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Recurrent Peripheral Facial Palsy in a Child With Familial Mediterranean Fever
Pediatric Neurology 49 (2013) 289e291
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Clinical Observations
Recurrent Peripheral Facial Palsy in a Child With Familial Mediterranean Fever Ünsal Yılmaz MD a, *, Nesrin Gülez MD b, Duygu Çubukçu MD c, Orkide Güzel MD a, Gülçin Akinci MD a, Aysel Öztürk MD a a
Department of Pediatric Neurology, Dr. Behçet Uz Children’s Hospital, Izmir, Turkey Department of Pediatric Immunology, Dr. Behçet Uz Children’s Hospital, Izmir, Turkey c Department of Physical Medicine and Rehabilitation, Dr. Behçet Uz Children’s Hospital, Izmir, Turkey b
article information
abstract
Article history: Received 14 February 2013 Accepted 2 May 2013
BACKGROUND: Recurrent peripheral facial palsy is uncommon in children. It mostly occurs as
Keywords: recurrent peripheral facial palsy familial Mediterranean fever children
an idiopathic disorder and to a lesser extent in the setting of some infectious, genetic, or systemic disorders. However, its association with familial Mediterranean fever has not been reported before. PATIENT: We present a 14-year-old girl who experienced three episodes of right-sided peripheral facial palsy during a 9-month interval. She had a diagnosis of familial Mediterranean fever (homozygous with M694V mutation) and she had been receiving colchicine for 8 years. Recurrent peripheral facial palsy could be a neurological manifestation of vasculitis in familial Mediterranean fever. CONCLUSION: Recurrent peripheral facial palsy may be a manifestation of familial Mediterranean fever in children. Ó 2013 Elsevier Inc. All rights reserved.
Introduction
Recurrent peripheral facial palsy is an uncommon disorder occurring in 6% of children with idiopathic peripheral facial palsy.1 It may also be associated with a variety of infectious, inflammatory, neoplastic, traumatic, genetic, or congenital disorders (Table). Familial Mediterranean fever is an autosomal recessive autoinflammatory disease associated with mutations in the Mediterranean fever (MEFV) gene, which encodes the protein pyrin.2 Pyrin is expressed mainly in granulocytes and is thought to be a negative regulator of inflammation.3 The typical features of the disease are recurrent self-limiting episodes of fever and painful polyserositis, affecting mainly the peritoneum, pleura, and synovium. Unexplained migration of granulocytes into the serosal and synovial spaces during these episodes may contribute to the sterile inflammation. Neurological involvement in familial Mediterranean fever is rare, but myalgia, headache, demyelinating lesions, * Communications should be addressed to: Dr. Yılmaz; Department of Pediatric Neurology, Dr. Behçet Uz Children’s Hospital, Konak, Izmir, 35210 Turkey. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2013.05.003
stroke, posterior reversible leukoencephalopathy syndrome, aseptic meningitis, convulsions, and cranial neuropathy have been documented adults.4-6 However, its association with recurrent peripheral facial palsy has not been reported before in children or adults. Case Report A 14-year-old girl with right peripheral facial palsy attended our outpatient clinic. She was known to have familial Mediterranean fever (homozygous with M694V mutation) and she had been treated with colchicine for 8 years. On admission, the patient had a 2-day history of right facial pain, progressive facial asymmetry, inability to close the right eye, and liquid loss through the right corner of her mouth while drinking. Neurological examination revealed a moderately severe dysfunction on the right side of the face with incomplete eye closure and moderate forehead movement (grade IV peripheral facial palsy according to the HouseBrackmann facial palsy scale). Of note, she had a fissured tongue (Fig). She had no rash suggestive of a viral infection. Her examination was otherwise unrevealing. She had an upper respiratory tract infection 15 days prior to the onset of symptoms. The family history was negative for peripheral facial palsy or any other neurologic disorders. After the exclusion of other causes of peripheral facial palsy based on clinical findings and laboratory investigations, the patient was diagnosed with Bell palsy and she recovered fully in 3 weeks with oral prednisolone and
290
Ü. Yılmaz et al. / Pediatric Neurology 49 (2013) 289e291
TABLE. Causes of recurrent peripheral facial palsy
Idiopathic (recurrent Bell palsy) Genetic Familial recurrent peripheral facial palsy Melkersson-Rosenthal syndrome Nerve compression Intracranial compressive tumors Leukemia Lymphoma Facial neurinoma* Fibrous dysplasia of the temporal bone* Osteopetrosis* Dental procedures* Infectious Recurrent otitis media Recurrent parotitis Lyme disease Mycoplasma infection Ramsey Hunt syndrome Human immunodeficiency virus infection* Inflammatory Celiac disease Sarcoidosis Behçet disease Systemic lupus erythematosus* Diabetes mellitus Dysglobulinemia Recurrent facial nerve palsy associated with anti-GQ1b immunoglobulin G antibodies* Multiple sclerosis* Vascular Hypertension Migraine Other Hereditary neuropathy with liability to pressure palsy* Plasmapheresis in Guillain-Barré syndrome* Pregnancy *
Case reports and thus could be coincidental.
