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<meta name="keywords" content="C5436882, als26, amyotrophic lateral sclerosis 26 with or without frontotemporal dementia, disease or syndrome, tia1, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="Amyotrophic lateral sclerosis-26 with or without frontotemporal dementia (ALS26) is an autosomal dominant neurodegenerative disorder characterized by adult onset of upper and low motor neuron disease causing bulbar dysfunction and limb weakness (ALS). Patients may also develop frontotemporal dementia (FTD) manifest as primary progressive aphasia, memory impairment, executive dysfunction, and behavioral or personality changes. Although patients may present with 1 or the other diseases, all eventually develop ALS. Neuropathologic studies of the brain and spinal cord show TDP43 (605078)-immunoreactive cytoplasmic inclusions that correlate with clinical features and Lewy body-like cytoplasmic inclusions in lower motor neurons (summary by Mackenzie et al., 2017).&#13; For a discussion of genetic heterogeneity of amyotrophic lateral sclerosis, see ALS1 (105400)." /><meta name="robots" content="index,nofollow,noarchive" />
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<title>Amyotrophic lateral sclerosis 26 with or without frontotemporal dementia (Concept Id: C5436882)
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<!--
UID=1771903
ConceptID=C5436882
-->
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Amyotrophic lateral sclerosis 26 with or without frontotemporal dementia<span class="h1sub">(ALS26)</span></div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1771903</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information."><span class="highlight" style="background-color:">C5436882</span></a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonyms:</td>
<td>ALS26; AMYOTROPHIC LATERAL SCLEROSIS 26 WITH OR WITHOUT FRONTOTEMPORAL DEMENTIA</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td><a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#target-gene-loc">Gene (location):<img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a><div class="display-none" id="target-gene-loc">
Gene(s) directly associated with<br />
this condition or phenotype.</div></td>
<td><a target="_blank" title="TIA1 - ID: 7072 - NCBI Gene" href="/gene/7072" class="medgenPMinfo">TIA1</a> (2p13.3)</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td>Monarch Initiative:</td>
<td><a href="https://monarchinitiative.org/disease/MONDO:0030885" target="_blank">MONDO:0030885</a></td></tr>
<tr><td>OMIM<span class="superscript">®</span>:</td>
<td><a href="https://omim.org/entry/619133" target="_blank">619133</a></td></tr>
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<div class="portlet mgSection" id="ID_100">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">Amyotrophic lateral sclerosis-26 with or without frontotemporal dementia (ALS26) is an autosomal dominant neurodegenerative disorder characterized by adult onset of upper and low motor neuron disease causing bulbar dysfunction and limb weakness (ALS). Patients may also develop frontotemporal dementia (FTD) manifest as primary progressive aphasia, memory impairment, executive dysfunction, and behavioral or personality changes. Although patients may present with 1 or the other diseases, all eventually develop ALS. Neuropathologic studies of the brain and spinal cord show TDP43 (605078)-immunoreactive cytoplasmic inclusions that correlate with clinical features and Lewy body-like cytoplasmic inclusions in lower motor neurons (summary by Mackenzie et al., 2017).&#13; For a discussion of genetic heterogeneity of amyotrophic lateral sclerosis, see ALS1 (105400). [from <a title="Online Mendelian Inheritance in Man" href="http://www.omim.org" class="defSource" target="_blank">OMIM</a>]</div>
</div>
<div class="portlet mgSection" id="ID_102">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Clinical_features">Clinical features</h1><a sid="102" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln clinfeat"><strong>From HPO</strong><br />
<div class="divPopper rprt" id="clin_107956"><div><strong>Limb muscle weakness</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>107956</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0587246</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove">Reduced strength and weakness of the muscles of the arms and legs.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/107956">Feature record</a> | <a href="/medgen?term=%22Limb%20muscle%20weakness%22%5BClinical%20Features%5D%20OR%20107956%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_274"><div><strong>Amyotrophic lateral sclerosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>274</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0002736</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Amyotrophic lateral sclerosis (ALS) is a progressive disease that affects motor neurons, which are specialized nerve cells that control muscle movement. These nerve cells are found in the spinal cord and the brain. In ALS, motor neurons die (atrophy) over time, leading to muscle weakness, a loss of muscle mass, and an inability to control movement.