|Year : 2017 | Volume
| Issue : 1 | Page : 112-119
Bilateral facial palsy and neurosarcoidosis – An approach to a difficult diagnosis
Mark W Fegley1, Lauren E Stone2, Rodrigo Duarte-Chavez3, Amitoj Singh3, Santo Longo4, Sudip Nanda3
1 Department of Family Medicine, St. Luke's University Health Network, Bethlehem, Pennsylvania 18015, USA
2 Lewis Katz School of Medicine at Temple University, 3500 N Broad St, Philadelphia, PA 19140, USA
3 Department of Internal Medicine, St. Luke's University Health Network, Bethlehem, Pennsylvania 18015, USA
4 Department of Pathology, St. Luke's University Health Network, Bethlehem, Pennsylvania 18015, USA
|Date of Web Publication||7-Jul-2017|
Department of Internal Medicine, St. Luke's University Health Network, 801, Ostrum Street, Bethlehem, Pennsylvania-18015
Source of Support: None, Conflict of Interest: None
We present a 71-year-old Caucasian female who presented with right upper quadrant abdominal pain and flank pain. As an inpatient, she subsequently developed bilateral facial palsy. After extensive workup, she was diagnosed with neurosarcoidosis. We present an algorithmic approach to diagnosing facial palsy and specific consideration when bilateral. Bilateral facial palsy accounts for <2% of all facial palsies. Unlike unilateral facial palsy, bilateral facial palsy is often associated with significant underlying disease. This systematic approach was helpful in providing the diagnosis of neurosarcoidosis and review of the disease helped confirm the diagnosis and direct further workup.
The following core competencies are addressed in this article: Patient care, Medical knowledge.
Keywords: Acute facial palsy, bilateral facial palsy, facial palsy, neurosarcoidosis, nontraumatic facial palsy
|How to cite this article:|
Fegley MW, Stone LE, Duarte-Chavez R, Singh A, Longo S, Nanda S. Bilateral facial palsy and neurosarcoidosis – An approach to a difficult diagnosis. Int J Acad Med 2017;3:112-9
|How to cite this URL:|
Fegley MW, Stone LE, Duarte-Chavez R, Singh A, Longo S, Nanda S. Bilateral facial palsy and neurosarcoidosis – An approach to a difficult diagnosis. Int J Acad Med [serial online] 2017 [cited 2019 Jul 15];3:112-9. Available from: http://www.ijam-web.org/text.asp?2017/3/1/112/209850
| Introduction|| |
Facial palsy has a wide differential diagnosis and is grossly divided into the following categories: Mechanism either traumatic or nontraumatic, time either acute or delayed, and laterality either unilateral or bilateral. Knowing the mechanism, time and laterality are useful for considering a differential diagnosis and further workup.
| Case Report|| |
A 71-year-old Caucasian female presented to our service for acute right-sided abdominal pain and flank pain. On admission, our workup included a computed tomography (CT) scan of the abdomen which revealed diffuse subcentimeter low-density nodules in the liver and spleen. Suspecting intestinal pathology a colonoscopy was performed which revealed submucosal lymphoid aggregation. Our initial working diagnosis was Chron's disease. As an inpatient, 5–6 days after the onset of abdominal pain she developed progressive left-sided facial weakness, drooling of saliva from corner of the mouth, and an inability to close her left eye. Subsequently, the same constellation of findings developed on the right side of the face within 3–4 days [Figure 1]. The ENT examination was normal. Other cranial nerves were normal.
|Figure 1: A 71-year-old Caucasian female with bilateral facial palsy on physical exam|
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Her complete blood counts, erythrocyte sedimentation rate, serum chemistries, serum protein electrophoresis, delta-aminolevulinic acid, 24 h urine porphobilinogen, angiotensin converting enzyme levels were normal. Tests for autoantibodies including ANA, antineutrophil cytoplasm antibodies, SMA, anticardiolipin antibodies, Ro-SSA, La-SSB, and acetylcholine receptor antibodies were negative. The purified protein derivative was negative. Serologies for herpes simplex, varicella zoster, Epstein–Barr, coxsackie, HIV, mycoplasma, and Borrelia burgdorferi i were negative. The cerebral spinal fluid (CSF) examination was normal. CT scan of the chest was negative. Magnetic resonance imaging (MRI) of the brain was normal. A liver biopsy revealed noncaseating granulomas [Figure 2].
