Exam III Random Mix Flashcards
4 Pillars of Psychological Assessment
- clinical interviews
- observations
- norm referenced tests
- informal data gathering
Acting Out
•immature defence mechanism •occurs when an individual becomes overwhelmed with feelings that are not socially acceptable and act them out
ADHD
•inattention or hyperactivity/impulsivity •6 months, maladaptive and inconsistent with what is expected for developmental level, must be present in at least two separate settings and before age 12 Diagnostic Criteria •inattention •hyperactivity/impulsivity Assessment •psychiatric evaluation Treatment •stimulant medications - methylphenidate and amphetamine salts •atomoxetine •alpha 2 agonists - guanfacine and clonidine •behavioural interventions •psychoeducation
agnosia
•lack of insight into one’s condition or lack of awareness of one’s deficits
agraphia
writing disorders
alexia
reading impairment
Altruism
•mature defence mechanism •subordination of your own needs and interested to those of others
Alzheimer’s
- most common form of degenerative dementia in US
- atrophy begins in entorhinal cortex and hippocampus
- as disease progresses, loss of brain volume and spreads more globally to involve most of areas of the cortex except the occipital poles
- There is no blood or cerebrospinal fluid study that is diagnostic. An elevated level of phosphorylated tau in the CSF with a decreased level of beta-amyloid is highly suggestive of the diagnosis of Alzheimer’s.
Drug development for the treatment of dementias has almost exclusively been for Alzheimer’s. This is partially because Alzheimer’s is the most common form of dementia and impacts the most people. Rivastigmine did get approval for use in Parkinson’s with dementia. Acetylcholinesterase inhibitors are the main treatment when it comes to Alzheimer’s dementia. Donepezil (Aricept), Galantamine (Razadyne ER) and Rivastigmine (Exelon) are the three medications that are available in this group. Efficacy and side effect profile are generally the same between the medications. As the group name implies, these medications work to increase the amount of acetylcholine available for cognitive function. Memantine (Namenda) is the only other approved medication for the management of Alzheimer’s. It is a N-methyl-D-aspartate antagonist. By blocking the activity of NMDA themedication is felt to keep brain cells alive longer. It is most often used along with a cholinesterase inhibitor as it shows the greatest benefit in combination therapy. Many trials are underway to develop other treatments. Many of these medications are looking at ways to decrease the amyloid build up in the brain. Patients tend to benefit from staying active, mentally as well as physically. Diets may be helpful in the maintenance of cognition. Decreasing stroke risk factors seems to be beneficial as well.
Anaplastic Astrocytoma
If mitotic figures are identified in a diffuse astrocytoma, this tumor is graded as anaplastic (WHO grade II). In general these tumors show increased pleomorphism, anaplasia and cellularity over the low grade tumors. The finding of mitotic figures in a diffuse astrocytoma reduces the prognosis to 2-4 years, although it is likely that the IDH mutant tumors will do better. On imaging these tumors look similar to WHO grade II diffuse astrocytomas.
anomia
naming impairment
Anomic Aphasia
- A fluent aphasia with relatively good auditory comprehension and repetition. Conversational speech is fluent, with normal utterance length and grammatically well-formed sentences.
- Auditory comprehension is good for everyday conversation, but there may be some difficulty with complex syntax or in difficult listening situations.
- Repetition is generally preserved even for full-length sentences.
- Naming impairment, in the absence of other significant language deficits, is the hallmark of this aphasia type.
- Lesion location. Lesions in acute anomic aphasia are usually located outside the perisylvian language zone and involve the angular gyrus or the inferior temporal region. Moderate and mild aphasias of various types caused by perisylvian lesions may evolve to chronic anomic aphasia.
- Prognosis. Acute anomic aphasia frequently resolves to minimal language impairment in the form of occasional word-finding difficulty. Anomic aphasia is the most common evolutionary endpoint for mild-to-moderate aphasia of all types.
