Absence of Lyme borreliosis among patients with presumed Bell's palsy.

In a prospective study, 69 patients with a presumed idiopathic (Bell's) peripheral facial palsy were clinically and serologically evaluated for the presence of Lyme borreliosis. In addition, their clinical spectrum was compared with clinical manifestations collected retrospectively in nine patients with symptomatic peripheral facial palsy due to Lyme borreliosis. The seroprevalence of Borrelia burgdorferi antibodies, determined by flagellum enzyme-linked immunosorbent assay, among 69 patients with idiopathic peripheral facial palsy (6%) and 153 healthy controls (4.5%) was not significantly different (odds ratio, 1.28; 95% confidence interval, 0.27 to 5.25). None of the patients with idiopathic peripheral facial palsy had or experienced the development of Lyme borreliosis. All patients with Lyme peripheral facial palsy had additional manifestations not present in patients with idiopathic peripheral facial palsy. These findings show that patients with a Lyme peripheral facial palsy can be differentiated from patients with idiopathic peripheral facial palsy by clinical examination. Therefore, screening of antibodies to B burgdorferi among patients with idiopathic peripheral facial palsy without additional manifestations is not recommended.     

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.     

Acute Cerebellar Ataxia in a Pediatric Case of Lyme Disease and a Review of Literature

A broad range of neurologic disorders have been described in children with Lyme disease, of which peripheral facial nerve palsy and aseptic meningitis are among the most common. In contrast, there are few reports of cerebellar involvement in pediatric Lyme disease patients. We report the case of a 5-year-old girl seropositive for antibodies against the causative Lyme disease pathogen Borrelia burgdorferi presenting with severe acute cerebellar ataxia from the in southern coast of Anatolia (Mediterranean region).     

Autoantibodies to human manganese superoxide dismutase (MnSOD) in children with facial palsy due to neuroborreliosis

AIM: Acute peripheral facial palsy due to neuroborreliosis is associated with a distal neuritis. In patients with Lyme disease the activity of antioxidant enzymes is decreased. With respect to the pathogenesis of neuroborreliosis, sera of children with acute peripheral facial palsy were investigated for autoantibodies against human manganese superoxide dismutase (MnSOD), which were suspected of raising the oxidative injury of infected tissues. METHODS: Sera of 20 children with acute peripheral palsy with neuroborreliosis, sera of 20 children with facial palsy without reference to Lyme disease and sera of 14 blood donors were tested for antibodies against human MnSOD using an ELISA. RESULTS: The concentrations of IgM autoantibodies to MnSOD of the children with neuroborreliosis were significantly increased, compared with the two control groups. CONCLUSIONS: We propose that the antibodies detected block the protective effects of MnSOD resulting in an increased oxidative inflammation.     

EMG prognosis of facial palsy associated with herpes zoster oticus (Ramsay-Hunt's syndrome). A longitudinal study of 11 cases (author's transl

Eleven patients with peripheral facial palsy associated with geniculate herpes zoster (Ramsay-Hunt's syndrome) have been followed-up clinically and electromyographically. Each patient was examined three times: within the first week, at the end of the third week and 3-4 months after the onset of symptoms. Only in three cases the facial palsy evolved satisfactorily, with an almost total recovery within three weeks. In eight cases the recovery was delayed and incomplete, with residual, and often severe, hemifacial spasm. This study confirms the rather poor prognosis of peripheral facial palsy in Ramsay-Hunt's syndrome. The importance of detecting even slight signs of herpetic eruption in any case of apparently "idiopathic" peripheral facial palsy is emphasized.     

Annualized incidence and spectrum of illness from an outbreak investigation of Bell's palsy

BACKGROUND: There are limited clinical and epidemiological data on patients diagnosed with Bell's palsy. While investigating an apparent clustering of Bell's palsy, we sought to characterize the spectrum of illness in patients with this diagnosis. METHODS: A telephone survey of persons with idiopathic facial (Bell's) palsy in the Greater Toronto Area (GTA, population = 4.99 million) and Nova Scotia (population = 0.93 million) from August 1 to November 15, 1997 collected information on subject demographics, neurological symptoms, constitutional symptoms, medical investigation and management. Information regarding potential risks for exposure to infectious agents, past medical history, and family history of Bell's palsy was also collected. Subjects with other secondary causes of facial palsy were excluded. RESULTS: In the GTA and Nova Scotia, 222 and 36 patients were diagnosed with idiopathic facial (Bell's) palsy, respectively. The crude annualized incidence of Bell's palsy was 15.2 and 13.1 per 100,000 population in the GTA and Nova Scotia, respectively. There was no temporal or geographical clustering, and symptomatology did not differ significantly between the two samples. The mean age was 45 years, with 55% of subjects being female. The most common symptoms accompanying Bell's palsy were increased tearing (63%), pain in or around the ear (63%), and taste abnormalities (52%). A significant number of patients reported neurological symptoms not attributable to the facial nerve. CONCLUSION: No clustering of cases of Bell's palsy was observed to support an infectious etiology for the condition. Misdiagnosis of the etiology of facial weakness is common. Patients diagnosed with Bell's palsy have a variety of neurological symptoms, many of which cannot be attributed to a facial nerve disorder.     

