PMID- 30298045
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 1664-2295 (Print)
IS  - 1664-2295 (Electronic)
IS  - 1664-2295 (Linking)
VI  - 9
DP  - 2018
TI  - Neuropsychological and Psychological Functioning Aspects in Myotonic Dystrophy 
      Type 1 Patients in Italy.
PG  - 751
LID - 10.3389/fneur.2018.00751 [doi]
LID - 751
AB  - 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.
FAU - Callus, Edward
AU  - Callus E
AD  - Clinical Psychology Service, IRCCS Policlinico San Donato, San Donato Milanese, 
      Italy.
FAU - Bertoldo, Enrico G
AU  - Bertoldo EG
AD  - Clinical Psychology Service, IRCCS Policlinico San Donato, San Donato Milanese, 
      Italy.
FAU - Beretta, Maria
AU  - Beretta M
AD  - Clinical Psychology Service, IRCCS Policlinico San Donato, San Donato Milanese, 
      Italy.
FAU - Boveri, Sara
AU  - Boveri S
AD  - Scientific Directorate, IRCCS Policlinico San Donato, San Donato Milanese, Italy.
FAU - Cardani, Rosanna
AU  - Cardani R
AD  - Laboratory of Muscle Histopathology and Molecular Biology, IRCCS Policlinico San 
      Donato, San Donato Milanese, Italy.
FAU - Fossati, Barbara
AU  - Fossati B
AD  - Department of Neurology, IRCCS Policlinico San Donato, San Donato Milanese, 
      Italy.
FAU - Brigonzi, Elisa
AU  - Brigonzi E
AD  - Department of Neurology, IRCCS Policlinico San Donato, San Donato Milanese, 
      Italy.
FAU - Meola, Giovanni
AU  - Meola G
AD  - Department of Neurology, IRCCS Policlinico San Donato, San Donato Milanese, 
      Italy.
AD  - Department of Biomedical Sciences for Health, University of Milan, Milan, Italy.
LA  - eng
PT  - Journal Article
DEP - 20180919
PL  - Switzerland
TA  - Front Neurol
JT  - Frontiers in neurology
JID - 101546899
PMC - PMC6160752
OTO - NOTNLM
OT  - myotonic dystrophy
OT  - neuropsychological assessment
OT  - neuropsychological functioning
OT  - patient empowerment
OT  - psychological assessment
OT  - psychological funtioning
EDAT- 2018/10/10 06:00
MHDA- 2018/10/10 06:01
PMCR- 2018/09/19
CRDT- 2018/10/10 06:00
PHST- 2018/03/19 00:00 [received]
PHST- 2018/08/17 00:00 [accepted]
PHST- 2018/10/10 06:00 [entrez]
PHST- 2018/10/10 06:00 [pubmed]
PHST- 2018/10/10 06:01 [medline]
PHST- 2018/09/19 00:00 [pmc-release]
AID - 10.3389/fneur.2018.00751 [doi]
PST - epublish
SO  - Front Neurol. 2018 Sep 19;9:751. doi: 10.3389/fneur.2018.00751. eCollection 2018.