Posterior Inferior Cerebellar Artery Aneurysm Shaped Like a "Calabash Gourd" and Mimicking a Tumor.

Posterior inferior cerebellar artery aneurysms are likely to be fusiform, yet they hardly enlarge to mimic a tumor in the posterior fossa on radiology. They constitute about 3%-4% of all cerebral aneurysms. A 65-year-old woman presented with tremor in her right upper limb for 1 year and intermittent dizziness for 8 months. Interestingly, magnetic resonance imaging revealed 2 unanimously enhanced masses like mother and daughter located in the right cerebellum hemisphere. The lesion was resected via surgery, and histopathology established the diagnosis of an aneurysm. Her tremor and dizziness subsided 3 months after the surgery, and at her 2-year follow-up she was well with no further neurologic deficits.

Scuba Diving-Induced Inner-Ear Pathology: Imaging Findings of Superior Semicircular Canal and Tegmen Tympani Dehiscence.

BACKGROUND Superior semicircular canal dehiscence is an inner-ear pathology which presents with vertigo, disequilibrium, and hearing loss. Although the exact etiology of superior semicircular canal dehiscence is unknown, it is thought that an increase in middle-ear pressure disrupts a thin overlying temporal bone. Superior semicircular canal dehiscence is frequently seen in association with dehiscence of the tegmen tympani, which overlies the middle ear. Here, we present a case report of a 52-year-old Puerto Rican man with vertigo, dizziness, vomiting, and mild hearing loss associated with superior semicircular canal and tegmen tympani dehiscence after performing improper scuba diving techniques. CASE REPORT A 52-year-old Puerto Rican man presented to the emergency department with vertigo, dizziness, vomiting, and mild hearing loss in the right ear. The symptoms began shortly after scuba diving with inadequate decompression techniques on ascent. He was treated with recompression therapy with mild but incomplete improvement in symptoms. Bilateral temporal magnetic resonance imaging was suggestive of segmental dehiscence of the right superior semicircular canal and tegmen tympani. High-resolution computed tomography of the temporal bone confirmed right superior semicircular canal and tegmen tympani dehiscence with an intact left inner ear. CONCLUSIONS The increased inner-ear pressure that occurs during scuba diving can lead to dehiscence of the superior semicircular canal and tegmen tympani, causing vertigo and hearing loss. Performance of improper diving techniques can further increase the risk of dehiscence. Therefore, appropriate radiologic evaluation of the inner ear should be performed in such patients.

Case Report: Encephalitis Caused by Balamuthia mandrillaris in a 3-Year-Old Iranian Girl.

It is about half a century since free-living amoebae were recognized as pathogenic organisms, but there is still much we should learn about these rare fatal human infectious agents. A recently introduced causative agent of granulomatous amoebic encephalitis, Balamuthia mandrillaris, has been reported in a limited number of countries around the world. A 3-year-old girl was referred to our tertiary hospital because of inability to establish a proper diagnosis. She had been experiencing neurologic complaints including ataxia, altered level of consciousness, dizziness, seizure, and left-sided hemiparesis. The patient's history, physical examination results, and laboratory investigations had led to a wide differential diagnosis. Computed tomography (CT) scan and magnetic resonance imaging analyses revealed multiple mass lesions. As a result, the patient underwent an intraoperative frozen section biopsy of the brain lesion. The frozen section study showed numerous cells with amoeba-like appearances in the background of mixed inflammatory cells. Medications for free-living amoebic meningoencephalitis were administered. PCR assay demonstrated B. mandrillaris as the pathogenic amoeba. Unfortunately, the patient died 14 days after her admission. To our knowledge, this is the first report of B. mandrillaris meningoencephalitis in the Middle East and the first time we have captured the organism during a frozen-section study.

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.

Neurological manifestations of COVID-19: available evidences and a new paradigm.

