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Year : 2022  |  Volume : 28  |  Issue : 2  |  Page : 174-177

Facial nerve palsy - A rare and underrecognized neurological manifestation of rhino-orbito-cerebral mucormycosis with potential for misdiagnosis

Department of ENT, Mahatma Gandhi University of Medical Sciences and Technology, Jaipur, Rajasthan, India

Date of Submission09-Dec-2021
Date of Acceptance04-Feb-2022
Date of Web Publication21-Sep-2022

Correspondence Address:
Dr. Priyanshi Gupta
Department of ENT, Mahatma Gandhi University of Medical Sciences and Technology, D-502, Mahima Panache, Jagatpura, Jaipur - 302 017, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/indianjotol.indianjotol_172_21

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Rhino-orbito-cerebral Mucormycosis is a rapidly aggressive disease having various clinical presentation like facial pain, nasal stuffiness, headache, retro-orbital pain, orbital swelling, ophthalmoplegia and visual loss. Multiple cranial nerves can be involved, however facial nerve palsy has been rarely reported. We aim to highlight the involvement of facial nerve as a presenting symptom. Mucormycosis should be kept as a differential in these cases for rapid diagnosis and treatment initiation to provide a good overall outcome.

Keywords: Amphotericin B, facial nerve palsy, rhino-orbito-cerebral mucormycosis

How to cite this article:
Ojha T, Gupta P, Kataria V, Sharma K. Facial nerve palsy - A rare and underrecognized neurological manifestation of rhino-orbito-cerebral mucormycosis with potential for misdiagnosis. Indian J Otol 2022;28:174-7

How to cite this URL:
Ojha T, Gupta P, Kataria V, Sharma K. Facial nerve palsy - A rare and underrecognized neurological manifestation of rhino-orbito-cerebral mucormycosis with potential for misdiagnosis. Indian J Otol [serial online] 2022 [cited 2022 Sep 25];28:174-7. Available from: https://www.indianjotol.org/text.asp?2022/28/2/174/356447

  Introduction Top

Mucormycosis is a rare but aggressive opportunistic fungal infection. The genera most commonly responsible are Mucor or Rhizopus spp.[1] Mucormycosis is an invasive fungal infection first described by Paulltauf A. in 1885. The incidence of mucormycosis reported in literature is approximately 1.7 cases per 1,000,000 populations per year.[2] It affects mainly the immunocompromised patients, especially those with uncontrolled diabetes mellitus. In addition, patients with organ transplant or hematopoietic stem-cell transplant, neutropenia, or malignancy are at high risk.[3] The causative agents of mucormycosis are the filamentous fungi of the Mucoraceae family, of the order Mucorales, subphylum Mucoromycotina.[4] On the basis of clinical presentation, six manifestations of the disease can be described: Rhino-orbito-cerebral (30%–50%), pulmonary, cutaneous, gastrointestinal, disseminated, and localized infections not otherwise belonging to the previous categories.[5] The “second wave” of the COVID epidemic in India due to the delta variant (B.1.617.2) lead to indiscriminate use of steroids, antibiotics, and uncontrolled diabetes which prompted a sudden increase in cases of mucormycosis. Early diagnosis is very important factor not only to cure the disease completely but also to improve the outcome by reducing the need for extensive surgical resection. Typical presentation of rhino-orbito-cerebral mucormycosis (ROCM) includes nasal stuffiness, headache, retro-orbital pain, orbital swelling, ophthalmoplegia, and visual loss in diabetic patients.

Here, we present an unusual case of a patient who presented with unilateral right lower motor neuron facial nerve palsy. On further evaluation, he was diagnosed as rhino-orbito-cerebral mucormycosis. The purpose of this case report is to acquaint the clinicians about this uncommon presentation of ROCM to prompt early diagnosis and treatment which directly affects the outcome in terms of survival.

