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Dementia

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

Overview

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

Classification

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

 
 
 
 
 
 
 
 
 
 
 
 
 
Dementia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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

Causes

Dementia may be caused by:

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


Overview

There are several theories behind the formation of senile plaques and neurofibrillary tangles. The amyloid hypothesis is currently the most commonly accepted explanation.

A second theory is that Alzheimer disease (AD) is caused by an abnormal aggregation of the tau protein, a microtubule-associated protein that stabilizes microtubules in the cell. Dementia is caused by a neurodegenerative disease .These produce overlapping clinical syndromes. Less common neurodegenerative disorders such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), multisystem atrophy, and Huntington disease can also be associated with dementia[1]

Causes

Common causes include:

  1. Alcoholism
  2. Alzheimer’s Disease
  3. Binswanger’s Disease
  4. Drugs
  5. Multi-infarct disease
  6. Parkinson’s Disease
  7. Smoking
  8. Midlife obesity

There several other causes of dementia mentioned in table below :

Cardiovascular Chronic heart failure, Dural arteriovenous fistula,Subdural hematoma, Vascular dementia, Feigenbaum-Bergeron-Richardson syndrome
Chemical / poisoning Aluminium poisoning , Benzene , Camphor , Carbon Tetrachloride , Cidofovir, Cocaine, Hydrogen Sulfide, Lead poisoning, Manganese poisoning, Marijuana abuse, Mercury poisoning, Methanol , Toluene , Xylene
Dermatologic Dykes-Markes-Harper syndrome
Drug Side Effect Amphetamines, Anticholinergics, Anxiolytics, Barbiturates, Bromides, Conjugated estrogens/bazedoxifene, Estropipate, Hypnotics, Nabilone, Pramipexole, Progesterone, Sedatives, Tranquilizers, Tricyclic antidepressants
Ear Nose Throat No underlying causes
Endocrine Alcohol-induced pseudo-Cushing syndrome , Cushing’s syndrome, Hashimoto’s encephalitis , Schilder’s Disease , Hypothyroidism, Hyperparathyroidism, Hypoparathyroidism, Hyperthyroidism
Environmental Decompression sickness
Gastroenterologic Aguecheek disease , Dykes-Markes-Harper syndrome , Liver failure, Wilson’s disease
Genetic Aceruloplasminemia , Adult Polyglucosan Body Disease , Alexander Syndrome , Alpers Syndrome ,Amelo-cerebro-hypohidrotic syndrome , Batten Disease , CADASIL , Down syndrome , Familial histiocytic reticulosis, Hemochromatosis, Menke disease, Mohr-Tranebjaerg syndrome, Wilson’s disease, Wolfram’s disease ,Wright dick syndrome
Hematologic Familial histiocytic reticulosis, Macrocytosis, Pernicious anemia
Iatrogenic Aluminium (dialysis related) toxicity
Infectious Disease Acute Bokhoror , AIDS, Borreliosis, HHV-6 encephalitis, HIV, Intracranial abscess / granuloma, Kuru, Lyme disease , Neurosyphilis , Whipple’s disease
Musculoskeletal / Ortho No underlying causes
Neurologic Adult Polyglucosan Body Disease , Akinetic-rigid syndromes, Alexander Syndrome , Alpers Syndrome ,Alzheimer disease , Amelo-cerebro-hypohidrotic syndrome , Amyloidosis, Amyotrophic lateral sclerosis, Autoimmune limbic encephalitis , Batten Disease , Binswanger Disease , Bovine spongiform encephalopathy , Brain tumor,CADASIL , Cerebellar ataxias, Cerebral Atrophy , Cerebrotendinous Xanthomatosus , Cerebrovascular accident,Ceroid lipofuscinosis, Childhood disintegrative disorder, Choreoacanthocytosis amyotrophic , Creutzfeldt-Jakob disease, Dural arteriovenous fistula, Ependymoma , Fahr’s Syndrome , Feigenbaum-Bergeron-Richardson syndrome ,Gerstmann-Straussler-Scheinker disease, Giant axonal neuropathy , Gliomatosis cerebri , Guam disease ,Hallervorden-Spatz Syndrome , HARP syndrome, Hartnup’s disease, Haw River syndrome, Heidenhain syndrome ,Huntington’s disease, Hydrocephalus, Intracranial space-occupying lesion, Jensen syndrome, Juvenile pilocytic astrocytoma , Lafora disease , Leukoencephalopathy , Low press hydrocephalus, Marchiafava-Bignami disease ,MELAS , Mesulam syndrome, Metachromatic Leukodystrophy , Mitochondrial encephalopathies, Mohr-Tranebjaerg syndrome, Multiple sclerosis, Nasu-Hakola disease, Neuroaxonal dystrophy, Neuroferritinopathy, Neurosarcoidosis, Normal pressure hydrocephalus , Parkinson’s disease, Pick’s disease , Prion diseases, Rasmussen encephalitis, Schilder’s Disease , Spinocerebellar ataxia, Shy-Drager Syndrome, Steel-Richardson Syndrome,Spongiform encephalopathy , Stroke, Subdural hematoma, Tome-Brune-Fardeau syndrome , Tuberous sclerosis, Van Bogaert disease , Van der Knaap disease, Westphal-Leyden ataxia , Wright dick syndrome
Nutritional / Metabolic Aceruloplasminemia , Acute intermittent porphyrias, Cerebrotendinous Xanthomatosus , Ceroid lipofuscinosis, Chronic hypocalcemia, Chronic hypoglycemia , Decreased folate , Lesch-Nyhan syndrome, Lysosomal storage diseases, MELAS , Menke disease, Mucopolysaccharidosis, Niacin deficiency, Niemann-Pick Disease,Nutritional deficiency ,Pellagra, Siderosis , Tay Sachs , Thiamine deficiency, Uremic encephalopathy ,Vitamin B12 deficiency, Vitamin B6 deficiency, Wernicke-Korsakoff syndrome
Obstetric/Gynecologic Postpartum thyroiditis
Oncologic Brain tumor, Ependymoma , Juvenile pilocytic astrocytoma , Melanoma , Meningioma,Paraneoplastic syndromes
Opthalmologic No underlying causes
Overdose / Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary No underlying causes
Renal / Electrolyte Kidney failure
Rheum / Immune / Allergy Autoimmune limbic encephalitis , Sarcoid, SLE, Tuberous sclerosis, Vasculitis
Sexual No underlying causes
Trauma Head injury
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Alcohol Withdrawal , Alcoholism , Glue sniffing, Prion diseases, Punch-drunk syndrome, Post anoxia