Lyme borreliosis and cranial neuropathy

In a 2-year study of 37 consecutive adult patients with isolated cranial nerve affection of primarily unknown origin, seen at a neurological clinic, borrelia infection was identified as the cause in six cases. Four patients had a peripheral facial palsy and two had a sixth nerve palsy. The patients with borreliosis had headaches or other pain considerably more often than patients with other or unknown aetiology. All six patients had accompanying symptoms and/or signs; in five cases these were obvious, and pointed to a borrelia infection. This study indicates that a careful history to elicit other symptoms of Lyme borreliosis will usually identify the cranial nerve affections with borrelial aetiology in adult patients. To verify the diagnosis, both serum and CSF analysis should be performed. Routine testing for borrelia serology in all patients with cranial neuropathy is generally not indicated.     

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     

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.     

The role of facial nerve conduction studies and electromyography in predicting the outcome of Bell's palsy

Many electrophysiological tests have been used to determine prognosis and extent of recovery in Bell's palsy but the reliability and sensitivity of the different parameters used is still controversial. We performed bilateral percutaneous facial nerve conduction studies, and volitional needle electromyography on 23 patients within 10–14 days post onset of their Bell's palsy. The following parameters were assessed: denervation and recruitment of the frontalis and orbicularis oris muscles, latency of the compound muscle action potential (CMAP), and CMAP amplitude ratio. The patients were re-examined 6 months later and their recovery graded according to the House-Brackman classification. The CMAP amplitude ratio and the recruitment scores of the frontalis and orbicularis oris muscles were the only parameters to reliably predict outcome (p = 0.016, 0.007 and 0.036, respectively). All patients with a CMAP amplitude ratio above 10% had a complete recovery. Since Bell's palsy is probably caused by herpes simplex virus, the active disease process is completed within 10–14 days; therefore, facial nerve conduction studies and electromyography at that time are appropriate to predict prognosis.     

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.     

Astroglial and neuronal proteins in cerebrospinal fluid as markers of CNS involvement in Lyme neuroborreliosis

Is Lyme neuroborreliosis, even in its early phase, a parenchymatous disorder in the central nervous system (CNS), and not merely a meningitic process? We quantified cerebrospinal fluid (CSF) levels of four nerve and glial cell marker proteins in Lyme neuroborreliosis patients with pretreatment durations of 7–240 days. AH 23 patients had meningo-radiculitis, and six had objective signs of encephalopathy. Glial fibrillary acidic protein (GFAp) pretreatment levels in CSF, and the light subunit of neurofilament protein (NFL) levels were related to clinical outcome and declined significantly after treatment (P < 0.001 and P < 0.01, respectively). NFL was detectable in 11 out of 22 patients, and pre-and post-treatment NFL levels were associated with the duration of neurological symptoms within 100 days prior to treatment. Neuron-specific enolase (NSE) concentrations also decreased after therapy (P < 0.001), while CSF levels of glial S-100 protein remained unchanged. The pretreatment duration of disease was related to postinfectious sequelae. GFAp, NSE and NFL levels in CSF are unspecific indicators of astroglial and neuronal involvement in CNS disease. The findings in the present study are in agreement with the hypothesis that early and late stages of Lyme neuroborreliosis damage the CNS parenchyma.     

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.     

The phenomenon of ‘chronic Lyme’; an observational study

Purposes: To chart clinical, laboratory, and psychometric profiles in patients who attribute their complaints to chronic Lyme disease. Methods: We assessed the patients by clinical examination, laboratory tests, and questionnaires measuring fatigue, depression, anxiety, health‐related quality of life, hypochondriasis, and illness perceptions. Results: We found no evidence of ongoing Borrelia burgdorferi (Bb) infection in any of the 29 included patients using current diagnostic guidelines and an extended array of tests. Eight (28%) had other well‐defined illnesses. Twenty‐one (72%) had symptoms of unknown cause, of those six met the suggested criteria for post‐Lyme disease syndrome. Fourteen (48%) had presence of anti‐Bb antibodies. The patients had more fatigue and poorer health‐related quality of life as compared to normative data, but were not more depressed, anxious, or hypochondriacal. Their beliefs about the illness were characterized by negative expectations. Conclusion: Our patients, who all attributed their symptoms to chronic Lyme disease, were heterogeneous. None had evidences of persistent Bb infection, but whether current diagnostic criteria are functional in patients with longstanding complaints is controversial. Other well‐defined illnesses or sequelae from earlier Lyme disease were probable as main explanatory factor in some cases. The patients were not more depressed, anxious, or hypochondriacal than the normal population, but they had poorer health‐related quality of life, more fatigue, and negative expectations about their illness.     

