Background

Dementia is a broad umbrella term. There is a whole range of conditions that affect the memory and thinking of an individual. This, in turn, influences their day-to-day living.

Dementia is a decline in neurocognitive function. Dementia is a progressive condition that persistently causes the deterioration of memory and thinking abilities. The loss of cognitive functioning can range from loss of thinking to loss of remembering and reasoning. It can be mild to severe, ranging from little impact on daily functioning to complete dependence on others for even basic functioning.

Frontal lobe: It controls an individual’s ability to solve problems, control emotions, and make plans and decisions. It also controls speech and language along with motor skills.

Temporal lobe- It processes language, sound and music. It is important for memory and learning. It helps in recognising people’s faces.

Occipital lobe- It is helpful for visual perception.

Parietal lobe- It controls spatial awareness, hand-eye coordination, skills such as maths and spelling, and perception of hard and soft objects.

Classification/Classifying dementia 

There are different ways to classify dementia:

1. Based on aetiology
2. Based on the progress of dementia
3. Based on how common dementia is
4. Based on Degenerative or non-degenerative
5. Based on cortical or non-cortical

Based on aetiology
Neurodegenerative disorders Cerebrovascular disorders Neurogenetic disorders Prion-associated disorders Infections Toxins Metabolic encephalopathies

Table 1: Classification of dementia based on aetiology. Dementia Pathology: Dementia, Alzheimer’s Disease, Vascular Dementia. (2020). eMedicine. [online] Available at: https://emedicine.medscape.com/article/2003174-overview#a1

Metabolic Encephalopathy – an overview | ScienceDirect Topics. [online] Available at: https://www.sciencedirect.com/topics/medicine-and-dentistry/metabolic-encephalopathy#:~:text=Metabolic%20encephalopathy%20may%20then%20be%20difficult%20to%20distinguish[Accessed 15 May 2023].

Based on the progress of dementia
Slow progressive dementia Rapid progressive dementia Fixed memory loss

Table 2: Classification of Dementia based on the progress of Dementia. Geschwind, M.D., Haman, A. and Miller, B.L. (2007). Rapidly Progressive Dementia. Neurologic Clinics, [online] 25(3), pp.783–807. doi:https://doi.org/10.1016/j.ncl.2007.04.001.

Based on Degenerative or non-degenerative
Degenerative diseases Alzheimer’s dementia Lewy Body Dementia Fronto-temporal Dementia Parkinson’s Disease Dementia Huntington’s Disease Progressive Supranuclear palsy Corticobasal Degeneration Wilsons Disease, Kearns-Sayre Disease Non-Degenerative diseases Vascular Dementia Infections- HIV, Syphilis, Lymes, tuberculosis, Meningitis, Encephalitis Prion disease- Creutzfeldt Jacobs, Gerstmann Tumours- Brain tumours Toxins- Alcohol, drugs, Heavy metals, Carbon monoxide poisoning Metabolic – Thyroid disease, Cushing’s, Vitamin deficiency Trauma- Head Injury Depression, Hydrocephalus, NPH Dementia, Multiple Sclerosis

Table 3: Classification of Dementia based on Degeneration Rone-Adams, S., Stern, D.F., Olivier, T.W. and Blodgett, M.G. (2013). Understanding Dementia. Strength and Conditioning Journal, 35(4), pp.88–98. doi:https://doi.org/10.1519/ssc.0b013e31829ed301.

Based on how common dementia is
Alzheimer’s Dementia Vascular Dementia Lewy Body dementia Frontotemporal Dementia Mixed Dementia Alcohol-related brain damage Rarer types Posterior Cortical atrophy Huntington’s Disease Parkinson’s Dementia

Table 4: Classification of Dementia based on how common Dementia is. Robinson, L., Tang, E. and Taylor, J.-P. . (2015). Dementia: timely diagnosis and early intervention. BMJ, [online] 350(jun15 14), pp.h3029–h3029. doi:https://doi.org/10.1136/bmj.h3029.

Based on Cortical vs non-Cortical
Cortical Dementia	Non-Cortical Dementia
Cortical dementia is the involvement of the outer cortex- there is generalised cortical atrophy, especially involving frontal and temporal lobes.	Subcortical grey matter is affected in the subcortical type of dementia- the lesions occur predominantly in the basal ganglia, the brainstem nuclei and the cerebellum.
Examples include Alzheimer’s dementia, Frontotemporal dementia and Creutzfeldt-Jacob.	Examples include Huntington’s, Parkinson’s and AIDS Dementia complex.
Individuals with the cortical type of dementia are generally alert and attentive, and their personality is preserved. However, they experience memory loss and can be aphasic and apraxic.	In the subcortical type of dementia, attention and executive function are both impaired, and they can be apathetic. Whilst these patients can be forgetful with poor language, they are not apraxic.
Perception and visuospatial difficulties can be noticed in both types of Dementia.

Table 5: Classification of Dementia based Cortical vs Non-Cortical. Contributors, W.E. (n.d.). What Is Dementia? [online] WebMD. Available at: https://www.webmd.com/alzheimers/types-dementia#:~:text=Alzheimer%27s%20and%20Creutzfeldt-Jakob%20disease%20are%20two%20forms%20of[Accessed 15 May 2023].