The recent pandemic outbreak of coronavirus is pathogenic and a highly transmittable viral infection caused by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV2). In this time of ongoing pandemic, many emerging reports suggested that the SARS-CoV-2 has inimical effects on neurological functions, and even causes serious neurological damage. The neurological symptoms associated with COVID-19 include headache, dizziness, depression, anosmia, encephalitis, stroke, epileptic seizures, and Guillain-Barre syndrome along with many others. The involvement of the CNS may be related with poor prognosis and disease worsening. Here, we review the evidence of nervous system involvement and currently known neurological manifestations in COVID-19 infections caused by SARS-CoV-2. We prioritize the 332 human targets of SARS-CoV-2 according to their association with brain-related disease and identified 73 candidate genes. We prioritize these 73 genes according to their spatio-temporal expression in the different regions of brain and also through evolutionary intolerance analysis. The prioritized genes could be considered potential indicators of COVID-19-associated neurological symptoms and thus act as a possible therapeutic target for the prevention and treatment of CNS manifestations associated with COVID-19 patients.

Diffusion tensor imaging in orthostatic tremor: a tract-based spatial statistics study.

OBJECTIVE: The pathogenesis of orthostatic tremor (OT) is unknown. We investigated OT-related white matter changes and their correlations with scores from a neuropsychological testing battery. METHODS: Diffusion tensor imaging measures were compared between 14 OT patients and 14 age- and education-matched healthy controls, using whole-brain tract-based spatial statistics analysis. Correlations between altered diffusion metrics and cognitive performance in OT group were assessed. RESULTS: In all cognitive domains (attention, executive function, visuospatial ability, verbal memory, visual memory, and language), OT patients' cognitive performance was significantly worse than that of healthy controls. OT patients demonstrated altered diffusivity metrics not only in the posterior lobe of the cerebellum (left cerebellar lobule VI) and in its efferent cerebellar fibers (left superior cerebellar peduncle), but also in medial lemniscus bilaterally (pontine tegmentum), anterior limb of the internal capsule bilaterally, right posterior limb of the internal capsule, left anterior corona radiata, right insula, and the splenium of corpus callosum. No relationship was found between diffusion measures and disease duration in OT patients. Diffusion white matter changes, mainly those located in right anterior limb of the internal capsule, were correlated with poor performance on tests of executive function, visuospatial ability, verbal memory, and visual memory in OT patients. INTERPRETATION: White matter changes were preferentially located in the cerebellum, its efferent pathways, as well as in the pontine tegmentum and key components of the frontal-thalamic-cerebellar circuit. Further work needs to be done to understand the evolution of these white matter changes and their functional consequences.

Hereditary Angioedema Type 1 with Recurrent Dizziness.

A 41-year-old woman presented with recurrent dizziness. After an attack of dizziness, she felt edematous sensations in her hands. However, according to photographs taken during the attack, the edema on the back of the patient's hands and fingers appeared mild. Laboratory examinations revealed a low C4 and C1 inhibitor (INH) activity. A direct sequencing analysis of C1INH revealed a pathogenic gene mutation. Based on these results, she was diagnosed with hereditary angioedema (HAE) type 1. These findings indicate that HAE can cause recurrent dizziness, and it should therefore be included in the differential diagnosis in patients with recurrent neurologic symptoms, even in the absence of severe edema.

Cervical Intervertebral Disc Degeneration Contributes to Dizziness: A Clinical and Immunohistochemical Study.

