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Objectives: Recent advancements in high-resolution imaging have improved the diagnostic assessment of magnetic resonance imaging (MRI) for intralabyrinthine schwannoma (ILS). This systematic review aimed to evaluate the diagnostic performance of MRI for patients with ILS., Methods: Ovid-MEDLINE and EMBASE databases were searched for related studies on the diagnostic performance of MRI for patients with ILS published up to February 10, 2020. The primary endpoint was the diagnostic performance of MRI for ILS. The quality of the enrolled studies was assessed using tailored questionnaires and the Quality Assessment of Diagnostic Accuracy Studies-2 criteria., Results: Overall, 6 retrospective studies that included 122 patients with ILS from a parent population of 364 were included. The sample size, parent population and its composition, reference standard, detailed parameters of MRI, and even the diagnostic methods varied between the studies. The studies had moderate quality. The sensitivity of combination of T2WI and CE-T1WI was over 90%. Relative sensitivity of T2WI comparative to CE-T1WI ranged from 62% to 100%, and the specificity were 100%., Conclusions: MRI has acceptable diagnostic performance for ILS. There is a need for well-organized research to reduce the factors causing heterogeneity., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

The aim of this study was to determine the frequency, clinical and radiological features, and efficacy of clinical evaluation and perfusion-weighted imaging (PWI) for the prediction of final stroke in patients with DWI/MRI-negative posterior circulation stroke (PCS) presenting acute dizziness/vertigo. From our comprehensive prospective stroke registry of acute ischemic stroke during a 7-year period, we identified 1846 consecutive patients with PCS, 850 of whom presented with acute dizziness/vertigo. Of these 850 patients, initial DWI-MRI was negative in 35 (4.1%). In these 35 patients, dizziness/vertigo was acute prolonged in 31 and recurrent transient in 4. Focal neurological signs or profound imbalance were present in 16/35 or 18/34, respectively. Spontaneous nystagmus was absent in 21/35; the HINTS protocol (head impulse, nystagmus, and test-of-skew) was not applicable to them. In 12/26 patients, PWI was positive and the same time as DWI was negative. The usual site of lesion was the lateral medulla (n = 18). Twenty-nine patients (83%) had small strokes, while 19 (54%) had large vessel strokes. The sensitivity of systematic clinical evaluation adopting neurological examination, HINTS plus, and assessment of equilibrium was 83%, for prediction of final stroke and 100% when combined with PWI. An integrated approach using systematic neurological and neuro-otological examinations combined with PWI accurately diagnoses PCS presenting with acute dizziness/vertigo. Although most patients with acute vertigo and MRI-negative PCS have small brainstem strokes, about a half have large vessel stroke with greater risk of progression requiring prompt treatment.

Background and Purpose: The aim of this study was to determine the prevalence of stroke and efficacy of bedside evaluation in diagnosing stroke in acute transient vestibular syndrome (ATVS)., Methods: We performed a prospective, single-center, observational study that had consecutively recruited 86 patients presenting with ATVS to the emergency department of Pusan National University Yangsan Hospital from January to December 2014. All patients received a constructed evaluation, including HINTS plus (head impulse, nystagmus patterns, test of skew, and finger rubbing) and brain magnetic resonance imagings. Patients without an obvious cause further received perfusion-weighted imaging. Multivariable logistic regression was used to determine clinical parameters to identify stroke in ATVS., Results: The prevalence of stroke was 27% in ATVS. HINTS plus could not be applied to the majority of patients because of the resolution of the vestibular symptoms, and magnetic resonance imagings were falsely negative in 43% of confirmed strokes. Ten patients (12%) showed unilateral cerebellar hypoperfusion on perfusion-weighted imaging without an infarction on diffusion-weighted imaging, and 8 of them had a focal stenosis or hypoplasia of the corresponding vertebral artery. The higher risk of stroke in ATVS was found in association with craniocervical pain (odds ratio, 9.6; 95% confidence interval, 2.0-45.2) and focal neurological symptoms/signs (odds ratio, 15.2; 95% confidence interval, 2.5-93.8)., Conclusions: Bedside examination and routine magnetic resonance imagings have a limitation in diagnosing strokes presenting with ATVS, and perfusion imaging may help to identify strokes in ATVS of unknown cause. Associated craniocervical pain and focal neurological symptoms/signs are the useful clues for strokes in ATVS., (© 2017 American Heart Association, Inc.)

