Bilateral Cranial Nerve Palsy Research Articles

Vogt‐Koyanagi‐Harada‐like findings as the first manifestation of neuroleukaemia

Aims/Purpose: To report a well‐documented case of a 62‐year‐old woman presenting with VKH‐like findings as the first manifestation of neuroleukaemia.Methods: Case study.Results: A 52‐year‐old woman who had received a diagnosis of acute myeloid monoblastic leukaemia (AML) one month prior and was in remission after the induction phase presented with floaters and gradual visual loss in both eyes. Visual acuity was 20/200 and 20/25, respectively. Anterior segment exam was normal in both eyes. Funduscopy showed reduced foveal reflex. On spectral‐domain OCT, subretinal fluid was found in both eyes. Fluorescein angiography showed hypofluorescent areas compatible with multifocal serous retinal detachments and multiple hyperfluorescent dots surrounding the detached areas. Days after, the patient debuted with facial palsy and binocular diplopia due to bilateral VI cranial nerve (CN) palsy, left VII CN palsy and right complete III CN palsy. An MRI was performed with no signs of infiltration. A cerebrospinal fluid sample was drawn by lumbar puncture and its analysis denoted infiltration of AML cells with similar immunophenotype to that on initial bone marrow biopsy. Our patient presented with an atypical case of Vogt‐Koyanagi‐Harada (VKH) with multiple leakages along the periphery of the detached retinal area. This sparing of the macula should be considered a suspicious pattern, and combined with the finding of CN palsies was enough to suspect a relapse in the form of CSF infiltration, making the diagnosis of neuroleukaemia possible.Conclusions: Exudative retinal detachment can be a primary presentation of AML or denote a relapse. VKH is a diagnosis of exclusion, as one of the main criteria for its diagnosis is the absence of other systemic diseases. This becomes especially important in atypical presentations and older patients. So as not to miss life‐threatening underlying conditions, leukaemia and lymphoma should be considered in the differential diagnosis of VKH.

Paralisi periferica bilateale del VII nervo cranico da infezione da virus di Epstein-Barr

Following a primary Epstein-Barr virus infection, a 4- years-old boy develops bilateral peripheral VII cranial nerve palsy. Ruled out the other bilateral facial palsy’s causes (first of all borreliosis), the cause of the paralysis is recognized in the Epstein-Barr virus infection. We start a steroid therapy associated with physiotherapy treatment for functional rehabilitation. Bilateral peripheral facial palsy is a rare condition. Un- like unilateral palsy, which is idiopathic in about half of the cases, bilateral palsy is idiopathic in only 20% of cas- es. Both in the case of unilateral and bilateral involve- ment, Borrelia burgdorferi infection stands out among the recognized causes in first place. Another bilateral facial palsy’s cause is Epstein-Barr virus infection, because of its important neurotropism, which causes palsy with a still unclear mechanism. About 2 months after the onset of neurological symptoms our boy is gradually recovering facial nerve functions.

Sudden Onset Auditory Neuropathy Spectrum Disorder: A Rare Case Report of Brown Vialetto Van Laere Syndrome.

Brown Vialetto Van Laere syndrome is a rare disorder characterized by progressive pontobulbar palsy with bilateral sensorineural hearing loss and lower cranial nerve palsies. Fifty-eight cases have been reported in the last hundred years. As the most common presenting complaint of this disorder is hearing loss, audiological evaluation plays a vital role in pointing towards and narrowing its diagnosis. We present a case report of a 12-year male child affected by this disorder.

Bilateral third and fourth cranial nerve palsy presenting secondary to central nervous system tuberculoma

Central nervous system (CNS) tuberculoma can have variable presentations depending on the site and number of tuberculomas. We report a rare case of a 48-year-old male presenting with ptosis and binocular double vision. Clinical examination revealed bilateral third-nerve palsy and fourth-nerve palsy with nystagmus. Magnetic resonance imaging (MRI) of the brain revealed ring-enhancing lesion in the dorsal midbrain, suggestive of tuberculoma. Radiological search for tuberculous foci in other internal organs proved pulmonary tuberculosis. Mantoux was positive, confirming the exposure to tuberculous bacilli. He was started on low-dose steroids along with antitubercular therapy (ATT). There was no improvement after continuing ATT for one-and-a-half years. Thus, the prognosis of CNS tuberculoma is related to the timing of initiating appropriate therapy. Delays in diagnosis and treatment will result in poor prognoses and severe neurological sequelae.