Anterior Cingulate Cortex
all prefrontal regions connect with the anterior cingulate cortex, whcih plays a role in decision making
aphasia
- acquired language deficit
- characterized by impaired word selection, language production, and language comprehension
- central language impairment, affects spoken language as well as comprehension and production of written language
- most common cause is ischemic stroke - left MCA
- traumatic brain injuries, cerebral hemorrhage - acute
- brain tumors - slow onset
- progressive aphasia - syndrome associated with focal cortical atrophy
- TIAs, migraine, seizures - transient aphasias
- lesions that isolate brain regions involved in semantic processing and the production of volitional speech
- extrasylvan aphasias are called transcortical aphasias
Aphasia Classification System
1) Fluency
- Fluent aphasias are characterized by plentiful verbal output consisting of wellarticulated, easily produced utterances of relatively normal length and prosody (i.e., variations of pitch, loudness, rhythm). Fluent aphasias are associated with posterior lesions that spare anterior cortical regions critical for motor control for speech.
- Nonfluent aphasias are characterized by sparse, effortful utterances of short phrase length and disrupted prosody. Nonfluent aphasias are associated with anterior lesions that compromise motor and premotor cortical regions involved in speech production.
2) Auditory Comprehension
- auditory processing is defective in most aphasic patients
- anterior lesions = mild
- posterior lesions = more significant
3) Repetition
- Repetition of spoken utterances requires an intact perisylvian region: Wernicke’s area, Broca’s area, and the arcuate fasciculus. Therefore, lesions anywhere in the perisylvian region are likely to disrupt repetition.
- Extrasylvian lesions are characterized by preserved repetition despite severe reduction of spontaneous speech, severe comprehension disturbance, or both.
4) Naming
All individuals with aphasia exhibit naming impairment, or anomia, usually in combination with other language deficits. However, naming impairment can also occur in relative isolation in patients with anomic aphasia. Due to its ubiquitous nature, anomia is considered the least useful localizing sign in aphasia.
apraxia of speech
a motor speech disorder that reflects impaired motor programming for speech that result in difficulty planning and sequencing speech sounds
Brainstem Centers for Consciousness - ARAS
We now know that cholinergic cells in the midbrain reticular formation, together with monoaminergic fibers passing through it, are necessary to maintain a state of consciousness. This brainstem system responsible for maintaining consciousness is called the Ascending Reticular Activating System (often abbreviated ARAS). The ARAS works to promote wakefulness in two general ways, by affecting both thalamus and cortex. It projects to thalamic relay and association nuclei, shifting their cells into tonic mode; it also projects to some of the midline and intralaminar nuclei of the thalamus, which in turn project to widespread cortical areas, in some way “activating” them. The monoaminergic fibers also project through the medial forebrain bundle directly to widespread cortical areas. Reticular structures in the rostral medulla and caudal pons periodically participate in turning the ARAS off and on, producing sleep and wakefulness. Hence, a midpontine lesion that disconnects these more caudal structures leaves the ARAS on constantly.
Autism Spectrum Disorder
Diagnostic Criteria •deficits in social communication and social interactions across multiple contexts -social-emotional reciprocity -nonverbal communicative behaviors -developing, maintains and understanding relationships •restricted or repetitive patterns of behavior, interests or activities -stereotyped or repetitive motor movements - echolalia -insistence on sameness -highly restricted, fixated interests -Hoyer or hyporeactivity to sensory input Assessment •M-CHAT - preschool •standard psychiatric evaluation - ADI, ADOS •medical asssessment •psychological assessment of cognitive ability and adaptive skills for treatment planning •communication assessment Treatment •behavioural theory - ABA •pharmacological therapy - none for ASD -irritability/aggression - risperidone, aripiprazole -hyperactivity and inattention - methylphenidate, amphetamine salts, clonidine and guanfacine
Automaticity of Breathing Control
The automaticity of breathing is controlled a respiratory pattern generator located at the brainstem. The pacemaker neurons for breathing are located in the medulla called the pre-Boztinger complex. The neurons in the pre-Botzinger complex are glutaminergic but they have receptors for substance P and for 𝜇 −opioids; subtance P accelerates breathing whereas opioids slow breathing (this makes sense since opioids cause respiratory depression). The output of the medullary pattern generator is conveyed to lower motor neuron in the cervical spine that innervate the diaphragm and the intercostal muscles. `Neurons in the medulla receive modulatory inputs from the pons and the hypothalamus. Therefore, damage to the hypothalamus and pontine centers cause changes in respiratory rhythm, but do not abolish the rhythmicity of breathing. Damage to the medullary centers, especially the pre-Botzinger complex, interrupts the activity of pacemaker neurons and cause apnea and death.
