Paraneoplastic cerebellar degeneration

Changed by Jeremy Jones, 26 Feb 2018

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Paraneoplastic cerebellar degeneration is a type of paraneoplastic syndrome characterised by the progressive onset of cerebellar dysfunction not explained by tumoral invasion, metastasis, or treatment side effects. 

Epidemiology

It is an uncommon paraneoplastic syndrome that has been reported in patients with small-cell lung cancer, ovary malignancies, breast carcinoma, and Hodgkin's lymphoma 1

Clinical presentation

Cerebellar dysfunction may manifest as dizziness, nausea/vomiting, ataxia, diplopia, dysarthria, or dysphagia 1. A rapidly progressing gait unsteadiness is usually the key symptom that prompt for neuroimaging. 

Radiographic features

Brain imaging with CT or MRI may show either normal cerebellum, particularly in initial presentation, to eventual diffuse cerebellar atrophy. Cerebellar hemispheric enlargement and cortical-meningeal enhancement have been reported 2

Treatment and prognosis

The management is focused on the tumour treatment and supportive therapies. 

  • -<p><strong>Paraneoplastic cerebellar degeneration</strong> is a type of <a title="Paraneoplastic syndromes" href="/articles/paraneoplastic-syndromes">paraneoplastic syndrome</a> characterised by the progressive onset of cerebellar dysfunction not explained by tumoral invasion, metastasis, or treatment side effects. </p><h4>Epidemiology</h4><p>It is an uncommon paraneoplastic syndrome that has been reported in patients with small-cell lung cancer, ovary malignancies, breast carcinoma, and Hodgkin's lymphoma <sup>1</sup>. </p><h4>Clinical presentation</h4><p>Cerebellar dysfunction may manifest as dizziness, nausea/vomiting, ataxia, diplopia, dysarthria, or dysphagia <sup>1</sup>. A rapidly progressing gait unsteadiness is usually the key symptom that prompt for neuroimaging. </p><h4>Radiographic features</h4><p>Brain imaging with CT or MRI may show either normal cerebellum, particularly in initial presentation, to eventual diffuse <a title="Diffuse cerebellar atrophy" href="/articles/diffuse-cerebellar-atrophy">cerebellar atrophy</a>. Cerebellar hemispheric enlargement and cortical-meningeal enhancement have been reported <sup>2</sup>. </p><h4>Treatment and prognosis</h4><p>The management is focused on the tumour treatment and supportive therapies. </p>
  • +<p><strong>Paraneoplastic cerebellar degeneration</strong> is a type of <a href="/articles/paraneoplastic-syndromes">paraneoplastic syndrome</a> characterised by the progressive onset of cerebellar dysfunction not explained by tumoral invasion, metastasis, or treatment side effects. </p><h4>Epidemiology</h4><p>It is an uncommon paraneoplastic syndrome that has been reported in patients with small-cell lung cancer, ovary malignancies, breast carcinoma, and Hodgkin's lymphoma <sup>1</sup>. </p><h4>Clinical presentation</h4><p>Cerebellar dysfunction may manifest as dizziness, nausea/vomiting, ataxia, diplopia, dysarthria, or dysphagia <sup>1</sup>. A rapidly progressing gait unsteadiness is usually the key symptom that prompt for neuroimaging. </p><h4>Radiographic features</h4><p>Brain imaging with CT or MRI may show either normal cerebellum, particularly in initial presentation, to eventual diffuse <a href="/articles/diffuse-cerebellar-atrophy">cerebellar atrophy</a>. Cerebellar hemispheric enlargement and cortical-meningeal enhancement have been reported <sup>2</sup>. </p><h4>Treatment and prognosis</h4><p>The management is focused on the tumour treatment and supportive therapies. </p>

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