Epidemiology

The WHO has estimated that around 50 million people globally have dementia, with a 20% increase each year. As per the 2022 January census, there are 944,000 people with dementia in the UK. This is projected to increase to 1.6 million people in 2040. Important reasons include increasing lifespan and accelerated ageing. There are 40,000 people with Early-onset dementia. Alzheimer’s dementia is the commonest of all other types of dementia, constituting about 50-75%. Vascular dementia is the second most common type of dementia. Almost one-fifth of all cases of dementia are of this type. Roughly, around 150,000 people in the UK are diagnosed with Vascular dementia. Lewy Body dementia is the third most common and is seen in 10-15% of cases. Frontotemporal dementia is seen in about 2% of cases.

References

• Turner, M.A., Moran, N.F. and Kopelman, M.D. (2002). Subcortical dementia. British Journal of Psychiatry, 180(2), pp.148–151. doi:https://doi.org/10.1192/bjp.180.2.148.
• Aarsland, D., Larsen, J.P., Lim, N.G., Janvin, C., Karlsen, K., Tandberg, E. and Cummings, J.L. (1999). Range of neuropsychiatric disturbances in patients with Parkinson’s disease. Journal of Neurology, Neurosurgery & Psychiatry, 67(4), pp.492–496. doi:https://doi.org/10.1136/jnnp.67.4.492.
• Brown, R.G. and Marsden, C.D. (1988). ‘Subcortical dementia’: The neuropsychological evidence. Neuroscience, 25(2), pp.363–387. doi:https://doi.org/10.1016/0306-4522(88)90246-1.
• Cummings, J.L. (1986). Subcortical Dementia Neuropsychology, Neuropsychiatry, and Pathophysiology. British Journal of Psychiatry, 149(6), pp.682–697. doi:https://doi.org/10.1192/bjp.149.6.682.
• Dementia Statistics Hub. (2022). Numbers of people in the UK. [online] Available at: https://www.dementiastatistics.org/statistics/numbers-of-people-in-the-uk-2/
• Dementia UK (2011). Dementia UK. [online] Dementia UK. Available at: https://www.dementiauk.org
• NICE. (n.d.). CKS is only available in the UK. [online] Available at: https://cks.nice.org.uk/topics/dementia/background-information/causes/
• Emmady, P.D. and Tadi, P. (2022). Dementia. [online] Available at: https://www.ncbi.nlm.nih.gov/books/NBK557444/#article-20337.s2
• Anon, (n.d.). Dementia Care Central Homepage. [online] Available at: https://www.dementiacarecentral.com/
• Geschwind, M.D., Haman, A. and Miller, B.L. (2007). Rapidly Progressive Dementia. Neurologic Clinics, [online] 25(3), pp.783–807. doi:https://doi.org/10.1016/j.ncl.2007.04.001.

Alzheimer’s dementia

This is the commonest form of dementia in the UK and globally as well. 50-75% of patients with dementia experience Alzheimer’s type of dementia. It is also a common form of young onset dementia, constituting one-third of dementia seen in people less than 65 years. 

An individual with Alzheimer’s dementia usually presents with memory loss and cognitive difficulties. They have trouble thinking, organising, problem-solving and communicating.

Associations/ risk factors

1. Age: Generally, 1% less than 65 years, 5% around 65 years and increases steadily to 40% by 80 years of age 
2. Familial: 5% of all cases
3. Genes: ApoE4, Presenilin 1/ 2 (young onset dementia)
4. Head injury- People who have sustained head injury are at risk of dementia in general and Alzheimer’s in particular. 
5. Females: 2 times more than males
6. Poor educational attainment
7. Sedentary lifestyle
8. Down’s syndrome, Learning disability
9. Depression
10. Medical illnesses like Diabetes, Hypertension, Stroke, Hyperlipidaemia, Obesity

Pathophysiology

In Alzheimer’s dementia, there is an accumulation of two different abnormal structures. These, in turn, affect the entire brain, causing shrinkage of the brain.

1. Beta Amyloid plaques
   • These are dense protein and cellular deposits that accumulate outside and around nerve cells in the brain. This leads to cell death and dementia.
2. Neurofibrillary tangles
   • This is hyperphosphorylated tau protein. The tau proteins start folding and clumping, resulting in these twisted fibres inside the nerve cells, causing dementia.

Clinical Features

1. Memory
   • They usually have an insidious onset with a gradual progression of memory difficulties. It can include both short and long-term memory loss. Impaired ability to learn and remember new information. 
     • Losing keys and glasses
     • Forgetting the names of people
     • Forgetting medications
     • Missing appointments
     • Repeating conversations
2. Orientation
   • They might experience problems such as poor orientation to time, place and people.
     • Waking up in the night- and being active
     • Losing track of days and dates
3. Language and Communication
   • Dysphasia, difficulty in word finding, Difficulty in understanding conversations and communicating
     • Speaking, reading and writing problems
     • Errors during conversations
4. Executive function
   • Difficulty in initiating, planning and organising. Difficulty in problem-solving, judging and decision-making. Inability to manage finances and complex tasks
5. Visuospatial difficulties
   • Inability to recognise people, face
   • Inability to orient to clothes, routes
   • Wandering
   • Parking car
   • Climbing up and down the stairs
6. Activities of Daily Living
   • Initially, complex activities like cooking, shopping, and organising can be impacted
   • Later, simple activities like bathing, showering, eating, swallowing, dressing, and walking can be impacted
7. Behavioural and Psychological Symptoms of dementia
   • Depression
   • Anxiety
   • Psychosis
     • Delusions
     • Hallucinations
   • Agitation
     • Argumentative
     • Verbally, physically challenging behaviour
   • Emotional lability
     • Easily upset
     • Mood swings
   • Apathy
   • Motor disturbances
     • Restless
     • Wandering behaviour
   • Sleep cycle disturbances
   • Social disinhibition

