Not seen on ct scan due to artefacts caused by bony structures, the hypertrophic olivary degeneration hod is more recently detected in vivo by mri and is characterized by an hypersignal of the olive of the medulla oblongata on pdt2 images with a variable enlargement of the olive itself. It occurs secondary to lesions which disrupt any tract within the dentatorubo olivary pathway which is made up of a triangle including the dentate nucleus, red nucleus and inferior olivary body. Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around 34 weeks following an injury to the guillain. Primary lesion of the dentatorubral olivary pathway may lead to secondary degeneration of the inferior olivary nucleus ion, resulting in a rare but clinically relevant condition called hypertrophic olivary degeneration hod. Imaging features of hypertrophic olivary degeneration.
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 occurs after focal lesions to the dentatorubro olivary pathway, typically following a pontine haemorrhage involving the ipsilateral central tegmental tract, the. Hypertrophic olivary degeneration hod is a rare abnormality that is caused by a lesion in the guillainmollaret triangle in the brainstem. Holmes tremor in association with bilateral hypertrophic. 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. Letters to editor hypertrophic olivary degeneration. Symptoms and treatment hypertrophic olivary degeneration.
Hypertrophic olivary degeneration hod is a rare type of neuronal degeneration involving the dentorubroolivary pathway and presents clinically as palatal tremor. Hypertrophic olivary degeneration inferior olivary nucleus hypertrophy. A lesion within the dentatorubro olivary pathway drop in the posterior fossa can cause secondary neurodegeneration of the inferior olivary nucleus. Hypertrophic olivary degeneration with an eggshaped. Not seen on ct scan due to artefacts caused by bony structures, the hypertrophic olivary degeneration hod is more recently detected in vivo by mri and is characterized by an hypersignal of the olive of.
This means the trait for the disease is found in the first twentytwo chromosomes of the. Jun 29, 2017 hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor pt or oculopalatal tremor opt. Figure 2 omitted hypertrophic olivary degeneration is a form of transsynaptic degeneration caused by an insult to the neuronal connections of the dentatorubroolivary pathway. 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. The clinical syndrome of hod occurs slowly over months and may be overlooked in progressive neurooncological diseases. Mri and tractography in hypertrophic olivary degeneration volume 45 issue 3 qilun lai, chaobo zheng, rui zhang, xiaoli liu, yaguo li, qi xu. Hod usually occurs uni and ipsilaterally to the lesion if the lesion is in the brainstem or contralaterally to the lesion if the lesion is in the cerebellum, as has been shown by mri. Hypertrophic olivary degeneration hod is a pathological phenomenon that occurs after injury to the dentato olivary pathway. Delayed occurrence of hypertrophic olivary degeneration. Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around. 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. The dentatorubro olivary pathway is a neural network involved in fine voluntary motor control and consists of the red nucleus, the ipsilateral inferior. 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. Injury to the dentatorubro olivary pathway causes hypertrophy and enlargement of the inferior olivary nuclei, which is called hypertrophic olivary degeneration hod.
Palatal myoclonus secondary to hypertrophic olivary. Mri and tractography in hypertrophic olivary degeneration. The forgotten triangle of guillain and mollaret sir, hypertrophic olivary degeneration hod, a form of transsynaptic degeneration, results from a lesion of the dentorubro. If your institution subscribes to this resource, and you dont have a myaccess profile, please contact your librarys reference desk for information on how to gain access to this resource from offcampus.
Pdf mri and neurological presentation of hypertrophic olivary. Hypertrophic olivary degeneration hod is a transneuronal degeneration secondary to focal lesions involving the dentatorubralolivary pathway, also know as guillainmollaret triangle. 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. Clinical and radiologic hallmarks of this condition are palatal myoclonus and t2 hyperintensity of the inferior olivary complex on magnetic resonance imaging mri, respectively. Hypertrophic olivary degeneration hod is a very rare transsynaptic degeneration that results from disruption of the guillainmollaret triangle gmt and affects the inferior olivary nucleus ion. Imagingwise hypertrophic olivary degeneration seen as unilateral or bilateral enlargement of ventro medial portion of medulla with t2 hyperintensity which corresponds to ion. Cerebelloolivary degeneration of holmes by autumn brown.