To investigate structural, metabolic, and functional connectivity changes in visual and oculomotor structures in a patient with paraneoplastic opsoclonus-myoclonus syndrome, serial resting-state functional and structural MRI, and FDG-PET data were collected during the acute stage and later on when the opsoclonus had resolved. In the acute stage, an FDG-PET scan demonstrated a substantially increased metabolism in structures around the deep cerebellar nuclei [e.g., fastigial nucleus (FN)] and a relatively reduced metabolism in the bilateral occipital lobes which normalized over 12 months. Functional connectivity increased initially between the seeds of the oculomotor and visual systems, including the primary and motion-sensitive visual cortex, frontal eye fields, superior colliculus, and cerebellar oculomotor vermis (OMV), and then decreased in the chronic stage as the symptoms resolved. The functional connectivity between the OMV and FN showed a positive correlation during the acute stage, which decreased later on. We provide a descriptive presentation of the changes of abnormal functional connectivity throughout visuo-oculomotor brain areas during opsoclonus and suggest directions for further research on the pathogenesis of opsoclonus.

Internuclear ophthalmoplegia (INO) indicates a lesion involving the medial longitudinal fasciculus (MLF) that interconnects the abducens nucleus and medial rectus subnucleus of the oculomotor nuclear complex. In fact, rostral-caudal localization value of the INO is often limited except when it accompanies symptoms and signs owing to involvement of nearby structures. Ataxia is often observed in lesions involving the cerebellum or the fibers to and from it anywhere in the brainstem. Herein, we sought to determine the localizing value of INO plus ataxia in the rostrocaudal axis of the brainstem. Thirty patients with INO plus limb or truncal ataxia were subjected to analyses. For comparison, 20 patients with isolated INO without any ataxia served as the control. We determined the lesion extent in the MRIs responsible for INO plus ataxia using a probabilistic lesion mapping and subtraction analysis and analyzed the neuro-otologic findings using video-oculography. In patients with INO with limb or truncal ataxia, the responsible lesions were mostly restricted to the paramedian tegmentum at the pontomesencephalic junction. In contrast, the lesions causing isolated INO without ataxia were mostly located in the caudal or mid-pontine area. The rostro-caudal distribution of the lesions was similar among the patients with only limb ataxia (n = 3), both limb and truncal ataxia (n = 10), and only truncal ataxia (n = 17). INO plus ataxia indicates a lesion involving the MLF at the pontomesencephalic junction. Damage to the brachium conjunctivum or mesencephalic locomotor region may explain the ataxia in association with INO in lesions involving this area.

A 33-year-old woman with Wernicke's encephalopathy (WE) due to poor oral intake after allogeneic stem cell transplantation for acute myeloid leukemia showed a sequential development of bilateral gaze-evoked nystagmus (GEN), rightward gaze palsy, and upbeat nystagmus. Initial MRIs obtained when she had GEN only showed a lesion involving the medullary tegmentum, and follow-up MRIs revealed additional lesions in the pontine and midbrain tegmentum along with development of rightward gaze palsy, and finally bilateral medial thalamus lesions in association with upbeat nystagmus. The evolution of abnormal ocular motor findings and serial MRI changes in our patient with WE provide imaging evidence on relative vulnerability of the neural structures, and on the progression of lesions and ocular motor findings in thiamine deficiency., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Given the characteristics of typewriter tinnitus, the paroxysmal nature with an excellent response to carbamazepine, the tinnitus may be ascribed to NVCC affecting the cochlear nerve [[2], [6]]. Dear Sirs, Vestibular paroxysmia (VP) is characterized by brief and recurrent vertigo that respond well to carbamazepine or oxcarbazepine [[1]]. Our patient presented with paroxysmal attacks of vertigo and typewriter tinnitus that resolved with carbamazepine. [Extracted from the article]

