delirium and dementia

Question 1

Delirium vs dementia (time course, conciousness, attention, memory, speech/language, toxic and metabolic causes, reversibility

Answer 1

Delirium: acute, fluctuating levels of conciousness, impaired attention, poor registration of memory, incoherent speech, toxic and metabolic causes typical, commonly reversible. Dementia: chronic, normal levels of conciousness, normal attention, amnesia, aphasia, toxic and metabolic causes unusual, reversibility is uncommon.

Question 2

1. Define the syndrome of delirium.

Answer 2

rapidly developing disorder of attention characterized by an inability to maintain a coherent line of thought. Usually hypoaroused, but can be hyperaroused

Question 3

Alternate names for delirium

Answer 3

acute confusional state, favored by many because it emphasizes the acute nature of the syndrome and the prominence of the attentional disorder, and toxic-metabolic encephalopathy, which highlights the most common etiologies.

Question 4

Delirium pathophys

Answer 4

disruption of normal brain homeostasis. Neuronal dysfunction is widespread, affecting arousal systems in the brainstem and diencephalon as well as cortical regions, but the most vulnerable neurons are thought to be those involved in cholinergic, dopaminergic, histaminergic, noradrenergic, and serotonergic neurotransmission. If the insult causing delirium is corrected within a few days, normal brain function can usually be restored, but a prolonged insult may damage neurons irreversibly and limit recoverability.

Question 5

Delirium differential diagnosis

Answer 5

Aphasia/Wernicke’s aphasia (resembles delirum b/c impaired speech and language, but aphasia usually follows a stroke). Schizophrenia and other psychotic diseases can mimic delirium, but there is typically no fluctuation in level of consciousness. In both mania and depression, prominent affective features are typical, but no fluctuation in level of consciousness.

Question 6

Etiology of delirium

Answer 6

Drugs/toxins (prescription meds, OTC, recreational), intoxication and withdrawal, Metabolic disorders, infectious/inflammatory disease ( meningitis, encephalitis, vasculitis, systemic infection), TBI, stroke, seizure disorders,

Question 7

evaluation of delirium

Answer 7

History/PE, MSE is NOT necessary b/c patient is too confused, CMP, urinalysis, urine toxicology, EKG, chest radiograph, CT/MRI of brain, lumbar puncture if infection is suspected, EEG (for status epilepticus)

Question 8

Treatment of delirium

Answer 8

Treat the cause, manipulations such as providing a clock and calendar, provision of adequate sleep and restoration of the sleep-wake cycle, and judicious use of calming medications such as the atypical neuroleptics.

Question 9

3. Define the syndrome of dementia.

Answer 9

acquired and persistent impairment in intellectual function with deficits in at least three of the following areas - memory, language, visuospatial skills, emotion and personality, and complex cognition - that is sufficient to interfere with usual social and occupational activities. By definition, dementia is neither progressive nor irreversible.

Question 10

4. Discuss the reversible etiologies of dementia.

Answer 10

Reversible (10-20%): drugs/toxins, mass lesions, normal pressure hydrocephalus, hypothyroidism, Vitamin B12 deficiency, neurosyphilis, CNS inflammatory dz, mild TBI.

Question 11

4. Discuss the irreversible etiologies of dementia.

Answer 11

Irreversible (80-90%): Alzheimers, frontotemporal dementia, vascular dementia, huntingtons, parkinsons, lewy body dementia, Creutzfeldt-jakobs, MS, HIV, Severe TBI

Question 12

Evaluation of dementia

Answer 12

Detect reversible causes! CBC, CMP, TSH, B12, RPR, MRI/CT, lumbar puncture, EEG, HIV, ESR, Abs for autoimmune

Question 13

Types of cortical dementia

Answer 13

Alzheimers dz and frontotemporal dementia

Question 14

Survival after onset of Alzheimers

Answer 14

6-12 yrs

Question 15

Alzheimers clinical stages

Answer 15

stage I (1-2 years), when amnesia is most notable; stage II (2-10 years), when dementia is obvious; and stage III (8-12 years), when the patient has severe mental and physical incapacity

Question 16

Mild cognitive impairment and relationship to Alzheimers

Answer 16

Mild cognitive impairment (MCI) is a transitional stage between normal aging and AD; memory complaints, normal general cognitive function and intact daily living activities. No dementia yet. in a person over 65 with MCI, the conversion rate to AD is 10-15% per year as opposed to 1-2% without MCI.

Question 17

Alzheimers neuropath

Answer 17

Cerebral atrophy (loss of cortical neurons/synapses), damage to limbic and association cortices, neuritic (amyloid) plaques and neurofibrillary tangles in neocortex and hippocampus (occur in normal aging, but less dramatically)

Question 18

Alzheimers etiology

Answer 18

Amyloid precursor protein mutations on chrom 21 (or overexpression in down syndrome), Presenilin-1 mutations on chrom 14 or presenilin-2 mutation on chrom 1 are linked to early onset AD (these mutations only account for 1% of all cases). Late onset cases often have epsilon-4 allele of apolipoprotein E gene

Question 19

Cholinergic hypothesis for AD

Answer 19

acetylcholine is relatively deficient in AD, and loss of cholinergic cells in the basal forebrain is correlated with cognitive impairments in AD

Question 20

Treatment of Alzheimers

Answer 20

Donepezil, rivastigmine, and galantamine are cholinesterase inhibitors approved for AD. memantine is an NMDA antagonist that is available as a neuroprotective agent. Standard drug treatment for AD now includes a cholinesterase inhibitor and memantine.

