Surgical Considerations in Addressing Cholesterol Granuloma of the Petrous Apex through an Infracochlear Approach.

Cholesterol granuloma of the petrous bone is a foreign body giant cell reaction to cholesterol deposits with symptoms including hearing loss, vestibular dysfunction, and cranial nerve deficit as a result of cystic mass compression. Surgical treatment is often difficult to plan due to limited access to the lesion and possible injury to surrounding structures. We report on a case of petrous apex cholesterol granuloma drainage through an infracochlear approach. A 27-year-old female patient presented with acute diplopia due to left-sided abducens paralysis. Multislice computed tomography (MSCT) and magnetic resonance (MR) imaging described a 3.5-cm well-marginated lesion in petrous bone apex, compressing the left abducens nerve at the point of entry into the cavernous sinus, corresponding to cholesterol granuloma. The patients was surgically treated through a transcanal infracochlear approach, since preserving the external and middle ear conduction mechanisms was paramount for the patient. The patient was discharged on the second postoperative day and diplopia resolved within 5 days postoperatively. Six months after the surgery, her hearing on the left side is normal, and she remains symptom-free. This case underpins the value of preoperative planning when approaching the petrous apex, an anatomically complex area due to abundance of important neurovascular structures crowded in a narrow and confined region.

Herpes simplex virus type 2 meningitis as a manifestation of Good's syndrome.

Good's syndrome is a primary immunodeficiency phenocopy characterized for thymoma and immunodeficiency. The most frequent clinical presentation is recurrent or opportunistic infections, hematological alterations, and chronic diarrhea. We treated a 66-year-old man who consulted for 5 days of headache and diplopia with right sixth cranial nerve palsy at examination. Patient reported chronic diarrhea and prolonged febrile syndrome accompanied by weight loss of 23 kg in the last year. Exhaustive evaluation revealed Herpes simplex virus (HSV) type 2 meningitis, eosinophilic colitis, and type A thymoma. Severe antibody deficiency (hypogammaglobulinemia) associated with thymoma confirmed the diagnosis of Good's syndrome.

Intravenous Glucocorticoid Treatment for Korean Graves' Ophthalmopathy Patients.

BACKGROUND: High-dose intravenous steroids are the first-line treatment for patients with moderate-to-severe and active Graves' ophthalmopathy (GO). We aimed to investigate the response rate of methylprednisolone (MPD) treatment among Korean patients with active moderate-to-severe GO and to identify predictive factors of treatment response. METHODS: This is a retrospective observational study. We included 54 active moderate-to-severe GO patients treated with 4.5 g intravenous MPD over 12 weeks between November 2011 and November 2018. Response was defined as an improvement in at least two of five indicators (clinical activity score [CAS], soft-tissue involvement, exophthalmos, diplopia, and visual acuity) at immediate and 3 months after treatment completion. We examined predictive factors for response using logistic regression analysis. RESULTS: Twenty-four (44.4%) and 22 (40.7%) patients showed response at immediate and 3 months after intravenous (IV) steroid treatment. Of the five ophthalmic parameters, all patients in the responsive group (100.0%) showed a decrease in CAS and 90.9% showed less soft tissue involvement after IV steroid treatment. Among variables, the sum of extraocular muscle width was positively (odds ratio [OR], 1.163; 95% confidence interval [CI], 0.973-1.389; P = 0.096) associated with treatment response. While, the OR of age was 0.918 (95% CI, 0.856-0.985; P = 0.017) and thyrotropin binding inhibitory immunoglobulin (TBII) was 0.921 (95% CI, 0.864-0.982; P = 0.012). CONCLUSION: In Korean active moderate-to-severe GO patients, intravenous steroid treatment is not as effective as previously reported. Parameters associated with CAS and soft-tissue involvement were found to be influenced by IV MPD treatment. Extraocular muscle enlargement, younger age and lower TBII are predictive factors for a good steroid treatment response.

Measurement of Medial Wall Bowing and Clinical Associations in Thyroid Eye Disease.

