Major Neurocognitive Disorder and the role of Frontal Subcortical Circuits in Neuropsyhciatry and Neuropsychology

The progressive loss of dopamine neurons between the substantia nigra and striatum is associated with which type of dementia?

The loss of dopamine neurons in the Basal Ganglia disease affects which subcortical circuit?

Which of the following are symptoms of Parkinson's Disease?

Depression occurs in approximately [blank_start]50%[blank_end] of Parkinson's patients.

Which statement best describes the genetic component of Parkinson's

Definite diagnosis of Parkinson's is possible post mortem, if depigmentation is observed in the substantia nigra.

Autonomic dysfunction, depression, memory complaints, and Bradykinesia that develops gradually over time, rather than within a year is suggestive of:

The one year rule regarding Lewy Bodies Dementia and Parkinson's:

Frontal subcortical circuits are effector mechanisms that allow the organism to act on the environment. Impaired executive functions, apathy, and impulsivity are hallmarks of frontal subcortical circuit dysfunction. The [blank_start]dorsolateral prefrontal[blank_end] circuit allows the organization of information to facilitate a response; the [blank_start]anterior cingulate[blank_end] circuit is required for motivated behavior; and the [blank_start]orbitofrontal[blank_end] circuit allows the integration of limbic and emotional information into behavioral responses.

[blank_start]Parkinson's[blank_end] is the world's 2nd most common neurodegenerative disease

Bradykinesia refers to:

The gait associated with [blank_start]Parkinson's[blank_end] is characterized by a forward stooped posture, diminished or absent arm swing and slow shuffling steps, with difficulty starting to walk and once walking, difficulty stopping.

Which of the following describes the speech and writing characteristic of Parkinson's disease

Parkinsonism refers to a

Which of the following best describes the Risk factors associated with Parkinson's disease?

Parkinson's involves which of the following?

Parkinson's Disease may begin two or more decades before the first symptoms develop.

Progression of [blank_start]Parkinson's disease[blank_end] tends to be slow with most patients surviving ten to 15 years after first symptoms are noticed.

The majority of Parkinson's patients survive beyond 75 years

Earlier disease onset, tremor dominant, non-tremor dominant and rapid disease progression with dementia ar sub groups of

Select the appropriate incidence of each of the following Parkinson's subtypes: Earlier disease onset [blank_start]25%[blank_end] Tremor dominant [blank_start]31%[blank_end] Non-tremor dominant [blank_start]35%[blank_end] Rapid disease progression with dementia [blank_start]8%[blank_end]

Which subtype of Parkinson's disease has the slowest progression in terms of cognitive decline?

When using the Unified Parkinson's Disease Rating Scale (UPDRS) a higher scores represents

Diagnosis of Parkinson's disease is confirmed as INCORRECT in one fourth of autopsied cases.

Which of the following disorders closely resemble Parkinson's Disease?

Which of the following represent the early signs of Parkinson's Disease

The cognitive deficits typical of the early stages of Parkinson's are most similar to

Vocabulary, grammar, and syntax tend to remain intact in PD

Diagnosis of depression in Parkinson's patients can be complicated by the presence of bradykinesia. Bradykinetic patients can lack facial expressiveness giving off the impression of depression, in addition to reduced motor activity and slowed responding. This is further compounded by the unreliability of self-reports in patients who are cognitively impaired.

Depression in Parkinson's Disease tends to respond well to conventional medication used to treat idiopathic depression.

Diagnosis of [blank_start]dementia with Lewy bodies[blank_end] is typically made when the diagnosis of dementia precedes or coincides within one year of the onset of motor symptoms. Whereas [blank_start]Parkinson's disease with dementia[blank_end] is usually made when dementia develops within the context of established [blank_start]PD[blank_end].

Which of the following deficits tend to distinguish between Parkinson's with cognitive impairment and Parkinson's with dementia?

The positive effects of L - Dopa on movement

Parkinson's - Tango dance classes twice a week has been shown to improve balance, functional mobility and walking compared to a no dance group.

