Adult leukodystrophies

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Adult-onset leukodystrophies are uncommon compared to those that present in childhood~~-onset but~~ and in most instances are ana delayed and atypical presentation of conditions more common in childhood. They are important ~~consideration~~differential considerations when assessing ~~diffuse~~ adults with white matter ~~disease~~diseases. ~~Most leukodystrophies diagnosed in adults, with only a few exceptions, are conditions that are also seen with greater frequency in younger populations.~~

Terminology

It should be noted~~, however,~~ that the term leukodystrophy and which conditions are considered to be leukodystrophies varies from publication to publication. ~~For example,~~This is discussed in ~~some instances, CADASIL is included~~greater detail in the general article: leukodystrophy.

Adult-onset leukodystrophies

~~Work~~The vast majority of adult-onset leukodystrophies are conditions that are also, and most frequently, diagnosed in ~~progress~~childhood. Some rare entities, however, are exclusively seen in adults ¹. Below are the more common conditions seen in each instance ^1,2.

Adult-onset of leukodystrophies usually diagnosed in childhood

Alexander disease
cerebrotendinous xanthomatosis
globoid cell leukodystrophy (Krabbe disease)
L-2-hydroxyglutaric aciduria
leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation
leukoencephalopathy with ataxia
leukoencephalopathy with calcifications and cysts (Labrun syndrome)
megalencephalic leukoencephalopathy with subcortical cysts
metachromatic leukodystrophy
Sjogren-Larsson syndrome
vanishing white matter disease
x-linked adrenoleukodystrophy
- adult cerebral adrenoleukodystrophy
- adrenomyeloneuropathy

Leukodystrophies exclusively diagnosed in adults

-<p><strong>Adult-onset leukodystrophies</strong> are uncommon compared to childhood-onset but are an important consideration when assessing diffuse white matter disease. Most leukodystrophies diagnosed in adults, with only a few exceptions, are conditions that are also seen with greater frequency in younger populations.</p><h4>Terminology</h4><p>It should be noted, however, that which conditions are considered leukodystrophies varies from publication to publication. For example, in some instances, CADASIL is included.</p><h4>Adult-onset leukodystrophies </h4><p> </p><p><em>Work in progress</em></p>
+<p><strong>Adult-onset leukodystrophies</strong> are uncommon compared to those that present in childhood and in most instances are a delayed and atypical presentation of conditions more common in childhood. They are important differential considerations when assessing adults with <a title="White matter diseases" href="/articles/white-matter-disorders">white matter diseases</a>. </p><h4>Terminology</h4><p>It should be noted that the term leukodystrophy and which conditions are considered to be leukodystrophies varies from publication to publication. This is discussed in greater detail in the general article: <a title="Leukodystrophy" href="/articles/leukodystrophies">leukodystrophy</a>. </p><h4>Adult-onset leukodystrophies </h4><p>The vast majority of adult-onset leukodystrophies are conditions that are also, and most frequently, diagnosed in childhood. Some rare entities, however, are exclusively seen in adults <sup>1</sup>. Below are the more common conditions seen in each instance <sup>1,2</sup>. </p><h5>Adult-onset of leukodystrophies usually diagnosed in childhood </h5><ul>
+<li><a href="/articles/alexander-disease">Alexander disease</a></li>
+<li><a title="Cerebrotendinous xanthomatosis" href="/articles/cerebrotendinous-xanthomatosis">cerebrotendinous xanthomatosis</a></li>
+<li>
+<a href="/articles/krabbe-disease">globoid cell leukodystrophy</a> (Krabbe disease)</li>
+<li><a href="/articles/l-2-hydroxyglutaric-aciduria-1">L-2-hydroxyglutaric aciduria</a></li>
+<li><a href="/articles/leukoencephalopathy-with-brainstem-and-spinal-cord-involvement-and-lactate-elevation">leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation</a></li>
+<li><a title="leukoencephalopathy with ataxia" href="/articles/leukoencephalopathy-with-ataxia">leukoencephalopathy with ataxia</a></li>
+<li>
+<a href="/articles/leukoencephalopathy-with-calcifications-and-cysts-1">leukoencephalopathy with calcifications and cysts</a> (Labrun syndrome)</li>
+<li><a href="/articles/megalencephalic-leukoencephalopathy-with-subcortical-cysts-1">megalencephalic leukoencephalopathy with subcortical cysts</a></li>
+<li><a href="/articles/metachromatic-leukodystrophy">metachromatic leukodystrophy</a></li>
+<li><a href="/articles/sjogren-larsson-syndrome">Sjogren-Larsson syndrome</a></li>
+<li><a href="/articles/vanishing-white-matter-disease">vanishing white matter disease</a></li>
+<li>
+<a href="/articles/adrenoleukodystrophies">x-linked adrenoleukodystrophy</a><ul>
+<li>adult cerebral adrenoleukodystrophy</li>
+<li><a title="Adrenomyeloneuropathy" href="/articles/adrenomyeloneuropathy">adrenomyeloneuropathy</a></li>
+</ul>
+</li>
+</ul><h5>Leukodystrophies exclusively diagnosed in adults</h5><ul>
+<li><a href="/articles/adult-onset-leukoencephalopathy-with-axonal-spheroids-and-pigmented-glia">adult-onset leukoencephalopathy with axonal spheroids and pigmented glia</a></li>
+<li><a title="autosomal dominant adult-onset demyelinating leukodystrophy" href="/articles/autosomal-dominant-adult-onset-demyelinating-leukodystrophy">autosomal dominant adult-onset demyelinating leukodystrophy</a></li>
+<li>
+<a title="adult polyglucosan body disease" href="/articles/adult-polyglucosan-body-disease">adult </a><a title="adult polyglucosan body disease" href="/articles/adult-polyglucosan-body-disease">polyglucosan</a><a title="adult polyglucosan body disease" href="/articles/adult-polyglucosan-body-disease"> body disease</a>
+</li>
+</ul>

References changed:

1. Köhler W, Curiel J, Vanderver A. Adulthood leukodystrophies. (2018) Nature reviews. Neurology. 14 (2): 94-105. <a href="https://doi.org/10.1038/nrneurol.2017.175">doi:10.1038/nrneurol.2017.175</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29302065">Pubmed</a> <span class="ref_v4"></span>
2. Resende LL, de Paiva ARB, Kok F, et al. Adult Leukodystrophies: A Step-by-Step Diagnostic Approach. (2019) Radiographics : a review publication of the Radiological Society of North America, Inc. 39 (1): 153-168. <a href="https://doi.org/10.1148/rg.2019180081">doi:10.1148/rg.2019180081</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/30620693">Pubmed</a> <span class="ref_v4"></span>

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