Association of Amyotrophic Lateral Sclerosis and Alzheimer’s Disease: New Entity or Coincidence? A Case Series

2021 ◽  
pp. 1-8
Author(s):  
Agathe Vrillon ◽  
Vincent Deramecourt ◽  
Florence Pasquier ◽  
Éloi Magnin ◽  
David Wallon ◽  
...  

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia have a strong clinical, genetic, and pathological connection but association of ALS with Alzheimer’s disease (AD) is seldom reported. We report a series of 5 cases of AD associated with ALS. Our patients presented with cognitive deterioration with episodic memory impairment meeting criteria for AD. ALS occurred subsequently in all cases and its phenotype was not homogenous. Amyloid process was confirmed in four cases with cerebrospinal fluid biomarkers. One case underwent postmortem exam, demonstrating hallmarks lesions of both diseases. This series highlights that ALS-AD phenotype could be a specific underexplored entity.

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Yui Nakayama ◽  
Satoru Morimoto ◽  
Misao Yoneda ◽  
Shigeki Kuzuhara ◽  
Yasumasa Kokubo

Objective. Amyotrophic lateral sclerosis/parkinsonism-dementia complex is classified as one of the tauopathies. Methods. The total tau, phosphorylated tau, and amyloid β42 levels were assayed in cerebrospinal fluid from patients with Kii amyotrophic lateral sclerosis/parkinsonism-dementia complex (), Alzheimer’s disease (), Parkinson’s disease (), amyotrophic lateral sclerosis (), and controls () using specific enzyme-linked immunosorbent assay methods. Results. Total tau and phosphorylated tau did not increase and amyloid β42 was relatively reduced in Kii amyotrophic lateral sclerosis/parkinsonism-dementia complex. Relatively reduced amyloid β42 might discriminate Kii amyotrophic lateral sclerosis/parkinsonism-dementia complex from amyotrophic lateral sclerosis and Parkinson’s disease, and the ratios of phosphorylated-tau to amyloid β42 could discriminate Kii amyotrophic lateral sclerosis/parkinsonism-dementia complex from Alzheimer’s disease. Conclusions. Cerebrospinal fluid analysis may be useful to differentiate amyotrophic lateral sclerosis/parkinsonism-dementia complex from Alzheimer’s disease, amyotrophic lateral sclerosis, and Parkinson’s disease.


2019 ◽  
Vol 1 ◽  
pp. 22-27
Author(s):  
J. Tarasiuk ◽  
K. Kapica-Topczewska ◽  
M. Chorąży ◽  
A. Borowik-Zaręba ◽  
B. Mroczko ◽  
...  

<b>Introduction:</b>S100 calcium-binding protein B (S100B) is a biochemical marker of astroglial damage. <br/><b>Purpose:</b> To assess the pathophysiological implications of S100B concentrations in blood plasma and cerebrospinal fluid of patients with neurodegenerative central nervous system disorders. <br/><b>Materials and Methods:</b> In this study, we determined and compare S100B concentrations in blood plasma and cerebrospinal fluid obtained from subjects diagnosed with Alzheimer's disease (n=20), amyotrophic lateral sclerosis (n=12), multiple sclerosis (n=40) and the reference group (n=20), using enzyme-linked immunosorbent assay. <br/><b>Results:</b> Concentrations of S100B in plasma collected from patients diagnosed with Alzheimer's disease (252,38±183,50 pg/mL) and multiple sclerosis (164,92±250,14 pg/mL) were above laboratory standards, but in patients with amyotrophic lateral sclerosis (53,96±56,92 pg/mL) and the reference group (2,12 pg/mL) were below laboratory norms (N>75 pg/mL). Concentrations of S100B in plasma collected from patients with Alzheimer's disease (252,38±183,50 pg/mL) were significantly higher than in patients with amyotrophic lateral sclerosis (53,96±56,92 pg/mL) (p<0,029). Concentrations of S100B in CSF collected from the reference group (546,96±236,62 pg/mL) and from patients with Alzheimer's disease (587,53±189,57 pg/mL), amyotrophic lateral sclerosis (404,41±179,56 pg/mL), multiple sclerosis (462,03±146,01 pg/mL) were very similar, and none of pairwise comparisons reached statistical significance. <br/><b>Conclusions:</b> Results of our studies indicate the importance of S100B protein concentration assessment in blood in central nervous system disorders differential diagnostics.


2015 ◽  
Vol 39 (5-6) ◽  
pp. 287-293 ◽  
Author(s):  
Anna Kämäläinen ◽  
Sanna-Kaisa Herukka ◽  
Päivi Hartikainen ◽  
Seppo Helisalmi ◽  
Virpi Moilanen ◽  
...  

Background: The C9ORF72 expansion is one of the most common causes of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The C9ORF72 expansion is associated with TDP-43 and p62 neuropathology, and amyloid plaques and neurofibrillary tangles are not common in patients with the C9ORF72 expansion. Therefore, we hypothesized that cerebrospinal fluid (CSF) biomarkers for Alzheimer's disease [AD; Aβ1-42, total tau (T-tau) and phospho-tau] are normal in these patients. Methods: The CSF Aβ1-42, T-tau and phospho-tau levels were measured in 40 Finnish patients with the C9ORF72 expansion (29 FTLD, 10 ALS and 1 FTLD-ALS) using ELISA. Results: A decreased Aβ1-42 level was found in 25% of cases, while there were only single cases with changes in the t-Tau or phospho-tau level. The patients with abnormal biomarkers fulfilled the clinical criteria of the behavioral variant frontotemporal dementia and expressed no clinical signs of AD. Conclusions: In clinical diagnostics, a decreased CSF Aβ1-42 level does not exclude the C9ORF72 expansion associated with FTLD.


2021 ◽  
pp. 1-6
Author(s):  
Jagan A. Pillai ◽  
James Bena ◽  
Lynn M. Bekris ◽  
Nancy Foldvary-Schaefer ◽  
Catherine Heinzinger ◽  
...  

Sleep dysfunction has been identified in the pathophysiology of Alzheimer’s disease (AD); however, the role and mechanism of circadian rhythm dysfunction is less well understood. In a well-characterized cohort of patients with AD at the mild cognitive impairment stage (MCI-AD), we identify that circadian rhythm irregularities were accompanied by altered humoral immune responses detected in both the cerebrospinal fluid and plasma as well as alterations of cerebrospinal fluid biomarkers of neurodegeneration. On the other hand, sleep disruption was more so associated with abnormalities in circulating markers of immunity and inflammation and decrements in cognition.


2019 ◽  
Vol 10 (2) ◽  
pp. 470 ◽  
Author(s):  
Ashok K. Shetty ◽  
Raghavendra Upadhya ◽  
Leelavathi N. Madhu ◽  
Maheedhar Kodali

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