Amiotrofična lateralna skleroza

Amiotrofična lateralna skleroza (ALS) ili Lou Gehrigova bolest pripada grupi neuroloških bolesti, koja zahvaća gornje i donje motorne neurone. Jedna je od najčešćih bolesti motorike, a može se javiti pojedinačno ili kao nasljedna obiteljska bolest (10% svih slučajeva ALS-a). Za 20% nasljeđenih ALS-a dokazana je mutacija gena za citosol, bakar/cink superoksid dismutazu (SOD 1). Do danas je identificirano gotovo 50 SOD 1 mutacija kod osoba s obiteljskim ALS-om. Oboliti može svatko, no ALS najčešće pogađa sredovječne osobe.

Čak 20 000 ljudi u SAD boluje od ALS-a, prema podacima Nacionalnog instituta za neurološke poremećaje i moždani udar, a svake godine dijagnosticira se 5 000 novih slučajeva.

U početku ljudi mogu primijetiti slabost u mišićima, trzanje ili grčenje, a potom bolest progresivno onesposobljava hod, govor, gutanje i na kraju disanje. Mnogi provedu posljednje dane života u potpunosti paralizirani, dok im mozak i dalje funkcionira.

Klinička slika karakterizirana je progresivnim gubitkom mišićne mase i snage, fascikulacijama, spasticitetom, hiperrefleksijom i ekstenzornim plantarnim odgovorom (pozitivan znak Babinski refleksa), kao i razvojem dizartrije i disfagije.

Bolest ima brzi progresivni tijek, a jedini preporučeni lijek od strane FDA je riluzol. Iako tretman riluzolom može produljiti život pacijenta, njegovi učinci su skromni.

Reference

Vanjske veze 
 Les Turner ALS Foundation
 Misao for a Cause  A t-shirt charity created by Misao Apparel, to help benefit those diagnosed with ALS.
 Extra Hands for ALS
 ALS Association
 Stephen Hawking's Website 
 Clinical Trial of Neurodex for Pseudobulbar Affect in People with Amyotrophic Lateral Sclerosis
 Lou Gehrig's Website
 ALS-Project - Free communication software for people suffering from ALS
 ALS Therapy Development Foundation
 PatientsLikeMe - a free online tool for ALS patients to track and share progress
 Duke ALS Clinic The Muscular Dystrophy Association (MDA) has recognized the Duke ALS Clinic as one of its distinguished ALS Research and Clinical Centers.
 Robert Packard Center for ALS Research at Johns Hopkins, a leader in ALS Research
 ALS Support Group ALSforums is an international online support group for individuals affected by ALS (Lou Gehrig's Disease).
 ALS-TDF forums are maintained by the ALS Therapy Development Foundation and focuses on answering scientific questions pertaining to ALS
 BrainTalk communities  was one of the largest ALS/MND forums in the world but following a 2-month hardware failure is currently rebuilding its community.
 BUILD UK  is for people affected by ALS/MND in the UK, but also has overseas members
 PatientsLikeMe is a telesocial medicine site which allows ALS/MND patients to record their symptoms and share information about treatments
 The ALS Advocacy Community  is the ALS Association's online community offering. Members can partake in blogs, discussions, social network and many other features.
 The Patrick O'Brien Foundation A non-profit dedicated to promoting awareness of ALS, established in honor of Patrick Sean O'Brien - an artist diagnosed with ALS at the uncommonly early age of 30, who is chronicling his experience of the disease for a feature film.
 A Ride for 3 Reasons  One man's solo ride across America on a bicycle to raise awareness and money for ALS research. Includes his daily weblog during the ride and profiles of families and individuals living with ALS.

Bolesti
Palijativna medicina
Sistemske atrofije koje prvenstveno utiču na centralni nervni sistem