The clinical syndrome of hod occurs slowly over months and may be overlooked in progressive neurooncological diseases. In vertebrates, the ion is known to coordinate signals from the spinal cord to the cerebellum to regulate motor coordination and learning. Hod is considered unique because the degenerating olive initially becomes hypertrophic rather than atrophic. Cerebelloolivary degeneration of holmes by autumn brown on prezi. Although the imaging features have been well described, the temporal course of hypertrophy and t2 signal increase in the inferior olivary nucleus ion has not been fully. The guillainmollaret triangle is a dentatorubroolivary pathway connecting the main inferior olivary nucleus, the contralateral dentate nucleus, and the ipsilateral red nucleus habas et al. Hypertrophic olivary degeneration hod is a rare transsynaptic form of degeneration occurring secondary to the disruption of the dentatorubro olivary pathway guillainmollaret triangle. Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around 34 weeks following an injury to the guillainmollaret triangle. Hypertrophic olivary degeneration hod is seen following lesions in the guillainmollaret triangle. Palatal myoclonus secondary to hypertrophic olivary. We performed a search of a radiologic report database for patients who were radiologically diagnosed as having hod.
Imagingwise hypertrophic olivary degeneration seen as unilateral or bilateral enlargement of ventro medial portion of medulla with t2 hyperintensity which corresponds to ion. Imaging features of hypertrophic olivary degeneration. The tract from the inferior olive to the dentate nucleus crosses the inferior cerebellar peduncle. Guillainmollaret triangle, hypertrophic olivary degeneration, magnetic resonance imaging introduction hypertrophic olivary degeneration hod is a rare occurrence in which different pathological processes including enlargement and vacuolation of the neurons, demyelination of the white matter, and fibrillary gliosis of the inferior olivary nucleus take place. This database includes the patients examined at the mayo clinic in florida and arizona. The pathophysiological basis for hypertrophic olivary.
The inferior olivary nucleus ion, is a structure found in the medulla oblongata underneath the superior olivary nucleus. Hypertrophic olivary degeneration symptoms, treatments. Bilateral hypertrophic olivary degeneration is more common than unilateral involvement. Although the imaging features have been well described, the temporal course of hypertrophy and t2 signal increase in the inferior olivary nucleus ion has not been fully characterized. These connections have been shown to be tightly associated, as degeneration of either the cerebellum or the ion results in. Hypertrophic olivary degeneration secondary to a guillain. Hypertrophic olivary degeneration hod is a rare condition characterized by a unique pattern of transsynaptic degeneration. Figure 4 t2weighted mri was done 2 years after pica stroke showing hypertrophic olivary degeneration hod of the right inferior olivary nucleus ion. Hod occurs when a lesion, usually a haemorrhage, causes an interruption of the guillainmollaret triangle fig1. Majority of idiopathic nonlesional hypertrophic olivary degeneration is bilateral.
Pdf hypertrophic olivary degeneration secondary to neuro. Posterior fossa tumors are often located near these. The forgotten triangle of guillain and mollaret sir, hypertrophic olivary degeneration hod, a form of transsynaptic degeneration, results from a lesion of the dentorubro olivary pathway also called the anatomical triangle of guillain and mollaret. A lesion within the dentatorubro olivary pathway drop in the posterior fossa can cause secondary neurodegeneration of the inferior olivary nucleus. Hypertrophic olivary degeneration hod is a rare neurological condition caused by degeneration in the brain stem, the structure that connects the brain to the spinal cord. Hypertrophic olivary degeneration hod is the result of an insult to the dentatorubro olivary pathway fig. After an insult infarct, hemorrhage, trauma, tumor, surgery that disrupts the gmt, hypertrophic degeneration of the affected inferior olivary nucleus ion develops. Holmes disease is a rare autosomallyinherited disease. T2 hyperintensity and enlargement of the inferior olivary nucleus ion are the radiological hallmarks of this entity. Hypertrophic olivary degeneration hod is a rare transsynaptic neuronal degeneration affecting the dentato. Frontiers hypertrophic olivary degeneration and palatal. The dentatorubro olivary pathway is a neural network involved in fine voluntary motor control and consists of the red nucleus, the ipsilateral inferior. Lesions involving this circuit may produce palatal myoclonus, one of the few involuntary movements that do not. Hypertrophic olivary degeneration a report of two cases.
