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     

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.     

The triad of neurologic manifestations of Lyme disease: meningitis, cranial neuritis, and radiculoneuritis

We studied 38 patients with Lyme meningitis, a newly recognized spirochetal infection. The patients characteristically had intermittent attacks of severe headache, mild meningismus, and a predominantly lymphocytic pleocytosis. In addition to meningitis, 11 patients experienced subtle encephalitic signs, 19 had cranial neuritis, most commonly unilateral or bilateral facial palsy, and 12 developed peripheral radiculoneuritis, plexitis, or mononeuritis multiplex. Without antibiotic therapy, the duration of neurologic involvement was 3 to 18 months. Although sometimes incomplete, the triad of neurologic manifestations of Lyme disease--meningitis, cranial neuritis, and radiculoneuritis--presents a unique clinical picture.     

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.     

Lyme borreliosis in Bell's palsy. Long Island Neuroborreliosis Collaborative Study Group.

Lyme borreliosis (LB) causes a range of neurologic manifestations, the most common of which is facial nerve paralysis. To evaluate nervous system LB, we organized a neurologic collaborative study group in Suffolk County, NY, a region of high LB incidence. Between July and September 1989, LB serologies were performed on all patients with new-onset Bell's palsy. Seven of 32 had serologic evidence of LB at onset. One, initially seronegative, was highly seropositive 5 weeks later. In the five in whom we examined CSF, there was no evidence of intrathecal synthesis of specific antibody. In highly endemic areas, LB may be responsible for 1/4 of cases of Bell's palsy. Rarely, the palsy may occur prior to the development of a measurable antibody response, indicating a need for follow-up serologic testing.     

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).     

Molecular mimicry and Lyme borreliosis: a shared antigenic determinant between Borrelia burgdorferi and human tissue

The pathogenesis of chronic manifestations in Lyme borreliosis, a disease induced by Borrelia burgdorferi, is at present unresolved. By testing monoclonal antibodies directed against various borrelia antigens, we found an antigenic determinant shared by the 41 kDa flagella protein and human tissue, especially prominent on myelinated fibers of human peripheral nerve, on nerve cells and axons of the central nervous system, as well as on certain epithelial cells (including joint synovia) and on heart muscle cells. Immune reactions against such a shared antigen could play a pathogenetic role in chronic organ manifestations of Lyme borreliosis.     

Clinical features of double infection with tick-borne encephalitis and Lyme borreliosis transmitted by tick bite

Background

In Latvia and other endemic regions, a single tick bite has the potential to transmit both tick‐borne encephalitis (TBE) and Lyme borreliosis.

Objective

To analyse both the clinical features and differential diagnosis of combined tick‐borne infection with TBE and Lyme borreliosis, in 51 patients with serological evidence, of whom 69% had tick bites.

Results

Biphasic fever suggestive of TBE occurred in 55% of the patients. Meningitis occurred in 92%, with painful radicular symptoms in 39%. Muscle weakness occurred in 41%; in 29% the flaccid paralysis was compatible with TBE. Only two patients presented with the bulbar palsy typical of TBE. Typical Lyme borreliosis facial palsy occurred in three patients. Typical TBE oculomotor disturbances occurred in two. Other features typical of Lyme borreliosis detected in our patients were distal peripheral neuropathy (n = 4), arthralgia (n = 9), local erythema 1–12 days after tick bite (n = 7) and erythema chronicum migrans (n = 1). Echocardiogram abnormalities occurred in 15.