Causes in Alphabetical Order


Mnemonics to remember causes

Dementia: treatable causes

DEMENTIA:

  • Drug toxicity
  • Emotional (depression, anxiety, OCD, etc.)
  • Metabolic (electrolytes, liver dz, kidney dz, COPD)
  • Eyes/ Ears (peripheral sensory restrictions)
  • Nutrition (vitamin, iron deficiencies/ NPH [Normal Pressure Hydrocephalus]
  • Tumors/ Trauma (including chronic subdural hematoma)
  • Infection (meningitis, encephalitis, pneumonia, syphilis)
  • Arteriosclerosis and other vascular disease

Dementia: some common causes

DEMENTIA:

  • Diabetes
  • Ethanol
  • Medication
  • Environmental (eg CO poisoning)
  • Nutritional
  • Trauma
  • Infection
  • Alzheimer’s[2]

Dementia: reversible dementia causes

DEMENTIA:

References

  1. ↑ Clarfield AM (September 1988). “The reversible dementias: do they reverse?”. Ann Intern Med. 109 (6): 476–86. doi:10.7326/0003-4819-109-6-476. PMID 3046450.
  2. ↑ Morris JC (2003). “Dementia update 2003”. Alzheimer Dis Assoc Disord. 17 (4): 245–58. doi:10.1097/00002093-200310000-00010. PMID 14657790.
  3. ↑ Caselli RJ (September 2003). “Current issues in the diagnosis and management of dementia”. Semin Neurol. 23 (3): 231–40. doi:10.1055/s-2003-814743. PMID 14722819.
  4. ↑ Knopman DS, Boeve BF, Petersen RC (October 2003). “Essentials of the proper diagnoses of mild cognitive impairment, dementia, and major subtypes of dementia”. Mayo Clin. Proc. 78 (10): 1290–308. doi:10.4065/78.10.1290. PMID 14531488.

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Differential Diagnosis

Differential Diagnosis

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
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 +++ ++ – ++ +++ ++ ++ ++ Has the following clinical stages:
Lewy body dementia ++ – Helpful +++ + + +++ + +++ +++ +++
Frontotemporal lobar degeneration +/- – Helpful +++ + – +++ +++ ++ +++ –
  • Onset in young age
Vascular dementia + (Dysexecutive syndrome) – Helpful + + + ++ – ++ +++ +
Normal pressure hydrocephalus
Parkinson’s disease
Binswanger’s disease
Thiamine deficiency
Marijuana abuse
AIDS
Neurosyphilis
Vitamin B12 deficiency
Vitamin B6 deficiency

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References

References

References

  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.
  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.

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