Purpose: Möbius syndrome is characterized by abducens and facial nerve palsy. However, the presence/absence of corresponding cranial nerves on MRI was not fully evaluated. The purpose of this study was to investigate the mechanism of Möbius syndrome by associating the presence of abducens and facial nerves on MR imagings with clinical features.Methods: We retrospectively reviewed the medical records and MR imagings of nine patients with Möbius syndrome between January 2004 and October 2015. The presence/absence of abducens and facial nerves on MR imaging, as well as corresponding neuro-ophthalmologic clinical features, was investigated.Results: Facial palsy was bilateral in six and unilateral in three patients. Abduction was limited bilaterally in five and unilaterally in four patients. The degrees of facial palsy and abduction limitation were variable, and asymmetric even in the bilateral cases. MR imaging documented bilateral absence of the abducens and facial nerves in six, absence of unilateral facial nerve and bilateral abducens nerves in one, and absence of facial and abducens nerves unilaterally on the same side in another. Both abducens and facial nerves were visualized bilaterally only in the one remaining patient.Conclusions: The absence of abducens and facial nerves on MR imaging was mostly correlated with the findings of facial palsy and abduction limitation in patients with Möbius syndrome. MR imaging aids in diagnosis of Möbius syndrome by documenting the absence or presence of abducens and facial nerves. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 75-year-old woman reported two episodes of spontaneous vertigo with illusory self-motion lasting several minutes. It mentions that the patient underwent brain magnetic resonance imaging revealed an acute infarction involving the deep white matter of the left superior temporal gyrus. She was treated with clopidogrel and atorvastatin.

Isolated vestibular syndrome may occur all along the vestibular pathways from the peripheral labyrinth to the brain. By virtue of recent developments in clinical neurotology and neuroimaging, however, diagnosis of isolated central vestibulopathy is increasing. Here, we review five distinct syndromes of isolated central vestibular syndrome from lesions restricted to the vestibular nuclei, the nucleus prepositus hypoglossi, the flocculus, the tonsil, and the nodulus, and introduce a new vestibular syndrome from isolated involvement of the inferior cerebellar peduncle. Decreased responses to head impulses do not exclude a central lesion as a cause of isolated vestibular syndrome. Brain imaging, including diffusion-weighted magnetic resonance imaging (MRI), may be falsely negative during the acute phase in patients with isolated vestibular syndrome because of a stroke. Central signs should be sought carefully in patients with isolated vertigo, even when the patients show the features of peripheral vestibulopathy and negative MRIs. Recognition of these isolated central vestibular syndromes would aid in defining the lesions responsible for various vestibular manifestations in central vestibulopathy. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 49-year-old man who was taken to the emergency room due to left ear tinnitus and vertigo. Medical diagnosis of the patient with the use of magnetic imaging resonance for the Meniere disease (MD) and response of the vestibular-evoked myogenic potentials (VEMPs) was performed. The article discusses the effect of the VEMPs leading to the labyrinthine ischemia in the patient.

Crossed vertical gaze palsy refers to a rare combination of elevation paresis in one eye and depression palsy in the fellow eye. It was once reported in a patient with unilateral infarction involving the mesodiencephalic junction, and was ascribed to selective disruption of the fibers projecting from the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) to the oculomotor nuclear complex. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare cause of ophthalmoplegia and crossed vertical gaze palsy has not been described in this disorder. Our patient with a circumscribed acute infarction involving the left mesodiencephalic junction due to CADASIL showed both upward and downward gaze palsy in both eyes, but more marked depression paresis in the ipsilesional eye and more conspicuous elevation deficit in the contralesional eye, which was consistent with crossed vertical gaze palsy. We provide alternate explanation for this rare phenotype of vertical gaze palsy. Selective disruption of riMLF fibers may cause crossed vertical gaze palsy in unilateral mesodiencephalic lesion. [ABSTRACT FROM AUTHOR]

Abstract: In polycythemia vera (PV), even though thrombotic complications are common, ophthalmoplegia rarely occurs, and oculomotor nerve palsy has not been described as an initial manifestation. We present a 53-year-old woman who developed right oculomotor nerve palsy and investigation disclosed PV as the cause of ophthalmoplegia. MRI also revealed an acute silent infarction in the right frontal subcortex. PV may manifest with oculomotor nerve palsy owing to a thrombotic complication and should be considered in the differential diagnosis of acute ophthalmoplegia. [Copyright &y& Elsevier]

