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. Hypertrophic olivary degeneration and palatal or oculopalatal tremor. Axial, t2weighted ffe image at the level of the bulb a and axial t2weighted image b shows hypersignal in the region of the left inferior olivary nucleus, as well as accentuation of. 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 dentaterubro olivary pathways triangle of guillainmollaret. Evidencebased studies linked those diagnosed to one family line. 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 is considered a transsynaptic degeneration that results from the disruption of the guillainmollaret triangle. Hypertrophic olivary degeneration genetic and rare. We present a striking example of hypertrophic olivary degeneration after cerebellar hemorrhage. Hypertrophic olivary degeneration with an eggshaped. This means the trait for the disease is found in the first twentytwo chromosomes of the autosomes.
Hypertrophic olivary degeneration a report of two cases. 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 and palatal or oculopalatal tremor article pdf available in frontiers in neurology 8 june 2017 with 401 reads how we measure reads. It mostly develops secondary to a destructive lesion involving the guillainmollaret pathway.
Imagingwise hypertrophic olivary degeneration seen as unilateral or bilateral enlargement of ventro medial portion of medulla with t2 hyperintensity which corresponds to ion. This section provides sciencebased information for. Cerebellar mr changes in patients with olivary hypertrophic degeneration. Hypertrophic olivary degeneration hod was first reported in 1887 by oppenheim. The forgotten triangle of guillain and mollaret 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. Hypertrophic olivary degeneration clinical correlates. Hypertrophic olivary degeneration hod is a rare abnormality that is caused by a lesion in the guillainmollaret triangle in the brainstem. They all refer to mulberryshaped abnormal blood vessels in the brain or spinal cord. Cavernous angioma, cavernoma, cavernous malformation, cavernous hemangioma, and ccm. Transneuronal degeneration of the inferior olivary nucleus occurs following its deafferentation by interruption of the dentatorubral or the rubroolivary pathways figure 6b. 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. Ioh is usually associated with the interruption of the dentatoolivary tract, most often from cerebrovascular. Clinical and radiologic hallmarks of this condition are palatal myoclonus and t2 hyperintensity of the inferior olivary complex on magnetic. Hypertrophic olivary degeneration and cerebrovascular.
Hypertrophic olivary degeneration is a degeneration of the inferior olivary nucleus that occurs secondary to a lesion in the dentorubroolivary pathway drop. Angioma alliance common hispanic mutation on ccm1 gene. T2 hyperintensity and enlargement of the inferior olivary nucleus ion are the radiological hallmarks of this entity. Hypertrophic olivary degeneration hod is a transneuronal degeneration secondary to focal lesions involving the dentatorubralolivary pathway, also know as guillainmollaret triangle. Hypertrophic olivary degeneration secondary to central. Cerebelloolivary degeneration of holmes by autumn brown. Hypertrophic olivary degeneration hod is a rare condition characterized by a unique pattern of transsynaptic degeneration. 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. 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. 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 hypertrophy 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.
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 genetic and rare diseases nih. Imaging features of hypertrophic olivary degeneration ruth van eetvelde, m. Symptoms and treatment hypertrophic olivary degeneration. 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. This is a rare case of hypertrophic olivary degeneration hod in a patient with previous history of pontine hemorrhagic infarct. 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 usually caused by a lesion along the dentaterubroolivary pathway, also known as the anatomic. Hypertrophic olivary degeneration cerebrovascular disease.
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. Bilateral hypertrophic olivary degeneration in wilson disease. 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. 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 following pontine haemorrhage. Transneuronal degeneration of the inferior olivary nucleus occurs following its deafferentation by interruption of the dentatorubral or the rubro olivary pathways figure 6b. A 59yearold man developed the acute onset of diplopia, dizziness, left hemiparesis, and slurred speech. Pdf hypertrophic olivary degeneration and palatal or. Holmes disease is a rare autosomallyinherited disease. Hypertrophic olivary degeneration hod is a rare type of neuronal degeneration involving the dentatorubroolivary pathway. 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. Patients with hod often present with palatal myoclonus, ataxia, tremor, dysarthria andor hemiparesis. The degeneration occurring in hypertrophc olivary degeneration hod is unique, as it causes hypertrophy rather than atrophy, a change which may last up to 35 years. These are usually formed in response to degenerative changes and these are examples of hypertrophic degeneration.