acyclovir treatment and motor rehabilitation. Five months and 9 months after the initial episode, peripheral facial palsy recurred on the same side, again preceded by an upper respiratory tract infection. The second and third clinical attacks resolved spontaneously in 10 and 20 days, respectively.
FIGURE. A deep central and a few radiating tongue fissures in a 14-year-old girl with recurrent peripheral facial palsy and familial Mediterranean fever.
After the last episode, a more detailed investigation was carried out. Blood count, blood chemistry, erythrocyte sedimentation rate, C-reactive protein, antistreptolysin-O titer, thyroid function tests, and vitamin B12 values were within normal limits. A chest radiograph was normal. Imaging scans of the brain including contrast-enhanced magnetic resonance imaging, diffusion magnetic resonance imaging, magnetic resonance angiography, and computerized tomography of temporal bone did not reveal neurologic lesions suggestive of infarct, hemorrhage, tumor, trauma, demyelinating lesions, infectious lesions, aneurysm, sinus cavernous thrombosis, vasculitis, or sarcoidosis. Cerebrospinal fluid examination, including cell count, culture, glucose, protein, and immunoglobulin G and M antibodies against Borrelia burgdorferi and polymerase chain reaction tests for herpes simplex virus 1 and 2, adenovirus, and enterovirus were negative. No antibodies against Borrelia, mycoplasma, salmonella, brucella, toxoplasma, rubella, cytomegalovirus, Epstein-Barr virus, human immunodeficiency virus, and hepatitis A, B and C viruses were detected in the serum. Antineutrophil cytoplasmic, antinuclear, antigliadin, and antiendomysium antibodies and rheumatoid arthritis latex were also negative. To rule out a subclinical peripheral neuropathy, we performed median and peroneal motor and sensory nerve conduction studies and conventional needle electromyography, which were all normal. We did not perform electrophysiologic studies of the facial nerve because the patient showed rapid recovery from all episodes.
Discussion
A tumor, infection, or a traumatic injury involving the petrous bone may cause recurrent peripheral facial palsy, which requires urgent intervention.7,8 Therefore, the possibility of these disorders was eliminated through extensive neuroimaging of the temporal bone and brain, which also largely excluded the diagnosis of a stroke or multiple sclerosis. There was no serologic evidence of a viral infection such as herpes simplex virus, Ebstein-Barr virus, cytomegalovirus, enterovirus, rubella, varicella, human immunodeficiency virus, or a bacterial infection such as Brucella or mycoplasma. There were no eruptions suggestive of Ramsay Hunt syndrome or any other viral infections. Tuberculosis was unlikely because there was no family history or any suggestive finding on chest x-ray. Lyme disease is among the common causes of bilateral or recurrent facial palsy.9 However, no clinical findings of Lyme disease such as erythema migrans, fever, fatigue, or arthritis were observed during the physical examination. Immunoglobulin G and M antibodies against Borrelia burgdorferi were negative both in cerebrospinal fluid and in serum, which excluded the possibility of Lyme disease in the differential diagnosis. One of the differential diagnosis of recurrent peripheral facial palsy is Melkersson-Rosenthal syndrome. The pathophysiology of this condition is unknown, but autoimmune, allergic, infectious, and genetic factors have been proposed. Our patient had a furrowed tongue and recurrent peripheral facial palsy, which fulfill the diagnostic criteria of Melkersson-Rosenthal syndrome. However, fissured tongue has also been reported in other autoinflammatory disorders such as psoriasis. Moreover, recurrent peripheral facial palsy also occurs in association with autoimmune disorders such as multiple sclerosis, Behçet disease, or celiac disease.10 Behçet disease was unlikely because there were no typical clinical characteristics such as oral aphthae, genital or cutaneous ulcerations, transitory ischemic attacks, sinus thrombosis, pseudotumor cerebri, aseptic meningitis, or encephalitis. It is not easy to demonstrate an exact etiopathogenic relationship between autoinflammatory disorders
Ü. Yılmaz et al. / Pediatric Neurology 49 (2013) 289e291
