Bell's palsy in children: analysis of clinical findings and course

To evaluate treatment of Bell's palsy (acute idiopathic peripheral facial nerve paralysis) of children, the authors analyzed 38 cases (18 females, 20 males) of Bell's palsy in children aged below 16 years old. The mean age of all cases was 6.8 +/- 6.2 years old. All cases resulted in complete recovery within 6 months. Clinical score of facial motor functions were adapted to 17 patients who were more than 5 years old. They were divided into two groups: early recovery group (clinical symptoms recovered within 3 months; 10 cases) and later recover group (over 3 months; 7 cases). Clinical scores evaluated in the first week from the onset were not significantly different. Steroid therapy was used for 9 patients of early group and 6 patient of later group. All patients of this study were recovered, thus we could not evaluate effect of steroid therapy for Bell's palsy in children. Use of steroid therapy for Bell's palsy needs more concretely administration. We consider how the region locates near to the center is an important prognostic factor.     

Facial palsy in Kawasaki syndrome

Facial nerve palsy, a very rare complication of Kawasaki syndrome, has been reported in only 25 patients. We treated a 12-week-old boy with bilateral coronary artery aneurysms due to Kawasaki syndrome who developed marked unilateral peripheral facial nerve palsy on day 36 of illness. None of the 25 previously reported patients with this complication were treated with immunoglobulin; they required 7 to 90 days to recover. In our patient, treatment with this agent was associated with complete resolution of facial nerve palsy within 36 hours. Review of prior cases demonstrates that children with Kawasaki-associated facial nerve palsy have more than twice the risk for coronary artery aneurysm (52% vs <25%) as that of children who do not develop this neurological complication. Unexplained facial nerve paralysis in young children with a prolonged febrile illness should provoke consideration of Kawasaki syndrome and of echocardiography to exclude coronary artery aneurysms. Although facial palsy appears likely to resolve in all patients that survive the acute phase of Kawasaki syndrome, treatment with intravenous immunoglobulin appears to considerably shorten the time to full recovery and provides an important clue to the mechanisms of neurological injury in this illness.     

Ramsay Hunt syndrome

The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. Hunt's analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndrome. Compared with Bell's palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely. Studies suggest that treatment with prednisone and acyclovir may improve outcome, although a prospective randomised treatment trial remains to be undertaken. In the only prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy. Further, Bell's palsy is significantly associated with herpes simplex virus (HSV) infection. In the light of the known safety and effectiveness of antiviral drugs against VZV or HSV, consideration should be given to early treatment of all patients with Ramsay Hunt syndrome or Bell's palsy with a 7-10 day course of famciclovir (500 mg, three times daily) or acyclovir (800 mg, five times daily), as well as oral prednisone (60 mg daily for 3-5 days). Finally, some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva. This indicates that a proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete. Treatment of these patients with acyclovir and prednisone within 7 days of onset has been shown to improve the outcome of recovery from facial palsy.     

Peripheral facial paralysis and HIV infection: report of four African cases and review of the literature

Summary Four cases of infranuclear facial palsy associated with infection by the human immunodeficiency virus in young heterosexual African patients are reported. Two cases were healthy HIV carriers, one patient manifested AIDS-related complex, and one case fulfilled the CDC criteria for AIDS. Two patients had a typical Bell's palsy, one presented with manifest cephalic Herpes zoster infection and one, who suffered from facial diplegia, could be considered to have a cephalic form of Guillain-Barré syndrome. A review of the literature confirmed that peripheral facial palsy can occur at any stage of HIV infection and in various clinical contexts. In stages I and II of the HIV infection, patients may develop either Bell's palsy or Guillain-Barré syndrome. In stages III and IV, when the cellular immunity has begun to decline, Herpes zoster-related facial paralysis, seventh cranial nerve involvement secondary to meningeal lymphomatosis, and peripheral facial paralysis as one aspect of widespread chronic peripheral neuropathy may also occur.     

Melkersson-Rosenthal综合征(附3例报告)

目的 探讨Melkersson-Rosenthal综合征（MRS）的临床特点及发病机制。方法 分析本院近10年来收治的3例完全型MRS患者临床及随访资料。结果 3例患者临床资料符合完全性MRS的诊断，即间歇性面瘫、再生性唇面肿及皱襞舌，其中1例有家族性皱襞舌。结论 MRS发病机制可能与免疫功能异常所致组织炎性肉芽肿有关，激素治疗有效。     

A case of brainstem encephalitis caused by herpes simplex virus type 1 with possible infection via trigeminal nerve]

