scholarly journals Holmes tremor in association with bilateral hypertrophic olivary degeneration and palatal tremor: chronological considerations. Case report

2003 ◽  
Vol 61 (2B) ◽  
pp. 473-477 ◽  
Author(s):  
Carlos R.M. Rieder ◽  
Ricardo Gurgel Rebouças ◽  
Marcelo Paglioli Ferreira
Keyword(s):  
Neuronal Degeneration ◽  
High Signal ◽  
Proton Density ◽  
Rare Type ◽  
Hypertrophic Olivary Degeneration ◽  
Palatal Tremor ◽  
Holmes Tremor ◽  
Nervous System Function ◽  
Uncommon Condition ◽  
Inferior Olivary

Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration involving the dento-rubro-olivary pathway and presents clinically as palatal tremor. We present a 48 year old male patient who developed Holmes' tremor and bilateral HOD five months after brainstem hemorrhage. The severe rest tremor was refractory to pharmacotherapy and botulinum toxin injections, but was markedly reduced after thalamotomy. Magnetic resonance imaging permitted visualization of HOD, which appeared as a characteristic high signal intensity in the inferior olivary nuclei on T2- and proton-density-weighted images. Enlargement of the inferior olivary nuclei was also noted. Palatal tremor was absent in that moment and appears about two months later. The delayed-onset between insult and tremor following structural lesions of the brain suggest that compensatory or secondary changes in nervous system function must contribute to tremor genesis. The literature and imaging findings of this uncommon condition are reviewed.

scholarly journals Bilateral Hypertrophic Olivary Degeneration and Holmes Tremor without Palatal Tremor: An Unusual Association

2016 ◽  
Vol 6 (0) ◽  
pp. 400 ◽  
Author(s):  
Carlos Cosentino ◽  
Miriam Velez ◽  
Yesenia Nuñez ◽  
Henry Palomino ◽  
Darko Quispe ◽  
...  
Keyword(s):  
Hypertrophic Olivary Degeneration ◽  
Palatal Tremor ◽  
Unusual Association ◽  
Holmes Tremor

scholarly journals Bilateral surgical damage of the central tegmental tract resulting in bilateral hypertrophic olivary degeneration: An MRI case report

2017 ◽  
Vol 31 (2) ◽  
pp. 182-185 ◽  
Author(s):  
Silvia Lana ◽  
Chiara Ganazzoli ◽  
Girolamo Crisi
Keyword(s):  
Cranial Nerve ◽  
Cranial Nerve Palsy ◽  
Nerve Palsy ◽  
Neuronal Degeneration ◽  
Inferior Olivary Nucleus ◽  
Hypertrophic Olivary Degeneration ◽  
Seventh Cranial Nerve ◽  
Central Tegmental Tract ◽  
Inferior Olivary ◽  
Olivary Nucleus

Hypertrophic olivary degeneration (HOD) is a rare trans-synaptic neuronal degeneration of the inferior olivary nucleus caused by an injury to the dentato-rubro-olivary connection, also known as Guillain-Mollaret triangle. It leads to hypertrophy of the affected nucleus rather than atrophy and is characterized by hyperintensity on T2-weighted images. Unilateral and bilateral cases are described. We present a case of a 70-year-old patient affected by a tumor inside the fourth ventricle who suffered from diplopia and right seventh cranial nerve palsy. He underwent surgery and developed left seventh cranial nerve palsy. Three months after resection, magnetic resonance imaging revealed the appearance of bilateral HOD. This is the first report of bilateral HOD occurrence after surgical bilateral damage of the rubro-olivary fibers running in central tegmental tracts.

scholarly journals Multicenter Prospective Analysis of Hypertrophic Olivary Degeneration Following Infratentorial Stroke (HOD-IS): Evaluation of Disease Epidemiology, Clinical Presentation, and MR-Imaging Aspects

