Department of Neurology, San Camillo Hospital IRCCS, Venice, ItalyItaly
Multiple Sclerosis Center, Wayne State University School of Medicine, Detroit, MI, USAUnited States
Also available as PDF (figures and tables appear in the PDF).
Multiple Sclerosis (MS) is an autoimmune neurodegenera-tive disease that affects several people especially in North America and Canada.
Open Journal PUBLISHERS Editorial The Origin of Facial Palsy in Multiple Sclerosis 1* 2 Arianna Di Stadio, MD, PhD1*; Evanthia Bernitsas, MD2 1Department of Neurology, San Camillo Hospital IRCCS, Venice, Italy 2Multiple Sclerosis Center, Wayne State University School of Medicine, Detroit, MI, USA * *Corresponding author Arianna Di Stadio, MD, PhD Assistant Professor, Department of Neurology, San Camillo Hospital IRCCS, Venice, Italy; E-mail: ariannadistadio@hotmail.com Article information th th th Received: April 16 , 2018; Accepted: April 27 , 2018; Published: April 30 , 2018 Cite this article Di Stadio A, Bernitsas E. the origin of facial palsy in multiple sclerosis. Otolaryngol Open J. 2018; 4(1): e1-e4. doi: 10.17140/OTLOJ-4-e006 ultiple Sclerosis (MS) is an autoimmune neurodegenera- tive disease that affects several people especially in North
tries to just 0.04 per 100,000 in the low-income countries.1 Women are more affected than men (2.3-3.5:1), but in men, the disease is usually more aggressive and has a worse prog- nosis than in women.2 MS is a demyelinating disorder in which the immune system attacks the neural structures in the central nervous system (CNS)3; this mechanism is determined by a particular class of cells- Microglia- thathave been identified as responsible of the degenera- tive process. These cells, that belong to the macrophage family, have the ability to move and they can easily migrate in different part of CNS4 and due to their two different phenotypes, an aggres- sive one, called M1, that induce neurodegeneration and, a protec- tive phenotype (M2) that has neuroprotective ability3 they may be induce demyelination and regeneration in different phases of MS, as for example, in the relapsing and remitting disease. Authors report that around 7% of patients affected by MS experienced one episode of FP at least once in their life, but In the active (relapsing) stage of MS, microglia M1 in- some controversies are still open about the origin of this symptom. duces demyelination in the area of CNS where they currently are located. If the area interested is a motor one, the patient manifests How many of these FPs are central and how many may movement disorders as stiffness or motor fatigue, in case of an at- arise from a peripheral involvement of facial nerve? tack in a sensory area the sensorial perception will be the affected one.
We reviewed the literature from 1997 to 2014 and we identified from a total of 16 articles, that most of the MS patients The facial pathway, that starts in the periphery with the suffered from central facial involvement when they presented with terminal branch of the facial nerve and ends up to the M1 area of a FP (Table 1).
cortex (Figure 1), may be attacked by microglia and a demyelinat- ing process that involves one or more areas of this pathway may be Three authors, unfortunately, did not report details about symptomatic as facial palsy (FP).
the site of the lesions so we were not able to completely under- cc Copyright 2018 by Di Stadio A. This is an open-access article distributed under Creative Commons Attribution 4.0 International License (CC BY 4.0), which
Authors Year Study type Sex Facial MRI results Three lesions in the supratentorial, periventricular region.Two on the right side, one on Saleh 2016 Case Report Man yes Right side the left (sizes between 8-13 mm). In spine small lesion at the level of the right caudal pons, inferior to the facial nucleus yes Right side th 8 Lesion in the floor of 4 ventricle at the pons and two in the right cerebral white matter Jens 2015 Case Report Woman started with and cerebellum numbness Sarigul 2014 Case Report Woman yes Left side Lesions in right parietal and left fronto-parietal areas 10 73% women, 27% Lassemi 2014 Cross-Sectional 17% of sample Not available men (400 patients) 32% men, 68 % 11 yes 19% of Danesh-Sani 2013 Prospective women (500 Lesion in the brain subjects patients) Lee 2013 Case Report Man yes Right side Lesion in the midline of dorsal pons Lesions in the left Renard 2012 Case Report Woman yes Left side pontine tegmentu Uzawa 2011 Case Report Woman yes Left side Lesion in the left facial colliculus Lesions in the supra and infratentorial region of the brain (periventricular, corpus cal- Ivankovic 2011 Case Report Woman yes Right side losum, pons, cerebellum Lin 2010 Case Report Man yes Left side Lesion in the lateral walls of the lateral ventricles Soltanzadeh 2008 Prospective 41 Women, 12 men yes Not available yes Right side Kwon 2008 Case Report Woman started with Lesion in the right cerebellopontine angle involving the Roof Entry Zone (REZ) numbness Zadro 2008 Retrospective 18 not defined yes not defined 66% of the subject presented brain lesions Critchley 2004 Case Report Man yes Right side Lesions diffused in the brain Thomke 1997 Prospective 2 men,1 woman yes not defined No identify cablevision in MRI 22 25 subjects (men vs.