A 24-year-old man was admitted to our hospital because of consciousness disturbance, a stiff neck and various brainstem symptoms including a right one-and-a-half syndrome and right peripheral facial palsy a week after an episode of pharyngitis and right facial herpes simplex. Magnetic resonance imaging of the brain on admission showed high-signal intensities in the right pontine tegmentum, right cerebellar peduncle and vermis on fluid-attenuated inversion recovery imaging. Examination of cerebrospinal fluid yielded mononuclear pleocytosis, elevated protein and increased IgM antibodies to herpes simplex virus (HSV) by enzyme immunoassay. HSV-1 specific antibodies also were detected in serum by neutralization test. We gave a diagnosis of brainstem encephalitis caused by HSV-1. The patient was successfully treated with high dose of acyclovir, steroid and intravenous immunoglobulin. He was discharged without any neurologic sequelae. We herein presented a case of atypical encephalitis due to HSV-1 involving mainly the brainstem with possible infection via right trigeminal nerve and summarized recent 35 cases with herpetic brainstem encephalitis since 1990.     

Abstracts

Bell’s palsy causes about two thirds of cases of acute peripheral facial weakness. Although the majority of cases completely recover spontaneously, about 30% of cases do not and are at risk from persisting severe facial paralysis and pain. It has been suggested that herpes simplex virus type 1 (HSV-1) may be the etiological agent that causes Bell’s palsy. Although corticosteroid therapy is now universally recognized as improving the outcome of Bell’s palsy, the question as to whether or not a combination of antiviral agents and corticosteroids result in a better rate of complete facial recovery compared with corticosteroids alone is now a highly contentious issue. The evidence obtained from laboratory studies of animals and humans that HSV-1 may be linked to facial nerve paralysis is first outlined. The discussion then focuses on the results of different clinical trials of the efficacy of antiviral agents combined with corticosteroids in increasing the rate of complete recovery in Bell’s palsy. These have often given different results leading to opposite conclusions as to the efficacy of antivirals. Of three recent meta-analyses of previous trials, two concluded that antivirals produce no added benefit to corticosteroids alone in producing complete facial recovery, and one concluded that such combined therapy may be associated with additional benefit. Although it is probably not justified at the present time to treat patients with Bell’s palsy with antiviral agents in addition to corticosteroids, it remains to be shown whether antivirals may be beneficial in treating patients who present with severe or complete facial paralysis.     

An adult case of peripheral facial nerve palsy following acute cerebellitis associated with high antibody titers against varicella-zoster virus]

Here we report a case of acute cerebellitis, in which the patient developed right peripheral facial palsy during the recovery phase of cerebellar ataxia. A 67-year-old man developed truncal and limb ataxia following a fever, general fatigue and anorexia. He was diagnosed to have acute cerebellitis. While the ataxia symptoms were improving without any treatment, right peripheral facial nerve palsy developed and an MRI revealed an enhancement of the right facial nerve proximal to the geniculate ganglion. After treatment with acyclovir and corticosteroids, his facial nerve palsy and ataxia both gradually improved. There has been no previous report of an adult case who developed peripheral facial nerve palsy during the recovery phase of acute cerebellitis. This case indicates that a wide spectrum of neurological complications may develop in association with a varicella-zoster virus infection.     

Melkersson-Rosenthal syndrome as a rare cause of recurrent facial nerve palsy

Melkersson-Rosenthal syndrome is a rare cause of recurrent facial nerve palsy. The syndrome is classically characterized by a triad of signs consisting of facial edema, recurrent peripheral facial nerve paralysis, and congenital fissured tongue, although it may also present in a mono- or oligosymptomatic form. The paper presents a 24-year-old woman with right-sided peripheral facial nerve palsy and a history of left-sided episodes of facial nerve palsy. Magnetic resonance angiography of the cerebral vessels suggested compression of the right seventh and eighth cranial nerves by a vascular loop. The authors describe the patient with a complete picture of Melkersson-Rosenthal syndrome and discuss the diagnosing process and treatment.     

Prognosis and course of Bell's palsy

Summary The clinical picture of ischemic palsy of the facial nerve was analyzed by symptoms in 87 patients. The level of the lesion of the facial nerve in the channel was determined by topesthesia. Mutagenic disorder usually causes incomplete or defective recovery. The speed of development of palsy does not affect its course. Retroaural pain has little significance in the prognosis of palsy. Recorvery may be defective even in cases where no pain is present. If the nasopalpebral reflex is absent and Bell's phenomenon is positive in the 3rd week the prognosis is infavourable. In half the patients no precipitating or etiological factor was found.Clinical appraisal was made in five muscles (M. frontalis, M. orbicularis oculi et oris, M. zygomaticus, M. depressor labii inferioris). The patients were divided into three groups according to the degree of their recovery: complete, incomplete and defective recovery. Recovery was defective in elderly patients with vascular hypertension. Voluntary activity in Group 1 (complete recovery) is symmetrical by the 1st–2nd month and in Group 2 (incomplete recovery) by the 5th–7th month; in Group 3 it is permanently insufficient.     