Top 41 on hypertrophic degenerative facet disease healthtap. Hypertrophic olivary degeneration radiology reference. 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. Hypertrophic olivary degeneration hod is an unusual neuronal degenerative disease involving inferior olivary nuclei with increased t2. Hod is a rare condition characterized by a unique pattern of transsynaptic degeneration caused by damage dentatorubroolivary pathway.
The dentatorubro olivary pathway is a neural network involved in fine voluntary motor control and consists of the red nucleus, the ipsilateral inferior. Pdf hypertrophic olivary degeneration secondary to. Histologically, there is vacuolar cytoplasmic degeneration with hypertrophy of the olivary nucleus due to an increase in the number of astrocytes. 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. Mri and neurological presentation of hypertrophic olivary. The dentatorubroolivary pathway is a neural network involved in fine voluntary motor control and consists of the red nucleus, the ipsilateral inferior. A rare presentation of hypertrophic olivary degeneration. It is associated with hypertrophic inferior olivary degeneration that is characterized by enlarged and vacuolated neurons, increased number. Full text a case of hypertrophic olivary degeneration after resection. Hypertrophic olivary degeneration hod is a rare transsynaptic degeneration that usually appears at around 34 weeks following an injury to the 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. Hypertrophic degeneration of the inferior olive is mainly observed in patients developing palatal tremor pt or oculopalatal tremor opt. 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. Four years earlier, a ruptured vascular malformation caused a hemorrhagic stroke on the left cerebellar hemisphere, evacuated by surgery. The degeneration is unique in that it is associated. 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.
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 with an eggshaped appearance. Mri revealed right hypertrophic olivary degeneration hod and reduction of cerebellarrubro pathways. In musculoskeletal system margins of joints form osteophyte which are outgrowths of bones at margins of joints. 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. T1 mri findings in nonlesional hypertrophic olivary degeneration. Letters to editor hypertrophic olivary degeneration. This case highlights some important teaching points. 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 after cerebellar or. 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 is a transsynaptic form of degeneration, which is also a. Early mri shows t2 lengthening in the dentate nucleus, superior cerebellar peduncle. Hypertrophic olivary degeneration and holmes tremor. Imaging features of hypertrophic olivary degeneration. Magnetic resonance imaging demonstrated hypertrophic olivary degeneration hod secondary to central tegmental tract injury. There patterns of hod in relation to location of primary lesion. Hypertrophic olivary degeneration resulting from lesions of the dentorubroolivary pathway, also called guillainmollarettriangle, has been described previously in a number of cases. Bilateral hypertrophic olivary degeneration europe pmc. Bilateral hypertrophic olivary nucleus degeneration on. Mri findings in nonlesional 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. It is a relatively normal finding in the spine as we age.
Hypertrophic olivary degeneration hod is a disease that can be a. Hypertrophic olivary degeneration hod hypertrophic olivary degeneration is a rare finding secondary to focal lesions of the brain stem involving guillainmollaret triangle. A 28yearold woman presented with hemidystonic movements on the left. Hypertrophic olivary degeneration after cerebellar hemorrhage. The pathophysiological basis for hypertrophic olivary.
The size of the hypertrophic olivary nucleus is variable, depending on the time interval between the event and the scanning procedure, with a normal size in the. Hod occurs when a lesion, usually a haemorrhage, causes an interruption of the guillainmollaret triangle fig1. Sometimes, bone spur formation can put pressure on nearby nerves and pinch them, causing lots of pain, weakness, or numbness. Hypertrophic olivary degeneration hod is usually caused by a lesion in the triangle of guillain and mollaret and presents clinically as palatal tremor. In cns the degeneration of an anatomical structure. Frontiers hypertrophic olivary degeneration and palatal. Hypertrophic olivary degeneration hod occurs because of posterior fossa or brainstem lesions that disrupt the dentatorubro olivary tract, well known as the guillainmollaret triangle. Ancestors of spanish settlers who came to new mexico around 1600 may have a genetic mutation on the ccm1 gene that causes cavernous angioma, also known as cavernous malformation or cavernoma.
Ipsilateal hod, when primary lesion is in brain stem central tegmental tract. Hypertrophic olivary degeneration secondary to neuro. Hypertrophic olivary degeneration hod is a rare entity that develops after an injury to the dentatorubro olivary pathway drop also called the guillainmollaret triangle, or gmt. This mutation is known as the common hispanic mutation. Hypertrophic olivary degeneration secondary to central tegmental tract injury article pdf available in radiologia brasileira 483. 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.