Hypertrophic olivary degeneration hod is a rare phenomenon in the dentorubro olivary pathway that was first described by oppenheim in 1887 1, 2. Information on how to subscribe to neurology and neurology. Haemorrhagic lesion within the left superior cerebellar peduncle causes secondary degeneration of the contralateral corticospinal tracts, which causes this unique. In vertebrates, the ion is known to coordinate signals from the spinal cord to the cerebellum to regulate motor coordination and learning. Even if the inferior olives are not the source of 2hz palatal tremor, they are undergoing a progressive hypertrophic degeneration with severe neuronal loss. The degeneration is unique in that it is associated. Letters to editor hypertrophic olivary degeneration. In this case, the previous hemorrhagic lesion in the right dentate nucleus caused the interruption that led to hypertrophic olivary. There patterns of hod in relation to location of primary lesion. We used the search terms hypertrophic olivary degeneration, hod, and olivary in the reports recorded from 1995 to 2015. Hypertrophic olivary degeneration secondary to pontine.
Images in hypertrophic olivary degeneration secondary to. 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. 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. Frontiers hypertrophic olivary degeneration and palatal. Mri findings in nonlesional hypertrophic olivary degeneration chris n.
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. Hypertrophic olivary degeneration a report of two cases. Hypertrophic olivary degeneration cerebrovascular disease. Imaging features of hypertrophic olivary degeneration. We present a 48 year old male patient who developed holmes tremor and bilateral hod five months after brainstem hemorrhage. Hypertrophic olivary degeneration does not reduce essential. It is caused by a lesion in the triangle of guillain and mollaret, resulting in hypertrophy of the inferior olivary nucleus. To date, the only known disorder which specifically targets the ion is an extremely rare form of degeneration called hypertrophic olivary degeneration hod. Hypertrophic olivary degeneration and holmes tremor. Klinisches bildhypertrophic olivary degenerationclinical correlates. 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. This syndrome manifests as a synchronous tremor of the palate pt andor eyes opt that may also involve other muscles from the branchial arches.
It usually occurs as a response to primary injury of dentorubro olivary pathways. Hypertrophic olivary degeneration mimics relapse in. It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and. Hypertrophic olivary degeneration symptoms, treatments.
Mri images showed an incidental finding of left hypertrophic olivary degeneration figure 1 and figure 2. Hypertrophic olivary degeneration after pontine hemorrhage. Hypertrophic olivary degeneration in a child following midbrain. This means the trait for the disease is found in the first twentytwo chromosomes of the autosomes. Hod is caused by lesions in the dentatorubral or central tegmental tracts.
There is little known about damage to the inferior olivary nucleus io independent from the cerebellum. A lesion within the dentatorubroolivary pathway drop in the posterior fossa can cause secondary neurodegeneration of the inferior olivary. Characteristic hypertrophic changes are because of transsynaptic degeneration, because of. Posterior fossa tumors are often located near these. 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. Imagingwise hypertrophic olivary degeneration seen as unilateral or bilateral enlargement of ventro medial portion of medulla with t2 hyperintensity which corresponds to ion. 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. In cns the degeneration of an anatomical structure. It has also been narrowed down to a few blood types, including b. Ioh is usually associated with the interruption of the dentato olivary tract, most often from cerebrovascular lesions in the dentate nucleus or red nucleus. Cerebelloolivary degeneration of holmes by autumn brown on prezi. New mr imaging observations regarding hypertrophic olivary degeneration 25 march 2017 clinical anatomy, vol.
There was atrophy evident in the midbrain, vermis, and cerebellar hemispheres. The dentatorubro olivary pathway is a neural network involved in fine voluntary motor control and consists of the red nucleus, the ipsilateral inferior. Discussion hod is a unique form of degeneration because it results in enlargement of the affected structure rather than atrophy 4,5. 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. This database includes the patients examined at the mayo clinic in florida and arizona. The inferior olivary nucleus ion, is a structure found in the medulla oblongata underneath the superior olivary nucleus.