2021 ◽  
Vol 12 ◽  
Author(s):  
Martin A. Schaller-Paule ◽  
Eike Steidl ◽  
Manoj Shrestha ◽  
Ralf Deichmann ◽  
Helmuth Steinmetz ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Clinical Symptoms ◽  
Inferior Olivary Nucleus ◽  
Index Event ◽  
Hypertrophic Olivary Degeneration ◽  
Palatal Tremor ◽  
Prophylactic Measures ◽  
Holmes Tremor ◽  
Dti Tractography ◽  
Tractography Data

Introduction: Ischemic and hemorrhagic strokes in the brainstem and cerebellum with injury to the functional loop of the Guillain-Mollaret triangle (GMT) can trigger a series of events that result in secondary trans-synaptic neurodegeneration of the inferior olivary nucleus. In an unknown percentage of patients, this leads to a condition called hypertrophic olivary degeneration (HOD). Characteristic clinical symptoms of HOD progress slowly over months and consist of a rhythmic palatal tremor, vertical pendular nystagmus, and Holmes tremor of the upper limbs. Diffusion Tensor Imaging (DTI) with tractography is a promising method to identify functional pathway lesions along the cerebello-thalamo-cortical connectivity and to generate a deeper understanding of the HOD pathophysiology. The incidence of HOD development following stroke and the timeline of clinical symptoms have not yet been determined in prospective studies—a prerequisite for the surveillance of patients at risk.Methods and Analysis: Patients with ischemic and hemorrhagic strokes in the brainstem and cerebellum with a topo-anatomical relation to the GMT are recruited within certified stroke units of the Interdisciplinary Neurovascular Network of the Rhine-Main. Matching lesions are identified using a predefined MRI template. Eligible patients are prospectively followed up and present at 4 and 8 months after the index event. During study visits, a clinical neurological examination and brain MRI, including high-resolution T2-, proton-density-weighted imaging, and DTI tractography, are performed. Fiberoptic endoscopic evaluation of swallowing is optional if palatal tremor is encountered.Study Outcomes: The primary endpoint of this prospective clinical multicenter study is to determine the frequency of radiological HOD development in patients with a posterior fossa stroke affecting the GMT at 8 months after the index event. Secondary endpoints are identification of (1) the timeline and relevance of clinical symptoms, (2) lesion localizations more prone to HOD occurrence, and (3) the best MR-imaging regimen for HOD identification. Additionally, (4) DTI tractography data are used to analyze individual pathway lesions. The aim is to contribute to the epidemiological and pathophysiological understanding of HOD and hereby facilitate future research on therapeutic and prophylactic measures.Clinical Trial Registration: HOD-IS is a registered trial at https://www.drks.de/drks_web/navigate.do?navigationId=trial.HTML&TRIAL_ID=DRKS00020549.

scholarly journals High‐Frequency Palatal Tremor and Stimulus‐Sensitive Leg Myoclonus with Degeneration of Inferior Olivary Nuclei in Celiac Disease

2020 ◽  
Vol 7 (S3) ◽  
Author(s):  
Deepak K. Gupta ◽  
Angel Viñuela ◽  
Peter H.R. Green ◽  
Michael J. Pitman ◽  
Jean P. Vonsattel ◽  
...  
Keyword(s):  
Celiac Disease ◽  
High Frequency ◽  
Palatal Tremor ◽  
Inferior Olivary

scholarly journals MRI Signal Abnormalities of the Inferior Olivary Nuclei in Spinocerebellar Ataxia Type 2

2017 ◽  
Vol 9 (3) ◽  
pp. 267-271 ◽  
Author(s):  
Fumihito Yoshii ◽  
Hitoshi Tomiyasu ◽  
Ryo Watanabe ◽  
Masafuchi Ryo
Keyword(s):  
Spinocerebellar Ataxia ◽  
Autosomal Dominant ◽  
Spinocerebellar Ataxia Type ◽  
Proton Density ◽  
Spinocerebellar Ataxias ◽  
Spinocerebellar Ataxia Type 2 ◽  
Cerebellar Peduncles ◽  
Magnetic Resonance Imaging Mri ◽  
Inferior Olivary

