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Elderly Care & Frailty

Question 126 of 180

A 68 year old woman is brought to the Emergency Department by her concerned son. He describes several months of mild confusion and disinhibition. He has not noted any change in her mood. She has frequent falls and has become incontinent of urine over the last month. She has no significant past medical history and does not smoke or drink alcohol. General physical examination is unremarkable. On neurological examination you note normal cranial nerves and upper limb neurology however the lower limbs have an increased tone with upgoing plantars. What is the diagnosis?

Answer:

  • Whilst falls and urinary incontinence can occur in Alzheimer’s disease, these occur late in disease progression and do not fit with the patient’s young age or mild cognitive impairment.
  • Lewy body dementia has hallucinations as a prominent feature (which are not mentioned) and commonly coexists with parkinsonian movement symptoms. This woman seems to have pyramidal signs in the legs and no arm symptoms.
  • Vascular dementia occurs with multiple strokes or transient ischaemic attacks (TIAs), which this woman does not have a history of, or risk factors for.
  • Benign intracranial hypertension does not commonly cause dementia, and was mainly included to confuse, with its mention of intracranial pressure. It presents with headaches, visual disturbance (papilloedema, which would be absent with normal pressure) and sometimes VI cranial nerve palsies.
  • Normal pressure hydrocephalus commonly presents with the triad of dementia, gait disturbance and urinary incontinence.

Dementia is a typically progressive clinical syndrome of deteriorating mental function significant enough to interfere with activities of daily living (ADLs). It affects cognitive domains (such as memory, thinking, language, orientation and judgement) and social behaviour (such as emotional control and motivation).

Causes

  • Alzheimer’s disease (50 – 75% of cases)
    • Atrophy of the cerebral cortex
    • Formation of amyloid plaques and neurofibrillary tangles
    • Reduced neuronal acetylcholine production
  • Vascular dementia (up to 20% of cases)
    • Reduced blood supply to the brain
  • Dementia with Lewy bodies (DLB) (10 – 15% of cases)
    • Cortical and subcortical Lewy bodies (abnormal deposits of protein inside nerve cells)
  • Frontotemporal dementia (FTB) (2% of cases)
    • Progressive degeneration of the frontal and/or temporal lobe
  • Rarer causes of dementia:
    • Parkinson’s disease dementia
    • Progressive supranuclear palsy
    • Huntington’s disease
    • Prion disease (such as Creutzfeldt-Jakob disease [CJD])
    • Normal pressure hydrocephalus (NPH)
    • Chronic subdural haematoma
    • Benign tumours
    • Metabolic and endocrine disorders (such as chronic hypocalcaemia and recurrent hypoglycaemia)
    • Vitamin deficiencies (such as B12 and thiamine deficiency)
    • Infections (such as HIV infection and syphilis)

Risk factors

  • Age - strongest risk factor for dementia
  • Mild cognitive impairment
  • Learning difficulties
  • Genetics
  • Cardiovascular disease risk factors (such as diabetes, smoking, hypercholesterolemia, and hypertension)
  • Parkinson's disease
  • Stroke
  • Depression
  • Heavy alcohol consumption
  • Low educational attainment
  • Low social engagement and support

Clinical features

Dementia can be difficult to identify as it usually has an insidious onset and non-specific signs and symptoms, which vary from person to person. People with early dementia may deny symptoms or accommodate to cognitive change and functional ability.

Suspect dementia if any of the following are reported by the person and/or their family/carer:

  • Cognitive impairment, including:
    • Memory problems
    • Receptive or expressive dysphasia
    • Difficulty in carrying out coordinated movements such as dressing
    • Disorientation and unawareness of the time and place
    • Impairment of executive function, such as difficulties with planning and problem solving
  • Behavioural and psychological symptoms of dementia (BPSD) tend to fluctuate, may last for 6 months or more and include:
    • Psychosis
    • Agitation and emotional lability
    • Depression and anxiety
    • Withdrawal or apathy
    • Disinhibition
    • Motor disturbance
    • Sleep cycle disturbance or insomnia
    • Tendency to repeat phrases or questions
  • Difficulties with activities of daily living (ADLs):
    • In the early stages of dementia this may lead to neglect of household tasks, nutrition (causing weight loss), personal hygiene, and grooming. People with dementia who are in employment may find that they are increasingly making mistakes at work.
    • In the later stages, basic ADLs such as dressing, eating, and walking become affected.

