Differences in Motor Unit Loss and Axonal Regeneration Rate between Sporadic and Familiar Amyotrophic Lateral Sclerosis: An Undervalued Field of Research?

Changes in Motor Unit Loss and Axonal Regeneration Rate in Sporadic and Familiar Amyotrophic Lateral Sclerosis (ALS) — Possible Different Pathogenetic Mechanisms?

Differential Motor Neuron Impairment and Axonal Regeneration in Sporadic and Familiar Amyotrophic Lateral Sclerosis with SOD-1 Mutations: Lessons from Neurophysiology

UNLABELLED Amyotrophic Lateral Sclerosis (ALS) is a degenerative disorder of the motor system. About 10% of cases are familial and 20% of these families have point mutations in the Cu/Zn superoxide dismutase 1 (SOD-1) gene. SOD-1 catalyses the superoxide radical (O(-2)) into hydrogen peroxide and molecular oxygen. The clinical neurophysiology in ALS plays a fundamental role in differential diag...

An Amyotrophic Lateral Sclerosis Like Symptoms Associated with Lead Toxicity

Abstract Lead is a heavy metal that affects many organs such as nervous system, liver, and kidney. The most important affected organ is central nervous system. The present study reported a case similar to Amyotrophic lateral sclerosis (ALS) due to lead exposure in an opium addicted person. The patient complaint was the weakness of upper and lower limbs in addition to the unsteadiness of gait...

An Iranian familial amyotrophic lateral sclerosis pedigree with p.Val48Phe causing mutation in SOD1: a genetic and clinical report

Objective(s): Amyotrophic lateral sclerosis (ALS), a fatal progressive neurodegenerative disorder, is the most common motor neuron disease in European populations. Approximately 10% of ALS cases are familial (FALS) and the other patients are considered as sporadic ALS (SALS). Among many ALS causing genes that have been identified, mutations in SOD1 and C9orf72 are the most common genetic causes...

White matter alterations differ in primary lateral sclerosis and amyotrophic lateral sclerosis.

Primary lateral sclerosis is a sporadic disorder characterized by slowly progressive corticospinal dysfunction. Primary lateral sclerosis differs from amyotrophic lateral sclerosis by its lack of lower motor neuron signs and long survival. Few pathological studies have been carried out on patients with primary lateral sclerosis, and the relationship between primary lateral sclerosis and amyotro...