PMID- 28945811
OWN - NLM
STAT- MEDLINE
DCOM- 20171023
LR  - 20220310
IS  - 1932-6203 (Electronic)
IS  - 1932-6203 (Linking)
VI  - 12
IP  - 9
DP  - 2017
TI  - Retinal thinning in amyotrophic lateral sclerosis patients without ophthalmic 
      disease.
PG  - e0185242
LID - 10.1371/journal.pone.0185242 [doi]
LID - e0185242
AB  - IMPORTANCE: Amyotrophic lateral sclerosis (ALS) is a fatal, rapidly progressive 
      neurodegenerative disease that primarily affects motor neurons. Recently, three 
      causative genes have been implicated in both ALS and glaucoma. However, it is 
      still uncertain whether patients with ALS have neurodegeneration in their 
      retinas. If so, retinal thickness measurements might be a useful biomarker for 
      ALS progression. Previous work in this area has been inconclusive, as it has not 
      taken into account the effect of ophthalmic diseases on retinal thinning. 
      OBJECTIVE: To determine whether there are differences in retinal neurons in ALS 
      patients utilizing spectral-domain optical coherence tomography (SD-OCT). We 
      tested the hypothesis that ALS patients exhibit retinal neurodegeneration that is 
      not associated with ophthalmic diseases. DESIGN, SETTINGS AND PARTICIPANTS: 
      Observational, comparative, cross-sectional study performed on patients recruited 
      from the Duke University Medical Center ALS clinic. Patients underwent a 
      comprehensive ophthalmologic examination to rule out ocular pathology. 21 
      patients met inclusion criteria. Two eyes with ocular pathology were excluded, 
      leading to a total of 40 eyes of 21 patients included in the study. Retinal 
      neurodegeneration was assessed by retinal nerve fiber layer (RNFL) thickness 
      measurement using SD-OCT (Spectralis; Heidelberg Engineering). MAIN OUTCOMES AND 
      MEASURES: ALS disease severity, determined through the ALS Functional Rating 
      Scale (ALSFRS-R); mean and six sector RNFL thickness values compared to 
      age-adjusted values in the normative database provided by Heidelberg Engineering; 
      RNFL thickness correlation with ALSFRS-R, ALSFRS-R progression rate, forced vital 
      capacity (FVC), and visual acuity. RESULTS: ALSFRS-R mean score was 30+/-10. Mean 
      RNFL thickness in ALS patients was 88.95 +/- 10.8 microns, significantly thinner 
      than values in the normative database (95.81 +/- 0.8). These RNFL thickness 
      values did not demonstrate correlation to ALSFRS-R score, ALSFRS-R progression 
      rate, FVC, intraocular pressure, or visual acuity. CONCLUSIONS: Using SD-OCT, our 
      study shows that ALS patients without ocular pathology exhibit thinned retinal 
      layers. Future studies are warranted to clarify the clinical relationship between 
      retinal thinning and motor neuron loss in ALS.
FAU - Mukherjee, Nisha
AU  - Mukherjee N
AD  - Department of Ophthalmology, Duke University Medical Center, Durham, North 
      Carolina, United States of America.
FAU - McBurney-Lin, Shan
AU  - McBurney-Lin S
AUID- ORCID: 0000-0001-9829-5748
AD  - Duke University School of Medicine, Durham, North Carolina, United States of 
      America.
FAU - Kuo, Anthony
AU  - Kuo A
AD  - Department of Ophthalmology, Duke University Medical Center, Durham, North 
      Carolina, United States of America.
FAU - Bedlack, Richard
AU  - Bedlack R
AD  - Duke ALS Clinic, Duke University Medical Center, Durham, North Carolina, United 
      States of America.
FAU - Tseng, Henry
AU  - Tseng H
AD  - Department of Ophthalmology, Duke University Medical Center, Durham, North 
      Carolina, United States of America.
LA  - eng
PT  - Journal Article
PT  - Observational Study
DEP - 20170925
PL  - United States
TA  - PLoS One
JT  - PloS one
JID - 101285081
SB  - IM
MH  - Adult
MH  - Aged
MH  - Amyotrophic Lateral Sclerosis/genetics/*pathology/physiopathology
MH  - Cross-Sectional Studies
MH  - Disease Progression
MH  - Female
MH  - Humans
MH  - Intraocular Pressure
MH  - Male
MH  - Middle Aged
MH  - Nerve Degeneration/pathology
MH  - Nerve Fibers/pathology
MH  - Retina/*pathology
MH  - Retinal Neurons/pathology
MH  - Tomography, Optical Coherence/methods
MH  - Visual Acuity
PMC - PMC5612691
COIS- Competing Interests: The authors have declared that no competing interests exist.
EDAT- 2017/09/26 06:00
MHDA- 2017/10/24 06:00
PMCR- 2017/09/25
CRDT- 2017/09/26 06:00
PHST- 2017/03/16 00:00 [received]
PHST- 2017/09/09 00:00 [accepted]
PHST- 2017/09/26 06:00 [entrez]
PHST- 2017/09/26 06:00 [pubmed]
PHST- 2017/10/24 06:00 [medline]
PHST- 2017/09/25 00:00 [pmc-release]
AID - PONE-D-17-10479 [pii]
AID - 10.1371/journal.pone.0185242 [doi]
PST - epublish
SO  - PLoS One. 2017 Sep 25;12(9):e0185242. doi: 10.1371/journal.pone.0185242. 
      eCollection 2017.