Early mri shows t2 lengthening in the dentate nucleus, superior cerebellar peduncle. 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. Palatal tremor and hypertrophic olivary degeneration as. Inferior olivary hypertrophy is uncommon in progressive. 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.
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. The hypertrophy, expected location, and time course led us to diagnose hypertrophic olivary degeneration from dentatorubro olivary circuit mollaret triangle interruption. A 59yearold man developed the acute onset of diplopia, dizziness, left hemiparesis, and slurred speech. 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. Hypertrophic olivary degeneration hod is usually caused by a lesion in the triangle of guillain and mollaret and presents clinically as palatal tremor.
In this form of transneuronal degeneration there is enlargement rather than atrophy of the affected structure. Hypertrophic olivary degeneration is a rare condition characterized by a unique pattern of transsynaptic degeneration. 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. Hod is considered unique because the degenerating olive initially becomes hypertrophic rather than atrophic. Hypertrophic olivary degeneration hod is a rare transsynaptic form of degeneration occurring secondary to the disruption of the dentatorubro olivary pathway guillainmollaret triangle. Konno t, broderick df, tacik p, caviness jn, wszolek zk. Hod occurs when a lesion, usually a haemorrhage, causes an interruption of the guillainmollaret triangle fig1. Hypertrophic olivary degeneration hod is a rare abnormality that is caused by a lesion in the guillainmollaret triangle in the brainstem. 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. Hypertrophic olivary degeneration after cerebellar or brain. Bilateral hypertrophic olivary degeneration in wilson disease.
Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around 34 weeks following an injury to the guillainmollaret. 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. A lesion within the dentatorubro olivary pathway drop in the posterior fossa can cause secondary neurodegeneration of the inferior olivary nucleus. University of massachusetts medical school faculty publications. These connections have been shown to be tightly associated, as degeneration of either the cerebellum or the ion results in.
The clinical syndrome of hod occurs slowly over months and may be overlooked in progressive neurooncological diseases. 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 genetic and rare. The tract from the inferior olive to the dentate nucleus crosses the inferior cerebellar peduncle. Hypertrophic olivary degeneration hod is a rare condition characterized by a unique pattern of transsynaptic degeneration. Open access case report case of bilateral hypertrophic. Hypertrophic olivary degeneration hod is a rare type of neuronal degeneration involving the dentorubro olivary pathway and presents clinically as palatal tremor. Lesions involving this circuit may produce palatal myoclonus, one of the few involuntary movements that do not. Inferior olivary nucleus an overview sciencedirect topics.
Frontiers hypertrophic olivary degeneration and palatal or. 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. Hod is a rare condition characterized by a unique pattern of transsynaptic degeneration caused by damage dentatorubro olivary pathway. Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around 34 weeks following an injury to the guillain. After an insult infarct, hemorrhage, trauma, tumor, surgery that disrupts the gmt, hypertrophic degeneration of the affected inferior olivary nucleus ion develops. Jun 29, 2017 hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor pt or oculopalatal tremor opt. This is unique because the inferior olivary nucleus hypertrophies following degeneration unlike the typical atrophy seen in other structures. 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. Jan 30, 2015 hypertrophic olivary degeneration hod is seen following lesions in the guillainmollaret triangle. 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.
Palatal tremor and hypertrophic olivary degeneration as sequelae of basilar artery occlusion. 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. Hypertrophic olivary degeneration hod is seen following lesions in the guillainmollaret triangle. Bilateral hypertrophic olivary degeneration is more common than unilateral involvement. T2 hyperintensity and enlargement of the inferior olivary nucleus ion are the radiological hallmarks of this entity. Magnetic resonance imaging of the brain showed an area of increased signal on diffusionweighted images of the dorsal pontomesencephalic junction on the right side. 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. 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 transneuronal degeneration secondary to focal lesions involving the dentatorubral olivary pathway, also know as guillainmollaret triangle. A rare presentation of hypertrophic olivary degeneration.
