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 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 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 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.

Perioperative posterior reversible encephalopathy syndrome in 2 pediatric neurosurgery patients with brainstem ependymoma

2011 ◽  
Vol 7 (3) ◽  
pp. 235-237 ◽  
Author(s):  
Melanie G. Hayden Gephart ◽  
Bonnie P. Taft ◽  
Anne-Katrin Giese ◽  
Raphael Guzman ◽  
Michael S. B. Edwards
Keyword(s):  
Mr Imaging ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Clinical Symptoms ◽  
Pediatric Neurosurgery ◽  
Altered Mental Status ◽  
Hemodynamic Instability ◽  
Imaging Features ◽  
Posterior Reversible Encephalopathy ◽  
Reversible Encephalopathy ◽  
Pediatric Neurooncology

Posterior reversible encephalopathy syndrome (PRES) has been described in pediatric neurooncology patients, although it has not been documented perioperatively in pediatric neurosurgery patients not actively receiving chemotherapy. Recently at the authors' facility, 2 cases of PRES were diagnosed perioperatively in children with brainstem ependymoma. Both patients had presented with hypertension, altered mental status, and seizures and demonstrated MR imaging features consistent with PRES. The patients were treated with antiseizure and antihypertension medications, leading to improvement in both clinical symptoms and neuroimaging findings. These cases are the first to document PRES in perioperative pediatric neurosurgery patients not actively receiving chemotherapy. Both patients had ependymoma involving the brainstem, which may have led to intra- and perioperative hemodynamic instability (including hypertension) and predisposed them to this syndrome. An awareness of PRES in similar scenarios will aid in the prevention, diagnosis, and treatment of pediatric neurosurgery patients with this syndrome.

scholarly journals Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis

2019 ◽  
Vol 8 (12) ◽  
pp. 2222
Author(s):  
Martin A. Schaller-Paule ◽  
Christian Foerch ◽  
Sara Kluge ◽  
Peter Baumgarten ◽  
Jürgen Konczalla ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Mass Effect ◽  
Clinical Syndrome ◽  
Inferior Olivary Nucleus ◽  
Posterior Fossa Tumors ◽  
Hypertrophic Olivary Degeneration ◽  
Oncological Patients ◽  
Oncological Diseases ◽  
Therapeutic Measures ◽  
The University

(1) Background: A lesion within the dentato-rubro-olivary pathway (DROP) in the posterior fossa can cause secondary neurodegeneration of the inferior olivary nucleus: so-called hypertrophic olivary degeneration (HOD). The clinical syndrome of HOD occurs slowly over months and may be overlooked in progressive neuro-oncological diseases. Posterior fossa tumors are often located near these strategic structures. The goal of this study was to analyze the systematics of HOD occurrence in neuro-oncological patients. (2) Methods: The neuroradiological database of the university healthcare center was scanned for HOD-related terms from 2010 to 2019. After excluding patients with other causes of HOD, 12 datasets from neuro-oncological patients were analyzed under predetermined criteria. (3) Results: Patients received multimodal tumor treatments including neurosurgery, radiotherapy, and chemotherapy. HOD occurred both unilaterally (left n = 4; right n = 5) and bilaterally (n = 3). Though the mass effect of posterior fossa tumors had already affected strategic structures of the DROP, none of the patients showed signs of HOD on MRI until therapeutic measures including neurosurgery affecting the DROP were applied. HOD was visible on MRI within a median of 6 months after the neurosurgical intervention. In 67%, the presumed underlying surgical lesion in the DROP lay in the contralateral dentate nucleus. (4) Conclusion: In a selected cohort of neuro-oncological patients, therapeutic lesions within the DROP were associated with HOD occurrence.

Reversible tonsillar prolapse and syringomyelia after embolization of a tectal arteriovenous malformation

2007 ◽  
Vol 107 (2) ◽  
pp. 412-415 ◽  
Author(s):  
Georges Rodesch ◽  
Bernard Otto ◽  
Micheline Mouchamps ◽  
Jacques Born
Keyword(s):  
Magnetic Resonance ◽  
Mr Imaging ◽  
Arteriovenous Malformation ◽  
Chiari Malformation ◽  
Clinical Symptoms ◽  
Type I ◽  
Imaging Findings ◽  
Mr Image ◽  
Chiari Malformation Type I ◽  
Straight Sinus

✓ The authors report the case of a 21-year-old woman who presented with headaches, frequent sensations of loss of equilibrium, and intermittent strabismus. A tectal arteriovenous malformation (AVM) was diagnosed based on magnetic resonance (MR) imaging findings. The AVM drained toward the straight sinus and was associated with a tonsillar prolapse (Chiari malformation Type I [CM-I]) and cervical syringomyelia. The tectal AVM was embolized with N-butyl cyanoacrylate, and disconnection of about 80% of the lesion was obtained. All clinical symptoms resolved after embolization, and radiosurgery was proposed to treat the malformation remnant. A control MR image confirmed the regression of the tonsillar prolapse and the disappearance of the syrinx. This report emphasizes that CM-I and syringomyelia may be acquired and related to hydrovenous disorders.

Suprapineal recess: an alternate site for third ventriculostomy?

2004 ◽  
Vol 101 (3) ◽  
pp. 518-520 ◽  
Author(s):  
Roy Thomas Daniel ◽  
Gabriel Yin Foo Lee ◽  
Peter Lawrence Reilly
Keyword(s):  
Mr Imaging ◽  
Clinical Symptoms ◽  
Aqueductal Stenosis ◽  
Third Ventriculostomy ◽  
Content Type ◽  
Cerebrospinal Fluid Flow ◽  
Shunt Dysfunction ◽  
Symptoms And Signs ◽  
Endoscopic Ventriculostomy ◽  
Triventricular Hydrocephalus

✓ This 30-year-old woman presented with clinical symptoms and signs of intracranial hypertension and Parinaud syndrome secondary to ventriculoperitoneal shunt dysfunction. Magnetic resonance (MR) imaging revealed gross triventricular hydrocephalus with a large suprapineal recess due to aqueductal stenosis. Using an endoscopic approach, a ventriculostomy was performed within the floor of the dilated suprapineal recess. Following this procedure the patient experienced alleviation of all her neurological symptoms and signs. Postoperative MR imaging and cerebrospinal fluid flow studies demonstrated a functioning ventriculostomy. The anatomy of the suprapineal recess and its suitability for endoscopic ventriculostomy are discussed.

Sign in / Sign up

Export Citation Format

Share Document