\n\nThere are many different types of ALS; these types are distinguished by their signs and symptoms and their genetic cause or lack of clear genetic association. Most people with ALS have a form of the condition that is described as sporadic, which means it occurs in people with no apparent history of the disorder in their family. People with sporadic ALS usually first develop features of the condition in their late fifties or early sixties. A small proportion of people with ALS, estimated at 5 to 10 percent, have a family history of ALS or a related condition called frontotemporal dementia (FTD), which is a progressive brain disorder that affects personality, behavior, and language. The signs and symptoms of familial ALS typically first appear in one's late forties or early fifties. Rarely, people with familial ALS develop symptoms in childhood or their teenage years. These individuals have a rare form of the disorder known as juvenile ALS.\n\nThe first signs and symptoms of ALS may be so subtle that they are overlooked. The earliest symptoms include muscle twitching, cramping, stiffness, or weakness. Affected individuals may develop slurred speech (dysarthria) and, later, difficulty chewing or swallowing (dysphagia). Many people with ALS experience malnutrition because of reduced food intake due to dysphagia and an increase in their body's energy demands (metabolism) due to prolonged illness. Muscles become weaker as the disease progresses, and arms and legs begin to look thinner as muscle tissue atrophies. Individuals with ALS eventually lose muscle strength and the ability to walk. Affected individuals eventually become wheelchair-dependent and increasingly require help with personal care and other activities of daily living. Over time, muscle weakness causes affected individuals to lose the use of their hands and arms. Breathing becomes difficult because the muscles of the respiratory system weaken. Most people with ALS die from respiratory failure within 2 to 10 years after the signs and symptoms of ALS first appear; however, disease progression varies widely among affected individuals.\n\nApproximately 20 percent of individuals with ALS also develop FTD. Changes in personality and behavior may make it difficult for affected individuals to interact with others in a socially appropriate manner. Communication skills worsen as the disease progresses. It is unclear how the development of ALS and FTD are related. Individuals who develop both conditions are diagnosed as having ALS-FTD.\n\nA rare form of ALS that often runs in families is known as ALS-parkinsonism-dementia complex (ALS-PDC). This disorder is characterized by the signs and symptoms of ALS, in addition to a pattern of movement abnormalities known as parkinsonism, and a progressive loss of intellectual function (dementia). Signs of parkinsonism include unusually slow movements (bradykinesia), stiffness, and tremors. Affected members of the same family can have different combinations of signs and symptoms.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/274">Feature record</a> | <a href="/medgen?term=%22Amyotrophic%20lateral%20sclerosis%22%5BClinical%20Features%5D%20OR%20274%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_8159"><div><strong>Aphasia</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>8159</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0003537</a></dd><dt><span class="dotprefix"></span></dt><dd>Mental or Behavioral Dysfunction</dd></dl></div></div></div>
<div class="spaceAbove">An acquired language impairment of some or all of the abilities to produce or comprehend speech and to read or write.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/8159">Feature record</a> | <a href="/medgen?term=%22Aphasia%22%5BClinical%20Features%5D%20OR%208159%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_43126"><div><strong>Lewy bodies</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>43126</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0085200</a></dd><dt><span class="dotprefix"></span></dt><dd>Cell Component</dd></dl></div></div></div>
<div class="spaceAbove">Intracytoplasmic, eosinophilic, round to elongated inclusions found in vacuoles of injured or fragmented neurons. The presence of Lewy bodies is the histological marker of the degenerative changes in LEWY BODY DISEASE and PARKINSON DISEASE but they may be seen in other neurological conditions. They are typically found in the substantia nigra and locus coeruleus but they are also seen in the basal forebrain, hypothalamic nuclei, and neocortex.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen?term=%22Lewy%20bodies%22%5BClinical%20Features%5D%20OR%2043126%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_68579"><div><strong>Memory impairment</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>68579</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0233794</a></dd><dt><span class="dotprefix"></span></dt><dd>Mental or Behavioral Dysfunction</dd></dl></div></div></div>