Conclusions

Patients with double infection with TBE and Lyme borreliosis fell into three main clinical groups: febrile illness, 3 (6%); meningitis, 15 (30%); central or peripheral neurological deficit (meningoencephalitis, meningomyelitis, meningoradiculitis and polyradiculoneuritis), 33 (65%). Systemic features pointing to Lyme borreliosis were found in 25 patients (49%); immunoglobulin (Ig)M antibodies to borreliosis were present in 18 of them. The clinical occurrence of both Lyme borreliosis and TBE vary after exposure to tick bite, and the neurological manifestations of each disorder vary widely, with considerable overlap. This observational study provides no evidence that co‐infection produces unusual manifestations due to unpredicted interaction between the two diseases. Patients with tick exposure presenting with acute neurological symptoms in areas endemic for both Lyme borreliosis and TBE should be investigated for both conditions. The threshold for simultaneous treatment of both conditions should be low, given the possibility of co‐occurrence and the difficulty in ascribing individual neurological manifestations to one condition or the other.The Baltic region is an endemic focus for both tick‐borne encephalitis (TBE) and Lyme borreliosis transmitted by ticks.^1,2,3,4 In Latvia, 7061 cases of TBE and 3566 cases of Lyme borreliosis were registered between 1994 and 2003, out of a population of 2.4 million. Both tick species present in Latvia, Ixodes ricinus and persulcatus, can transmit the encephalitis virus, the borreliosis spirochete and more rarely erlichiosis. A single tick bite has the potential to transmit both infections.⁵ Despite their different clinical courses, TBE and Lyme borreliosis have neurological features in common: lymphocytic meningitis, flaccid or spastic limb weakness and cranial nerve involvement. Thus, differentiating between these disorders is important, given different approaches to treatment.Of the two infections, only TBE runs a biphasic course with the initial prodomal period of influenza‐like symptoms usually developing 1–2 weeks after the tick bite. Hence, after an asymptomatic period lasting 2–10 days, about a third of infected patients enter a second phase with aseptic meningitis.² Subsequently, 2–10% in Western TBE subtype or 10–25% in Eastern TBE subtype develop encephalitis, myelitis or meningoencephalomyelitis typically manifesting as combinations of flaccid paresis of the limbs, usually arms and neck, bulbar dysfunction, disorientation, aphasia and spastic paresis.^1,2 A poliomyelitis‐like syndrome is described in central European TBE.⁶ Manifestations of TBE in the Baltic may be heterogeneous, given that infection with the Western, Far Eastern and Siberian subtypes all cause human infection in Latvia.⁷ Although severe manifestations usually subside after 3–6 weeks, the convalescence period of TBE may be very long, with nearly 40% having a postencephalitic syndrome at 4 years.⁸ The uptake of TBE vaccination is increasing in the Baltic region.Classical Lyme borreliosis differs considerably from TBE and produces local and generalised forms, systemic involvement, and development over several stages. Its acute and chronic courses pose problems of diagnosis and management.^1,9 Diagnosis of neuroborreliosis requires a definite or possible tick bite, erythema migrans or seropositivity, and typical peripheral or central nervous system involvement.¹⁰ In early neuroborreliosis (2–10 weeks after tick bite) the most common neurological abnormalities are meningitis, meningoradiculoneuritis and cranial neuritis, particularly facial palsy.^1,9,10,11 Progressive chronic encephalomyelitis, polyneuritis and cerebrovascular disorders are later manifestations of Lyme borreliosis, usually occurring months after the initial infection. Neurological features are noted in 10–12% of all patients with Lyme borreliosis in Europe¹ and in 10–15% of patients in Northern America.¹¹ Neurological manifestations in 330 European patients with Lyme borreliosis included radicular pain (70%), headache (18%), peripheral paresis (45%), central paresis (4%), sensory disturbances (44%) and facial palsy (39%).¹ Borrelia infection takes a subclinical or minimally symptomatic course in up to 80% of the population after tick bites.¹² Importantly, borreliosis is treatable with antibiotics.TBE infection can be proven by specific and sensitive ELISA detection of antibody in cerebrospinal fluid (CSF), or by detection of genome through polymerase chain reaction.¹³ Serum IgM antibodies can remain positive for ⩾10 months.^2,14 By contrast, serological tests for Lyme borreliosis infection are less sensitive and specific to variable onset and occurrence of specific IgM and IgG antibodies, with recognised persistent seronegatives; direct detection of a pathogen is rarely possible, and reliance must be placed on interpreting the laboratory investigations in the light of the clinical picture.^13,15,16 Demonstration of intrathecal antibody production provides a specific test,¹⁷ but is not sensitive in detecting all forms of neuroborreliosis.¹⁵ Despite their different clinical courses, TBE and Lyme borreliosis have neurological features in common: lymphocytic meningitis, flaccid or spastic limb weakness, and cranial nerve involvement. Pain, particularly in a radicular distribution, and sensory disturbance are regarded as features more typical of Lyme borreliosis than TBE.Only limited information on double infection with TBE and Lyme borreliosis is available. Single cases, small series or serologically defined series with limited clinical information are described from Germany, Slovenia, Central Russia and Finland.^{18,19,20,21,22,23,24} This retrospective clinical observational study analyses the clinical features and problems of differential diagnosis in patients with evidence of both TBE and Lyme borreliosis infection in Latvia.     

