Medial medullary syndrome

Changed by Rohit Sharma, 26 Feb 2024
Disclosures - updated 18 Aug 2023: Nothing to disclose

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Medial medullary syndrome, also known as Déjerineéjerine syndrome, is secondary to thrombotic or embolic occlusion of small perforating branches from vertebral or proximal basilar artery supplying the medial aspect of medulla oblongata1,2.

Epidemiology

Represents less than 1% of brainstem stroke syndromes1,2.

Clinical presentation

It is characterizedcharacterised by contralateral hemiplegia/hemiparesis as well as hemisensory loss with ipsilateral hypoglossal palsy (ipsilateral tongue weakness and atrophy) from involvement of CN XII nucleus nucleus 1,2. Other manifestations such as vertigo, nausea, or contralateral limb ataxia are also reported 1,2.

Radiographic features

MRI

MRI with DWI is the best diagnostic test to confirm the infarct in the medial medulla, whereby the infarcted area has high DWI signal and is low signal on ADC. If bilateral medial medullary infarcts are present, the heart sign may be observed 4.

History and etymology

The syndrome was first described by Joseph Jules Déjerineéjerine (1849-1917), a a French neurologist, in 1915 3.

  • -<p><strong>Medial medullary syndrome</strong>, also known as <strong>Dé</strong><strong>jerine syndrome,  </strong>is secondary to thrombotic or embolic occlusion of small perforating branches from vertebral or proximal basilar artery supplying the medial aspect of <a href="/articles/medulla-oblongata">medulla oblongata</a><sup>1,2</sup>.</p><h4>Epidemiology</h4><p>Represents less than 1% of <a href="/articles/brainstem-stroke-syndromes">brainstem stroke syndromes</a> <sup>1,2</sup>.</p><h4>Clinical presentation</h4><p>It is characterized by contralateral hemiplegia/hemiparesis as well as hemisensory loss with ipsilateral hypoglossal palsy (ipsilateral tongue weakness and atrophy) from involvement of <a href="/articles/hypoglossal-nerve">CN XII</a> nucleus <sup>1,2</sup>. Other manifestations such as vertigo, nausea, or contralateral limb ataxia are also reported <sup>1,2</sup>.</p><h4>History and etymology</h4><p>The syndrome was first described by <strong>Joseph Jules Dé</strong><strong>jerine </strong>(1849-1917), a French neurologist, in 1915 <sup>3</sup>.</p>
  • +<p><strong>Medial medullary syndrome</strong>, also known as <strong>Déjerine syndrome, &nbsp;</strong>is secondary to thrombotic or embolic occlusion of small perforating branches from vertebral or proximal basilar artery supplying the medial aspect of <a href="/articles/medulla-oblongata">medulla oblongata</a><sup>1,2</sup>.</p><h4>Epidemiology</h4><p>Represents less than 1% of <a href="/articles/brainstem-stroke-syndromes">brainstem stroke syndromes</a>&nbsp;<sup>1,2</sup>.</p><h4>Clinical presentation</h4><p>It is characterised by contralateral hemiplegia/hemiparesis as well as hemisensory loss with ipsilateral hypoglossal palsy (ipsilateral tongue weakness and atrophy) from involvement of <a href="/articles/hypoglossal-nerve-1">CN XII</a>&nbsp;nucleus <sup>1,2</sup>. Other manifestations such as vertigo, nausea, or contralateral limb ataxia are also reported <sup>1,2</sup>.</p><h4>Radiographic features</h4><h5>MRI</h5><p>MRI with DWI is the best diagnostic test to confirm the infarct in the medial medulla, whereby the infarcted area has high DWI signal and is low signal on ADC. If bilateral medial medullary infarcts are present, the <a href="/articles/heart-sign-medulla" title="Heart sign (medulla)">heart sign</a> may be observed <sup>4</sup>.</p><h4>History and etymology</h4><p>The syndrome was first described by <strong>Joseph Jules Déjerine </strong>(1849-1917),&nbsp;a French neurologist, in 1915 <sup>3</sup>.</p>

References changed:

  • 4. Duarte-Celada W, Montalvan V, Bueso T, Davila-Siliezar P. Bilateral Medial Medullary Stroke: “The Heart Sign”. Radiology Case Reports. 2024;19(4):1329-32. <a href="https://doi.org/10.1016/j.radcr.2024.01.008">doi:10.1016/j.radcr.2024.01.008</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/38292797">Pubmed</a>

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