Evaluation

1. Detailed history is taken from family members who know him well.
2. Thorough physical examination
3. Neurological Examination
   • Gait, coordination
   • Sensory
   • Motor
4. Vision, Auditory problems
5. Systemic Examination
6. Blood investigations:
   • FBC, ESR, CRP, Urea & Electrolytes, Calcium, HbA1c, LFT, TFT, Serum B12 & Folate
   • Radiology, serology, ECG, Neuroimaging based on needs
7. Cognitive assessment:
   • AMTS
   • MMSE- Mini-mental state examination
   • 10-point cognitive screener
   • 6 CIT- 6 item Cognitive impairment test
   • Mini-Cog
   • ACE-R- Addenbrooke’s Cognitive Examination- Revised
   • MOCA – Montreal Cognitive Assessment
8. Carers assessment

Differential Diagnosis

• Normal age-related Memory difficulties
• Mild Cognitive impairment
• Mixed dementia
• Depression
• Delirium
• Medical causes:
  • Hypothyroidism, B12 deficiency, adverse drug effects
• Sensory difficulties
• NPH- Normal Pressure Hydrocephalus

Management

Generally, a biopsychosocial approach is employed in Psychiatry.

Bio

• Acetylcholinesterase inhibitors- Donepezil, Galantamine, Rivastigmine
• NMDA antagonist- Memantine

Psycho

• Psychoeducation
• Cognitive stimulation therapy
• Cognitive rehabilitation
• Group reminiscence therapy
• Skills training for the family to manage behavioural changes

Social

• Occupational therapy- To assess risks at home and arrange for handrail and stair lift
• Physiotherapy- to assess mobility and arrange walking aids
• Social services- Day care, respite care, meals on wheels
• Music, art, dance, pet, massage therapies

Prognosis

Dementia, in general, is a progressive condition. There is no treatment available to cure it. Dementia and Alzheimer’s disease are the leading cause of death for women. It accounts for about 13% of deaths among women and is the second leading cause of death among men. It is almost half of that of women (7%)

Mild cognitive decline has a life expectancy of about 10 years. It drops down as the severity increases from moderate to severe cognitive decline, from 3-8 years to less than 4 years respectively.

References

• Gunhild Waldemar and Burns, A.S. (2017). Alzheimer’s disease. Oxford: Oxford University Press.
• Dening, T., Thomas, A., Stewart, R. and J -P Taylor (2020). Oxford textbook of old age psychiatry. Oxford: Oxford University Press.
• Taylor, J.-P. and Underwood, B.R. (2020). Alzheimer’s disease. Oxford Textbook of Old Age Psychiatry, pp.443–478. doi:https://doi.org/10.1093/med/9780198807292.003.0030.
• bestpractice.bmj.com. (n.d.). Alzheimer’s disease – Symptoms, diagnosis and treatment | BMJ Best Practice. [online] Available at: https://bestpractice.bmj.com/topics/en-gb/317?q=Alzheimer%27s%20disease&c=suggested [Accessed 21 Feb. 2023].
• bestpractice.bmj.com. (n.d.). Assessment of dementia – Differential diagnosis of symptoms | BMJ Best Practice. [online] Available at: https://bestpractice.bmj.com/topics/en-gb/242.
• bestpractice.bmj.com. (n.d.). Assessment of memory deficit – Differential diagnosis of symptoms | BMJ Best Practice. [online] Available at: https://bestpractice.bmj.com/topics/en-gb/710
• Alzheimer’s Society (2019). Alzheimer’s Society – United against dementia | Alzheimer’s Society. [online] Alzheimers.org.uk. Available at: https://www.alzheimers.org.uk
• Anon, (n.d.). Dementia Care Central Homepage. [online] Available at: https://www.dementiacarecentral.com/

Vascular Dementia

It is the second most common type of dementia. It is a progressive neurodegenerative cognitive decline that constitutes approximately around 20% of all cases of dementia.

It occurs mainly due to reduced blood supply to the brain. This hypoperfusion could be due to damaged or diseased blood vessels, TIA, or mini TIA’s. This hypoperfusion deprives the oxygen and nutrients supply to the brain and can affect cognition and day-to-day living.

Background

Epidemiology

• Unlike Alzheimer’s dementia, the incidence of vascular dementia is higher in males.
• It is common in people more than 65 years of age.
• It constitutes 20% of dementias

Risk factors

• Age- As age increases, risk increases. People above 65 are at higher risk
• Sex- More in males compared to females
• Ethnicity- South Asian and African-Caribbean
• Physical health problems- Cardiovascular risk factors, Arterial disease, Hypertension, Stroke, Diabetes Mellitus, Hyperlipidaemia, Obesity
• Smoking, excess alcohol intake
• Social isolation
• Physical inactivity
• Head injury

Types of Vascular Dementia

1. Mild Vascular cognitive impairment
2. Multi-infarct dementia- the commonest form. It is caused by a series of small strokes or TIAs. It is a cortical type of vascular dementia. The white matter of the cerebral hemisphere and the deep grey nuclei, including the striatum and thalamus, are affected more, causing cognitive decline. It mainly affects the area associated with memory, learning and language skills. Though it can be temporary, multiple episodes can lead to permanent damage.
3. Vascular dementia due to a strategic single infarct
4. Vascular dementia due to lacunar lesions- small cavities or lacunas are formed in the brain parenchyma due to occluding vessels.
5. Vascular dementia due to haemorrhagic lesions
6. Binswanger disease- affects small blood vessels in the white matter
7. Subcortical Vascular dementia
8. Mixed dementia (Alzheimer’s dementia plus Vascular dementia)