PURPOSE: To propose and validate a measure of medial wall bowing in thyroid eye disease (TED) and to assess the clinical correlates of bowing in TED. METHODS: In this cross-sectional cohort study, all patients affected with TED seen by a single specialist over a 2-year period were screened for study entry. Eligible participants were adults with clinical evidence of TED and either CT or MRI of the orbits. Exclusion criteria included prior history of decompression surgery and/or medical or other ophthalmic conditions that could alter the orbital anatomy. The primary outcome measure was prevalence of medial wall bowing. Secondary outcomes included the associations between medial wall bowing and exophthalmometry, diplopia, rectus muscle restriction, dysthyroid optic neuropathy (DON), strabismus, and quality of life. Medial wall bowing was defined as medial divergence of the medial wall from a straight line drawn between the anterior lacrimal crest and the middle of the optic canal and measured radiographically using axial images of the orbits. Volumetric analysis of bowing was performed on a sample of orbits with and without bowing. Volumetric and axial single slice measurements were compared. Bivariate statistics were performed. RESULTS: The final sample included 112 orbital images from 56 patients with TED. Medial wall bowing was found to be evident in 11.6% (n = 13/112) of orbits. Medial wall bowing was significantly associated with greater Hertel measurements, horizontal and vertical muscle restriction, ocular surface symptoms and Clinical Activity Score, as well as lower quality of life scores. Patients with demonstrable medial wall bowing were more likely to be affected by optic neuropathy, diplopia (Gorman score ≥ 1), strabismus, or horizontal muscle restriction. Analysis of the volume for medial wall bowing demonstrated that the height of the arc at the peak of bowing on an axial image of a 2-dimensional CT scan correlated highly with the total volume of bowing (r = 0.90, p < 0.001). CONCLUSIONS: The identification of medial wall bowing on CT or MRI is associated with clinical measures of disease severity, including diplopia and DON.

Diplopia as isolated presentation of varicella zoster central nervous system reactivation.

Here, we report a patient who developed diplopia secondary to a right cranial nerve III and IV palsy, as well as fever and headache. Cerebrospinal fluid analysis (CSF) showed high varicella-zoster virus (VZV)-DNA viral load (>300,000,000 copies/ml). VZV antibodies in CSF was ≥1:16. Diagnosis of neurological reactivation of VZV infection was made without the presence of characteristic vesicular rash. Quantitative real-time PCR for VZV and intrathecal dosage of VZV IgM and IgG should be performed in cases suspected for viral encephalitis and also in all patients with not otherwise attributable cranial nerve lesions.

Preoperative evaluation and surgical technique of functional and cosmetic aspects in zygomatic complex fracture patients.

The zygomatico-maxillary complex functions as the principle buttress of the face and is the cornerstone to an individual’s aesthetic appearance. Its fracture not only creates cosmetic deformities owing to its position and facial contour, but can also cause disruption of ocular and mandibular functions. The aim of this study was to evaluate the quality, efficacy and impact of internal fixation of zygomatic complex fractures on functional and cosmetic outcomes. A prospective study was carried out on 100 patients who were divided according to the classification and the severity of injury. Subjective evaluation was submitted based on the patient’s perception of signs and symptoms in the preoperative and postoperative periods. Intraoperative and postoperative assessment of bone reduction quality was made according to the type of the fracture and related difficulties; also, the difference between these groups was observed as functional and esthetic outcome. To optimize the treatment of zygomatic bone fractures, a pre-designed questionnaire was used for subjective evaluation of symptoms and treatment outcome. In 70% of cases, ophthalmologic consultation was taken and was most common in type VII fractures (100% cases). Neurosensory disturbance was the most common finding (60%), followed by diplopia (56R%), pain upon mouth opening (54%) and malar depression (50%). Out of all possible 400 fracture sites in 100 patients of zygomatic complex fractures, 266 (66.5%) fractures were detected by clinical examination, in contrast to 330 (82.5%) on radiological examination, which were highest at zygomatic-maxillary buttress (93%) followed by infraorbital rim (91%) and almost equal among fronto-zygomatic site (72%) and zygomatic arch (74%). The scores from the questionnaire for annoyance were significantly higher for paraesthesia (23%) than for trismus (10%), pain (8.5%), or deformity (8.25%). Residual deformity and pain significantly influenced the total satisfaction. Conclusively, there are many treatment modalities available for zygomatic complex fractures, and the preferred methods should be selected on the basis of fracture type, fracture severity, pre-operative signs and symptoms. Regarding the requirements of fracture site exposure and actual fixation, one priority should be to minimize postoperative complications, morbidity and residual deformities.

Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression.

Progressive multifocal leukoencephalopathy (PML) is a viral demyelinating disease due to the reactivation of the JC virus (JCV), which usually occurs in the context of immunosuppression in HIV infection, malignancy, or in patients on disease modifying therapy for autoimmune diseases, such as multiple sclerosis (MS) and Crohn's disease. Notably, there is growing recognition that PML can occur in patients with transient immune dysfunction. Here, we present a case of a 55-year-old man without history of immunosuppression or evidence of ICL who was diagnosed with PML on brain biopsy. We will discuss the potential etiologies of mild and transient immunosuppression that can lead to PML with non-apparent immunosuppression.