<div class="spaceAbove">An impairment of memory as manifested by a reduced ability to remember things such as dates and names, and increased forgetfulness.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/68579">Feature record</a> | <a href="/medgen?term=%22Memory%20impairment%22%5BClinical%20Features%5D%20OR%2068579%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_66817"><div><strong>Personality changes</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>66817</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0240735</a></dd><dt><span class="dotprefix"></span></dt><dd>Mental or Behavioral Dysfunction</dd></dl></div></div></div>
<div class="spaceAbove">An abnormal shift in patterns of thinking, acting, or feeling.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/66817">Feature record</a> | <a href="/medgen?term=%22Personality%20changes%22%5BClinical%20Features%5D%20OR%2066817%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_83266"><div><strong>Frontotemporal dementia</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>83266</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0338451</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">In general, frontotemporal dementia (FTD) refers to a clinical manifestation of the pathologic finding of frontotemporal lobar degeneration (FTLD). FTD, the most common subtype of FTLD, is a behavioral variant characterized by changes in social and personal conduct with loss of volition, executive dysfunction, loss of abstract thought, and decreased speech output. A second clinical subtype of FTLD is 'semantic dementia,' characterized by specific loss of comprehension of language and impaired facial and object recognition. A third clinical subtype of FTLD is 'primary progressive aphasia' (PPA), characterized by a reduction in speech production, speech errors, and word retrieval difficulties resulting in mutism and an inability to communicate. All subtypes have relative preservation of memory, at least in the early stages. FTLD is often associated with parkinsonism or motor neuron disease (MND) resembling amyotrophic lateral sclerosis (ALS; 105400) (reviews by Tolnay and Probst, 2002 and Mackenzie and Rademakers, 2007). Mackenzie et al. (2009, 2010) provided a classification of FTLD subtypes according to the neuropathologic findings (see PATHOGENESIS below).&#13; Clinical Variability of Tauopathies&#13; Tauopathies comprise a clinically variable group of neurodegenerative diseases characterized neuropathologically by accumulation of abnormal MAPT-positive inclusions in nerve and/or glial cells. In addition to frontotemporal dementia, semantic dementia, and PPA, different clinical syndromes with overlapping features have been described, leading to confusion in the terminology (Tolnay and Probst, 2002). Other terms used historically include parkinsonism and dementia with pallidopontonigral degeneration (PPND) (Wszolek et al., 1992); disinhibition-dementia-parkinsonism-amyotrophy complex (DDPAC) (Lynch et al., 1994); frontotemporal dementia with parkinsonism (FLDEM) (Yamaoka et al., 1996); and multiple system tauopathy with presenile dementia (MSTD) (Spillantini et al., 1997). These disorders are characterized by variable degrees of frontal lobe dementia, parkinsonism, motor neuron disease, and amyotrophy.&#13; Other neurodegenerative disorders associated with mutations in the MAPT gene include Pick disease (172700) and progressive supranuclear palsy (PSP; 601104).&#13; Inherited neurodegenerative tauopathies linked to chromosome 17 and caused by mutation in the MAPT gene have also collectively been termed 'FTDP17' (Lee et al., 2001).&#13; Kertesz (2003) suggested the term 'Pick complex' to represent the overlapping syndromes of FTD, primary progressive aphasia (PPA), corticobasal degeneration (CBD), PSP, and FTD with motor neuron disease. He noted that frontotemporal dementia may also be referred to as 'clinical Pick disease' and that the term 'Pick disease' should be restricted to the pathologic finding of Pick bodies.&#13; Genetic Heterogeneity of Frontotemporal Lobar Degeneration&#13; Mutations in several different genes can cause frontotemporal dementia and frontotemporal lobar degeneration, with or without motor neuron disease. See FTD2 (607485), caused by mutation in the GRN gene (138945) on chromosome 17q21; FTDALS7 (600795), caused by mutation in the CHMP2B gene (609512) on chromosome 3p11; inclusion body myopathy with Paget disease and FTD (IBMPFD; 167320), caused by mutation in the VCP gene (601023) on chromosome 9p13; ALS6 (608030), caused by mutation in the FUS gene (137070) on 16p11; ALS10 (612069), caused by mutation in the TARDBP gene (605078) on 1p36; and FTDALS1 (105550), caused by mutation in the C9ORF72 gene (614260) on 9p21.