Frontotemporal lobar degeneration: Difference between revisions

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List of terms related to Frontotemporal lobar degeneration

	Frontotemporal lobar degeneration;
	A human brain showing frontotemporal lobar degeneration causing frontotemporal dementia.
OMIM	600274
DiseasesDB	10034
MeSH	D003704

VisualWikitext

Revision as of 22:49, 23 September 2017

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Synonyms and keywords: Disinhibition-dementia-parkinsonism-amyotrophy complex; ddpac; frontotemporal dementia-amyotrophic lateral sclerosis; ftd-als pallidopontonigral degeneration; ppnd FTLD; frontotemporal lobar degeneration with tau inclusions; ftld with tau inclusions; frontotemporal dementia with parkinsonism; frontotemporal lobe dementia; fldem; ftdp17; multiple system tauopathy with presenile dementia; mstd;

Overview

Frontotemporal lobar degeneration (FTLD) is a pathologic process involving degeneration of gray matter in the frontal lobe and anterior portion of the temporal lobe of the cerebrum, with sparing of the parietal and occipital lobes.

Pathophysiology

The gross finding of frontotemporal lobar degeneration is likely produced by several etiologically distinct processes, with unique genetic and histopathological findings. Accordingly, there are a number of possible histopathological findings at post-mortem:

tau inclusions (either with Pick bodies or without)
ubiquitin positive (tau-negative) inclusions - in the majority of cases that have this type of pathology the ubiquitinated inclusions contain a protein called TDP-43. There are three subtypes of this type of pathology. Mackenzie et al (and subsequently Davidson et al) describe the following: type 1 with intranuclear inclusions; type 2 with neurites predominantly and type 3 with cytoplasmic inclusions predominantly. It should be noted that not all ubiquitin-positive, tau negative cases stain for TDP-43 e.g. the CHMP2B cases but also other cases.
Dementia lacking distinctive histology (DLDH) - A rare and controversial entity - new analyses have allowed many cases to be reclassified into one of the positively-defined subgroups.

Genetics

Many cases (possibly up to 50%) of FTLD are genetic rather than sporadic. Mutations in the Tau gene (on chromosome 17q21 - known as MAPT or Microtubule Associated Protein Tau) can cause FTLD and there are over 40 known mutations at present. A series of new mutations associated with FTLD has been recently described in the progranulin gene which is remarkably also on chromosome 17q21. Patients with progranulin mutations have type 1 TDP-43 positive, tau negative pathology at post-mortem. Progranulin is associated with tumorgenesis when overproduced, whereas the mutations seen in the progranulin gene associated with FTLD suggests a deficit in progranulin may be the problem. There are currently 2 other known genes that can cause FTLD: CHMP2B (on chromosome 3) which is associated with a behavioural syndrome (mainly in a large Jutland cohort); and VCP (valosin-containing protein, on chromosome 9) which is associated with the IBMPFD syndrome (inclusion body myopathy, Paget's disease and frontotemporal dementia). These 2 genes only account for a tiny proportion of cases. A locus on chromosome 9 is associated with FTD-MND (or FTD-ALS) i.e. frontotemporal dementia associated with motor neurone disease (or amyotrophic lateral sclerosis) - the hunt for this gene is currently the focus of a number of research labs around the world.

Differential Diagnosis Of Major or Mild Frontotemporal Neurocognitive Disorder

Other neurocognitive disorders

Other neurological conditions

Other mental disorders and medical conditions

The following table outlines the main findings of the diseases which must be differentiated from dementia with frontotemporal lobar degeneration as they may share common characteristics of cognitive impairment:^[2]^[3]^[4]^[5]^[6]^[7]^[8]^[9]^[10]^[11]^[12]^[13]^[14]^[15]^[16]^[17]