Determinants of visual and endocrinological outcome after early endoscopic endonasal surgery for pituitary apoplexy.

Patients diagnosed with pituitary apoplexy and presenting with acute visual deterioration require urgent surgical resection. This is also commonly associated with pituitary hypopituitarism that requires hormonal replacement for correction. This study was undertaken to evaluate the clinical recovery of 45 patients diagnosed with symptomatic pituitary apoplexy who underwent early (within 72 h of symptom onset) endoscopic transsphenoidal surgical resection with an emphasis on visual, ocular craniopathy, and endocrinological outcome. This is a retrospective analysis of a consecutive series of patients diagnosed with pituitary apoplexy between 2011 and 2020 treated by early (within 72 h of symptom onset) endoscopic transsphenoidal surgical resection. All tumors were histologically proven as pituitary adenomas. Clinical and neuro-ophthalmological examinations, imaging studies, and endocrinological evaluation were retrospectively reviewed. Patients with a minimum of 6 months follow-up were included in the study. The influence of patients' demographics, extent of visual and ocular motility impairment, preoperative endocrinological dysfunction, tumor size and extent, degree of resection, and surgical complications were analyzed as potential prognostic factors for recovery. Forty-five patients were included in this retrospective study. Ages ranged from 27 to 57 years (mean: 42 years). All patients presented with headache and variable degrees of visual loss and visual field deficit. Ophthalmoplegia was present in 22 (48.9%) patients with 17 (37.8%) having bilateral ocular cranial nerve palsy. All patients had variable degrees of endocrinological deficiencies. All patients showed evidence of low cortisol, 14 (31.1%) showed hypothyroidism and 11 (24.4%) showed hypogonadism. There was evidence of hyperprolactinemia in 16 (35.6%) patients. All patients harbored a pituitary macroadenoma. Tumor resection was complete in 33 (73%) of patients, with residual tumor related to cavernous sinus or retrosellar extension. Operative complications were mainly related to short-term nasal complications occurring in 14 (31%) patients. Cerebrospinal fluid leak requiring revision surgery occurred in only one patient harboring a modified SIPAP Grade 3s tumor. Transient diabetes insipidus occurred in 9 (20%) patients, with 2 (4.4%) requiring long-term hormonal replacement. The mean follow-up was 25 months. Baseline visual improvement was achieved in 39 (86.7%) patients. Ocular cranial nerve palsy showed complete recovery in 17 (77.2%) patients. Endocrine follow-up showed that patients with panhypopituitarism (11 [24.4%]) failed to recover. The current surgical series showed safety in terms of low complication rate and efficacy in terms of clinical outcome. The significant prognostic factor related to visual recovery was the degree of preoperative visual deficit. Recovery of ocular cranial neuropathy showed a higher recovery rate when it was unilateral as opposed to bilateral. Pituitary hormonal recovery was less favorable with pituitary panhypopituitarism being a poor prognostic factor.