Hypertrophic olivary degeneration radiology reference. Figure 2 omitted hypertrophic olivary degeneration is a form of transsynaptic degeneration caused by an insult to the neuronal connections of the dentatorubro olivary pathway i. Hypertrophic olivary degeneration hod was first reported in 1887 by oppenheim. Jul 16, 2014 in this patient with a history of resected pilocytic astrocytoma of the midbrain and uvulopalatal tremor, a stable lesion is present in the right meduallary olive remote from the surgical site. We used the search terms hypertrophic olivary degeneration, hod, and olivary in the reports recorded from 1995 to 2015. Mri findings in nonlesional hypertrophic olivary degeneration. Symptoms and treatment hypertrophic olivary degeneration. Jan 27, 2017 hypertrophic olivary degeneration hod is a rare neurological condition caused by degeneration in the brain stem, the structure that connects the brain to the spinal cord. Bilateral hypertrophic olivary degeneration in wilson disease. Hypertrophic olivary degeneration is a rare and unique type of neuronal degeneration mostly due to infarction, infection, demyelination and postsurgery and trauma. This means the trait for the disease is found in the first twentytwo chromosomes of the autosomes. The hypertrophy, expected location, and time course led us to diagnose hypertrophic olivary degeneration from dentatorubro olivary circuit mollaret triangle interruption. Failure to recognize a medullary lesion as hypertrophic olivary degeneration from a prior insult along the. Information on how to subscribe to neurology and neurology.
Hypertrophic olivary degeneration hod is a rare form of transneuronal degeneration of the inferior olivary nucleus. As in vivo diagnosis of this condition has only become possible with the advent of mri, the number of reported cases remains relatively small and they are almost exclusively in adults. This anatomical pathway was described by guillain and mollaret 1 in 1931. It is caused by a lesion in the triangle of guillain and mollaret, resulting in hypertrophy of the inferior olivary nucleus. Hod is caused by lesions in the dentatorubral or central tegmental tracts. Hypertrophic olivary degeneration hod is a rare abnormality that is caused by a lesion in the guillainmollaret triangle in the brainstem. Klinisches bildhypertrophic olivary degenerationclinical correlates. Hypertrophic olivary degeneration resulting from lesions of the dentorubro olivary pathway, also called guillainmollarettriangle, has been described previously in a number of cases. Hod is a rare condition characterized by a unique pattern of transsynaptic degeneration caused by damage dentatorubro olivary pathway. The guillainmollaret triangle is a triangular circuit connecting the dentate nucleus of the cerebellum on one side with the red nucleus and the inferior olivary nucleus on the other side, via the. Inferior olivary hypertrophy is uncommon in progressive. Hypertrophic olivary degeneration after pontine hemorrhage.
Konno t, broderick df, tacik p, caviness jn, wszolek zk. University of massachusetts medical school faculty publications. Images in hypertrophic olivary degeneration secondary to. Palatal tremor and hypertrophic olivary degeneration as. In some case series, half of the patients with hod have developed it as a result of a brainstem cavernous angioma hemorrhage or surgery. A rare presentation of hypertrophic olivary degeneration secondary to primary central nervous system lymphoma. Ioh is usually associated with the interruption of the dentato olivary tract, most often from cerebrovascular lesions in the dentate nucleus or red nucleus.
Open access case report case of bilateral hypertrophic. Hypertrophic olivary degeneration hod is a rare form of neuronal degeneration that occurs secondary to injuries that disrupt normal function of the afferent fibers to the inferior olivary nucleus ion as part of the dentaterubroolivary pathways triangle of guillainmollaret. Hod is considered a transsynaptic degeneration because it occurs following loss of neuronal input to a cell, in this case the neurons of the inferior olivary nucleus. A 59yearold man developed the acute onset of diplopia, dizziness, left hemiparesis, and slurred speech. We present a 48 year old male patient who developed holmes tremor and bilateral hod five months after brainstem hemorrhage. A lesion within the functional brainstem loop of the dentatorubro olivary pathway drop leads to secondary transsynaptic neurodegeneration of the inferior olivary nucleus, a condition called hypertrophic olivary degeneration hod 1,2,3,4,5. Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor pt or oculopalatal tremor opt. This is a rare case of hypertrophic olivary degeneration hod in a patient with previous history of pontine hemorrhagic infarct. Hypertrophic olivary degeneration hod is a rare type of neuronal degeneration involving the dentorubro olivary pathway and presents clinically as palatal tremor. Sir, hypertrophic olivary degeneration hod, a form of transsynaptic degeneration, results from a lesion of the dentorubro olivary pathway also called the anatomical triangle of guillain and mollaret. Cerebellar mr changes in patients with olivary hypertrophic degeneration. Hypertrophic olivary degeneration mimics relapse in. In cns the degeneration of an anatomical structure. Hod is a unique form of transneuronal degeneration in which the inferior olivary nucleus undergoes hypertrophy followed by atrophy, as a consequence of lesions to the dentorubro olivary pathway.