Cause of dementia	Clinical features											Associated features	Nature of progression	Histopathological findings
Cause of dementia	Cognitive impairment
	Recall	Recollection	Cue requirement for recall	Infirngement of thoughts	Semantic memory	Procedural memory	Working memory	Awareness	Attention	Executive functioning issues	Visuo-spatial skills
Alzheimer's disease	+++ (Slow cognitive and functional decline with early loss of awareness)	+++	Not helpful	+++	++	-	++	+++	++	++	++	Focal neurological signs absent during early stage (may develop later) Mild extrapyramidal signs	Has the following clinical stages: Pre-clinical Mild cognitive impairment Advanced dementia (full blown dementia)	Neurofibrillary tangles Senile (neuritic) plaques
Lewy body dementia	++	-	Helpful	+++	+	+	+++	+	+++	+++	+++	Focal neurological signs present in majority Strongly associated with extrapyramidal signs Autonomic changes	Early visual hallucinations Muscle rigidity Sleep disturbance	α-synuclein deposits present in neurons
Frontotemporal lobar degeneration	+/-	-	Helpful	+++	+	-	+++	+++	++	+++	-	Focal neurological signs present (motor neuron disease) Extrapyramidal signs present (familial frontotemporal lobar degeneration) Onset in young age Strong familial association Focal atrophy of frontal and temporal lobes Knife-edge atrophy noted on MRI Personality and behavioral changes Language impairment	Two major variants: Progressive nonfluent aphasia Semantic variant Progressive supranuclear palsy	Pick bodies Corticobasal degeneration Ubiquitinated inclusions
Vascular dementia	+ (Dysexecutive syndrome)	-	Helpful	+	+	+	++	-	++	+++	+	Focal neurological signs present typically Mild extrapyramidal signs Depression Mild motor signs in subcortical vascular dementia Slowed processing speed Presentation might be similar to Alzheimer's disease	Stepwise progression and focal neurologic signs (also known as multi-infarct dementia or poststroke dementia)	Multifocal and/or diffuse lesions (lacunes and microinfarcts) The following brain areas may be involved: Subcortical area Thalamus Frontobasallimbic system White matter lesions Hippocampal sclerosis Diffuse post-ischemic lesions

Epidemiology and Demographics Of Major or Mild Frontotemporal Neurocognitive Disorder

Prevalence

The prevalence of major or mild frontotemporal neurocognitive disorder is 2,000-10,000 per 100,000 (2%-10%) of the overall population.^[1]

Age

In the over 65 age group, FTLD is probably the fourth most common cause of dementia after Alzheimer's disease, Dementia with Lewy bodies and vascular dementia. In the below 65 age group, it is the second most common cause after Alzheimer's disease. The process of FTLD is thought to be the main cause of three clinical syndromes: frontotemporal dementia, semantic dementia, and progressive nonfluent aphasia.

Risk Factors Of Major or Mild Frontotemporal Neurocognitive Disorder

Genetic predisposition
Motor neuron disease^[1]

Diagnosis

Symptoms

Physical Examination

Neuro

Ocular motility abnormalities
Pyramidal tract dysfunction
Frontal lobe release signs
Perseverative vocalizations
Bradykinesia
Postural instability

DSM-V Diagnostic Criteria for Major Or Mild Frontotemporal Neurocognitive Disorder^[1]

“

A.The criteria are met for major or mild neurocognitive disorder.

AND

B.The disturbance has insidious onset and gradual progression.

AND

C.Either (1) or (2);

1.Behavioral variant;

a.Three or more of the following behavioral symptoms:

i.Behavioral disinhibition.
ii.Apathy or inertia.
iii.Loss of sympathy or empathy.
iv.Perseverative, stereotyped or compulsive/ritualistic behavior.
v.Hyperorality and dietary changes.

b.Prominent decline in social cognition and/or executive abilities.

2.Language variant:

a.Prominent decline in language ability, in the form of speech production, word finding, object naming, grammar, or word comprehension.

AND

D.Relative sparing of learning and memory and perceptual-motor function.

AND

E.The disturbance is not better explained by cerebrovascular disease, another neurodegenerative disease, the effects of a substance, or another mental, neurological, or systemic disorder.

Probable frontotemporal neurocognitive disorderis diagnosed if either of the following is present; otherwise, possible frontotemporal neurocognitive disorder should be diagnosed:

1.Evidence of a causative frontotemporal neurocognitive disorder genetic mutation, from either family history or genetic testing.

2.Evidence of disproportionate frontal and/or temporal lobe involvement from neuro imaging.

Possible frontotemporal neurocognitive disorderis diagnosed if there is no evidence of a genetic mutation, and neuro imaging has not been performed.

