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

Depression occurs in approximately 50% 10% 80%( 50%, 10%, 80% ) of Parkinson's patients.

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 dorsolateral prefrontal anterior cingulate orbitofrontal( dorsolateral prefrontal, anterior cingulate, orbitofrontal ) circuit allows the organization of information to facilitate a response; the anterior cingulate dorsolateral prefrontal orbitofrontal( anterior cingulate, dorsolateral prefrontal, orbitofrontal ) circuit is required for motivated behavior; and the orbitofrontal anterior cingulate dorsolateral prefrontal( orbitofrontal, anterior cingulate, dorsolateral prefrontal ) circuit allows the integration of limbic and emotional information into behavioral responses.

Parkinson's Alzheimer's Huntington's( Parkinson's, Alzheimer's, Huntington's ) is the world's 2nd most common neurodegenerative disease

Progression of Parkinson's disease Delirium Lewy Bodies Dementia Creutzfeld Jacob’s disease( Parkinson's disease, Delirium, Lewy Bodies Dementia, Creutzfeld Jacob’s disease ) tends to be slow with most patients surviving ten to 15 years after first symptoms are noticed.

Select the appropriate incidence of each of the following Parkinson's subtypes:
Earlier disease onset 25% 31% 35% 8%( 25%, 31%, 35%, 8% )
Tremor dominant 31% 25% 8% 70%( 31%, 25%, 8%, 70% )
Non-tremor dominant 35% 25% 8% 70%( 35%, 25%, 8%, 70% )
Rapid disease progression with dementia 8% 60% 25% 31%( 8%, 60%, 25%, 31% )

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

Huntington's disease Alzheimer's disease Parkinson's disease Lewy bodies dementia Major Depressive Disorder( Huntington's disease, Alzheimer's disease, Parkinson's disease, Lewy bodies dementia, Major Depressive Disorder ) results from an excessive number of trinucleotide CAG GAG TAG( CAG, GAG, TAG ) repeats (cytosine, adenine, guanine tycoon, adenine, guanine guanine, adenine, guanine( cytosine, adenine, guanine, tycoon, adenine, guanine, guanine, adenine, guanine )) in the HD gene PD gene AD gene MD gene( HD gene, PD gene, AD gene, MD gene ) that encodes a protein known as huntingtin tryptone parkintin globin( huntingtin, tryptone, parkintin, globin ) located on chromosome 4 5 6 7( 4, 5, 6, 7 ). People without the disease will have fewer than 35 more than 35 fewer than 15 more than 15( fewer than 35, more than 35, fewer than 15, more than 15 ) repeats. Huntington's Disease Alzheimer's Disease Parkinson's Disease Major depressive disorder Lewy bodies dementia( Huntington's Disease, Alzheimer's Disease, Parkinson's Disease, Major depressive disorder, Lewy bodies dementia ) is considered autosomal dominant recessive( dominant, recessive ), so that 50% 35% 10%( 50%, 35%, 10% ) of all offspring of a single carrier parent will develop the disease.

Huntington's disease is more prominent in Caucasian Asian African( Caucasian, Asian, African ) populations than either Asian or African Caucasian or African Asian or Caucasian( Asian or African, Caucasian or African, Asian or Caucasian ) populations.

Huntington's disease - The most consistent change in neurotransmitters occurs as reduced levels of GABA GATA Glutamate Norepinephrine( GABA, GATA, Glutamate, Norepinephrine ), with a consistent concomitant increase in excitatory inhibitory( excitatory, inhibitory ) neurotransmitters.

There is a negative positive( negative, positive ) correlation between the number of CAG repeat length and age at onset for huntington's disease. Additionally, disease severity is positively negatively( positively, negatively ) correlated with CAG repeat lengths.

In Huntington's Parkinson's( Huntington's, Parkinson's ) disease early onset is associated with more severe symptoms and faster degeneration. Conversely, early onset Parkinson's Huntington's( Parkinson's, Huntington's ) disease is associated with a slower progression.

Huntington's Disease, Neuropsychological Profile:

Attention Span: Drops as disease progresses Preserved( Drops as disease progresses, Preserved )Set Shifting: Impaired Preserved( Impaired, Preserved )
Working Memory: Impaired Preserved( Impaired, Preserved )
Memory Deficit: Mild initially, worsen with time Early development( Mild initially, worsen with time, Early development )
Memory deficits tend to be due to retention strategy primary retention deficit( retention strategy, primary retention deficit )
Vocabulary and Grammar - Preserved early, develops last Early sign( Preserved early, develops last, Early sign )
Executive Function: Impaired Preserved( Impaired, Preserved )

Emotional disturbances of personality change develop in almost all 10% of 30% of 50% of( almost all, 10% of, 30% of, 50% of ) patients with Huntington's Disease.

A lacunes or microinfarct is a damaged region of the brain < 2mm 10mm 0.5mm( 2mm, 10mm, 0.5mm ) in area, and typically involve central white matter tracts and subcortical cortical( subcortical, cortical ) regions.

Normal Pressure Hydrocephalus has an incidence of approximately 5.5 10.5 20.5 205( 5.5, 10.5, 20.5, 205 ) in 100,000. Progressive supranuclear palsy has an incidence of approximately 1.4 5.5 150 500( 1.4, 5.5, 150, 500 ) in 100,000.

What proportion of the following populations have dementia?

