【摘要】 目的：报道1例罕见的视神经视网膜炎合并Bell麻痹。
方法：病例报道。
结果：男孩16岁，右眼视物模糊1wk就诊，伴高热以及两侧面部不对称。视力右眼0.1(6/60)，左眼1.0(6/6)。右眼眼底表现为视盘充血水肿及黄斑部存在星芒状皱褶。左侧下运动神经麻痹，Humphrey视野检查表现为旁中心暗点和生理盲点扩大。全血细胞记数显示中性粒细胞增多及血沉加快。血清学检测梅毒、弓形体病、莱姆病、猫抓病阴性。单纯疱疹、带状疱疹、艾滋病毒检测均为阴性。立克次体也为阴性，脑部及眼眶MRI均正常，未发现脱髓鞘病变。起始为甲强龙每日4次250mg静滴3d，出院后继续口服大剂量强的松1mg/kg 11d。6mo后，随访视力达1.0(6/6)，视盘略显苍白。Bell麻痹也明显改善，无明显两侧面部不对称。
结论：对于视神经炎，临床医生检查其他颅神经有无病变是非常重要的。视神经视网膜炎同时合并面神经麻痹非常罕见，需要进行全面的检查和问诊以排除一些重要的感染性致病原因。视神经炎合并面神经麻痹对大剂量激素治疗敏感。
【关键词】 视神经视网膜炎；Bell麻痹；黄斑部星芒状皱褶
AbstractAIM: To report an unusual case of neuroretinits with coexistent Bells Palsy.METHODS: A case report.RESULTS: A 16yearold boy presented with right eye blurring of vision for one week. It was associated with high fever and facial asymmetry. The visual acuity of the right eye was 6/60 and the left eye was 6/6. Funduscopy of the right eye showed swollen and hyperemic optic disc with presenceof macular star. There was also left lower motor neuron nerve palsy. Humphrey visual field perimetry showed smallparacentral scotoma with enlargement of blind spot. Complete blood count showed neutrophilia with increased erythrocyte sedimentation rate (ESR). Serology for syphilis,toxoplasmosis, Lyme disease and catscratch disease was negative. Herpes simplex virus, Herpes zoster virus, and human immunodeficiency virus investigations were negative. Rickettsia was also negative. MRI of the brain and orbit were normal without demyelinating lesion. He was started with high doseintravenous methylprednisolone 250mg four times per day for three days. He was then discharged home with oral prednisolone 1mg/kg for another 11 days. During the followup, the visual acuity was improved to 6/6 after six months with slightly pale disc. The Bells palsy was also improved without obvious facial asymmetry.CONCLUSION: It is important for clinician to examine other cranial nerves in pticneuritis. Althoughneuroretinitis with coexistent facial nerve palsy is a rare condition, thorough examinations and investigations should be made to exclude the important infective causes. Optic neuritis with coexistent facial nerve palsy responds well to the high dose steroids.
KEYWORDS: neuroretinitis; Bells palsy; macular star
INTRODUCTION
Optic neuritis can present as optic papillitis, neuroretinits or retrobulbar optic neuritis. The primary pathology in neuroretinitis is the optic nerve, and not the retinal vasculature. About 50% of the neuroretinitis are caused by infection or inflammatory process such as syphilis, Lyme disease (Borrelia species), catscratch disease (Bartonella species), toxoplasmosis, herpes virus, rickettsia, multiple sclerosis and autoimmune disorder. The remained 50% of the cases were idiopathic[13]. Neuroretinitis is characterized by swelling of the optic disc, peripapillary and macular hard exudates[4].
Facial nerve palsy can be caused by infection, vascular ischemia, autoimmune inflammatory disorders, Bells palsy or congenital. Bells palsy is an acute, idiopathic, unilateral paralysis of peripheral facial nerve of lower motor neuron type.
CASE REPORT
A 16yearold man presented with right eye blurring of vision for one week. It was associated with high fever, vomiting and headache. He noticed that he was not able to smile and close his left eyelids fully. The left angle of his mouth was also drooping. On examination he appeared lethargic. However, all the vital signs were stable. The visual acuity of the right eye was 6/60 and the left eye was 6/6. Funduscopy of the right eye showed swollen and hyperemic optic disc (Figure 1) with presence of macular star (Figure 2). Humphrey visual field perimetry showed small paracentral scotomawith enlargement of blind spot (Figure 3). There was also left lower motor neuron facial nerve palsy (Figure 4). The left eye and other cranial nerves were normal. There was no peripheral neurological deficit.
