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dir="ltr"><div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Group of neurodegenerative disorders with associated iron accumulation in the brain</div> <div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Medical condition</div><style data-mw-deduplicate="TemplateStyles:r1257001546">.mw-parser-output .infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox ib-medical-condition"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Neurodegeneration with brain iron accumulation</th></tr><tr><th scope="row" class="infobox-label">Other names</th><td class="infobox-data">NBIA</td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="/wiki/Neurology" title="Neurology">Neurology</a> <span class="penicon autoconfirmed-show"><span class="mw-valign-text-top" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q16892735?uselang=en#P1995" title="Edit this on Wikidata"><img alt="Edit this on Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></span></td></tr></tbody></table> <p><b>Neurodegeneration with brain iron accumulation</b> is a heterogenous group of inherited <a href="/wiki/Neurodegenerative_disease" title="Neurodegenerative disease">neurodegenerative diseases</a>, still under research, in which iron accumulates in the <a href="/wiki/Basal_ganglia" title="Basal ganglia">basal ganglia</a>, either resulting in progressive <a href="/wiki/Dystonia" title="Dystonia">dystonia</a>, <a href="/wiki/Parkinsonism" title="Parkinsonism">parkinsonism</a>, <a href="/wiki/Spasticity" title="Spasticity">spasticity</a>, <a href="/wiki/Optic_atrophy" class="mw-redirect" title="Optic atrophy">optic atrophy</a>, <a href="/wiki/Retinopathy" title="Retinopathy">retinal degeneration</a>, <a href="/wiki/Neuropsychiatric" class="mw-redirect" title="Neuropsychiatric">neuropsychiatric</a>, or diverse neurologic abnormalities.<sup id="cite_ref-1" class="reference"><a href="#cite_note-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> Some of the NBIA disorders have also been associated with several genes in synapse and lipid metabolism related pathways.<sup id="cite_ref-2" class="reference"><a href="#cite_note-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> NBIA is not one disease but an entire group of disorders, characterized by an accumulation of brain iron, sometimes in the presence of <a href="/wiki/Axonal_spheroid" class="mw-redirect" title="Axonal spheroid">axonal spheroids</a> in the <a href="/wiki/Central_nervous_system" title="Central nervous system">central nervous system</a>.<sup id="cite_ref-Gregory2009_3-0" class="reference"><a href="#cite_note-Gregory2009-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>Iron accumulation can occur anywhere in the brain, with accumulation typically occurring in <a href="/wiki/Globus_pallidus" title="Globus pallidus">globus pallidus</a>, <a href="/wiki/Substantia_nigra" title="Substantia nigra">substantia nigra</a>, pars reticula, <a href="/wiki/Striatum" title="Striatum">striatum</a> and cerebellar <a href="/wiki/Dentate_nucleus" title="Dentate nucleus">dentate nuclei</a>.<sup id="cite_ref-Dusek2012_4-0" class="reference"><a href="#cite_note-Dusek2012-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Symptoms can include various movement disorders, neuropsychiatric issues, seizures, visual disturbances, and cognitive decline, usually in different combinations.<sup id="cite_ref-Dusek2012_4-1" class="reference"><a href="#cite_note-Dusek2012-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Ten to fifteen genetic NBIA disorders involving various cell processes have been identified: iron metabolism, <a href="/wiki/Coenzyme_A" title="Coenzyme A">coenzyme A</a> biosynthesis, <a href="/wiki/Phospholipid" title="Phospholipid">phospholipid</a> metabolism, <a href="/wiki/Ceramide" title="Ceramide">ceramide</a> metabolism, lysosomal disorders, as well as mutations in genes with unknown functions.<sup id="cite_ref-Spaull2021_5-0" class="reference"><a href="#cite_note-Spaull2021-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Dusek2012_4-2" class="reference"><a href="#cite_note-Dusek2012-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Onset can occur at different ages, from early childhood to late adulthood.<sup id="cite_ref-Dusek2012_4-3" class="reference"><a href="#cite_note-Dusek2012-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p><p>As of 2021<sup class="plainlinks noexcerpt noprint asof-tag update" style="display:none;"><a class="external text" href="https://en.wikipedia.org/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit">[update]</a></sup> there were no curative treatments for any of the NBIA disorders, though several medications have been subject to clinical trial including the iron <a href="/wiki/Chelation" title="Chelation">chelator</a> <a href="/wiki/Deferiprone" title="Deferiprone">deferiprone</a>.