Facial palsy in Melkersson-Rosenthal syndrome and Bell's palsy: familial history and recurrence tendency.

Melkersson-Rosenthal syndrome.

Key words: Bell's palsy, brainstem glioma, magnetic reso- nance imaging Introduction Facial palsy of the peripheral type is generally seen in the pain clinic and is often treated with a stellate gang- lion block. The most common cause of peripheral facial nerve palsy is Bell's palsy, although its etiology remains controversial. The diagnosis of Bell's palsy is usually made by exclusion of other conditions such as herpes zoster oticus (Ramsay Hunt syndrome), trauma (including skull base fracture and surgery), otitis media, and neoplasm [1]. Isolated peripheral facial nerve palsy of neoplastic origin is uncommon. We herein describe a case of peripheral facial nerve palsy which was initially diag- nosed as Bell's palsy but was later found to be caused by an intrinsic brain stem tumor. Case report A 9-year-old boy presented to the Pediatric Depart- ment of our University Hospital in August 1990 with left facial weakness. His mother noticed the hyperemic conjunctiva and lacrimation of his left eye at the end of June. Consultation with the ophthalmologist revealed no abnormality in his left eye and the hyperemia im- proved with conservative therapy. In July, facial asym- metry became obvious. He was diagnosed as having Bell's palsy by a pediatrician in August and was referred to. our pain clinic. Address correspondence to: K. Kodama Received for publication on May 31, 1993; accepted on January 6, 1994 Upon examination, the patient had a left facial nerve palsy of the peripheral type (score of the facial paresis was 24/40), however, no other neurological deficits were seen. An audiogram failed to reveal a hearing abnormality. Although repeated stellate ganglion block was given, his facial palsy progressed slowly over a 2- month period, suggesting an etiology other than Bell's palsy. Magnetic resonance imaging (MRI) in September demonstrated a tumor in the left pons and brachium pontis extending into the left cerebellopontine angle (Fig. 1). The lesion was seen as a hypointense and hyperintense area on T1- and T2-weighted images, re- spectively. He was admitted to the Neurosurgical De- partment on September 29. The positive neurological findings on admission were Bruns' nystagmus, absence of left corneal reflex, decreased gag reflex, and mild trunkal ataxia, in addition to left facial nerve palsy. He underwent a wide suboccipital decompressive craniec- tomy, and biopsy of the tumor indicated low-grade glioma. In spite of postoperative radiation (60 Gy) and chemotherapy including Ranimustine and tumor necro- sis factor, he died due to tumor progression 17 months from the time of his initial symptom. Discussion Eighty percent of peripheral facial nerve palsy cases represent idiopathic or Bell's palsy, of which approxi- mately 20% can be demonstrated to have a specific etiology [2]. Peripheral facial nerve palsy with neoplas- tic origin is uncommon, and is estimated to be the cause in approximately 5% of all cases [3]. The diagnosis of Bell's palsy is unjustified unless an accurate history is taken along with a careful examina- tion of the ear and central nervous system (CNS). The differential diagnosis of neoplastic facial palsy is vast

To report the causes and clinical evaluation of children with facial nerve palsy (FNP) admitted to an affiliated university hospital during a 5-year period (2011-2015). A total of 124 children were retrospectively categorised into two groups: idiopathic Bell's palsy (109 patients) and the second group into other FNP aetiologies (15 patients). All children received a standardised work-up and follow-up. Therapy consisted of steroid administration associated with antiviral treatment when a viral infection was suspected. All children of the first group had a full recovery under oral steroids within 2 months of treatment. From the second group, seven children (46%) had a viral infection based on serological findings, two of them were positive for neurotropic herpes viruses, and one had Ramsay Hunt syndrome; six children with infectious FNP had recurrent FNP on the ipsilateral or contralateral side. Five patients had FNP as a complication of acute otitis media; three of them (60%) had partial or full recovery postoperatively. One child developed FNP following temporal bone trauma that had an uneventful recovery with conservative treatment. One child suffered from Melkersson-Rosenthal syndrome, and another child presented with FNP associated with unilateral hemiparesis following an ischaemic cerebral infarct. Facial palsy in children is a manifestation of a heterogeneous group of causes. The most common aetiology of FNP in children in our study was idiopathic (Bell's palsy), followed by infective causes, such as acute otitis media and neurotropic herpes viruses. Therefore, treatment should be adapted to each patient depending on the underlying disease and severity of FNP.