Complete blood count showed neutrophilia as high as 83.0%. Erythrocyte sedimentation rate (ESR) was elevated to 28mm/h. Toxoplasmosis serology showed positive IgG as 19.7kU/L, but negative for IgM. Serology for syphilis, Lyme disease, catscratch disease was negative. Investigations for herpes simplex virus (HSV), herpes zoster virus (HZV) and human immunodeficiency virus (HIV) were negative. The serology for Rickettsia (investigation for Rocky Mountain spotted fever) was negative as well. All serological screening tests for autoimmune disorders including rheumatic factor, dsDNA, antinuclear antibody were negative. CT scan of the brain and orbit were normal. MRI of the brain and orbit were normal as well, without demyelinating lesion.
He was started with high dose intravenous methylprednisolone 250mg four times per day for three days. He was then discharged home with oral prednisolone 1 mg/kg for another 11 days. During the followup the visual acuity was improved to 6/6 after six months with slightly pale disc. The Bells palsy also improved without obvious facial asymmetry.
DISCUSSION
Neuroretinitis usually occurs in children or young adults whose ages are between 6 and 50 years old. They usually present with unilateral loss of vision and a nonspecific "viral illness" which precedes or accompanies the loss of vision. Men and women are equally affected. The macular star in neuroretinitis is due to hard exudate accumulation around the fovea. The primary pathology is in the optic nerve which is characterized by hyperemic and swollen disc. It is important to identify the infective causes for neuroretinitis because certain organism can be treated with antimicrobial agents[57].
The most common form of facial nerve palsy is idiopathic lower motor neuron facial nerve palsy, also referred as Bells palsy, which was named after Sir Charles Bell (17741842). The incidence is approximately 20 to 30 persons in 100000. The exact pathogenesis of Bells palsy is still a mystery. However, most people believe that inflammation causes facial nerve swelling, resulting in an entrapment of the nerve within the meatal portion of the labyrinth segment. Herpeszoster infection of geniculate ganglion, known as Ramsay Hunt syndrome, can lead to Bells palsy. The other reported infections which cause facial nerve palsy are herpes simplex virus type 1, catscratch disease, human herpes virus type 6 and influenza virus[8]. The treatment of Bells palsy is still controversial, but patients usually recover well after 6 months with steroid[9].
Although all the investigations for infective agents are negative in this case but we must not forget the main cause of neuroretinitis is mainstay infection by microorganism. Patient presented with high fever for one week during the episode of reduced vision and facial nerve palsy, which may be suggestive more of infective cause of inflammation of the optic and facial nerves. It was important to keep the patientin optimized condition. He responded well to high dose of systemic prednisolone. Neuroretinits and facial nerve palsy can occur in multiple sclerosis but there was no demyelinating lesion shown in the MRI of the patients brain. Thesediseases can be found in autoimmune disorder but they usually presented with low grade fever. All the screening tests for autoimmune disorders including rheumatic factor, dsDNA, antinuclear antibody were negative.
It is difficult to identify the microorganism by current available serology test such as enzymelinked immonusorbent assay (ELISA) which has a high index of false negative results as well. The polymerase chain reaction (PCR) is more sensitive and has higher specificity, but it is not cost effective in most medical centers. The wide variety of microorganism that causes neuroretinitis and facial nerve palsy further makes the diagnosis more complicated.
It is rare to have neuroretinitis with coexistent facial nerve palsy. There was once a case of neuroretinitis with coexistent facial nerve palsy, which was caused by catscratch disease reported by Thompson et al [10]in 1999.
Therefore, it is important to examine other cranial nerves in optic neuritis. Neuroretinitis and facial nerve palsy have a very similar inflammatory process. It usually precedes or concurs with viruslike infection. Thus, the treatment and prognosis for both diseases are very much similar. It is crucial to have prompt investigation and treatment in patients with neuroretinitis with coexistent facial nerve palsy to in order to obtain good outcome[11,12].
In conclusion, it is important for clinician to examine other cranial nerves in optic neuritis. Although neuroretinitis with coexistent facial nerve palsy is a rare condition, thorough examinations and investigations are necessary to exclude the important infective causes. Optic neuritis with coexistent facial nerve palsy respond well to the high dose steroids. In most cases the causes for neuroretinitis or facial nerve palsy remain a mystery. More researches and case reviews should be carried out for coexistent optic neuritis and facial nerve palsy.
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