Brain Regions Involved in Language
- Broca’a Area (opercular and triangular parts of the inferior frontal gyrus)
- planum temporale (superior surface of the temporal lobe behind auditory cortex)
- Wernicke’s Area (posterior superior temporal gyrus)
- angular and supramarginal gyri
*Broca’s Area, Wernicke’s Area and the arcuate fasciculus {arcuate fasciculus (Latin: curved bundle) is a bundle of axons that connects Broca’s area and Wernicke’s area in the brain. It is an association fiber tract connecting caudal temporal cortex and inferior frontal lobe} are called “perisylvan” because they are located aroud the left sylvan fissure (lateral sulcus)
Broca’s Aphasia
- A nonfluent aphasia in which there is relatively good comprehension and poor repetition. Conversational speech is nonfluent, with slow, halting speech production. Utterances are of reduced length (typically less than four words) with simplified grammar.
- Auditory comprehension is relatively good for conversational speech, but there is considerable difficulty with complex syntax or multistep commands.
- Repetition is limited to single words and short phrases, typically commensurate with the length of spontaneous utterances.
- Naming is always impaired to some degree, especially for low frequency words. Motor speech production deficits may interfere with intelligibility.
- Lesion location. Lesions restricted to Broca’s area cause only transient disruption of speech production and fluency. Persistent Broca’s aphasia results from much larger perisylvian lesions encompassing much more of the left frontal lobe.
- Prognosis. Broca’s aphasia typically evolves toward anomic aphasia. As grammatical complexity, utterance length, and prosody improve, spoken output may be characterized as borderline fluent.
Chronic Traumatic Encephalopathy
CTE is increasingly being recognized as a serious consequence of repetitive mild traumatic brain injury. CTE was originally known as dementia pugilistica based on its early descriptions in boxers. The first description in retired NFL football plays was made by Omalu et al. in 2005 and large series of cases has been reported by McKee et al, in 2012. While this entity is currently being actively debated, from a Neuropathology standpoint, the evidence is entirely convincing that the repeated head trauma of professional sports results in a neurodegenerative disease. Microscopically, CTE it is characterized by the abnormal accumulation of hyperphosphorylated tau protein in a distinct pattern. In CTE, immunohistochemistry shows tau deposition in neurons and astrocytes surrounding blood vessels. There is a predilection for tau at the depths of the sulci. Neurofibrillary tangles are also identified in the superficial layers of the cerebral cortex. Tau deposition is associated with widespread axonal disruption (producing swellings) and axonal degeneration
Circadian Rhythm Sleep Disorder
•transient
- acute work shift change
- jet lag
•persistent
-constant changing of work shifts
Cognitive Executive Functions
Verbal reasoning
Problem solving
Planning and sequencing
The ability to sustain attention
The ability to resist distractors/interference
Judicious use of feedback
Flexible thinking
Optimal response to novelty
Depends on the dorsolateral PFC (DLPFC)
Linked to cognitive functions
Comprehenesion of Language
- The auditory pathway for spoken language involves the cochlea, 8th cranial nerve, brainstem (bilaterally after the cochlear nuclei, up through the lateral lemniscus to the inferior colliculus), then through the inferior brachium to the thalamus (medial geniculate nucleus), and finally through the internal capsule to the primary auditory cortex (Heschl’s gyrus). Auditory information from primary auditory areas projects to Wernicke’s area.
- The visual pathway for written language involves the retina, optic nerve, optic tract, lateral geniculate nucleus of the thalamus, and through the internal capsule to primary visual cortex in the occipital lobe. Higher level processing of written information relies on adjacent areas in the temporo-parietal association areas.
