IMPORTANCE TAR DNA-binding protein of 43 kDa (TDP-43) plays a major role in the pathogenesis of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Although a pathological continuity between FTLD and ALS has been suggested, the neuropathological changes of the lower motor neuron (LMN) systems have not been assessed in TDP-43-associated FTLD (FTLD-TDP), to our knowl...

BACKGROUND Cognitive impairment is associated with a negative prognosis in amyotrophic lateral sclerosis (ALS), as well as with clinical specificity. We investigate neuropsychological function in ALS patients without known genetic mutations in a Korean tertiary clinic. METHODS Three hundred and eighteen patients were enrolled in a prospective longitudinal cohort from September 2008 to Februar...

Introduction: Amyotrophic lateral sclerosis (ALS) is a neurogenic progressive disease that leads to muscle atrophy. The purpose of this study was to evaluate pulmonary function test (PFT) in patients with ALS and its correlation with ASL symptoms. Materials and Methods: This cross-sectional study was performed on 32 ALS patients at Ghaem Hospital, Mashhad...

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder characterized by progressive muscular paralysis reflecting degeneration of motor neurons in the primary motor cortex, corticospinal tracts, brainstem and spinal cord. The causes of ALS remain largely unknown more than 140 years after the disease was first described. The overall aims of this thesis were to describe t...

Even though neuroimaging and clinical studies indicate that amyotrophic lateral sclerosis (ALS) manifests with distinct clinical phenotypes, no objective test exists to assess upper motor degeneration in ALS. There is great interest in identifying biomarkers of ALS to allow earlier diagnosis and to recognize disease subtypes. Current quantitative neuroimaging techniques such as T2 relaxometry a...

Few analyses in the United States have examined geographic variation and socioeconomic disparities in amyotrophic lateral sclerosis (ALS) incidence, because of lack of population-based incidence data. In this analysis, we used population-based ALS data to identify whether ALS incidence clusters geographically and to determine whether ALS risk varies by area-based socioeconomic status (SES). Thi...

OBJECTIVE To identify the metabolic signature of the various levels of cognitive deficits in amyotrophic lateral sclerosis (ALS) using 18F-2-fluoro-2-deoxy-d-glucose-PET (18F-FDG-PET). METHODS A total of 170 ALS cases consecutively enrolled at the ALS Center of Turin underwent brain 18F-FDG-PET and were classified as displaying normal cognition (ALS-Cn; n = 94), full-blown frontotemporal deme...

Amyotrophic lateral sclerosis (ALS) is a disease characterized by progressive degeneration of motor neurons in the motor cortex, brainstem, and spinal cord. The incidence of sporadic ALS is 1.5 to 2.7 in 100,000, and the prevalence is 5.2 to 6.0 in 100,000. Conjugal ALS is even rarer than sporadic ALS. We report a case of conjugal ALS encountered in our outpatient neurology clinic.

BACKGROUND Hyperechogenicity of the substantia nigra was recently reported in patients with sporadic ALS with a frequency similar to PD. Data on the diagnostic utility compared to key differential diagnoses of ALS do not exist yet. METHODS We prospectively enrolled 43 patients with ALS, 29 with myasthenia gravis, 25 patients with inflammatory neuropathy, and 13 with cervical canal stenosis. A...