What am I? Neurodegenerative disorder Hereditary, Autosomal dominant Typical Age at onset: 35-45 years Duration: 15-20 years Symptoms include: Bradykinesia and/or Chorea, Apathy, Executive Dysfunction, Working Memory Deficit, Psychiatric disorder, and elevated suicide rate

All Huntington's patients develop dementia, unless they die before the disease has run it's course.

What number of trinucleotide CAG repeats (Cytosine, Adenine, Guanine) is considered pathological and linked to the development of Huntington's disease?

[blank_start]Huntington's disease[blank_end] results from an excessive number of trinucleotide [blank_start]CAG[blank_end] repeats ([blank_start]cytosine, adenine, guanine[blank_end]) in the [blank_start]HD gene[blank_end] that encodes a protein known as [blank_start]huntingtin[blank_end] located on chromosome [blank_start]4[blank_end]. People without the disease will have [blank_start]fewer than 35[blank_end] repeats. [blank_start]Huntington's Disease[blank_end] is considered autosomal [blank_start]dominant[blank_end], so that [blank_start]half[blank_end] of all offspring of a single carrier parent will develop the disease.

Huntington's disease is more prominent in [blank_start]Caucasian[blank_end] populations than either [blank_start]Asian or African[blank_end] populations.

Huntington's disease - The most consistent change in neurotransmitters occurs as reduced levels of [blank_start]GABA[blank_end], with a consistent concomitant increase in [blank_start]excitatory[blank_end] neurotransmitters.

There is a [blank_start]negative[blank_end] correlation between the number of CAG repeat length and age at onset for huntington's disease. Additionally, disease severity is [blank_start]positively[blank_end] correlated with CAG repeat lengths.

In [blank_start]huntington's[blank_end] disease early onset is associated with more severe symptoms and faster degeneration. Conversely, early onset [blank_start]Parkinson's[blank_end] disease is associated with a slower progression.

Cognitive Impairment is often the first expression of the disease and may predate motor symptoms by as much as two year. This statement is consistent with

Huntington's Disease, Neuropsychological Profile: Attention Span: [blank_start]Drops as disease progresses[blank_end]Set Shifting: [blank_start]Impaired[blank_end] Working Memory: [blank_start]Impaired[blank_end] Memory Deficit: [blank_start]Mild initially, worsen with time[blank_end] Memory deficits tend to be due to [blank_start]retention strategy[blank_end] Vocabulary and Grammar - [blank_start]Preserved early, develops last[blank_end] Executive Function: [blank_start]Impaired[blank_end]

Emotional disturbances of personality change develop in [blank_start]almost all[blank_end] patients with Huntington's Disease.

Select ALL correct responses regarding depression in Huntington's Disease:

Deep Brain Stimulation is more effective as a treatment of Huntington's Disease than Parkinson's

Which of the following is NOT classed as a subcortical dementia?

Multi Infarct Dementia (DIM), CADASIL (Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoaraiosis) and HCHWA-D (Hereditary cerebral hemorrhage with amyloidosis-Dutch type) are subtypes of which of the following?

The umbrella term used in the DSM 5 to refer to what is commonly called dementia

A lacunes or microinfarct is a damaged region of the brain < [blank_start]2mm[blank_end] in area, and typically involve central white matter tracts and [blank_start]subcortical[blank_end] regions.

Strategic infarct dementia is

A slowly progressive form of Vascular dementia that involves subcortical white matter lesions produced by chronic ischemia.

Huntington's Disease, Parkinson's Disease and Vascular Dementia all involve which of the following symptoms? Choose characteristic symptoms!