<sup id="cite_ref-Spaull2021_5-1" class="reference"><a href="#cite_note-Spaull2021-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Variants">Variants</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=1" title="Edit section: Variants"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <table class="wikitable"> <caption>Overview of monogenic NBIA disorders<sup id="cite_ref-Spaull2021_5-2" class="reference"><a href="#cite_note-Spaull2021-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-7" class="reference"><a href="#cite_note-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </caption> <tbody><tr> <th>NBIA variant</th> <th><a href="/wiki/Gene" title="Gene">Gene</a></th> <th><a href="/wiki/Heredity" title="Heredity">Inheritance</a> </th></tr> <tr> <td><a href="/wiki/Pantothenate_kinase-associated_neurodegeneration" title="Pantothenate kinase-associated neurodegeneration">Pantothenate kinase-associated neurodegeneration</a> (PKAN)<sup id="cite_ref-8" class="reference"><a href="#cite_note-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup></td> <td><i><a href="/wiki/PANK2_(gene)" title="PANK2 (gene)">PANK2</a></i></td> <td><a href="/wiki/Autosome" title="Autosome">autosomal</a> <a href="/wiki/Dominance_(genetics)" title="Dominance (genetics)">recessive</a> </td></tr> <tr> <td><a href="/w/index.php?title=PLA2G6-associated_neurodegeneration&action=edit&redlink=1" class="new" title="PLA2G6-associated neurodegeneration (page does not exist)"><i>PLA2G6</i>-associated neurodegeneration</a> (PLAN)<sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup></td> <td><i><a href="/wiki/PLA2G6" title="PLA2G6">PLA2G6</a></i></td> <td>autosomal recessive </td></tr> <tr> <td><a href="/wiki/Mitochondrial_membrane_protein-associated_neurodegeneration" title="Mitochondrial membrane protein-associated neurodegeneration">Mitochondrial membrane protein-associated neurodegeneration</a> (MPAN)<sup id="cite_ref-10" class="reference"><a href="#cite_note-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup></td> <td><i>C19orf12</i></td> <td>autosomal <a href="/wiki/Dominance_(genetics)" title="Dominance (genetics)">recessive or dominant</a> </td></tr> <tr> <td><a href="/wiki/Beta-propeller_protein-associated_neurodegeneration" class="mw-redirect" title="Beta-propeller protein-associated neurodegeneration">Beta-propeller protein-associated neurodegeneration</a> (BPAN)<sup id="cite_ref-11" class="reference"><a href="#cite_note-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup></td> <td><i><a href="/wiki/WDR45" title="WDR45">WDR45</a></i></td> <td><a href="/wiki/X-linked_dominant_inheritance" title="X-linked dominant inheritance">X-linked dominant</a> (mostly <a href="/wiki/De_novo_mutation" title="De novo mutation">de novo mutations</a>) </td></tr> <tr> <td><a href="/w/index.php?title=Fatty_acid_hydroxylase-associated_neurodegeneration&action=edit&redlink=1" class="new" title="Fatty acid hydroxylase-associated neurodegeneration (page does not exist)">Fatty acid hydroxylase-associated neurodegeneration</a> (FAHN)<sup id="cite_ref-12" class="reference"><a href="#cite_note-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup></td> <td><i><a href="/wiki/FA2H" title="FA2H">FA2H</a></i></td> <td>autosomal recessive </td></tr> <tr> <td><a href="/wiki/Kufor%E2%80%93Rakeb_syndrome" title="Kufor–Rakeb syndrome">Kufor–Rakeb syndrome</a></td> <td><i><a href="/wiki/ATP13A2" title="ATP13A2">ATP13A2</a></i></td> <td>autosomal recessive </td></tr> <tr> <td><a href="/wiki/Neuroferritinopathy" title="Neuroferritinopathy">Neuroferritinopathy</a></td> <td><i><a href="/wiki/Ferritin_light_chain" title="Ferritin light chain">FTL</a></i></td> <td>autosomal <a href="/wiki/Dominance_(genetics)" title="Dominance (genetics)">dominant</a> </td></tr> <tr> <td><a href="/wiki/Aceruloplasminemia" title="Aceruloplasminemia">Aceruloplasminemia</a></td> <td><i><a href="/wiki/Ceruloplasmin" title="Ceruloplasmin">CP</a></i></td> <td>autosomal recessive </td></tr> <tr> <td><a href="/wiki/Woodhouse%E2%80%93Sakati_syndrome" title="Woodhouse–Sakati syndrome">Woodhouse–Sakati syndrome</a></td> <td><i><a href="/wiki/DCAF17" title="DCAF17">DCAF17</a></i></td> <td>autosomal recessive </td></tr> <tr> <td><a href="/w/index.php?title=COASY_protein-associated_neurodegeneration&action=edit&redlink=1" class="new" title="COASY protein-associated neurodegeneration (page does not exist)">COASY protein-associated neurodegeneration</a> (CoPAN)</td> <td><i><a href="/wiki/COASY" title="COASY">COASY</a></i></td> <td>autosomal recessive </td></tr> <tr> <td>NBIA7<sup id="cite_ref-Drecourt2018_13-0" class="reference"><a href="#cite_note-Drecourt2018-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup></td> <td><i><a href="/wiki/REPS1" title="REPS1">REPS1</a></i></td> <td>autosomal recessive </td></tr> <tr> <td>NBIA8<sup id="cite_ref-Drecourt2018_13-1" class="reference"><a href="#cite_note-Drecourt2018-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup></td> <td><i><a href="/wiki/Carnitine_O-acetyltransferase" title="Carnitine O-acetyltransferase">CRAT</a></i></td> <td>autosomal recessive </td></tr></tbody></table> <div class="mw-heading mw-heading2"><h2 id="Symptoms_and_signs">Symptoms and signs</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=2" title="Edit section: Symptoms and signs"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1251242444">.mw-parser-output .ambox{border:1px solid #a2a9b1;border-left:10px solid #36c;background-color:#fbfbfb;box-sizing:border-box}.mw-parser-output .ambox+link+.ambox,.mw-parser-output .ambox+link+style+.ambox,.mw-parser-output .ambox+link+link+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+style+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+link+.ambox{margin-top:-1px}html body.mediawiki .mw-parser-output .ambox.mbox-small-left{margin:4px 1em 4px 0;overflow:hidden;width:238px;border-collapse:collapse;font-size:88%;line-height:1.25em}.mw-parser-output .ambox-speedy{border-left:10px solid #b32424;background-color:#fee7e6}.mw-parser-output .ambox-delete{border-left:10px solid #b32424}.mw-parser-output .ambox-content{border-left:10px solid #f28500}.mw-parser-output .ambox-style{border-left:10px solid #fc3}.mw-parser-output .ambox-move{border-left:10px solid #9932cc}.mw-parser-output .ambox-protection{border-left:10px solid #a2a9b1}.mw-parser-output .ambox .mbox-text{border:none;padding:0.25em 0.5em;width:100%}.mw-parser-output .ambox .mbox-image{border:none;padding:2px 0 2px 0.5em;text-align:center}.mw-parser-output .ambox .mbox-imageright{border:none;padding:2px 0.5em 2px 0;text-align:center}.mw-parser-output .ambox .mbox-empty-cell{border:none;padding:0;width:1px}.mw-parser-output .ambox .mbox-image-div{width:52px}@media(min-width:720px){.mw-parser-output .ambox{margin:0 10%}}@media print{body.ns-0 .mw-parser-output .ambox{display:none!important}}</style><table class="box-Empty_section plainlinks metadata ambox mbox-small-left ambox-content" role="presentation"><tbody><tr><td class="mbox-image"><span typeof="mw:File"><a href="/wiki/File:Wiki_letter_w_cropped.svg" class="mw-file-description"><img alt="[icon]" src="//upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/20px-Wiki_letter_w_cropped.svg.png" decoding="async" width="20" height="14" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/30px-Wiki_letter_w_cropped.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/40px-Wiki_letter_w_cropped.svg.png 2x" data-file-width="44" data-file-height="31" /></a></span></td><td class="mbox-text"><div class="mbox-text-span"><b>This section is empty.</b> You can help by <a class="external text" href="https://en.wikipedia.org/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=">adding to it</a>. <span class="date-container"><i>(<span class="date">October 2024</span>)</i></span></div></td></tr></tbody></table> <div class="mw-heading mw-heading2"><h2 id="Cause">Cause</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=3" title="Edit section: Cause"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1251242444"><table class="box-Empty_section plainlinks metadata ambox mbox-small-left ambox-content" role="presentation"><tbody><tr><td class="mbox-image"><span typeof="mw:File"><a href="/wiki/File:Wiki_letter_w_cropped.svg" class="mw-file-description"><img alt="[icon]" src="//upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/20px-Wiki_letter_w_cropped.svg.png" decoding="async" width="20" height="14" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/30px-Wiki_letter_w_cropped.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/40px-Wiki_letter_w_cropped.svg.png 2x" data-file-width="44" data-file-height="31" /></a></span></td><td class="mbox-text"><div class="mbox-text-span"><b>This section is empty.</b> You can help by <a class="external text" href="https://en.wikipedia.org/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=">adding to it</a>. <span class="date-container"><i>(<span class="date">October 2024</span>)</i></span></div></td></tr></tbody></table> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=4" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/DaT_scan" title="DaT scan">DaT scans</a>, <a href="/wiki/Transcranial_Doppler" title="Transcranial Doppler">transcranial Doppler</a> sonography (TCD), <a href="/wiki/PET_scan" class="mw-redirect" title="PET scan">PET scans</a>, and, in some cases, <a href="/wiki/Magnetic_resonance_imaging" title="Magnetic resonance imaging">magnetic resonance imaging</a> (MRI) (type of scans depending on the symptoms)<sup id="cite_ref-14" class="reference"><a href="#cite_note-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> are used to distinguish between the different forms of NBIA due to the accumulation of iron in different areas of the brain.<sup id="cite_ref-Hayflick2006_15-0" class="reference"><a href="#cite_note-Hayflick2006-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> Patients typically fall into two different categories: (1) early onset, rapid progression or (2) late onset, slow progression.<sup id="cite_ref-Hayflick2006_15-1" class="reference"><a href="#cite_note-Hayflick2006-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> The first type is considered to be the classic presentation, while the second type is thought to be a more atypical presentation. Phenotypes of the different disorders appear to be dependent on age, i.e. amount of iron accumulation and cognitive abilities.<sup id="cite_ref-Schneider2012_16-0" class="reference"><a href="#cite_note-Schneider2012-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Treatments">Treatments</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=5" title="Edit section: Treatments"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Effective disease-modifying treatments have not yet been found for any of the NBIA disorders.<sup id="cite_ref-Spaull2021_5-3" class="reference"><a href="#cite_note-Spaull2021-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> Treatment is supportive and focused on improving symptoms: <a href="/wiki/Dystonia" title="Dystonia">Dystonia</a> is a common debilitating symptom and can be managed with oral medications, and sometimes with <a href="/wiki/Deep_brain_stimulation" title="Deep brain stimulation">deep-brain electrical stimulation</a>, therapy support for walking, eating, and manual tasks is essential. Later, in many of the diseases, slowing and stopping of movement (known as parkinsonism) can become common. Removal of iron, using medications known as iron <a href="/wiki/Chelation" title="Chelation">chelators</a>, has been tested in clinical trial but was not definitively shown to be effective.