”

References

↑ ^1.0 ^1.1 ^1.2 ^1.3 Diagnostic and statistical manual of mental disorders : DSM-5. Washington, D.C: American Psychiatric Association. 2013. ISBN 0890425558.
↑ Jellinger KA (2008). "The pathology of "vascular dementia": a critical update". J. Alzheimers Dis. 14 (1): 107–23. PMID 18525132.
↑ Murayama S (2008). "[Neuropathology of frontotemporal dementia]". Rinsho Shinkeigaku (in Japanese). 48 (11): 998. PMID 19198143.
↑ Hodges JR, Patterson K (1996). "Nonfluent progressive aphasia and semantic dementia: a comparative neuropsychological study". J Int Neuropsychol Soc. 2 (6): 511–24. PMID 9375155.
↑ Hodges JR, Patterson K, Oxbury S, Funnell E (1992). "Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy". Brain. 115 ( Pt 6): 1783–806. PMID 1486461.
↑ "Dementia, Globalization and Contemporary Art".
↑ Helkala EL, Laulumaa V, Soininen H, Riekkinen PJ (1988). "Recall and recognition memory in patients with Alzheimer's and Parkinson's diseases". Ann. Neurol. 24 (2): 214–7. doi:10.1002/ana.410240207. PMID 3178177.
↑ Weintraub S, Wicklund AH, Salmon DP (2012). "The neuropsychological profile of Alzheimer disease". Cold Spring Harb Perspect Med. 2 (4): a006171. doi:10.1101/cshperspect.a006171. PMC 3312395. PMID 22474609.
↑ Goldman JG, Williams-Gray C, Barker RA, Duda JE, Galvin JE (2014). "The spectrum of cognitive impairment in Lewy body diseases". Mov. Disord. 29 (5): 608–21. doi:10.1002/mds.25866. PMC 4126402. PMID 24757110.
↑ Metzler-Baddeley C (2007). "A review of cognitive impairments in dementia with Lewy bodies relative to Alzheimer's disease and Parkinson's disease with dementia". Cortex. 43 (5): 583–600. PMID 17715794.
↑ Uversky VN (2008). "Alpha-synuclein misfolding and neurodegenerative diseases". Curr. Protein Pept. Sci. 9 (5): 507–40. PMID 18855701.
↑ Bennett DA, Schneider JA, Wilson RS, Bienias JL, Arnold SE (2004). "Neurofibrillary tangles mediate the association of amyloid load with clinical Alzheimer disease and level of cognitive function". Arch. Neurol. 61 (3): 378–84. doi:10.1001/archneur.61.3.378. PMID 15023815.
↑ Brion JP (1998). "Neurofibrillary tangles and Alzheimer's disease". Eur. Neurol. 40 (3): 130–40. PMID 9748670.
↑ Lee JS, Jung NY, Jang YK, Kim HJ, Seo SW, Lee J, Kim YJ, Lee JH, Kim BC, Park KW, Yoon SJ, Jeong JH, Kim SY, Kim SH, Kim EJ, Park KC, Knopman DS, Na DL (2017). "Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy". J Clin Neurol. 13 (3): 234–242. doi:10.3988/jcn.2017.13.3.234. PMC 5532319. PMID 28748674.
↑ Pao WC, Dickson DW, Crook JE, Finch NA, Rademakers R, Graff-Radford NR (2011). "Hippocampal sclerosis in the elderly: genetic and pathologic findings, some mimicking Alzheimer disease clinically". Alzheimer Dis Assoc Disord. 25 (4): 364–8. doi:10.1097/WAD.0b013e31820f8f50. PMC 3107353. PMID 21346515.
↑ Tsolaki M, Kokarida K, Iakovidou V, Stilopoulos E, Meimaris J, Kazis A (2001). "Extrapyramidal symptoms and signs in Alzheimer's disease: prevalence and correlation with the first symptom". Am J Alzheimers Dis Other Demen. 16 (5): 268–78. doi:10.1177/153331750101600512. PMID 11603162.
↑ McGuinness B, Barrett SL, Craig D, Lawson J, Passmore AP (2010). "Executive functioning in Alzheimer's disease and vascular dementia". Int J Geriatr Psychiatry. 25 (6): 562–8. doi:10.1002/gps.2375. PMID 19810010.

