Degenerative Disorders Flashcards

1
Q

Delirium - Definition

A

AKA acute confusional state, toxic-metabolic encephalopathy

An acute disorder of attention characterized by an inability to maintain a coherent line of thought; reflects diffuse brain dysfunction related to a disruption of normal brain homeostasis, affecting arousal systems in the brainstem, diencephalon, and cortical regions

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2
Q

Delirium - etiology

A
Drugs - Intoxication and withdrawal
Environmental toxins 
Metabolic disorders
Infection
Inflammation
Structural lesions - TBI, stroke
Seizure disorders
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3
Q

Evaluation of delirium

A

History
Physical exam, including neurologic exam (no MSE)
Labs - CMP, CBC, urinalysis, urine toxicology
ECG, Chest X Ray
CT/MRI of brain

LP if brain infection is suspected
EEG if non-convulsive status epilepticus is suspected

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4
Q

Dementia - definition

A

An acquired and persistent impairment in intellectual function with deficits in at least 3 areas - memory, language, visuospatial skills, emotion, personality, and complex cognition - which interferes with social and occupational activities

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5
Q

Reversible causes of dementia

A

Account for 10-20% of dementias

Drugs & Toxins 
Mass lesions 
Hypothyroidism
Vitamin B12 deficiency
Neurosyphilis 
CNS inflammatory diseases
Severe depression 
Mild TBI
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6
Q

Irreversible Causes of dementia

A

Accounts for 80-90% of dementias

Alzheimer's 
Frontotemporal Dementia (FTD)
Huntington's Disease 
Parkinson's Disease 
Lewy Body Dementia
Creutzfeldt-Jakob Disease
Multiple Sclerosis 
HIV-associated dementia
Severe TBI
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7
Q

Evaluation of dementia

A

History
Physical, including neurologic exam and MSE
Labs - CMP, CBC, TSH, B12, RPR (syphilis)
Imaging - MRI or CT of brain

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8
Q

Cortical Dementia - 2 examples

A
Alzheimer's Disease
Frontotemporal Dementia (FTD)
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9
Q

Alzheimer’s Disease - Clinical features

A

Most common cause of dementia in older people (50-70% of cases); survival after onset is 6-12 years

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10
Q

Stages of Alzheimer’s Disease

A

Stage I (1-2 years): Notable amnesia

Stage II (2-10 years): Obvious dementia

Stage III (8-12 years): Severe mental and physical incapacity

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11
Q

Mild Cognitive Impairment (MCI)

A

A transitional stage between normal aging and AD; in a person over 65 with MCI, the conversion rate to AD is 10-15% per year

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12
Q

Alzheimer’s - Neuropathology

A

Gross - cerebral atrophy, reflecting loss of cortical neurons and synapses

Microscopic - neuritic (amyloid) plaques and neurofibrillary tangles

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13
Q

Genetic risk for early onset Alzheimer’s

A

Down syndrome (via trisomy of APP gene on chromosome 21); other mutations of APP

Mutations in Presenilin-1 on Chromosome 14

Mutations in Presenilin-2 on Chromosome 1

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14
Q

Genetic Risk - Late onset Alzheimer’s

A

Epsilon-4 allele of the apolipoprotein E (APOE) gene

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15
Q

Alzheimer’s - Treatment

A

Acetylcholinesterase inhibitors - Donepezil, Rivastigmine, Galantamine

NMDA antagonist - Memantine

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16
Q

Frontotemporal Dementia

A

A degenerative disease of the frontal and temporal lobes; patients typically present with disinhibition, apathy, and impairment of executive function with sparing of memory until late in the course of the disease

17
Q

Subcortical dementias - 2 examples

A

Parkinson’s Disease
Huntington’s Disease

Subcortical dementias affect the deeper gray matter structures (basal ganglia, thalamus, brainstem) and so they feature some variety of movement disorder in addition to dementia

18
Q

Parkinson’s Disease - Clinical characteristics

A
Resting tremor
Bradykinesia 
Rigidity
Postural instability
80% develop dementia
19
Q

Parkinson’s Disease - Pathology

A

Lewy bodies in the substantia nigra
Abnormal synuclein protein
Dopamine deficiency

20
Q

Huntington’s Disease - Clinical characteristics

A

Dementia
Chorea
Personality Changes
Neuropsychiatric features

21
Q

Huntington’s Disease - Genetics & Pathology

A

Autosomal dominant inheritance of an abnormal CAG trinucleotide repeat in the HTT gene; genetic testing is available

Imaging shows caudate atrophy

22
Q

White matter dementias - Examples

A

Binswanger’s Disease

Normal pressure hydrocephalus

23
Q

Binswanger’s Disease - Mechanism

A

Long-standing hypertension leads to ischemia and lacunar infarction of cerebral white matter; dementia is slowly progressive

24
Q

Normal Pressure Hydrocephalus (NPH)

A

Imaging shows enlarged ventricles with normal sulci, indicating hydrocephalus and neuropathology affecting the periventricular white matter

Presents with dementia, gait disorder, and urinary incontinence

25
Mixed Dementias - 2 examples
Multi-infarct Dementia | Creutzfeldt-Jakob Disease
26
Multi-infarct dementia
Caused by a series of sequential strokes which erode cognitive function in a step-wise fashion until dementia appears; infarcts may be large vessel (affecting the cerebral cortex) or small vessel lacunar infarcts (affecting the subcortical gray and white matter)
27
Creutzfeldt-Jakob Disease - Mechanism
Caused by a proteinaceous infectious particle (prion); 85-90% are sporadic and most of the rest are familial; infectious causes are rare but include transmission by dura mater grafts and neurosurgical instruments
28
CJD - Clinical features
Patients present with dementia that progresses over weeks to months, often with confusion, psychotic features, and myoclonus; death occurs within 4-12 months
29
Treatment of dementia
Treatment of underlying, reversible causes Avoidance of drugs that worsen mental status (benzos, anticholinergics) Low-dose atypical anti-psychotics (quetiapine, risperidone) for neuropsychiatric syndromes SSRI treatment for depression
30
Lewy Body Dementia
Characterized by dementia, parkinsonism, visual hallucinations, and confusion
31
Binswanger's Disease
Caused by long standing hypertension leading to the development of white matter ischemia Dementia is slowly progressive
32
Frontotemporal Dementia - Pathology
Abnormal tau metabolism Neurofibrillary Tangles Ubiquitin inclusions