Contrast-enhanced MR images (at 1.5 T) were obtained in 11 patients with facial palsy. The group included five people with acute idiopathic facial (Bell's) palsy, three with chronic idiopathic facial palsy, and one each with acute facial palsy after local radiation therapy, acute facial palsy resulting from herpes zoster virus infection, and facial palsy caused by facial neuroma. Eight of the 11 patients demonstrated marked enhancement of the affected facial nerve from the labyrinthine portion through the descending canal. Three patients also demonstrated mild enhancement of the distal canalicular portion of the facial nerve, simulating small distal acoustic neuromas. No difference in the pattern of enhancement between the acute or chronic Bell's palsy patients was seen. Radiographic resolution appeared to lag behind clinical resolution. The facial neuroma appeared distinct from the other lesions as a focally enhancing mass. The enhancement pattern in the Bell's group correlated with the histopathologic features of Bell's palsy and is consistent with the viral hypothesis of the syndrome. Thin-section contrast-enhanced MR scans are recommended for individuals with atypical presentation of facial paralysis. In the proper clinical setting, contrast-enhanced MR imaging may permit a positive radiographic diagnosis of Bell's palsy, which has previously been a diagnosis of exclusion.

Article1 August 1942THE OCCURRENCE OF PERIPHERAL FACIAL PARALYSIS IN HYPERTENSIVE VASCULAR DISEASEHAROLD R. MERWARTH, M.D., F.A.C.P.HAROLD R. MERWARTH, M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-17-2-298 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptAlthough paralysis of the peripheral portion of the facial nerve is a very common disorder, its occurrence as a result of localized compressive bleeding in hypertensive vascular disease has been regarded as rare. Whereas hemorrhage into the facial aqueduct as a cause of facial palsy was recognized by earlier observers, their pathological findings and opinions have been ignored recently in the tabulated causal classifications of facial paralysis.Since 1925, 468 cases of facial paralysis peripheral in location have been observed. Eighteen of this number were myoclonic facial palsies. Although the ultimate picture of myoclonic paralysis resembles that of the contractured...Bibliography1. MOXON : Transactions of the Pathologic Society of London, 1869, xx, 420. Google Scholar2. GOWERS WR: Diseases of the nervous system (Am. Edition), 1888, Blakiston & Son, Philadelphia, p. 648. Google Scholar3. OPPENHEIM H: Textbook of nervous diseases, 1910, Darien Press, Edinburgh, i, p. 482. Google Scholar4. KEITHWAGENERKERNOHAN NHPJW: The syndrome of malignant hypertension, Arch. Int. Med., 1928, xli, 141. CrossrefGoogle Scholar5. AMBERG S: Hypertension in the young, Am. Jr. Dis. Child., 1929, xxxvii, 335. Google Scholar6. MAY E: Néphrite chronique et paralysie faciale, Bull. et mém. Soc. méd. d. hôp. de Paris, 1930, liv, 915-917. Google Scholar7. MONIER-VINARDPUECH P: Néphrite chronique et paralysie faciale, Bull. et mém. Soc. méd. d. hôp. de Paris, 1930, liv, 977-980. Google Scholar8. GRIFFITH JQ: Involvement of the facial nerve in malignant hypertension, Arch. Neurol. and Psychiat., 1923, xxix, 1194. Google Scholar9. GALLAVARDIN ML: Hypertension artérielle et paralysie faciale périphérique, Médecine, 1936, xvii, 186-190. Google Scholar10. MERWARTH HR: The recurrence of facial paralysis, Am. Jr. Med. Sci., 1935, clxxxix, 2, 270. CrossrefGoogle Scholar11. POLITZER A: Diseases of the ears, 1926, Lea and Febiger, Philadelphia, p. 37. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: HAROLD R. MERWARTH, M.D., F.A.C.P.Affiliations: Brooklyn, New York*Read before the Brooklyn Neurological Society January 28, 1941. Received for publication August 8, 1941. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byThe Use of Phototherapy for Bell’s PalsyBell's palsy: an overviewAnatomical consideration of the temporal bone as a pathogenesis of Bell’s palsyPeripheral facial palsy after varicella. Report of two cases and review of the literatureCorrelates of degree of nerve involvement in early Bell's palsyBell's PalsyIdiopathic facial (Bell's) palsy: a clinical survey of prognostic factorsBell's palsy: factors affecting the prognosis in 200 patients with reference to hypertension and diabetes mellitus‘Bell's palsy’ in accelerated hypertensionIdiopathic Facial Paralysis, Pregnancy, and the Menstrual CycleIdiopathic Facial Palsy and PregnancyMelkersson-Rosenthal syndromeTransitory unilateral facial parallysis (Bell's palsy)Tratamiento de la parálisis facialXCVI The Otological Concept of Bell's Palsy and its TreatmentSymposium: The Treatment of Facial ParalysisLXIV The Present Position of Facial Nerve Surgery 1 August 1942Volume 17, Issue 2 Page: 298-307 Keywords Facial nerve Hemorrhage Paralysis Vascular diseases ePublished: 1 December 2008 Issue Published: 1 August 1942 PDF downloadLoading ...