Isolated monolateral neurosensory hearing loss as a rare sign of neuroborreliosis

Abstract. Lyme disease, or borreliosis, is a zoonosis transmitted by Borrelia burgdorferi which also involves the central nervous system (CNS), in 15% of affected individuals, with the occurrence of aseptic meningitis, fluctuating meningoencephalitis, or neuropathy of cranial and peripheral nerves. Encephalopathy with white matter lesions revealed by magnetic resonance imaging (MRI) scans in late, persistent stages of Lyme disease has been described. In this report, we describe a patient with few clinical manifestations involving exclusively the eighth cranial nerve, monolaterally and diffuse bilateral alterations of the white matter, particularly in the subcortical periventricular regions at cerebral MRI. This single patient study shows that the search for antibodies against Borrelia burgdoferi should always be performed when we face a leukoencephalopathy of unknown origin. An isolated lesion of the eighth cranial nerve can be the only neurologic sign in patients with leukoencephalopathy complicating Lyme disease.     

Carpal tunnel syndrome in Lyme borreliosis

Neurophysiologic evidence of median nerve entrapment in the carpal tunnel was present in 25% of patients with late Lyme borreliosis. Sixty-eight of 76 consecutive, prospectively studied patients with late Lyme underwent neurophysiologic testing. Nineteen reported intermittent hand paresthesias; 17 had neurophysiologically confirmed carpal tunnel syndrome. This was not consistently associated with clinically apparent wrist arthritis or with neurophysiologically evident peripheral neuropathy. We conclude that a significant proportion of patients with late Lyme borreliosis develop carpal tunnel syndrome.     

Chronic polyneuropathy and Lyme disease

Infection of the peripheral nervous system with Borrelia burgdorferi can present as a cranial neuropathy or radiculopathy with cerebrospinal fluid (CSF) pleocytosis and intrathecal antibody production against B. burgdorferi , or as an asymmetric peripheral neuropathy with acrodermatitis chronica atrophicans (ACA) and normal CSF findings. According to North American studies, it can also present as a symmetric chronic polyneuropathy without ACA or other Lyme manifestations. Our purpose was to investigate the prevalence of B. burgdorferi antibodies in patients presenting with isolated chronic polyneuropathy (PN) in a European region with high incidence of Lyme disease. Sera from 209 PN patients and 247 healthy blood donors from Vest-Agder County, Norway, were examined. Borrelia burgdorferi antibodies were detected in 43 (21%) PN patients and in 45 (18%) healthy blood donors ( P  = 0.553). The prevalence of B. burgdorferi antibodies was similar ( P  = 0.311) in cryptogenic PN (24/102, 24%) and PNs of identified etiologies (19/107, 18%). PN patients with B. burgdorferi antibodies had normal spinal fluid white cell count and they did not differ clinically or electrophysiologically from PN patients without antibodies. None of 20 antibody-positive PN patients responded to antimicrobial treatment. The study shows that, in Europe, chronic distal PN without ACA or other Lyme manifestations is very rarely caused by a B. burgdorferi infection.     

PCR detection of Borrelia burgdorferi DNA in cerebrospinal fluid of Lyme neuroborreliosis patients.

We used the polymerase chain reaction (PCR), a method useful in the detection of Borrelia burgdorferi in vitro, to evaluate CSF in patients thought to have neuroborreliosis. Nested pairs of oligonucleotide primers were designed to recognize the C-terminal region of B burgdorferi OspA. CSF samples were obtained from (1) patients with immunologic evidence of systemic B burgdorferi infection and clinical manifestations suggestive of CNS dysfunction, (2) seronegative patients with clinical disorders consistent with Lyme borreliosis, and (3) patient and contamination controls; all were analyzed in a blinded fashion. PCR detected B burgdorferi OspA DNA in CSF of (1) 10 of 11 patients with Lyme encephalopathy, (2) 28 of 37 patients with inflammatory CNS disease, (3) seven of seven seronegative patients with Lyme-compatible disorders, and (4) zero of 23 patient controls. Zero of 83 additional contamination controls were PCR-positive. In eight patients from whom we obtained CSF before and after parenteral antimicrobial therapy, PCR results invariably predicted clinical outcome accurately.     

Magnetic resonance imaging findings in bilateral Bell's palsy.

Bell's palsy (idiopathic facial paralysis) is the most common cause of unilateral peripheral facial neuropathy. Bilateral involvement occurs in less than 10% of cases. The authors describe a 20-year-old man with bilateral idiopathic facial weakness. Brain magnetic resonance imaging (MRI) showed abnormal bilateral enhancement of the proximal intracanalicular segments of VII/VIII nerve complexes. The enhancement was most prominent in the leptomeningeal regions. There was no facial nerve swelling. Three months later he had improving residual bifacial weakness. To the authors' knowledge, this is the first report of abnormal MRI findings in bilateral Bell's palsy.     