OBJECTIVES: Dizziness often happens in patients with chronic neck pain with only cervical disc degeneration but without cervical radiculopathy or myelopathy. We prospectively selected a series of patients who showed cervical disc degeneration with concomitant chronic neck pain and intractable dizziness who did not respond to conservative treatment to test a new diagnostic method for this dizziness, to analyze the results of anterior cervical discectomy and fusion (ACDF) surgery based on the test, and to explore its pathogenesis. METHODS: Seventy-seven patients who had a transient neck pain and dizziness relief after injection of bupivacaine into a suspected disc were included in the study. In total, 52 underwent ACDF as surgery group, and 25 refused surgery and accepted conservative treatments as conservative group from June, 2015 to October, 2016 with subsequent follow-up to 1 year. The outcomes were visual analogue scale for neck pain, Neck Disability Index, and intensity and frequency of dizziness. During ACDF, the 72 specimens of degenerative cervical discs were collected to determine the innervation in degenerative cervical discs immunohistochemically. RESULTS: After surgery, the patients experienced a significant reduction in neck pain and dizziness. Symptomatic relief in surgery group was obviously better than conservative group at each time point of follow-up (P = 0.001). Ruffini corpuscles and substance P-positive free nerve fibers were obviously increased in the number and deeply ingrown into the inner degenerative cervical discs. CONCLUSIONS: Current clinical and immunohistochemical studies strongly suggest that chronic neck pain and intractable dizziness in this series of patients stem from the degenerative cervical discs.

Oxidative Stress in Cerebral Small Vessel Disease Dizziness Patients, Basally and After Polyphenol Compound Supplementation.

BACKGROUND: Leukoaraiosis (LA) is a common radiological finding in elderly, frequently associated with several clinical disorders, including unexplained dizziness. The pathogenesis of LA is multifactorial, with a dysfunction of cerebral microcirculation resulting in chronic hypoperfusion and tissue loss, with oxidative stress involved in this cascade. OBJECTIVE: The aim of this study was to analyse some oxidative stress biomarkers in a cohort of LA patients. METHOD: Fifty-five consecutive patients (33 males, median age 75 years) with LA were recruited. In a subgroup of 33 patients with LA and unexplained dizziness, we have then performed an open study to evaluate if 60-day supplementation with a polyphenol compound may modify these biomarkers and influence quality of life, analysed with the Dizziness Handicap Inventory (DHI) scale. RESULTS: At baseline, blood oxidative stress parameters values were outside normal ranges and compared to matched healthy controls. After the two months supplementation, we observed a significant decrement of advanced oxidation protein products values and a significant improvement of DHI. CONCLUSION: Oxidative stress biomarkers may be useful to detect redox imbalance in LA and to provide non-invasive tools to monitor disease status and response to therapy.

The impact of diabetes on mobility, balance, and recovery after repositioning maneuvers in individuals with benign paroxysmal positional vertigo.

AIM: The prevalence of benign paroxysmal positional vertigo (BPPV) is higher in people with type 2 diabetes (DM). The impact of DM on mobility, balance, and management of BPPV is unknown. This prospective study compared symptom severity, mobility and balance before and after the canalith repositioning maneuver (CRM) in people with posterior canal BPPV canalithiasis, with and without DM. METHODS: Fifty participants, BPPV (n=34) and BPPV+DM (n=16) were examined for symptom severity (dizziness handicap inventory, DHI), mobility (functional gait assessment, FGA), and postural sway (using an accelerometer in five conditions) before and after the CRM. The number of maneuvers required for symptom resolution was recorded. RESULTS: At baseline, no differences in DHI or FGA scores were seen between groups, however, people with BPPV+DM had higher sway velocity in the medio-lateral direction in tandem stance (p<0.01). After treatment, both groups improved in DHI and FGA scores (p<0.01), with no differences between groups. Decrease in sway velocity in the mediolateral direction (p=0.003) were seen in tandem stance in persons with BPPV+DM. There were no differences between the groups in the number of CRMs provided. CONCLUSIONS: This pilot study showed no differences in symptom severity, mobility deficits or efficacy of CRM treatments in people with posterior canal BPPV canalithiasis with and without DM. Future studies examining the impact of the severity and duration of diabetes, as well as the influence of diabetic peripheral neuropathy on functional performance are essential.

Detection of Leptomeningeal Metastasis by Contrast-Enhanced 3D T1-SPACE: Comparison with 2D FLAIR and Contrast-Enhanced 2D T1-Weighted Images.

