MRI findings and cognitive functions in a small cohort of myotonic dystrophy type 1: Retrospective analyses

Bajrami, Arsida; Azman, Filiz; Yayla, Vildan; Cagirici, Sultan; Keskinkılıç, Cahit; Sozer, Nejla

doi:10.1177/1971400916678223

Cited by 16 publications

(16 citation statements)

References 23 publications

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“… 42 Bajrami et al . 43 suggest that the WM hyperintense lesions (WMHLs) located at the temporal poles seem to be characteristics of the disease, while others, small and diffuse WMHLs would be similar to the age-related alterations. 43 …”

Section: Discussionmentioning

confidence: 99%

Cognitive impairment in neuromuscular diseases: A systematic review

et al. 2018

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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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Section: Discussionmentioning

confidence: 99%

Cognitive impairment in neuromuscular diseases: A systematic review

et al. 2018

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“…The purpose of this research was to investigate both cognition and a psychological symptomatology association. We opted for a battery containing screening tests (MMSE, for a general cognitive screening; FAB for the assessment of the executive functions) and we decided to go for ENB-2 battery vs. WAIS, because it is validated on the Italian population and it offers the advantage of a shorter assessment time ( 7 , 11 , 12 , 14 , 15 , 18 , 22 , 26 , 27 , 30 , 35 , 39 , 51 , 53 , 56 , 58 , 61 , 62 , 66 – 69 , 74 , 76 , 78 ).…”

Section: Methodsmentioning

confidence: 99%

Neuropsychological and Psychological Functioning Aspects in Myotonic Dystrophy Type 1 Patients in Italy

et al. 2018

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Introduction: Myotonic Dystrophy Type 1 (DM1) is an autosomal dominant genetic illness, characterized by a progressive loss of strength. Important deficits in cognitive functioning and a significant prevalence of psychiatric disorders have been previously reported.Methods:A neuropsychological and psychological assessment was carried out in 31 DM1 patients (61% males) in order to measure the cognitive functioning and explore their personality profiles. The MMSE Mini-Mental State Examination, Frontal Assessment Battery (FAB), ENB-2 Battery assessing memory (short term, long term and working memory), integration capacities, visual-spatial ability, attention (selective, divided, shifting/switching) executive functions, praxis, discrimination and logic capabilities and psychopathology Symptom Check List 90-R (SCL-90-R) were administered. The neuropsychological and psychological evaluation of DM1 patients was carried out taking into consideration the clinical parameters (CTG repeat, age at onset, disease duration, Muscular Impairment Rate Scale (MIRS), Medical Research Council Scale (MRC) and the Epworth Sleepiness Scales (EPS)).Results: Regarding psychopathology 19.4% of patients scored a moderate or high level of symptoms intensity index (GSI), 12.9% reported a high number of symptoms (PST) and 16.1% reported a high intensity level of the perceived symptoms (PSDI). Fatigue and daytime sleepiness resulted as being associated with higher levels of psychoticism (PSY). Only 1 patient reported a severe impairment in the spatial and temporal orientation, memory, language, praxis, attention and calculation. Longer disease duration was also associated with cognitive impairment evaluated through ENB-2 (p < 0.05).Discussions and Conclusions:There are indications of the utility of neuropsychological and psychological screening and support for these patients and their families due to the link between disease duration and cognitive performances. A proposal of a clinical protocol, with an illustration of a clinical case report of a family is presented.

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“…Studies have shown through the used PET, and MRI brain scanning technology, those new neural cells are generated through life as well as new neural pathways [17] [18]. So, old brains are capable of creating measurable changes in brain organization.…”

Section: Discussionmentioning

confidence: 99%

“…Focuses on this issue, researchers are confident that the brain continually adapts and reorganizes. Research about cognitive functions using fMRI found that the neuronal connections in many brain regions appear to be organized differently each time [18] [27].…”

Section: Discussionmentioning

confidence: 99%

Can Digital Games Be a Way of Improving the Neuroplasticity in Stroke Damage? Can the Adult Brain Grow New Cells or Rewire Itself in Response to a New Experience?

Valentin¹

2017

OJMP

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Exploratory studies developed at several neurosciences laboratories at universities around the world show us through the experience that there is a biological process called neuroplasticity. Because of this oldest concept about the neuronal formation, scientists also thought that if a particular area of the adult brain was damaged, the nerve cells could not form new connections and the functions controlled by this field of the brain would be permanently lost or could not be regenerate. However, studies have overturned this old view, and currently, scientists recognize that the brain continues to reorganize itself by forming new neural connections during the life. This phenomenon is called neuroplasticity that refers to the potential which the brain should be reorganized by creating new neural pathways to adapt, as it needs.

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MRI findings and cognitive functions in a small cohort of myotonic dystrophy type 1: Retrospective analyses

Cited by 16 publications

References 23 publications

Cognitive impairment in neuromuscular diseases: A systematic review

Cognitive impairment in neuromuscular diseases: A systematic review

Neuropsychological and Psychological Functioning Aspects in Myotonic Dystrophy Type 1 Patients in Italy

Can Digital Games Be a Way of Improving the Neuroplasticity in Stroke Damage? Can the Adult Brain Grow New Cells or Rewire Itself in Response to a New Experience?

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