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Nervous System II > Degenerative Disorders > Flashcards

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
Q

Mixed Dementias - 2 examples

A

Multi-infarct Dementia

Creutzfeldt-Jakob Disease

26
Q

Multi-infarct dementia

A

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
Q

Creutzfeldt-Jakob Disease - Mechanism

A

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
Q

CJD - Clinical features

A

Patients present with dementia that progresses over weeks to months, often with confusion, psychotic features, and myoclonus; death occurs within 4-12 months

29
Q

Treatment of dementia

A

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
Q

Lewy Body Dementia

A

Characterized by dementia, parkinsonism, visual hallucinations, and confusion

31
Q

Binswanger’s Disease

A

Caused by long standing hypertension leading to the development of white matter ischemia

Dementia is slowly progressive

32
Q

Frontotemporal Dementia - Pathology

A

Abnormal tau metabolism
Neurofibrillary Tangles
Ubiquitin inclusions

Nervous System II (12 decks)

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