INTRODUCTION: To compare the diagnostic accuracy of contrast-enhanced 3D(dimensional) T1-weighted sampling perfection with application-optimized contrasts by using different flip angle evolutions (T1-SPACE), 2D fluid attenuated inversion recovery (FLAIR) images and 2D contrast-enhanced T1-weighted image in detection of leptomeningeal metastasis except for invasive procedures such as a CSF tapping. MATERIALS AND METHODS: Three groups of patients were included retrospectively for 9 months (from 2013-04-01 to 2013-12-31). Group 1 patients with positive malignant cells in CSF cytology (n = 22); group 2, stroke patients with steno-occlusion in ICA or MCA (n = 16); and group 3, patients with negative results on MRI, whose symptom were dizziness or headache (n = 25). A total of 63 sets of MR images are separately collected and randomly arranged: (1) CE 3D T1-SPACE; (2) 2D FLAIR; and (3) CE T1-GRE using a 3-Tesla MR system. A faculty neuroradiologist with 8-year-experience and another 2nd grade trainee in radiology reviewed each MR image- blinded by the results of CSF cytology and coded their observations as positives or negatives of leptomeningeal metastasis. The CSF cytology result was considered as a gold standard. Sensitivity and specificity of each MR images were calculated. Diagnostic accuracy was compared using a McNemar's test. A Cohen's kappa analysis was performed to assess inter-observer agreements. RESULTS: Diagnostic accuracy was not different between 3D T1-SPACE and CSF cytology by both raters. However, the accuracy test of 2D FLAIR and 2D contrast-enhanced T1-weighted GRE was inconsistent by the two raters. The Kappa statistic results were 0.657 (3D T1-SPACE), 0.420 (2D FLAIR), and 0.160 (2D contrast-enhanced T1-weighted GRE). The 3D T1-SPACE images showed the highest inter-observer agreements between the raters. CONCLUSIONS: Compared to 2D FLAIR and 2D contrast-enhanced T1-weighted GRE, contrast-enhanced 3D T1 SPACE showed a better detection rate of leptomeningeal metastasis.

The conundrum of cervicogenic dizziness.

Cervicogenic or cervical dizziness is debated as an entity. However, there exists both a physiologic basis and a multitude of clinical data to make such a disease concept at least possible and worth considering. In addition, the interaction of proprioceptive and vestibular mechanisms may amplify dizziness of other origin. Cervical pain and dizziness are both common symptoms and may coincide, and neck pain or obvious dysfunction does not necessarily cause dizziness or balance disturbances. So far, there is also the lack of a proper diagnostic test for cervicogenic dizziness. On the other hand, there is growing evidence that cervical proprioceptive input is important for balance and postural control not only in animals but also in humans, and that intervention in disorders affecting the human cervical segment may relieve dizziness in some patients. It is advocated that the diagnosis should be used with care and that there is a need for better diagnostic tests. In the absence of such a test, one has to rely on preliminary criteria and a diagnosis ex juvantibus. A possible approach would require patients to present with neck pain before or in close temporal relation with dizziness; that other causes should be made at least unlikely; and that treatment of a cervical dysfunction reduces also dizziness or balance disturbance.

Imaging alterations due to squamous metaplasia in intracranial neurenteric cysts: A report of two cases.

Intracranial neurenteric cysts are rare congenital abnormalities with a broad imaging spectrum, and therefore are occasionally mistaken for other common intracranial cysts such as epidermoid and arachnoid cysts. We report two cases of neurenteric cysts in the posterior cranial fossa that were initially mistaken for other types of cysts. They exhibited signal intensity alterations in magnetic resonance imaging with significant volume expansion during their long-term observation. Both cases received surgical treatment because of clinical deterioration. Histologically, the cysts were lined by flattened or cuboidal epithelium, occasionally showing squamous metaplasia. Xanthogranulomatous inflammation and accumulation of cholesterol clefts, dry keratin and proteinaceous substance were observed in the cysts. These findings may indicate that chronic inflammation in neurenteric cysts induces squamous metaplasia, keratinization and high proteinaceous content, and causes MRI signal intensity alterations and volume expansion. We propose that MRI signal intensity alterations in neurenteric cysts may be a warning sign of their volume expansions, and thus require closer follow-up imaging and eventually surgical treatment.