Specific features of subtypes of dementia:

  • For Alzheimer’s disease:
    • Early impairment of episodic memory — this may include memory loss for recent events, repeated questioning, and difficulty learning new information.
  • For vascular dementia:
    • Stepwise increases in the severity of symptoms — subcortical ischaemic vascular dementia may present insidiously with gait and attention problems and changes in personality.
      Focal neurological signs (such as hemiparesis or visual field defects) may be present.
  • For dementia with Lewy bodies:
    • Repeated falls, syncope or transient loss of consciousness, severe sensitivity to antipsychotics, delusions, and hallucinations may be present. Memory impairment may not be apparent in early stages. Parkinsonian motor features (such as shuffling gait, rigidity, bradykinesia, and loss of spontaneous movement) and autonomic dysfunction (such as postural hypotension, difficulty in swallowing, and incontinence or constipation) may be present.
  • For frontotemporal dementia (FTD):
    • Personality change and behavioural disturbance (such as apathy or social/sexual disinhibition) may develop insidiously. Other cognitive functions (such as memory and perception) may be relatively preserved.

Differential diagnosis

Conditions that can present with similar symptoms to dementia include:

  • Normal-age related memory changes - Normal ageing is associated with a mild decline in cognitive function, and memory lapses are common, especially during times of physical illness or stress.
  • Mild cognitive impairment (MCI) - MCI differs from dementia in that symptoms do not fulfil the diagnostic criteria for dementia, for example only one cognitive domain may be affected or activities of daily life may not be significantly affected. Of people with MCI, 50% will later develop dementia.
  • Depression - Symptoms of depression include low mood, loss of interest, anhedonia, and self-neglect which can be similar to those of dementia.In older people, features of depression may be less obvious, with somatic symptoms (such as reduced appetite, fatigue, and insomnia) are more common.
  • Delirium - Delirium is an acute, fluctuating syndrome of disturbed consciousness, attention, cognition, and perception. It is a common condition in the differential diagnosis for dementia. People with cognitive impairment are at increased risk of delirium, and the two conditions often coexist.
  • Vitamin deficiency - Thiamine deficiency can lead to Wernicke encephalopathy and Korsakoff psychosis. Symptoms include confusion, memory loss, problems with learning new information and gait disturbances. Vitamin B12 deficiency can lead to ataxia, psychiatric abnormalities, memory loss, and gait disturbance.
  • Hypothyroidism - Symptoms of hypothyroidism can include low mood, and impaired concentration and memory.
  • Adverse drug effects - Many drugs, including benzodiazepines, analgesics (such as opioids, naproxen, and ibuprofen), anticholinergics, antidepressants (such as tricyclics), antipsychotics (such as haloperidol), anticonvulsants (especially older preparations, such as phenytoin and phenobarbital), and corticosteroids can affect cognition.
  • Normal pressure hydrocephalus - Normal pressure hydrocephalus can present with symptoms of early cognitive impairment, urinary incontinence, and gait disorder. In NPH, cerebrospinal fluid (CSF) flow has been impeded but compensatory mechanisms have prevented raised pressure. The lateral ventricles are prominently dilated, and these ventricles exert local pressure on certain brain areas to give the classical clinical picture. Pressure on the frontal lobes gives the dementia and pressure on the medial side of the motor cortex, and the pyramidal tract fibres, cause incontinence and pyramidal leg weakness. Diagnosis is by lumbar puncture (to demonstrate a normal CSF opening pressure) followed by head computed tomography (CT)/magnetic resonance imaging (MRI) (showing enlarged ventricles). Treatment is with ventriculoperitoneal shunting.
  • Sensory deficits - Problems with vision and hearing can contribute significantly to an apparent decline in cognitive ability.