Factor V Leiden Thrombophilia Causing Recurrent Thrombosis

Normal hemostasis requires a delicate balance between the natural procoagulant and anticoagulant systems. Thrombophilia is an abnormality of the coagulation or fibrinolytic system that results in a hypercoagulable state and increases the risk for thrombotic event by forming intravascular thrombus. The predisposition to form such blood clot may be inherited or acquired. In inherited thrombophilia, there is deficiency of natural anti-coagulants, e.g. anti-thrombin III, protein C and S or increase formation of human coagulant factors, e.g., factor V Leiden or prothrombin gene 20210A. Factor V Leiden thrombophilia is extremely rare in Bangladesh. We report this unique case of a young lady of 46-year-old presented with continuous headache and found to have extensive cerebral venous thrombosis with bilateral 6th cranial nerve palsy. She had suffered from acute arterial occlusion of left upper limb in September 2016, 2 years prior to current presentation. We suspect thrombophilia according to her presentation. The subsequent step is to screen for-human natural anti-coagulant factors deficiency or presence of mutated form of factor V (factor V Leiden) and prothrombin gene mutation. We screened for and found to have presence of factor V Leiden in our patient. We always should remember that, thrombotic events are increasingly recognized as a significant source of mortality and morbidity, so screening should be considered in selected individuals if criteria suggests. Bangladesh Crit Care J September 2021; 9 (2): 104-108

Suspected botulism outbreak after the consumption of vegetarian pâté in the south of Viet Nam

Botulism and other botulinum neurotoxins-producing clostridia are potentially life-threatening diseases caused by toxins produced by Clostridium botulinum. Here we reported a case series of six patients who presented with botulism following ingestion of commercially made pâté. The key features of presentation were acute onset of bilateral cranial nerve palsies and symmetrical descending weakness in the absence of fever resulting in the need for mechanical ventilation in all six patients. The clinical diagnosis of botulism was confirmed through the identification of C. botulinum from the suspected food source. Given that botulinum antitoxin was not available in Vietnam at the time, and their severe status, all patients received a trial of plasma exchange therapy, but no clear benefit was seen. Due to its rarity, diagnosing botulism is a challenge, demanding high clinical suspicion. Successful outcomes depend upon early recognition and rapid initiation of specific treatment with botulinum antitoxin. There is a need to improve global access to antitoxin. These cases, the first in Viet Nam, serve as a reminder of the need to maintain the highest possible food hygiene and preservation practices.

Botulism outbreak after the consumption of vegetarian pâté in the south of Viet Nam

Botulism is a potentially life-threatening disease caused by toxins produced by Clostridium botulinum. Here we reported a case series of six patients who presented with botulism following ingestion of commercially made pâté. The key features of presentation were acute onset of bilateral cranial nerve palsies and symmetrical descending weakness in the absence of fever resulting in the need for mechanical ventilation in all six patients. The clinical diagnosis of botulism was confirmed through the identification of C. botulinum from the suspected food source. Given that botulinum antitoxin was not available in Vietnam at the time, and their severe status, all patients received a trial of plasma exchange therapy, but no clear benefit was seen. Due to its rarity, diagnosing botulism is a challenge, demanding high clinical suspicion. Successful outcomes depend upon early recognition and rapid initiation of specific treatment with botulinum antitoxin. There is a need to improve global access to antitoxin. These cases, the first in Viet Nam, serve as a reminder of the need to maintain the highest possible food hygiene and preservation practices.

Hydrocephalus Revealed by Relapsing Bilateral Fourth Cranial Nerve Palsy

Introduction: Uni- or bi-lateral fourth cranial nerve palsy due to hydrocephalus and/or after VPS placement is a very rare oculomotor manifestation. We report a case of relapsing bilateral fourth nerve palsies demonstrating recurring hydrocephalus. We reviewed the literature (table1) in order to inform the clinician about the clinical assessment, the past medical history and the radiological findings that prompt research for this peculiar entity and to avoid misdiagnoses like palsies of the sixth cranial nerve. Diagnosis, intervention and outcome: The patient presented with recurrence of diplopia in reading position, partially resolved after a second VPS placement. A diagnosis of bilateral fourth nerves palsies was done after complete neuro-ophthalmological evaluation. A close follow-up demonstrated fluctuating level of diplopia by changing VPS valve resistance. An optimal placement of the VPS offered reduction and stability of diplopia. A final strabismus surgery was necessary to obtain complete symptoms release.