Clinical features

1. A classic history of stepwise progression is suggestive of vascular dementia but is not always seen. The onset of illness and cognitive problems could be related to cerebrovascular disease. 
2. General symptoms as seen in Alzheimer’s disease may be noted
3. Specific features include:
   • Mild to severe Depression
   • Weakness of limbs and movement difficulties
   • Bladder and bowel control can be lost
   • Speech and language difficulties
4. Behavioural problems:
   • Anger, agitation
   • Psychotic symptoms
   • Laughing, crying inappropriately
   • Problems following instructions, handling money
5. Mood and behavioural changes are more prominent in small vessel diseases like Binswanger’s, lacunar disease

Differential Diagnosis

• Other types of dementias
• Parkinson’s disease dementia
• Depression
• HIV-associated neurocognitive disorder
• Huntington’s disease dementia

Evaluation

1. Very similar to Alzheimer’s dementia.
2. Specific tests and investigations
   • Physical health assessment
   • Special tests- serology (VDRL, HIV) in specific situations
3. Neuroimaging- CT, MRI

Management

There is no specific treatment as such. These treatment options can reduce the progress of vascular dementia. A biopsychosocial approach is taken, like the management of other dementias. 

Bio

• Mainstay is managing medical illnesses such as high blood pressure, high cholesterol, diabetes Mellitus and heart conditions
• Anti-dementia medications are not as effective as in Alzheimer’s dementia
  • Anti-dementia medications can be considered only if there is associated Alzheimer’s dementia, Parkinson’s Disease dementia, Dementia with Lewy Bodies
  • Consider antipsychotics for behavioural and psychological symptoms of dementia, weighing the pros and cons of antipsychotics and physical health.

Psycho

• Apart from the ones mentioned in Alzheimer’s dementia, CBT for anxiety and depression.

Social

• Apart from the ones mentioned in Alzheimer’s dementia, patients should be educated on a healthy lifestyle: smoking cessation, reducing alcohol excess, increasing exercise and maintaining a healthy diet. 
• Physiotherapy and Occupational therapy input
• Speech and Language therapy
• Carer support, Respite care

Prognosis

Vascular dementia has a higher mortality rate compared to Alzheimer’s dementia. People with a diagnosis live for approximately 5 years following the diagnosis. This could be due to multiple physical health risk factors.

References

• Stewart, R. (2020). Vascular and mixed dementia. Oxford Textbook of Old Age Psychiatry, [online] pp.479–494. doi:https://doi.org/10.1093/med/9780198807292.003.0031.
• bestpractice.bmj.com. (n.d.). Vascular dementia – Symptoms, diagnosis and treatment | BMJ Best Practice. [online] Available at: https://bestpractice.bmj.com/topics/en-gb/319
• Wolters, F.J. and Ikram, M.A. (2019). Epidemiology of Vascular Dementia. Arteriosclerosis, Thrombosis, and Vascular Biology, 39(8), pp.1542–1549. doi:https://doi.org/10.1161/atvbaha.119.311908.
• Series, H. and Esiri, M. (2012). Vascular dementia: a pragmatic review. Advances in Psychiatric Treatment, 18(5), pp.372–380. doi:https://doi.org/10.1192/apt.bp.110.008888.
• Series, H. and Esiri, M. (2020). Vascular Dementia. [online] Cambridge University Press. Available at: https://www.cambridge.org/core/books/abs/clinical-topics-in-old-age-psychiatry/vascular-dementia/EA86DA225E0DDA48E03FCAA884EE685A [Accessed 21 Feb. 2023].

Lewy Body Dementia

It is the third commonest type of dementia. It is a progressive neurodegenerative cognitive decline, which constitutes approximately around 10% of all cases of dementia. It is generally characterised by difficulties in attention, focusing on tasks and executive function. There is a fluctuating cognitive problem noted rather than learning. They also present with visual hallucinations and mobility problems.

Background

Epidemiology

• Unlike Alzheimer’s dementia, this is higher in males.
• It is a disease of middle-aged and older people.
• It constitutes 10% of dementias.

Pathophysiology

Lewy Body dementia is a broad term that includes two subtypes:

1. Dementia with Lewy bodies- the individual develops fluctuating cognition-related problems that affect the way they think and behave. They would also experience changes in their visual perception, resulting in visual hallucinations. These symptoms occur first or together with movement and mobility problems.
2. Parkinson’s disease dementia- In this type of dementia, Parkinsonian symptoms are seen earlier than cognitive difficulties. The cognitive difficulties may be seen many years later in general.

In both these types of dementia, the common factor is the presence of Lewy bodies. The site of location of the Lewy bodies determines these two subtypes. They are small round neuronal inclusions comprised of altered neurofilament proteins (Tau proteins and tangles). 

In dementia with Lewy Body, the Lewy bodies are mainly concentrated in the cerebral cortex. In Parkinson’s disease, it is more in the substantia nigra, whilst in Parkinson’s disease dementia; the Lewy bodies are seen in both the cerebral cortex and substantia nigra.

Risk factors

• No specific risk factors. It is generally multifactorial.