Hypertrophic olivary degeneration occurs in lesions involving the dentatorubro olivary system, an important feedback circuit responsible for modulating spinal cord motor activity. Hypertrophic olivary degeneration hod is a dynamic process caused by disruptive lesions affecting components of the guillainmollaret. Mri images showed an incidental finding of left hypertrophic olivary degeneration figure 1 and figure 2. Discussion hod is a unique form of degeneration because it results in enlargement of the affected structure rather than atrophy 4,5. Mri findings in nonlesional hypertrophic olivary degeneration chris n. Hypertrophic olivary degeneration hod is a transneuronal degeneration secondary to focal lesions involving the dentatorubral olivary pathway, also know as guillainmollaret triangle. Hypertrophic olivary degeneration after cerebellar or brain. Idiopathic bilateral hypertrophic olivary degeneration. This is unique because the inferior olivary nucleus hypertrophies following degeneration unlike the typical atrophy seen in other structures. Holmes tremor in association with bilateral hypertrophic.
Hypertrophic olivary degeneration genetic and rare. Injury to the drop causes hypertrophy and enlargement of the inferior olivary nuclei ion, in contrast to the atrophy usually observed in other parts of the central nervous system cns 1. Signs and symptoms include palatal tremors, lack of movement coordination ataxia, holmes tremor, vision problems, muscle weakness, and gait impairment, along with an mri showing enlargement hypertrophy of the inferior olivary nucleus. Korean j radiol 142, marapr 20 317 bilateral hypertrophic olivary degeneration in wilson disease weighted images swis was revealed in the formatio reticularis, globus pallidus, putamen, and caudate nucleus. Hypertrophic olivary degeneration is a rare condition characterized by a unique pattern of transsynaptic degeneration. To date, the only known disorder which specifically targets the ion is an extremely rare form of degeneration called hypertrophic olivary degeneration hod. Bilateral hypertrophic olivary degeneration in wilson disease discussion hypertrophic olivary degeneration represents a unique form of transneuronal transsynaptic degeneration in which the inferior olivary nucleus undergoes an initial hypertrophic alteration rather than atrophy, which often occurs several years later 1, 2, 57. Inferior olivary nucleus an overview sciencedirect topics.
Letters to editor hypertrophic olivary degeneration. Hypertrophic olivary degeneration and holmes tremor. Hypertrophic olivary degeneration genetic and rare diseases. Jan 30, 2015 hypertrophic olivary degeneration hod is seen following lesions in the guillainmollaret triangle. A lesion within the dentatorubroolivary pathway drop in the posterior fossa can cause secondary neurodegeneration of the inferior olivary. In this case, the previous hemorrhagic lesion in the right dentate nucleus caused the interruption that led to hypertrophic olivary. The present paper contribute to a better understanding of hod clinical characteristic, which will be helpful for neurologists and radiologists. Delayed occurrence of hypertrophic olivary degeneration after. Hypertrophic olivary degeneration secondary to pontine. Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around 34 weeks following an injury to the guillain. Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around 34 weeks following an injury to the guillainmollaret. Bilateral hypertrophic olivary nucleus degeneration on. The degeneration is unique in that it is associated, at least for a period of time, with the hypertrophy rather than atrophy of inferior olivary nucleus. The degeneration is unique in that it is associated.
It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and. Signs and symptoms include palatal tremors, lack of movement coordination ataxia, holmes tremor, vision problems, muscle weakness, and gait impairment, along with an mri showing enlargement. Hypertrophic olivary degeneration hod is usually caused by a lesion in the triangle of guillain and mollaret and presents clinically as palatal tremor. Jul 25, 2016 hypertrophic olivary degeneration hod is a rare type of neuronal degeneration caused by damage to the dentatorubro olivary pathway or the triangle of guillainmollaret figures 4 and 5.
