Lateral Medullary Syndrome Following Injury of Lateral Vestibulospinal Tract: Diffusion Tensor Imaging Study.

BACKGROUND: Unilateral lesions of vestibular nucleus can cause lateral medullary syndrome. Little is known about injury of medial and lateral vestibulospinal tract (VST) after dorsolateral medullary infarct. We investigated injury of the lateral VST in patients with typical central vestibular disorder using diffusion tensor tractography (DTT). METHODS: Seven patients with lateral medullary syndrome and ten control subjects were recruited. For the medial VST, we determined seed region of interest (ROI) as medial vestibular nuclei of pons and target ROI on posteromedial medulla. For the lateral VST, the seed ROI was placed on lateral vestibular nuclei of pons, and the target ROI on posterolateral medulla. Fractional anisotropy (FA), mean diffusivity (MD), and tract volume were measured. RESULT: Reconstructed lateral VST on both sides had significantly lower FA values in patients than controls (p<0.05). Tract volume of lateral VST in affected side was significantly lower than unaffected side and control group (p<0.05). However, no DTI parameters of the medial VST differed between patients and controls (p>0.05). CONCLUSION: Injury of the lateral VST was demonstrated in patients with lateral vestibular syndrome following dorsolateral medullary infarct. Analysis of the lateral VST using DTT would be helpful in evaluation of patients with lateral medullary syndrome.

Vestibular nuclear infarction: Case series and review of the literature.

BACKGROUND AND PURPOSE: Acute vestibular syndrome (AVS) is a common cause of emergency admittance and has very rarely been reported due to a vestibular nucleus infarction. Initial magnetic resonance imaging studies (MRIs) including diffusion-weighted images may reveal normal results and even bedside examination tests like HINTS battery which involves head impulse test (HIT), nystagmus and test of skew can be challenging in differing a peripheral vestibulopathy from a central lesion. METHODS: Four patients seen in the emergency department with AVS and evaluated with HINTS battery, cervical vestibular-evoked myogenic potentials (cVEMP) and cranial MRI revealing infarcts restricted to vestibular nuclei were evaluated. RESULTS: In two patients spontaneous nystagmus beating towards the unaffected side was present. In one patient spontaneous nystagmus changed direction on looking to the affected side. In the fourth gaze evoked nystagmus was present without any spontaneous nystagmus. In all, HIT was positive to the affected side. In three cVEMPs was studied revealing delayed latency, reduced amplitude p13/n23 potentials on the lesioned side in two of them. Initial MRIs including diffusion-weighted images disclosed acute infarction in the area of the vestibular nuclei in two patients, with normal results in the other two. Follow-up MRI's performed 48 hours later revealed vestibular nuclear infarction. CONCLUSION: It is not always easy to differentiate small lesions restricted to central vestibular structures from peripheral vestibular lesions both on clinical and radiological grounds. Follow-up cranial MRI is necessary in patients with known vascular risk factors.

Ramsay Hunt syndrome associated with solitary nucleus, spinal trigeminal nucleus and tract, and vestibular nucleus involvement on sequential magnetic resonance imaging.

Ramsay Hunt syndrome (RHS) is an acute peripheral facial nerve paralysis typically accompanied by erythematous vesicular lesions of the auricular skin. The etiology is considered to be geniculate ganglionitis due to reactivation of varicella-zoster virus (VZV). Encephalitis is a rare but serious complication of VZV reactivation. Clarifying the regional and temporal evolution of the lesions on magnetic resonance imaging (MRI) would help with understanding the pathology of the lesion, but this information is lacking in encephalitis with RHS. Therefore, here, we reviewed sequential MR images in three RHS cases complicated by brainstem lesions. All the regions of the lesions represent specific neuronal structures-the ipsilateral solitary nucleus (SN) and spinal trigeminal nucleus and tract (STNT) in case 1; bilateral SN, ipsilateral STNT, and vestibular nucleus in case 2; ipsilateral SN and vestibular nucleus in case 3-and this seems to account for the persistent robust symptoms. Case 1 initially showed no abnormalities on MRI and cases 2 and 3 showed weak signals on the first MRI which subsequently plateaued. These observations suggest the timeframe within which it becomes possible to detect regional and temporal evolution, namely, that the distribution of the affected regions expands between weeks 2 and 5 after onset of facial paralysis. These observations and the findings of a literature review indicate that the SN, STNT, and vestibular nucleus are relatively prone to developing encephalitis after RHS.

The medial vestibular nuclei, a vulnerable target in thiamine deficiency.