@@ Line 43: / Line 43: @@
 :*[[Major depression]]
 :*[[Schizophrenia]]<ref name=DSMV>{{cite book | title = Diagnostic and statistical manual of mental disorders : DSM-5 | publisher = American Psychiatric Association | location = Washington, D.C | year = 2013 | isbn = 0890425558 }}</ref>
+* The following table outlines the main findings of the diseases which must be differentiated from dementia with frontotemporal lobar degeneration as they may share common characteristics of [[cognitive impairment]]:<ref name="pmid18525132">{{cite journal |vauthors=Jellinger KA |title=The pathology of "vascular dementia": a critical update |journal=J. Alzheimers Dis. |volume=14 |issue=1 |pages=107–23 |year=2008 |pmid=18525132 |doi= |url=}}</ref><ref name="pmid19198143">{{cite journal |vauthors=Murayama S |title=[Neuropathology of frontotemporal dementia] |language=Japanese |journal=Rinsho Shinkeigaku |volume=48 |issue=11 |pages=998 |year=2008 |pmid=19198143 |doi= |url=}}</ref><ref name="pmid9375155">{{cite journal |vauthors=Hodges JR, Patterson K |title=Nonfluent progressive aphasia and semantic dementia: a comparative neuropsychological study |journal=J Int Neuropsychol Soc |volume=2 |issue=6 |pages=511–24 |year=1996 |pmid=9375155 |doi= |url=}}</ref><ref name="pmid1486461">{{cite journal |vauthors=Hodges JR, Patterson K, Oxbury S, Funnell E |title=Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy |journal=Brain |volume=115 ( Pt 6) |issue= |pages=1783–806 |year=1992 |pmid=1486461 |doi= |url=}}</ref><ref name="urlDementia, Globalization and Contemporary Art">{{cite web |url=https://kohlibri.e-bookshelf.de/products/reading-epub/product-id/4033550/title/Dementia.html |title=Dementia, Globalization and Contemporary Art |format= |work= |accessdate=}}</ref><ref name="pmid3178177">{{cite journal |vauthors=Helkala EL, Laulumaa V, Soininen H, Riekkinen PJ |title=Recall and recognition memory in patients with Alzheimer's and Parkinson's diseases |journal=Ann. Neurol. |volume=24 |issue=2 |pages=214–7 |year=1988 |pmid=3178177 |doi=10.1002/ana.410240207 |url=}}</ref><ref name="pmid22474609">{{cite journal |vauthors=Weintraub S, Wicklund AH, Salmon DP |title=The neuropsychological profile of Alzheimer disease |journal=Cold Spring Harb Perspect Med |volume=2 |issue=4 |pages=a006171 |year=2012 |pmid=22474609 |pmc=3312395 |doi=10.1101/cshperspect.a006171 |url=}}</ref><ref name="pmid24757110">{{cite journal |vauthors=Goldman JG, Williams-Gray C, Barker RA, Duda JE, Galvin JE |title=The spectrum of cognitive impairment in Lewy body diseases |journal=Mov. Disord. |volume=29 |issue=5 |pages=608–21 |year=2014 |pmid=24757110 |pmc=4126402 |doi=10.1002/mds.25866 |url=}}</ref><ref name="pmid17715794">{{cite journal |vauthors=Metzler-Baddeley C |title=A review of cognitive impairments in dementia with Lewy bodies relative to Alzheimer's disease and Parkinson's disease with dementia |journal=Cortex |volume=43 |issue=5 |pages=583–600 |year=2007 |pmid=17715794 |doi= |url=}}</ref><ref name="pmid18855701">{{cite journal |vauthors=Uversky VN |title=Alpha-synuclein misfolding and neurodegenerative diseases |journal=Curr. Protein Pept. Sci. |volume=9 |issue=5 |pages=507–40 |year=2008 |pmid=18855701 |doi= |url=}}</ref><ref name="pmid15023815">{{cite journal |vauthors=Bennett DA, Schneider JA, Wilson RS, Bienias JL, Arnold SE |title=Neurofibrillary tangles mediate the association of amyloid load with clinical Alzheimer disease and level of cognitive function |journal=Arch. Neurol. |volume=61 |issue=3 |pages=378–84 |year=2004 |pmid=15023815 |doi=10.1001/archneur.61.3.378 |url=}}</ref><ref name="pmid9748670">{{cite journal |vauthors=Brion JP |title=Neurofibrillary tangles and Alzheimer's disease |journal=Eur. Neurol. |volume=40 |issue=3 |pages=130–40 |year=1998 |pmid=9748670 |doi= |url=}}</ref><ref name="pmid28748674">{{cite journal |vauthors=Lee JS, Jung NY, Jang YK, Kim HJ, Seo SW, Lee J, Kim YJ, Lee JH, Kim BC, Park KW, Yoon SJ, Jeong JH, Kim SY, Kim SH, Kim EJ, Park KC, Knopman DS, Na DL |title=Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy |journal=J Clin Neurol |volume=13 |issue=3 |pages=234–242 |year=2017 |pmid=28748674 |pmc=5532319 |doi=10.3988/jcn.2017.13.3.234 |url=}}</ref><ref name="pmid21346515">{{cite journal |vauthors=Pao WC, Dickson DW, Crook JE, Finch NA, Rademakers R, Graff-Radford NR |title=Hippocampal sclerosis in the elderly: genetic and pathologic findings, some mimicking Alzheimer disease clinically |journal=Alzheimer Dis Assoc Disord |volume=25 |issue=4 |pages=364–8 |year=2011 |pmid=21346515 |pmc=3107353 |doi=10.1097/WAD.0b013e31820f8f50 |url=}}</ref><ref name="pmid11603162">{{cite journal |vauthors=Tsolaki M, Kokarida K, Iakovidou V, Stilopoulos E, Meimaris J, Kazis A |title=Extrapyramidal symptoms and signs in Alzheimer's disease: prevalence and correlation with the first symptom |journal=Am J Alzheimers Dis Other Demen |volume=16 |issue=5 |pages=268–78 |year=2001 |pmid=11603162 |doi=10.1177/153331750101600512 |url=}}</ref><ref name="pmid19810010">{{cite journal |vauthors=McGuinness B, Barrett SL, Craig D, Lawson J, Passmore AP |title=Executive functioning in Alzheimer's disease and vascular dementia |journal=Int J Geriatr Psychiatry |volume=25 |issue=6 |pages=562–8 |year=2010 |pmid=19810010 |doi=10.1002/gps.2375 |url=}}</ref>