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant spinocerebellar degeneration, associated with extended repeats of the trinucleotide CAG in the ATXN2 gene on the long arm of chromosome 12. Magnetic resonance imaging (MRI) of SCA2 showed significant atrophies of the brainstem, middle cerebellar peduncles, and cerebellum. We report two genetically proven SCA2 patients who showed hypertrophy of the inferior olivary nuclei on proton density- and T2-weighted MRI. This pattern has never been reported in patients with SCA1, SCA3, or SCA6, and may make it possible to differentiate SCA2 from other hereditary spinocerebellar ataxias.

scholarly journals Neuro-Behçet: Pons Involvement with Longitudinal Extension to Midbrain and Hypertrophic Olivary Degeneration

2015 ◽  
Vol 7 (2) ◽  
pp. 148-151 ◽  
Author(s):  
Emilio Franco-Macías ◽  
Florinda Roldán-Lora ◽  
Paula Martínez-Agregado ◽  
Nuria Cerdá-Fuertes ◽  
Francisco Moniche
Keyword(s):  
Brain Mri ◽  
Inferior Olivary Nucleus ◽  
Intravenous Methylprednisolone ◽  
Hypertrophic Olivary Degeneration ◽  
Oral Ulcers ◽  
Pyramidal Syndrome ◽  
Extensive Lesion ◽  
Longitudinal Extension ◽  
Inferior Olivary ◽  
Olivary Nucleus

A 21-year-old right-handed man developed progressive dysarthria and gait disturbance over 4 months (associated with intermittent hiccups). During that time, he also suffered from uveitis. A physical examination showed pseudobulbar and pyramidal signs and genital and oral ulcers. A brain MRI revealed an extensive lesion mainly located in the ventral pons, with an extension upwards to the midbrain. The inferior olivary nucleus also showed hyperintensity. After the treatment with intravenous methylprednisolone and pulses of cyclophosphamide, he improved. As observed on his MRI, his lesions also improved, except for an increase of the inferior olivary nucleus, consistent with hypertrophic olivary degeneration. Neuro-Behçet tropism for ventral brainstem explains the usual presentation with pyramidal syndrome. Hypertrophic olivary degeneration due to pons involvement could explain the hiccup attacks in a few known cases.

scholarly journals Comparison of 3T and 7T MRI for the visualization of globus pallidus sub-segments

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Shuki Maruyama ◽  
Masaki Fukunaga ◽  
Hans-Peter Fautz ◽  
Robin Heidemann ◽  
Norihiro Sadato
Keyword(s):  
Globus Pallidus ◽  
Signal Intensity ◽  
Single Channel ◽  
Spin Echo ◽  
High Signal ◽  
Proton Density ◽  
Internal Globus Pallidus ◽  
Internal Structures ◽  
Electrode Localization ◽  
Straight Lines

AbstractThe success of deep brain stimulation (DBS) targeting the internal globus pallidus (GPi) depends on the accuracy of electrode localization inside the GPi. In this study, we sought to compare visualization of the medial medullary lamina (MML) and accessory medullary lamina (AML) between proton density-weighted (PDW) and T2-weighted (T2W) sequences on 3T and 7T MRI scanners. Eleven healthy participants (five men and six women; age, 19–28 years; mean, 21.5) and one 61-year-old man were scanned using two-dimensional turbo spin-echo PDW and T2W sequences on 3T and 7T MRI scanners with a 32-channel receiver head coil and a single-channel transmission coil. Profiles of signal intensity were obtained from the pixel values of straight lines over the GP regions crossing the MML and AML. Contrast ratios (CRs) for GPe/MML, GPie/MML, GPie/AML, and GPii/AML were calculated. Qualitatively, 7T visualized both the MML and AML, whereas 3T visualized the MML less clearly and hardly depicted the AML. The T2W sequence at 7T yielded significantly higher CRs for GPie/MML, GPie/AML, and GPii/AML than the PDW sequence at 7T or 3T. The T2W sequence at 7T allows visualization of the internal structures of GPi segments with high signal intensity and contrast.

Sign in / Sign up

Export Citation Format

Share Document