento, e per molti pazienti subentra la difficoltà a respirare a causa del mancato funzionamento dei muscoli respiratori, con conseguente insufficienza respiratoria, una delle più comuni cause di mortalità tra chi è affetto da sclerosi laterale amiotrofica.
Solo in pochi casi la malattia colpisce le funzioni cerebrali del pensiero, e perciò la persona è pienamente cosciente di quanto sta accadendo al proprio corpo.
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