and recurrent peripheral facial palsy. In one study, upon observation of a patient with recurrent peripheral facial palsy who manifested symptoms of celiac disease, the authors performed diagnostic investigations for celiac disease in eight patients with recurrent peripheral facial palsy, and they found that three patients had celiac disease.10 Moreover, two of them had no symptoms suggestive of celiac disease at the time of second peripheral facial palsy. Of note, two of the patients had furrowed tongues.10 To rule out presymptomatic celiac disease, we obtained antigliadin and antiendomysium antibodies, which were found negative. Based on these observations, we speculated that the coexistence of furrowed tongue and recurrent peripheral facial palsy was not specific for Melkersson-Rosenthal syndrome and that it may also be seen in other autoinflammatory disorders. The presence of preceding upper respiratory tract infections in all attacks in this case may be regarded as a triggering factor for immune system activation, which in turn possibly led to vasculitis. Several forms of systemic and cerebral vasculitis, including polyarteritis nodosa, Henoch-Schönlein purpura, Behçet disease, and acute vasculitis, with multiorgan involvement were previously described in association with familial Mediterranean fever. Thus, recurrent peripheral facial palsy could be attributed to vasculitis associated with familial Mediterranean fever instead of Melkersson-Rosenthal syndrome in our patient. Indeed peripheral facial nerve palsy has been reported as a manifestation of familial Mediterranean fever associated with the same mutation (M694V) in an adult patient. The authors in one study postulated that neurological abnormalities in familial Mediterranean fever could be attributed to cerebral vasculitis or amyloidosis.6 Although colchicine-induced myoneuropathy with proximal muscle weakness can rarely occur in patients with familial Mediterranean fever,11 its association with peripheral facial palsy seems unlikely because symptoms disappeared spontaneously in a relatively short period of time while the patient was still on colchicine treatment. Furthermore, electroneuromyography did not reveal any finding of myopathy or peripheral neuropathy in our patient.
291
Conclusion
Recurrent peripheral facial palsy remains a complex and unresolved entity from a pathophysiologic perspective. It appears that recurrent peripheral facial palsy may be a manifestation of familial Mediterranean fever in children. However, the role of coincidence or association is controversial and needs to be explored. This work was completed at the Department of Pediatric Neurology, Dr. Behçet Uz _ Children’s Hospital (Izmir, Turkey). The authors thank all colleagues for their help in follow-up of the patient.
References 1. Eidlitz-Markus T, Gilai A, Mimouni M, Shuper A. Recurrent facial nerve palsy in paediatric patients. Eur J Pediatr. 2001;160:659-663. 2. International FMF Consortium. Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever. Cell. 1997;90:797-807. 3. Gross O, Thomas CJ, Guarda G, et al. The inflammasome. An integrated view. Immunol Rev. 2011;243:136-151. 4. Gedalia A, Zamir S. Neurologic manifestations in familial Mediterranean fever. Paediatr Neurol. 1993;9:301-302. 5. Kalyoncu U, Eker A, Oguz KK, et al. Familial Mediterranean fever and central nervous system involvement: a case series. Medicine. 2010; 89:75-84. 6. Finsterer J, Stollberger C, Shinar Y. Cranial nerve lesions and abnormal visually evoked potentials associated with the M694V mutation in familial Mediterranean fever. Clin Rheumatol. 2002;21: 317-321. 7. May M, Hardin WB. Facial palsy: interpretation of neurological findings. Laryngoscope. 1978;88:1352-1362. 8. Bodénez C, Vargaftig J, Barré P, Mansour G, Lamas G, Tankere E. Bilateral and recurrent facial paralysis due to lymphoma: a case report. Rev Laryngol Otol Rhinol. 2007;128:69-72. 9. Halperin JJ. Lyme disease and the peripheral nervous system. Muscle Nerve. 2003;28:133-143. 10. Capone F, Batocchi AP, Cammarota G, Pilato F, Profice P, Di Lazzaro V. Gluten-related recurrent peripheral facial palsy. J Neurol Neurosurg Psychiatry. 2012;83:667-668. 11. Cantarini L, Volpi N, Galeazzi M, et al. Colchicine myopathy and neuromyopathy: two cases with different characteristics. J Clin Rheumatol. 2010;16:229-232.