Bell's palsy in children

Bell's palsy is the most common cause of peripheral facial nerve paralysis. Although herpes simplex virus type 1 (HSV-1) infection has been strongly suggested as a cause of Bell's palsy, the pathomechanism of the facial nerve paralysis is unclear. Previously, we had succeeded in producing an animal model of acute, transient and homolateral facial paralysis by inoculating HSV-1 into the auricle, simulating Bell's palsy. To clarify the mechanism of this facial nerve paralysis, electrophysiological testing of the trigemino-facial reflex (blink reflex) and electroneuronography (ENoG) were carried out, and a histopathological study of the facial nerve was subsequently performed. The blink reflex and ENoG were examined twice ; during facial paralysis on day 10 and after recovery on day 17. The R1 latency of the blink reflex was prolonged or disappeared on the paralyzed side during facial paralysis, but recovered in all animals with the corresponding recovery of facial nerve paralysis. ENoG values were inconsistent during facial nerve paralysis and did not normalize even after complete recovery of the facial nerve paralysis. The histopathological studies demonstrated that mixed nerve damage, demyelination and axonotomesis, were present, although demyelination was dominant. Collectively, the electrophysiological and histopathological findings suggested that the pathomechanism of facial nerve paralysis caused by HSV-1 infection is mainly due to demyelination of the nerve, which was represented as a conduction block in electrophysiological testing. The present study suggested that the facial nerve damage underlying Bell's palsy involves mixed nerve damage including demyelination and axonotomesis, and the prognosis of facial nerve paralysis is dependent on the balance of these two kinds of nerve damage.

Conclusions: Herpes simplex virus 1 (HSV-1) and varicella-zoster virus (VZV) DNA were not detected in the cerebrospinal fluid (CSF) of patients with acute idiopathic peripheral facial palsy (Bell's palsy). Our results indicate either the absence of these viruses or the presence of technical shortcomings. The role of human herpesvirus 6 (HHV-6) in this disorder and the significance of a positive HHV-6 DNA finding in the central nervous system need further investigation. Objective: Our goal was to determine whether DNA of HSV-1, VZV, or HHV-6 can be found by polymerase chain reaction (PCR) in the CSF of peripheral facial palsy patients. Materials and methods: We used PCR to detect the presence of HSV-1, VZV, and HHV-6 DNA in CSF. This was a retrospective case control study with 33 peripheral facial palsy patients (34 CSF samples) in the study group (26 with Bell's palsy, 5 with simultaneously diagnosed herpesvirus infection, 1 with puerperal facial palsy, 1 with Melkersson-Rosenthal syndrome). The control group included 36 patients, most with diagnosed or suspected Borreliosis and facial palsy or sudden deafness. Results: One patient with Bell's palsy had HHV-6 DNA in CSF. Neither HSV-1 nor VZV DNA was detected in patients or controls.

IntroductionIsolated facial nerve palsy usually manifests as Bell's palsy. Lacunar infarct involving the lower pons is a rare cause of solitary infranuclear facial paralysis. The present unusual case is one in which the patient appeared to have Bell's palsy but turned out to have a pontine infarct.Case presentationA 47-year-old Asian Indian man with a medical history of hypertension presented to our institution with nausea, vomiting, generalized weakness, facial droop, and slurred speech of 14 hours' duration. His physical examination revealed that he was conscious, lethargic, and had mildly slurred speech. His blood pressure was 216/142 mmHg. His neurologic examination showed that he had loss of left-sided forehead creases, inability to close his left eye, left facial muscle weakness, rightward deviation of the angle of the mouth on smiling, and loss of the left nasolabial fold. Afferent corneal reflexes were present bilaterally. MRI of the head was initially read as negative for acute stroke. Bell's palsy appeared less likely because of the acuity of his presentation, encephalopathy-like imaging, and hypertension. The MRI was re-evaluated with a neurologist's assistance, which revealed a tiny 4 mm infarct involving the left dorsal aspect of the pons. The final diagnosis was isolated facial nerve palsy due to lacunar infarct of dorsal pons and hypertensive encephalopathy.ConclusionThe facial nerve has a predominant motor component which supplies all muscles concerned with unilateral facial expression. Anatomic knowledge is crucial for clinical localization. Bell's palsy accounts for around 72% of facial palsies. Other causes such as tumors and pontine infarcts can also present as facial palsy. Isolated dorsal infarct presenting as isolated facial palsy is very rare. Our case emphasizes that isolated facial palsy should not always be attributed to Bell's palsy. It can be a presentation of a rare dorsal pontine infarct as observed in our patient.

