BMB 2 - Dementia; Movement Disorders Flashcards
The left hippocampus is more responsible for which aspects of memory?
Language / verbal memory
The right hippocampus is more responsible for which aspects of memory?
Visuospatial memory
Which brain structure is largely responsible for linguistic and verbal memory?
The left hippocampus
Which brain structure is largely responsible for visuospatial memory?
The right hippocampus
Which brain structure is largely responsible for emotional memory?
The amygdala
Which brain structure is largely responsible for procedural memory?
The basal ganglia and cerebellum
Name two memory-processing structures connected by the fornix.
The hippocampus and the mamillary bodies
Which neural loop involves the hippocampus and is largely responsible for episodic, declarative memory?
The Papez circuit
Which neural loop involves the amygdala and is largely responsible for emotional memory?
The Yakolev circuit
What are the major structures of one hemisphere of the Papez neural loop?
The cingulate gyrus, parahippocampal gyrus, hippocampus, fornix, and mamillary body
What are the major structures of one hemisphere of the Yakolev neural loop?
Tha amygdala, thalamus, and prefrontal/temporal cortices
(the hypothalamus and brainstem also have input)
What is the most important risk factor for Parkinson’s disease?
Age
You tap on a patient’s forhead between their eyes (on their glabella); they blink slowly and late.
What is this called?
The Myerson (or glabella) sign
(indicates Parkinson’s)
What percentage of Parkinson’s disease patients have depression?
50%
Name some of the S/Sy of Parkinson’s disease.
Gait disturbance;
resting tremor;
depression;
Myerson (glabella) sign;
bradykinesia;
cogwheel rigidity;
cognitive impairment;
pin-wheel tremor;
postural instability;
ANS dysfunction
What is the major etiology of Parkinson’s disease?
Degeneration of the substantia nigra pars compacta
What is the classic triad of Parkinson’s disease?
Resting tremor
Bradykinesia
Rigidity
Parkinson’s disease has close associations with which form of neuropathology and dementia?
Lewy body
Are Lewy bodies present in Parkinson’s disease?
Yes, in the substantia nigra pars compacta
Where within the cell are Parkinsonian Lewy bodies found?
As intranuclear deposits
Parkinsonian symptoms with early-onset dementia is likely _______________.
Parkinsonian symptoms with late-onset dementia is likely _______________.
Parkinsonian symptoms with early-onset dementia is likely Lewy body dementia.
Parkinsonian symptoms with late-onset dementia is likely Parkinson’s disease.
What are some of the general pharmacological treatment options for Parkinson’s disease?
Levodopa
Dopamine agonists
Amantadine
MAO inhibitors (monoamine oxidase)
CMOT inhibitors (catechol-o-methyl transferase)
(also therapy and deep-brain stimulation)
What is the general pharmacological treatment for Lewy body dementia?
Cholinesterase inhibitors
Alzheimer’s disease most severely damages which portions of the brain?
The hippocampus;
the posterior cortical areas
Alzheimer’s disease is associated with a loss of which neurotransmitter from which nucleus?
Acetylcholine;
the basal nucleus of Meynert
Alzheimer’s disease is associated with what neuropathology?
Tau neurofibrillary tangles and beta-amyloid plaques
What is the most important risk factor for Alzheimer’s disease?
Age
What are the genetics of Alzheimer’s disease?
APoE4 on chromosome 19 increases risk
(APoE2 is protective)
Early-onset Alzheimer’s disease has an onset before what age?
65 years
(usually in one’s 40s or 50s)
What are the genetics of early-onset, familial Alzheimer’s disease?
APP, PSEN1, PSEN2
What is the mode of inheritance of familial (early-onset) Alzheimer’s disease?
Autosomal dominant
Most cases of Alzheimer’s disease are ____________ (sporadic/familial).
Most cases of Alzheimer’s disease are sporadic (>95%).
True/False.
Most cases of both Parkinson’s disease and also Alzheimer’s disease are sporadic and not likely to be inherited.
True.
What is the most common form of dementia?
And second?
Alzheimer’s disease (40%)
Vascular dementia (20%)
True/False.
Vascular dementias can result due to both large-vessel multiinfarcts or chronic small-vessel disease.
True.
Describe some of the features of vascular dementia.
Symptoms of Alzheimer’s disease
+
chronic microvascular white matter changes
Describe some of the features of frontotemporal dementia (Pick’s disease).
Associated with chromosome 17 abnormalities
Pick bodies in frontal and temporal lobes
Loss of inhibition
Progressive aphasia
Describe some of the features of Lewy body dementia.
