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Zachariah Campbell

PSYC31 Lecture 08: Neuropathology for NP Continued • Performances of those with FTD are filled with cursery responses (they don’t seem to care), with minimal effort and slow response initiations (e.g., they are slow in the finger tapping test because of a lack of care). They are also very perseverative o The use little words/language (an economy of output), show echolalia, stereotypy, and concreteness ▪ They may stop speaking altogether at the end of disease progression o Their perception, however, is preserved, as the parietal lobes are unaffected ▪ This can be used to differentiate DA from FTD ▪ Spatial function is also preserved as a result o Poor memory because of frontal lobe atrophy; frontal lobes involved in encoding and retrieval ▪ HERA – hemispheric encoding/retrieval asymmetry • The right hemisphere is involved in retrieval, and the left is encoding (I’m not sure if I heard this right, don’t take my word on this) • Recognition tasks can be used to test if encoding or retrieval is damaged o Concrete executive function; they can’t shift sets in a WCST, and they are disorganized • There are three main subtypes of FTD; disinhibited, apathetic, and stereotypic o The deficits of the disinhibited subtypes can be more debilitating than their cognitive deficits ▪ They show a jocularity; they laugh a lot, often in inappropriate moments ▪ They are restless, purposeless, overactive, unconcerned, and perform poorly in social situations o The apathetic subtype show inertia/perseveration, aspontaneity (they won’t start tasks on their own), and early incontinence o Stereotypic subtype show pronounced behavioural stereotypies and compulsive, ritualistic traits • Two other subtypes of FTD are Primary Progressive Aphasia and Semantic Dementia o PPA has a late onset; typically 50-65 years, but can range from 45-70 years ▪ Like other dementias, has a mean duration of 8 years, but can last 4-12 years ▪ They have a “progressive Broca’s aphasia” which gets worse with time, although cognitive impairment remains the same • Their speech is non-fluent, agrammatic, and stuttered • They perform poorly in confrontation naming because of phonemic errors and paraphasias, and they perform poorly in word fluency tests o In Word Fluency Tests, participants must name as many words that they can think of that start with a certain letter, and then as many words that they can think of that belong in a certain category, like animals o They perform better in the animals condition than they do in the letter condition, as they are less likely to make phonemic errors here ▪ Atrophy is found most in frontal areas o Semantic dementia has a similar onset; typically 50-65 years, but can range from 48-71 ▪ Atrophy is found most in temporal areas ▪ It is more common in women than man, whereas PPA is sex equivalent ▪ They have a progressive Wernicke’s aphasia, and as a result are compromised on most tests ▪ They show semantic paraphasias as opposed to phonemic ones, and instead of naming words they describe the word (it’s called circum-something, I didn’t hear what he said) ▪ Their biographical memory is intact, but their semantic memory is impaired (hence the term semantic dementia) Subcortical Dementias • Progressive Supranuclear Palsy is rare. Supranuclear Palsy, an impairment in intentional eye movement, is the most prominent feature (they can still move their eyes in reflexes) o Nuclei involved in gaze are destroyed o They have a slow, awkward gait, often leading to misdiagnoses of Parkinson’s ▪ The thalamus, brainstem, cerebellum, and even the spinal cord are damaged ▪ Patients have neurofibrillary tangles; their symptoms can’t be ameliorated with L-DOPA like Parkinson’s symptoms o They have a slowed cognition, and emotional/personality disturbances o The basal ganglia and superior colliculi are damaged, resulting in difficulties in eye movement, and gait ▪ Also have inaccurate visuospatial skills because of lost eye movement capability o Prefrontal functioning is altered, resulting in poor executive functioning and slowed response times • Parkinson’s disease symptoms include: resting tremors, muscle rigidity, shuffling gait (small steps – helps to maintain balance), cogwheel rigidity (when their arm is pulled back, they then have difficulty extending the arm back to its original position, for example), akinesia, and bradykinesia o They have poor executive function, slowed response times, and inaccurate visual spatial skills (like in those