Intact disease insight tends to be present in Vascular Dementia

Name this disease. Non-familial (i.e non-genetic) Prevalence of 1.4 per 100,000 No known disease specific biomarker Patients often display a inability to look downwards Primary lesion sites begin in the upper brainstem and extend to the basal ganglia. In particular, this process appears to disconnect ascending pathways from these subcortical structures to the prefrontal cortex. Dopamine levels drop drastically in the striatum Alzheimer's often co-occurs (69% of cases)

Which disease displays the following symptoms: Onset after the age of 40 Early Symptoms: Postural Instability Dysarthria Bradykinesia Within the first year: Cognitive or behavioural changes begin to develop. Rarely develop first Can include: deficits typical of frontal lesions: apathy, initiation difficulties, flexibility. Memory ability may be normal but significantly slowed. Memory impairment can occur, though not to the same extent as AD Forward digit span may be within normal limits Language will often remain intact, but primary progressive aphasia can occur. Also note Dysarthria. Later: Vertical gaze palsy, when patients try to bend their head to compensate their eyes roll up reflexively. Others: Oculomotor defects Death often results from respiratory arrest, either secondary to pneumonia or due to degenerative processes involving brainstem respiratory centres.

Name this disease: Primary symptomatic features include: Progressive gait disturbance Urinary Incontinence Confusion, disorientation and memory problems. Memory problems usually develop after Urinary incontinence and gait disturbance. Though Executive dysfunction can present relatively early in the disease. Perseveration, Typical imaging shows enlargement of the ventricle due to excess CSF and white matter damage.

Which of the following diseases/disorders can often be successfully treated with a "shunt"

Normal Pressure Hydrocephalus has an incidence of approximately [blank_start]5.5[blank_end] in 100,000. Progressive supranuclear palsy has an incidence of approximately [blank_start]1.4[blank_end] in 100,000.

Name this disease: Progressive dementia with rapid onset Death usually occurs after 4-5 months Atypical EEG Myoclonus, gait disturbance, possible seizures Psychiatric disturbance Hallucinations

To receive a diagnosis of MCI the memory problems have to interfere with daily functioning

Histopathological hallmark of Alzheimer’s disease

Sudden onset, fluctuating cognition and attention

Visual hallucinations, most often

When does normal biological decay begin?

What proportion of the following populations have dementia? Of 80+ population: [blank_start]20%[blank_end] dementia Of 85+ population: [blank_start]40%[blank_end] dementia

A patient who presents with dysfunction on memory tasks (or any other domain), despite some difficulties day to day they are still able to function independently at home. What would be the most appropriate diagnosis?

Deficits in Mild Cognitive Impairment can only occurs within the cognitive domain of memory

All patients who develop true Mild Cognitive Impairment will progress to dementia

Non-amnestic MCI though to be predictive of:

Approximately [blank_start]5.6 - 15%[blank_end] of MCI patients convert to Dementia each year. However in many cases MCI will not develop any further, and in some cases it is even alleviated after time.

Walking 72 blocks a week was associated with greater grey matter volume and a two fold reduced risk developing a cognitive impairment more than a decade later in a longitudinal study.

[blank_start]Amnestic MCI[blank_end] - Poor memory performance, below expected for the patient's age with no other cognitive deficits and not serious enough to warrant a diagnosis of dementia. [blank_start]Nonamnestic MCI[blank_end] - Poor performance in any domain other than memory, below expected for the patient's age with no other cognitive deficits and not serious enough to warrant a diagnosis of dementia. [blank_start]Multi domain MCI[blank_end] - Poor performance in at least two domains, below expected for the patient's age with no other cognitive deficits and not serious enough to warrant a diagnosis of dementia.

Dementia applies to a condition in which both cognitive decline AND [blank_start]functional impairment[blank_end] are observed.

Alzheimer's Disease accounts for [blank_start]60 - 80%[blank_end] of dementia, with an estimated incidence of [blank_start]8.45 million[blank_end] affected in europe alone with an annual cost of [blank_start]141 billion[blank_end] Euro per year

A definite diagnosis of Alzheimer's is only possible after post-mortem exam in which amyloid plaques and neurofibrillary tangles are confirmed present.