Bilateral hypertrophic olivary degeneration in wilson disease. Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor pt or oculopalatal tremor opt. This means the trait for the disease is found in the first twentytwo chromosomes of the autosomes. Bilateral hypertrophic olivary nucleus degeneration on. Hypertrophic olivary degeneration hod is a transneuronal degeneration of the inferior olivary nucleus in which patients develop hypertrophy of the inferior olivary nucleus following a primary lesion in the dentatorubroolivary pathway. Hod is a rare condition characterized by a unique pattern of transsynaptic degeneration caused by damage dentatorubro olivary pathway. 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 genetic and rare diseases. Hypertrophic olivary degeneration is a rare occurrence in which different pathological processes including enlargement and vacuolation of the neurons, demyelination of the white matter. Primary lesion of the dentatorubralolivary pathway may lead to secondary degeneration of the inferior olivary nucleus ion, resulting in a rare but clinically relevant condition called hypertrophic olivary. It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and.
Dec 31, 2019 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. 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. Imagingwise hypertrophic olivary degeneration seen as unilateral or bilateral enlargement of ventro medial portion of medulla with t2. Cerebelloolivary degeneration of holmes by autumn brown on prezi. Signs and symptoms include palatal tremors, lack of movement coordination ataxia, holmes tremor, vision problems, muscle weakness, and gait impairment, along with an mri. Hypertrophic olivary degeneration after cerebellar or. 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. Hypertrophic olivary degeneration secondary to pontine hemorrhage. 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. Hypertrophic olivary degeneration may occur after damage to the dentatorubroolivary pathway. Jul 01, 2009 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. Frontiers hypertrophic olivary degeneration and palatal or.
This is a rare case of hypertrophic olivary degeneration hod in a patient with previous history of pontine hemorrhagic infarct. This syndrome manifests as a synchronous tremor of the palate pt andor eyes opt that may also involve other muscles from the branchial arches. A lesion within the dentatorubroolivary pathway drop in the posterior fossa can cause secondary neurodegeneration of the inferior olivary. Bilateral hypertrophic olivary degeneration has been reported in a few metabolic, genetic, neurodegenerative and toxic disorders. In cns the degeneration of an anatomical structure. Posterior fossa tumors are often located near these.
Hypertrophic olivary degeneration genetic and rare. Hypertrophic olivary degeneration hod is a very rare transsynaptic degeneration that results from disruption of the guillainmollaret triangle gmt and affects the inferior olivary nucleus. It has also been narrowed down to a few blood types, including b. Bilateral hypertrophic olivary nucleus degeneration on magnetic. Hypertrophic olivary degeneration hod is a rare type of neuronal degeneration caused by damage to the dentatorubroolivary pathway or the triangle of guillainmollaret figures 4 and 5. To date, adult cases of hod have usually been reported, and there are only a few individual paediatric cases with limited radiological emphasis in the literature. Holmes disease is a rare autosomallyinherited disease. If your institution subscribes to this resource, and you dont have a myaccess profile, please contact your librarys reference desk for information on how to gain access to this resource. Peter bouz, rafeek oj woods, and kamal rm woods department of neurological surgery, loma linda university medical center, usa. Hypertrophic olivary degeneration a report of two cases.
Hod is a rare condition characterized by a unique pattern of transsynaptic. Its hallmarks include hypertrophy of the olive with increased t2 signal intensity on magnetic resonance imaging, and it often manifests with palatal tremor and oscillopsia clinically. The pathophysiological basis for hypertrophic olivary. Hypertrophic olivary degeneration is a rare type of transsynaptic degeneration. 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. Pdf hypertrophic olivary degeneration secondary to central. Hypertrophic olivary degeneration occurs after focal lesions to the dentatorubroolivary pathway, typically following a pontine haemorrhage involving the ipsilateral central tegmental tract, the. Hypertrophic olivary degeneration and holmes tremor. Delayed occurrence of hypertrophic olivary degeneration after.