Hypertrophic olivary degeneration radiology reference. The present paper contribute to a better understanding of hod clinical characteristic, which will be helpful for neurologists and radiologists. Hypertrophic olivary degeneration genetic and rare diseases. Cerebellar mr changes in patients with olivary hypertrophic degeneration. Symptoms and treatment hypertrophic olivary degeneration. 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. Mayank goyal, eric versnick, paul tuite, jean saint cyr, walter kucharczyk, waltrer montanera, robert willinsky, and david mikulis background and purpose. Hypertrophic olivary degeneration secondary to a guillain.
Unusual clinical manifestation associated with hypertrophic. Idiopathic bilateral hypertrophic olivary degeneration. In some case series, half of the patients with hod have developed it as a result of a brainstem cavernous angioma hemorrhage or surgery. 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. Palatal myoclonus secondary to hypertrophic olivary. 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. Hypertrophic olivary degeneration hod is a rare transsynaptic neuronal degeneration affecting the dentato. Hypertrophic olivary degeneration hod is the result of an insult to the dentatorubro olivary pathway fig. A rare presentation of hypertrophic olivary degeneration secondary to primary central nervous system lymphoma. Hypertrophic olivary degeneration is considered a transsynaptic degeneration that results from the disruption of the guillainmollaret triangle. 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. 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.
Pdf hypertrophic olivary degeneration secondary to neuro. 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. Majority of idiopathic nonlesional hypertrophic olivary degeneration is bilateral. 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.
Hypertrophic olivary degeneration hod is a rare form of transneuronal degeneration of the inferior olivary nucleus. Injury to the dentatorubroolivary pathway causes hypertrophy and enlargement of the inferior olivary nuclei, which is called hypertrophic olivary degeneration. Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor pt or oculopalatal tremor opt. Bilateral hypertrophic olivary nucleus degeneration on. 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. 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. Patients with hod often present with palatal myoclonus, ataxia, tremor, dysarthria andor hemiparesis. Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around 34 weeks following an injury to the guillainmollaret triangle. Mri findings in nonlesional hypertrophic olivary degeneration.
Hypertrophic olivary degeneration is a rare and unique type of neuronal degeneration mostly due to infarction, infection, demyelination and postsurgery and trauma. Delayed occurrence of hypertrophic olivary degeneration. Hypertrophic olivary degeneration hod is a disease that can be a complication of brainstem or cerebellar hemorrhage or surgery including radiosurgery. Lesions of the superior cerebellar peduncle can also result in contralateral hod, whereas primary lesions of the central tegmental tract cause ipsilateral hod. Holmes disease is a rare autosomallyinherited disease. Hypertrophic olivary degeneration hod hypertrophic olivary degeneration is a rare finding secondary to focal lesions of the brain stem involving guillainmollaret triangle.
This is a functional pathway composed by neural connections between. Oculomandibular tremor and bilateral hypertrophic olivary. This is a rare case of hypertrophic olivary degeneration hod in a patient with previous history of pontine hemorrhagic infarct. 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. Figure 4 t2weighted mri was done 2 years after pica stroke showing hypertrophic olivary degeneration hod of the right inferior olivary nucleus ion.
Pdf hypertrophic olivary degeneration and holmes tremor. Holmes tremor in association with bilateral hypertrophic. 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. Hypertrophic olivary degeneration resulting from lesions of the dentorubro olivary pathway, also called guillainmollarettriangle, has been described previously in a number of cases. The pathophysiological basis for hypertrophic olivary. 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. The drop, or socalled guillainmollaret triangle gmt, consists of three anatomic structures. 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. Delayed occurrence of hypertrophic olivary degeneration after. 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. We performed a search of a radiologic report database for patients who were radiologically diagnosed as having hod. We report two cases of hod in two different clinical scenarios. Failure to recognize a medullary lesion as hypertrophic olivary degeneration from a prior insult along the. 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.
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