Whether [blank_start]Alzheimer's[blank_end] disease evolves from neurofibrillary tangles and amyloid plaques or whether they are a by product of the disease [blank_start]in unknown.[blank_end]

[blank_start]Probable Alzheimer's disease:[blank_end] Dementia has been established by clinical and neuropsychological examination. Cognitive impairments also have to be progressive (ie decline from previous functioning) and be present in two or more areas of cognition. Deficit interferes with ability to work/daily functioning. Onset between the ages of 40 and 90 years and finally there must be an absence of other diseases capable of producing a dementia syndrome. [blank_start]Definite Alzheimer's disease:[blank_end] The patient meets the criteria for probable Alzheimer's disease and has histopathologic evidence of AD via autopsy or biopsy. [blank_start]Unlikely Alzheimer's disease:[blank_end] The patient presents a dementia syndrome with a sudden onset, focal neurologic signs, or seizures or gait disturbance early in the course of the illness [blank_start]Possible Alzheimer's disease[blank_end]: There is a dementia syndrome with an atypical onset, presentation or progression; and without a known etiology; but no co-morbid diseases capable of producing dementia are believed to be in the origin of it. Or a lack of sufficient patient history or objective cognitive documentation.

According to the DSM-IV a diagnosis of probable Alzheimer's must include a decline in at least two cognitive domains. One of these deficits MUST involve an inability to learn new information or recall previously learned information. The decline must also be sufficient enough to impair social or occupational functioning.

[blank_start]30%[blank_end] of all dementia cases are due to Alzheimer's ALONE, [blank_start]38%[blank_end] are AD with infarcts

A common explanation to account for the fact that people with higher levels of education have a lower risk of developing AD is that these people have a higher [blank_start]cognitive reserve[blank_end] to compensate for the negative effects of the disease.

Higher rates of educational attainment is associated with [blank_start]faster[blank_end] rates of cognitive decline after a diagnosis of dementia but [blank_start]a delayed[blank_end] onset.

Alzheimer's likely does not have a genetic component.

Roughly [blank_start]25%[blank_end] of Alzheimer's disease is familial, this is especially the case for [blank_start]early[blank_end] onset AD in which [blank_start]60%[blank_end] of cases are familial.

What percentage of Alzheimer's Begins before the age of 60 - 65? I.e What proportion of AD is "early onset"

Mutations of the presenilin-1 gene on chromosome 14, presenilin-2 gene on chromosome 1 and Amyloid precursor protein gene on chromosome 21 are associated with which form of dementia?

Vascular risk factors such as high fat consumption, arteriosclerosis, hypertension and diabetes are risk factors for: A. Alzheimer's Disease B. Vascular Dementia

TBI increases the risk factor of which of the following diseases? A. Alzheimer's B. Parkinson's

Hormone Replacement Therapy (combined estrogen and progesterone) may as much as double the risk of developing Alzheimer's Dementia.

Daily light to moderate alcohol consumption (1-2 drinks per day) is associated with a lower risk of dementia.

[blank_start]Neurofibrillary tangles[blank_end] develop when the microtubules that transport substances from the nerve cell body to the end of the axon become twisted. The protein that helps to maintain these structures is known as [blank_start]tau[blank_end]. In [blank_start]Alzheimer's disease[blank_end], this protein is altered allowing the twisted tubules to aggregate into [blank_start]tangles[blank_end].

In AD which of the following cortical regions are comparatively spared, even in later stages?

Second to memory decline, which of the following presents the most sensitive measure when diagnosing AD

Label the symptom sets

In alzheimer's, [blank_start]older[blank_end] memories tend to be better preserved than [blank_start]new[blank_end] memories.

Central to language deterioration in Alzheimer's patients is a disintegration of [blank_start]semantic relationships and understanding[blank_end].

Although popular as a screening measure for AD clock drawing deficits are not specific to AD, in fact they tend to occur more frequently in Parkinson's Disease and Lewy Body Dementia.