Prevalence of taste disorders in idiopathic and B. burgdorferi-associated facial palsy

The frequency of taste disorders in idiopathic facial palsy (IFP) and B. burgdorferi-associated facial palsy (BFP) was retrospectively assessed in a cohort of patients with acute peripheral facial palsy (AFP). A significant (>10/μl) CSF pleocytosis was found in 17% of the patients who underwent lumbar puncture for AFP. In two centres, 26 patients with BFP were identified by CSF and serological criteria. The control group (patients with IFP) consisted of 59 patients from one of the centres in whom BFP was excluded by CSF examination. AFP patients of both centres are routinely questioned about taste disorders according to the hospitals’ standards. A taste disorder was found in 46% of the IFP and 31% of the BFP cases (not significant). About one-third of the BFP patients complained of radicular or back pain. We conclude that a history of taste disorder is not helpful in distinguishing clinically between BFP and IFP.     

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.     

Reactivation of type 1 herpes simplex virus and varicella zoster virus in an immunosuppressed patient with acute peripheral facial weakness

We describe a 26-year-old man treated with azathioprine for myasthenia gravis who developed acute left-sided peripheral facial weakness. Brain magnetic resonance imaging (MRI) revealed enhancement in the left geniculate ganglion and in the intracanalicular and tympanic segments of the facial nerve. Analysis of cerebrospinal fluid (CSF) and serum revealed intrathecal synthesis of anti-varicella zoster virus (VZV) IgG antibody. Although previous analyses of saliva, blood mononuclear cells, serum antibodies, middle ear fluid, and auricular and geniculate zone skin scrapings have shown that a small but definite proportion of patients with idiopathic peripheral facial palsy ("Bell's palsy") have the Ramsay Hunt syndrome zoster sine herpete (RHS ZSH), this is the first confirmation of RHS ZSH by intrathecal synthesis of anti-VZV IgG antibody. In addition, herpes simplex virus (HSV)-1 DNA was found in saliva of the patient on 3 consecutive days. Simultaneous reactivation of two alphaherpesviruses (HSV-1 and VZV) in our immunosuppressed patient underscores the need to consider opportunistic infection as a cause of facial weakness.     

Cefotaxime vs penicillin G for acute neurologic manifestations in Lyme borreliosis. A prospective randomized study

We randomly assigned 21 patients with painful Lyme neuroborreliosis radiculitis (Bannwarth's syndrome) and neuroborreliosis meningitis to a 10-day treatment with either penicillin G. 4 x 5 million U/d (n = 10) or cefotaxime sodium, 3 x 2 g/d (n = 11), intravenously. We were not able to demonstrate clinical differences between groups, either during the 10-day treatment period or at follow-up examination a mean of 7.7 months after antibiotic therapy. Cerebrospinal fluid cefotaxime concentrations reached the minimum inhibitory concentration at the 90% level for Borrelia burgdorferi in all patients, while none of the patients treated with penicillin G had cerebrospinal fluid concentrations above the minimum inhibitory concentration at the 90% value. We conclude that patients with acute neurologic manifestations of Lyme borreliosis may benefit from a 10-day treatment with cefotaxime or penicillin G. Cerebrospinal fluid antibiotic concentrations above the minimum inhibitory concentration at the 90% value, as observed in all patients treated with cefotaxime, offer the most hope for long-term prognosis.     

脑桥梗死的临床与神经影像学特征

目的阐述脑桥梗死的临床-影像-解剖学关系。方法回顾性分析69例急性孤立性脑桥梗死患者的临床特征及其与神经影像学改变的对应关系。结果主要临床特征有病灶对侧的中枢性面瘫和肢瘫、眩晕、构音障碍、偏身麻木、饮水呛咳、眼球水平运动异常等。9例（13．04％）患者在发病早期呈现进展性过程；51例（73．91％）梗死灶位于脑桥旁正中区域；59例（85．51％）患者病灶位于脑桥中上部水平；45例血管造影患者中．9人有椎-基底动脉狭窄。结论脑桥梗死多发生在脑桥中上部的旁正中区域，主要由椎-基底动脉狭窄、高血压性基底动脉穿通支闭塞所致。偏瘫、眩晕、构音障碍、感觉异常及眼球水平运动障碍等为主要临床表现。经典的脑桥综合征少见。少数患者在病程初期呈现进展过程，大多数患者预后良好。