PMID- 32335868
OWN - NLM
STAT- MEDLINE
DCOM- 20220222
LR  - 20220222
IS  - 2240-2993 (Electronic)
IS  - 0300-9009 (Linking)
VI  - 121
IP  - 6
DP  - 2021 Dec
TI  - Quality of life in hereditary neuropathy with liability to pressure palsies is as 
      impaired as in Charcot-Marie-Tooth disease type 1A.
PG  - 1481-1486
LID - 10.1007/s13760-020-01355-w [doi]
AB  - To date, only one study assessed quality of life (QoL) in patients with 
      hereditary neuropathy with liability to pressure palsies (HNPP). We aimed to fill 
      in this gap by investigating QoL in a cohort of patients with HNPP compared to 
      Charcot-Marie-Tooth type 1A (CMT1A) patients, as well as to analyze 
      sociodemographic and clinical features associated with QoL in HNPP. Eighteen 
      genetically confirmed HNPP patients were age-and gender-matched with 18 CMT1A 
      patients. SF-36 questionnaire was used to assess QoL. Medical Research Council 
      (MRC) Sum Score, CMT Neuropathy Score (CMTNS), Overall Neuropathy Limitation 
      Scale Score (ONLS), Falls Efficacy Score (FES), Visual Analog Pain Scale, Beck 
      Depression Inventory (BDI) and Fatigue Severity Scale (FSS) were also used in our 
      study. Although HNPP patients were less clinically impaired, no difference was 
      observed in these two cohorts regarding SF-36 scores. Worse QoL in HNPP patients 
      was associated with lower education (p < 0.01), physical work (p < 0.05), higher 
      number of clinically affected nerves during the disease course (p < 0.01), worse 
      MRC-SS score (p < 0.01), worse ONLS score (p < 0.01), and with more severe pain 
      (p < 0.01), depression (p < 0.01), and fatigue (p < 0.01). Worse pain at the 
      moment of testing appeared as a significant independent predictor of worse QoL in 
      HNPP patients (beta = - 0.93, p < 0.001). QoL was similarly impaired in patients 
      with HNPP and patients with CMT1A. We identified different factors associated 
      with QoL in HNPP, and many of these factors are amenable to treatment which is of 
      special interest in these still incurable disease.
CI  - (c) 2020. Belgian Neurological Society.
FAU - Bjelica, Bogdan
AU  - Bjelica B
AD  - Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of 
      Belgrade, 6, Dr Subotic Street, 11 000, Belgrade, Serbia.
FAU - Peric, Stojan
AU  - Peric S
AD  - Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of 
      Belgrade, 6, Dr Subotic Street, 11 000, Belgrade, Serbia. stojanperic@gmail.com.
FAU - Bozovic, Ivo
AU  - Bozovic I
AD  - Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of 
      Belgrade, 6, Dr Subotic Street, 11 000, Belgrade, Serbia.
FAU - Jankovic, Milena
AU  - Jankovic M
AD  - Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of 
      Belgrade, 6, Dr Subotic Street, 11 000, Belgrade, Serbia.
FAU - Brankovic, Marija
AU  - Brankovic M
AD  - Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of 
      Belgrade, 6, Dr Subotic Street, 11 000, Belgrade, Serbia.
FAU - Palibrk, Aleksa
AU  - Palibrk A
AD  - Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of 
      Belgrade, 6, Dr Subotic Street, 11 000, Belgrade, Serbia.
FAU - Rakocevic Stojanovic, Vidosava
AU  - Rakocevic Stojanovic V
AD  - Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of 
      Belgrade, 6, Dr Subotic Street, 11 000, Belgrade, Serbia.
LA  - eng
GR  - 175083/Ministarstvo Prosvete, Nauke i Tehnoloskog Razvoja/
PT  - Journal Article
DEP - 20200425
PL  - Italy
TA  - Acta Neurol Belg
JT  - Acta neurologica Belgica
JID - 0247035
RN  - Tomaculous neuropathy
SB  - IM
MH  - Adult
MH  - Arthrogryposis/diagnosis/*epidemiology/*psychology
MH  - Charcot-Marie-Tooth Disease/diagnosis/*epidemiology/*psychology
MH  - Cohort Studies
MH  - Cross-Sectional Studies
MH  - Female
MH  - Hereditary Sensory and Motor Neuropathy/diagnosis/*epidemiology/*psychology
MH  - Humans
MH  - Longitudinal Studies
MH  - Male
MH  - Middle Aged
MH  - Quality of Life/*psychology
MH  - Serbia/epidemiology
OTO - NOTNLM
OT  - Ability
OT  - Charcot-Marie-Tooth type 1A (CMT1A)
OT  - Hereditary neuropathy with liability to pressure palsies (HNPP)
OT  - Impairment
OT  - Quality of life
EDAT- 2020/04/27 06:00
MHDA- 2022/02/23 06:00
CRDT- 2020/04/27 06:00
PHST- 2020/01/28 00:00 [received]
PHST- 2020/04/06 00:00 [accepted]
PHST- 2020/04/27 06:00 [pubmed]
PHST- 2022/02/23 06:00 [medline]
PHST- 2020/04/27 06:00 [entrez]
AID - 10.1007/s13760-020-01355-w [pii]
AID - 10.1007/s13760-020-01355-w [doi]
PST - ppublish
SO  - Acta Neurol Belg. 2021 Dec;121(6):1481-1486. doi: 10.1007/s13760-020-01355-w. 
      Epub 2020 Apr 25.