|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 2 | Page : 152-153
Procerus sign revisited
Eswaradass Prasanna Venkatesan1, Balakrishnan Ramasamy1, Ramadoss Kalidoss1, Ali Raza2
1 Department of Neurology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India
2 Department of Psychiatry, Howard University Hospital, Washinton DC, USA
|Date of Web Publication||30-Jun-2016|
Eswaradass Prasanna Venkatesan
3, Vijayarahavachari Road Gandhi Road, Salem - 636 007, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Venkatesan EP, Ramasamy B, Kalidoss R, Raza A. Procerus sign revisited. Muller J Med Sci Res 2016;7:152-3
We report a 68-year-old male who presented to us with a history of recurrent backward falls of 6 months duration associated with bruises over the forehead and Colles' fracture of the right hand. He also had a history of slowness of all movements for the duration of 1 year and history of cognitive decline associated with emotional lability and emotional incontinence. On examination, he had impaired frontal executive functions, supranuclear vertical gaze palsy, and features of Parkinsonism More Details. He had more axial rigidity than appendicular rigidity. His forehead showed vertical wrinkling suggestive of procerus sign [Figure 1] and [Figure 2]. Magnetic resonance imaging of the brain showed midbrain and cerebral atrophy more in bifrontal areas and hence, a diagnosis of progressive supranuclear palsy (PSP) was made.
Procerus sign was first described in 2001 in two cases of PSP by Romano and Colosimo. It is defined as the appearance of vertical wrinkles in the glabellar region and the bridge of the nose due to dystonic contractions of the procerus muscle, associated with a reduced blinking, lid retraction, and gaze palsy. This occurs because the procerus muscle originates in the nasal bone and inserts in the skin at the center of the forehead between the eyebrows.  What it gives to the typical facial expression has been described as a "astonished," "worried," or "reptile-like" expression in PSP.
Later there was opposition to the procerus sign being solely attributed to vertical wrinkling on the forehead. Several muscles contribute to the wrinkling of the glabellar skin, giving rise to the typical expression of frowning.  These include the procerus, corrugator supercilii, and the orbicularis oculi. The procerus acts by drawing down the medial angle of the eyebrows and produces the transverse wrinkling over the bridge of the nose. Corrugator supercilii, on the other hand, draws the eyebrows medially and downward, and leads to the vertical wrinkling of the supranasal skin of the forehead.  Romano and Colosimo agreed that peculiar contractions of the upper facial muscles in PSP patients are complex and not easily explained by simple contraction of the procerus muscle.
Recently Batla et al. recommended that this should be called "vertical wrinkling of the forehead" to avoid confusion and emphasize the dystonic nature of this condition rather than contraction of a single muscle as indicated by "procerus sign."  Warabi et al. reported a case of procerus sign in corticobasal ganglionic degeneration (CBD).  PSP and CBD have characteristics that are common and are neuropathologically characterized by selective 4-repeat tau deposition. Many cases of PSP have been misdiagnosed as CBD clinically. 
Procerus sign is a useful clinical sign in PSP, which has gained importance recently in the literature as it can differentiate PSP from other Parkinson disorders. Its exact specificity or sensitivity is unknown. It can also occur in CBD. The mechanism for vertical wrinkling is not known.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Romano S, Colosimo C. Procerus sign in progressive supranuclear palsy. Neurology 2001;57:1928.
Moudgil SS. Procerus sign in progressive supranuclear palsy. Neurology 2002;58:1866-7.
Macdonald MR, Spiegel JH, Raven RB, Kabaker SS, Maas CS. An anatomical approach to glabellar rhytids. Arch Otolaryngol Head Neck Surg 1998;124:1315-20.
Batla A, Nehru R, Vijay T. Vertical wrinkling of the forehead or procerus sign in progressive supranuclear palsy. J Neurol Sci 2010;298:148-9.
Shibasaki Warabi Y, Nagao M, Bandoh M, Kanda T, Hirai S. Procerus sign in corticobasal degeneration. Intern Med 2002;41:1217-8.
Houlden H, Baker M, Morris HR, MacDonald N, Pickering-Brown S, Adamson J, et al
. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology 2001;56:1702-6.
[Figure 1], [Figure 2]