A case of typical Melkersson-Rosenthal syndrome with possible autosomal dominant inheritance]

Here we presented a case of 40-year-old woman who suffered from bilateral facial palsy and headache. She had allegedly had an episode of facial palsy, and facial edema at her age of 14 years. Physical examination revealed swelling of the lips, upward disturbance of the left eye, hypogeusia, the fissured tongue, and bilateral facial palsy. Oral administration of prednisolone 20 mg/day yielded gradual but complete improvement of the facial palsy and hypogeusia within two weeks. Careful analysis of family history disclosed that four members had oro-facio-cervical edema and three had the fissured tongue. A diagnosis of Melkersson-Rosenthal syndrome with possible autosomal dominant inheritance was made based on the clinical findings and familial aggregation of the incomplete form of this syndrome.     

Electrophysiologic findings and prognosis in Bell's palsy

Electrophysiologic investigations were carried out on 45 patients with Bell's palsy at periodic intervals after the onset of paralysis. It was found that there was a good correlation between prognosis in Bell's palsy and the amplitude of evoked motor response obtained after six or more days of clinical paresis. When the average amplitude of evoked motor response was within normal limits (i.e., 504μV or greater), complete recovery with no residual deficits took place two to six weeks after the onset of facial palsy. When the evoked motor response was absent in all three major branches of the facial nerve, indicating complete nerve degeneration, electromyographic signs of recovery were apparent by the third or fourth month after the onset of paralysis. In these cases, recovery was relatively slow and incomplete, with some degree of residual deficit and synkinesis. Maximal return of voluntary facial movement was established 8 to 12 months after the initial symptom. When the mean amplitude of evoked motor response was below the lower limit of normal (i.e., less than 504μV), electromyographic signs of recovery were noted within 1 to 3 months, depending on the amplitude values. The final outcome of this intermediate group was similar, but not identical, to that of the previous group. The prognosis of facial paralysis in Bell's palsy was thus found to be directly related to the mean amplitude of evoked motor response, regardless of the extent of clinical paralysis.     

Atypical Fisher syndrome with optic nerve involvement]

We report a 38-year-old woman who developed what appeared to be Fisher syndrome associated with optic nerve involvement. One week after a common cold, she developed double vision and left facial palsy. Four days after the onset, she developed bilateral blurred vision, painful total ophthalmoplegia, and ataxic gait. Brain CT and MRI findings were normal. Her vision worsened but the optic fundi were normal. Serum anti-GQ 1 b antibody was elevated. She received steroid therapy at another hospital, and her vision, facial palsy and ocular pain improved. She was transferred to our hospital and we treated her by plasma exchange. She showed near complete recovery. Human optic and ocular nerves contain high amount of GQ 1 b. This may be a underlying mechanism for optic nerve involvement in Fisher syndrome. We thought that she had an atypical Fisher syndrome associated with optic nerve involvement.     

Idiopathic cranial polyneuropathies]

A series of 43 cases with multiple cranial nerve deficits was collected between 1972 and 1990. No diagnosis was established in 15 cases. The facial and the trigeminal nerves were most frequently affected. In 10 cases, a monophasic course was observed usually with recovery. Recurrence was present in 5 cases. An inflammatory mechanism was likely in 10 cases. Nosological relations between these cases and either Bell's palsy (idiopathic facial paralysis) or Tolosa-Hunt's syndrome are discussed.     

Three new patients with congenital unilateral facial nerve palsy due to chromosome 22q11 deletion.

We report three unrelated patients with congenital facial nerve palsy and chromosome 22q11 deletion, a condition hitherto poorly recognized. In the first case, facial palsy was associated with aortic coarctation, ductus arteriosus, and ostium secundum atrial septal defect. In the second case, facial palsy was associated with ostium secundum atrial septal defect, obstruction of the ureteropelvic junction, double ureteropelvic-calicial system, and distal metaphyseal widening of the forearm and leg bones. In both cases, facial palsy was the presenting feature. In the third case, an ostium secundum atrial septal defect was also present, but involvement of cranial nerves III, VI, and VIII, in addition to hypoplastic structures of cerebellar and cerebral peduncles, were the predominant features. There were no inherited deletions within chromosome band 22q11 and the de novo deletions detected in each case belonged to the paternally derived chromosome 22. Association of facial nerve palsy and congenital heart disease versus cardiofacial syndrome are different only on clinical grounds, so both conditions can be genetically identical and form part of the spectrum of defects associated with chromosome 22q11 deletions. We recommend investigation for chromosome 22q11 deletions in patients with complete nerve facial palsy.     

