An alarming sign for serious diseases in children: bilateral facial paralysis

Facial paralysis in children is most often idiopathic, and isolated facial nerve palsy resulting from leukemic infiltration is a rare occurrence. We report a 13-year-old male with acute lymphoblastic leukemia presenting with bilateral facial palsy, who was previously diagnosed with idiopathic facial palsy and treated with steroids. This rare presentation of acute lymphoblastic leukemia should be kept in mind as a diagnostic possibility in a patient with bilateral facial nerve paralysis.     

Cerebral venous thrombosis associated with tentorial subdural hematoma during oxymetholone therapy

Androgen was reported to cause cerebral venous thrombosis (CVT) during replacement therapy for aplastic anemia. Oxymetholone, a synthetic androgen analogue, has been widely used in the treatment of aplastic anemia. A 40-year-old woman with aplastic anemia visited our hospital because of severe headache, nausea, vomiting, blurred vision and diplopia for a period of 1 month. She had taken oxymetholone for 2 years. Neurological examination revealed bilateral papilledema and bilateral sixth nerve palsies. Brain magnetic resonance imaging (MRI), performed at the time of admission, demonstrated left-sided tentorial SDH, and focal cerebral thrombosis of the left superficial sylvian vein and sigmoid sinus. MR venography revealed multiple irregularities in the superior sagittal sinus and left transverse sinus. CVT with tentorial subdural hematoma (SDH) caused by oxymetholone was strongly suggested. Oxymetholone was immediately discontinued, and her symptoms and signs disappeared. Because of the thrombocytopenia, anticoagulation was not started. She was discharged and visited the outpatient clinic without neurological symptoms for 6 months. This report supports the cautions given about the risk of CVT with oxymetholone supplementation in aplastic anemia. To the best of our knowledge, this is the first report of CVT associated with tentorial SDH that was probably caused by oxymetholone.     

Vernet's Syndrome Associated with Internal Jugular Vein Thrombosis

Our objective is to present a case of Vernet's syndrome (cranial nerve (CN) IX, X, and XI palsy) associated with cerebral venous thrombosis (CVT) in an internal jugular vein. The patient presented with acutely developed dysphagia. The weakness of the left sternocleidomastoid and trapezius muscles was observed. The initial magnetic resonance imaging and computed tomography (CT) with contrast enhancement showed contrast-filling defect in the left internal jugular vein inside the jugular foramen. The magnetic resonance venography with contrast enhancement revealed a partial filling defect in the left sigmoid sinus and total occlusion of the left internal jugular vein. Under the diagnosis of CVT associated with CN IX, X palsy, anticoagulation therapy with low-molecular-weighted heparin was initiated. Despite the continued anticoagulation therapy for 3 months, neither the burden of thrombosis in the left sigmoid sinus and internal jugular vein on neck CT nor dysphagia symptoms improved. Clinicians need to be aware of internal jugular venous thrombosis as one of the differential diagnoses in Vernet's syndrome in patients in a hypercoagulable state. Further reporting of similar cases is needed to confirm the association between CVT and Vernet's syndrome.     

Idiopathic intracranial hypertension and facial palsy: case report and review of the literature

We present the case of an 11-year-old obese girl who presented with idiopathic intracranial hypertension affecting first the lateral abducens nerve. She received acetazolamide, but 5 days later she developed lateral, peripheral facial palsy. Imaging evaluation was normal, which primarily excluded cerebral venous thrombosis and sustained the initial diagnosis. Despite some complicating factors (obesity, elevated intracranial pressure), prednisolone was administered for a short-term period to counteract the facial palsy. Ophthalmological residuals resolved within almost 1.5 months, while facial palsy receded after 4 months. Peripheral facial palsy is an extremely rare, but not unknown condition in idiopathic intracranial hypertension. As a symptom, it should be investigated thoroughly, primarily to exclude cerebral venous sinus thrombosis, before it can be attributed to idiopathic intracranial hypertension. As far as treatment is concerned, corticosteroids can be added to the initial treatment with acetazolamide, without worsening already elevated intracranial hypertension or ophthalmologic findings.     

Preoperative Identification of Facial Nerve in Vestibular Schwannomas Surgery Using Diffusion Tensor Tractography

Objective

Facial nerve palsy is a common complication of treatment for vestibular schwannoma (VS), so preserving facial nerve function is important. The preoperative visualization of the course of facial nerve in relation to VS could help prevent injury to the nerve during the surgery. In this study, we evaluate the accuracy of diffusion tensor tractography (DTT) for preoperative identification of facial nerve.

Methods

We prospectively collected data from 11 patients with VS, who underwent preoperative DTT for facial nerve. Imaging results were correlated with intraoperative findings. Postoperative DTT was performed at postoperative 3 month. Facial nerve function was clinically evaluated according to the House-Brackmann (HB) facial nerve grading system.