&#13; In 1 family with FTD, a mutation was identified in the presenilin-1 gene (PSEN1; 104311) on chromosome 14, which is usually associated with a familial form of early-onset Alzheimer disease (AD3; 607822).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/83266">Feature record</a> | <a href="/medgen?term=%22Frontotemporal%20dementia%22%5BClinical%20Features%5D%20OR%2083266%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_898626"><div><strong>Bulbar palsy</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>898626</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C4082299</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Bulbar weakness (or bulbar palsy) refers to bilateral impairment of function of the lower cranial nerves IX, X, XI and XII, which occurs due to lower motor neuron lesion either at nuclear or fascicular level in the medulla or from bilateral lesions of the lower cranial nerves outside the brain-stem. Bulbar weakness is often associated with difficulty in chewing, weakness of the facial muscles, dysarthria, palatal weakness and regurgitation of fluids, dysphagia, and dysphonia.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/898626">Feature record</a> | <a href="/medgen?term=%22Bulbar%20palsy%22%5BClinical%20Features%5D%20OR%20898626%5Buid%5D">Search on this feature</a></div></div><div class="TreeLite" data-jigconfig="closed: 1"><div class="concept-def"><a class="small" href="#" onclick="jQuery(&quot;.TreeLite&quot;,&quot;#ID_102&quot;).TreeLite().openAll(); return false;">Show all</a><a class="small" href="#" onclick="jQuery(&quot;.TreeLite&quot;,&quot;#ID_102&quot;).TreeLite().closeAll(); return false;">Hide all</a></div><ul><li><span class="TLline">Abnormality of limbs</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_107956" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Limb muscle weakness</a></span></li></ul></li><li><span class="TLline">Abnormality of the nervous system</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_274" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Amyotrophic lateral sclerosis</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_8159" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Aphasia</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_898626" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Bulbar palsy</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_83266" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Frontotemporal dementia</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_43126" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Lewy bodies</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_68579" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Memory impairment</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_66817" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Personality changes</a></span></li></ul></li></ul></div></div>
</div>
<div class="portlet mgSection" id="ID_105">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
<div class="nl"><a target="_blank" href="/pubmed/36396270">Cognitive Impairment in Older Adults: Epidemiology, Diagnosis, and Treatment.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Pérez Palmer N,
Trejo Ortega B,
Joshi P</span><br />
<span class="medgenPMjournal">Psychiatr Clin North Am</span>
2022 Dec;45(4):639-661.
Epub 2022 Oct 14
doi: 10.1016/j.psc.2022.07.010.
<span class="bold">PMID: </span><a href="/pubmed/36396270" target="_blank">36396270</a></div>
<div class="nl"><a target="_blank" href="/pubmed/31358572">Speech and language therapy approaches to managing primary progressive aphasia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Volkmer A,
Rogalski E,
Henry M,
Taylor-Rubin C,
Ruggero L,
Khayum R,
Kindell J,
Gorno-Tempini ML,
Warren JD,
Rohrer JD</span><br />
<span class="medgenPMjournal">Pract Neurol</span>
2020 Apr;20(2):154-161.
Epub 2019 Jul 29
doi: 10.1136/practneurol-2018-001921.
<span class="bold">PMID: </span><a href="/pubmed/31358572" target="_blank">31358572</a><a href="/pmc/articles/PMC6986989" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26900156">The Differential Diagnosis and Treatment of Atypical Parkinsonism.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Levin J,
Kurz A,
Arzberger T,
Giese A,
Höglinger GU</span><br />
<span class="medgenPMjournal">Dtsch Arztebl Int</span>
2016 Feb 5;113(5):61-9.
doi: 10.3238/arztebl.2016.0061.
<span class="bold">PMID: </span><a href="/pubmed/26900156" target="_blank">26900156</a><a href="/pmc/articles/PMC4782269" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (455)</a></div></div>
</div>
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
<div class="portlet mgSection" id="ID_103">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
<div class="nl"><a target="_blank" href="/pubmed/35182511">Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Boeve BF,
Boxer AL,
Kumfor F,
Pijnenburg Y,
Rohrer JD</span><br />
<span class="medgenPMjournal">Lancet Neurol</span>
2022 Mar;21(3):258-272.
doi: 10.1016/S1474-4422(21)00341-0.