Unusual and benign course of idiopathic facial diplegia

Bilateral facial nerve palsy or facial diplegia is a rare condition that occurs mainly in the context of Guillain–Barré syndrome. Its natural history has never been studied. We report four patients with isolated idiopathic bilateral facial nerve palsy with meningitis, no evidence of Guillain–Barré syndrome and rapid and complete recovery. Our report aims to draw attention to an unusual variant of bilateral facial palsy.     

Sjogren’s syndrome associated with bilateral peripheral facial paralysis

Sjogren’s Syndrome (SS) is a chronic autoimmune disorder that may be complicated by neurological dysfunctions. The involvement of cranial nerves in SS was described as a very rare complication. Moreover, bilateral peripheral facial paralysis associated with SS has been described only in 3 patients in the literature and the first case was described by Henrik Sjogren himself in 1935. We report a 59-year-old female with bilateral peripheral facial paralysis associated with Sjogren’s syndrome. She was treated with 5-day IVIG consecutively and continued oral methylprednisolone 16mg/day and almost fully recovered at 2 months of follow-up examination. Acute bilateral peripheral facial palsy in SS is a very rare condition and Lyme disease, Guillain-Barré syndrome, HIV infection, and central nervous system lymphoma should be considered in the differential diagnosis. As a result, SS should be considered as an underlying cause of bilateral facial paralysis.

Sjögren’s Syndrome (SS) is a systemic chronic autoimmune and vasculitic disorder characterized by lymphocytic infiltration of lacrimal and salivary glands.¹ In 39% of the affected cases, the disease can start with neurological findings. The most common peripheral involvement of SS is sensory neuropathies.²Peripheral facial paralysis associated with primary SS has been described in very few cases, and most of them were unilaterally affected.³ Only three had bilateral facial nerve involvement.⁴ Here, we described a patient with SS, who presented with bilateral facial nerve involvement.     

Acute peripheral facial palsy in adults

Objective To collect epidemiological data of peripheral facial palsy, and especially to chart the incidence and clinical characteristics of Lyme associated facial palsy.Material and methods We included consecutive adult patients presenting with peripheral facial palsy in Vest–Agder County from January 1997 to December 1998. The facial palsy was graded according to the House and Brackman facial function scoring system,and cerebrospinal fluid and serum samples were examined for Borrelia burgdorferi antibodies and virus antibodies. Final outcome was evaluated by follow up visits or telephone interview.Results Sixty nine patients were included and followed until complete recovery, or for 5 years. Ten per cent were caused by Lyme disease, 17% by virus infection, 4% by other causes and 68% were classified as Bell’s palsy. All patients with Lyme facial palsy had additional neurological symptoms, and 87% reported constitutional complaints. The overall final outcome was good with complete recovery in 77%, slight sequelae in 20% and moderate sequelae in 3%. No patients experienced severe sequelae. Two of 28 patients examined with neurography had absent compound muscle action potentials in orbicularis oculi. Both made good recovery with only slight sequelae.Conclusions Peripheral facial palsy is a common disorder with a favourable prognosis. Lyme disease seems to be an infrequent cause of facial palsy in patients without constitutional symptoms or additional neurological findings.     

Cerebrospinal fluid in acute peripheral facial palsy

Cerebrospinal fluid (CSF) is rarely analyzed in peripheral facial palsy, and reports in the literature are scarce. We report the CSF findings in 265 patients with acute isolated peripheral facial palsy. The CSF findings were abnormal in 11% of 230 patients with idiopathic peripheral facial palsy, in 60% of 17 patients with Ramsay Hunt syndrome (pleocytosis), in 25% of 8 patients with Lyme disease, in all of 8 patients with HIV infection, and in 2 other patients (sarcoidosis and herpes simplex). We conclude from this large series that the CSF is usually normal in idiopathic peripheral facial palsy. If the CSF is abnormal, a specific cause should be sought. Received: 17 October 1997 Accepted: 24 June 1998     

Value of the facial nerve latency test in the prognosis of childhood Bell's palsy

In the present study we evaluated the facial nerve latency test (FNLT) as a prognostic tool in cases of childhood Bell's palsy. Twenty-five children aged 4–14 years were studied. We divided our subjects into three groups according to duration of latency time (LT). Group A patients had an LT within the normal range, with average of 3.27 ms, group B a slightly prolonged LT averaging 5.7 ms, and group C a markedly prolonged LT averaging 10.5 ms. Analysis of the recovery index by group showed that group A patients experienced complete and quick recovery, while in group B 50% had complete but delayed recovery and 50% slightly impaired facial nerve function, and in group C 50% had slightly impaired function and 50% incomplete recovery. The more prolonged the LT, the worse the clinical results. The FNLT is thus a valuable prognostic tool in cases of Bell's palsy in childhood.