Results

Facial nerve courses on preoperative tractography were entirely correlated with intraoperative findings in all patients. Facial nerve was located on the anterior of the tumor surface in 5 cases, on anteroinferior in 3 cases, on anterosuperior in 2 cases, and on posteroinferior in 1 case. In postoperative facial nerve tractography, preservation of facial nerve was confirmed in all patients. No patient had severe facial paralysis at postoperative one year.

Conclusion

This study shows that DTT for preoperative identification of facial nerve in VS surgery could be a very accurate and useful radiological method and could help to improve facial nerve preservation.     

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.     

T2*-Weighted MRI Detected Dilated Cerebral Veins in a Patient with Acute-Phase Cerebral Venous Sinus Thrombosis—A Case Report

We describe a 45-year-old man who presented with nausea, vomiting, and strong occipital headache on the right side. Although no abnormalities on neurological examination or computed tomography imaging were found on admission, peripheral blood cell counts showed polycythemia (hemoglobin 20.6 g/dL) and electrocardiography demonstrated atrial fibrillation. Therefore, anticoagulant treatment with heparin was started immediately. On the following day, the occipital headache continued. Brain T2*-weighted (T2*WI) magnetic resonance imaging (MRI) and, to a lesser extent, susceptibility-weighted imaging showed dilation of numerous cortical veins, suggesting the possibility of cerebral venous thrombosis (CVT). MR venography (MRV) showed a deficit of the right transverse sinus. Contrast-enhanced MRI revealed partial defects of the right transverse sinus, and led to the definite diagnosis of CVT, and the anticoagulation therapy was continued. On day 7 the headache disappeared, and MRV on day 16 showed the recanalization of the right transverse sinus. There were no complications subsequent to the CVT. On day 25, the patient was discharged with no after-effect. We speculate that the dilation of cortical veins on T2*WI is a helpful sign in detecting acute-phase CVT.     

Facial nerve palsy in childhood

Facial nerve palsy in children is usually idiopathic but can also result from many conditions such as neoplasias, systemic diseases, or congenital anomalies with poor prognosis. Children with idiopathic facial palsy (Bell’s palsy) have a very good prognosis, while treatment with prednisone does not certainly improve the outcome. The causes of facial nerve palsy in childhood differ from those in adults. A detailed investigation and differential diagnosis are recommended for facial palsy in children.     

Facial palsy in multiple sclerosis

Facial palsy occurred in 21 (19.6%) of 107 Japanese patients with multiple sclerosis (MS) during a mean follow-up period of 4.3 years. We observed residual signs of facial palsy in five other patients in whom acute onset was confirmed from medical records. Facial palsy began on average 7.6 years after the onset of MS but in five patients (4.7%) was the first symptom of MS, preceding the next MS symptom by 0.5–3 years. Facial palsy was usually associated with other brainstem signs, while two patients showed only facial palsy 1 and 3 years after the onset of MS. Twenty-one (84.0%) of the 25 patients who underwent brain magnetic resonance imaging (MRI) showed brainstem lesions in the pontine tegmentum ipsilateral to the facial palsy. However, the two patients without other symptoms or signs had no apparent causal lesion on MRI, which suggests difficulty in differentiating idiopathic Bell’s palsy from MS- associated facial palsy by MRI, although it has an excellent capacity to detect causal lesions of facial palsy associated with MS. Received: 6 March 1997 Received in revised form: 25 July 1997 Accepted: 12 August 1997     

Arachnoid cyst of the cerebellopontine angle associated with gliosis of the eighth cranial nerve.

The clinical syndrome produced by a cyst in the cerebellopontine angle (CPA) may closely mimic that of an acoustic neuroma, with sensorineural hearing loss, impaired corneal reflex, and cerebellar signs with increased intracranial pressure. Facial palsy is seldom reported. Gliosis of the eighth nerve is common but its association with CPA arachnoid cyst is very rare and not previously reported. We report a patient with a CPA arachnoid cyst associated with gliosis of the eighth cranial nerve. He presented with right peripheral facial palsy, and gliosis of the eighth nerve was diagnosed intraoperatively. CPA arachnoid cysts should be included in the differential diagnosis of peripheral facial palsy and the eighth cranial nerve should be examined during the resection and fenestration of the arachnoid cyst.     