Clinical features

1. General symptoms, as seen in Alzheimer’s, might be noted due to cognitive difficulties. However, the cognitive symptoms are generally fluctuating with varying levels of alertness and attention. Intellectual functioning is better preserved than in Alzheimer’s dementia.
2. Visual hallucinations- vivid
3. Executive functions and visuospatial deficits are more prominent here than in Alzheimer’s dementia.
4. Parkinsonian symptoms:
   • Masked facies
   • Shuffling gait
   • Rigid limbs
   • Bradykinesia
   • Stooped posture
   • Tremors
5. Supportive symptoms:
   • Repeated falls, balance problems
   • Syncope
   • Transient loss of consciousness
   • Neuroleptic sensitivity
   • Rapid eye movement sleep disorder
   • Systematised Delusions
   • Restless Leg Syndrome
   • Autonomic dysfunction- constipation, orthostatic hypotension, urinary incontinence
   • Speech and swallowing problems
6. Behavioural problems:
   • Anger, agitation
   • Depression, anxiety
   • Apathy
   • Excess daytime sleeping
   • Problems following instructions, handling money

Differential Diagnosis

• Other types of dementias
• Parkinson’s Disease dementia
• Depression
• HIV-associated neurocognitive disorder
• Huntington’s disease dementia

Evaluation

• Very similar to Alzheimer’s dementia. Basic screening for memory problems
• Specific tests, investigations
  1. Physical health assessment
  2. Special tests- serology (VDRL, HIV) in specific situations
• Neuroimaging- CT, MRI, PET, SPECT, DAT SCAN

Management

General principles 

• There are no specific treatments as such. These treatment options can provide symptomatic treatment. 
• It is pertinent to get the diagnosis accurately so the right symptomatic treatment and support can be offered. Hence, the importance of detailed collateral history and investigations.
• Medications used for visual hallucinations can worsen Parkinson’s, and treatment offered for Parkinson’s symptoms can worsen psychosis. Moreover, neuroleptic sensitivity can add to the difficulty of offering limited medications.
• Non-pharmacological treatment should be used in the first instance.

Bio

• Acetylcholinesterase inhibitors are used as the first choice for hallucinations and behavioural problems like agitation. They also can help with cognitive problems. The aim is to lead a better quality of life and manage activities of daily living.
• Should a need arise to offer antipsychotics, atypical neuroleptics/ antipsychotics are preferred (Quetiapine, Aripiprazole, Clozapine), especially if there is a poor response to cholinesterase inhibitors.
• For motor symptoms, anti-Parkinson medications such as levodopa and carbidopa are helpful in a few individuals.
• For the REM sleep disorder, Clonazepam and Melatonin (especially at lower doses) have been helpful.
• Consider antipsychotics, prn Benzodiazepine for behavioural and psychological symptoms of dementia, weighing the pros and cons of antipsychotics and the physical health.

Psycho

• Apart from the ones mentioned in Alzheimer’s dementia, CBT is used for anxiety and depression.
• Cognitive stimulation therapy

Social

• Apart from the ones mentioned in Alzheimer’s dementia, education regarding a healthy lifestyle (smoking cessation, reducing alcohol excess, exercise and a healthy diet)
• Physiotherapy and Occupational therapy input- adaptations at home for independent living
• Speech and Language therapy
• Carer support, Respite care
• Reminiscence therapy- sharing life experiences and memories
• Positive interactions and activities that interest and distract them
• Basic sleep hygiene

Prognosis

Dementia with Lewy body is a progressive illness, and it progresses faster than Alzheimer’s dementia. Mortality is associated with complications such as swallowing problems and poor nutrition. Dysphagia and mobility issues can worsen aspiration and pneumonia. Mobility problems can lead to frequent falls and injuries.

People with a diagnosis live approximately 5 years or so following the diagnosis. This could be due to multiple physical health risk factors.

References

1. Rongve, A. and Aarsland, D. (2020). The Lewy body dementias: Dementia with Lewy bodies and Parkinson’s disease dementia. academic.oup.com. [online] doi:https://doi.org/10.1093/med/9780198807292.003.0032.
2. McKeith, I.G. (2002). Dementia with Lewy bodies. The British Journal of Psychiatry, [online] 180(2), pp.144–147. doi:https://doi.org/10.1192/bjp.180.2.144.
3. Chin, K.S., Teodorczuk, A. and Watson, R. (2019). Dementia with Lewy bodies: Challenges in the diagnosis and management. Australian & New Zealand Journal of Psychiatry, 53(4), pp.291–303. doi:https://doi.org/10.1177/0004867419835029.
4. Vik-Mo, A.O., Giil, L.M., Borda, M.G., Ballard, C. and Aarsland, D. (2019). The individual course of neuropsychiatric symptoms in people with Alzheimer’s and Lewy body dementia: 12-year longitudinal cohort study. The British Journal of Psychiatry, 216(1), pp.43–48. doi:https://doi.org/10.1192/bjp.2019.195.
5. bestpractice.bmj.com. (n.d.). Dementia with Lewy bodies – Symptoms, diagnosis and treatment | BMJ Best Practice. [online] Available at: https://bestpractice.bmj.com/topics/en-gb/320?q=Lewy%20body%20dementia&c=suggested [Accessed 21 Feb. 2023].

Frontotemporal Dementia

Background

It is a rare form of dementia. It is a progressive neurodegenerative cognitive decline, which constitutes approximately around 2% of all cases of dementia. It is a condition characterised by deterioration of personality and cognitive issues. There is evidence of atrophy in the frontal and temporal lobes.

It is generally characterised by difficulties in thinking and behaviour. They usually present with unusual and unwarranted behaviours, emotional problems, difficulties in communication (speech and language), motivation and preservative behaviours.

