Oculopalatal Tremor Following Pontine Hemorrhage.
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ABSTRACT:
SUBMITTER: Wilks A
PROVIDER: S-EPMC6739662 | biostudies-literature | 2019 Oct
REPOSITORIES: biostudies-literature
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Oculopalatal Tremor Following Pontine Hemorrhage.
Wilks Anson A McCullough Austin A Day Gregory S GS
The Neurohospitalist 20190207 4
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Project description:Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor (PT) or oculopalatal tremor (OPT). This syndrome manifests as a synchronous tremor of the palate (PT) and/or eyes (OPT) that may also involve other muscles from the branchial arches. It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and vacuolated neurons, increased number and size of astrocytes, severe fibrillary gliosis, and demyelination. It appears on MRI as an increased T2/FLAIR signal intensity and enlargement of the inferior olive. There are two main conditions in which hypertrophic degeneration of the inferior olive occurs. The most frequent, studied, and reported condition is the development of PT/OPT and hypertrophic degeneration of the inferior olive in the weeks or months following a structural brainstem or cerebellar lesion. This "symptomatic" condition requires a destructive lesion in the Guillain-Mollaret pathway, which spans from the contralateral dentate nucleus via the brachium conjunctivum and the ipsilateral central tegmental tract innervating the inferior olive. The most frequent etiologies of destructive lesion are stroke (hemorrhagic more often than ischemic), brain trauma, brainstem tumors, and surgical or gamma knife treatment of brainstem cavernoma. The most accepted explanation for this symptomatic PT/OPT is that denervated olivary neurons released from inhibitory inputs enlarge and develop sustained synchronized oscillations. The cerebellum then modulates/accentuates this signal resulting in abnormal motor output in the branchial arches. In a second condition, PT/OPT and progressive cerebellar ataxia occurs in patients without structural brainstem or cerebellar lesion, other than cerebellar atrophy. This syndrome of progressive ataxia and palatal tremor may be sporadic or familial. In the familial form, where hypertrophic degeneration of the inferior olive may not occur (or not reported), the main reported etiologies are Alexander disease, polymerase gamma mutation, and spinocerebellar ataxia type 20. Whether or not these are associated with specific degeneration of the dentato-olivary pathway remain to be determined. The most symptomatic consequence of OPT is eye oscillations. Therapeutic trials suggest gabapentin or memantine as valuable drugs to treat eye oscillations in OPT.
| S-EPMC5490180 | biostudies-literature
Project description:The inferior olivary nuclei clearly play a role in creating oculopalatal tremor, but the exact mechanism is unknown. Oculopalatal tremor develops some time after a lesion in the brain that interrupts inhibition of the inferior olive by the deep cerebellar nuclei. Over time the inferior olive gradually becomes hypertrophic and its neurons enlarge developing abnormal soma-somatic gap junctions. However, results from several experimental studies have confounded the issue because they seem inconsistent with a role for the inferior olive in oculopalatal tremor, or because they ascribe the tremor to other brain areas. Here we look at 3D binocular eye movements in 15 oculopalatal tremor patients and compare their behaviour to the output of our recent mathematical model of oculopalatal tremor. This model has two mechanisms that interact to create oculopalatal tremor: an oscillator in the inferior olive and a modulator in the cerebellum. Here we show that this dual mechanism model can reproduce the basic features of oculopalatal tremor and plausibly refute the confounding experimental results. Oscillations in all patients and simulations were aperiodic, with a complicated frequency spectrum showing dominant components from 1 to 3 Hz. The model's synchronized inferior olive output was too small to induce noticeable ocular oscillations, requiring amplification by the cerebellar cortex. Simulations show that reducing the influence of the cerebellar cortex on the oculomotor pathway reduces the amplitude of ocular tremor, makes it more periodic and pulse-like, but leaves its frequency unchanged. Reducing the coupling among cells in the inferior olive decreases the oscillation's amplitude until they stop (at approximately 20% of full coupling strength), but does not change their frequency. The dual-mechanism model accounts for many of the properties of oculopalatal tremor. Simulations suggest that drug therapies designed to reduce electrotonic coupling within the inferior olive or reduce the disinhibition of the cerebellar cortex on the deep cerebellar nuclei could treat oculopalatal tremor. We conclude that oculopalatal tremor oscillations originate in the hypertrophic inferior olive and are amplified by learning in the cerebellum.
| S-EPMC2842510 | biostudies-literature
Project description:IntroductionOculopalatal tremor (OPT) is a late manifestation of a Guillain-Mollaret triangle lesion. Memantine has been shown to improve nystagmus in OPT, but its long-term efficacy and putative distinct effects on each plane of nystagmus and on associated phenomena (e.g., gravity perception) are largely unknown.MethodsWe conducted a 6-month open-label study to evaluate the effect of memantine in OPT patients. Baseline (visit 1), 2 (visit 2), and 6 months (visit 3) assessments included video-oculography, best corrected visual acuity (BCVA), visual function questionnaire (VFQ25), palatal tremor frequency, and subjective visual vertical (SVV). Memantine was titrated to 20 mg per day and stopped after 6 months.ResultsWe included six patients (5 females; mean age 68.5+/-9.7). At visit 2, nystagmus improved >50% only along the horizontal plane in two patients, while worsening >50% along the vertical and horizontal planes in 4 and 1 patients, respectively. At visit 3, previous improvement of nystagmus along the horizontal plane in two patients was not sustained, and it further worsened >50% along the vertical plane in 4. The mean vertical velocity and amplitude of nystagmus in the left eye significantly worsened from visit 2 to visit 3 (p = 0.028). Throughout the study, nystagmus frequency remained unchanged (p = 0.074), BCVA improved in both eyes (p = 0.047, p = 0.017), SVV progression was unpredictable (p = 0.513), and the mean VFQ-25 score (p = 0.223) and mean palatal frequency remained unchanged.ConclusionThe long-term use of memantine 20 mg per day in OPT produced a modest and only transient improvement in nystagmus, predominantly along the horizontal plane. Visual acuity improved, albeit without relevant changes in vision-related quality of life.