A comparison of facial nerve disability between patients with Bell’s palsy and vestibular schwannoma

We compared self-assessed morbidity of facial nerve dysfunction arising from surgical intervention with those in non-surgical clinical scenarios in a cross-sectional observational study. The validated patient-graded Facial Clinimetric Evaluation (FaCE) scale and a supplementary questionnaire were mailed to adult subjects with a history of facial nerve dysfunction. Seventy-five completed survey packets were returned: 53 (71%) cases of facial nerve dysfunction were caused by surgery for vestibular schwannoma and 22 (29%) were the result of Bell’s palsy. The vestibular schwannoma cohort reported less paralysis-related morbidity in the subscale domains of facial movement and social function (p < 0.03, one-way analysis of variance [ANOVA]). Within the surgical cohort, those patients expecting “likely” postoperative facial dysfunction experienced less morbidity in oral function and social function compared to those expecting “unlikely” postoperative facial dysfunction or those who did not recall having a preoperative discussion at all (p < 0.05, one-way ANOVA). Patient age, gender, history of rehabilitative measures, and duration of paralysis did not correlate with morbidity. We concluded that patients with facial nerve dysfunction arising from surgical intervention experienced less morbidity than those caused by non-surgical etiologies. Patient participation in the informed consent process and health provider setting of expectations were important factors to reduce perceived morbidity.     

The cavernous sinus syndrome an anatomical and clinical study

The cavernous sinus is often involved pathologically, which can cause ocular motor nerve palsies with or without facial sensory disturbances. Consequently several clinical features of ocular motor nerve palsies have been described.

In this article we present a study of the cavernous sinus syndrome, and compare this syndrome with other nerve palsy syndromes caused by lesions in or adjacent to the cavernous sinus.

The clinical features are explained by means of an anatomical study of the cavernous sinus.     

Facial nerve damage following surgery for cerebellopontine angle tumours. Prevention and comprehensive treatment

Facial nerve (CN VII) palsy or even its transient paresis causes physical disability but is also a psychosocial problem. Immediately after vestibular schwannoma removal, different degrees of CN VII paresis occur in 20-70% of patients. Facial nerve paresis is observed in 10-40% after surgery of cerebellopontine angle meningiomas. Postoperative facial nerve weakness significantly reduces or completely withdraws with time in the majority of cases. However, even if prognosis for CN VII regeneration is good, proper management is needed because of the potential for serious ophthalmic complications. In this paper, the authors raise the issue of perioperative prophylaxis and comprehensive treatment of postoperative paresis of CN VII. Prophylaxis and treatment of ophthalmic complications are discussed. Current trends in the treatment of intraoperative loss of facial nerve continuity, management of facial paresis with good prognosis and dealing with facial palsy with no spontaneous recovery are also described in the paper.     

Proximal conduction abnormality of the facial nerve in Miller Fisher syndrome: a study using transcranial magnetic stimulation.

OBJECTIVE: To investigate facial nerve conduction, including its proximal segment, in Miller Fisher syndrome. METHODS: Three patients underwent facial nerve conduction studies comprising stylomastoid electrical stimulation and transcranial magnetic stimulation at the entrance of the facial canal within the skull and of the cortical representation area. All 3 patients presented with acute bilateral complete ophthalmoplegia, areflexia, mild ataxia and varying other symptoms. One of the patients had bilateral facial palsy; the other two had normal facial innervation. RESULTS: Findings suggestive of demyelination of the proximal segment of the facial nerve were observed in each of the 3 patients with Miller Fisher syndrome. The patient with bilateral facial palsy had absent responses to canalicular stimulation on both sides, while the other two showed increased temporal dispersion and prolonged latency in the proximal nerve segments. CONCLUSIONS: Our findings suggest that the primary pathology of facial nerve lesion in Miller Fisher syndrome is demyelination and that it is localized to the proximal nerve segment. This is in line with the known vulnerability of proximal nerve segments (spinal roots) in other dysimmune demyelinating polyneuropathies. SIGNIFICANCE: Facial nerve conduction study with magnetic stimulation can localize and detect even subclinical facial nerve dysfunction in patients with Miller Fisher syndrome. The technique may contribute to the diagnosis of this disease, where electrophysiologic findings are scanty.     

Case of pontine infarction causing alternating hemiplegia with ipsilateral abducens nerve palsy and contralateral supranuclear facial nerve palsy]

We report a 73-year-old man with alternating abducent hemiplegia (Raymond syndrome) and contralateral supranuclear facial nerve palsy. On admission, he showed lateral gaze palsy of the right eye, left supranuclear facial nerve palsy, dysarthria and left hemiparesis. Brain MRI showed an infarct that was located in the paramedian and lateral area in the base of the caudal pons on the right side. MRA showed a mild stenosis of the basilar artery. Hemiplegia and supranuclear facial nerve palsy were considered to be caused by the involvement of corticospinal tract and corticobulbar tract that run at the ventromedial area of the pons. Abducens nerve palsy was considered to be caused by the involvement of infranuclear abducens nerve fibers. There has been one previously reported case of Raymond syndrome in which MRI determined the precise location of the lesion. In this case, a small hematoma was found at the ventral and medial pontomedullary junction, whereas the infarct in our case was located in the pontine base. We considered that documentation of our case was an important contribution to determine the pathogenesis of supranuclear facial nerve palsy due to caudal pontine lesions.     