Conditioned reinforcer
•associated with a primary reinforcer - something that derives its reinforcing value from primary reinforcers such as money and praise
Conduct Disorder
•repetitive pattern of behavior that violates the rights of other or major societal rules Diagnostic Criteria - 3 for at least 12 months, and one in the last 6 months •aggression to people/animals •serious violation of rules •destruction of property •deceitfulness/theft Treatment •psychotherapy •psychopharmacology -treatment of agression with alpha 2 agonists or mood stabilizers
Conduction Aphasia
- A fluent aphasia in which there is good comprehension but poor repetition. Conversational speech is fluent with relatively normal utterance length but marred by paraphasias.
- Auditory comprehension is relatively good for casual conversation. Single-word comprehension is well preserved, but patients may have difficulty with complex syntax or multi-step commands.
- Repetition impairment is the hallmark of conduction aphasia, despite relatively good spontaneous speech. Paraphasias tend to be particularly prominent during repetition tasks.
- Naming is always impaired.
- Lesion location. Conduction aphasia results from posterior perisylvian lesions affecting primarily the supramarginal gyrus in the parietal lobe and the arcuate fasciculus.
- Prognosis. Conduction aphasia may persist or may evolve into anomic aphasia.
Contusion
A contusion is often accompanied by altered consciousness secondary to a head injury. A contusion is caused by blunt trauma, similar to bruise in the rest of the body. The location of the brain damage depends on whether the head is moving or not. The injury will be at the site of impact (coup injury) if the head is stable and struck by a hard object (i.e. a baseball bat). If the head is moving, sudden deceleration will also result in the brain on the opposite side striking the skull. This results in a contrecoup injury which is often larger in size than the coup injury. Brain contusions are characterized by hemorrhage, tissue necrosis and edema. There is a progression of the pathological findings, with neutrophils, followed by macrophages and phagocytosis of necrotic tissue. The injuries are most pronounced at the surface of the brain (crests of gyri) since this is the site of the direct force. The inferior frontal lobes and the inferior surface of the temporal lobes are the most commonly affected because the inner surface of the skull is rough and irregular in these areas. Old contusions are characterized by depressed, yellowish brown patches.
•moderate to severe TBI
Countertransference
The physician’s response to the patient; the same emotional expectation that the relationship with the patient will be similar to one from the past.
Criterion Referenced Test
- Criterion-referenced testing or interpretation involves scoring an examinee’s test performance in terms of how much he/she has mastered the domain being assessed
- Results are giving meaning by comparing them with a preset standard (e.g. at Walden Grove High School - “Every student who scores above 75% will pass”)
- Also known as content- and domain- referenced testing
CTE
- chronic traumatic encephalopathy
- first called dementia pugilistica
- caused by repeated mild traumatic brain injury
- accumulation of tau protein in neurons and astrocytes distributed around small blood vessels at the depths of cortical sulci and in an irregular pattern
- tau positive tangles
Damage to DLPFC
Damage to the DLPFC impairs mainly “cold” executive functions that require cognitive flexibility such as planning and prioritizing, decision-making, abstract thinking, and working memory. The opposite of cognitive flexibility is perseverance. Cognitive flexibility is manifest when a person is required to shift from one set of the rules to another. The Wisconsin card-sorting task is a classic test of cognitive flexibility and whether there might be damage in the DLPFC. The patient is asked to sort a deck of cards but not how to sort or match the cards; however, the patient is told whether a particular match is right or wrong. As the patient continues you change the rules of how to sort the cards. Patients with DLPFC damage are unable to switch between rules and they perseverate (to repeat something insistently) in sorting the cards based on the first instruction even though they are told it is wrong. Perseveration is a major component of the frontal syndromes in frontotemporal dementia, in adult ADHD, and in multiple behavioral conditions related to trauma to the DLPFC. In other words, the DLPFC plays a role in being able to change cognitive tasks upon changing rules – or adapt quickly in figuring out tasks.
•Working Memory
- n-back and delayed response task
- dopamine
•problem solving, planning, sequencing
Damage to VMPFC
Damage to the VMPFC leads to preserved working memory and reasonable planning but severe disruption in social behavior, judgment, impulsivity, and personality. Typically patients with ventromedial PFC lesions become impulsive. Impulsivity can be tested by the Iowa gambling task.