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Clinical Observations
Recurrent Peripheral Facial Palsy in a Child With Familial Mediterranean Fever Ünsal Yılmaz MD a, *, Nesrin Gülez MD b, Duygu Çubukçu MD c, Orkide Güzel MD a, Gülçin Akinci MD a, Aysel Öztürk MD a a
Department of Pediatric Neurology, Dr. Behçet Uz Children’s Hospital, Izmir, Turkey Department of Pediatric Immunology, Dr. Behçet Uz Children’s Hospital, Izmir, Turkey c Department of Physical Medicine and Rehabilitation, Dr. Behçet Uz Children’s Hospital, Izmir, Turkey b
article information
abstract
Article history: Received 14 February 2013 Accepted 2 May 2013
BACKGROUND: Recurrent peripheral facial palsy is uncommon in children. It mostly occurs as
Keywords: recurrent peripheral facial palsy familial Mediterranean fever children
an idiopathic disorder and to a lesser extent in the setting of some infectious, genetic, or systemic disorders. However, its association with familial Mediterranean fever has not been reported before. PATIENT: We present a 14-year-old girl who experienced three episodes of right-sided peripheral facial palsy during a 9-month interval. She had a diagnosis of familial Mediterranean fever (homozygous with M694V mutation) and she had been receiving colchicine for 8 years. Recurrent peripheral facial palsy could be a neurological manifestation of vasculitis in familial Mediterranean fever. CONCLUSION: Recurrent peripheral facial palsy may be a manifestation of familial Mediterranean fever in children. Ó 2013 Elsevier Inc. All rights reserved.
Introduction
Recurrent peripheral facial palsy is an uncommon disorder occurring in 6% of children with idiopathic peripheral facial palsy.1 It may also be associated with a variety of infectious, inflammatory, neoplastic, traumatic, genetic, or congenital disorders (Table). Familial Mediterranean fever is an autosomal recessive autoinflammatory disease associated with mutations in the Mediterranean fever (MEFV) gene, which encodes the protein pyrin.2 Pyrin is expressed mainly in granulocytes and is thought to be a negative regulator of inflammation.3 The typical features of the disease are recurrent self-limiting episodes of fever and painful polyserositis, affecting mainly the peritoneum, pleura, and synovium. Unexplained migration of granulocytes into the serosal and synovial spaces during these episodes may contribute to the sterile inflammation. Neurological involvement in familial Mediterranean fever is rare, but myalgia, headache, demyelinating lesions, * Communications should be addressed to: Dr. Yılmaz; Department of Pediatric Neurology, Dr. Behçet Uz Children’s Hospital, Konak, Izmir, 35210 Turkey. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2013.05.003
stroke, posterior reversible leukoencephalopathy syndrome, aseptic meningitis, convulsions, and cranial neuropathy have been documented adults.4-6 However, its association with recurrent peripheral facial palsy has not been reported before in children or adults. Case Report A 14-year-old girl with right peripheral facial palsy attended our outpatient clinic. She was known to have familial Mediterranean fever (homozygous with M694V mutation) and she had been treated with colchicine for 8 years. On admission, the patient had a 2-day history of right facial pain, progressive facial asymmetry, inability to close the right eye, and liquid loss through the right corner of her mouth while drinking. Neurological examination revealed a moderately severe dysfunction on the right side of the face with incomplete eye closure and moderate forehead movement (grade IV peripheral facial palsy according to the HouseBrackmann facial palsy scale). Of note, she had a fissured tongue (Fig). She had no rash suggestive of a viral infection. Her examination was otherwise unrevealing. She had an upper respiratory tract infection 15 days prior to the onset of symptoms. The family history was negative for peripheral facial palsy or any other neurologic disorders. After the exclusion of other causes of peripheral facial palsy based on clinical findings and laboratory investigations, the patient was diagnosed with Bell palsy and she recovered fully in 3 weeks with oral prednisolone and
290
Ü. Yılmaz et al. / Pediatric Neurology 49 (2013) 289e291
TABLE. Causes of recurrent peripheral facial palsy
Idiopathic (recurrent Bell palsy) Genetic Familial recurrent peripheral facial palsy Melkersson-Rosenthal syndrome Nerve compression Intracranial compressive tumors Leukemia Lymphoma Facial neurinoma* Fibrous dysplasia of the temporal bone* Osteopetrosis* Dental procedures* Infectious Recurrent otitis media Recurrent parotitis Lyme disease Mycoplasma infection Ramsey Hunt syndrome Human immunodeficiency virus infection* Inflammatory Celiac disease Sarcoidosis Behçet disease Systemic lupus erythematosus* Diabetes mellitus Dysglobulinemia Recurrent facial nerve palsy associated with anti-GQ1b immunoglobulin G antibodies* Multiple sclerosis* Vascular Hypertension Migraine Other Hereditary neuropathy with liability to pressure palsy* Plasmapheresis in Guillain-Barré syndrome* Pregnancy *
Case reports and thus could be coincidental.
