Hypertrophic olivary degeneration

Changed by David Preston, 11 Mar 2018
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Hypertrophic olivary degeneration (HOD) is a rare condition characterised by a unique pattern of trans-synaptic degeneration. It is caused by a lesion in the triangle of Guillain and Mollaret, resulting in hypertrophy of the inferior olivary nucleus. The three corners of the triangle are:

Clinical presentation

Palatal myoclonus is a, rhythmic involuntary movement of the soft palate, uvula, pharynx, larynx and upper extremity are classically described clinical featurefeatures.

Pathology

Interruption of either connection between the dentate nucleus and contralateral red nucleus (dentatorubral tract, superior cerebellar peduncle) or the connection between the red nucleus and ipsilateral inferior olivary nucleus (central tegmental tract) leads to changes in the olive.

Pathologically, this is characterised by "trans-synaptic degeneration resulting in vacuolation of the neurons" and an increase in astrocytes. Isolated lesions of the inferior cerebellar peduncle do not cause HOD, as anatomically there are no direct connections between the inferior olivary nucleus and the contralateral dentate nucleus (fibres from the inferior olivary nucleus project to the cerebellar cortex via the olivocerebellar tracts and then to the dentate nucleus).

Radiographic features

It is often seen several months after the original insult.

MRI brain

In radiology, laymen terms the inferior olivary nucleus gets larger and increases in T2 signal intensity.

Typically, within a few months T2 signal increases and lasts 3-4 years, whereas hypertrophy occurs later (at about one year), and resolves by 3-4 years.

Differential diagnosis

General imaging differential considerations include:

  • -</ul><h4>Clinical presentation</h4><p>Palatal myoclonus is a classically described clinical feature.</p><h4>Pathology</h4><p>Interruption of either connection between the dentate nucleus and contralateral red nucleus (dentatorubral tract, superior cerebellar peduncle) or the connection between the red nucleus and ipsilateral inferior olivary nucleus (central tegmental tract) leads to changes in the olive.</p><p>Pathologically, this is characterised by "trans-synaptic degeneration resulting in vacuolation of the neurons" and an increase in astrocytes. Isolated lesions of the inferior cerebellar peduncle do not cause HOD, as anatomically there are no direct connections between the inferior olivary nucleus and the contralateral dentate nucleus (fibres from the inferior olivary nucleus project to the cerebellar cortex via the olivocerebellar tracts and then to the dentate nucleus).</p><h4>Radiographic features</h4><p>It is often seen several months after the original insult.</p><h5>MRI brain</h5><p>In radiology, laymen terms the inferior olivary nucleus gets larger and increases in T2 signal intensity.</p><p>Typically, within a few months T2 signal increases and lasts 3-4 years, whereas hypertrophy occurs later (at about one year), and resolves by 3-4 years.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>
  • +</ul><h4>Clinical presentation</h4><p>Palatal myoclonus, rhythmic involuntary movement of the soft palate, uvula, pharynx, larynx and upper extremity are classically described clinical features.</p><h4>Pathology</h4><p>Interruption of either connection between the dentate nucleus and contralateral red nucleus (dentatorubral tract, superior cerebellar peduncle) or the connection between the red nucleus and ipsilateral inferior olivary nucleus (central tegmental tract) leads to changes in the olive.</p><p>Pathologically, this is characterised by "trans-synaptic degeneration resulting in vacuolation of the neurons" and an increase in astrocytes. Isolated lesions of the inferior cerebellar peduncle do not cause HOD, as anatomically there are no direct connections between the inferior olivary nucleus and the contralateral dentate nucleus (fibres from the inferior olivary nucleus project to the cerebellar cortex via the olivocerebellar tracts and then to the dentate nucleus).</p><h4>Radiographic features</h4><p>It is often seen several months after the original insult.</p><h5>MRI brain</h5><p>In radiology, laymen terms the inferior olivary nucleus gets larger and increases in T2 signal intensity.</p><p>Typically, within a few months T2 signal increases and lasts 3-4 years, whereas hypertrophy occurs later (at about one year), and resolves by 3-4 years.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>

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