Effect of External Positive and Negative Pressure on Venous Flow in an Experimental Model.

OBJECTIVE: Positive external pressure is said to decrease transmural pressure; negative pressure in the pleural cavity is widely believed to result in negative pressure in systemic chest veins. The discrepancy between erect column height and foot venous pressure has been explained on this basis. METHODS: These core concepts rest on static closed models that may not be appropriate. This study examined the effects of external pressures in a dynamic open model that may better reflect in vivo conditions. Flow in a Penrose drain enclosed in a chamber that could be positively or negatively pressurized was used. Input and output reservoirs with pressures in the physiological range provided flow. Flow and pressure were monitored in horizontal and erect models with modifications to suit particular experiments. RESULTS: The discrepancy between foot venous pressure and erect venous column height was shown in this experimental model to be a result of two flows in opposite directions (superior and inferior vena cavae) meeting at the zero reference level at the heart; the upper column pressure therefore does not register at the foot. Positive external pressure results in slowing of velocity with conversion to pressure. Internal and transmural pressures therefore do not decrease. Negative external pressure has only a marginal effect on flow; importantly, internal pressure does not become negative. In an experimental set-up it was shown that negative pressure in chest veins was not necessary for air embolism to occur. CONCLUSION: Persistent negative pressure in systemic chest veins probably does not occur. The reason for the discrepant foot venous pressure is likely to be a result of dynamic flow and not negative pressure in chest veins. External positive pressure results in slowing of velocity but the transmural pressure remains largely unchanged.

Magnetic resonance brain imaging in patients with visual vertigo.

INTRODUCTION: Patients with visual vertigo (VV) report dizziness provoked by moving visual surroundings. It has been suggested that these subjects develop a compensation strategy for a vestibulo-proprioceptive deficit and rely excessively on visual input. We have postulated that patients with VV might have brain abnormalities that interfere with appropriate processing of visual stimulation and performed a brain MRI study to verify this hypothesis. MATERIALS AND METHODS: Patients with VV of more than 3 months duration were included. They were asked to complete the Situational Characteristic Questionnaire (SCQ) that scores for the symptoms of VV. Dizzy patients without VV served as controls. A brain MRI was performed with a Siemens 1.5 Tesla scanner in patients and controls. RESULTS: Twenty-four patients with VV were included. Their mean SCQ score was 1.45 ± 0.9 (normal 0.16 ± 0.28). In 50% of patients, abnormalities in MRI imaging were found. Thirty-three percent of 27 controls demonstrated an abnormal brain MRI. The two groups were similar in respect to the prevalence of a localized hemispheric or posterior fossa lesion (P = 0.13), but VV patients had more unspecific white matter brain changes than controls (P = 0.009). Patients and controls did not differ in age and gender distribution (P = 0.9) or the history of a neurotological event preceding their symptoms (P = 0.3). CONCLUSIONS: Our study suggests that multiple white matter lesions might contribute to occurrence of the phenomenon of VV. Future prospective large-scale studies by specific MR techniques are indicated to validate our preliminary findings and elucidate the pathological mechanism of VV.

Are white matter abnormalities associated with "unexplained dizziness"?