Simultaneous thrombosis of cerebral artery and venous sinus

Cerebral venous thrombosis (CVT) is infrequent among cerebrovascular diseases. The simultaneous thrombosis involving both cerebral artery and venous sinus is even extremely rare. We reported a 41-year-old woman who presented with acute headache and left hemiparesis due to concomitant arterial ischemic stroke and recurrent CVT. Extensive investigation disclosed acquired protein C and protein S deficiency, iron deficiency anemia (IDA) and cryoglobulinemia. She was treated with intravenous injection of heparin followed by oral anticoagulant therapy. The headache rapidly subsided; however, left hemiparesis persisted over five months. The rare condition of simultaneous thrombosis of cerebral artery and venous sinus may be caused by the synergistic effect of coagulation disorders, IDA and cryoglobulinemia.     

Facial palsy and idiopathic intracranial hypertension in twins with cystic fibrosis and hypovitaminosis A

Facial nerve palsies are uncommon in infants. We report on 10-week-old monozygotic twins, diagnosed with cystic fibrosis by newborn screening, who developed facial palsy and increased intracranial pressure. Cranial imaging and cerebrospinal fluid analysis produced normal results. Levels of serum vitamin A were below normal range. Low levels of vitamin A are associated with facial nerve paralysis, and are at least partly implicated in the development of increased intracranial pressure in infants with cystic fibrosis.     

A case of facial diplegia associated with herpes simplex reactivation

Facial nerve palsy is the commonest cranial neuropathy affecting 40/100 000 people in the Western World but is idiopathic in 50–70% of cases. This report describes a case of recurrent facial nerve palsy in association with herpes simplex virus (HSV) reactivation. HSV may be a major cause of racial nerve palsy. Further research is required to clarify this issue in order that targeted treatment strategies can be developed for this common problem.     

面静脉-眼上静脉入路填塞海绵窦

目的评价经面静脉-眼上静脉入路填塞海绵窦治疗颈动脉海绵窦瘘（CCF）的有效性。方法经股静脉-面静脉-眼上静脉入路到达患侧海绵窦，用GDC或EDC，游离弹簧圈，真丝线段等多种栓塞材料填塞海绵窦，同时闭塞瘘口。面静脉插管困难者，在下颌角附近切开皮肤显露面静脉，直视下穿刺面静脉放置相应导管，再经眼上静脉到达患侧海绵窦并将其填塞。结果经面静脉-跟上静脉入路对14例，16侧海绵窦进行了栓塞治疗，其中5例为外伤性、直接CCF（A型），经动脉途径球囊栓塞后复发，或微弹簧圈栓塞未能成功，或经岩下窦入路未能成功，9例为自发性、间接CCF（D型8例，C型1例）。13例经股静脉-面静脉-眼上静脉途径，1例通过直视下面静脉穿刺。11例栓塞治疗后即刻造影显示瘘消失，2例残留低流量的岩下窦引流，另有1例在微导管进入面静脉后，而静脉痉挛闭塞，未能继续进行栓塞治疗，造影仍见瘘存在，但眼静脉出现明显的造影剂滞留。1例A型CCF在球囊栓塞后出现外展神经麻痹，经面静脉-眼上静脉栓塞后亦无改善。因面静脉痉挛闭塞未能栓塞成功者，于术后即感眼部症状加重，但第2天感症状缓解，术后第21天症状明显改善，造影检查发现瘘门已经消失，术后1个月病人眼部症状完全消失。其他病例在栓塞术后眼部症状明显改善，最后消失。随访3个月至21个月未见复发。2例残留瘘口者，1例于3个月和12个月进行2次造影复查，另1例于3个月造影复查，瘘的流量均无明显变化，因无临床症状未再进行治疗。其他病例未进行造影复查。结论经面静脉-眼上静脉栓塞治疗CCF安全有效，对于A型CCF，可作为经动脉途径治疗失败后的补救措施，而对于B、C、D型CCF，应作为首选治疗。     

Facial nerve neurinoma presenting as middle cranial fossa and cerebellopontine angle mass: a case report

Facial nerve neurinomas are rare. The tumours arising from the geniculate ganglion may grow anteriorly and superiorly and present as a mass in the middle cranial fossa. Only a few cases of facial nerve neurinomas presenting as middle cranial fossa mass have so far been reported. These tumours present with either long standing or intermittent facial palsy along with cerebellopontine angle syndrome.