<span class="bold">PMID: </span><a href="/pubmed/35182511" target="_blank">35182511</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35039149">Progranulin as a therapeutic target in neurodegenerative diseases.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Rhinn H,
Tatton N,
McCaughey S,
Kurnellas M,
Rosenthal A</span><br />
<span class="medgenPMjournal">Trends Pharmacol Sci</span>
2022 Aug;43(8):641-652.
Epub 2022 Jan 15
doi: 10.1016/j.tips.2021.11.015.
<span class="bold">PMID: </span><a href="/pubmed/35039149" target="_blank">35039149</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34747778">Frontotemporal Dementia - Current Concepts.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Puppala GK,
Gorthi SP,
Chandran V,
Gundabolu G</span><br />
<span class="medgenPMjournal">Neurol India</span>
2021 Sep-Oct;69(5):1144-1152.
doi: 10.4103/0028-3886.329593.
<span class="bold">PMID: </span><a href="/pubmed/34747778" target="_blank">34747778</a></div>
<div class="nl"><a target="_blank" href="/pubmed/27637961">The epidemiology of amyotrophic lateral sclerosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Talbott EO,
Malek AM,
Lacomis D</span><br />
<span class="medgenPMjournal">Handb Clin Neurol</span>
2016;138:225-38.
doi: 10.1016/B978-0-12-802973-2.00013-6.
<span class="bold">PMID: </span><a href="/pubmed/27637961" target="_blank">27637961</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26595641">Frontotemporal dementia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bang J,
Spina S,
Miller BL</span><br />
<span class="medgenPMjournal">Lancet</span>
2015 Oct 24;386(10004):1672-82.
doi: 10.1016/S0140-6736(15)00461-4.
<span class="bold">PMID: </span><a href="/pubmed/26595641" target="_blank">26595641</a><a href="/pmc/articles/PMC5970949" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (4441)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/38081627">Frontotemporal dementia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Medina-Rioja R,
Gonzalez-Calderon G,
Masellis M</span><br />
<span class="medgenPMjournal">CMAJ</span>
2023 Dec 10;195(48):E1660.
doi: 10.1503/cmaj.230407.
<span class="bold">PMID: </span><a href="/pubmed/38081627" target="_blank">38081627</a><a href="/pmc/articles/PMC10718268" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/37511491">Frontotemporal Dementia, Where Do We Stand? A Narrative Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Antonioni A,
Raho EM,
Lopriore P,
Pace AP,
Latino RR,
Assogna M,
Mancuso M,
Gragnaniello D,
Granieri E,
Pugliatti M,
Di Lorenzo F,
Koch G</span><br />
<span class="medgenPMjournal">Int J Mol Sci</span>
2023 Jul 21;24(14)
doi: 10.3390/ijms241411732.
<span class="bold">PMID: </span><a href="/pubmed/37511491" target="_blank">37511491</a><a href="/pmc/articles/PMC10380352" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35182511">Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Boeve BF,
Boxer AL,
Kumfor F,
Pijnenburg Y,
Rohrer JD</span><br />
<span class="medgenPMjournal">Lancet Neurol</span>
2022 Mar;21(3):258-272.
doi: 10.1016/S1474-4422(21)00341-0.
<span class="bold">PMID: </span><a href="/pubmed/35182511" target="_blank">35182511</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28410663">Frontotemporal Dementia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Olney NT,
Spina S,
Miller BL</span><br />
<span class="medgenPMjournal">Neurol Clin</span>
2017 May;35(2):339-374.
doi: 10.1016/j.ncl.2017.01.008.
<span class="bold">PMID: </span><a href="/pubmed/28410663" target="_blank">28410663</a><a href="/pmc/articles/PMC5472209" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26595641">Frontotemporal dementia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bang J,
Spina S,
Miller BL</span><br />
<span class="medgenPMjournal">Lancet</span>
2015 Oct 24;386(10004):1672-82.
doi: 10.1016/S0140-6736(15)00461-4.
<span class="bold">PMID: </span><a href="/pubmed/26595641" target="_blank">26595641</a><a href="/pmc/articles/PMC5970949" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (4804)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/38178103">Gut microbiota, circulating cytokines and dementia: a Mendelian randomization study.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ji D,
Chen WZ,
Zhang L,
Zhang ZH,
Chen LJ</span><br />
<span class="medgenPMjournal">J Neuroinflammation</span>
2024 Jan 4;21(1):2.
doi: 10.1186/s12974-023-02999-0.