|Figure 2: Liver biopsy (×200) with hematoxylin and eosin stain shows a noncaseating granuloma|
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| Discussion|| |
Facial nerve palsy can be unilateral or bilateral. Bilateral facial palsy is defined as facial palsy involving both sides of the face within 28 days. Facial palsy is diagnosed clinically and can be rated in severity from I to VI using the House-Brackmann Classification scale which has been modified through the years and was updated to the Facial Nerve Grading System 2.0[Figure 3]., Both of these scales provide insight into the prognostic outcome. The mechanisms of facial palsy can occur either via traumatic or nontraumatic and further subdivided by time as acute or delayed. Congenital palsy is also known to occur. [Figure 4] and [Table 1] show anatomic course, full differential diagnosis, and radiologic workup.,,,, Diagnostic imaging studies may include CT, MRI, ultrasound, or diffusion tensor tractography [Table 1]. Additional diagnostic studies may include lumbar puncture if clinician suspects meningitis, vasculitis, multiple sclerosis, or meningeal carcinomatosis. Audiometric testing will determine type and degree of hearing loss which can aid in diagnosis. Electroneuronography may be performed to grade recovery of the nerve.
|Figure 3: Facial Nerve Grading Scale 2.0, adaptation of Vrabec. Grade I is normal score of 4, Grade II score of 5–9, Grade III score of 10–14, Grade IV score 15–19, Grade V 20–23, Grade VI–24 or more|
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|Figure 4: Anatomy and course of the facial nerve. Adaptation from practical neurology. The temporal branch of facial nerve receives input from both cerebral hemispheres. In an upper motor neuron injury, eyebrow raising is spared. Lesions at the level of pons or below will affect temporal branch and hence eyebrow raising. Please refer to Table 2 for a complete list of differential diagnosis|
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Unilateral facial palsy has an incidence of 20–25 per 100,000 population. Bilateral facial palsy is far less common, accounting for 0.3–2% of all cases of facial palsy. Bilateral facial palsy is an ominous sign and further workup should be strongly considered. Bell's palsy accounts for approximately 23% of all bilateral facial palsies., Other common causes include sarcoidosis, Lyme's disease, Miller Fisher syndrome, leukemia, meningitis, syphilis, and infectious mononucleosis [Table 1].
Bilateral facial palsy is difficult to diagnose as facial symmetry may be retained. Lesions of the cortex or corticobulbar fibers produce contralateral central-type facial palsy and hemiplegia. Peripheral facial palsy causes weakness of all facial muscles and results from the lesion of facial nerve nucleus or the nerve. For bilateral lower motor neuron palsy (peripheral) all voluntary movement fails, the angles of the mouth sag and whites of the eyes are seen with attempt to close both eyes. In bilateral upper motor neuron palsy (central voluntary) blinking is not affected, the mouth cannot be moved voluntarily but moves well during spontaneous conversation. In bilateral upper motor neuron emotional palsy (central involuntary), there are mask-like facies without normal expression, and weakness is more with spontaneity than with voluntary effort [Table 2].
|Table 2: Differential diagnosis of bilateral facial palsy based upon clinical exam|
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Our patient had a noncaseating granuloma which is a nonspecific microscopic nodular complex of lymphocytes, macrophages, multinucleated giants cells, and occasionally eosinophils and granulocytes believed to be a reaction to persistent antigens. This cellular complex may be seen as a reaction to drugs, infection, tumor antigens, and foreign bodies. Noncaseating granulomas are found in 2–20% of liver biopsies. In the US, sarcoid is the most common cause of hepatic granulomas. Tuberculosis, mycobacterium avium-intracellulare, primary biliary cirrhosis, and drugs are other common causes [Table 3]. Except for tuberculosis, syphilis, and necrotic rheumatoid nodules, all granuloma are noncaseating. The liver is involved in almost all patients with systemic sarcoidosis. Usually, asymptomatic, hepatic sarcoidosis may cause an elevated alkaline phosphatase, chronic hepatitis, cirrhosis, cholestasis and Budd-Chiari syndrome More Details.
In our patient, biopsy-proven noncaseating granulomas of the liver and bilateral seventh nerve palsy occurred in the absence of any detectable underlying conditions. The provisional diagnosis is sarcoidosis. Tuberculosis, fungal disease, lymphoma, berylliosis, collagen vascular disease, vasculitis, and malignancies are common mimickers and ruled out after workup. The pathological diagnosis was established in the liver. Contrast enhanced MRI of the CNS is the preferred mode of imaging. MRI imaging in neurosarcoidosis may reveal changes in any part of the CNS [Table 4]. In our patient, there were no radiological findings in the CNS to explain the bilateral facial palsy. However, this is not unusual in neurosarcoidosis, and a normal MRI cannot rule out the diagnosis.