INTRODUCTION: Although cerebral small vessel disease is a significant contributor to the development of imbalance and falls in the elderly, whether it causes dizziness is not known. METHODS: A retrospective case analysis was conducted for 122 dizzy patients referred to two neuro-otology tertiary centres in London and Pisa. Patients were divided into 'explained' causes of dizziness (e.g. benign positional vertigo, vestibular neuritis, orthostatic hypotension, cerebellar ataxias) and 'unexplained' dizziness. White matter hyperintensities (WMH) in MRI (T2 weighted and FLAIR sequences) were blindly rated according to the Fazekas scale. RESULTS: 122 patients; 58 (mean age=72, SD=7.95 years) in the 'unexplained' group and 64 (mean age=72.01, SD=8.28 years) in the 'explained' group were recruited. The overall frequency of lesions (Fazekas 1-3) significantly differed between groups (p=0.011). The frequency of severe lesions (Fazekas 3) was significantly higher in the 'unexplained' group (22%) than in the 'explained' group (5%; p=0.003). CONCLUSION: Increased severity of WMH in cases of unexplained dizziness suggests that such abnormalities are likely contributory to the development of dizziness. WM lesions may induce dizziness either because patients perceive a degree of objective unsteadiness or by a disconnection syndrome involving vestibular or locomotor areas of the brain.

[Application of vestibular rehabilitation with an introduction session in patients with chronic dizziness].

Pharmacological and non-pharmacological approaches are the treatment options for dizzy patients. Vestibular exercises are a potential treatment option for intractable dizzy patients who do not respond to conventional drug therapy. The retention rate of exercises is quite important for a successful outcome. We organized vestibular exercise courses to improve the retention rate in the outpatient groups; these courses comprised introduction sessions with a clinical psychologist and rehabilitation sessions with a clinical technologist. It is unclear whether the effects of vestibular rehabilitation are based on the improvement of the psychological parameters of vestibular function. Therefore, we evaluated the functional mechanism of vestibular rehabilitation by using questionnaires and balance function tests. Our study comprised 16 patients with chronic dizziness. The patients performing the self-administered home-based vestibular exercise courses were supervised in small groups. The patients were taught that the exercises were to be performed 4 times a day at their respective homes. The symptoms of dizziness were evaluated using the Japanese version of the dizziness handicap inventory (DHI). Anxiety and depression levels were assessed using a set of psychological questionnaires. The static and dynamic balance function was assessed using static posturography and foam posturography and by measuring the time required for performing a certain series of eye-head movement protocols at the initial visit, before rehabilitation, and 2 and 3 months after rehabilitation. Of 16 patients, 15 could successfully complete the study (93.8%). The DHI score significantly improved 1 month after the rehabilitation, whereas anxiety and depression levels did not improve. The results of static and foam posturography as well as the time required for the eye-head movements also significantly improved. The improvement in the DHI scores was not associated with psychological changes, but with the improvement in the static and dynamic balance function.

Effect of orthostasis on endothelial function: a gender comparative study.

As the vascular endothelium has multiple functions, including regulation of vascular tone, it may play a role in the pathophysiology of orthostatic intolerance. We investigated the effect of orthostasis on endothelial function using EndoPAT®, a non-invasive and user-independent method, and across gender. As sex steroid hormones are known to affect endothelial function, this study examined the potential effect of these hormones on the endothelial response to orthostasis by including females at different phases of the menstrual cycle (follicular and luteal-where the hormone balance differs), and females taking an oral contraceptive. A total of 31 subjects took part in this study (11 males, 11 females having normal menstrual cycles and 9 females taking oral contraceptive). Each subject made two visits for testing; in the case of females having normal menstrual cycles the first session was conducted either 1-7 (follicular) or 14-21 days (luteal) after the start of menstruation, and the second session two weeks later, i.e., during the other phase, respectively. Endothelial function was assessed at baseline and following a 20-min orthostatic challenge (active standing). The EndoPAT® index increased from 1.71 ± 0.09 (mean ± SEM) at baseline to 2.07 ± 0.09 following orthostasis in females (p<0.001). In males, the index increased from 1.60 ± 0.08 to 1.94 ± 0.13 following orthostasis (p<0.001). There were no significant differences, however, in the endothelial response to orthostasis between females and males, menstrual cycle phases and the usage of oral contraceptive. Our results suggest an increased vasodilatatory endothelial response following orthostasis in both females and males. The effect of gender and sex hormones on the endothelial response to orthostasis appears limited. Further studies are needed to determine the potential role of this post orthostasis endothelial response in the pathophysiology of orthostatic intolerance.