acyclovir treatment and motor rehabilitation. Five months and 9 months after the initial episode, peripheral facial palsy recurred on the same side, again preceded by an upper respiratory tract infection. The second and third clinical attacks resolved spontaneously in 10 and 20 days, respectively.
FIGURE. A deep central and a few radiating tongue fissures in a 14-year-old girl with recurrent peripheral facial palsy and familial Mediterranean fever.
After the last episode, a more detailed investigation was carried out. Blood count, blood chemistry, erythrocyte sedimentation rate, C-reactive protein, antistreptolysin-O titer, thyroid function tests, and vitamin B12 values were within normal limits. A chest radiograph was normal. Imaging scans of the brain including contrast-enhanced magnetic resonance imaging, diffusion magnetic resonance imaging, magnetic resonance angiography, and computerized tomography of temporal bone did not reveal neurologic lesions suggestive of infarct, hemorrhage, tumor, trauma, demyelinating lesions, infectious lesions, aneurysm, sinus cavernous thrombosis, vasculitis, or sarcoidosis. Cerebrospinal fluid examination, including cell count, culture, glucose, protein, and immunoglobulin G and M antibodies against Borrelia burgdorferi and polymerase chain reaction tests for herpes simplex virus 1 and 2, adenovirus, and enterovirus were negative. No antibodies against Borrelia, mycoplasma, salmonella, brucella, toxoplasma, rubella, cytomegalovirus, Epstein-Barr virus, human immunodeficiency virus, and hepatitis A, B and C viruses were detected in the serum. Antineutrophil cytoplasmic, antinuclear, antigliadin, and antiendomysium antibodies and rheumatoid arthritis latex were also negative. To rule out a subclinical peripheral neuropathy, we performed median and peroneal motor and sensory nerve conduction studies and conventional needle electromyography, which were all normal. We did not perform electrophysiologic studies of the facial nerve because the patient showed rapid recovery from all episodes.
Discussion
A tumor, infection, or a traumatic injury involving the petrous bone may cause recurrent peripheral facial palsy, which requires urgent intervention.7,8 Therefore, the possibility of these disorders was eliminated through extensive neuroimaging of the temporal bone and brain, which also largely excluded the diagnosis of a stroke or multiple sclerosis. There was no serologic evidence of a viral infection such as herpes simplex virus, Ebstein-Barr virus, cytomegalovirus, enterovirus, rubella, varicella, human immunodeficiency virus, or a bacterial infection such as Brucella or mycoplasma. There were no eruptions suggestive of Ramsay Hunt syndrome or any other viral infections. Tuberculosis was unlikely because there was no family history or any suggestive finding on chest x-ray. Lyme disease is among the common causes of bilateral or recurrent facial palsy.9 However, no clinical findings of Lyme disease such as erythema migrans, fever, fatigue, or arthritis were observed during the physical examination. Immunoglobulin G and M antibodies against Borrelia burgdorferi were negative both in cerebrospinal fluid and in serum, which excluded the possibility of Lyme disease in the differential diagnosis. One of the differential diagnosis of recurrent peripheral facial palsy is Melkersson-Rosenthal syndrome. The pathophysiology of this condition is unknown, but autoimmune, allergic, infectious, and genetic factors have been proposed. Our patient had a furrowed tongue and recurrent peripheral facial palsy, which fulfill the diagnostic criteria of Melkersson-Rosenthal syndrome. However, fissured tongue has also been reported in other autoinflammatory disorders such as psoriasis. Moreover, recurrent peripheral facial palsy also occurs in association with autoimmune disorders such as multiple sclerosis, Behçet disease, or celiac disease.10 Behçet disease was unlikely because there were no typical clinical characteristics such as oral aphthae, genital or cutaneous ulcerations, transitory ischemic attacks, sinus thrombosis, pseudotumor cerebri, aseptic meningitis, or encephalitis. It is not easy to demonstrate an exact etiopathogenic relationship between autoinflammatory disorders
Ü. Yılmaz et al. / Pediatric Neurology 49 (2013) 289e291
