PMID- 33065300
OWN - NLM
STAT- MEDLINE
DCOM- 20211125
LR  - 20211125
IS  - 1877-0665 (Electronic)
IS  - 1877-0657 (Linking)
VI  - 64
IP  - 5
DP  - 2021 Sep
TI  - Anosognosia in amyotrophic lateral sclerosis: A cross-sectional study of 85 
      individuals and their relatives.
PG  - 101440
LID - S1877-0657(20)30171-8 [pii]
LID - 10.1016/j.rehab.2020.08.004 [doi]
AB  - BACKGROUND: Amyotrophic lateral sclerosis (ALS) has long been considered a pure 
      motor neurodegenerative disease. However, now, extra-motor manifestations such as 
      cognitive-behavioral disorders are considered not rare and are even a severity 
      factor of the disease. Experiencing anosognosia (i.e., the inability to recognize 
      neurological symptoms) might affect care and treatment compliance in ALS. 
      Regardless, this pivotal feature has been little investigated. OBJECTIVES: By 
      comparing patients' and caregivers' reports, we analysed whether patients with 
      ALS would experience a lack of awareness about their executive disorders and 
      their apathy symptoms. METHODS: From the ALS reference center in Paris, we 
      included 85 patients (47 men, mean [SD] age 60.5 [12] years and ALS-Functional 
      Rating Scale-revised score 8 to 46) and their primary family caregivers who all 
      completed the Dysexecutive Questionnaire (DEX) and the Apathy Evaluation Scale 
      (AES). Overall scores and answers were compared by agreement/disagreement 
      statistical methods. RESULTS: Caregivers reported higher levels of 
      cognitive-behavioral disorders than did patients, but reports matched when 
      cognitive-behavioral disorders were absent or mild. With published DEX and AES 
      cutoffs, 32% and 51% of patients had executive disorders and apathy, 
      respectively. In these patients with significant impairment, Bland-Altman plots 
      (i.e., visual display agreement that represents the difference between the 
      patient's and caregiver's scores as a function of their average) showed a strong 
      discrepancy between joint reports: patients underestimated their symptoms by a 
      mean bias of -6.81 DEX points (95% confidence interval -11.88, -1.75) and -8.85 
      AES points (95% confidence interval -11.72, -5.98). We found no clear 
      relationship between bulbar or spinal ALS subtypes and anosognosia. CONCLUSIONS: 
      ALS patients with a cognitive-behavioral phenotype show anosognosia by a mismatch 
      between self and proxy reports, which warrants further investigation in 
      neuroimaging. Systematic longitudinal screening of anosognosia is needed to 
      propose targeted psychoeducation in patient-caregiver dyads showing disagreement.
CI  - Copyright (c) 2020 Elsevier Masson SAS. All rights reserved.
FAU - Salah, Amina Ben
AU  - Salah AB
AD  - Department of Physical Rehabilitation Medicine, Pitie-Salpetriere hospital 
      (AP-HP) and GRC 24 (Sorbonne Universite), 47, boulevard de l'Hopital, 75013 
      Paris, France.
FAU - Pradat, Pierre-Francois
AU  - Pradat PF
AD  - Laboratoire d'imagerie biomedicale (LIB), Sorbonne universite, Paris, France; 
      Department of Neurology and Reference ALS Center, Pitie-Salpetriere Hospital 
      (AP-HP), Paris, France.
FAU - Villain, Marie
AU  - Villain M
AD  - Department of Physical Rehabilitation Medicine, Pitie-Salpetriere hospital 
      (AP-HP) and GRC 24 (Sorbonne Universite), 47, boulevard de l'Hopital, 75013 
      Paris, France.
FAU - Balcerac, Alexander
AU  - Balcerac A
AD  - Department of Physical Rehabilitation Medicine, Pitie-Salpetriere hospital 
      (AP-HP) and GRC 24 (Sorbonne Universite), 47, boulevard de l'Hopital, 75013 
      Paris, France.
FAU - Pradat-Diehl, Pascale
AU  - Pradat-Diehl P
AD  - Department of Physical Rehabilitation Medicine, Pitie-Salpetriere hospital 
      (AP-HP) and GRC 24 (Sorbonne Universite), 47, boulevard de l'Hopital, 75013 
      Paris, France; Laboratoire d'imagerie biomedicale (LIB), Sorbonne universite, 
      Paris, France.
FAU - Salachas, Francois
AU  - Salachas F
AD  - Department of Neurology and Reference ALS Center, Pitie-Salpetriere Hospital 
      (AP-HP), Paris, France.
FAU - Lacomblez, Lucette
AU  - Lacomblez L
AD  - Department of Neurology and Reference ALS Center, Pitie-Salpetriere Hospital 
      (AP-HP), Paris, France.
FAU - Bayen, Eleonore
AU  - Bayen E
AD  - Department of Physical Rehabilitation Medicine, Pitie-Salpetriere hospital 
      (AP-HP) and GRC 24 (Sorbonne Universite), 47, boulevard de l'Hopital, 75013 
      Paris, France; Laboratoire d'imagerie biomedicale (LIB), Sorbonne universite, 
      Paris, France; Global Brain Health Institute, Memory and Aging Center, University 
      of California San Francisco, San Francisco, USA. Electronic address: 
      Eleonore.bayen@gbhi.org.
LA  - eng
PT  - Journal Article
DEP - 20210924
PL  - Netherlands
TA  - Ann Phys Rehabil Med
JT  - Annals of physical and rehabilitation medicine
JID - 101502773
SB  - IM
MH  - *Agnosia/etiology
MH  - *Amyotrophic Lateral Sclerosis/complications
MH  - *Apathy
MH  - Cross-Sectional Studies
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - *Neurodegenerative Diseases
OTO - NOTNLM
OT  - ALS
OT  - Amyotrophic lateral sclerosis
OT  - Anosognosia
OT  - Apathy
OT  - Dysexecutive disorders
EDAT- 2020/10/17 06:00
MHDA- 2021/11/26 06:00
CRDT- 2020/10/16 20:11
PHST- 2020/03/12 00:00 [received]
PHST- 2020/08/09 00:00 [revised]
PHST- 2020/08/26 00:00 [accepted]
PHST- 2020/10/17 06:00 [pubmed]
PHST- 2021/11/26 06:00 [medline]
PHST- 2020/10/16 20:11 [entrez]
AID - S1877-0657(20)30171-8 [pii]
AID - 10.1016/j.rehab.2020.08.004 [doi]
PST - ppublish
SO  - Ann Phys Rehabil Med. 2021 Sep;64(5):101440. doi: 10.1016/j.rehab.2020.08.004. 
      Epub 2021 Sep 24.