The closing in phenomenon demonstrated by some dementia patients in visuoconstruction tasks is more closely associated with: A: Alzheimer's Disease B: Vascular Dementia

[blank_start]Perseveration[blank_end] is the uncontrolled repetition or continuation of a response (e.g., behavior, word, thought, activity, strategy, or emotion) in the absence of an ongoing occasion or rationale for that behavior or emotion (e.g., the topic or task requirements have changed). Whereas, an [blank_start]intrusion[blank_end] is defined as the inappropriate repetition of a prior response to a task after intervening stimuli, that is on a later task.

Alzheimer's and EEG: slowing [blank_start]Increased[blank_end] theta activity, combined with [blank_start]Decreased[blank_end] alpha activity, and [blank_start]Decreased[blank_end] beta activity Late stages: [blank_start]Increased[blank_end] delta activity

[blank_start]Frontotemporal lobar[blank_end] dementia typically involved pathology of the frontal and temporal lobes with relative sparing of the posterior regions. Age of onset is relatively young (40 - 65) and there are three main subtypes a [blank_start]behavioural[blank_end] variant, a [blank_start]semantic[blank_end] type and [blank_start]primary progressive aphasia[blank_end].

Pick's Disease is the former name of which type of dementia/major neurocognitive disorder?

Which of the following are considered risk factors for FTLD

Pick's Bodies are found in about [blank_start]20%[blank_end] of patients with FTLD.

In pure/classic FTLD the parietal and occipital lobes remain intact.

Hippocampal atrophy in FTLD (Choose the BEST)

Which of the following types of dementia best fits the following symptom set in its classic form: extrapyramidal signs (shakiness, rigidity etc), apathy, relatively young onset (40 - 65), executive dysfunction, incontinence, and in particular profound social and behavioural change as an early stage symptom.

In a classic distinction Alzheimer's patients will present with [blank_start]memory[blank_end] problems as the primary symptom, whereas patients with FTLD will more often present with [blank_start]social and executive[blank_end] primary symptoms. However these distinctions should be taken lightly as co-occurrence and symptomatic overlap are common.

Dementia patients who have more temporal than frontal involvement, little to no posterior involvement, intact autobiographical memory but impaired knowledge of word meaning. This constellation of symptoms best matches

Semantic dementia (FTLD) - usually grammar and syntax remains intact. Prominent anterior temporal lobes atrophy is associated.

[blank_start]Primary progressive aphasia[blank_end] occurs without memory impairment or dementia in the early course. Many patients remain dementia free for at least 2 years and as many as 10. The disorder starts with anomia and proceeds to grammatical structure and language comprehension

[blank_start]Primary progressive aphasia[blank_end] starts with anomia and proceeds to grammatical structure and language comprehension. [blank_start]FTLD - Semantic Dementia[blank_end] word meanings are usually lost with an intact sense of grammar.

Primary progressive aphasia - Usually associated with [blank_start]FTLD[blank_end] although [blank_start]AD[blank_end] accounts for 30% of all cases. [blank_start]Unlike[blank_end] other FTLD women are more often affected than men.