Neurosarcoidosis is associated with a high rate of mortality 5–10% and often is refractory to treatment. Central and peripheral nervous system complications occur in 5% of patients with sarcoidosis. Approximately 50% of patients with neurosarcoidosis have the neurologic symptoms as their presenting symptoms. Facial nerve palsy is present in over 50% patients with neurosarcoidosis. It can be unilateral or bilateral and simultaneous or sequential. Optic nerve and vestibulocochlear nerves can also be involved. Parenchymal involvement of the hypothalamus leads to neuroendocrine dysfunction. Granulomatous involvement of perivascular tissue can cause cognitive dysfunction, seizure, and encephalopathy. Sarcoidosis involvement of the spinal cord can cause myelopathy and radiculopathy. Hydrocephalus and meningitis are other presentations. Peripheral nerve involvement causes mononeuropathy or mononeuritis multiplex. There can be little correlation between clinical signs and radiologic findings in neurosarcoidosis, i.e., clinical signs without imaging finding or imaging findings without clinical signs. Hence, the goal is to look for systemic sarcoidosis.
If neurosarcoidosis is suspected, one must search for extraneural disease. The skin, lymph nodes, lungs, eyes, lacrimal gland, nasal, and buccal mucosa are common sites of involvement. Biopsies are attempted from the safest, most accessible site of abnormal tissue. The confirmation of systemic sarcoidosis will include ophthalmologic evaluation, endoscopic ENT evaluation, CT scan of the chest and abdomen, ACE assay, MRI of the CNS, CSF examination, and biopsy from easily accessible tissues. Ophthalmologic symptoms most commonly found on examination are optic neuritis, uveitis and occulamotor nerve palsy., Sarcoidosis infrquently involves the upper respiratory tract; however, ENT examination is necessary when suspected and may show sinonasal sinusitis. Vocal cord involvement is possible but rare. Chest imaging usually reveals bilateral lymphadenopathy with or without lung parenchymal lesions. Pulmonary function tests results are those suggestive of interstitial lung disease. In bronchoalveolar lavage samples, the CD4:CD8 lymphocyte ratio ranges from 3 to 10:1, compared to 2:1 for normal people. Serum ACE levels are elevated in 40–90% of patients with clinically active disease. However it is also elevated in berylliosis, fungal and tubercular involvement, Gaucher's disease, hypothyroidism, cirrhosis, diabetes, and Hodgkin's disease. CSF may be normal or show a lymphocytic pleocytosis with or without elevated ACE levels. The definitive diagnosis of neurosarcoidosis must be based on biopsy evidence in the context of history, characteristic clinical signs, and workup ruling out other possible differentials.
The therapy of choice is high-dose glucocorticoids. Neurosarcoidosis is often refractory to traditional 20–40 mg/day of prednisone, often 80–100 mg/day is used. Duration of therapy is typically at least 3 months and often continued up to 6–18 months. The major therapeutic decision is when to treat. The potential for persistent damage is highest in the eye, heart, and the CNS, and aggressive therapy is indicated. For severely incapacitating disease, intravenous methylprednisolone is used. A very gradual taper of prednisone prevents recurrent flares of the disease. Other options for therapy are azathioprine, cladribine, chlorambucil, cyclophosphamide, cyclosporine, infliximab, methotrexate, mycophenolate, etc., Cyclosporine is ineffective for pulmonary sarcoidosis. Refractory sarcoidosis is diagnosed as failure to respond to at least 2 therapeutic alternatives. In such cases, radiation therapy has been attempted. Ventricular drain is needed for hydrocephalus. Endocrine insufficiencies need to be corrected.
| Conclusion|| |
Neurosarcoidosis has a 5–10% rate of mortality and treatment with high-dose glucocorticoids is recommended. Our patient had a bone marrow biopsy after 3 weeks of steroids which was free from granulomas. Subsequent lumbar puncture revealed lymphocytic pleocytosis. The patient has been tapered off steroids over 3 months. She had good recovery of her facial palsy. There is no drooling of saliva or slurring of speech. There remains some residual ptosis of the right eye for which she undergoes physiotherapy. There has been no new neurologic worsening.
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| References|| |
Teller DC, Murphy TP. Bilateral facial paralysis: A case presentation and literature review. J Otolaryngol 1992;21:44-7.
House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg 1985;93:146-7.
Vrabec JT, Backous DD, Djalilian HR, Gidley PW, Leonetti JP, Marzo SJ, et al
. Facial Nerve Grading System 2.0. Otolaryngol Head Neck Surg 2009;140:445-50.
Fattah AY, Gurusinghe AD, Gavilan J, Hadlock TA, Marcus JR, Marres H, et al.
Facial nerve grading instruments: Systematic review of the literature and suggestion for uniformity. Plast Reconstr Surg 2015;135:569-79.
Biller J. Practical Neurology. 4th
ed. Philadelphia: Wolters Kluwer/Lippincott Williams and Wilkins Heath; 2012. p. 143-51.