<sup id="cite_ref-Klopstock2019_17-0" class="reference"><a href="#cite_note-Klopstock2019-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=6" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap mw-references-columns"><ol class="references"> <li id="cite_note-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-1">^</a></b></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite id="CITEREFWardChrichton2019" class="citation book cs1">Ward, Roberta J.; Chrichton, Robert R. (2019). "Chapter 4. Ironing out the Brain". In Sigel, Astrid; Freisinger, Eva; Sigel, Roland K. O.; Carver, Peggy L. (eds.). <i>Essential Metals in Medicine:Therapeutic Use and Toxicity of Metal Ions in the Clinic</i>. Vol. 19. Berlin: de Gruyter GmbH. pp. 87–122. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1515%2F9783110527872-010">10.1515/9783110527872-010</a>. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/978-3-11-052691-2" title="Special:BookSources/978-3-11-052691-2"><bdi>978-3-11-052691-2</bdi></a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/30855105">30855105</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=bookitem&rft.atitle=Chapter+4.+Ironing+out+the+Brain&rft.btitle=Essential+Metals+in+Medicine%3ATherapeutic+Use+and+Toxicity+of+Metal+Ions+in+the+Clinic&rft.place=Berlin&rft.pages=87-122&rft.pub=de+Gruyter+GmbH&rft.date=2019&rft_id=info%3Apmid%2F30855105&rft_id=info%3Adoi%2F10.1515%2F9783110527872-010&rft.isbn=978-3-11-052691-2&rft.aulast=Ward&rft.aufirst=Roberta+J.&rft.au=Chrichton%2C+Robert+R.&rfr_id=info%3Asid%2Fen.wikipedia.org%3ANeurodegeneration+with+brain+iron+accumulation" class="Z3988"></span> <span class="cs1-visible-error citation-comment"><code class="cs1-code">{{<a href="/wiki/Template:Cite_book" title="Template:Cite book">cite book</a>}}</code>: </span><span class="cs1-visible-error citation-comment"><code class="cs1-code">|journal=</code> ignored (<a href="/wiki/Help:CS1_errors#periodical_ignored" title="Help:CS1 errors">help</a>)</span></span> </li> <li id="cite_note-2"><span class="mw-cite-backlink"><b><a href="#cite_ref-2">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFBettencourtForaboscoWiethoffHeidari2016" class="citation journal cs1">Bettencourt C, Forabosco P, Wiethoff S, Heidari M, Johnstone DM, Botía JA, Collingwood JF, Hardy J, Milward EA, Ryten M, Houlden H (March 2016). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4731015">"Gene co-expression networks shed light into diseases of brain iron accumulation"</a>. primary. <i>Neurobiology of Disease</i>. <b>87</b>: 59–68. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.nbd.2015.12.004">10.1016/j.nbd.2015.12.004</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely 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"Syndromes of neurodegeneration with brain iron accumulation". review. <i>Seminars in Pediatric Neurology</i>. <b>19</b> (2): 57–66. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.spen.2012.03.005">10.1016/j.spen.2012.03.005</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/22704258">22704258</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Seminars+in+Pediatric+Neurology&rft.atitle=Syndromes+of+neurodegeneration+with+brain+iron+accumulation&rft.volume=19&rft.issue=2&rft.pages=57-66&rft.date=2012-06&rft_id=info%3Adoi%2F10.1016%2Fj.spen.2012.03.005&rft_id=info%3Apmid%2F22704258&rft.aulast=Schneider&rft.aufirst=SA&rft.au=Bhatia%2C+KP&rfr_id=info%3Asid%2Fen.wikipedia.org%3ANeurodegeneration+with+brain+iron+accumulation" class="Z3988"></span></span> </li> <li id="cite_note-Klopstock2019-17"><span class="mw-cite-backlink"><b><a href="#cite_ref-Klopstock2019_17-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFKlopstockTrictaNeumayrKarin2019" class="citation journal cs1">Klopstock, Thomas; Tricta, Fernando; Neumayr, Lynne; Karin, Ivan; Zorzi, Giovanna; et al. 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class="Z3988"></span></span> </li> </ol></div></div> <div class="mw-heading mw-heading2"><h2 id="External_links">External links</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Neurodegeneration_with_brain_iron_accumulation&action=edit&section=7" title="Edit section: External links"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="navbox-styles"><style data-mw-deduplicate="TemplateStyles:r1236075235">.mw-parser-output .navbox{box-sizing:border-box;border:1px solid #a2a9b1;width:100%;clear:both;font-size:88%;text-align:center;padding:1px;margin:1em auto 0}.mw-parser-output .navbox .navbox{margin-top:0}.mw-parser-output .navbox+.navbox,.mw-parser-output .navbox+.navbox-styles+.navbox{margin-top:-1px}.mw-parser-output .navbox-inner,.mw-parser-output .navbox-subgroup{width:100%}.mw-parser-output .navbox-group,.mw-parser-output .navbox-title,.mw-parser-output .navbox-abovebelow{padding:0.25em 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("counter(listitem)"\a0 "}</style><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"></div><div role="navigation" class="navbox" aria-label="Navbox" style="width:100%; margin:0.5em 0 0.5em 0;;padding:3px"><table class="nowraplinks navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q16892735" class="extiw" title="d:Q16892735">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse10/2019/en#/G23.0">G23.0</a></li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=C538421">C538421</a></li><li><b><a href="/wiki/Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb35160.htm">35160</a></li></ul></div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">External resources</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus</a></b>: <a rel="nofollow" class="external text" href="https://www.nlm.nih.gov/medlineplus/ency/article/001225.htm">001225</a></li><li><b><a href="/wiki/GeneReviews" title="GeneReviews">GeneReviews</a></b>: <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/books/NBK121988/">Neurodegeneration with Brain Iron Accumulation Disorders Overview</a></li><li><b><a href="/wiki/GARD" class="mw-redirect" title="GARD">GARD</a></b>: <a rel="nofollow" class="external text" href="https://rarediseases.info.nih.gov/diseases/11899/Neurodegeneration-with-brain-iron-accumulation">Neurodegeneration with brain iron accumulation</a></li><li><b><a href="/wiki/Radiopaedia" title="Radiopaedia">Radiopaedia</a></b>: <a rel="nofollow" class="external text" href="https://radiopaedia.org/articles/neurodegeneration-with-brain-iron-accumulation">neurodegeneration-with-brain-iron-accumulation</a></li><li><b><a href="/wiki/Orphanet" title="Orphanet">Orphanet</a></b>: <a rel="nofollow" class="external text" href="https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=385">385</a></li></ul></div></div></td></tr></tbody></table></div> <ul><li><a rel="nofollow" class="external text" href="https://www.ninds.nih.gov/health-information/disorders/neurodegeneration-brain-iron-accumulation">Neurodegeneration with Brain Iron Accumulation</a> information page from the <a href="/wiki/National_Institute_of_Neurological_Disorders_and_Stroke" title="National Institute of Neurological Disorders and Stroke">National Institute of Neurological Disorders and Stroke</a></li></ul> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Metal_deficiency_and_toxicity_disorders" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output .navbar-mini abbr{font-variant:small-caps;border-bottom:none;text-decoration:none;cursor:inherit}.mw-parser-output .navbar-ct-full{font-size:114%;margin:0 7em}.mw-parser-output .navbar-ct-mini{font-size:114%;margin:0 4em}html.skin-theme-clientpref-night .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}@media(prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}}@media print{.mw-parser-output .navbar{display:none!important}}</style><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Metal_deficiency_and_toxicity_disorders" title="Template:Metal deficiency and toxicity disorders"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Metal_deficiency_and_toxicity_disorders" title="Template talk:Metal deficiency and toxicity disorders"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Metal_deficiency_and_toxicity_disorders" title="Special:EditPage/Template:Metal deficiency and toxicity disorders"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Metal_deficiency_and_toxicity_disorders" style="font-size:114%;margin:0 4em">Metal deficiency and toxicity disorders</div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Iron_metabolism_disorder" title="Iron metabolism disorder">Iron</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i>excess:</i></span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Iron_overload" title="Iron overload">Iron overload</a></li> <li><a href="/wiki/Hemochromatosis" class="mw-redirect" title="Hemochromatosis">Hemochromatosis</a> <ul><li><a href="/wiki/HFE_hereditary_haemochromatosis" class="mw-redirect" title="HFE hereditary haemochromatosis">Hemochromatosis/HFE1</a></li> <li><a href="/wiki/Juvenile_hemochromatosis" title="Juvenile hemochromatosis">Juvenile/HFE2</a></li> <li><a href="/wiki/Haemochromatosis_type_3" title="Haemochromatosis type 3">HFE3</a></li> <li><a href="/wiki/African_iron_overload" title="African iron overload">African iron overload/HFE4</a></li></ul></li> <li><a href="/wiki/Aceruloplasminemia" title="Aceruloplasminemia">Aceruloplasminemia</a></li> <li><a href="/wiki/Atransferrinemia" title="Atransferrinemia">Atransferrinemia</a></li> <li><a href="/wiki/Hemosiderosis" title="Hemosiderosis">Hemosiderosis</a></li> <li><a class="mw-selflink selflink">Neurodegeneration with brain iron accumulation</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i>deficiency:</i></span></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Iron_deficiency" title="Iron deficiency">Iron deficiency</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Copper_metabolism" class="mw-redirect" title="Copper metabolism">Copper</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i>excess:</i></span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Copper_toxicity" title="Copper toxicity">Copper toxicity</a></li> <li><a href="/wiki/Wilson%27s_disease" title="Wilson's disease">Wilson's disease</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i>deficiency:</i></span></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Copper_deficiency" title="Copper deficiency">Copper deficiency</a></li> <li><a href="/wiki/Menkes_disease" title="Menkes disease">Menkes disease</a>/<a href="/wiki/Occipital_horn_syndrome" title="Occipital