Chapter 6 Cognitive Impairment in the Motor Neuron Disorders

2003 ◽  
pp. 145-cp1
Author(s):  
Michael J. Strong ◽  
Catherine Lomen-Hoerth ◽  
Wenchang Yang
Keyword(s):  
Cognitive Impairment ◽  
Motor Neuron

Cognitive impairment, frontotemporal dementia, and the motor neuron diseases

2003 ◽  
Vol 54 (S5) ◽  
pp. S20-S23 ◽  
Author(s):  
Michael J. Strong ◽  
Catherine Lomen-Hoerth ◽  
Richard J. Caselli ◽  
Eileen H. Bigio ◽  
Wencheng Yang
Keyword(s):  
Cognitive Impairment ◽  
Motor Neuron ◽  
Frontotemporal Dementia ◽  
Motor Neuron Diseases

scholarly journals 042 Respiratory function and cognitive profile in motor neuron disease

2019 ◽  
Vol 90 (e7) ◽  
pp. A14.2-A14
Author(s):  
William Huynh ◽  
Lara E Sharplin ◽  
Jashelle Caga ◽  
Elizabeth Highton-Williamson ◽  
Matthew C Kiernan
Keyword(s):  
Cognitive Impairment ◽  
Cognitive Function ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Respiratory Function ◽  
Normal Function ◽  
Cognitive Testing ◽  
Respiratory Compromise ◽  
Respiratory Dysfunction ◽  
Non Invasive Ventilation

IntroductionMotor neuron disease (MND) is increasingly recognised as a multisystems disorder with 30–50% of patients having mild to moderate cognitive impairment. Mechanisms of cognitive dysfunction in MND are multifactorial but chronic hypoxia secondary to respiratory dysfunction may contribute to cognitive decline in patients.ObjectivesThe current study aimed to identify the relationship between respiratory function in MND patients and the presence and degree of cognitive impairment.MethodsMND patients were prospectively recruited from a multidisciplinary MND clinic. Patients meeting the criteria for frontotemporal dementia were excluded. Baseline clinical assessments including respiratory function as assessed by spirometry were recorded with FVC ≤ 75% considered to have reduced respiratory function. Cognitive testing was performed utilising the Addenbrooke’s Cognitive Examination (ACE).ResultsFrom a cohort of 100 MND patients 48% were categorised as having impaired respiratory function whilst 52% had normal function. Compared to the group with normal respiratory function (ACE: 86.83±1.5), patients with respiratory dysfunction had significantly reduced cognitive function (ACE: 90.68±0.89, P=0.025). Subscores demonstrated significant differences between the groups with respect to domains in memory, attention with a trend observed in fluency. There was a significant correlation between FVC and ACE scores as well as between FVC and memory and fluency subscores (P<0.01).ConclusionMND patients with respiratory compromise were more likely to develop reduced cognitive function. In addition to improving physical function, it remains plausible that non-invasive ventilation may alter the progression of cognitive impairment in MND patients, thereby potentially improving their overall quality of life and carer burden.

Cognitive impairment in motor neuron disease

2009 ◽  
Vol 71 (6) ◽  
pp. 480-484 ◽  
Author(s):  
R. Gallassi ◽  
P. Montagna ◽  
C. Ciardulli ◽  
S. Lorusso ◽  
V. Mussuto ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Motor Neuron ◽  
Motor Neuron Disease

Association of Clinically Evident Eye Movement Abnormalities With Motor and Cognitive Features in Patients With Motor Neuron Disorders

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012774
Author(s):  
Barbara Poletti ◽  
Federica Solca ◽  
Laura Carelli ◽  
Alberto Diena ◽  
Eleonora Colombo ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Motor Neuron ◽  
Eye Movement ◽  
Cognitive Behavioral ◽  
Cognitively Impaired ◽  
Clinical Markers ◽  
Behavioral Alterations ◽  
Neuropsychological Battery ◽  
Bulbar Onset ◽  
Als Patients

