Dementia

| Resident
Survival
Guide

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Dementia Microchapters

Patient Information

Overview

Classification

Causes

* Alzheimer's Disease
* Parkinson's Disease
* Binswanger's Disease
* Dementia with Lewy bodies
* Thiamine deficiency
* Vascular dementia
* Marijuana abuse
* AIDS
* Neurosyphilis
* Normal pressure hydrocephalus
* Vitamin B12 deficiency
* Vitamin B6 deficiency

Differential Diagnosis

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

Synonyms and keywords: dementia

## Overview

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Dementia is an acquired cognitive impairment in different cognitive areas such as attention, learning, memory, language, executive function, and motor function. Dementia may be classified according to the etiology into 5 groups including neurodegenerative, vascular, infectious, drug-related, and metabolic. The most common causes of dementia include alzheimer's Disease, parkinson's Disease, binswanger's Disease, dementia with Lewy bodies, frontotemporal lobar degeneration,thiamine deficiency,vitamin B12 deficiency, vitamin B6 deficiency, vascular dementia, marijuana abuse, AIDS neurosyphilis, and normal pressure hydrocephalus.

## Classification

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Dementia may be classified according to the etiology into 5 groups including neurodegenerative, vascular, infectious, drug-related, and metabolic.

| Dementia|

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| Neurodegenerative|

| Vascular|

| Infectious|

| Drug related|

| Metabolic|

| •Alzheimer's Disease
•Parkinson's Disease
•Binswanger's Disease
•Dementia with Lewy bodies
•Frontotemporal lobar degeneration|

| •Vascular dementia|

| •AIDS
•Neurosyphilis|

| •Marijuana abuse|

| •Thiamine deficiency
•Vitamin B12 deficiency
•Vitamin B6 deficiency|

## Causes

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Dementia may be caused by:

* Alzheimer's Disease
* Parkinson's Disease
* Binswanger's Disease
* Dementia with Lewy bodies
* Frontotemporal lobar degeneration
* Thiamine deficiency
* Vitamin B12 deficiency
* Vitamin B6 deficiency
* Vascular dementia
* Marijuana abuse
* AIDS
* Neurosyphilis
* Normal pressure hydrocephalus

## Differential Diagnosis

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Dementia causes must be differentiated from each other: [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]

Cause of dementia | Clinical features  | Associated features  | Nature of progression  | Histopathological findings

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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 the 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

Normal pressure hydrocephalus |  |  |  |  |  |  |  |  |  |  |  |  |  |
Parkinson's disease |  |  |  |  |  |  |  |  |  |  |  |  |  |
Binswanger's disease |  |  |  |  |  |  |  |  |  |  |  |  |  |
Thiamine deficiency |  |  |  |  |  |  |  |  |  |  |  |  |  |
Marijuana abuse |  |  |  |  |  |  |  |  |  |  |  |  |  |
AIDS |  |  |  |  |  |  |  |  |  |  |  |  |  |
Neurosyphilis |  |  |  |  |  |  |  |  |  |  |  |  |  |
Vitamin B12 deficiency |  |  |  |  |  |  |  |  |  |  |  |  |  |
Vitamin B6 deficiency |  |  |  |  |  |  |  |  |  |  |  |  |  |

## References

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1. ↑ Jellinger KA (2008). "The pathology of "vascular dementia": a critical update". J. Alzheimers Dis. 14 (1): 107–23. PMID 18525132.
2. ↑ Murayama S (2008). "[Neuropathology of frontotemporal dementia]". Rinsho Shinkeigaku (in Japanese). 48 (11): 998. PMID 19198143.CS1 maint: Unrecognized language (link)
3. ↑ 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.
4. ↑ 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.
5. ↑ "Dementia, Globalization and Contemporary Art".
6. ↑ 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.
7. ↑ 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.
8. ↑ 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.
9. ↑ 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.
10. ↑ Uversky VN (2008). "Alpha-synuclein misfolding and neurodegenerative diseases". Curr. Protein Pept. Sci. 9 (5): 507–40. PMID 18855701.
11. ↑ 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.
12. ↑ Brion JP (1998). "Neurofibrillary tangles and Alzheimer's disease". Eur. Neurol. 40 (3): 130–40. PMID 9748670.
13. ↑ 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.
14. ↑ 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.
15. ↑ 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.
16. ↑ 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.