Corticobasilar degeneration
Cognitive and motor symptoms
Mild memory loss, executive functioning problems, magnetic apraxia
What part of the brain routes input from the cerebellum and basal ganglia to the cortex for control of motor movement?
The ventral lateral thalamus
What part of the brain is targeted by deep-brain stimulation (e.g. for Parkinson’s disease or essential tremor)?
The ventral lateral thalamus
Describe the typical etiology and presentation of Huntington’s disease.
CAG repeats on chromosome 4 –>
Accumulation of abnormal huntingtin protein –>
Degeneration of the caudate –>
Loss of motor inhibition by basal ganglia –>
Chorea / Hemiballismus / Depression / Dementia
The motor S/Sy of Huntington’s disease largely result due to deposition of the ___________ protein in the medium _________ neurons of the caudate nucleus.
The motor S/Sy of Huntington’s disease largely result due to deposition of the huntingtin protein in the medium spiny neurons of the caudate nucleus.
What is the mode of inheritance of Huntington’s disease?
Autosomal dominant
Name the relevant, respective symptomatic treatment(s) for each of the following aspects of Huntington’s disease:
Chorea —
Depression —
Psychosis —
Cognition —
Name the relevant, respective symptomatic treatment(s) for each of the following aspects of Huntington’s disease:
Chorea — VMAT2 inhibitors (e.g. tetrabenazine) (decreasing dopamine)
Depression — SSRIs
Psychosis — Antipsychotics
Cognition — No known treatment
What are some of the general symptomatic treatments for dementia?
Acetylcholinesterase inhibitors: donepezil, galantamine, and rivastigmine
N-methyl-D-aspartate antagonists: memantine
Diet, exercise, sleep
Alzheimer’s disease alone is responsible for _____% of cases of dementia.
Vascular dementia alone is responsible for _____% of cases of dementia.
Frontal and mixed dementias (e.g. Pick’s, Lewy body, progessive aphasia, etc.) are responsible for _____% of cases of dementia.
Other types (e.g. Parkinson’s, TBIs, toxins, infections, metabolic issues, etc.) are responsible for _____% of cases of dementia.
Alzheimer’s disease alone is responsible for 40 % of cases of dementia.
Vascular dementia alone is responsible for 20 % of cases of dementia.
Frontal and mixed dementias (e.g. Pick’s, Lewy body, progessive aphasia, etc.) are responsible for 25 % of cases of dementia.
Other types (e.g. Parkinson’s, TBIs, toxins, infections, metabolic issues, etc.) are responsible for 15 % of cases of dementia.
What is a tic?
A premonitory urge to move
What is the definition of chronic tic disorder?
Either ≥ 1 motor or ≥ 1 verbal tics that began before age 18 and are present for ≥ 1 year
Define Tourette’s syndrome.
A tic disorder with onset of symptoms before 18 years of age;
≥ 2 motors tics and ≥ 1 vocal/phonetic tics throughout the overall course of the disease
What are the major comorbidities associated with Tourette’s syndrome?
OCD and ADHD
What are the phonetic tics often seen in Tourette’s syndrome?
Throat clearing, coughing, grunting, anything that makes sounds
Coprolalia: shouting of obscenities (only 5-15% of patients)
What are the motor tics often seen in Tourette’s syndrome?
Copropraxia: obscene movements
Name some of the treatments available for reducing the burden of tic disorders generally (and Tourette’s syndrome specifically).
Agents which deplete or block dopamine
Clonazepam
Botulinum toxin injections
Describe essential tremor.
A familial, bilateral movement disorder
Does alcohol help or exacerbate the involuntary motor movement of essential tremor?
Help
Name some of the treatments used for managing essential tremor.
Topiramate (first-line)
Propranolol (not for the elderly)
Primidone
Deep brain stimulation
How does cerebellar ataxia typically present?
Slow, progressive worsening of symptoms over the years involving speech, swallowing, coordination, gait, or ataxia
How is cerebellar ataxia managed?
Only by treating underlying causes
(e.g. repleting vitamin deficiency, giving steroids for inflammatory conditions, treating underlying cancers, etc.)
What is generically meant by the term ‘dystonia?’
Sustained, abnormal muscle contractions
(e.g. as an adverse reaction of antipsychotic use)
Name some of the adverse motor effects of antipsychotic use.
Dystonia
Akathisia
Tardive dyskinesia
How is generalized dystonia treated?
How about acute focal dystonia?
How about dystonia due to antipsychotic use?
Baclofen
Botulinum toxin
IV anticholinergics
How are tardive syndromes (i.e. dystonia, akathisia, dyskinesia) treated?
Removal of the offending agent (usually an antipsychotic);
tetrabenazine;
symptomatic management
