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Review article


Amyotrophic Lateral Sclerosis and its Mimics/Variants: A Comprehensive Review.

Vivek S Yedavalli1Abhijit Patil2Parinda Shah2
1Department of Neuroradiology and Neurointervention, Stanford University, alo Alto, California, 2Department of Radiology, Advocate Illinois Masonic Medical Center, Chicago, Illinois, USA
Date of Submission: 31-May-2018, Date of Acceptance: 11-Aug-2018, Date of Web Publication: 06-Dec-2018.
Corresponding Author:
Corresponding Author

Vivek S Yedavalli

E-mail: vyedav86@stanford.edu

Corresponding Author:
Corresponding Author

Vivek S Yedavalli

E-mail: vyedav86@stanford.edu

DOI: 10.4103/jcis.JCIS_40_18 Facebook Twitter Google Linkedin

ABSTRACT


Motor neuron diseases (MNDs) are a debilitating subset of diseases, which result in progressive neuronal destruction and eventual loss of voluntary muscular function. These entities are often challenging to distinguish and accurately diagnose given overlapping clinical pictures and overall rarity. This group of diseases has a high morbidity and mortality rate overall and delineating each type of disease can help guide appropriate clinical management and improve quality of life for patients. Of all MNDs, amyotrophic lateral sclerosis (ALS) is by far the most common comprising 80%–90% of cases. However, other mimics and variants of ALS can appear similar both clinically and radiographically. In this review, we delve into the epidemiological, physiological, neuroimaging, and prognostic characteristics and management of ALS and its most common MND mimics/variants. In doing so, we hope to improve accuracy in diagnosis and potential management for this rare group of diseases.
Keywords: Amyotrophic lateral sclerosis, postpolio syndrome, primary lateral sclerosis, progressive bulbar palsy, progressive muscular atrophy, spinal and bulbar muscular atrophy

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