  Case Report Top

A 63-year-old male patient reported to us with the chief complaint of pain and swelling in the right side of face and right eye along with inability to close the right eye, drooling of saliva, drooping of the corner of the mouth, the absence of the wrinkles on the right side of forehead (suggestive of lower motor neuron lesion), proptosis, epiphora, and blurring of vision. (The patient had previously consulted a local practitioner who initially treated the patient in the line of Bell's palsy with steroids and antivirals, but the pain and swelling progressed which prompted his diagnosis toward herpes infection for which he prescribed treatment, but even after that, when the swelling and pain progressed to involve the right eye with proptosis, epiphora, redness, and blurring of vision, he was referred to an ENT specialist) [Figure 1]a. Examination of nose by nasal endoscopy showed black eschar on the right middle turbinate and purulent discharge from the right middle meatus. Examination of ear showed bilateral intact dull lusterless tympanic membrane. Examination of eye showed proptosis, B/L pupil reacting to light, vision in right eye – 6/12 and in left eye – 6/9, rest of the ophthalmological examination was normal. Neurological examination showed right side lower motor neuron facial nerve palsy, grade V (House–Brackmann facial grading system), and rest of the cranial nerve examination was normal. Routine blood investigations revealed leukocytosis with neutrophilia (ANC-10126) and lymphopenia (ALC-464). Random blood sugar was 357mg/dl, fasting blood sugar – 255mg/dl, and HbA1C – 10.6%. Erythrocyte sedimentation rate was 120mm/Hr. Rest of the investigations was within normal limits. Noncontrast CT scan of the brain, nose, and paranasal sinuses was ordered which showed soft-tissue mucosal thickening in bilateral maxillary and ethmoid sinuses with mild cerebral atrophy [Figure 2]a. Gadolinium contrast-enhanced magnetic resonance imaging (MRI) was also done which suggested mucosal enhancement of all sinuses on both sides with right premaxillary, infratemporal, right inferior extraconal space of the orbit with proptosis of eye globe, and irregularities of bony walls suggestive of invasive fungal sinusitis with adjacent cellulitis and intraorbital extension [Figure 3]a. Preoperative biopsy was taken and sent for HPE which was positive for mucormycosis. On high index of clinical suspicion, injection liposomal amphotericin B (5 mg/kg/day) with tablet posaconazole 300 mg once a day, injection insulin according to the sugar levels, and regular supportive treatment was started. The patient was taken for endoscopic endonasal sinus debridement, and the disease was found involving all the sinuses on right side along with premaxillary space, pterygopalatine, and infratemporal fossa. Right orbit appeared normal. On left side, there was edematous mucosa in maxillary sinus. Biopsy was sent separately from different areas which later revealed clusters of fungal hyphae, broad, and irregular wide branch angle infiltrating the fibrovascular and necrotic tissue, suggestive of mucormycosis [Figure 4]a, [Figure 4]b, [Figure 4]c. The patient was kept for 45 days postsurgery and continued on AmB when his facial palsy improved to grade III. Postoperative computed tomography scan and MRI on 2-month follow-up shows no remanent disease with improvement in facial palsy to grade II along with normal vision in both eyes [Figure 1]b, [Figure 2]b and [Figure 3]b. The patient is on regular physiotherapy and oral antifungal medication.
Figure 1: (a) Preoperative facial nerve palsy Grade V. (b) 2 month postoperative facial nerve palsy improved Grade II

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Figure 2: (a) Preoperative NCCT showing mucosal thickening in maxillary and ethnocide sinuses heterogeneous opacities with irregular bony outlines, suggestive of invasive fungal sinusitis. (b) Postoperative NCCT showing operated disease-free paranasal sinuses and nasal cavities

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Figure 3: (a) Preoperative Gadolinium enhanced magnetic resonance imaging showing enhancing mucosal thickening all sinuses with restricted diffusion edema of soft tissues in pre maxillary right infratemporal fossa and inferior extra conal space and irregularities of bony walls. (b) Postoperative contract-enhanced magnetic resonance imaging showing healthy disease free nasal cavities and sinuses

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Figure 4: (a) 10% KOH of the tissues showing broad aesptate hyphae. (b) Lactophenol cotton blue mount of the culture (magnification X400) showing broad aesptate hyphae with champagne glass appearance of the sporangial sac. (c) culture grown on sabouraud's agar showing white cottony growth almost filling the culture tube

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  Discussion Top

The presentation of mucormycosis as facial palsy has been reported in few isolated cases. The involvement of various tissues in rhino-cerebral area can lead to clinical presentation mimicking other conditions such as cerebrovascular accidents.[6] The frequency of facial paralysis in ROCM is 11%. The exact mechanism of involvement is unknown with no definite pathophysiology.

Involvement of pterygopalatine fossa has been reported by some authors as a route of spread of mucormycosis to the facial nerve. Pterygopalatine fossa is additionally considered to be a reservoir of mucor from where it spreads to retro global space of orbit and infratemporal space.[7] Hence, the infection reaching the pterygopalatine fossa can spread to the inferior orbital fissure, orbital apex, and infratemporal fossa.

