Hon--Dementia, Movement Disorders Flashcards
1
Q
Causes of dementia–degenerative
A
- Alzheimers (80%)
- Lewy body disease
- Parkinson’s
- Frontotemporal Lobar degeneration
- Progressive supranuclear palsy
- corticobasilar degeneration
- Multiple systems atrophy
- Huntington’s Disease
- Olivopontocerebellar degeneration
2
Q
Causes of dementia–Vascular
A
- Multiple infarction
- single stroke
- biswanger’s disease (chronic hypertension)
- vasculitis
- subarachnoid hemorrhage
3
Q
Causes of dementia–infectious
A
- fungal meningitis
- syphillis
- AIDS
- Creutzfeldt-Jakob disease
- post-herpes simplex encephalitis
4
Q
Causes of dementia–psychiatric
A
- Depression
- Alcohol abuse
- Drug related
- Personality disorder
- Anxiety disorder
5
Q
Causes of dementia–toxic/metabolic
A
Vitamin B12 deficiency Thyroid deficiency System failure (liver, renal, cardiac, resp) Heavy metals Toxins
6
Q
Causes of dementia–traumatic
A
- Subdural hematoma
- chronic traumatic encephalopathy
- anoxic brain injury
7
Q
Causes of dementia–tumors
A
- glioblastoma/astrocytoma
- lymphoma
- metastatic tumor
8
Q
Diagnosis of Alzheimer’s disease
A
- dementia established by clinical exam and mini mental status exam
- deficits in 2 or more areas of cognition
- progressive worsening of memory and other cognitive functions
- no disturbance of consciousness
- onset–most >65 years of age
- absence of systemic disorders or other brain diseases
9
Q
Supported findings in diagnosis of Alzheimer’s disease
A
- progressive deterioration of cognitive functions
- impaired activities of daily living, altered behavior
- family history
- normal LP
- EEG–normal or generalized slowing
- progressive atrophy! MRI or CT
10
Q
Treatment of Alzheimer’s
A
- Acetylcholinesterase inhibitors (donepezil, rivastigmine, galantamine)
- NMDA R antagonist (memantine)
11
Q
Mild Cognitive impairment (MCI)
A
- memory complaint (often noted by patient–vs Alzheimer’s when patient thinks nothing is wrong)
- abnormal memory for age, but does not meet criteria for dementia–normal cognitive function, normal activities of daily living
- precursors to Alzheimer’s–5x more likely to develop
- treat–AchEl–slow progression to Alzheimer’s
12
Q
criteria for vascular dementia
A
- focal signs on neuro exam
- evidence of cerebrovascular disease
13
Q
Vascular dementia–presence of 1 or more
A
- onset of dementia within 3 months after stroke
- abrupt deterioration in cognitive function
- fluctuating, stepwise progression of cognitive deficits
14
Q
Lewy body–tetrad of symptoms
A
- dementia
- Parkinsonian symptoms (bradykinesia, rigidity, no tremor)
- Prominent psychotic symptoms (visual hallucinations)
- extreme sensitivity to antipsychotic agents
15
Q
Lewy body disease differs from Alzheimer’s how?
A
- progresses more rapidly
- unexplained periods of increased confusion that lasts days to weeks and then is better
- psychotic symptoms much earlier–visual hallucinations–animals, children
16
Q
Classic delusions in Alzheimer’s disease–when?
A
- late in disease!
- husband thinks wife is having an affair
- thinks someone is stealing from them
17
Q
Lewy body disease (vs Parkinson’s)
A
- cortical Lewy bodies
- dementia early in illness
- resting tremor is absent
- autonomic dysfunction prominent
- hallucinations common in absence of antiparkinsonian drugs
18
Q
Parkinson’s disease (vs Lewy body disease)
A
- midbrain Lewy bodies
- dementia late in disease
- resting tremor
- autonomic dysfunction only sometimes
- hallucinations only in response to antiparkinsonian drugs
19
Q
Frontotemporal degeneration
A
-dementia–deterioration of social skills and changes in personality, with impairment of intellect, memory and language
20
Q
Frontotemporal degeneration–core symptoms
A
- loss of memory
- lack of spontaneity
- difficulty in thinking or concentrating
- disturbances of speech
21
Q
Frontotemporal degeneration–onset, progression
A
- ages 40-60
- 2-10 years
22
Q
Normal pressure hydrocephalus–triad
A
- dementia
- gait disturbances
- urinary incontinence
- potentially reversible with ventriculoperitoneal shunting (gait disturbance)
23
Q
CADASIL’s disease–onset, manifests as?