Melkersson Rosenthal Syndrome (MRS) is a rare neurological disorder characterized by swelling of the face, particularly one or both lips (granulomatous cheilitis), facial muscle weakness (palsy) and a fissured tongue. We present a patient with Melkersson Rosenthal Syndrome, highlighting the clinical triad of symptoms and management. A74-year-old Nigerian male presented to the Oral Medicine Clinic of the University of Benin Teaching Hospital for evaluation of right-sided facial numbness, inability to close the right eye, left facial deviation suggestive of a lower motor neuron type facial palsy of 4 days, and painless swelling of the upper and lower lips for seven days. On examination, he was found to have swelling of the upper and lower lips, multiple fissures on the tongue, right facial paresthesia, and an isolated right-sided facial nerve paralysis. He was empirically managed with 50mg prednisone daily for seven days, which was tapered over two weeks and neurobion 1 tablet daily for a month. This resulted in remission of lip swelling, however fissured tongue remained. Two months later, facial deviation had become less apparent. Patient is being followed up in the outpatient clinic. Melkersson Rosenthal Syndrome is a rare disorder with features of facial swelling, facial nerve palsy and fissured tongue. Some affected individuals may have all three of these features and others may have only one or two. The diagnosis of MRS can be made clinically when there is a complete triad of symptoms as reported in our patient.Keywords: Melkersson Rosenthal Syndrome, clinical symptoms, management

OLIGOSYMPTOMATIC FORM OF MELKERSSON-ROSENTHAL SYNDROME: A CASE REPORT

To evaluate the incidence of recurrent facial palsy and the frequency of misdiagnosis as Bell's palsy in patients with intratemporal facial nerve schwannomas (FNSs)Methods:A systematic review of PubMed and Cochrane databases and a single-institutional analysis were conducted, covering studies from the past 10 years on adult cases of intratemporal FNS with documented facial nerve function at presentation. Inclusion criteria focused on patients presenting with facial paralysis to assess recurrence rates and misdiagnoses as Bell's palsy. Key outcomes included incidence, severity, and the number of prior facial paralysis episodes at the time of FNS diagnosis. From 284 studies identified, 77 full texts were reviewed, and 53 met inclusion criteria, totaling 531 patients. Among the 531 patients, 55.6% (295) initially presented with facial paralysis. We found that 4.5% (24) of all patients in the systematic review and 22.2% (2) of cases in our institutional review with an intratemporal FNS were initially misdiagnosed with Bell's palsy. Of those who presented with facial paralysis, misdiagnosis as Bell's palsy was noted in 8.14% (24) of the systematic review and 100% (2) of our institutional review. The average House-Brackmann (HB) scores worsened from initial presentation to pre-operative assessment (mean scores: 2.07 ± 1.49 vs 2.94 ± 1.73). Our single-institutional and systematic review emphasizes that facial paralysis is a common presenting symptom of FNS. Although idiopathic (Bell's) palsy is the most frequent cause of facial paralysis, it remains a diagnosis of exclusion and a neoplastic cause should be ruled out in certain cases. A high index of suspicion is warranted for persistent (>3 months) or recurrent facial palsy, particularly when accompanied by otologic symptoms. Early identification of FNS enables timely interventions, such as facial nerve decompression, which may preserve native nerve function.