<span class="bold">PMID: </span><a href="/pubmed/38178103" target="_blank">38178103</a><a href="/pmc/articles/PMC10765696" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36737245">UNC13A in amyotrophic lateral sclerosis: from genetic association to therapeutic target.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Willemse SW,
Harley P,
van Eijk RPA,
Demaegd KC,
Zelina P,
Pasterkamp RJ,
van Damme P,
Ingre C,
van Rheenen W,
Veldink JH,
Kiernan MC,
Al-Chalabi A,
van den Berg LH,
Fratta P,
van Es MA</span><br />
<span class="medgenPMjournal">J Neurol Neurosurg Psychiatry</span>
2023 Aug;94(8):649-656.
Epub 2023 Feb 3
doi: 10.1136/jnnp-2022-330504.
<span class="bold">PMID: </span><a href="/pubmed/36737245" target="_blank">36737245</a><a href="/pmc/articles/PMC10359588" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35006557">Pharmacological Management of Apathy in Dementia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Azhar L,
Kusumo RW,
Marotta G,
Lanctôt KL,
Herrmann N</span><br />
<span class="medgenPMjournal">CNS Drugs</span>
2022 Feb;36(2):143-165.
Epub 2022 Jan 10
doi: 10.1007/s40263-021-00883-0.
<span class="bold">PMID: </span><a href="/pubmed/35006557" target="_blank">35006557</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28674982">Motoneuron Disease: Clinical.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ilieva H,
Maragakis NJ</span><br />
<span class="medgenPMjournal">Adv Neurobiol</span>
2017;15:191-210.
doi: 10.1007/978-3-319-57193-5_7.
<span class="bold">PMID: </span><a href="/pubmed/28674982" target="_blank">28674982</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28144880">Use of Stimulants in Bipolar Disorder.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Perugi G,
Vannucchi G,
Bedani F,
Favaretto E</span><br />
<span class="medgenPMjournal">Curr Psychiatry Rep</span>
2017 Jan;19(1):7.
doi: 10.1007/s11920-017-0758-x.
<span class="bold">PMID: </span><a href="/pubmed/28144880" target="_blank">28144880</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia%22%20AND%20Therapy%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (997)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/37379561">Frontotemporal dementia: Past, present, and future.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ulugut H,
Pijnenburg YAL</span><br />
<span class="medgenPMjournal">Alzheimers Dement</span>
2023 Nov;19(11):5253-5263.
Epub 2023 Jun 28
doi: 10.1002/alz.13363.
<span class="bold">PMID: </span><a href="/pubmed/37379561" target="_blank">37379561</a></div>
<div class="nl"><a target="_blank" href="/pubmed/37308302">Global Prevalence and Incidence of Amyotrophic Lateral Sclerosis: A Systematic Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Wolfson C,
Gauvin DE,
Ishola F,
Oskoui M</span><br />
<span class="medgenPMjournal">Neurology</span>
2023 Aug 8;101(6):e613-e623.
Epub 2023 Jun 12
doi: 10.1212/WNL.0000000000207474.
<span class="bold">PMID: </span><a href="/pubmed/37308302" target="_blank">37308302</a><a href="/pmc/articles/PMC10424837" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30465257">Inflammation in ALS/FTD pathogenesis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">McCauley ME,
Baloh RH</span><br />
<span class="medgenPMjournal">Acta Neuropathol</span>
2019 May;137(5):715-730.
Epub 2018 Nov 21
doi: 10.1007/s00401-018-1933-9.
<span class="bold">PMID: </span><a href="/pubmed/30465257" target="_blank">30465257</a><a href="/pmc/articles/PMC6482122" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/28674982">Motoneuron Disease: Clinical.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ilieva H,
Maragakis NJ</span><br />
<span class="medgenPMjournal">Adv Neurobiol</span>
2017;15:191-210.
doi: 10.1007/978-3-319-57193-5_7.
