Eyelid retraction in dementia with Lewy bodies and Parkinson’s disease

Abstract

Dear Sirs, Eyelid retraction (ELR), indicates widening of the palpebral opening with white parts of the sclera appearing above and, often, below the limbus. ELR was indicated with different and controversial eponyms including Graefe’s, pseudo-Graefe’s, Stellwag’s, or Dalrymple’s sign [1], which were, however, originally ascribed to endocrinological conditions [1, 2]. Dalrymple’s, Stellwag’s and Graefe’s signs are typical of Grave’s disease: the first indicating retraction of the upper eyelid, causing abnormal wideness of the palpebral fissure, the second infrequent or incomplete blinking, the third tardy or jerky downward movement of the upper eyelids when the gaze is directed downward. Pseudo-Graefe’s (or Fuch’s) sign is characterized by elevation or retraction of the upper eyelid when the eye is looking downwards. Collier’s sign is the only accepted synonymous of neurogenic eyelid retraction [3]: it is defined as unilateral or bilateral lid retraction due to midbrain lesion, occurring at any age. ELR was initially reported as an early sign of typical and atypical parkinsonisms [4, 5]. With the more recent identification of progressive supranuclear palsy (PSP) [6], ELR became a mark sign of the disease or a red flag signalling possible diagnosis, although further ocular and extra-ocular neurological signs should accompany it, like ‘‘combined activation of frontalis/superior orbicularis and platysma muscles, giving a frightened expression’’ [6], vertical supranuclear gaze palsy, blepharospasm and blepharocolysis (apraxia of eyelid opening/closing). Despite this emphasis on ELR in PSP, ELR may be present in Parkinson’s disease (PD), as it was observed in patients with autoptically detected Lewy bodies [7]. Yet it is not clear whether ELR occurs early or late during the disease course, whether it is concealed or reduced by dopaminergic treatments, or whether it is present in different forms of atypical parkinsonism other than PSP. With the present report we evidence that ELR unaccompanied by other ocular motor signs is consistently observed in early phase of dementia with Lewy bodies (DLB), is more frequent than in PD and is responsive to dopaminergic treatment. Electronic supplementary material The online version of this article (doi:10.1007/s00415-011-5942-z) contains supplementary material, which is available to authorized users.