From ophthalmoplegic migraine to cranial neuropathy.

Ophthalmoplegic migraine (OM)/recurrent painful ophthalmoplegic neuropathy (RPON) is a rare disease consisting of recurrent unilateral headache accompanied or followed by ipsilateral ophthalmoplegia. Because MRI findings suggest neuropathy and the relationship to typical migraine remains unclear, the disease has been renamed from "ophthalmoplegic migraine" to "recurrent painful oculomotor neuropathy" in the third edition of the International Classification of Headache Disorders (ICHD). However, it remains a fact that most cases of OM/RPON described in the literature have a history of migraine and that the headache during OM/RPON often has migrainous features. A more detailed clinical description of the headache during OM/RPON and additional results from imaging and possibly histology will be needed to better understand the pathophysiology of the disease and its relationship to typical migraine.

A novel association of ocular neuromyotonia with brainstem demyelination: two case reports.

Ocular neuromyotonia (ONM) is a rare disorder of ocular mal-alignment in which painless, transient spontaneous or gaze-induced abnormal deviation of the eye manifests as episodic diplopia. With only a few cases reported in the literature, ONM mostly follows months to years after cranial irradiation for sellar or suprasellar lesions. Here we present two patients with this rare ocular condition, secondary to brainstem demyelination, the association of which is hitherto unreported in the literature. Both patients were 15-year-old girls who presented to us with episodic forced-eye deviation with diplopia. Examination during these attacks revealed ONM involving the superior rectus and medial rectus in the first and second patient, respectively. There was clinical evidence of intrinsic brainstem involvement with downbeat nystagmus and skew deviation in one patient without any other cerebellar or long tract signs. MRI showed evidence of demyelination involving the brainstem in both, with CSF showing positive immunological markers and with positive aquaporin-4 antibody in one patient. Both patients responded remarkably to immunomodulatory therapy and are asymptomatic at follow-up. That ONM can occur with brainstem demyelination has not been reported in the literature. This association may help in explaining the pathophysiology of ONM as secondary to segmental demyelination.

Suspected migration of cervical epidural catheter into the brainstem after a difficult catheter insertion.

We report a case of diplopia during continuous epidural injection presumably caused by catheter migration. A 61-year-old woman underwent shoulder surgery under general anesthesia with cervical epidural anesthesia. The epidural catheter was placed in the C6-C7 epidural space with some difficulty before general anesthesia. The depth of the catheter placed under the skin was 10 cm. On POD 2, the patient noticed diplopia and developed dysarthria despite of good pain control so far. She complained of sudden headache after the rate of continuous epidural infusion was increased to relieve postoperative pain. Computed tomography and T2-weighted cerebral magnetic resonance imaging revealed an air image and surrounding edema in the pons. Diplopia and dysarthria disappeared after ceasing continuous epidural injection. A 15-cm-long mark under the skin and leak of colorless clear fluid from the puncture site were noted at removal of the catheter. On POD 13, diplopia recurred, which improved gradually. On the 9-month radiologic follow-up, we considered that the symptoms on POD 2 were caused by migration of the epidural catheter into the pons and that her later diplopia was induced by intracranial hypotension syndrome. One should be aware that such an unexpected migration of the catheter can occur following a difficult insertion.

Intra-operative navigation for orbital reconstruction to correct diplopia and enophthalmos with endoscopic assisted technique: first case report in Thailand.

BACKGROUND AND OBJECTIVE: In an orbital fracture involving diplopia, enophthalmos is a major problem to be corrected because ofsoft tissue swelling and limited incision, which causes inaccurate restoration oforbital anatomy and reestablishing orbital volume. Pre-operative computerized planning combined with intra-operative navigation and endoscopy are used to create the accurate anatomical orbital position and effectively correct the posttraumatic diplopia and enophthalmos. CASE REPORT: An 18-year-old Thai male with diplopia and enophthalmospresented aposttraumatic left orbital fracture two months prior Three-dimensional CTscan of the facial bone confirmed the fracture. The patient required surgical treatment for correction of the orbital fracture. The intra-operative navigator and endoscopy-assisted technique were used. Pre- and post-operative pictures were compared, indicated the successful correction of enophthalmos and clinical correction of diplopia. CONCLUSION: Intra-operative navigator combined with endoscopy-assisted technique were a new surgical procedure that could correct the orbital deformity problem involving enophthalmos and diplopia more effective.

Partial third nerve palsy and ocular neuromyotonia from displacement of posterior communicating artery detected by high-resolution MRI.