BACKGROUND: Bilateral medial vestibular nuclei (MVN) is a common target in thiamine depletion and results in acute vestibular failure. Involvement of the MVN was present in 27 out of 38 brainstem sections reported in the largest thiamine deficiency autopsy cohort with Wernicke's encephalopathy. METHOD: Serial clinical, imaging and vestibulo-ocular reflex gain measured with the video head impulse (vHIT) in one patient with acute thiamine deficiency. RESULTS: Low horizontal VOR gain correlated with an abnormal manual head impulse and with MRI evidence of MVN in an alcohol-dependent patient with low thiamine levels. The vertical VOR gain was either normal or mildly abnormal. Thiamine replacement and normal diet restored the VOR gain and MRI signal changes to normal. CONCLUSION: This single case study provides clinical-imaging correlation for symmetric MVN compromise in thiamine deficiency, its effect on the VOR gain and the favorable response to thiamine and diet replacement when identified early.

Sensory overload and imbalance: Resting-state vestibular connectivity in PTSD and its dissociative subtype.

BACKGROUND: The vestibular system integrates multisensory information to monitor one's bodily orientation in space, and is influenced by interoceptive awareness. Post-traumatic stress disorder (PTSD) involves typically alterations in interoceptive and bodily self-awareness evidenced by symptoms of hyperarousal, as well as of emotional detachment, including emotional numbing, depersonalization, and derealization. These alterations may disrupt vestibular multisensory integration between the brainstem (vestibular nuclei) and key vestibular cortical regions (parieto-insular vestibular cortex, prefrontal cortex). Accordingly, this study examined functional connectivity of the vestibular system in PTSD and its dissociative subtype. METHODS: Using resting-state fMRI data in SPM12 and PickAtlas, a seed-based analysis was employed to examine vestibular nuclei functional connectivity differences among PTSD (n = 60), PTSD dissociative subtype (PTSD + DS, n = 41) and healthy controls (n = 40). RESULTS: Increased vestibular nuclei functional connectivity with the parieto-insular vestibular cortex and the dorsolateral prefrontal cortex (dlPFC) was observed in PTSD and in controls as compared to PTSD + DS, and greater connectivity with the posterior insula was observed in controls as compared to PTSD. Interestingly, whereas PTSD symptom severity correlated negatively with dlPFC connectivity, clinical measures of depersonalization/derealization correlated negatively with right supramarginal gyrus connectivity. DISCUSSION: Taken together, decreased vestibular nuclei functional connectivity with key cortical vestibular regions in the PTSD + DS as compared to PTSD group, and its negative correlations with PTSD and dissociative symptoms, suggest that dysregulation of vestibular multisensory integration may contribute to the unique symptom profiles of each group. Further research examining disruption of vestibular system neural circuitry in PTSD and its dissociative subtype will be critical in capturing the neurophenomenology of PTSD symptoms and in identifying psychotherapeutic techniques that target dysfunction related to the vestibular system.

Nucleus prepositus hypoglossi lesions produce a unique ocular motor syndrome.

OBJECTIVE: To describe the ocular motor abnormalities in 9 patients with a lesion involving the nucleus prepositus hypoglossi (NPH), a key constituent of a vestibular-cerebellar-brainstem neural network that ensures that the eyes are held steady in all positions of gaze. METHODS: We recorded eye movements, including the vestibulo-ocular reflex during head impulses, in patients with vertigo and a lesion involving the NPH. RESULTS: Our patients showed an ipsilesional-beating spontaneous nystagmus, horizontal gaze-evoked nystagmus more intense on looking toward the ipsilesional side, impaired pursuit more to the ipsilesional side, central patterns of head-shaking nystagmus, contralateral eye deviation, and decreased vestibulo-ocular reflex gain during contralesionally directed head impulses. CONCLUSIONS: We attribute these findings to an imbalance in the NPH-inferior olive-flocculus-vestibular nucleus loop, and the ocular motor abnormalities provide a new brainstem localization for patients with acute vertigo.

Sequential [(18)F]FDG µPET whole-brain imaging of central vestibular compensation: a model of deafferentation-induced brain plasticity.