Hypertrophic olivary degeneration cerebrovascular disease. Hypertrophic olivary degeneration is a rare form of neuronal degeneration that results from disruption of the afferent fibres to the inferior olive within the dentatorubroolivary tract, otherwise known as the triangle of guillainmollaret. Characteristic hypertrophic changes are because of transsynaptic degeneration, because of. Mri and neurological presentation of hypertrophic olivary. Hypertrophic olivary degeneration hod is a rare type of neuronal degeneration caused by damage to the dentatorubro olivary pathway or the triangle of guillainmollaret figures 4 and 5. Hypertrophic olivary degeneration hod hypertrophic olivary degeneration hod is a disease that can be a complication of brainstem or cerebellar hemorrhage or surgery including radiosurgery. Injury to the dentatorubroolivary pathway causes hypertrophy and enlargement of the inferior olivary nuclei, which is called hypertrophic olivary degeneration. We report a case of hypertrophic olivary degeneration hod detected by mri, in a 14yearold girl, months after surgical excision of a brainstem cavernous malformation. May 15, 2004 inferior olivary hypertrophy ioh is an uncommon disorder attributed to transsynaptic degeneration and characterized clinically by palatal myoclonus and histopathologically by swollen, fenestrated neurons and bizarre astrocytic gliosis. Although the imaging features have been well described, the temporal course of hypertrophy and t2 signal increase in the inferior olivary nucleus ion has not. This syndrome manifests as a synchronous tremor of the palate pt andor eyes opt that may also involve other muscles from the branchial arches. Hypertrophic olivary degeneration hod is a rare phenomenon, probably related to transsynaptic degeneration of the inferior olivary nucleus. Hypertrophic olivary degeneration hod hypertrophic olivary degeneration is a rare finding secondary to focal lesions of the brain stem involving guillainmollaret triangle. Lesions of the triangle of guillain and mollaret the dentatorubro olivary pathway may lead to hod, a secondary transsynaptic degeneration of the inferior olivary nucleus.
Even if the inferior olives are not the source of 2hz palatal tremor, they are undergoing a progressive hypertrophic degeneration with severe neuronal loss. Palatal tremor and hypertrophic olivary degeneration as sequelae of basilar artery occlusion. Haemorrhagic lesion within the left superior cerebellar peduncle causes secondary degeneration of the contralateral corticospinal tracts, which causes this unique. Hypertrophic olivary degeneration hod is a unique form of transneuronal degeneration caused by a disruption of the dentatorubro olivary pathway, also known as the triangle of guillainmollaret.
Even if the inferior olives are not the source of 2hz palatal tremor, they are undergoing a progressive hypertrophic degeneration with severe neuronal loss, which has not affected our patients hand tremor. Frontiers hypertrophic olivary degeneration and palatal or. Hypertrophic olivary degeneration in a child following midbrain. It has also been narrowed down to a few blood types, including b. Hypertrophic olivary degeneration hod is a rare entity that develops after an injury to the dentatorubroolivary pathway drop also called the guillainmollaret triangle, or gmt. There patterns of hod in relation to location of primary lesion. Lesions of the superior cerebellar peduncle can also result in contralateral hod, whereas primary lesions of the central tegmental tract cause ipsilateral hod. New mr imaging observations regarding hypertrophic olivary degeneration 25 march 2017 clinical anatomy, vol.
A rare presentation of hypertrophic olivary degeneration. Unusual clinical manifestation associated with hypertrophic. Hypertrophic olivary degeneration is a very rare form of transsynaptic degeneration that occurs over a long period of time when a lesion interrupts the triangle of guillainmollaret. Magnetic resonance imaging of the brain showed an area of increased signal on diffusionweighted images of the dorsal pontomesencephalic junction on the right side. Hypertrophic olivary degeneration is a rare form of neuronal degeneration that results from disruption of the afferent fibres to the inferior olive within the dentatorubro olivary tract, otherwise known as the triangle of guillainmollaret. Hypertrophic olivary degeneration does not reduce essential. Hypertrophic olivary degeneration is a rare occurrence in which different pathological processes including enlargement and vacuolation of the neurons, demyelination of the white matter, and fibrillary gliosis of the inferior olivary nucleus take place. Early mri shows t2 lengthening in the dentate nucleus, superior cerebellar peduncle. This is a functional pathway composed by neural connections between. Patients with hod often present with palatal myoclonus, ataxia, tremor, dysarthria andor hemiparesis. Hypertrophic olivary degeneration hod is an atypical form of transsynaptic degeneration representing the final result of an alteration in the neuronal connection of the dentatorubral olivary pathway. Mayank goyal, eric versnick, paul tuite, jean saint cyr, walter kucharczyk, waltrer montanera, robert willinsky, and david mikulis background and purpose.
Pdf hypertrophic olivary degeneration and holmes tremor. There was atrophy evident in the midbrain, vermis, and cerebellar hemispheres. Hypertrophic olivary degeneration is considered a transsynaptic degeneration that results from the disruption of the guillainmollaret triangle. Jun 29, 2017 hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor pt or oculopalatal tremor opt. In this form of transneuronal degeneration there is enlargement rather than atrophy of the affected structure.
This would have triggered a process of transynaptic hypertrophic degeneration of the nucleus ending in the generation of an abnormal oscillatory activity eventually. It usually occurs as a response to primary injury of dentorubro olivary pathways. We report two cases of hod in two different clinical scenarios. Hypertrophic olivary degeneration hod is a disease that can be a complication of brainstem or cerebellar hemorrhage or surgery including radiosurgery.
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