Recent studies have demonstrated the spread of Mucorales species along peripheral nerves.[6] The juxtaposition of the pterygopalatine fossa and presence of the many connections of vascular and neural tissue make it a possible route of perineural invasion to the cranial tissues.[6] Perineural extension through Vidian nerve is also one of the possibilities.[6] The Vidian nerve is a continuation of greater superficial petrosal nerve. An isolated involvement of the intracanalicular facial nerve within the temporal bone can occur along the nerve sheath of this nerve.[7] This explains facial nerve affection without involvement of any other cranial nerves as seen in our study.

Facial nerve may also be affected by direct spread through the  Eustachian tube More Details or through the vascular channels into middle ear.[8] This fungus is reported to be a normal commensal within the nose and nasopharynx of healthy individuals and may get opportunistic in immunocompromised diabetic state.

Another reason of involvement of facial nerve palsy can be the pathology of resistance arteries in diabetic patients which may cause edema and localize facial nerve ischemia. This would compromise the blood supply to the nerve leading to palsy.[9] In most of the diabetic cases with facial palsy, the chorda tympani is not affected, and hence, the taste sensation remains unaffected. This occurs because the bifurcation of chorda tympani nerve is proximal to the site of lesion.[10] Unlike in cases of facial nerve palsy with viral or any other etiology, where the entire facial nerve is involved. Facial nerve palsy can also be incidental and may be idiopathic as in Bell's palsy.[8]

Spread of mucormycosis can be identified using contrast-enhanced MRI. Facial nerve involvement may be diagnosed by widening of pterygopalatine fossa, seen on MRI. Perineural invasion has been documented using contrast-enhanced MRI.[11] However, imaging studies carry their own limitations and tissue culture is the gold standard for diagnosis of mucormycosis. Diagnosis is confirmed histologically by detection of aseptate hyphae with right-angled branching which is pathognomonic of mucormycosis.[11] The prognostic value of facial nerve involvement in cases of ROCM is yet to be established.

  Conclusion Top

Unilateral lower motor neuron facial nerve palsy usually prompts the diagnosis of Bell's palsy and sometimes any neurological deficit but almost never of mucormycosis. Thorough ENT examination, keeping mucormycosis as a differential will bring us to a rapid diagnosis and treatment initiation which is of paramount value for overall good outcome and survival.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Khan AR, Khan MU, Ullah S. Rhinocerebral mucormycosis. J Coll Physicians Surg Pak 2002;12:639-41.  Back to cited text no. 1
Bouza E, Muñoz P, Guinea J. Mucormycosis: An emerging disease? Clin Microbiol Infect 2006;12:7-23.  Back to cited text no. 2
Mane RS, Watve JK, Mohite AA, Patil BC. Rhinocerebral mucormycosis: A deadly disease on the rise. Indian J Otolaryngol Head Neck Surg 2007;59:112-5.  Back to cited text no. 3
Hibbett DS, Binder M, Bischoff JF, Blackwell M, Cannon PF, Eriksson OE, et al. A higher-level phylogenetic classification of the Fungi. Mycol Res 2007;111:509-47.  Back to cited text no. 4
Spellberg B, Edwards J Jr., Ibrahim A. Novel perspectives on mucormycosis: Pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18:556-69.  Back to cited text no. 5
Aloosi SN. Facial nerve palsy as frequent presentation in patient with rhinocerebral mucormycosis. J Oral Dent Res 2018;5:55-69. Available from: https://www.iasj.net/iasj/article/145763. [Last accessed on 2021 Dec 03].  Back to cited text no. 6
Hosseini SM, Borghei P. Rhinocerebral mucormycosis: Pathways of spread. Eur Arch Otorhinolaryngol 2005;262:932-8.  Back to cited text no. 7
Bakshi SS. An unusual cause for facial nerve palsy: Mucormycosis. Int J Diabetes Dev Ctries 2016;36:385-8.  Back to cited text no. 8
Shekar V, Sikander J, Rangdhol V, Naidu M. Facial nerve paralysis: A case report of rare complication in uncontrolled diabetic patient with mucormycosis. J Nat Sci Biol Med 2015;6:226-8.  Back to cited text no. 9
Pecket P, Schattner A. Concurrent Bell's palsy and diabetes mellitus: A diabetic mononeuropathy? J Neurol Neurosurg Psychiatry 1982;45:652-5.  Back to cited text no. 10
Safdar A, Dommers MP Jr., Talwani R, Thompson CR. Intracranial perineural extension of invasive mycosis: A novel mechanism of disease propagation by Aspergillus fumigatus. Clin Infect Dis 2002;35:e50-3.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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