| S-EPMC9428258 | biostudies-literature
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| S-EPMC6974824 | biostudies-literature
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| S-EPMC8175821 | biostudies-literature
Project description:INTRODUCTION:We report a case of hypertrophic olivary degeneration due to pontine hemorrhage. A 59-year-old male with untreated hypertension suffered a primary pontine hemorrhage, which caused horizontal eye-movement limitation. Progressive neurological deterioration with involuntary eye and palatal movements began months after hemorrhage. This was accompanied by magnetic resonance imaging evidence of hypertrophic olivary degeneration at 4.5 months. BACKGROUND:Primary pontine hemorrhage often leads to impairment of eye movements and diplopia. Hypertrophic olivary degeneration can also emerge months after hemorrhage, producing involuntary pendular eye movements. Neither the natural history of voluntary eye movements nor the emergence of involuntary eye movements after pontine hemorrhage has been previously quantified. METHODS:We used an optokinetic task that enabled measurement of eye movements. It provided real-time feedback on the ability to track continuously and saccade quickly in a pursuit task. The feedback motivated the patient to use the system repeatedly in his home. From 3 months after hemorrhage, the patient used the system for 9 months, allowing us to quantify changes in his eye movements. RESULTS:Horizontal gaze impairments were manifest in our task as limitation in horizontal range of motion, as well as delay in initiation of the right eye's movement during left-to-right pursuit. Improvement in these impairments was measured over the course of months 3-7 post hemorrhage. In addition, the emergence of vertical pendular nystagmus was identified in the subject at 4 months. Analysis of the eye-movement records revealed presymptomatic oscillatory eye movements whose amplitude had grown steadily over the course of 3 weeks, prior to a sharp increase in amplitude that coincided with the patient's first report of oscillopsia. Horizontal pendular nystagmus emerged 7.4 months after the hemorrhage, primarily in the left eye. CONCLUSION:An eye-tracking system deployed in a patient's home enabled prospective longitudinal quantification of the natural history and improvement in voluntary eye-movement impairments after pontine hemorrhage. It also characterized prospectively for the first time, the emergence of involuntary eye movements resulting from the rare complication of hypertrophic olivary degeneration. Results suggest that brief weekly measurements with an eye-tracker may allow early detection of this complication.
| S-EPMC5411421 | biostudies-literature
Project description:Prognosis of primary pontine hemorrhage (PPH) is important for treatment planning and patient management. However, only few clinical factors were reported to have prognostic value to PPH. Here, we propose a deep learning (DL) model that mines high-dimensional prognostic information from computed tomography (CT) images and combines clinical factors for predicting individualized prognosis of PPH. We proposed a multi-task DL model to learn high-dimensional CT features of hematoma and perihematomal areas for predicting the risk of 30-day mortality, 90-day mortality and 90-day functional outcome of PPH simultaneously. We further explored the combination of the DL model and clinical factors by building a combined model. All the models were trained in a training cohort (n = 219) and tested in an independent testing cohort (n = 35). The DL model achieved area under the curve (AUC) of 0.886, 0.886, and 0.759 in predicting 30-day mortality, 90-day mortality and 90-day functional outcome of PPH in the independent testing cohort, which improved over the previously reported new PPH score and the clinical model. When combining the DL model and clinical factors, the combined model achieved improved performance (AUC = 0.920, 0.941, and 0.894), indicating that DL model mines CT information that complements clinical factors. Through DL visualization technique, we found that the internal structure of hematoma and its expansion to perihematomal regions are important for predicting the prognosis of PPH. This DL model provides an easy-to-use way for predicting individualized prognosis of PPH by mining high-dimensional information from CT images, and showed improvement over clinical factors and present methods.
| S-EPMC9668666 | biostudies-literature
Project description: Not available
| S-EPMC7781080 | biostudies-literature
Project description:Background:Essential tremor (ET) is a common movement disorder characterized by kinetic and postural tremor in the upper extremities and frequently in the midline. Persons with ET often also exhibit gait ataxia. Previous studies have observed associations between midline tremor severity and gait ataxia in persons with ET, suggesting a common pathophysiology distinct from that of upper extremity tremor. However, a causal link between midline tremor and gait impairment has not been established. Methods:We investigated tremor and gait in 24 persons with ET before and after implantation of unilateral deep brain stimulation into the ventralis intermedius nucleus of the thalamus. Results:Stimulation significantly improved tremor in the targeted upper extremity and midline. However, gait was unaffected at the cohort level. Furthermore, improvement in midline tremor was not significantly associated with gait improvement. Discussion:These findings revealed that midline tremor and gait impairment may be dissociable in persons with ET.
| S-EPMC6744814 | biostudies-literature
Project description:It is difficult to determine the pathoanatomical correlates of dystonia because of its complex pathophysiology, and most cases with secondary dystonia are associated with basal ganglia lesions. Moreover, it is a challenging issue that patients with abnormal postures accompanied by other neurological findings in the affected body part (e.g., sensory loss) can be diagnosed with true dystonia or pseudodystonia. Here, we report a case of abnormal postures with loss of proprioception in the left extremities after right dorsal pontine hemorrhage.
| S-EPMC6987531 | biostudies-literature