Botulism outbreak after the consumption of vegetarian pâté in the south of Viet Nam

Botulism is a potentially life-threatening disease caused by toxins produced by Clostridium botulinum. Here we reported a case series of six patients who presented with botulism following ingestion of commercially made pâté. The key features of presentation were acute onset of bilateral cranial nerve palsies and symmetrical descending weakness in the absence of fever resulting in the need for mechanical ventilation in all six patients. The clinical diagnosis of botulism was confirmed through the identification of C. botulinum from the suspected food source. Given that botulinum antitoxin was not available in Vietnam at the time, and their severe status, all patients received a trial of plasma exchange therapy, but no clear benefit was seen. Due to its rarity, diagnosing botulism is a challenge, demanding high clinical suspicion. Successful outcomes depend upon early recognition and rapid initiation of specific treatment with botulinum antitoxin. There is a need to improve global access to antitoxin. These cases, the first in Viet Nam, serve as a reminder of the need to maintain the highest possible food hygiene and preservation practices.

Botulism outbreak after the consumption of vegetarian pâté in the south of Viet Nam

Botulism is a potentially life-threatening disease caused by toxins produced by Clostridium botulinum. Here we reported a case series of six patients who presented with botulism following ingestion of commercially made pâté. The key features of presentation were acute onset of bilateral cranial nerve palsies and symmetrical descending weakness in the absence of fever resulting in the need for mechanical ventilation in all six patients. The clinical diagnosis of botulism was confirmed through the identification of C. botulinum from the suspected food source. Given that botulinum antitoxin was not available in Vietnam at the time, and their severe status, all patients received a trial of plasma exchange therapy, but no clear benefit was seen. Due to its rarity, diagnosing botulism is a challenge, demanding high clinical suspicion. Successful outcomes depend upon early recognition and rapid initiation of specific treatment with botulinum antitoxin. There is a need to improve global access to antitoxin. These cases, the first in Viet Nam, serve as a reminder of the need to maintain the highest possible food hygiene and preservation practices.

RECURRENT NASOPHARYNGEAL CARCINOMA: PROBLEM AND MANAGEMENT

Abstract Introduction: Treatment results for nasopharyngeal carcinoma (NPC) have been improved, but there were still some patients who developed localized persistent or local recurrent. Local recurrence still represents a major cause of mortality and morbidity also management still challenging issues. Case Report: A 27-year-old woman presented with bilateral conductive hearing loss, dysphagia, rhinorrhoea, epistaxis, and cranial nerve palsy after chemo radiation for NPC. The results of nasopharyngoscopy, CT scan, and PET scan were not found a mass in the nasopharynx. The result of nasopharyngeal biopsy result was undifferentiated carcinoma. Patient diagnosed with recurrent NPC and planned for re-radiotherapy. Conclusion: After complete treatment for NPC we must immediately ensure recurrence and choose the next appropriate treatment.

Neuromyelitis Optica Spectrum Disorder Presenting as Symmetric, Bilateral Cranial Nerve Palsies (2215)

Neuromyelitis Optica Spectrum Disorder Presenting as Symmetric, Bilateral Cranial Nerve Palsies (2215)

Lyme Neuroborreliosis presenting as multiple bilateral cranial nerve palsies (4838)

Lyme Neuroborreliosis presenting as multiple bilateral cranial nerve palsies (4838)

Acute myeloid leukemia diagnosed by dysphagia due to bilateral vagus nerve palsy: a case report

This is the rare case report that bilateral vagus nerve paralysis was emerged as the initial symptom of acute myelogenous leukemia (AML). An 83-year-old man admitted to our hospital because of dysphagia. His dysphagia progressed two months prior to admission. Although physical examination revealed no abnormality, videoendoscopy and videofluorography examination clearly revealed bilateral vagus nerve palsy. Brain MRI showed hypointense signals at the bilateral clivus on T1 weighted images, suggesting tumor infiltration to bilateral petroclivus. He was diagnosed as AML by blood samples and bone marrow biopsy. After initiation of the treatment including radiation therapy, dysphagia shows mild improvement. Although bilateral cranial nerve palsy due to malignant tumor involving at the clivus is very uncommon, we should pay attention to the symptom.