horn syndrome">Occipital horn syndrome</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Zinc_metabolism" class="mw-redirect" title="Zinc metabolism">Zinc</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i>excess:</i></span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Zinc_toxicity" title="Zinc toxicity">Zinc toxicity</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><i>deficiency:</i></span></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Acrodermatitis_enteropathica" title="Acrodermatitis enteropathica">Acrodermatitis enteropathica</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Inborn_errors_of_metabolism" title="Inborn errors of metabolism">Inborn errors of metabolism</a></li></ul> </div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Diseases_of_the_nervous_system,_primarily_CNS" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1239400231"><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Central_nervous_system_disease" title="Template:Central nervous system disease"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Central_nervous_system_disease" title="Template talk:Central nervous system disease"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Central_nervous_system_disease" title="Special:EditPage/Template:Central nervous system disease"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Diseases_of_the_nervous_system,_primarily_CNS" style="font-size:114%;margin:0 4em">Diseases of the <a href="/wiki/Nervous_system" title="Nervous system">nervous system</a>, primarily <a href="/wiki/Central_nervous_system_disease" title="Central nervous system disease">CNS</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Inflammation" title="Inflammation">Inflammation</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="Brain" scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Human_brain" title="Human brain">Brain</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Encephalitis" title="Encephalitis">Encephalitis</a> <ul><li><a href="/wiki/Viral_encephalitis" title="Viral encephalitis">Viral encephalitis</a></li> <li><a href="/wiki/Herpesviral_encephalitis" class="mw-redirect" title="Herpesviral encephalitis">Herpesviral encephalitis</a></li> <li><a href="/wiki/Limbic_encephalitis" title="Limbic encephalitis">Limbic encephalitis</a></li> <li><a href="/wiki/Encephalitis_lethargica" title="Encephalitis lethargica">Encephalitis lethargica</a></li></ul></li> <li><a href="/wiki/Cavernous_sinus_thrombosis" title="Cavernous sinus thrombosis">Cavernous sinus thrombosis</a></li> <li><a href="/wiki/Brain_abscess" title="Brain abscess">Brain abscess</a> <ul><li><a href="/wiki/Amoebic_brain_abscess" title="Amoebic brain abscess">Amoebic</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Brain and <a href="/wiki/Spinal_cord" title="Spinal cord">spinal cord</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Encephalomyelitis" title="Encephalomyelitis">Encephalomyelitis</a> <ul><li><a href="/wiki/Acute_disseminated_encephalomyelitis" title="Acute disseminated encephalomyelitis">Acute disseminated</a></li></ul></li> <li><a href="/wiki/Meningitis" title="Meningitis">Meningitis</a></li> <li><a href="/wiki/Meningoencephalitis" title="Meningoencephalitis">Meningoencephalitis</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Human_brain" title="Human brain">Brain</a>/<br /><a href="/wiki/Encephalopathy" title="Encephalopathy">encephalopathy</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Neurodegenerative_disease" title="Neurodegenerative disease">Degenerative</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Extrapyramidal_system" title="Extrapyramidal system">Extrapyramidal</a> and<br /><a href="/wiki/Movement_disorders" class="mw-redirect" title="Movement disorders">movement disorders</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Basal_ganglia_disease" title="Basal ganglia disease">Basal ganglia disease</a> <ul><li><a href="/wiki/Parkinsonism" title="Parkinsonism">Parkinsonism</a> <ul><li><a href="/wiki/Parkinson%27s_disease" title="Parkinson's disease">PD</a></li> <li><a href="/wiki/Postencephalitic_parkinsonism" class="mw-redirect" title="Postencephalitic parkinsonism">Postencephalitic</a></li> <li><a href="/wiki/Neuroleptic_malignant_syndrome" title="Neuroleptic malignant syndrome">NMS</a></li></ul></li> <li><a class="mw-selflink selflink">NBIA</a> <ul><li><a href="/wiki/Pantothenate_kinase-associated_neurodegeneration" title="Pantothenate kinase-associated neurodegeneration">PKAN</a></li></ul></li> <li><a href="/wiki/Tauopathy" title="Tauopathy">Tauopathy</a> <ul><li><a href="/wiki/Progressive_supranuclear_palsy" title="Progressive supranuclear palsy">PSP</a></li></ul></li> <li><a href="/wiki/Multiple_system_atrophy" title="Multiple system atrophy">Striatonigral degeneration</a></li> <li><a href="/wiki/Hemiballismus" title="Hemiballismus">Hemiballismus</a></li> <li><a href="/wiki/Huntington%27s_disease" title="Huntington's disease">HD</a></li> <li><a href="/wiki/Olivopontocerebellar_atrophy" class="mw-redirect" title="Olivopontocerebellar atrophy">OA</a></li></ul></li></ul> <ul><li><a href="/wiki/Dyskinesia" title="Dyskinesia">Dyskinesia</a> <ul><li><a href="/wiki/Dystonia" title="Dystonia">Dystonia</a> <ul><li><a href="/wiki/Status_dystonicus" title="Status dystonicus">Status dystonicus</a></li> <li><a href="/wiki/Spasmodic_torticollis" title="Spasmodic