with PSP). Also attentional deficits (both for shifting attention and sustained attention) • Huntington’s disease has a clear genetic component (CAG repeats). They show chorea (irregular jerks, almost dance-like) and cognitive and personality disturbances o Degeneration of the frontal lobes; poor executive function and psychotic symptoms (these psychotic symptoms distinguish it from Parkinson’s) o Impaired recall, slow response time, and inaccurate visual spatial skills. Their recognition is better than their recall • Multiple Sclerosis can present in many, MANY, different ways o Subtypes include: ▪ Relapse/remitting; lesions found at the ventricular system (periventricular lesions). Perhaps this is where the immune system gains access to white matter • They go through asymptomatic and symptomatic phases ▪ Chronic, progressive; symptoms gets worse o Symptoms include dysarthria, eye muscle imbalance (this causes double vision), transient blindness, numbness, fatigue, and weakness o It progresses at different rates in different people, and so neuropsychological tests findings are equivocal ▪ But, the WCST is predictive of progression Neurobehavioural Variables and Diagnostic Issues • Behavioural change can be affected by multiple factors, including lesion size, location, duration of condition, age of onset, cerebral dominance, psychological makeup, etc. o Also by whether the condition is diffuse or ocal. Focal injuries are often accompanied by diffuse damage, however Diffuse Brain Injury • Diffuse brain injuries typically result from widespread conditions like infections, anoxia, closed TBIs, and some degenerative conditions like CJD (Creutzfeldt-Jakob disease) o CJD characterized by sudden stroke-like symptoms, although the patient has not had a stroke. They die quickly (up the 45 days after onset; rapidly dementing) ▪ It creates holes in prions/neurons and spreads ▪ Incredibly rare. Sometimes spread genetically, sometimes through infection • Impairment in those with diffuse brain injury are in sustained and focused attention, memory, conceptual ability, and reasoning. These impairments affect all of their test scores o They perform accurately on tests, but slowly. This mental slowing reduces their test scores on attentional tasks. They will make errors on more cognitive demanding tasks o Short-term memory is generally intact. Repetition helps consolidate information when encoding and retrieval are impaired ▪ When a CVLT is given to test memory, they show a typical sequence of results • They do better on trials after repetition, and do better in cued recall and recognition trials • Attentional deficits can be tested with: o Digit symbol; they perform accurately but don’t finish much of the test o Ruff 2 and 7; participant is presented a line of numbers and must cross out all the 2s and 7s. Then shown a line of letters, 2s and 7s, and must cross out the 2s and 7s ▪ Failing in the second trial is indicative of impairment ▪ Those with diffuse brain injury are accurate (they don’t cross out letters, or the wrong numbers) but their speed is impaired o Brief Test of Attention; used to measure divided attention. Scores are impaired because it is cognitively demanding • Higher level cognition is impaired, resulting in conceptual concretism and inflexibility o They have borderline WVST scores, but no gross perseverations o They are slowed in the Tower of London task, but they ultimately solve the problem o Their TMTB score is markedly lower than their TMTA score because of cognitive demand o BADS, a multi-tasking task, shows poor scores because of cognitive demand and attentional difficulties • Response slowing is a hallmark of both cognitive and motor ability o Patients perform poorly on Finger Tapping Test and Grooved Pegboard regardless of hand – not a lateralized impairment Focal Brain Injuries • Showa clear lateralized difference in Finger Tapping and Grooved Pegboard, as the lesion is typically found mostly in one hemisphere • Do not exclusively use a cookbook approach to infer the location of the lesion (they are impaired in x, therefore their lesion is in y), but still consider some broad correlations o The Tower of London, TMT, BADS, RFFT, TVCF, and WCST are sensitive to frontal lobe PSYC31 Lecture 09: Neurobehavioural Variables • To test integrity of frontal lobes, use tests of executive function and conceptualization o Behavioural Assessment of the Dysexecutive Syndrome (BADS), Wisconsin Card Sorting Test (WCST), Ruff Figur
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