Notes in L30 Neuropath 3 (Neurodengenerative disease)

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Published	09/09/2023	What class of diseases is characterized by anticipation?{{c1::Trinucleotide repeat diseases (e.g. Huntington disease)}}
Published	09/09/2023	What class of diseases is Huntington disease a part of?{{c1::Trinucleotide repeat expansion diseases}}
Published	09/09/2023	What sequence of nucleotides is repeated in Huntington disease?{{c1::CAG}}
Published	09/09/2023	Do upper motoneuron lesions present with weakness? {{c1::Yes}}
Published	09/09/2023	Do upper motoneuron lesions typically present with atrophy? {{c1::No*}}
Published	09/09/2023	Do upper motoneuron lesions present with fasciculations? {{c1::No}}
Published	09/09/2023	Do upper motoneuron lesions present with the Babinski sign? {{c1::Yes}}
Published	09/09/2023	Do upper motoneuron lesions present with clasp knife spasticity? {{c1::Yes}}
Published	09/09/2023	Do lower motoneuron lesions present with weakness? {{c1::Yes}}
Published	09/09/2023	Do lower motoneuron lesions present with atrophy? {{c1::Yes}}
Published	09/09/2023	Do lower motoneuron lesions present with fasciculations? {{c1::Yes}}
Published	09/09/2023	Does amyotrophic lateral sclerosis (ALS) damage upper or lower motoneurons?{{c1::Both :)}}
Published	09/09/2023	Amyotrophic lateral sclerosis (ALS) typically begins at {{c1::cervical}} levels of the spinal cord
Published	09/09/2023	ALS presents with asymmetric {{c2::flaccid}} paralysis in the {{c1::upper}} limbs
Published	09/09/2023	ALS presents with asymmetric {{c2::spastic}} paralysis in the {{c1::lower}} limbs
Published	09/09/2023	Does ALS present with symmetric or asymmetric weakness?{{c1::Asymmetric}}
Published	09/09/2023	An early sign of ALS is atrophy and weakness of {{c1::hands}}
Published	09/09/2023	What sensory deficits does ALS present with?{{c1::None :)}}
Published	09/09/2023	What bladder/bowel deficits does ALS present with? {{c1::None :)}}
Published	09/09/2023	Amyotrophic lateral sclerosis (ALS) is commonly known as {{c1::Lou Gehrig}} disease
Published	09/09/2023	Most cases of ALS are sporadic and arise in {{c1::middle-aged adults::age group}}
Published	09/09/2023	Familial cases of {{c2::ALS}} may be associated with {{c1::zinc-copper superoxide dismutase 1 (SOD1)}} mutations, which leads to free radical injury i…
Published	09/09/2023	What drug is used to treat ALS? {{c1::Riluzole}}
Published	09/09/2023	What disease is associated with this spinal cord lesion? {{c1::Amyotrophic lateral sclerosis (ALS)}}
Published	09/09/2023	Which part of the brain initiates and provides gross control over skeletal muscle movement? {{c1::Basal ganglia}}
Published	09/09/2023	One component of the basal ganglia is the {{c1::striatum}}, which consists of the {{c2::putamen::motor}} and {{c3::caudate::cognitive}}
Published	09/09/2023	In both the direct and indirect basal ganglia pathways, neurons from the cerebral cortex lead to {{c2::excitation}} of neurons of the {{c1::striatum}}
Published	09/09/2023	What neurotransmitter is released by the motor cortex in the basal ganglia pathway (direct and indirect)? {{c1::Glutamate (excitatory)}}
Published	09/09/2023	In the direct basal ganglia pathway, the neurons of the striatum lead to {{c2::inhibition}} of neurons of the {{c1::globus pallidus internus}}
Published	09/09/2023	What neurotransmitters are released by the striatum in the direct basal ganglia pathway? {{c1::GABA and Substance P (Inhibitory)::2}}
Published	09/09/2023	What neurotransmitter is released by the globus pallidus internus in the basal ganglia pathway (direct and indirect)? {{c1::GABA (Inhibitory)}}
Published	09/09/2023	In the indirect basal ganglia pathway, the neurons of the striatum lead to {{c2::inhibition}} of neurons of the {{c1::globus pallidus externus}}
Published	09/09/2023	What neurotransmitters are released by the striatum in the indirect basal ganglia pathway? {{c1::GABA and Enkephalin (Inhibitory)::2}}
Published	09/09/2023	In the indirect basal ganglia pathway, inhibition of the globus pallidus externus leads to {{c2::excitation}} of neurons of the {{c1::subthalamic nucl…