Causes and surgical treatment of diplopia and strabismus secondary to myasthenia gravis

Abstract Objective To present a report of the ocular motility disorders, treatment and outcomes of myasthenia gravis (MG). Material and method A retrospective study was performed on the data of patients with MG. An evaluation was made using mean age, gender, initial diagnosis, ocular deviation, time of onset of clinical characteristics, treatment and results. Resolution of diplopia and/or ocular deviation in primary and reading gaze was considered a good outcome at the end of follow-up. Results A total of 14 cases were included. The mean age of the sample was 55.64 years, of which 9 were women, and 10 cases were bilateral. The diagnosis was made by ophthalmologists in 4 cases. The initial diagnoses were diverse: bilateral cranial third nerve palsy in 3, unilateral third nerve palsy in 1, superior or inferior rectus palsy in 3, sixth nerve palsy in 2, fourth nerve palsy in 1, exotropia in 3 and esotropia in 1. Diplopia was presented in 14 cases and 9 associated ptosis. The different types of strabismus were horizontal ocular deviation in 11 cases: 8 with exotropia, and 4 with vertical deviation. Strabismus surgery was performed in 4 cases that did not respond to medical treatment, with a good final outcome. Pharmacological treatment resolved diplopia in 6 cases, and prisms in one. Ptosis surgery was only necessary in one patient. Outcome was favorable in 78.57% at the end of follow-up. Conclusion Acute onset diplopia caused by strabismus with variable angle or oculomotor palsy, associated or not with a ptosis can indicate MG. There were favorable outcomes with strabismus surgery. Pharmacological treatment did not resolve the diplopia in all cases.

Causas y tratamiento quirúrgico de la diplopía y estrabismo en la miastenia gravis

ObjectiveTo present a report of the ocular motility disorders, treatment and outcomes of myasthenia gravis (MG). Material and methodA retrospective study was performed on the data of patients with MG. An evaluation was made using mean age, gender, initial diagnosis, ocular deviation, time of onset of clinical characteristics, treatment and results. Resolution of diplopia and/or ocular deviation in primary and reading gaze was considered a good outcome at the end of follow-up. ResultsA total of 14 cases were included. The mean age of the sample was 55.64 years, of which 9 were women, and 10 cases were bilateral. The diagnosis was made by ophthalmologists in 4 cases. The initial diagnoses were diverse: bilateral cranial third nerve palsy in 3, unilateral third nerve palsy in 1, superior or inferior rectus palsy in 3, sixth nerve palsy in 2, fourth nerve palsy in 1, exotropia in 3 and esotropia in 1. Diplopia was presented in 14 cases and 9 associated ptosis. The different types of strabismus were horizontal ocular deviation in 11 cases: 8 with exotropia, and 4 with vertical deviation. Strabismus surgery was performed in 4 cases that did not respond to medical treatment, with a good final outcome. Pharmacological treatment resolved diplopia in 6 cases, and prisms in one. Ptosis surgery was only necessary in one patient. Outcome was favourable in 78.57% at the end of follow-up. ConclusionAcute onset diplopia caused by strabismus with variable angle or oculomotor palsy, associated or not with a ptosis can indicate MG. There were favourable outcomes with strabismus surgery. Pharmacological treatment did not resolve the diplopia in all cases.

Bilateral Third Nerve Palsy in Mirror Aneurysms of the Posterior Communicating Arteries.