A
(Cerebral Autosomal Dominant Arteriopathy with subcortical infarcts and Leukoencephalopathy)
- 40-50 years of age
- heriditary stroke disorder–NOTCH3
- degeneration of smooth m cells in BVs
- migraine headaches and TIA’s or strokes
- MRI–multiple areas of ischemia prior to onset of symptoms
- progresses to dementia
24
Q
Bradykinetic disorders
A
- most common–Parkinsonism
- idiopathic Parkinson’s disease (most common)
- postencephalitic
- toxin-induced
- MPTP (meperidine analog)
25
Parkinson's disease--cardinal features
- tremor (resting tremor, unilateral at 1st, pill rolling, mouth/chin tremor)
- rigidity
- bradykinesia
26
Parkinson's disease--other features
- postural instability
- hypotonia
- hypo mimic (masked fancies)
- decreased eye blinking
- gait disturbance--slow, stooped forward, small steps
- dysautonomia
- many develop dementia
27
Other Akinetic Rigid Syndromes
PSP
MSA
CBD
28
Progressive Supranuclear Palsy
Bradykinesia and rigidity
| Loss of voluntary control of eye movements (vertical gaze)
29
Multiple Systems Atrophy (MSA)
- bradykinesia and rigidity
| - pronounced autonomic dysfunction
30
Cortical Basal Degeneration (CBD)
- cortical and basal ganglionic dysfunction
- bradykinesia and rigidity
- cortical sensory loss, apraxia, myoclonus, aphasia (cortical functions)
31
Chorea
Brief, dance like movements
32
Writing, sinuous movements (often in combo with chorea)
Athetosis
33
Dystonia
Sustained muscle contractions that produce twisting and repetitive movements and abnormal postures
-cervical--Classic
34
Ballism
-large amplitude, flinging movement
35
Tic
-brief, rapid, repetitive, purposeless that may involve single or multiple muscle groups
36
Huntington's disease onset
- 30-50 years of age
| - lifespan--15 years after onset
37
Sydenham's chorea
- previous infection with group A hemolytic streptococcus
- may be a form of arterteritis
- unilateral choreiform movements, can be confused for restlessness or fidgeting
- treatment--bed rest, antibiotics
38
Idiopathic torsion dystonia
- dystonia movements and postures without other signs
| - remains throughout life
39
Idiopathic torsion dystonia--clinical findings
- Torticollis (neck twisted)
- blepharospasm (forced closure of eyelids)
- oromandibular dystonia (spasm of muscles around mouth)
- arm--hyperpronated position, wrist flexed, fingers extended
- leg--extension, pronation and inversion of foot
40
Focal torsion dystonia
Dystonia confined to focal area
- blepharospasm, oromandibular dystonia, spasmodic Torticollis
- writer's cramp (dystonic posturing of hand and forearm when used for specific task)
41
Focal torsion dystonia--medication
BOTOX
42
Wilson's disease--onset, organ dysfunction, clinical features
- decreased binding of copper to cerruloplasmin
- childhood or young adult life!
- neurological and hepatic dysfunction!
- bradykinetic and hyperkinetic!
43
Wilson's disease--clinical features
- bradykinetic and hyperkinetic!!
- tremor
- choreiform
- rigidity
- bradykinesia
- dysarthria
- ataxia
- personality changes, dementia, psychosis if not caught!
44
Wilson's disease--diagnosis
- increased copper excretion in urine
- decreased cerruloplasmin levels
- kayser-fleischer ring
45
Wilson's disease--treatment
- penicillamine
| - restriction of dietary copper
46
Tic disorders
- single motor tics
| - Gilles de La Tourette's syndrome--multiple motor and vocal tics with onset before age 21
47
Gilles De La Tourette's syndrome
- sporadic, males
- vocal tics--barks, hisses, grunts, throat clearing, coughing
- may also see:
- coprolalia (vulgar speech)
- echolalia (parroting speech of others)
- echopraxia (imitation of other's movements)
- palilalia (repition of words)
48
Essential Tremor (benign familial tremor)--clinical presentation
- postural or kinetic tremor of both hands, may involve head or voice
- often not until later in life
- progresses slowly
- social embarrassment
- alcohol--decreases tremor
49
Essential tremor--treatment
- B blockers
- Primidone
- Benzodiazepines
50
Dementia definition
- decline in memory and at least 1 other cognitive function
| - decline impairs social or occupational functional in comparison with previous functioning