Published	09/09/2023	What neurotransmitter is released upon stimulation of the globus pallidus externus (basal ganglia pathway)? {{c1::GABA (Inhibitory)}}
Published	09/09/2023	In the indirect basal ganglia pathway, the neurons of the subthalamic nucleus lead to {{c2::excitation}} of neurons of the {{c1::globus pallidus inter…
Published	09/09/2023	What neurotransmitter is released by the subthalamic nucleus in the indirect basal ganglia pathway? {{c1::Glutamate (Excitatory)}}
Published	09/09/2023	Dopamine excites the {{c1::direct}} basal ganglia pathway through {{c2::D1}} receptors
Published	09/09/2023	Dopamine inhibits the {{c1::indirect}} basal ganglia pathway through {{c2::D2}} receptors
Published	09/09/2023	Multiple sclerosis is more commonly seen in caucasians in regions {{c1::away from::towards or away from}} the equator
Published	09/09/2023	Degeneration of the neurons of the {{c1::cerebral cortex}} leads to dementia (e.g. Alzheimer's)
Published	09/09/2023	Degeneration of the neurons of the brainstem and {{c1::basal ganglia}} leads to movement disorders (e.g. Parkinson's, Huntington's)
Published	09/09/2023	{{c1::Dementia}} is defined as decreased cognitive ability, memory, or function with intact consciousness
Published	09/09/2023	What is the most common cause of dementia in the elderly? {{c1::Alzheimer disease}}
Published	09/09/2023	Alzheimer disease presents with {{c1::slow::speed}}-onset memory loss, beginning with short-term and progressing to long-term loss
Published	09/09/2023	Are focal neurologic deficits seen in early Alzheimer disease? {{c1::No}}
Published	09/09/2023	Over 90% of Alzheimer disease cases are {{c1::sporadic::sporadic or familial}}
Published	09/09/2023	The {{c2::ε4}} allele of apolipoprotein E is associated with {{c1::increased}} risk for Alzheimer disease
Published	09/09/2023	The {{c2::ε2}} allele of apolipoprotein E is associated with {{c1::decreased}} risk for Alzheimer disease
Published	09/09/2023	Familial cases of Alzheimer disease (10%) are characterized by an {{c1::earlier}} age of onset (relative to sporadic cases)
Published	09/09/2023	Familial cases of Alzheimer disease are associated with mutations in {{c1::presenilin-1}} and {{c1::presenilin-2}}
Published	09/09/2023	Alzheimer disease is characterized by widespread cortical {{c1::atrophy}}
Published	09/09/2023	Alzheimer disease is characterized by {{c1::widening}} of sulci
Published	09/09/2023	Alzheimer disease is characterized by {{c1::narrowing}} of gyri
Published	09/09/2023	What type of hydrocephalus may be seen with Alzheimer disease? {{c1::Hydrocephalus ex vacuo}}
Published	09/09/2023	Alzheimer disease is characterized histologically by {{c1::neuritic (senile)}} plaques in gray matter
Published	09/09/2023	The neuritic plaques seen in Alzheimer's are composed of {{c1::Aβ amyloid}} with entangled neuritic processes
Published	09/09/2023	The APP receptor on neurons is normally broken down by {{c1::alpha::alpha or beta}} cleavage
Published	09/09/2023	The APP receptor on neurons is occasionally broken down by {{c1::beta}} cleavage, resulting in formation of Aβ amyloid
Published	09/09/2023	What feature of Alzheimer disease is represented in the image below?{{c1::Senile (neuritic) plaque}}
Published	09/09/2023	Alzheimer disease is characterized by {{c1::neurofibrillary tangles}}, which are intracellular aggregates of {{c2::hyperphosphorylated}} {{c3::tau}} p…
Published	09/09/2023	The tau protein that aggregates in Alzheimer's normally functions as a {{c1::microtubule}}-associated protein
Published	09/09/2023	The number of {{c2::neurofibrillary tangles}} in Alzheimer's correlates with degree of {{c1::dementia}}
Published	09/09/2023	What feature of Alzheimer disease is represented in the image below?{{c1::Neurofibrillary tangles}}
Published	09/09/2023	Alzheimer's disease is characterized by {{c1::decreased}} acetylcholine
Published	09/09/2023	The diagnosis of Alzheimer's disease is confirmed by histology at {{c1::autopsy}} (when possible)