BackgroundBilateral third cranial nerve palsy has only been reported in a handful of conditions including some with inflammatory, tumoural and vascular causes. An urgent imaging study is mandatory to rule out vascular aetiology, mainly aneurysmal subarachnoid haemorrhage (aSAH).Case presentationA 28-year-old Hispanic woman presented to the emergency department with a 21-day history of a sudden-onset and severe headache that on three occasions was accompanied by transient loss of awareness, the last episode occurring a week previously. The simple CT image showed minimal bleeding at the level of the perimesencephalic cisterns, with evidence of SAH. An angioCT revealed a 5×6 mm bilobed saccular aneurysm of the right posterior communicating artery and a 2×2 mm saccular aneurysm in the posterior left communicating artery.ConclusionsA mirror aneurysm is found in 2–25% of aSAH cases. To date there is no consensus about the optimal management of patients with these findings.LEARNING POINTSThe presence of third cranial nerve palsy should always raise the suspicion of an aneurysm.Subarachnoid haemorrhage is the most common cause of a thunderclap headache.Aneurysmal subarachnoid haemorrhage requires surgical management in all cases.

Cavernous sinus involvement in human papillomavirus associated oropharyngeal squamous cell carcinoma: case report of an atypical site of distant metastasis

BackgroundHPV-associated OSCC (HPV-OSCC) has been determined to be a distinct disease entity from non-HPV associated OSCC. Patients affected by HPV-OSCC generally have a more favourable prognosis, with improved rates of locoregional control and survival compared with their non-HPV counterparts. Despite this, HPV-OSCC has a similar rate of distant metastases. Interestingly, recent evidence has emerged that demonstrates more frequent atypical metastasis patterns when compared to non-HPV associated disease. To the best of our knowledge, this report describes the first case of a confirmed HPV-OSCC with distant metastasis to the cavernous sinus.Case PresentationA 62-year-old non-smoking male presented to the head and neck oncology clinic with a five-month history of enlarging right neck mass causing neck pain, dysphagia, and dysphonia. HPV-associated base of tongue squamous cell carcinoma (cT4aN2c) was diagnosed, and he was treated with primary chemoradiation. Shortly after treatment, he presented with progressive bilateral cranial nerve palsies including left cranial nerve III and right cranial nerve VI involvement. Imaging identified masses in the left cavernous sinus with extension of tumor into the sella and in the right cavernous sinus at the level of Dorello’s canal. Endoscopic Image Guided Transsphenoidal biopsy of the left sellar mass confirmed distant metastases from the previously treated HPV-OSCC primary to the cavernous sinus. The patient was palliated with carboplatin and paclitaxel.ConclusionThe presented report is the first documented case of confirmed HPV-associated oropharyngeal squamous cell carcinoma metastasizing to the cavernous sinus, and the only HPV confirmed head and neck cancer case to present with metastasis to the cavernous sinus and limited extracranial disease. This case demonstrates the importance of recognizing presentations of atypical metastasis that are possible in HPV-associated oropharyngeal cancer. Given the rarity of metastasis to this region, vigilance in follow up is instrumental in early identification and treatment for these patients.

63. Guillain-Barrè syndrome with marked pleocytosis in the cerebrospinal fluid: A case report

A 71 years old female patient referred to her Physician for left hip and abdominal, nigh-time worsening pain. For this reason, she underwent a lumbosacral vertebral MRI, that highlighted the presence of L3-S1 left radiculopathy. After two weeks, she developed a left VI and VII cranial (peripheral) nerves palsy. Admitted to hospital, she underwent brain CT and MRI, that resulted unremarkable. In Neurology division, on examination a left VI cranial nerve palsy, a bilateral VII cranial nerve palsy, dysarthria, dysphagia and increased tendon reflexes were observed. The neurophysiology investigation revealed diffuse delayed F-waves with axonal response, absent H reflex, asymmetrical skin reflex, normal distal M-waves latencies, central and peripheral motor and sensitive conduction, and EEG. In light of these findings, matching the hypothesis of acute polyradiculoneuritis a lumbar puncture was performed. Increased CSF proteins and white cell count were found, while microbiological exams were unremarkable. A combination iv treatment (ceftriaxon, acyclovir, human IG) was established. Because of the onset of tetraparesis and respiratory failure, the patient was admitted to the intensive care unit. Even if case of CSF pleocytosis (>50/ μ l) a differential diagnosis should be considered 1–2 this case report suggests that Guillain-Barre diagnosis can not defectively ruled out.