Objective:Although oculomotor abnormalities (OMAs) are not usually considered prominent features of amyotrophic lateral sclerosis (ALS), they may represent potential clinical markers of neurodegeneration, especially when investigated together with cognitive and behavioral alterations. The aim of our study was to identify patterns of clinically evident OMAs in ALS patients and to correlate such findings with cognitive-behavioral data.Methods: three consecutive, inpatient cohorts of Italian ALS patients and controls were retrospectively evaluated to assess the frequency of OMAs and cognitive-behavioral alterations. The ALS population was divided in a discovery and a replication cohort. Controls included a cohort of cognitively impaired individuals and of patients with Alzheimer’s disease (AD). Subjects underwent bedside eye movement evaluation to determine the presence and pattern of OMAs. Cognitive assessment was performed using a standard neuropsychological battery (discovery ALS cohort, and AD cohort), and the Italian Edinburgh Cognitive and Behavioural ALS Screen – ECAS (replication ALS cohort).Results:We recruited 864 ALS (635 discovery, 229 replication), 798 cognitively unimpaired, and 171 AD subjects. OMAs were detected in 10.5% of our ALS cohort vs 1.6% of cognitively unimpaired controls (p=1.2x10-14) and 11.4% of AD patients (p=ns). The most frequent deficits were smooth pursuit and saccadic abnormalities. OMAs frequency was higher in patients with bulbar onset, prominent upper motor neuron signs, and advanced disease stages. Cognitive dysfunction was significantly more frequent in patients with OMAs in both ALS cohorts (p=1.1x10-25). Furthermore, OMAs significantly correlated with the severity of cognitive impairment and with pathological scores at the ECAS ALS-specific domains. Lastly, OMAs could be observed in 35.0% of cognitively impaired ALS vs 11.4% of AD patients (p=6.4x10-7), suggesting a possible involvement of frontal oculomotor areas in ALS.Discussion:ALS patients showed a range of clinically evident OMAs, and these alterations were significantly correlated with cognitive, but not behavioral, changes. OMAs may be a marker of neurodegeneration and bedside assessment represents a rapid, highly specific tool for detecting cognitive impairment in ALS.

scholarly journals Relationship between neuropsychiatric disorders and cognitive and behavioural change in MND

2019 ◽  
Vol 91 (3) ◽  
pp. 245-253 ◽  
Author(s):  
Caroline A McHutchison ◽  
Danielle Jane Leighton ◽  
Andrew McIntosh ◽  
Elaine Cleary ◽  
Jon Warner ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Family History ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Frontotemporal Dementia ◽  
Mood Disorders ◽  
Behavioural Change ◽  
Neuropsychiatric Disorders ◽  
Neuropsychiatric Disorder ◽  
History Of

ObjectiveIn this population-based study, we aimed to determine whether neuropsychiatric history, medication or family history of neuropsychiatric disorders predicted cognitive and/or behavioural impairment in motor neuron disease (MND).MethodsPeople with MND (pwMND) on the Scottish Clinical, Audit, Research and Evaluation of MND (CARE-MND) register, diagnosed from January 2015 to January 2018, with cognitive and/or behavioural data measured using the Edinburgh Cognitive and Behavioural ALS Screen were included. Data were extracted on patient neuropsychiatric, medication and family history of neuropsychiatric disorders. We identified patients with cognitive impairment (motor neuron disease with cognitive impairment (MNDci)), behavioural impairment (motor neuron disease with behavioural impairment (MNDbi), both (motor neuron disease with cognitive and behavioural impairment (MNDcbi)) or motor neuron disease–frontotemporal dementia (MND-FTD).ResultsData were available for 305 pwMND (mean age at diagnosis=62.26 years, SD=11.40), of which 60 (19.7%) had a neuropsychiatric disorder. A family history of neuropsychiatric disorders was present in 36/231 (15.58%) of patients. Patient premorbid mood disorders were associated with increased apathy (OR=2.78, 95% CI 1.083 to 7.169). A family history of any neuropsychiatric disorder was associated with poorer visuospatial scores, MNDbi (OR=3.14, 95% CI 1.09 to 8.99) and MND-FTD (OR=5.08, 95% CI 1.26 to 20.40). A family history of mood disorders was associated with poorer overall cognition (exp(b)=0.725, p=0.026), language, verbal fluency and visuospatial scores, and MND-FTD (OR=7.57, 95% CI 1.55 to 46.87). A family history of neurotic disorders was associated with poorer language (exp(b)=0.362, p<0.001), visuospatial scores (exp(b)=0.625, p<0.009) and MND-FTD (OR=13.75, 95% CI 1.71 to 110.86).ConclusionNeuropsychiatric disorders in patients and their families are associated with cognitive and behavioural changes post-MND diagnosis, with many occurring independently of MND-FTD and C9orf72 status. These findings support an overlap between MND, frontotemporal dementia and neuropsychiatric disorders, particularly mood disorders.