+{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align="center"
+|+
+! rowspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Cause of dementia}}
+! colspan="11" align="center" style="background:#4479BA; color: #FFFFFF; width: 400px;" + | Clinical features
+! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated features
+! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Nature of progression
+! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Histopathological findings
+|-
+! colspan="11" align="center" style="background:#4479BA; color: #FFFFFF;" + |Cognitive impairment
+|-
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Recall
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Recollection
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Cue requirement for recall
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Infirngement of thoughts
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Semantic memory
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Procedural memory
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Working memory
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Awareness
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Attention
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Executive functioning issues
+! align="center" style="background:#4479BA; color: #FFFFFF;" + |Visuo-spatial skills
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Alzheimer's disease]]
+| style="padding: 5px 5px; background: #F5F5F5;" | +++
+(Slow [[cognitive]] and functional decline with early loss of awareness)
+|<nowiki>+++</nowiki>
+|Not helpful
+|<nowiki>+++</nowiki>
+|<nowiki>++</nowiki>
+|<nowiki>-</nowiki>
+|<nowiki>++</nowiki>
+|<nowiki>+++</nowiki>
+|<nowiki>++</nowiki>
+|<nowiki>++</nowiki>
+|<nowiki>++</nowiki>
+|
+* [[Focal neurologic signs|Focal neurological signs]] absent during early stage (may develop later)
+* Mild [[extrapyramidal]] signs
+|Has the following clinical stages:
+* Pre-clinical
+* [[Mild cognitive impairment]]
+* Advanced [[dementia]] (full blown dementia)
+|
+* [[Neurofibrillary tangle|Neurofibrillary tangles]]
+* [[Senile plaques|Senile (neuritic) plaques]]
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |[[Lewy body dementia]]
+| style="padding: 5px 5px; background: #F5F5F5;" | ++
+|<nowiki>-</nowiki>
+|Helpful
+|<nowiki>+++</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>+++</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>+++</nowiki>
+|<nowiki>+++</nowiki>
+|<nowiki>+++</nowiki>
+|
+* Focal neurological signs present in majority
+* Strongly associated with [[extrapyramidal]] signs
+* [[Autonomic]] changes
+|
+* Early [[visual hallucinations]]
+* [[Muscle rigidity]]
+* [[Sleep disturbance]]
+|
+* [[Alpha-synuclein|α-synuclein]] deposits present in [[Neuron|neurons]]
+|-
+| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[Frontotemporal lobar degeneration]]
+| style="padding: 5px 5px; background: #F5F5F5;" | +/-
+|<nowiki>-</nowiki>
+|Helpful
+|<nowiki>+++</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>-</nowiki>
+|<nowiki>+++</nowiki>
+|<nowiki>+++</nowiki>
+|<nowiki>++</nowiki>
+|<nowiki>+++</nowiki>
+|<nowiki>-</nowiki>
+|
+* [[Focal neurologic signs|Focal neurological signs]] present ([[motor neuron disease]])
+* [[Extrapyramidal|Extrapyramidal signs]]  present (familial [[Frontotemporal lobar degenerations|frontotemporal lobar degeneration]])
+* Onset in young age
+* Strong [[familial]] association
+* Focal [[atrophy]] of [[Frontal lobe|frontal]] and [[temporal lobes]]
+* Knife-edge [[atrophy]] noted on [[Magnetic resonance imaging|MRI]]
+* [[Personality changes|Personality]] and behavioral changes
+* Language impairment
+|
+* Two major variants:
+** Progressive nonfluent [[aphasia]]
+** [[Semantic Dementia|Semantic]] variant
+* [[Progressive supranuclear palsy]]
+|
+* [[Pick bodies]]
+* [[Corticobasal degeneration]]
+* [[Ubiquitination|Ubiquitinated]] inclusions
+|-
+| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |[[Vascular dementia]]
+| style="padding: 5px 5px; background: #F5F5F5;" | + (Dysexecutive syndrome)
+|<nowiki>-</nowiki>
+|Helpful
+|<nowiki>+</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>+</nowiki>
+|<nowiki>++</nowiki>
+|<nowiki>-</nowiki>
+|<nowiki>++</nowiki>
+|<nowiki>+++</nowiki>
+|<nowiki>+</nowiki>
+|
+* [[Focal neurologic signs|Focal neurological signs]] present typically
+* Mild [[extrapyramidal]] signs
+* [[Depression]]
+* Mild [[Motor disorders|motor signs]] in subcortical [[vascular dementia]]
+* Slowed processing speed
+* Presentation might be similar to Alzheimer's disease
+|
+* Stepwise progression and focal neurologic signs (also known as [[multi-infarct dementia]] or poststroke [[dementia]])
+|
+* Multifocal and/or diffuse lesions (lacunes and microinfarcts)
+* The following [[brain]] areas may be involved:
+** [[Subcortical|Subcortical area]]
+** [[Thalamus]]
+** Frontobasal[[limbic system]]
+** [[White matter]] lesions
+** [[Hippocampal sclerosis]]
+** Diffuse post-ischemic lesions
+|}
 ==Epidemiology and Demographics Of Major or Mild Frontotemporal Neurocognitive Disorder==