Published	09/09/2023	{{c2::Pick}} disease is a degenerative disease of the {{c1::frontal}} and {{c1::temporal}} cortex
Published	09/09/2023	{{c2::Frontotemporal dementia (Pick disease)}} is characterized by {{c1::round::shape}} aggregates of hyperphosphorylated tau protein known as {{…
Published	09/09/2023	Frontotemporal dementia (Pick disease) may have ubiquitinated {{c1::TDP-43 cytoplasmic}}  inclusions seen on histology
Published	09/09/2023	{{c3::Frontotemporal}} dementia is characterized by {{c1::behavioral}} and/or {{c2::language}} symptoms early in the disease course
Published	09/09/2023	Frontotemporal dementia may have associated {{c1::movement}} disorders
Published	09/09/2023	{{c3::Parkinson}} disease is characterized by degeneration of {{c1::dopaminergic}} neurons in the {{c2::substantia nigra}} of the basal ganglia
Published	09/09/2023	Parkinson disease is characterized grossly by {{c1::depigmentation}} of the substantia nigra pars compacta
Published	09/09/2023	Parkinson disease is a common disorder related to {{c1::aging}} (risk factor)
Published	09/09/2023	What contaminant in illicit drugs historically is associated with Parkinson disease? {{c1::MPTP}}
Published	09/09/2023	The clinical features of {{c6::Parkinson}} disease may be remembered with the mnemonic "TRAPS": T: {{c1::Tremor (pill-rolling tremor at rest; disappea…
Published	09/09/2023	Which movement disorder presents as an uncontrolled movement of distal appendages at rest that is alleviated by intentional movement? {{c1::Resting tr…
Published	09/09/2023	{{c3::Parkinson}} disease is characterized histologically by {{c1::Lewy bodies}}, which are round, eosinophilic inclusions composed of {{c2::α-synucle…
Published	09/09/2023	What feature of Parkinson disease is represented in the image below?{{c1::Lewy body}}
Published	09/09/2023	Parkinson disease is characterized by {{c1::increased}} acetylcholine
Published	09/09/2023	Parkinson disease is characterized by {{c1::decreased}} dopamine
Published	09/09/2023	Parkinson disease is characterized by {{c1::decreased}} serotonin
Published	09/09/2023	Dementia is a common feature of {{c1::late::early or late}} Parkinson disease
Published	09/09/2023	Early-onset dementia with Parkinsonian features is suggestive of {{c1::Lewy body}} dementia
Published	09/09/2023	Lewy body dementia is characterized initially by dementia with {{c1::fluctuating}} cognition / alertness, a {{c2::REM::which stage?}} sleep behavior d…
Published	09/09/2023	Lewy body dementia is characterized by intracellular lewy bodies primarily in the {{c1::cortex::location in the brain}}
Published	09/09/2023	{{c3::Huntington}} disease is characterized by degeneration of {{c1::GABAergic}} neurons in the {{c2::caudate nucleus}} and putamen of the basal gangl…
Published	09/09/2023	What is the mode of inheritance of Huntington disease? {{c1::Autosomal dominant}}
Published	09/09/2023	Huntington disease is caused by expanded trinucleotide repeats of CAG in the {{c1::huntingtin}} gene on chromosome {{c2::4}}
Published	09/09/2023	Further expansion of CAG repeats (Huntington disease) occurs during {{c1::spermatogenesis}} in males, which leads to anticipation in future generation…
Published	09/09/2023	What movement disorders are associated with Huntington disease? {{c1::Chorea}} and {{c2::Athetosis}}
Published	09/09/2023	Which movement disorder presents as a sudden, jerky, purposeless movements? {{c1::Chorea}}
Published	09/09/2023	Which movement disorder presents as a slow, writhing movements, especially in the fingers? {{c1::Athetosis}}
Published	09/09/2023	Symptoms of Huntington disease typically manifest between ages {{c1::20}} and {{c1::50}}
Published	09/09/2023	Huntington disease presents with chorea that can progress to {{c1::depression}} and {{c2::dementia}}; also may have {{c3::aggression}}
Published	09/09/2023	Huntington disease is sometimes initially mistaken for {{c1::substance abuse}}
Published	09/09/2023	Huntington disease is characterized by {{c1::decreased}} GABA
Published	09/09/2023	Huntington disease is characterized by {{c1::decreased}} acetylcholine