<span class="bold">PMID: </span><a href="/pubmed/28674982" target="_blank">28674982</a></div>
<div class="nl"><a target="_blank" href="/pubmed/25531687">Frontotemporal dementia: diagnosis, deficits and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bott NT,
Radke A,
Stephens ML,
Kramer JH</span><br />
<span class="medgenPMjournal">Neurodegener Dis Manag</span>
2014;4(6):439-54.
doi: 10.2217/nmt.14.34.
<span class="bold">PMID: </span><a href="/pubmed/25531687" target="_blank">25531687</a><a href="/pmc/articles/PMC4824317" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia%22%20AND%20Prognosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (2072)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/37379561">Frontotemporal dementia: Past, present, and future.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ulugut H,
Pijnenburg YAL</span><br />
<span class="medgenPMjournal">Alzheimers Dement</span>
2023 Nov;19(11):5253-5263.
Epub 2023 Jun 28
doi: 10.1002/alz.13363.
<span class="bold">PMID: </span><a href="/pubmed/37379561" target="_blank">37379561</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35678399">Behavioral Variant Frontotemporal Dementia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Boeve BF</span><br />
<span class="medgenPMjournal">Continuum (Minneap Minn)</span>
2022 Jun 1;28(3):702-725.
doi: 10.1212/CON.0000000000001105.
<span class="bold">PMID: </span><a href="/pubmed/35678399" target="_blank">35678399</a><a href="/pmc/articles/PMC9578563" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30925617">Frontotemporal Dementia: A Clinical Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sivasathiaseelan H,
Marshall CR,
Agustus JL,
Benhamou E,
Bond RL,
van Leeuwen JEP,
Hardy CJD,
Rohrer JD,
Warren JD</span><br />
<span class="medgenPMjournal">Semin Neurol</span>
2019 Apr;39(2):251-263.
Epub 2019 Mar 29
doi: 10.1055/s-0039-1683379.
<span class="bold">PMID: </span><a href="/pubmed/30925617" target="_blank">30925617</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30465257">Inflammation in ALS/FTD pathogenesis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">McCauley ME,
Baloh RH</span><br />
<span class="medgenPMjournal">Acta Neuropathol</span>
2019 May;137(5):715-730.
Epub 2018 Nov 21
doi: 10.1007/s00401-018-1933-9.
<span class="bold">PMID: </span><a href="/pubmed/30465257" target="_blank">30465257</a><a href="/pmc/articles/PMC6482122" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/25531687">Frontotemporal dementia: diagnosis, deficits and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Bott NT,
Radke A,
Stephens ML,
Kramer JH</span><br />
<span class="medgenPMjournal">Neurodegener Dis Manag</span>
2014;4(6):439-54.
doi: 10.2217/nmt.14.34.
<span class="bold">PMID: </span><a href="/pubmed/25531687" target="_blank">25531687</a><a href="/pmc/articles/PMC4824317" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (3559)</a></div></div>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_systematic_reviews">Recent systematic reviews</h1><a sid="104" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">
<div class="nl"><a target="_blank" href="/pubmed/37308302">Global Prevalence and Incidence of Amyotrophic Lateral Sclerosis: A Systematic Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Wolfson C,
Gauvin DE,
Ishola F,
Oskoui M</span><br />
<span class="medgenPMjournal">Neurology</span>
2023 Aug 8;101(6):e613-e623.
Epub 2023 Jun 12
doi: 10.1212/WNL.0000000000207474.
<span class="bold">PMID: </span><a href="/pubmed/37308302" target="_blank">37308302</a><a href="/pmc/articles/PMC10424837" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35366021">Sleep apnea and the risk of dementia: A systematic review and meta-analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Guay-Gagnon M,
Vat S,
Forget MF,
Tremblay-Gravel M,
Ducharme S,
Nguyen QD,
Desmarais P</span><br />
<span class="medgenPMjournal">J Sleep Res</span>
2022 Oct;31(5):e13589.
Epub 2022 Apr 2
doi: 10.1111/jsr.13589.
<span class="bold">PMID: </span><a href="/pubmed/35366021" target="_blank">35366021</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34313331">Mini-Mental State Examination (MMSE) for the early detection of dementia in people with mild cognitive impairment (MCI).</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Arevalo-Rodriguez I,
Smailagic N,
Roqué-Figuls M,
Ciapponi A,
Sanchez-Perez E,
Giannakou A,
Pedraza OL,
Bonfill Cosp X,
Cullum S</span><br />
<span class="medgenPMjournal">Cochrane Database Syst Rev</span>
2021 Jul 27;7(7):CD010783.
doi: 10.1002/14651858.CD010783.pub3.