Ocular neuromyotonia is an unusual condition in which sustained, undesired contraction of one or more extraocular muscles occurs after normal muscle activation. Although most commonly reported after paraseller cranial irradiation for tumor, chronic nonaneurysmal vascular compression of the third nerve can produce partial ocular motor nerve paresis and ocular neuromyotonia. A 75-year-old woman presented with intermittent left-gaze-evoked binocular diplopia. She had an incomplete right third nerve palsy but became symptomatically diplopic and esotropic upon sustained left gaze. High-resolution brain magnetic resonance imaging showed displacement of the right posterior communicating artery and contact with the right third nerve. Gaze-evoked diplopia resolved with carbamazepine, but a partial third nerve paresis remained.

CT and MRI diagnosis of silent sinus syndrome.

PURPOSE: The purpose of this study was to determine computed tomography (CT) and magnetic resonance (MR) findings of silent sinus syndrome (SSS) - a rare clinical entity with the constellation of progressive enophthalmos and hypoglobus, facial asymmetry and possible diplopia - due to otherwise asymptomatic maxillary sinus disease. MATERIALS AND METHODS: We reviewed the pre- and postoperative CT and MR images of six patients with a definitive diagnosis of SSS and compared the radiological and clinical findings with those reported in the literature. RESULTS: The CT and MR studies demonstrated in all cases the most characteristic imaging features of SSS reported in the literature. CONCLUSIONS: Both CT and MR imaging enable a diagnosis of SSS to be made, but CT provides a better depiction of all features of SSS necessary for diagnosis and differentiation from other sinus conditions, even in patients without a clinical suspicion of SSS.

Near-real time oculodynamic MRI: a feasibility study for evaluation of diplopia in comparison with clinical testing.

OBJECTIVE: To demonstrate feasibility of near-real-time oculodynamic magnetic resonance imaging (od-MRI) in depicting extraocular muscles and correlate quantitatively the motion degree in comparison with clinical testing in patients with diplopia. METHODS: In 30 od-MRIs eye movements were tracked in the horizontal and sagittal plane using a a TrueFISP sequence with high temporal resolution. Three physicians graded the visibility of extraocular muscles by a qualitative scale. In 12 cases, the maximal monocular excursions in the horizontal and vertical direction of both eyes were measured in od-MRIs and a clinical test and correlated by the Pearson test. RESULTS: The medial and lateral rectus muscles were visible in the axial plane in 93% of the cases. The oblique, superior and inferior rectus muscles were overall only in 14% visible. Horizontal (p = 0,015) and vertical (p = 0,029) movements of the right eye and vertical movement of the left eye (p = 0,026) measured by od-MRI correlated positively to the clinical measurements. CONCLUSIONS: Od-MRI is a feasible technique. Visualization of the horizontal/vertical rectus muscles is better than for the superior/inferior oblique muscle. Od-MRI correlates well with clinical testing and may reproduce the extent of eye bulb motility and extraocular muscle structural or functional deteriorations. Key Points • Oculodynamic MRI technique helps clinicians to assess eye bulb motility disorders • MRI evaluation of eye movement provides functional information in cases of diplopia • Oculodynamic MRI reproduces excursion of extraocular muscles with good correlation with clinical testing • Dynamic MRI sequence supplements static orbital protocol for evaluation of motility disorders.

Occurrence and clinicotopographical correlates of brainstem infarction in patients with diabetes mellitus.

BACKGROUND: The goal of the study was to clarify the association between diabetes mellitus (DM) and brainstem infarctions (BSIs) and to investigate the clinicotopographic characteristics of BSIs in patients with diabetes. METHODS: Data were retrospectively reviewed for 1026 consecutive patients admitted to our hospital because of acute cerebral infarctions from January 2004 to August 2010. Acute symptomatic BSIs were explored on radiologic images and classified into multiple infarctions with BSIs, multifocal BSIs, and monofocal BSIs. Isolated BSIs were further classified based on the vertical distribution into midbrain, pontine, and medullary infarctions, and on the horizontal distribution into anterior-dominant, posterior-dominant, and anterior/posterior BSIs. Neurologic symptoms of BSIs and clinical background were compared between DM and non-DM patients. RESULTS: The prevalence of BSIs was 2.6-fold higher (P < .0001) in DM patients. Logistic regression analysis including age, sex, smoking, previous stroke, atrial fibrillation, other cardiac diseases, hypertension, hyperlipidemia, and DM showed that DM was independently associated with BSIs (odds ratio [OR] 2.814; 95% confidence interval [CI] 1.936-4.090; P < .0001). Compared with non-DM patients, DM patients showed more frequent monofocal BSIs (P < .0001) and multifocal BSIs (P = .0296). Monofocal BSIs (n = 114) more frequently involved the pons (P < .0001) and medulla (P = .0212). Anterior-dominant BSIs (P < .0001) were more common in DM patients than in non-DM patients. Symptoms of BSIs included more frequent motor paresis (P = .0180) and less frequent diplopia (P = .0298) in DM patients than in non-DM patients. CONCLUSIONS: DM is important in the development of BSIs, and the associated clinical characteristics include more frequent motor paresis and less frequent diplopia.