scholarly journals Motor Neuron Syndrome as a New Phenotypic Manifestation of Mutation 9185T>C in Gene MTATP6

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Marisa Brum ◽  
Cristina Semedo ◽  
Rui Guerreiro ◽  
José Pinto Marques
Keyword(s):  
Cognitive Impairment ◽  
Motor Neuron ◽  
Muscle Weakness ◽  
Mitochondrial Disease ◽  
Muscular Atrophy ◽  
Leigh Syndrome ◽  
Exercise Intolerance ◽  
Laboratory Findings ◽  
Progressive Muscle Weakness ◽  
Atp6 Gene

Background.The mutation 9185T>C in ATP6 gene, associated with Leigh syndrome, was reported in only few families. Motor neuron disease (MND), both clinically and electrophysiologically, was not previously described in association with this mutation.Case Report.33-year-old male, with family history of mitochondrial disease, presented with cognitive impairment, exercise intolerance, and progressive muscle weakness. Examination revealed global hypotonia, and proximal tetraparesis, without atrophy or fasciculation, pyramidal signs, or sensory symptoms. The laboratory findings revealed an increase of lactate and lactate/pyruvate ratio; electromyogram showed chronic neurogenic compromise; muscle biopsy was suggestive of spinal muscular atrophy and mitochondriopathy; genetic study of SMN1 was negative but detected a homoplasmic mutation 9185T>C in ATP6 gene. His younger sister, with the same mutation, had cognitive impairment, ataxia, and muscle weakness. EMG showed axonal peripheral neuropathy.Conclusion.This case is unique because of the benignity and the coexistence of clinical, neurophysiological, and pathological findings suggestive of MND that, although described in mitochondrial disease, have not yet been reported in association with 9185T>C mutation. The present case contributes to the expansion of the phenotypic expressions of this particular mutation.

scholarly journals Cognitive impairment in neuromuscular diseases: A systematic review

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Marco Orsini ◽  
Ana Carolina Andorinho de F. Ferreira ◽  
Anna Carolina Damm de Assis ◽  
Thais Magalhães ◽  
Silmar Teixeira ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Genetic Analysis ◽  
Motor Neuron ◽  
Case Reports ◽  
Experimental Studies ◽  
Neuromuscular Diseases ◽  
Mitochondrial Disorders ◽  
Motor Neuron Diseases ◽  
Fiber Damage ◽  
Molecular Alterations

Neuromuscular diseases are multifactorial pathologies characterized by extensive muscle fiber damage that leads to the activation of satellite cells and to the exhaustion of their pool, with consequent impairment of neurobiological aspects, such as cognition and motor control. To review the knowledge and obtain a broad view of the cognitive impairment on Neuromuscular Diseases. Cognitive impairment in neuromuscular disease was explored; a literature search up to October 2017 was conducted, including experimental studies, case reports and reviews written in English. Keywords included Cognitive Impairment, Neuromuscular Diseases, Motor Neuron Diseases, Dystrophinopathies and Mitochondrial Disorders. Several cognitive evaluation scales, neuroimaging scans, genetic analysis and laboratory applications in neuromuscular diseases, especially when it comes to the Motor Neuron Diseases, Dystrophinopathies and Mitochondrial Disorders. In addition, organisms model using rats in the genetic analysis and laboratory applications to verify the cognitive and neuromuscular impacts. Several studies indicate that congenital molecular alterations in neuromuscular diseases promote cognitive dysfunctions. Understanding these mechanisms may in the future guide the proper management of the patient, evaluation, establishment of prognosis, choice of treatment and development of innovative interventions such as gene therapy.

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