<span class="bold">PMID: </span><a href="/pubmed/34313331" target="_blank">34313331</a><a href="/pmc/articles/PMC8406467" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30891742">Memantine for dementia.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">McShane R,
Westby MJ,
Roberts E,
Minakaran N,
Schneider L,
Farrimond LE,
Maayan N,
Ware J,
Debarros J</span><br />
<span class="medgenPMjournal">Cochrane Database Syst Rev</span>
2019 Mar 20;3(3):CD003154.
doi: 10.1002/14651858.CD003154.pub6.
<span class="bold">PMID: </span><a href="/pubmed/30891742" target="_blank">30891742</a><a href="/pmc/articles/PMC6425228" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26760674">Mini-Mental State Examination (MMSE) for the detection of dementia in clinically unevaluated people aged 65 and over in community and primary care populations.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Creavin ST,
Wisniewski S,
Noel-Storr AH,
Trevelyan CM,
Hampton T,
Rayment D,
Thom VM,
Nash KJ,
Elhamoui H,
Milligan R,
Patel AS,
Tsivos DV,
Wing T,
Phillips E,
Kellman SM,
Shackleton HL,
Singleton GF,
Neale BE,
Watton ME,
Cullum S</span><br />
<span class="medgenPMjournal">Cochrane Database Syst Rev</span>
2016 Jan 13;2016(1):CD011145.
doi: 10.1002/14651858.CD011145.pub2.
<span class="bold">PMID: </span><a href="/pubmed/26760674" target="_blank">26760674</a><a href="/pmc/articles/PMC8812342" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (195)</a></div></div>
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<div class="portlet_content ln"><ul><li><a href="/gtr/tests?term=C5436882%5bDISCUI%5d&amp;filter=method%3A2%5F8" target="_blank">Deletion/duplication analysis (3)</a></li>
<li><a href="/gtr/tests?term=C5436882%5bDISCUI%5d&amp;filter=method%3A2%5F7" target="_blank">Sequence analysis of the entire coding region (1)</a></li>
<li class="portletSeeAll portletSeeAllPad"><total><a href="/gtr/tests?term=C5436882%5bDISCUI%5d" target="_blank">See all (3)</a></total></li>
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<div class="portlet_content ln"><ul><li><a href="https://www.omim.org/search?index=entry&amp;start=1&amp;limit=10&amp;sort=score%20desc&amp;field=number&amp;search=619133" target="_blank">OMIM</a></li><li><a href="https://clinicaltrials.gov/search?cond=Amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia" target="_blank">ClinicalTrials.gov</a></li></ul></div>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Practice_guidelines">Practice guidelines</h1><a sid="121" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><ul class="a_poppers"><li><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">PubMed</a><div class="help-popup">See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li></ul></div>
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<div class="portlet_content ln"><ul><li><a href="https://www.malacards.org/card/amyotrophic_lateral_sclerosis_26_with_or_without_frontotemporal_dementia" target="_blank">MalaCards</a></li><li><a href="https://vsearch.nlm.nih.gov/vivisimo/cgi-bin/query-meta?v:project=medlineplus&amp;query=Amyotrophic%20lateral%20sclerosis%2026%20with%20or%20without%20frontotemporal%20dementia" target="_blank">MedlinePlus</a></li><li><a href="https://rarediseases.info.nih.gov/diseases/16425/disease" target="_blank">NCATS Office of Rare Diseases Research (GARD)</a></li></ul></div>
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<a class="brieflinkpopperctrl" href="/gene?LinkName=medgen_gene_diseases&amp;from_uid=1771903" ref="log$=recordlinks">Gene</a>
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<a class="brieflinkpopperctrl" href="/gtr/tests?term=C5436882[DISCUI]" ref="log$=recordlinks">GTR</a>
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<a class="brieflinkpopperctrl" href="/omim?LinkName=medgen_omim_gene&amp;from_uid=1771903" ref="log$=recordlinks">OMIM(Genes)</a>
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