and recurrent peripheral facial palsy. In one study, upon observation of a patient with recurrent peripheral facial palsy who manifested symptoms of celiac disease, the authors performed diagnostic investigations for celiac disease in eight patients with recurrent peripheral facial palsy, and they found that three patients had celiac disease.10 Moreover, two of them had no symptoms suggestive of celiac disease at the time of second peripheral facial palsy. Of note, two of the patients had furrowed tongues.10 To rule out presymptomatic celiac disease, we obtained antigliadin and antiendomysium antibodies, which were found negative. Based on these observations, we speculated that the coexistence of furrowed tongue and recurrent peripheral facial palsy was not specific for Melkersson-Rosenthal syndrome and that it may also be seen in other autoinflammatory disorders. The presence of preceding upper respiratory tract infections in all attacks in this case may be regarded as a triggering factor for immune system activation, which in turn possibly led to vasculitis. Several forms of systemic and cerebral vasculitis, including polyarteritis nodosa, Henoch-Schönlein purpura, Behçet disease, and acute vasculitis, with multiorgan involvement were previously described in association with familial Mediterranean fever. Thus, recurrent peripheral facial palsy could be attributed to vasculitis associated with familial Mediterranean fever instead of Melkersson-Rosenthal syndrome in our patient. Indeed peripheral facial nerve palsy has been reported as a manifestation of familial Mediterranean fever associated with the same mutation (M694V) in an adult patient. The authors in one study postulated that neurological abnormalities in familial Mediterranean fever could be attributed to cerebral vasculitis or amyloidosis.6 Although colchicine-induced myoneuropathy with proximal muscle weakness can rarely occur in patients with familial Mediterranean fever,11 its association with peripheral facial palsy seems unlikely because symptoms disappeared spontaneously in a relatively short period of time while the patient was still on colchicine treatment. Furthermore, electroneuromyography did not reveal any finding of myopathy or peripheral neuropathy in our patient.
291
Conclusion
Recurrent peripheral facial palsy remains a complex and unresolved entity from a pathophysiologic perspective. It appears that recurrent peripheral facial palsy may be a manifestation of familial Mediterranean fever in children. However, the role of coincidence or association is controversial and needs to be explored. This work was completed at the Department of Pediatric Neurology, Dr. Behçet Uz _ Children’s Hospital (Izmir, Turkey). The authors thank all colleagues for their help in follow-up of the patient.
References 1. Eidlitz-Markus T, Gilai A, Mimouni M, Shuper A. Recurrent facial nerve palsy in paediatric patients. Eur J Pediatr. 2001;160:659-663. 2. International FMF Consortium. Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever. Cell. 1997;90:797-807. 3. Gross O, Thomas CJ, Guarda G, et al. The inflammasome. An integrated view. Immunol Rev. 2011;243:136-151. 4. Gedalia A, Zamir S. Neurologic manifestations in familial Mediterranean fever. Paediatr Neurol. 1993;9:301-302. 5. Kalyoncu U, Eker A, Oguz KK, et al. Familial Mediterranean fever and central nervous system involvement: a case series. Medicine. 2010; 89:75-84. 6. Finsterer J, Stollberger C, Shinar Y. Cranial nerve lesions and abnormal visually evoked potentials associated with the M694V mutation in familial Mediterranean fever. Clin Rheumatol. 2002;21: 317-321. 7. May M, Hardin WB. Facial palsy: interpretation of neurological findings. Laryngoscope. 1978;88:1352-1362. 8. Bodénez C, Vargaftig J, Barré P, Mansour G, Lamas G, Tankere E. Bilateral and recurrent facial paralysis due to lymphoma: a case report. Rev Laryngol Otol Rhinol. 2007;128:69-72. 9. Halperin JJ. Lyme disease and the peripheral nervous system. Muscle Nerve. 2003;28:133-143. 10. Capone F, Batocchi AP, Cammarota G, Pilato F, Profice P, Di Lazzaro V. Gluten-related recurrent peripheral facial palsy. J Neurol Neurosurg Psychiatry. 2012;83:667-668. 11. Cantarini L, Volpi N, Galeazzi M, et al. Colchicine myopathy and neuromyopathy: two cases with different characteristics. J Clin Rheumatol. 2010;16:229-232.