Question 21

Frontotemporal Dementia presentation, brain regions affected, differential diagnosis

Answer 21

Aka Picks disease. Presents as changes in behavior and comportment, not memory. disinhibition, apathy, and executive dysfunction. Degenerative dz of frontal and temporal lobes, sparing hippocampus until later in dz. Many are misdiagnosed with psychiatric dz (ie. bipolar)

Question 22

What are subcortical dementias and give examples

Answer 22

diseases in which subcortical gray matter structures (basal ganglia, thalamus, brainstem nuclei) bear the major burden of neuropathology. All of these disorders feature some variety of abnormal movement in addition to dementia. Parkinsons and huntingtons are examples

Question 23

Parkinsons presentation, histology, treatment

Answer 23

resting tremor, bradykinesia, rigidity, and postural instability. Dementia develops in up to 80%. Lewy bodies in the substantia nigra and dopamine deficiency due to loss of dopaminergic cells in midbrain substantia nigra. Standard PD drugs may have a modest effect on cognition, and cholinesterase inhibitors may also help

Question 24

Lewy Body Dementia symptoms, treatment

Answer 24

characterized by dementia, parkinsonism, visual hallucinations, and fluctuating confusion. Cholinesterase inhibitors can help the dementia of LBD, but this disease is difficult to treat because PD drugs may worsen psychosis while neuroleptics may worsen parkinsonism

Question 25

Huntingtons gnetics, symptoms, physiology, diagnosis, treatment

Answer 25

Autosomal dominant. Dementia or chorea may be presenting, plus premonitory personality changes, and many neuropsychiatric features. Caudate atrophy. Diagnosed by genetic testing for CAG repeat. Treated with neuroleptic drugs and tetrabenzine for chorea, but no drugs available for dementia

Question 26

Examples of white matter dementia

Answer 26

genetic, demyelinative, infectious, inflammatory, toxic, metabolic, traumatic, Binswangers disease and normal pressure hydrocephalus

Question 27

Binswangers disease description/cause, clinical profile

Answer 27

vascular dementia in which long-standing hypertension leads to the development of ischemia and lacunar infarction that falls heavily on the cerebral white matter. Seen as slowly progressive dementia, apathy, confusion, depression, gait disorder, rigidity, incontinenc

Question 28

Binswangers disease pathology

Answer 28

White matter volume loss, especially in periventricular regions > hydrocephalus ex vacuo, Thickened and hyalinized penetrating arterioles (“small vessels”), Ischemic demyelination, mainly of association fibers, sparing U fibers, “Incomplete infarction” of white matter, Cerebral cortex is essentially normal

Question 29

Normal pressure hydrocephalus presentation

Answer 29

present with reversible dementia, gait disorder, and urinary incontinence, and have enlarged ventricles with normal sulci on neuroimaging indicating hydrocephalus and neuropathology affecting the periventricular white matter

Question 30

What procedure improves gait and sometimes cognition in normal pressure hydrocephalus

Answer 30

high volume lumbar tap test (30-50cc) then ventriculo-peritoneal, ventriculo-atrial, and lumboperitoneal shunts show some improvement
high volume lumbar tap test (30-50cc) then ventriculo-peritoneal, ventriculo-atrial, and lumboperitoneal shunts show some improvement

Question 31

Multi-infarct dementia cause, symptoms, treatment

Answer 31

A series of sequential strokes erodes cognitive function so that eventually, when enough brain tissue is destroyed, dementia appears. These strokes can be large vessel ischemic infarcts affecting the cerebral cortex or small vessel lacunar infarcts involving subcortical gray and white matter. The classic course is that of step-wise deterioration of function, with progressively more cognitive loss evident after each new stroke. The obvious treatment is to prevent strokes from occurring at all.

Question 32

Creutzfeldt-Jakob Disease cause, presentation, prognosis,

Answer 32

rapidly progressive, always fatal, and potentially transmissible. Human prion disase (most are sporadic, some are familial). Infectious causes are rare but have been found with transplants. Present with dementia progressing over weeks-months, with confusion or psychotic features, myoclonus, cortical or subcortical hyperintensities on diffusion weighted MRI. Death usually within 4-12months.

Question 33

Variant Creutzfeldt-Jakob Disease

Answer 33

human bovine spongiform encephalopathy or “mad cow disease” is a very rare prion disease acquired by consuming infected English beef, and recently a few cases have occurred after blood transfusion. vCJD has an earlier age of onset and a slightly longer course than CJD

Question 34

Treatment of irreversible dementia

Answer 34

Avoid benzodiazepines and anticholinergic drugs which make mental status worse, use lose dose atypical antipsychotic drugs (e.g. quetiapine or risperidone) for disruptive neuropsychiatric syndromes, a trial of an antidepressant (SSRI) treatment if depression is significant

Question 35

Treatment of Alzheimers

Answer 35

Cholinesterase inhibitors: Tacrine (Cognex), donepezil (Aricept), rivastigmine (Exelon), and galantamine (Reminyl, Razadyne); all these drugs have been shown to have similar efficacy, but donepezil is often better tolerated, and is easier to use because of once daily dosing. Rivastigmine can now be given transdermally to reduce GI side effects. Memantine (Namenda) is an NMDA antagonist with similar efficacy that can be used in conjunction with a cholinesterase inhibiton