The VMPFC computes the value of rewards, punishments, risks, and even of regret based on the time elapsed between the stimulus and the outcome.The VMPFC computes the value of rewards, punishments, risks, and even of regret based on the time elapsed between the stimulus and the outcome.
The VMPFC computes the value of rewards, punishments, risks, and even of regret based on the time elapsed between the stimulus and the outcome.
Decerebrate Posturing
•Decerebrate posture is an abnormal body posture that involves the arms and legs being held straight out, the toes being pointed downward, and the head and neck being arched backward. The muscles are tightened and held rigidly. This type of posturing usually means there has been severe damage to the brain.
Decorticate Posturing
•Decorticate posture is an abnormal posturing in which a person is stiff with bent arms, clenched fists, and legs held out straight. The arms are bent in toward the body and the wrists and fingers are bent and held on the chest. This type of posturing is a sign of severe damage in the brain.
Dementia with Lewy Bodies
Dementia with Lewy bodies is the second most common form of degenerative dementia after Alzheimer’s disease. As with any dementia a decline in cognition is seen in dementia with Lewy bodies. Associated findings include delusions and hallucinations. These are seen early in the process of the illness. Additionally, individuals are sensitive to medications. Dopaminergic medications may exacerbate the experience of psychosis. Dopamine blocking agents may lead to rigidity and features of Parkinson’s. Despite the overlap with Parkinson’s, dementia is the prominent symptoms and motor signs are less severe. Often the course of the illness is recognized as fluctuating and families will report that at one moment the patient seems completely normal, while at another they are completely confused. Another associated symptom is REM sleep behaviors. The person may start talking in their sleep, walking in their sleep or even acting out their dreams. Unlike Parkinson’s where Lewy bodies are visualized in the midbrain, especially the substantia nigra, in dementia with Lewy bodies, Lewy bodies are found in the brainstem as well as widespread throughout the cerebral cortex.
Denial
•immature defence mechanism •direct disavowal of a painful reality that is overwhelmingly disturbing
Diffuse Astrocytoma
These diffuse infiltrating astrocytomas show only a mild increase in cellularity, minimal atypia and no mitoses. MRI scans show an ill-defined, non-enhancing expansion of brain parenchyma because of infiltration of the neoplastic cells. IDH mutation is present in the majority of cases. The median time survival of a patient IDH mutant diffuse astrocytoma is approximately 10 years. Without IDH mutation, the median time for survival is 4-5 years.
Diffuse Astrocytoma
The diffuse astrocytoma is the most common primary brain tumor. Diffuse astrocytomas are commonly seen in the cerebral hemispheres of adults but can occur at any age and in any location. A diffuse astrocytoma is characterized by neoplastic astrocytes that migrate and infiltrate as single cells within brain parenchyma. For that reason they are commonly termed diffuse and infiltrating.
Three Grades: no Grade I because it cannot be resected
- diffuse astrocytoma (WHO Grade II)
- anaplastic astrocytoma (WHO Grade III)
- glioblastoma (WHO Grade IV)
Diffuse Axonal Injury
Severe head trauma resulting in a coma is often accompanied by diffuse axonal injury. This is commonly seen within the deep white matter especially the corpus callosum and periventricular white matter. It is caused when there is movement of the brain within the skull and axons are injured by mechanical forces. Rotational acceleration and deceleration cause shearing forces which tears axons. Microscopically, diffuse axonal injury is characterized by widespread axonal swelling. Axonal transport continues even after an axons is torn, which leads to a build-up of proteins in the segment still attached to the cell body. Ultimately, diffuse axonal injury causes degeneration of the fiber tracts distal to the torn axons
Diffuse Axonal Injury
- severe head trauma
- deep white matter, especially corpus callosum and periventricular white matter
- mechanical forces - rotaional acceleration and deceleration
- widespread axonal swelling
- ultimately diffuse axonal injury causes degeneration of fiber tracts distal to the torn axons
- any severity of injury
Displacement
•immature defence mechanism •unconscious process by which feelings attached to one source are redirected to another
Disruptive Mood Dysregulation Disorder (DMDD)
•frequent, severe, recurrent temper outbursts and chronically irritable and/or angry mood - decrease misdiagnosis of pediatric bipolar disorder Diagnostic Criteria •severe outbursts Treatment •still working on it…
Dissociation
•immature defence mechanism •the splitting off one’s thoughts and associated feelings from conscious awareness
dysarthria
Imprecise articulation due to muscle weakness or incoordination that results from damage to motor areas (including motor cortex, basal ganglia, cerebellum, upper motor neurons, lower motor neurons, neuromuscular junction, or muscle)
Emotional Executive Functions
Regulation of one’s own emotions
Decision making and risk taking
Impulse control
Insight and foresight
The ability to maintain social relationships
Depends on the ventromedial PFC (VMPFC)
Linked to reward and punishment
Ependymoma
Not surprisingly, ependymomas are typically located in the ventricles (IV ventricle> lateral ventricles> III ventricle> aqueduct of Sylvius).