1st Step: Neurodegenerative disease: Shows [blank_start]progressive deterioration[blank_end] of behaviour and/or cognition by observation or history (as provided by a knowledgeable informant). II. [blank_start]Possible[blank_end] bvFTLD Three of the following behavioural/cognitive symptoms (A–F) must be present to meet criteria. Ascertainment requires that symptoms be persistent or recurrent, rather than single or rare events. A. Early* behavioural disinhibition [one of the following symptoms (A.1–A.3) must be present]: A.1. [blank_start]Socially inappropriate behaviour[blank_end] A.2. [blank_start]Loss of manners or decorum[blank_end] A.3. [blank_start]Impulsive, rash or careless actions[blank_end] B. Early apathy or inertia [one of the following symptoms (B.1–B.2) must be present]: B.1. Apathy B.2. Inertia C. Early loss of [blank_start]sympathy or empathy[blank_end] [one of the following symptoms (C.1–C.2) must be present]: C.1. Diminished response to other people’s needs and feelings C.2. Diminished social interest, interrelatedness or personal warmth D. Early [blank_start]perseverative[blank_end], stereotyped or compulsive/ritualistic behaviour [one of the following symptoms (D.1–D.3) must be present]: D.1. Simple repetitive movements D.2. Complex, compulsive or ritualistic behaviours D.3. Stereotypy of speech E. [blank_start]Hyperorality[blank_end] and dietary changes [one of the following symptoms (E.1–E.3) must be present]: E.1. Altered food preferences E.2. Binge eating, increased consumption of alcohol or cigarettes E.3. Oral exploration or consumption of inedible objects F. Neuropsychological profile: executive/generation deficits with relative sparing of [blank_start]memory and visuospatial functions[blank_end] [all of the following symptoms (F.1–F.3) must be present]: F.1. Deficits in executive tasks F.2. [blank_start]Relative sparing of episodic memory[blank_end] F.3. Relative sparing of visuospatial skills III. Probable bvFTLD All of the following symptoms (A–C) must be present to meet criteria. A. Meets criteria for possible bvFTD B. Exhibits significant functional decline (by caregiver report or as evidenced by Clinical Dementia Rating Scale or Functional Activities Questionnaire scores) C. Imaging results consistent with bvFTD [one of the following (C.1–C.2) must be present]: C.1. [blank_start]Frontal and/or anterior temporal atrophy[blank_end] on MRI or CT C.2. [blank_start]Frontal and/or anterior temporal[blank_end] hypoperfusion or hypometabolism on PET or SPECT IV. Behavioural variant FTD with definite FTLD Pathology Criterion A and either criterion B or C must be present to meet criteria. A. Meets criteria for possible or probable bvFTD B. Histopathological [blank_start]evidence of on biopsy or at post-mortem[blank_end] C. Presence of a known pathogenic mutation V. [blank_start]Exclusionary criteria[blank_end] for bvFTLD Criteria A and B must be answered negatively for any bvFTLD diagnosis. Criterion C can be positive for possible bvFTLD but must be negative for probable bvFTLD A. Pattern of deficits is better accounted for by other non-degenerative nervous system or medical disorders B. Behavioural disturbance is better accounted for by a psychiatric diagnosis C. Biomarkers strongly indicative of Alzheimer’s disease or other neurodegenerative process

Difficulty in language as most prominent clinical feature Principal cause of impaired ADL Aphasia most prominent deficit at onset and initial phase Sounds like?

Dementia, Delirium or Depression?

Delirium, Depression or Dementia?

What is the relationship between the following list and dementia? Hypothyroidism, Neurology (tumor, hematoma's), Intoxications (medication, alcohol), Infections (Lues, HIV, Lyme), Deficiencies (vit. B12, B1), Psychiatric Health.

Name the dementia: Unrecognized until the 1970s Major features include extrapyramidal signs (parkinsonisms), fluctuating cognition, visual hallucinations Often deficits in attention, visuoperceptual and executive function. Not neatly classified as primarily sub-cortical nor cortical

Lewy bodies are found in:

Amyloid Plaques are uncommon in Dementia with Lewy bodies, though neurofibrillary tangles are common.

Lewy bodies in largely concentrated in the substantia nigra alone are characteristic of:

Lewy bodies in the substantia nigra, limbic and cortical areas, without neruofibrillary tangles (tau) is suggestive of

Decline in [blank_start]DLB[blank_end] is usually more rapid than decline in [blank_start]AD[blank_end]

It is well known that some normally functioning elderly people at the time of death have abundant neuropathological signs of AD (etc) in post mortem exam

Label these symptoms

Label the functions associated with these frontal-subcortical circuits: DLPFC - [blank_start]Executive Functioning[blank_end] Anterior Cingulate - [blank_start]Motivation and Drive[blank_end] Orbito Frontal/Lateral Orbital PFC - [blank_start]Inhibition, Social rules[blank_end]

Depression can result in biases in A Attention B Processing C Memory D All of the above