Epidemiology

• Unlike Alzheimer’s dementia, this is higher in males.
• It is a disease of middle-aged and older people.
• It constitutes 2% of all cases of dementia

Pathophysiology

There is prominent frontal and temporal lobar atrophy.

FTD is a broad term that includes a few subtypes:

1. Behavioural FTD or frontal variant FTD (bvFTD) – 60% of patients with FTD have this type of behavioural FTD. It causes changes in judgement, personality, behaviour and emotions. Behavioural changes are seen initially.
2. Semantic dementia or primary progressive aphasia: (sv FTD or PPA): Here, mainly there is language difficulty (speech production is spared). However, during early stages, the other aspects such as judgement, behaviour and personality are not disabled; during later stages, they are impacted.
3. Progressive non-fluent Aphasia (PNFA): Here mainly there is language difficulty- mainly speaking. Grammar is affected more so in PNFA.
4. Progressive Supranuclear palsy: This involves the brainstem, frontal cortex and basal ganglia.
5. Corticobasal degeneration: This is a neurodegenerative disorder classified as an atypical Parkinsonian syndrome
6. FTD with Motor Neuron disease: Early cognitive and behavioural changes with MND

Risk factors

• No specific risk factors. 
• Men and women are equally affected
• More likely to run in families

Clinical features

1. General symptoms of behavioural variant FTD:
   • Behavioural changes:
     • Making inappropriate comments
     • Overfamiliar, socially disinhibited
     • Impulse-losing inhibitions
     • Commenting on others’ appearances, being insensitive and rude, staring
     • Sexual gestures in public
     • Aggressive verbally, physically
   • Changes in food and drinking behaviours: Overeating, craving sweet food, poor table manners, drinking excessively, and not knowing when to stop eating or drinking.
   • Poor organising skills, planning and decision-making.
   • Poor insight- lack of awareness of changes in themselves (Anosognosia)
   • Poor empathy- fail to understand others and less considerate of their feelings (alexithymia)
   • Poor motivation to do things, anhedonia
   • Reduced concentration and ability to focus (gets distracted easily)
   • Repetitive and or obsessive behaviours- Repeating phrases, gestures, hoarding
2. Semantic variant PPA:
   • Although this mainly involves language, other FTD symptoms might present too
     • They lose their vocabulary over the days: Initially complicated words and then later simple ones.
     • They may forget the functions of familiar objects: The use of fridge, microwave
     • Loss of skill to use simple objects in day-to-day life: use of comb, toothbrush
3. Non-fluent variant PPA:
   • Although this mainly involves language, other FTD symptoms might be present too. As the name suggests, the problem is in speaking fluently
     • Poor grammatical sentences (agrammatism)
     • Short telegraphic speech, sound errors
     • Saying the opposite of what they want to
     • They might understand a particular word but not when in a sentence
4. Late stages of FTD:
   • Cognitive impairment
   • Psychotic symptoms
   • BPSD
   • Poor recognition of people
5. Progressive Supranuclear palsy:
   • Postural instability
   • Vertical Supranuclear ophthalmoparesis
   • Very similar to multiple system atrophy, Parkinson’s
   • Axial rigidity
6. Cortico-basal degeneration:
   • Asymmetric ideomotor apraxia
   • Alien limb phenomenon
   • Visual, sensory hemineglect
   • Myoclonus
   • Non-fluent aphasia
   • Extrapyramidal dysfunction
   • Repeated falls, balance problems
   • Syncope
   • Transient loss of consciousness
   • Neuroleptic sensitivity
   • Rapid eye movement sleep disorder
   • Systematised Delusions
   • Restless Leg Syndrome
   • Autonomic dysfunction- constipation, orthostatic hypotension, urinary incontinence
   • Speech and swallowing problems
7. Behavioural problems:
   • Anger, agitation
   • Depression, Anxiety
   • Apathy
   • Excess daytime sleeping
   • Problems following instructions, handling money

Differential Diagnosis

• Very similar to Alzheimer’s dementia. Basic screening for memory problems

Evaluation

• Very similar to Alzheimer’s dementia. Basic screening for memory problems
• Most patients with FTD have normal neurological examination findings. However, frontal lobe dysfunction may have these various reflexes positive- glabellar, sucking, Rooting reflexes, etc
• Neuroimaging- CT, MRI, PET, SPECT, DAT SCAN 

Management

1. There is no specific treatment as such. These treatment options can provide symptomatic treatment. 
2. It is pertinent to get the diagnosis accurately so the right symptomatic treatment and support can be offered. Hence, the importance of detailed collateral history and investigations.
3. Non-pharmacological treatment should be used in the first instance.
4. It is symptomatic management as in other dementias. There is not much benefit to giving acetylcholinesterase inhibitors.

Prognosis

People with a diagnosis live for less than 5 years or so approximately following the diagnosis. 