Frontotemporal lobar degeneration: Difference between revisions

Revision as of 22:49, 23 September 2017

Contents

Overview

Pathophysiology

Genetics

Differential Diagnosis Of Major or Mild Frontotemporal Neurocognitive Disorder

Epidemiology and Demographics Of Major or Mild Frontotemporal Neurocognitive Disorder

Prevalence

Age

Risk Factors Of Major or Mild Frontotemporal Neurocognitive Disorder

Diagnosis

Symptoms

Physical Examination

Neuro

DSM-V Diagnostic Criteria for Major Or Mild Frontotemporal Neurocognitive Disorder^[1]

References

See also

Navigation menu


Frontotemporal lobar degeneration
A human brain showing frontotemporal lobar degeneration causing frontotemporal dementia.
OMIM	600274
DiseasesDB	10034
MeSH	D003704

Frontotemporal lobar degeneration: Difference between revisions

Revision as of 22:49, 23 September 2017

Overview

Pathophysiology

Genetics

Differential Diagnosis Of Major or Mild Frontotemporal Neurocognitive Disorder

Epidemiology and Demographics Of Major or Mild Frontotemporal Neurocognitive Disorder

Prevalence

Age

Risk Factors Of Major or Mild Frontotemporal Neurocognitive Disorder

Diagnosis

Symptoms

Physical Examination

Neuro

DSM-V Diagnostic Criteria for Major Or Mild Frontotemporal Neurocognitive Disorder[1]

References

See also

Navigation menu

DSM-V Diagnostic Criteria for Major Or Mild Frontotemporal Neurocognitive Disorder^[1]