torticollis">Spasmodic torticollis</a></li> <li><a href="/wiki/Meige%27s_syndrome" title="Meige's syndrome">Meige's</a></li> <li><a href="/wiki/Blepharospasm" title="Blepharospasm">Blepharospasm</a></li></ul></li> <li><a href="/wiki/Athetosis" title="Athetosis">Athetosis</a></li> <li><a href="/wiki/Chorea" title="Chorea">Chorea</a> <ul><li><a href="/wiki/Choreoathetosis" title="Choreoathetosis">Choreoathetosis</a></li></ul></li> <li><a href="/wiki/Myoclonus" title="Myoclonus">Myoclonus</a> <ul><li><a href="/wiki/Myoclonic_epilepsy" title="Myoclonic epilepsy">Myoclonic epilepsy</a></li></ul></li> <li><a href="/wiki/Akathisia" title="Akathisia">Akathisia</a></li></ul></li></ul> <ul><li><a href="/wiki/Tremor" title="Tremor">Tremor</a> <ul><li><a href="/wiki/Essential_tremor" title="Essential tremor">Essential tremor</a></li> <li><a href="/wiki/Intention_tremor" title="Intention tremor">Intention tremor</a></li></ul></li> <li><a href="/wiki/Restless_legs_syndrome" title="Restless legs syndrome">Restless legs</a></li> <li><a href="/wiki/Stiff-person_syndrome" title="Stiff-person syndrome">Stiff-person</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Dementia" title="Dementia">Dementia</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Tauopathy" title="Tauopathy">Tauopathy</a> <ul><li><a href="/wiki/Alzheimer%27s_disease" title="Alzheimer's disease">Alzheimer's</a> <ul><li><a href="/wiki/Early-onset_Alzheimer%27s_disease" title="Early-onset Alzheimer's disease">Early-onset</a></li></ul></li> <li><a href="/wiki/Primary_progressive_aphasia" title="Primary progressive aphasia">Primary progressive aphasia</a></li></ul></li> <li><a href="/wiki/Frontotemporal_dementia" title="Frontotemporal dementia">Frontotemporal dementia</a>/<a href="/wiki/Frontotemporal_lobar_degeneration" title="Frontotemporal lobar degeneration">Frontotemporal lobar degeneration</a> <ul><li><a href="/wiki/Pick%27s_disease" class="mw-redirect" title="Pick's disease">Pick's</a></li></ul></li> <li><a href="/wiki/Lewy_bodies_dementia" class="mw-redirect" title="Lewy bodies dementia">Lewy bodies dementia</a></li> <li><a href="/wiki/Posterior_cortical_atrophy" title="Posterior cortical atrophy">Posterior cortical atrophy</a></li></ul> <p><a href="/wiki/Creutzfeldt%E2%80%93Jakob_disease" title="Creutzfeldt–Jakob disease">Creutzfeldt–Jakob disease</a> </p> <ul><li><a href="/wiki/Vascular_dementia" title="Vascular dementia">Vascular dementia</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mitochondrial_disease" title="Mitochondrial disease">Mitochondrial disease</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Leigh_syndrome" title="Leigh syndrome">Leigh syndrome</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Demyelinating_disease" title="Demyelinating disease">Demyelinating</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/CNS_demyelinating_autoimmune_diseases" title="CNS demyelinating autoimmune diseases">Autoimmune</a></li> <li><a href="/wiki/Inflammatory_demyelinating_diseases_of_the_central_nervous_system" title="Inflammatory demyelinating diseases of the central nervous system">Inflammatory</a></li> <li><a href="/wiki/Multiple_sclerosis" title="Multiple sclerosis">Multiple sclerosis</a></li> <li>For more detailed coverage, see <a href="/wiki/Template:Demyelinating_diseases_of_CNS" title="Template:Demyelinating diseases of CNS">Template:Demyelinating diseases of CNS</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Episodic/<br /><a href="/wiki/Paroxysmal_attack" title="Paroxysmal attack">paroxysmal</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Epileptic_seizure" class="mw-redirect" title="Epileptic seizure">Seizures</a> and <a href="/wiki/Epilepsy" title="Epilepsy">epilepsy</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Focal_seizure" title="Focal seizure">Focal</a></li> <li><a href="/wiki/Generalized_epilepsy" title="Generalized epilepsy">Generalised</a></li> <li><a href="/wiki/Status_epilepticus" title="Status epilepticus">Status epilepticus</a></li> <li>For more detailed coverage, see <a href="/wiki/Template:Epilepsy" class="mw-redirect" title="Template:Epilepsy">Template:Epilepsy</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Headache" title="Headache">Headache</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Migraine" title="Migraine">Migraine</a></li> <li><a href="/wiki/Cluster_headache" title="Cluster headache">Cluster</a></li> <li><a href="/wiki/Tension_headache" title="Tension headache">Tension</a></li> <li>For more detailed coverage, see <a href="/wiki/Template:Headache" title="Template:Headache">Template:Headache</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Cerebrovascular_disease" title="Cerebrovascular disease">Cerebrovascular</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Transient_ischemic_attack" title="Transient ischemic attack">TIA</a></li> <li><a href="/wiki/Stroke" title="Stroke">Stroke</a></li> <li>For more detailed coverage, see <a href="/wiki/Template:Cerebrovascular_diseases" title="Template:Cerebrovascular diseases">Template:Cerebrovascular diseases</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Sleep_disorders" class="mw-redirect" title="Sleep disorders">Sleep disorders</a> <ul><li>For more detailed coverage, see <a href="/wiki/Template:Sleep" title="Template:Sleep">Template:Sleep</a></li></ul></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Cerebrospinal_fluid" title="Cerebrospinal