Pupil-sparing inferior divisional oculomotor nerve palsy is rare and has not been described in Tolosa-Hunt syndrome (THS). A 24-year-old man with right supraorbital pain and binocular vertical diplopia for 2 weeks underwent full neuro-ophthalmolgical evaluations including brain magnetic resonance imaging (MRI). The patient showed right exo- and hypertropia with impaired depression and adduction of the right eye. The pupils were normal. Other findings of neurological examination and brain MRI were normal. The headache and ophthalmoplegia improved within 2 days of corticosteroid treatment. In view of the pupil sparing, the patient may have a lesion selectively involving the shorter branch of the oculomotor inferior division in the orbit. THS may manifest with pupil-sparing inferior divisional oculomotor palsy. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: This paper aims to determine the frequency of isolated labyrinthine infarction as an initial manifestation of anterior inferior cerebellar artery (AICA) territory infarction and discuss its clinical implication. Methods: We studied 54 consecutive patients with AICA infarction diagnosed by brain MRI from the acute stroke registry. Results: We identified 4 patients (7.4%) with AICA territory infarction who initially presented with vertigo and hearing loss mimicking acute labyrinthitis and subsequently suffered from delayed neurological deficits. All patients had normal brain MRIs including diffusion-weighted images at the time of isolated audiovestibular loss, but all subsequently exhibited acute infarct(s) in the AICA territory on follow-up brain MRIs when they developed additional neurological deficits. Conclusion: Labyrinthine infarction may be a warning sign of impending pontocerebellar infarction in the AICA territory. Our results indicate that even a classical peripheral presentation, e.g., acute hearing loss, and acute vertigo with unidirectional nystagmus, may be a manifestation of ischemic stroke in the posterior circulation. [Copyright &y& Elsevier]

Two women presented at our clinic with vision blurring following Varicella zoster virus (VZV) vaccination, 3 weeks and 1 week ago. Ophthalmologic examination and magnetic resonance imaging revealed bilateral and unilateral optic neuritis, respectively. One patient had a history of optic neuritis in the fellow eye 33 years ago without recurrence since then. Both patients completely recovered after treatment with high dose intravenous methylprednisolone followed by a tapered dose of oral prednisolone. This is the first report of optic neuritis occurring in relation to VZV vaccination. [ABSTRACT FROM AUTHOR]

Abstract: A 74-year-old woman with sudden vertigo and dysarthria showed spontaneous nystagmus beating rightward, upward and clockwise. She also exhibited dysfunction of the left utricle and superior and horizontal canals, which was documented by head impulse and heave tests, measurements of ocular torsion and subjective visual vertical tilt, bithermal caloric tests, and rotation axis analysis of the spontaneous nystagmus. In contrast, functions of the left posterior canal, saccule, and cochlea were remained intact, as evidenced by normal head impulse test, audiometry and vestibular evoked myogenic potential. MRI revealed an acute infarction in the left lateral pons of anterior inferior cerebellar artery (AICA) territory. Our patient with AICA infarction showed isolated damage to the superior vestibular labyrinth or its afferents with preservation of the inferior vestibular labyrinth and auditory function. Our patient provides further evidence that isolated damage to the superior vestibular labyrinth or its afferents may be a mechanism of isolated vascular vertigo. [Copyright &y& Elsevier]

Abstract: Antemortem diagnosis of isolated inner ear infarction remains a diagnostic challenge since current imaging techniques do not permit differentiation of isolated labyrinthine infarction from other more benign disorders involving the inner ear. An 81-year-old woman with diabetes, hypertension and hypercholesterolemia presented with vertigo and hearing loss. Initial examination revealed findings of left cochleovestibular labyrinthitis without brainstem or cerebellar signs. MRIs including the diffusion-weighted images were normal. Five days later, however, she developed dysarthria, and decreased facial sensation, subtle facial palsy, and dysmetria in the left side. Follow-up MRI was consistent with acute infarctions in the territory of anterior inferior cerebellar artery (AICA), involving the left lateral pons, left middle cerebellar peduncle, and inferolateral cerebellum. AICA infarction should be considered in acute audiovestibular syndrome, especially in aged patients with vascular risk factors, even though the classic brainstem or cerebellar signs are absent. [Copyright &y& Elsevier]

Abstract: A 66-year-old man developed primary position upbeat nystagmus and bilateral internuclear ophthalmoplegia (INO). Video-oculography showed primary position upbeat nystagmus with exponentially decreasing slow phases, which disappeared in darkness. Brain MRI disclosed enhancing lesions involving bilateral dorsomedial pons extending from the middle to upper portion. Upbeat nystagmus in association with bilateral INO may be attributed by the damage of the cell groups of the paramedian tracts (PMT), the projections from the interstitial nucleus of Cajal (INC) to PMT, or the connections between INC and the nucleus of Roller. [Copyright &y& Elsevier]