Unilateral inner ear damage is followed by a rapid behavioural recovery due to central vestibular compensation. In this study, we utilized serial [(18)F]Fluoro-deoxyglucose ([(18)F]FDG)-µPET imaging in the rat to visualize changes in brain glucose metabolism during behavioural recovery after surgical and chemical unilateral labyrinthectomy, to determine the extent and time-course of the involvement of different brain regions in vestibular compensation and test previously described hypotheses of underlying mechanisms. Systematic patterns of relative changes of glucose metabolism (rCGM) were observed during vestibular compensation. A significant asymmetry of rCGM appeared in the vestibular nuclei, vestibulocerebellum, thalamus, multisensory vestibular cortex, hippocampus and amygdala in the acute phase of vestibular imbalance (4 h). This was followed by early vestibular compensation over 1-2 days where rCGM re-balanced between the vestibular nuclei, thalami and temporoparietal cortices and bilateral rCGM increase appeared in the hippocampus and amygdala. Subsequently over 2-7 days, rCGM increased in the ipsilesional spinal trigeminal nucleus and later (7-9 days) rCGM increased in the vestibulocerebellum bilaterally and the hypothalamus and persisted in the hippocampus. These systematic dynamic rCGM patterns during vestibular compensation, were confirmed in a second rat model of chemical unilateral labyrinthectomy by serial [(18)F]FDG-µPET. These findings show that deafferentation-induced plasticity after unilateral labyrinthectomy involves early mechanisms of re-balancing predominantly in the brainstem vestibular nuclei but also in thalamo-cortical and limbic areas, and indicate the contribution of spinocerebellar sensory inputs and vestibulocerebellar adaptation at the later stages of behavioural recovery.

Imaging cortical activity after vestibular lesions.

In this article we will discuss our current knowledge of multisensory vestibular structures and their functions in the human cortex. Most of it derives from brain activation studies with PET and fMRI in humans conducted over the last decade. They have confirmed the existence of several separate and distinct cortical areas that were identified earlier by tracer and electrophysiological studies in animals, especially in monkeys. The patterns of activations and deactivations during vestibular stimulations in healthy subjects have been compared with those in patients with acute and chronic peripheral and central vestibular disorders. The following reviews what is presently known about the interconnections of vestibular structures, their activations and interactions with other sensory modalities, the correlations of perceptual and motor functions in normal humans, and the changes that result from strategic unilateral peripheral and central vestibular lesions such as vestibular neuritis and bilateral vestibular failure, on the one hand, and central vestibular nucleus lesions due to ischemic infarctions of the lateral medulla (Wallenberg's syndrome), on the other.

Vestibular imbalance associated with a lesion in the nucleus prepositus hypoglossi area.

BACKGROUND: The nucleus prepositus hypoglossi (NPH) is known to be a neural integrator of horizontal eye movements. Although the role of the human NPH is not well known, it may also function in postural balance, in view of its anatomic connections with the vestibular nuclei and vestibulocerebellum and of lesion studies in experimental animals. OBJECTIVE: To show that the human NPH contributes to vestibular function in addition to eye movement control. DESIGN: Case series. SETTING: University hospital. Patients Six patients with small and discrete brainstem infarctions that predominantly involved the NPH region.Main Outcome Measure Findings on magnetic resonance images. RESULTS: The NPH was affected at the lower pontine level in 2 patients and at the upper medullary level in 4. In addition to gaze-evoked nystagmus, all patients had vertigo, vomiting, and postural ataxia, suggesting vestibular dysfunction. The patients typically fell contralaterally or bilaterally to the lesion side. CONCLUSION: The NPH serves a vestibular function in addition to its oculomotor control function.

[Processing vestibular impulses in the central nervous system. Study based on positron emission tomography]. / Verarbeitung vestibulärer Impulse im Zentralnervensystem. Untersuchung anhand der Positronen-Emissions-Tomographie.

BACKGROUND AND OBJECTIVE: Functional imaging methods have opened new perspectives for vestibular research. Many authors have investigated the central connections of the system, but the differences between the reports leave further questions open. We investigated the cerebral projection of the vestibular system, using positron emission tomography in right-handed subjects. PATIENTS AND METHODS: Bilateral caloric stimulation was used in every volunteer (n = 6). This can be considered a standard method, which will make it possible to compare the results from different laboratories in the future. A detailed map of activated and deactivated brain regions is included. RESULTS: Changes caused by vestibular stimulation are portrayed. The activated regions partially correspond with previous results in the literature. We would like to point out the Brodmann 6 region as the cortical manifestation of involuntary isometric tightening of muscles. We have found many, previously unidentified regions showing decreased regional cerebral blood flow. CONCLUSIONS: We are the first to point out the functional connection between the hippocampus and the vestibular system in this report.

Morphologic changes in superior vestibulo-ocular neurons and vestibular nerve following labyrinthectomy in the cat.