Electrodiagnosis of the cranial nerves

Isolated facial weakness suggests either a contralateral hemispheric lesion or a disease of the facial nerve per se. The presence of sensory symptoms usually indicates a central facial weakness, which characteristically involves the lower part of the face. In contrast, the absence of sensory disturbances suggests a peripheral nerve lesion, some system diseases such as amyotrophic lateral sclerosis, or a stroke sparing the sensory cortex. Sporadic cases of Bell's palsy rank the first in incidence. Although its exact etiology remains unknown, accumulating evidence suggests reactivation of herpes simplex virus type I. A facial palsy that develops in patients with diabetes mellitus tends to show a more severe involvement with substantial denervation. Acoustic neuroma, strategically located at the cerebellopontine angle, may compress the facial nerve. Peripheral facial palsy may herald other symptoms of multiple sclerosis in young adults. Serial electrodiagnostic studies help delineate the course of the illness. The amplitude of the direct response elicited by stimulation of the facial nerve after the fourth to fifth day of onset serves as the best means predicting the eventual outcome of recovery. Blink reflex studies usually show an absent or delayed R1, implicating the central reflex arc, which includes the intrapontine portion of the facial nerve.     

Delayed facial weakness in Guillain‐Barré and miller fisher syndromes

Introduction: Dr. C. Miller Fisher described the appearance of unilateral facial palsy after resolution of ataxia in a patient with the eponymic Miller Fisher syndrome (MFS). However, there have been very few reports of delayed appearance of facial weakness in Guillain‐Barré syndrome (GBS) and MFS when the other neurological signs reached nadir or started improving. Methods: In this study we reviewed the clinical and laboratory findings of consecutive patients with GBS (n = 195) and MFS (n = 68). Results: Delayed facial weakness occurred in 12 (6%) GBS and 4 (6%) MFS patients and was unilateral in 5 (42%) GBS and 2 (50%) MFS patients. In those patients with delayed facial weakness, neither limb weakness nor ataxia progressed, and facial weakness disappeared without immunotherapy. Conclusions: Because facial weakness can lead to further morbidity, it would be prudent for clinicians to warn patients of this possibility, although additional immunotherapy is usually not required. Muscle Nerve 51 : 811–814, 2015     

Bilateral facial nerve palsy associated with COVID‐19 and Epstein–Barr virus co‐infection

COVID‐19 can occasionally be associated with cranial nerve involvement, but facial palsy, particularly if bilateral, is exceptional. We here report a patient who presented with severe bilateral facial palsy and evidence of SARS‐CoV‐2 infection preceded by upper respiratory symptoms. He also had serological evidence of coinfection with Epstein‐Barr virus, which could have also played a role in his neurological manifestations. PCR in the cerebrospinal fluid was negative for both EBV and SARS‐CoV‐2, which suggests an indirect, immune‐mediated mechanism rather than direct, viral‐induced damage. The patient was treated with prednisone 60 mg/24h with a tapering schedule and had a favorable outcome, with an almost complete recovery in 3 weeks. SARS‐CoV‐2 adds to the list of infectious agents causative of bilateral facial palsy. Coinfection with SARS‐CoV‐2 is not rare and should be considered in the differential diagnosis.     

Exploring sexual problems among patients with multiple sclerosis

Bosco D, Plastino M, Bosco F, Consoli A, Labate A, Pirritano D, Consoli D, Fava A. Bell’s palsy: a manifestation of prediabetes?
Acta Neurol Scand: 2011: 123: 68–72.
© 2010 John Wiley & Sons A/S. Background – Idiopathic peripheral facial nerve palsy or Bell’s palsy (BP) is the most common cause of facial nerve palsy. Objective – To evaluate the role of glucose metabolism abnormalities in BP. Methods– We identified 148 patients with unilateral BP and 128 control subjects. In all we evaluated glucose level at fasting and after a 2‐h oral glucose tolerance test (2h‐OGTT). In addition we determined insulin resistance (IR), by HOMA‐index. Patients and controls were divided in to two groups, according to their Body Mass Index (BMI). Results – Following a 2h‐OGTT, the prevalence of glucose metabolism abnormalities was significantly higher in patients with BP than in controls (P < 0.001). Impaired glucose tolerance (IGT) was found in 57 (38%) patients and in 23 (18%) controls, while a new‐diagnosed DM (NDDM) was found in 29 (19%) patients and in 8 (6%) controls. The IR was significantly increased only in BP patients with BMI ≥ 24.9 (P = 0.005). BMI, waist circumference, blood pressure, tryglicerides, serum lipid, drugs use were not significantly different between patients and controls. Conclusions – In this study we found that prediabetes is frequently associated with facial palsy. We propose to perform a 2h‐OGTT in patients with peripheral facial palsy and normal fasting glycaemia. HOMA‐index should be evaluated in obese facial palsy patients.     

Early diagnosis of zoster sine herpete and antiviral therapy for the treatment of facial palsy

The effect of antiviral agents on recovery from facial palsy in patients with zoster sine herpete (ZSH; varicella zoster virus reactivation without zoster) has not been evaluated because ZSH is difficult to diagnose early after onset. In this study, all 13 patients who received acyclovir-prednisone treatment within 7 days of onset, as confirmed by a positive PCR result, showed complete recovery. PCR-based early diagnosis of ZSH and antiviral therapy elicited an excellent outcome for recovery from facial palsy due to ZSH.