Investigations

  • Patient's should be assessed using a standardised cognitive assessment tool.
  • To help identify reversible causes of dementia and exclude other causes of symptoms initial investigations include:
    • In most cases:
      • Full blood count
      • Erythrocyte sedimentation rate (ESR)
      • Urea and electrolytes
      • Calcium
      • HbA1c
      • Liver function tests
      • Thyroid function tests
      • Serum B12 and folate levels
    • If clinically indicated:
      • A midstream urine, for example when delirium is a possibility
      • Chest X-ray, electrocardiogram (ECG), syphilis serology, and HIV testing
  • Patients with suspected dementia should be referred to a specialist memory assessment service for further specialist assessment and management.
  • Specialist investigations for suspected dementia:
    • Should include structural imaging (MRI or CT scan) to: exclude non-dementia cerebral pathology such as normal pressure hydrocephalus and to identify dementia subtype.
    • May also include:
      • HMPAO SPECT and FDG PET as second line investigations if diagnosis or subtype is unclear
      • (FP-CIT) SPECT where a potential diagnosis of dementia with Lewy bodies (DLB) is in doubt
      • Cerebrospinal fluid examination to exclude inflammatory, infective, or malignant causes of dementia, in cases where dementia is rapidly progressive, the presentation is unusual, or the person is younger than 55 years of age
      • Electroencephalography if a diagnosis of delirium, frontotemporal dementia, or Creutzfeldt–Jakob disease is suspected, or in the assessment of associated seizure disorder in people with dementia
      • Brain biopsy in highly selected people whose dementia is thought to be due to a potentially reversible condition (such as cerebral vasculitis) that cannot be diagnosed in another way

Management

  • Specialist non-pharmacological interventions for cognitive symptoms of dementia:
    • Structured group cognitive stimulation programs
    • Reminiscence therapy with discussion of past experiences
    • Tools such as life histories, shared memories, and familiar objects from the past
  • Specialist drug treatments for cognitive symptoms of dementia:
    • Acetylcholinesterase (AChE) inhibitors (donepezil, galantamine, and rivastigmine) - These drugs can be used for mild to moderate Alzheimer's disease.
    • Memantine (a N-methyl-D-aspartic acid receptor antagonist) - Memantine is an option for managing Alzheimer's disease for people with moderate Alzheimer's disease who are intolerant of, or have a contraindication to, AChE inhibitors or in severe Alzheimer's disease.
  • Specialist drug treatment for behavioural and psychological symptoms of dementia (BPSD) or non-cognitive symptoms of dementia and challenging behaviour:
    • No antipsychotics (except risperidone in very specific circumstances) are licensed for BPSD in the UK. Specialists may prescribe an antipsychotic drug off-label in certain circumstances (e.g. if they are severely distressed or there is an immediate risk of harm to the person or others) following a thorough clinical assessment and discussion (including the risks and benefits) with the carer and (if possible) the person with dementia. Antipsychotics have potentially serious adverse effects, including increased risk of stroke and mortality, Parkinsonism, and cognitive impairment. They should be used with caution, at low dose, and for the shortest time possible.
    • In specific situations, specialists may offer AChE inhibitors for BPSD if non-pharmacological methods and anti-psychotic drugs are inappropriate or ineffective.

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  • Biochemistry
  • Blood Gases
  • Haematology
Biochemistry Normal Value
Sodium 135 – 145 mmol/l
Potassium 3.0 – 4.5 mmol/l
Urea 2.5 – 7.5 mmol/l
Glucose 3.5 – 5.0 mmol/l
Creatinine 35 – 135 μmol/l
Alanine Aminotransferase (ALT) 5 – 35 U/l
Gamma-glutamyl Transferase (GGT) < 65 U/l
Alkaline Phosphatase (ALP) 30 – 135 U/l
Aspartate Aminotransferase (AST) < 40 U/l
Total Protein 60 – 80 g/l
Albumin 35 – 50 g/l
Globulin 2.4 – 3.5 g/dl
Amylase < 70 U/l
Total Bilirubin 3 – 17 μmol/l
Calcium 2.1 – 2.5 mmol/l
Chloride 95 – 105 mmol/l
Phosphate 0.8 – 1.4 mmol/l
Haematology Normal Value
Haemoglobin 11.5 – 16.6 g/dl
White Blood Cells 4.0 – 11.0 x 109/l
Platelets 150 – 450 x 109/l
MCV 80 – 96 fl
MCHC 32 – 36 g/dl
Neutrophils 2.0 – 7.5 x 109/l
Lymphocytes 1.5 – 4.0 x 109/l
Monocytes 0.3 – 1.0 x 109/l
Eosinophils 0.1 – 0.5 x 109/l
Basophils < 0.2 x 109/l
Reticulocytes < 2%
Haematocrit 0.35 – 0.49
Red Cell Distribution Width 11 – 15%
Blood Gases Normal Value
pH 7.35 – 7.45
pO2 11 – 14 kPa
pCO2 4.5 – 6.0 kPa
Base Excess -2 – +2 mmol/l
Bicarbonate 24 – 30 mmol/l
Lactate < 2 mmol/l

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