Morphologic changes in ipsilateral superior vestibulo-ocular neurons (SVON) and the vestibular nerve were measured in 4 cats 8 weeks after labyrinthectomy and 4 cats 1 year after labyrinthectomy. There is a 20% decrease in SVON size and 30% decrease in rough endoplasmic reticulum and ribosomes with no change in the volume fractions of Golgi apparatus or mitochondria. In the central nervous system degeneration of the vestibular nerve terminals ipsilateral to the labyrinthectomy was represented by a 25% loss of synaptic profiles (SP) on SVON at 8 weeks and 57% loss of SP at 1 year after labyrinthectomy. There was no significant loss of fiber number in the vestibular nerve at 8 weeks post lesion but a 35% loss of fibers primarily of the large size at 1 year post lesion.

Injections of calmidazolium chloride into the ipsilateral medial vestibular nucleus or fourth ventricle reduce spontaneous ocular nystagmus following unilateral labyrinthectomy in guinea pigs.

The effects of three injections (0.5-4.5 h post-operation) of 1-[bis-(p-chlorophenyl)methyl]-3-[2,4-dichloro-beta-(2,4- dichlorobenzyloxy)phenethyl]-imidazolium chloride (calmidazolium chloride, R24571), into the ipsilateral medial vestibular nucleus or fourth ventricle, on vestibular compensation for unilateral labyrinthectomy was studied in guinea pigs. R24571, a calmodulin antagonist and inhibitor of several Ca(2+)-dependent enzymes, caused a significant reduction in the average frequency of spontaneous ocular nystagmus (spontaneous nystagmus) during the first 53 h following unilateral labyrinthectomy (n = 5), compared with vehicle-injected animals (n = 5). Although a statistical analysis was not performed on the yaw head tilt and roll head tilt data because of the large variability between animals over the 53-h period of compensation, most R24571-treated animals had less yaw head tilt (4/4 animals) and roll head tilt (4/5 animals) at 9-11 h post-labyrinthectomy than the average values for the vehicle groups at that time. The decrease in the frequency of spontaneous nystagmus following R24571 treatment was not associated with general ataxia or sedation. These results are consistent with recent biochemical studies in suggesting that intracellular pathways associated with Ca2+ may be involved in the neuronal mechanisms of vestibular compensation following unilateral labyrinthectomy.

Direct electrical stimulation of the cochlear nucleus: surface vs. penetrating stimulation.

Prosthetic stimulation of the cochlear nucleus (CN) has been used for rehabilitation of profoundly deaf patients who are not suitable candidates for cochlear implants. The goal of this article was to assess the relative effectiveness of surface vs. penetrating stimulation of the CN. Electrophysiologic and autoradiographic measures were used to study central auditory system activation elicited by direct stimulation of the CN. Eighteen pigmented guinea pigs, divided into three groups, underwent acute implantation of bipolar electrodes in the CN. One group was not stimulated and acted as a control (n = 7). Electrodes were placed on the surface of the CN in one test group (n = 4) and within the CN in a second test group (n = 7). Thresholds for electrically evoked middle latency responses (EMLR) were determined and input/output (I/O) functions were obtained. The two test groups were then pulsed with [14C]-2-Deoxyglucose (2-DG) intramuscularly and stimulated for 1 hour with biphasic; charge-balanced pulses having a total duration of 400 microseconds, a repetition rate of 100/sec, and an amplitude of 200 microA. After stimulation, animals were killed and brains were harvested and prepared for autoradiography using standard techniques. Threshold current for EMLRs in the surface-stimulated group had a mean of 67.5 +/- 23.9 microA (range, 40 to 100 microA). Thresholds for in-depth stimulated group had a mean of 11.4 +/- 3.5 microA (range, 10 to 20 microA). The saturation level of the I/O function for the surface-stimulated group had a mean of 287.5 +/- 41.5 microA (range, 250 to 350 microA). The saturation level for the in-depth stimulated group had a mean of 192.9 +/- 49.5 mciroA (range, 100 to 250 microA). The dynamic range for the surface electrodes had a mean of 13.1 +/- 2.7 dB (range, 9.9 to 15.9 dB), whereas the dynamic range for the penetrating electrodes had a mean of 24.5 +/- 2.6 dB (range, 20 to 28.0 dB). Autoradiographs generated by CNS tissue from stimulated animals demonstrated no significant difference in metabolic activity of the CN between surface and in-depth stimulated groups. However, there were highly significant differences in 2-DG uptake in the contralateral superior olivary complex, contralateral inferior colliculus, and ipsilateral and contralateral lateral lemniscus, with greater uptake in in-depth stimulated preparations. Electrophysiologic and autoradiographic data suggest that a penetrating CN prosthesis is capable of activating the auditory tract at a lower threshold, with a relatively wider dynamic range than a surface prosthesis.(ABSTRACT TRUNCATED AT 400 WORDS)