PMID- 36136731
OWN - NLM
STAT- MEDLINE
DCOM- 20230515
LR  - 20230830
IS  - 1537-2677 (Electronic)
IS  - 0740-9303 (Linking)
VI  - 39
IP  - 3
DP  - 2023 May-Jun 01
TI  - Debunking the Puzzle of Eyelid Apraxia: The Muscle of Riolan Hypothesis.
PG  - 211-220
LID - 10.1097/IOP.0000000000002291 [doi]
AB  - PURPOSE: Apraxia of eyelid opening (AEO) has been defined by the presence of an 
      intermittent nonparalytic bilateral loss of the volitional ability to open the 
      eyes or to maintain the eyelids in a sustained elevated position. It is not known 
      whether the condition represents an apraxia, a dystonia, or a freezing 
      phenomenon, and several different nomenclatorial terms have been suggested for 
      this condition including the so-called AEO (scAEO), blepahrocolysis, focal eyelid 
      dystonia, and so on. The primary goal of this review is to attempt to clarify the 
      pathogenetic mechanisms underlying scAEO as a clinical phenomenon. This review 
      also addresses the issue of whether scAEO is part of the spectrum of 
      blepharospasm (BSP) which includes BSP, dystonic blinks and other dystonic eyelid 
      conditions, or whether it is a separate phenomenologically heterogeneous disease 
      with clinical features that merely overlap with BSP. METHODS: A literature review 
      was conducted in PubMed, MEDLINE, PubMed Central (PMC), NCBI Bookshelf, and 
      Embase for several related keywords including the terms "apraxia of eyelid 
      opening," "pretarsal blepharospasm," "blepharocolysis," "eyelid freezing," 
      "eyelid akinesia," "levator inhibition," "blepharospasm-plus," as well as 
      "blepharospasm." The clinical findings in patients with scAEO who fulfilled the 
      classic diagnostic criteria of the disease that were originally set by Lepore and 
      Duvoisin were included, while patients with isolated blepharospasm or dystonic 
      blinks (DB) were excluded. In addition, electromyographic (EMG) studies in 
      patients with scAEO were reviewed in detail with special emphasis on studies that 
      performed synchronous EMG recordings both from the levator muscle (LPS) and the 
      pretarsal orbicularis oculi muscle (OO). RESULTS: The apraxia designation is 
      clearly a misnomer. Although scAEO behaves clinically as a hypotonic freezing 
      phenomenon, it also shares several cardinal features with focal dystonias. The 
      authors broadly categorized the EMG data into 3 different patterns. The first 
      pattern (n = 26/94 [27.6%]) was predominantly associated with involuntary 
      discharges in the OO muscle and has been termed pretarsal blepharospasm (ptBSP). 
      The commonest pattern was pattern no. 2 (n = 53/94 [56.38%]), which was 
      characterized by involuntary discharges in the OO muscle, together with a 
      disturbed reciprocal innervation of the antagonist levator muscle and is dubbed 
      disturbed reciprocal innervation (DRI). This EMG pattern is difficult to discern 
      from the first pattern. Pattern no. 3 (n = 15/94 [15.9%]) is characterized by an 
      isolated levator palpebrae inhibition (ILPI). This levator silence was observed 
      alone without EMG evidence of contractions in the pretarsal orbicularis or a 
      disturbed reciprocal relation of both muscles. CONCLUSION: EMG evidence shows 
      that the great majority (84%) of patients show a dystonic pattern, whereas ILPI 
      (16%) does not fit the dystonic spectrum. The authors propose that a spasmodic 
      contraction of the muscle of Riolan may be the etiological basis for levator 
      inhibition in patients with ILPI. If this is true, all the 3 EMG patterns 
      observed in scAEO patients (ptBSP, DRI, and ILPI) would represent an atypical 
      form of BSP. The authors suggest coining the terms Riolan muscle BSP ( rmBSP ) 
      for ILPI, and the term atypical focal eyelid dystonia ( AFED ) instead of the 
      term scAEO, as both terms holistically encompass both the clinical and EMG data 
      and concur with the authors' theorem.
CI  - Copyright (c) 2022 The American Society of Ophthalmic Plastic and Reconstructive 
      Surgery, Inc.
FAU - Tawfik, Hatem A
AU  - Tawfik HA
AD  - Department of Ophthalmology, Ain Shams University, Cairo, Egypt.
FAU - Dutton, Jonathan J
AU  - Dutton JJ
AD  - Department of Ophthalmology, University of North Carolina School of Medicine, 
      Chapel Hill, North Carolina, U.S.A.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20220922
PL  - United States
TA  - Ophthalmic Plast Reconstr Surg
JT  - Ophthalmic plastic and reconstructive surgery
JID - 8508431
SB  - IM
MH  - Humans
MH  - *Blepharospasm/diagnosis
MH  - *Eyelid Diseases/etiology
MH  - *Dystonia/complications
MH  - Facial Muscles
MH  - *Apraxias/diagnosis/complications
COIS- The authors have no financial or conflicts of interest to disclose.
EDAT- 2022/09/23 06:00
MHDA- 2023/05/15 06:42
CRDT- 2022/09/22 12:23
PHST- 2023/05/15 06:42 [medline]
PHST- 2022/09/23 06:00 [pubmed]
PHST- 2022/09/22 12:23 [entrez]
AID - 00002341-202305000-00003 [pii]
AID - 10.1097/IOP.0000000000002291 [doi]
PST - ppublish
SO  - Ophthalmic Plast Reconstr Surg. 2023 May-Jun 01;39(3):211-220. doi: 
      10.1097/IOP.0000000000002291. Epub 2022 Sep 22.