References

• Bocchetta, M. and Rohrer, J.D. (2020). Frontotemporal dementia. Oxford Textbook of Old Age Psychiatry, [online] pp.513–526. doi:https://doi.org/10.1093/med/9780198807292.003.0033.
• Snowden, J.S., Neary, D. and Mann, D.M.A. (2002). Frontotemporal dementia. The British Journal of Psychiatry, [online] 180(2), pp.140–143. doi:https://doi.org/10.1192/bjp.180.2.140.
• Sivasathiaseelan, H., Marshall, C., Agustus, J., Benhamou, E., Bond, R., van Leeuwen, J., Hardy, C., Rohrer, J. and Warren, J. (2019). Frontotemporal Dementia: A Clinical Review. Seminars in Neurology, 39(02), pp.251–263. doi:https://doi.org/10.1055/s-0039-1683379.
• Dementia UK. (n.d.). Types of dementia. [online] Available at: https://www.dementiauk.org/about-dementia/types-of-dementia/?msclkid=df566113a1b61be45a3902e752c01159 [Accessed 21 Feb. 2023].
• Warren, J.D., Rohrer, J.D. and Rossor, M.N. (2013). Frontotemporal dementia. BMJ, [online] 347(aug12 3), pp.f4827–f4827. doi:https://doi.org/10.1136/bmj.f4827.
• bestpractice.bmj.com. (n.d.). Frontotemporal dementia – Symptoms, diagnosis and treatment | BMJ Best Practice. [online] Available at: https://bestpractice.bmj.com/topics/en-gb/968.

Parkinson’s disease dementia

Background

This is a rare form of dementia. It is due to progressive neurodegenerative cognitive decline, which constitutes approximately around 2% of all cases of dementia worldwide. Patients with Parkinson’s disease have a higher chance of dementia, although almost 2/3rd do not suffer from dementia. It is a condition characterised by deterioration of cognition and day-to-day tasks. 

It is generally characterised by difficulties in thinking and concentration. They usually present with difficulties in communication (speech and language) and executive functioning.

It is a part of dementia with Lewy bodies. One of the entities is Parkinson’s disease dementia, while the other is Lewy Body dementia. 

Epidemiology

• Like Alzheimer’s dementia, this is a common neurodegenerative illness
• It is seen similarly in both sexes.
• It is a disease of middle-aged and older people.
• It constitutes 2% of all cases of dementia worldwide

Pathophysiology

Lewy body accumulates in vulnerable sites of the central nervous system. It contains a protein called alpha-synuclein. The alpha-synuclein is seen in Alzheimer’s dementia as well. There is also a deficiency of Acetylcholine.

Depending on the deposition of the Lewy body, they can be predominantly Nigral disease, limbic disease or diffuse neocortical disease.

Parkinson’s disease dementia is diagnosed based on the timing of cognitive difficulties and motor symptoms. If dementia was present at the onset of symptoms or within 1 year of motor symptoms, then it is Lewy Body dementia. However, if someone has had Parkinson’s dementia or if they have had motor symptoms for at least 1 year and following that, if they develop memory difficulties, it is diagnosed as Parkinson’s disease dementia.

Risk factors

• No specific risk factors 
• Men and women are equally affected
• More likely to run in families
• The presence of hallucinations in a person without dementia, excessive daytime sleepiness and postural instability can increase the chances of dementia with Parkinson’s disease

Clinical features

1. Core clinical symptoms
   • Loss of memory- slowed thinking- poor concentration (This makes it difficult to make decisions, judgement and planning activities. It also makes one to do daily activities like dressing, grooming or cleaning difficult)
   • Recurrent visual hallucinations
   • Rapid eye movement and sleep behavioural disorder
   • Spontaneous motor signs of Parkinsonism
     • Bradykinesia
     • Resting tremor
     • Rigidity
2. Supportive clinical features
   • Excess daytime sleeping and insomnia
   • Severe sensitivity to antipsychotics
   • Anosmia
   • Mental Health problems- Depression, Anxiety, Apathy, Delusions
   • Irritability and agitation
   • Postural instability and repeated falls
   • Autonomic disturbances- Low BP, urological disturbances, constipation, syncope

 

Evaluation

• Very similar to Alzheimer’s dementia. Basic screening for memory problems
• EEG, polysomnography

Management

1. Treatment is mainly symptomatic
2. It is pertinent to get the diagnosis accurately so the right symptomatic treatment and support can be offered. Hence, the importance of detailed collateral history and investigations.
3. Medications used for visual hallucinations can worsen Parkinson’s, and treatment offered for Parkinson’s symptoms can worsen psychosis. Moreover, Neuroleptic sensitivity can add to the difficulty of offering limited medications.
4. Non-pharmacological treatment should be used in the first instance.

Bio

• Antipsychotics should be avoided as much as possible. Non-pharmacological approach such as comfortable surroundings and environment, adequate lighting, appropriate visual and hearing aids and managing other problems like dehydration, pain, infections, etc., is essential.
• The rest of the treatment is like Lewy Body dementia
• Consider antipsychotics, prn Benzodiazepine for Behavioural and Psychological symptoms of Dementia, weighing the pros and cons of antipsychotics and the physical health.
• Symptomatic treatment of anxiety and depression

Psycho

• The treatment is like Lewy Body dementia

Social

• The treatment is like Lewy Body dementia

Prognosis

Parkinson’s disease dementia is a progressive illness, and it progresses faster than Alzheimer’s dementia. Mortality is associated with complications such as swallowing problems and poor nutrition. Dysphagia and mobility issues can worsen aspiration and pneumonia. Mobility problems can lead to frequent falls and injuries.

People with a diagnosis live for less than 5 years or so approximately following the diagnosis. 