Hypertrophic olivary degeneration with an eggshaped appearance. 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 hypertrophic olivary degeneration hod is a disease that can be a complication of brainstem or cerebellar hemorrhage or surgery including radiosurgery. Hypertrophic olivary degeneration hod is a rare form of transneuronal degeneration of the inferior olivary nucleus. Hypertrophic olivary degeneration hod is a rare condition characterized by a unique pattern of transsynaptic degeneration. It is caused by a lesion in the triangle of guillain and mollaret, resulting in hypertrophy of the inferior olivary nucleus. Patients with hod often present with palatal myoclonus, ataxia, tremor, dysarthria andor hemiparesis. Hypertrophic olivary degeneration after pontine hemorrhage.
Hypertrophic olivary degeneration is associated with lateonset neurological worsening after brainstem injury, but this has seldom been. 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 may occur after damage to the dentatorubro olivary pathway. It occurs secondary to lesions which disrupt any tract within the dentatoruboolivary pathway which is made up. We present a 48 year old male patient who developed holmes tremor and bilateral hod five months after brainstem hemorrhage. Hypertrophic olivary degeneration radiology reference. Hypertrophic olivary degeneration hod is a rare form of transsynaptic degeneration characterized by hypertrophy of the inferior olivary nucleus situated in the olivary body, part of the medulla oblongata.
There patterns of hod in relation to location of primary lesion. Hypertrophic olivary degeneration cerebrovascular disease. If it takes too long to load the home page, tap on the button below. 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.
Bilateral hypertrophic olivary degeneration in wilson disease discussion hypertrophic olivary degeneration represents a unique form of transneuronal transsynaptic degeneration in which the. Our radiology report database was queried for hod in all head mri reports between 712002 and 7120 and identified 8 patients. Hod usually occurs uni and ipsilaterally to the lesion if the lesion is. Mri findings in nonlesional hypertrophic olivary degeneration. Frontiers hypertrophic olivary degeneration and palatal. 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 hod is usually caused by a lesion in the triangle of guillain and mollaret and presents clinically as palatal tremor.
Hypertrophic olivary degeneration hod hypertrophic olivary degeneration is a rare finding secondary to focal lesions of the brain stem involving guillainmollaret triangle. Imaging features of hypertrophic olivary degeneration art. It mostly develops secondary to a destructive lesion involving the guillainmollaret pathway. Hypertrophic olivary degeneration hod is a rare phenomenon, probably related to transsynaptic degeneration of the inferior olivary nucleus. Investigate the relative frequency of nonlesional versus lesional hypertrophic olivary degeneration hod and potential explanations for nonlesional hod. Hypertrophic olivary degeneration hod is a rare type of neuronal degeneration involving the dentorubro olivary pathway and presents clinically as palatal tremor. Its hallmarks include hypertrophy of the olive with increased t2. Unusual clinical manifestation associated with hypertrophic. The hypertrophic olivary degeneration hod is one of such changes caused by the impairment of guillainmollaret triangle, the circuit connecting red nucleus, ipsilateral inferior olivary. Hypertrophic olivary degeneration neuroradiology case.
The present paper contribute to a better understanding of hod clinical characteristic, which will be helpful for neurologists and radiologists. 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. Sir, hypertrophic olivary degeneration hod, a form of transsynaptic degeneration, results from a lesion of the dentorubroolivary pathway also called the anatomical triangle of guillain and mollaret. A lesion within the dentatorubroolivary pathway drop in the posterior fossa can cause secondary neurodegeneration of the inferior olivary nucleus. Hypertrophic olivary degeneration hod is a rare form of transsynaptic degeneration characterized by hypertrophy of the inferior olivary nucleus situated in the olivary body, part of the medulla oblongata, representing a major source of input to the cerebellum. 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. It is caused by a lesion in the triangle of guillain and mollaret, resulting in.
Pdf hypertrophic olivary degeneration secondary to. Cerebellar mr changes in patients with olivary hypertrophic degeneration. Hypertrophic olivary degeneration hod is a pathological phenomenon that occurs after injury to the dentatoolivary pathway. 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. It usually occurs as a response to primary injury of dentorubro olivary pathways. 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. Hypertrophic olivary degeneration hod is a rare form of transsynaptic degeneration characterized by hypertrophy of the inferior olivary nucleus situated in the. It is caused by a lesion in the triangle of guillain and mollaret, resulting in hypertrophy of the inferior olivary nucl. Hypertrophic olivary degeneration hod occurs because of posterior fossa or brainstem lesions that disrupt the dentatorubro olivary tract, well known as the guillainmollaret triangle.