Lesions in the pontine tegmentum ipsilateral to the facial palsy (21 with multi nerve Fukazawa 1997 Prospective yes not defined women: 1: 2.7) involvement); no lesions in the two with pure facial palsy stand the origin of the FP.
bone study.6 In two cases (a man and a woman) the authors reported We are trying to understand the reason which, in pres- vestibular and auditory symptoms associated with the onset of fa- ence of a MS-related facial palsy, the central lesion mimics a pe- cial palsy, while another author reported a woman that showed ripheral involvement presenting with a complete involvement of only vertigo complicating his facial paralysis.
In only 1 case, as reported by Kwon et al18, the origin of Is it possible the majority of the lesions are located in the facial palsy may be directly correlated with the presence of a lesion medulla or, the lesion in the central pathway are associated with a in the roof entry zone (REZ).
peripheral involvement of facial nerve in its bone tract that is not visible in the magnetic resonance imaging (MRI)?
Overall, none of the authors reported cases of FP link- able with a peripheral demyelination process as for example an The particular anatomy of the facial pathway supports the involvement of the facial nerve external to the stylus-mastoid fora- hypothesis of the central involvement, in fact, both cerebral hemi- men, where the nerve comes out from the fallopian canal. spheres (area of motor cortex with the posterior limb of internal capsule and the corticobulbar tract) are responsible for the move- It is also important to remember that a peripheral demy- ment of the frontalis muscle, while only the contralateral cortex in- elination in MS patients is rarely described5 and the most of the nervates the lower motor neurons that drive the muscles under the authors believe that the motor disease observed in MS is related frontalis. So, due to the wide diffusion of MS lesions in the brain, to the lesion in brain, pons, and medulla more than a peripheral is very uncommon to observe the typical “central facial palsy” in involvement.
those patients (Figure 2), while is more common to observe a “pe- ripheral facial palsy”.
In case of MS-related vestibule-cochlear involvement, instead, a peripheral involvement of inner ear might happen4 in The action of the microglia supports the concept of su- fact the presence of macrophages/microglia has been identified in perior facial pathway involvement in the MS-related facial palsy, patients affected from auto-immune diseases in human temporal in fact, these cells (responsible of demyelization phenomena) are
Figure 2. Figure 2. The Image Illustrates the Differences between a Central Facial Palsy with Normal Frontalis Muscle Movement and a Peripheral Form in which all Muscles are Affected from Paralysis commonly found in the brain and they may migrate in other struc- 3. Luo C, Jian C, Liao Y, et al. The role of microglia in multi- tures, as for example the inner ear, but they are rarely identified in ple sclerosis. Neuropsychiatr Dis Treat. 2017; 13: 1661-1667. doi:
peripheral nerves when they come out from their bone tract. 10.2147/NDT.S140634 The involvement of facial nerve may be plausible in the 4. Di Stadio A, Ralli M. Inner ear involvement in multiple sclerosis: internal auditory canal (IAC) but an attack of microglia outside this An underestimated condition? Mult Scler. 2018: 1352458518750010. area is very improbable; furthermore, by reviewing the literature doi: 10.1177/1352458518750010 none of the authors reported lesion in the IAC in patients affected from FP so, we could deduct that the central lesions only, are re- 5. Misawa S, Kuwabara S, Mori M, Hayakawa S, Sawai S, Hattori sponsible for the onset of facial disease.
T. Peripheral nerve demyelination in multiple sclerosis. Clin Neuro- physiol. 2008; 119(8): 1829-1833. doi: 10.1016/j.clinph.2008.04.010 We may conclude that the central facial pathway is re- sponsible for the manifestations of MS-related facial paralysis. 6. O'Malley JT, Nadol JB Jr, McKenna MJ. Anti CD163+, Iba1+, and CD68+ Cells in the adult human inner ear: Normal distribu- tion of an unappreciated class of macrophages/microglia and im- In our opinion, additional studies are needed to evalu- plications for inflammatory otopathology in humans. Otol Neurotol. ate the prevalence of peripheral versus central involvement in MS- 2016; 37(1): 99-108.
related facial palsy, and to shed light on its pathophysiology. 7. Saleh C, Patsi O, Mataigne F, Beyenburg S. Peripheral (Seventh) CONFLICTS OF INTEREST nerve palsy and multiple sclerosis: A diagnostic dilemma - A case report. Case Rep Neurol. 2016; 8(1): 27-33. doi: 10.1159/000443681 The authors declare that they have no conflicts of interest. 8. Jens W, Ahmed A. Eight and a Half Syndrome from Multi- FUNDING ple Sclerosis. J Neurol Stroke. 2015; 3(4): 00099. doi: 10.15406/ jnsk.2015.03.00099 None.
9. Sarigül A, Akça S, Evin B, Singar E, Pınarcı EY. A case of mul-