Published	09/09/2023	Huntington disease is characterized by {{c1::increased}} dopamine
Published	09/09/2023	Neuronal death in Huntington disease occurs via NMDA-R binding and {{c1::glutamate}} excitotoxicity
Published	09/09/2023	What is a common unnatural cause of death in patients with Huntington disease? {{c1::Suicide}}
Published	09/09/2023	{{c2::Normal pressure}} hydrocephalus often presents with a characteristic {{c1::magnetic}} gait
Published	09/09/2023	What is the treatment for normal pressure hydrocephalus? {{c1::Ventriculoperitoneal (VP) shunting}}
Published	09/09/2023	Prion protein is normally expressed in CNS neurons in an {{c2::α-helical}} conformation, known as {{c1::PrPc}}
Published	09/09/2023	Prion disease arises with conversion to a {{c2::β-pleated}} conformation, known as {{c1::PrPsc}}
Published	09/09/2023	Creutzfeldt-Jakob disease (CJD) is usually {{c1::sporadic}}, but can rarely can arise due to exposure to prion-infected human tissue
Published	09/09/2023	Which movement disorder presents as a sudden, brief, uncontrolled muscle contraction? {{c1::Myoclonus}}
Published	09/09/2023	{{c3::Myoclonus}} is a movement disorder common in metabolic abnormalities, such as {{c1::renal}} and {{c2::liver}} failure
Published	09/09/2023	What pathology is characterized by degeneration of the basal nucleus of Meynert? {{c1::Alzheimer disease}}
Published	09/09/2023	Which area of the brain is particularly sensitive to damage in Alzheimer disease? {{c1::Hippocampus}}
Published	09/09/2023	What neurotransmitter is decreased in Alzheimer disease? {{c1::Acetylcholine}}
Published	09/09/2023	What neurotransmitter is increased in Huntington disease? {{c1::Dopamine}}
Published	09/09/2023	What neurotransmitters are decreased in Huntington disease? {{c1::Acetylcholine}}, {{c2::GABA}}, and substance P
Published	09/09/2023	What neurotransmitters are decreased in Parkinson disease? {{c1::Dopamine}} and {{c2::serotonin}}
Published	09/09/2023	Alzheimer disease is characterized by deposition of {{c1::Aβ-amyloid}} in the brain, which forms amyloid plaques
Published	09/09/2023	In older patients, REM sleep behavior disorder may be a sign of {{c1::neurodegeneration (e.g. Parkinson’s)}}
Published	09/09/2023	Patients with medically intractable symptoms (ex. bradykinesia, rigidity) of Parkinsons disease may benefit from deep brain stimulation (DBS) of the {…
Published	09/09/2023	{{c2::Pick}} disease is a degenerative disease of the {{c1::frontal}} and {{c1::temporal}} cortex.
Published	09/09/2023	What associated symptoms are sometimes present in REM sleep behavior disorder?{{c1::Parkinsonism (eg. changes in gait, anosmia, constipation)REM sleep…
Published	09/09/2023	Marked atrophy of the caudate nucleus is seen in {{c1::Huntington disease::condition}}
Published	09/09/2023	{{c1::Huntington Disease}} is a neurological disorder characterized by a classic triad of choreiform movement, dementia/depression, and behavioural ch…
Published	09/09/2023	Alzheimer disease = ↓ {{c1::ACh::neurotransmitter}} in basal nucleus of meynert
Published	09/09/2023	Huntington disease = ↑ {{c1::dopamine::neurotransmitter}}
Published	09/09/2023	Huntington disease = ↓ {{c1::ACh::neurotransmitter}} and ↓ {{c1::GABA::neurotransmitter}}
Published	09/09/2023	Protein aggregates in neurons from hyperphosphorylation of tau protein:{{c1::Pick bodies (Pick's Disease) and Neurofibrillary tangles (Alzheimer disea…
Published	09/09/2023	What CT finding may be found in patient's with late-stage Alzheimer's disease? {{c1::Diffuse cortical and subcortical atrophy}}
Published	09/09/2023	Dementia and visual hallucinations followed by early Parkinsonism is suggestive of {{c1::Lewy body dementia::condition}}
Published	09/09/2023	Patient presents with bradykinesia, resting tremor, cogwheel rigidity, postural instability, and shuffling gait is suggestive of {{c1::Parkinson's dis…
Published	09/09/2023	{{c3::Parkinson}} disease is characterized by degeneration of {{c1::dopaminergic}} neurons in the {{c2::substantia nigra}} of the basal ganglia.