References

• Rongve, A. and Aarsland, D. (2020). The Lewy body dementias: Dementia with Lewy bodies and Parkinson’s disease dementia. academic.oup.com. [online] doi:https://doi.org/10.1093/med/9780198807292.003.0032.
• McKeith, I. (2007). Dementia with Lewy bodies and Parkinson’s disease with dementia: where two worlds collide. Practical Neurology, 7(6), pp.374–382. doi:https://doi.org/10.1136/jnnp.2007.134163.
• Aarsland, D., Batzu, L., Halliday, G.M., Geurtsen, G.J., Ballard, C., Ray Chaudhuri, K. and Weintraub, D. (2021). Parkinson disease-associated cognitive impairment. Nature Reviews Disease Primers, 7(1). doi:https://doi.org/10.1038/s41572-021-00280-3.
• Gibb, W.R.G. (1989). Dementia and Parkinson’s Disease. British Journal of Psychiatry, 154(5), pp.596–614. doi:https://doi.org/10.1192/bjp.154.5.596.
• Parkinson’s UK. (n.d.). Dementia. [online] Available at: https://www.parkinsons.org.uk/information-and-support/dementia
• Alzheimer’s Society. (n.d.). Parkinson’s disease. [online] Available at: https://www.alzheimers.org.uk/about-dementia/types-dementia/parkinsons-disease
• Alzheimer’s Disease and Dementia. (2019). Parkinson’s Disease. [online] Available at: https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/parkinson-s-disease-dementia#:~:text=Prevalence.%20The%20Parkinson%27s%20Foundation%20estimates%20that%20one%20million.

Young Onset Dementia

When someone develops dementia before the age of 65, it is called young onset dementia. It causes cognitive and behavioural problems apart from neuropsychiatric symptoms. There is an increase in cases in the UK. It affects not only the individual but their entire family as well.

It is also called ‘early onset’ or ‘working age’ dementia. However, this is subjective and is becoming less pertinent as services are focused on not just the individual but also the impact. 

Unfortunately, individuals with early onset dementia postpone meeting the professionals for assessment and delay a diagnostic assessment. On the other hand, there are reports that patients and their families sometimes must move ‘from pillar to post’ before gaining access to appropriate services. The longer time to reach diagnosis can have a knock-on effect on family stress, work sickness and loss of earnings, delays in treatment, increased cognitive problems and functional decline, depression or anxiety and missed opportunities to participate in research.

Evaluation and management include detailed clinical assessment and bio-psycho-social management as per individual dementias mentioned above.

Epidemiology

• It is seen in individuals between 45-65 years.
• It is estimated that 42,325 people in the UK have been diagnosed with young-onset dementia. (Ref Dementia UK, 2nd edition 2014, Alzheimer’s Society).  
• They represent around 5% of the 850,000 people with dementia. 
• The actual figure could be higher because of the difficulties of diagnosing the condition and may be closer to 6-9% of all people with dementia. 
• Awareness in primary care is still comparatively low, and when individuals are still functioning or working, symptoms are often recognised as stress, depression or anxiety rather than dementia.
• Young onset dementia is not only uncommon but is challenging to distinguish. Since they are younger, they find it difficult to admit anything wrong, especially when they are healthy, resulting in delayed presentation with the doctors.
• People with young-onset dementia are more likely to have a hereditary form of dementia. 
• Young-onset dementia seems to be higher among BAME groups.  
• Likewise, another group of individuals with a higher prevalence is those with Intellectual disabilities. Studies have shown that 1 in 10 people with a learning disability develop young-onset Alzheimer’s disease between the ages of 50 to 65. 
• The number of people with Down’s syndrome who develop Alzheimer’s disease is even greater, with 1 in 50 developing the condition aged 30-39, 1 in 10 aged 40-49, and 1 in 3 people with Down’s syndrome will have Alzheimer’s in their 50s and almost 1 in 2 at 60 years.

Pathophysiology

• Presenile Alzheimer’s dementia can run in families and is autosomal dominant. It can be due to mutations of presenilin 1 or 2 genes. 
• Likewise, frontotemporal lobar degeneration can occur in individuals between ages 45 and 65 usually and could be due to genetic causes, especially involving a mutation of tau genes.
• Huntington’s dementia can be caused due to expansion of the CAG repeat sequence. It is inherited as an autosomal dominant condition. 
• Vascular dementia due to physical morbidities can also be responsible. 
• Cerebral vasculitis is one of the treatable causes

Common types/causes of Young onset dementia (This list is not exhaustive):

1. Young-onset- Alzheimer’s dementia
2. Young-onset- Vascular dementia
3. Young-onset- Frontotemporal dementia
4. Korsakoff’s syndrome
5. Young-onset- Lewy body dementia
6. Parkinson’s dementia
7. Huntington’s dementia
8. Creutzfeldt-Jacob dementia

References

• www.alzheimers.org.uk. (2021). Young-onset dementia | Alzheimer’s Society. [online] Available at: https://www.alzheimers.org.uk/about-dementia/types-dementia/young-onset-dementia
• Sampson, E.L. (2004). Young-onset dementia. Postgraduate Medical Journal, 80(941), pp.125–139. doi:https://doi.org/10.1136/pgmj.2003.011171.
• Young Dementia Network. (n.d.). Resources for clinicians. [online] Available at: https://www.youngdementianetwork.org/resources/resources-for-clinicians/ [Accessed 21 Feb. 2023].
•  www.rcpsych.ac.uk. (n.d.). Young-onset dementia in mental health services (CR217 Oct 2018). [online] Available at: https://www.rcpsych.ac.uk/improving-care/campaigning-for-better-mental-health-policy/college-reports/2018-college-reports/cr217 [Accessed 21 Feb. 2023]
• Jefferies, K. and Agrawal, N. (2020). Young-Onset Dementias. [online] Cambridge University Press. Available at: https://www.cambridge.org/core/books/abs/clinical-topics-in-old-age-psychiatry/youngonset-dementias/E45582319E62BC20D6756D760B34309D [Accessed 21 Feb. 2023].