A 28-year-old man presented with right-sided facial paralysis that had been worsening over the past eight months. He was initially diagnosed with Bell's palsy and treated with oral steroids and antiviral medication immediately after symptom onset, but experienced minimal improvement. He has a known history of conductive hearing loss in the right ear since age five due to a traumatic tympanic membrane perforation from q-tip use and subsequently underwent tympanoplasty at that time. He denied otalgia, otorrhea, vertigo, or tinnitus. Physical examination showed grade 6/6 paralysis on the right side. Audiogram from two months before presentation ago is shown in Figure 1. What is your diagnosis?Figure 1: Audiogram showing moderate-to-severe conductive hearing loss on the right side. Hearing loss, paralysis.Figure 2: Axial (horizontal) CT of the right temporal bone showing the cholesteatoma involving the middle ear, cochlea, and internal auditory canal. Hearing loss, paralysis.Figure 3: Axial (horizontal) CT of the right temporal bone showing cholesteatoma 1.2 mm above Figure 2 showing the involvement of the tympanic (middle ear) facial nerve by the cholesteatoma. Hearing loss, paralysis.Figure 4: Coronal (vertical parallel to ear) CT of the right temporal bone demonstrating that the cholesteatoma has eroded the tegmen tympani. The cholesteatoma appears to have originated medial to the malleus. Hearing loss, paralysis.Figure 5: Sagittal (vertical parallel to face) CT of the right temporal bone further highlighting the erosion of the tegmen tympani as the cholesteatoma extended medially to the cochlea and internal auditory canal. Hearing loss, paralysis.Figure 6: Sagittal (vertical parallel to face) CT Temporal bone showing the involvement of the cochlea 2 mm medial to the image in Figure 5. Hearing loss, paralysis.DIAGNOSIS: IATROGENIC CHOLESTEATOMA The most concerning aspect of this patient's presentation is the duration of his facial paralysis. Although Bell's palsy is the most frequent diagnosis for facial paralysis, the physician must begin to consider other etiologies and obtain imaging with MRI of the internal auditory canals (IACs) if the paralysis persists beyond six months. The majority of patients with facial paralysis are diagnosed with Bell's palsy, also called idiopathic facial nerve paralysis. By definition, the exact cause of Bell's palsy is unknown; however, it is believed that a large number of cases are due to edema in and around the facial nerve and is caused by the herpes simplex virus (HSV). HSV, which also causes cold sores, has been found to be present within the geniculate ganglion of affected individuals. Viral replication and reactivation within the ganglion are thought to cause edema and subsequent compression of facial nerve fibers, resulting in blockage of electrical conduction and subsequent facial paralysis. The surrounding bony architecture of the facial nerve helps to explain this phenomenon. As it courses through the labyrinthine segment of the temporal bone (the narrowest portion of the fallopian canal measuring approximately 0.68 mm), the facial nerve is completely surrounded by bone, and therefore vulnerable to compression in the event of swelling. The extent of nerve injury depends on both the degree of inflammation and how quickly treatment with steroids and antivirals can be given to reduce swelling. In cases of mild edema, there is transient compression and blockage of nerve conduction until the inflammation subsides. However, in more severe cases the nerve fibers may be crushed and rapidly degenerate. In these cases, axon regeneration usually occurs, but the new nerve fibers may not reach the intended target muscles. This results in synkinesis, in which voluntary movement in one facial muscle group causes involuntary activity of another. For example, a patient may experience involuntary blinking when trying to smile. In the case of this patient, we obtained a CT scan of the temporal bones given the history of conductive hearing loss and previous surgery (Figs. 2, 3, 4, 5). On the right side, we see bony erosion with soft tissue opacification within the petrous and mastoid temporal bone segments, including regional involvement of the labyrinthine and tympanic segments of the facial nerve, basal turn of the cochlea, vestibule, IAC, tegmen tympani, and middle ear cavity including the ossicles. Though initially treated for Bell's palsy, our patient was ultimately diagnosed with a middle ear cholesteatoma that invaded the skull base and involved the facial nerve. The diagnosis was confirmed surgically. Cholesteatomas are benign masses comprised of abnormal squamous epithelium within the temporal bone. Over time, these masses can grow large enough to cause local bony destruction with surrounding inflammation and granulation tissue. Cholesteatomas are often classified into congenital and acquired types (primary or secondary). In the primary acquired type, cholesteatomas typically arise in the setting of chronic tympanic membrane (TM) retraction. Alternatively, secondary acquired cholesteatomas occur in the setting of TM perforation with epithelial migration into the middle ear space. Given the patient's history of q-tip injury and subsequent surgery, the cholesteatoma was most likely caused by traumatic implantation of squamous epithelium or iatrogenic, i.e., caused by the surgeon not removing or implanting squamous epithelium from the middle ear. Facial nerve palsy due to cholesteatoma has been rarely reported in the literature.1 While the mechanism by which cholesteatoma causes facial nerve palsy remains unclear, several theories have been proposed. The first hypothesis is that direct compression by the cholesteatoma is responsible for causing nerve edema and subsequent ischemia. A second hypothesis is that direct contact between the cholesteatoma and facial nerve promotes an inflammatory reaction that leads to injury. This theory is supported by histological studies showing degeneration of the epineurium in facial nerve segments exposed to cholesteatoma or granulation tissue.2 A third hypothesis is that nerve injury is mediated by neurotoxic or enzymatic substances secreted by the cholesteatoma, although the significance of these factors remains controversial.3 It is important to accurately diagnose and treat cholesteatomas, as they have a strong propensity to become infected and erode through local bony structures.4 The infections and associated pathogens in cholesteatoma can be especially hard to eradicate as they are frequently polymicrobial and resistant to antibiotics. Skull base invasion of cholesteatomas carries an increased risk of deafness, facial paralysis, and intracranial complications given their location. In this patient, we see the cholesteatoma is already eroding the cochlea, creating an increased likelihood of sensorineural hearing loss in the right ear. After evaluating the extent of the disease on CT scan, surgical treatment is undertaken with the goals of removing all of the cholesteatoma and repairing damaged structures when possible. Various surgical approaches can be used, depending on the involved structures as well as surgeon comfort level. In this patient, a right middle cranial fossa or translabyrinthine approach could be undertaken. Generally, when the hearing is intact, the best approach is the middle cranial fossa. The translabyrinthine approach is reserved for non-serviceable hearing patients. If the facial nerve function does not return, the patient may receive a hypoglossal-facial jump graft. In the future, a medical device in development may allow restoration of function for the patient.5-6 BONUS ONLINE VIDEOS: VISUAL DIAGNOSIS Read this month's Clinical Consultation case, then watch the accompanying videos from Hamid R. Djalilian, MD, to review the patient's imaging for yourself. Video 1. Axial (horizontal) CT of the right temporal bone showing the extent of the cholesteatoma in the axial plane and involvement of tympanic facial nerve and geniculate ganglion. Video 2. Coronal (vertical parallel to ear) CT of the right temporal bone showing the extent of the cholesteatoma in the coronal plane and invasion of the tegmen and IAC. Video 3. Sagittal (vertical parallel to face) CT of the right temporal bone showing the extent of the cholesteatoma in the sagittal plane and invasion of the cochlea. Video 4. Axial (horizontal) CT of the left temporal bone showing the normal anatomy of the facial nerve in the axial plane. Video 5. Coronal (vertical parallel to ear) CT of the left temporal bone showing the normal anatomy of the tegmen tympani. Video 6. Sagittal (vertical parallel to face) CT of the left temporal bone showing the normal cochlear anatomy in the sagittal plane. Watch the patient videos online at thehearingjournal.com