Published	09/09/2023	Patient presents with a pill-rolling resting tremor, cogwheel rigidity, bradykinesia, postural instability, and shuffling gait is suggestive of {{c1:…
Published	09/09/2023	Patient presents with chorea, dementia, and caudate/putamen degeneration is suggestive of {{c1:: Huntington disease::diagnosis}}
Published	09/09/2023	REM sleep behavior disorder is associated with {{c1::alpha-synuclein}} neurodegenerative disorders
Published	09/09/2023	{{c3::Huntington}} disease is characterized by degeneration of {{c1::GABAergic}} neurons in the {{c2::caudate nucleus}} and putamen of the basal gangl…
Published	09/09/2023	What symptom is characteristic of early Alzheimer's disease?{{c1::Short-term memory loss}}
Published	09/09/2023	{{c1::Riluzole}} is a {{c2::glutamate}} inhibitor that is approved for use in patients with {{c3::amyotrophic lateral sclerosis (ALS)}}
Published	09/09/2023	Alzheimer disease = generalized cortical atrophy but also {{c1::temporal}} lobe atrophy
Published	09/09/2023	Parkinson = {{c1::hypo}}kinetic gait
Published	09/09/2023	Presenilin-1 / presenilin-2 mutations lead to {{c1::Alzheimer disease (early onset)::condition}}Apo-E4 mutations lead to {{c1::Alzheimer disease (late…
Published	09/09/2023	What is required for diagnosis of Lewy body dementia in contrast to dementia secondary to Parkinson disease?{{c1::Cognitive and motor symptom onset &l…
Published	09/09/2023	{{c2::Cerebral amyloid angiopathy}} is a localized amyloidosis associated with {{c1::Alzheimer's}} disease
Published	09/09/2023	Long term (e.g. over 5 years), patients with Alzheimer's disease may present with {{c1::personality}} changes, {{c3::urinary incontinence::autonomic f…
Published	09/09/2023	Amyotrophic lateral sclerosis will show {{c1::fibrillation potentials}} and {{c2::positive sharp}} waves on EMG and nerve conducti…
Published	09/09/2023	Alzheimer's Disease presents with low acetylcholine levels due to decreased activity of {{c1::choline acetyltransferase::enzyme}}
Published	09/09/2023	Alzheimer disease may present with {{c1::executive cognitive dysfunction}}, characterized by difficulty organizing or planning
Published	09/09/2023	One feature of Alzheimer disease is {{c1::visuospatial}} impairment, which is a loss of sense of "self" in relation to surroundings
Published	09/09/2023	Onset of frontotemporal dementia typically occurs in the fifth or sixth decades of life with 25% inherited in a(n) …
Published	09/09/2023	Is ALS associated with cognitive impairment?{{c1::Yes (frontotemporal dementia)}}
Published	09/09/2023	One clinical manifestation of Parkinson's disease is a {{c1::hypo}}kinetic gait
Published	09/09/2023	{{c3::Amyotrophic lateral sclerosis}} is a neurological disorder that on EMG and nerve conduction studies will show {{c1::fibrillation potentials}} an…
Published	09/09/2023	If parkinsonism predates cognitive impairment by >1 year, this is suggestive of {{c1::Parkinson disease dementia::Lewy body dementia or Parkinson d…
Published	09/09/2023	If cognitive impairment predates parkinsonism, this is suggestive of {{c1::Lewy body dementia::Lewy body dementia or Parkinson disease dementia}}
Published	09/09/2023	If cognitive deficits precede depressive symptoms, {{c1::Alzheimer disease::Alzheimer disease or major depressive disorder}} is more likely
Published	09/09/2023	Lewy body dementia is characterized by dementia and {{c2::visual hallucinations::early finding}} followed by {{c1::parkinsoni…
Published	09/09/2023	{{c1::Amytriohic Lateral Sclerosis}} is death of the {{c2::LMN}} in the {{c3::spinal cord & brain stem}} + {{c4::UML}} in the {{c5::motor cortex}}
Published	09/09/2023	{{c1::Amytriohic Lateral Sclerosis}} is due to {{c2::abnormal  RNA or protein processing }}. Mutations occur on {{c3::chromosome 9 hexanucle…
Published	09/09/2023	Histological  & Gross Pathology of ALS: Found in {{c1::anterior roots}} of the spinal cord → thin & gray {{c2::↓ # of anterior horn …
Published	09/09/2023	Histological  & Gross Pathology of ALS: Found in {{c1::anterior roots}} of the spinal cord → thin & gray {{c2::↓ # of anterior horn …
Published	09/09/2023	{{c1::Intelect, sensation, sphincter control, eye movements}} are {{c2::SPARED}} in ALS
Published	09/09/2023	ALS causes degenration what what spinal tract = {{c1::lateral corticospinal tract (lateral sclerosis) }}
Published	09/09/2023	Upper motor neuron symptoms of ALS  → {{c3::paresis}}, spasticity, {{c2::hyperreflexia}}, {{c1::Babinski}} sign, degeneration of {{c3::later…
Published	09/09/2023	Lower motor neuron symptoms of ALS → muscle denervation → {{c1::muscle atrophy, weakness, fasciculations }}
Published	09/09/2023	Huntington Chorea pathological features:{{c1::Intranuclear inclusions}} contian {{c2::ubiqyutnated huntungtin }}Atrophy of  {{c3::caudate &a…
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