Menezo V, Lobo A, Yeo TK, du Bois RM, Lightman S. Ocular features in neurosarcoidosis. Ocul Immunol Inflamm 2009;17:170-8.
Long CM, Smith TL, Loehrl TA, Komorowski RA, Toohill RJ. Sinonasal disease in patients with sarcoidosis. Am J Rhinol 2001;15:211-5.
Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, et al.
American College of Rheumatology classification criteria for Sjögren's syndrome: A data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken) 2012;64:475-87.
Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al.
The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-7.
Rogers RS 3rd
. Melkersson-Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin 1996;14:371-9.
Reichenberger E, Chen IP. Craniometaphyseal dysplasia, autosomal dominant. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJ, et al
., editors. GeneReviews (R). Seattle (WA): University of Washington, Seattle University of Washington, Seattle. All Rights Reserved; 1993.
Bird TD. Hereditary neuropathy with liability to pressure palsies. In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJ, et al
., editors. GeneReviews (R). Seattle (WA): University of Washington, Seattle University of Washington, Seattle. All Rights Reserved.; 1993.
Li G, Gu HM, Zhang DW. ATP-binding cassette transporters and cholesterol translocation. IUBMB Life 2013;65:505-12.
Watanabe K, Hagura R, Akanuma Y, Takasu T, Kajinuma H, Kuzuya N, et al.
Characteristics of cranial nerve palsies in diabetic patients. Diabetes Res Clin Pract 1990;10:19-27.
Rice JP, Horowitz M, Chin D. Wernicke-Korsakoff syndrome with bilateral facial nerve palsies. J Neurol Neurosurg Psychiatry 1984;47:1356-7.
Oztürk O, Serin GM, Inanli S, Tutkun A. Acute extratemporal facial nerve paralysis after a suicide attempt by hanging. Otolaryngol Head Neck Surg 2005;133:644.
Gupta S, Mends F, Hagiwara M, Fatterpekar G, Roehm PC. Imaging the facial nerve: A contemporary review. Radiol Res Pract 2013;2013:248039.
Price T, Fife DG. Bilateral simultaneous facial nerve palsy. J Laryngol Otol 2002;116:46-8.
Brazis PW, Masdeu JC, Biller J. Localization in Clinical Neurology. 5th
ed. Philadelphia: Lippincott Williams and Wilkins; 2007. p. 287-306.
Keane JR. Bilateral seventh nerve palsy: Analysis of 43 cases and review of the literature. Neurology 1994;44:1198-202.
Stahl N, Ferit T. Recurrent bilateral peripheral facial palsy. J Laryngol Otol 1989;103:117-9.
Gilden DH. Clinical practice. Bell's palsy. N Engl J Med 2004;351:1323-31.
James DG. A clinicopathological classification of granulomatous disorders. Postgrad Med J 2000;76:457-65.
Wainwright H. Hepatic granulomas. Eur J Gastroenterol Hepatol 2007;19:93-5.
Lower EE, Weiss KL. Neurosarcoidosis. Clin Chest Med 2008;29:475-92, ix.
Smith JK, Matheus MG, Castillo M. Imaging manifestations of neurosarcoidosis. AJR Am J Roentgenol 2004;182:289-95.
Krumholz A, Stern BJ. Neurologic manifestations of sarcoidosis. Handb Clin Neurol 2014;119:305-33.
Jain V, Deshmukh A, Gollomp S. Bilateral facial paralysis: Case presentation and discussion of differential diagnosis. J Gen Intern Med 2006;21:C7-10.
Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol 1985;42:909-17.
Mitchell DG. Sarcoidosis. London: Hodder Arnold; 2012. p. 25-70.
Koczman JJ, Rouleau J, Gaunt M, Kardon RH, Wall M, Lee AG. Neuro-ophthalmic sarcoidosis: The University of Iowa experience. Semin Ophthalmol 2008;23:157-68.
Mastan S, Advani R, Stobbs N, Kumar N. A rare manifestation of a multisystemic disease: A case of vocal cord palsy secondary to sarcoidosis. BMJ Case Rep 2015;2015. pii: Bcr2015209728.
Baughman RP, Lower EE. Treatment of sarcoidosis. Clin Rev Allergy Immunol 2015;49:79-92.
Agnihotri SP, Singhal T, Stern BJ, Cho TA. Neurosarcoidosis. Semin Neurol 2014;34:386-94.
Lorentzen AO, Sveberg L, Midtvedt Ø, Kerty E, Heuser K. Overnight response to infliximab in neurosarcoidosis: A case report and review of infliximab treatment practice. Clin Neuropharmacol 2014;37:142-8.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2], [Table 3], [Table 4]