The revision of MEDLINE from 1966 to 2003 did not report any association between multiple sclerosis (MS) and Melkersson-Rosenthal syndrome (MRS). This is a case report of a 51-year-old woman, with history of four recurrent Bell's palsies. In 1999 she developed a right facial paralysis due to a supranuclear pyramidal lesion with right monoparesis. The family history showed five relatives with recurrent Bell's paralysis and plicata tongue. right Bell's paralysis, left supranuclear facial paralysis, furrowed tongue, right hemiparesis with pallor of the optic disks. Brain magnetic resonance imaging (MRI) demonstrated the typical lesions of MS and CSF oligoclonal bands. This is the first observation of a patient with hereditary MRS and MS. The link between both diseases is discussed.

The aim of this study is to evaluate the types and clinical characteristics of peripheral facial palsy in children. The hospital charts of children diagnosed with peripheral facial palsy were reviewed retrospectively. A total of 81 children (42 female and 39 male) with a mean age of 9.2 ± 4.3 years were included in the study. Causes of facial palsy were 65 (80.2%) idiopathic (Bell palsy) facial palsy, 9 (11.1%) otitis media/mastoiditis, and tumor, trauma, congenital facial palsy, chickenpox, Melkersson-Rosenthal syndrome, enlarged lymph nodes, and familial Mediterranean fever (each 1; 1.2%). Five (6.1%) patients had recurrent attacks. In patients with Bell palsy, female/male and right/left ratios were 36/29 and 35/30, respectively. Of them, 31 (47.7%) had a history of preceding infection. The overall rate of complete recovery was 98.4%. A wide variety of disorders can present with peripheral facial palsy in children. Therefore, careful investigation and differential diagnosis is essential.