Dementias

Intro

Definition: The global impairment of cognition with normal levels of consciousness.

This is versus acute confusional state, where conscious level is impaired too.

- It is important to try and make a diagnosis, even though it’s really hard and it’s probably
going to be wrong.
- If they have anxiety, it probably isn’t proper dementia, because proper ones have no
insight.
- Treatable causes:
- Depression - Do a MMSE to find out if they are just depressed pseudo-dementia.
But note a lot of dementia patients have depression.
- Normal pressure hydrocephalus - (build up of CSF in ventricles) - Has triad of
dementia, incontinence and gait disturbance, but therapeutic lumbar puncture can
lead to overt improvement
- Subdural haematoma could be no trauma with just cognitive impairment.
- Intracranial tumours - subacute cognitive decline.
- Hypothyroid could have mild to moderate dementia
- Chronic severe hypoNa
- Vit B12 def
- Neurosyphillis or general paralysis of the insane
- Vasculitis
- Paraneoplastic syndromes rarely cause pure dementia
- Tumour-associated limbic encephalitis
- Autoimmune encelopathy
- due to antivoltage-gated K chanenl antibodies
- Whipple’s disease
- Whipple's disease is a rare, systemic infectious disease caused by the bacterium
Tropheryma whipplei.
- Whipple's disease primarily causes malabsorption but may affect any part
of the body including the heart, lungs, brain, joints, and eyes.

- All patients should have

- CT head
- Vit B12
- Thyroid Function
- Syphilis serology
- FBC
- Biochem tests for renal and hepatic function
- ESR for systemic diseases producing cognitive impairment.

Alzheimer’s disease

- Most frequently with gradual impairement of episodic memory and then eventually all
decreased cognitive function (They can’t form new memories and reserves childhood
memories in the beginning, but soon forgets everything)
- MRI shows temporal atrophy
- 5% familial show early (APP amyloid precursor gene and some ApoE polymorphisms
predispose to alzheimers)
- Pathologically there is cortical neuron loss, including loss of ACh neurons, where ACh
inhib comes into effect
- Prognosis is not good, progresses relentlessly, death from pneumonia or inanition after 8-
10y.
- Key to mx is treat symptom-to-symptom. Take pressure off care-giver.

Frontotemporal dementia (Pick’s Disease)

Pick's disease, also known as Pick disease and PiD, is a rare neurodegenerative
disease. While the term Pick's disease was once used to represent a specific group of
clinical syndromes with symptoms attributable to frontal and temporal lobe dysfunction, it is
now used (at least among professionals in the field) to mean a specific pathology that is
just one of the causes of the clinical syndrome now known as frontotemporal lobar
degeneration. Some people still use the term Pick's disease to mean the more general
clinical syndrome of frontotemporal lobar degeneration, but this has previously led to
confusion among both professionals and patients and so its use should be restricted to the
specific pathological subtype described below.

Pick's disease (the pathology) causes progressive destruction of nerve cells in the brain
and causes tau proteins in neurons to accumulate into silver-staining, spherical
aggregations known as "Pick bodies" that are a defining characteristic of the disease.[1]

Vascular dementia

Hx is stepwise evolution of cognitive impairment and focal neurological signs, in someone

with appropriate vascular risk factors. Patient also have visuoperpectual tasking deficits.
MRI shows atrophy with diffuse white and frey matter vascular lesions.
Treat with vascular disease and risk factors and aspirin may slow progression.

Dementia with Lewy Bodies

- Causes Parkinson’s
- Typical features
- Fluctuating cognition
- Nocturnal visual hallucinations
- Disturbe REM sleep
- L-DOPA exacerbate pschiatric symptoms, antipsychotics can lead to catastrophic and
potentially fatal decline in motor performance.

Prion Diseases

- Mutated proteins that induce a conformational change in certain constitutive brain

proteins, so producing non-functional, non-metabolizable forms.
- Gradually build up, producing brain dysfunction
- Sporadic and inherited CJD have rapidly progressive dementia with myoclonus, ataxia
and cortical blindness over months.
- EEG shows characteristic repetitive complexes.

Cognitive impairment in younger patients

i.e. other reasons for dementia, esp in young people

- Recreational drugs (glue)

- HIV related dementia
- Cerebral vasculitis
- End-stage MS
- Adrenoleukodystophies
- CADASIL (white matter disease)
- Wilson’s usually before 30yo, neuropsych problems with dystonia of mouth and tongue
assicated with high amplitude limb tremor.
- Uncontrolled epilepsy produces ‘slight twilight phase’, which is when seizures are so
frequent it disallows full recovery in between. Rarely, if they perform repetitive movements,
this is called ‘non-convulsive status’, they may have rapidly progressive dementia. EEG
diagnostic.