Of 80+ population: 20% 40% 60%( 20%, 40%, 60% ) dementia
Of 85+ population: 40% 20% 60%( 40%, 20%, 60% ) dementia

Approximately 5.6 - 15% 30 - 35% 50%( 5.6 - 15%, 30 - 35%, 50% ) 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.

Dementia applies to a condition in which both cognitive decline AND functional impairment vascular impairment atrophy of the hippocampus dysexecutive syndrome( functional impairment, vascular impairment, atrophy of the hippocampus, dysexecutive syndrome ) are observed.

Alzheimer's Disease accounts for 60 - 80% 10 - 20% 45%( 60 - 80%, 10 - 20%, 45% ) of dementia, with an estimated incidence of 8.45 million 4.45 million 10.45 million 50 million( 8.45 million, 4.45 million, 10.45 million, 50 million ) affected in europe alone with an annual cost of 141 billion 20 billion 300 billion( 141 billion, 20 billion, 300 billion ) Euro per year

Whether Alzheimer's Stroke Normal Pressure Hydrocephalus( Alzheimer's, Stroke, Normal Pressure Hydrocephalus ) disease evolves from neurofibrillary tangles and amyloid plaques or whether they are a by product of the disease is unknown. is certain. depends of the type of dementia( is unknown., is certain., depends of the type of dementia )

Probable Alzheimer's disease Definite Alzheimer's disease Unlikely Alzheimer's disease Possible Alzheimer's disease( Probable Alzheimer's disease, Definite Alzheimer's disease, Unlikely Alzheimer's disease, Possible Alzheimer's disease ) 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.

Definite Alzheimer's disease: Probable Alzheimer's disease Possible Alzheimer's disease Unlikely Alzheimer's disease( Definite Alzheimer's disease:, Probable Alzheimer's disease, Possible Alzheimer's disease, Unlikely Alzheimer's disease ) The patient meets the criteria for probable Alzheimer's disease and has histopathologic evidence of AD via autopsy or biopsy.

Unlikely Alzheimer's disease: Definite Alzheimer's disease Probable Alzheimer's disease Possible Alzheimer's disease( Unlikely Alzheimer's disease:, Definite Alzheimer's disease, Probable Alzheimer's disease, Possible Alzheimer's disease ) 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

Possible Alzheimer's disease Probable Alzheimer's disease Definite Alzheimer's disease Unlikely Alzheimer's disease( Possible Alzheimer's disease, Probable Alzheimer's disease, Definite Alzheimer's disease, Unlikely Alzheimer's disease ): 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.

30% 60 - 80% 10 - 20%( 30%, 60 - 80%, 10 - 20% ) of all dementia cases are due to Alzheimer's ALONE, 38% 20% 50% 12%( 38%, 20%, 50%, 12% ) are AD with infarcts

Higher rates of educational attainment is associated with faster slower the same( faster, slower, the same ) rates of cognitive decline after a diagnosis of dementia but a delayed an early( a delayed, an early ) onset.

Roughly 25% 60% 80%( 25%, 60%, 80% ) of Alzheimer's disease is familial, this is especially the case for early late( early, late ) onset AD in which 60% 25% 80%( 60%, 25%, 80% ) of cases are familial.

Neurofibrillary tangles Amyloid Plaques Lewy Bodies( Neurofibrillary tangles, Amyloid Plaques, Lewy Bodies ) 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 tau amyloid Lewy protein( tau, amyloid, Lewy protein ). In Alzheimer's disease Lewy Bodies Dementia Huntington's Disease( Alzheimer's disease, Lewy Bodies Dementia, Huntington's Disease ), this protein is altered allowing the twisted tubules to aggregate into tangles plaques( tangles, plaques ).

In alzheimer's, older newer( older, newer ) memories tend to be better preserved than new old( new, old ) memories.

Central to language deterioration in Alzheimer's patients is a disintegration of semantic content of words and sentences syntax and linguistic organisation word pronounciation( semantic content of words and sentences, syntax and linguistic organisation, word pronounciation ).

Alzheimer's and EEG: slowing
Increased Decreased( Increased, Decreased ) theta activity, combined with
Decreased Increased( Decreased, Increased ) alpha activity, and
Decreased Increased( Decreased, Increased ) beta activity
Late stages: Increased Decrease( Increased, Decrease ) delta activity

Pick's Bodies are found in about 20% 90% 70% 0%( 20%, 90%, 70%, 0% ) of patients with FTLD.

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

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

Primary progressive aphasia - Usually associated with FTLD AD PDD HD( FTLD, AD, PDD, HD ) although AD FTLD PDD HD( AD, FTLD, PDD, HD ) accounts for 30% of all cases. Unlike Like( Unlike, Like ) other FTLD women are more often affected than men.

Decline in DLB AD( DLB, AD ) is usually more rapid than decline in AD DLB( AD, DLB )

Label the functions associated with these frontal-subcortical circuits:

DLPFC - Executive Functioning Inhibition, Social rules Motivation and Drive( Executive Functioning, Inhibition, Social rules, Motivation and Drive )
Anterior Cingulate - Motivation and Drive Executive Functioning Inhibition, Social rules( Motivation and Drive, Executive Functioning, Inhibition, Social rules )
Orbito Frontal/Lateral Orbital PFC - Inhibition, Social rules Executive Functioning Motivation and Drive( Inhibition, Social rules, Executive Functioning, Motivation and Drive )