fluid">CSF</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Intracranial_pressure" title="Intracranial pressure">Intracranial hypertension</a> <ul><li><a href="/wiki/Hydrocephalus" title="Hydrocephalus">Hydrocephalus</a></li> <li><a href="/wiki/Normal_pressure_hydrocephalus" title="Normal pressure hydrocephalus">Normal pressure hydrocephalus</a></li> <li><a href="/wiki/Choroid_plexus_papilloma" title="Choroid plexus papilloma">Choroid plexus papilloma</a></li> <li><a href="/wiki/Idiopathic_intracranial_hypertension" title="Idiopathic intracranial hypertension">Idiopathic intracranial hypertension</a></li></ul></li> <li><a href="/wiki/Cerebral_edema" title="Cerebral edema">Cerebral edema</a></li> <li><a href="/wiki/Spontaneous_cerebrospinal_fluid_leak" class="mw-redirect" title="Spontaneous cerebrospinal fluid leak">Intracranial hypotension</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Brain_herniation" title="Brain herniation">Brain herniation</a></li> <li><a href="/wiki/Reye_syndrome" title="Reye syndrome">Reye syndrome</a></li> <li><a href="/wiki/Hepatic_encephalopathy" title="Hepatic encephalopathy">Hepatic encephalopathy</a></li> <li><a href="/wiki/Toxic_encephalopathy" title="Toxic encephalopathy">Toxic encephalopathy</a></li> <li><a href="/wiki/Hashimoto%27s_encephalopathy" title="Hashimoto's encephalopathy">Hashimoto's encephalopathy</a></li> <li><a href="/wiki/Fetal_alcohol_spectrum_disorder" title="Fetal alcohol spectrum disorder">Static encephalopathy</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Both/either</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="Degenerative" scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Neurodegenerative_disease" title="Neurodegenerative disease">Degenerative</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Spinocerebellar_ataxia" title="Spinocerebellar ataxia">SA</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Friedreich%27s_ataxia" title="Friedreich's ataxia">Friedreich's ataxia</a></li> <li><a href="/wiki/Ataxia%E2%80%93telangiectasia" title="Ataxia–telangiectasia">Ataxia–telangiectasia</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Motor_neuron_diseases" title="Motor neuron diseases">MND</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i><a href="/wiki/Upper_motor_neuron" title="Upper motor neuron">UMN</a> only:</i> <ul><li><a href="/wiki/Primary_lateral_sclerosis" title="Primary lateral sclerosis">Primary lateral sclerosis</a></li> <li><a href="/wiki/Pseudobulbar_palsy" title="Pseudobulbar palsy">Pseudobulbar palsy</a></li> <li><a href="/wiki/Hereditary_spastic_paraplegia" title="Hereditary spastic paraplegia">Hereditary spastic paraplegia</a></li></ul></li></ul> <ul><li><i><a href="/wiki/Lower_motor_neuron" title="Lower motor neuron">LMN</a> only:</i> <ul><li><a href="/wiki/Distal_hereditary_motor_neuronopathies" title="Distal hereditary motor neuronopathies">Distal hereditary motor neuronopathies</a></li> <li><a href="/wiki/Spinal_muscular_atrophies" title="Spinal muscular atrophies">Spinal muscular atrophies</a> <ul><li><a href="/wiki/Spinal_muscular_atrophy" title="Spinal muscular atrophy">SMA</a></li> <li><a href="/wiki/Spinal_and_bulbar_muscular_atrophy" title="Spinal and bulbar muscular atrophy">SMAX1</a></li> <li><a href="/wiki/X-linked_spinal_muscular_atrophy_type_2" title="X-linked spinal muscular atrophy type 2">SMAX2</a></li> <li><a href="/wiki/Distal_spinal_muscular_atrophy_type_1" title="Distal spinal muscular atrophy type 1">DSMA1</a></li> <li><a href="/wiki/Congenital_distal_spinal_muscular_atrophy" title="Congenital distal spinal muscular atrophy">Congenital DSMA</a></li> <li>Spinal muscular atrophy with lower extremity predominance (SMALED) <ul><li><a href="/wiki/Spinal_muscular_atrophy_with_lower_extremity_predominance_1" title="Spinal muscular atrophy with lower extremity predominance 1">SMALED1</a></li> <li><a href="/wiki/Spinal_muscular_atrophy_with_lower_extremity_predominance_2A" title="Spinal muscular atrophy with lower extremity predominance 2A">SMALED2A</a></li> <li><a href="/wiki/Spinal_muscular_atrophy_with_lower_extremity_predominance_2B" title="Spinal muscular atrophy with lower extremity predominance 2B">SMALED2B</a></li></ul></li> <li><a href="/wiki/Pontocerebellar_hypoplasia" title="Pontocerebellar hypoplasia">SMA-PCH</a></li> <li><a href="/wiki/Spinal_muscular_atrophy_with_progressive_myoclonic_epilepsy" title="Spinal muscular atrophy with progressive myoclonic epilepsy">SMA-PME</a></li></ul></li> <li><a href="/wiki/Progressive_muscular_atrophy" title="Progressive muscular atrophy">Progressive muscular atrophy</a></li> <li><a href="/wiki/Progressive_bulbar_palsy" title="Progressive bulbar palsy">Progressive bulbar palsy</a> <ul><li><a href="/wiki/Fazio%E2%80%93Londe_disease" title="Fazio–Londe disease">Fazio–Londe</a></li> <li><a href="/wiki/Infantile_progressive_bulbar_palsy" title="Infantile progressive bulbar palsy">Infantile progressive bulbar palsy</a></li></ul></li></ul></li></ul> <ul><li><i>both:</i> <ul><li><a href="/wiki/Amyotrophic_lateral_sclerosis" class="mw-redirect" title="Amyotrophic lateral sclerosis">Amyotrophic lateral sclerosis</a></li></ul></li></ul> </div></td></tr></tbody></table><div></div></td></tr></tbody></table><div></div></td></tr></tbody></table></div>    </div><noscript><img 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