Pineal germinoma in a young adult: A case report.

BACKGROUND: Intracranial germinomas (GN) are rare cancers that primarily affect children, making them rarer still in adults. Standard treatment for this neoplasm includes neoadjuvant chemotherapy (NC) followed by radiotherapy (RT) or RT at a higher dose and larger field. These recommendations are based on studies focused mostly on children; it is currently unclear whether this treatment is applicable to adults. CASE: We present a case of a 23-year-old adult male with no underlying pathologies, drug allergies, or family history of cancer, who presented for medical evaluation with blurred vision, diplopia, forgetfulness, and weight loss starting 3-4 months before the evaluation. Clinical examination indicated Parinaud's Syndrome. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a pineal tumor with ependymal dissemination in both lateral ventricles, which was causing obstructive hydrocephalus. The patient had surgery consisting of ventriculostomy, Holter shunt insertion, cisternal ventricular intubation, and cisterna magna anastomosis to improve ventricular drainage. Pathology confirmed pineal germinoma. Cerebrospinal fluid cytology and MRI of the axis were negative. Four cycles of NC were given to the patient (carboplatin, etoposide, and ifosfamide), with reduced dosage. Once a partial volumetric response was confirmed, whole-ventricular radiotherapy (WVR) was initiated with a total tumor bed dose of 45 Gy over 25 sessions in 5 weeks. Optimum clinical results were observed, and no short-term (<90 day) radiation toxicity was observed. The patient was able to resume his normal activities soon after treatment. Follow-ups over 2 years post-surgery indicated continued control of the lesion and absence of symptoms except for mild diplopia. CONCLUSION: Although this is a case report, these data suggest that a reduced NC course and WVR may effectively treat adult GN. This protocol likely decreases the risk of undesirable NC and RT secondary effects, while providing excellent local control; however, using a narrower RT field is not recommended.

Population pharmacokinetic model development and its relationship with adverse events of oxcarbazepine in adult patients with epilepsy.

This study aimed to develop a pharmacokinetic (PK) model of oxcarbazepine (OXC) and analyse the relationship between monohydroxylated derivative (MHD), an active metabolite of OXC, and the adverse events of OXC. We obtained 711 OXC samples from 618 patients with epilepsy who were enrolled in the Epilepsy Registry Cohort of Seoul National University Hospital from February 2011 to January 2014. The plasma PK model was developed using a nonlinear mixed-effect modelling method with NONMEM (ver 7.3). A one-compartment model with a first-order absorption model and proportional residual error adequately described the MHD concentration-time profiles. The only covariate incorporated for CL/F and V/F was body weight. Of the 447 patients analysed, 28 (6.26%) had dose-related adverse events (DRAEs), which were dizziness, somnolence, headache, and diplopia. For DRAE occurrence, the cut-off values of the MHD trough and AUC were 12.27 mg/L (specificity 0.570, sensitivity 0.643) and 698.5 mg h/L (specificity, sensitivity 0.571), respectively. Multivariate analysis showed the sole dizziness symptom was significantly associated with both the MHD trough and the AUC (p = 0.013, p = 0.038, respectively). We newly developed a population PK model using sparse sampling data from patients with epilepsy, and the model better reflects the actual clinical situation.

Rapid recovery of motor and cognitive functions after resection of a right frontal lobe meningioma in a child.

PURPOSE: The purpose of this study is to provide longitudinal data on neurological and neuropsychological restitution following resection of an extra-axial space-occupying lesion. CASE REPORT: A comprehensive neuropsychological test battery was utilised preoperatively and 1, 4, 11, and 24 months, respectively, after removal of a parasagittal meningioma compressing the right frontal lobe in a right-handed 6-year-old boy with average intelligence. RESULTS: Symptoms related to brain compression (left-sided hemiparesis, diplopia) resolved shortly after surgery. Recovery of specific cognitive functions (short- and long-term memory, attention, and dichotic listening performance) was more protracted. CONCLUSION: Here, we illustrate the potential of a structured follow-up analysis, based on neuropsychological testing. We were able to distinguish separate time-courses for neurological functions but even more distinct within complex neuropsychological processes. This time-dependent recovery should be considered when designing longitudinal follow-up studies.