Growth of ependymomas into a ventricle will obstruct of the flow of CSF resulting in hydrocephalus from. These tumors also have a tendency to spread through the CSF.
Microscopically these tumors show a fibrillary background with structures termed rosettes. The rosettes are arrangements of tumor nuclei around a lumen (true ependymal rosette) or around a capillary (perivascular pseudorosettes).
The survival after the diagnosis of ependymoma is variable depending on the extent of resection and location.
Epidural Hematoma
Between the dura and the skull - associated with skull fractures.
Often due to a tear in the middle meningeal artery.
Sudden, rapidly evolving neurologic symptoms
Extinction
•withholding the consequences that reinforce behavior •extinction burst
Familial Tumor Syndromes
- variety, primarily involve the CNS and others that include the CNS along with many other systemic sites
- may be due to a mutation in an ioncogene resulting in an increase in function driving toward cancer cell proliferation or loss of function of a gene that normally acts as a tumor suppressor
ex. neurofibromatosis 1 and 2, tuberous sclerosis, Von Hipple-Landau disease
Fixation
•immature defence mechanism •seen when one avoids seeing their own mistakes and continue behavior, or react to a situation, even though it has been determined to be maladaptive
Fixed Interval Schedule
•No behavior will be reinforced until the time has elapsed •engaging in the behavior is pointless until the time elapses •rate of behavior increases near the end of the interval in a rapid and steady manner
Fixed Ratio Schedules
•a behavior is reinforced when it has occurred a fixed number of times •perform at a high rate, often punctuated by short pauses after the reinforcement - post reinforcement pauses •FR1 = CRF Ex: paid for each shirt sewn or each bushel picked
Forebrain Centers for Consciousness
Clinical observations indicate that damage around the walls of the third ventricle in the mid-to-caudal hypothalamus cause drowsiness and lethargy. Conversely, damage in the vicinity of the lamina terminalis causes inability to sleep. Thus, there appear to be additional sleep and wakefulness centers in the forebrain. Yet another collection of monoaminergic neurons is located in the hypothalamus near the mammillary bodies (in the tuberomammillary nucleus). Like neurons of the locus ceruleus and raphe nuclei, they project to widespread CNS areas, in this case releasing histamine (Fig. 5). Histamine depolarizes both thalamic and cortical neurons, and turning these histaminergic neurons off causes drowsiness or sleep (like antihistamines such as Benadryl® that block the H1 receptor). In addition, a set of neurons in the lateral midhypothalamus has widespread excitatory connections that use a neuropeptide called orexin (also known as hypocretin) (Fig.6) that may be the master controller since they communicate with the histamine neurons and the ARAS of the brainstem. Finally, cholinergic neurons of the basal nucleus increase the excitability of cortical neurons. GABAergic neurons of the preoptic region and anterior hypothalamus periodically inhibit the orexin and histamine neurons and the other wakefulness-promoting neurons, helping to cause sleep. These neurons get input from the suprachiasmatic nucleus.
Frontal Syndrome
The symptoms of the frontal syndrome include loss of desired behavior and gain of undesired behavior. The losses of desired behaviors include: abulia (lack of will), apathy, lack of spontaneity, akinetic mutism, pseudodepressive state, lack of drive, poor motivation. The gains of undesired behaviors include: unjustified euphoric state, distractibility, impulsivity, disinhibition, irritability, restlessness, moria (childish excitement, not Middle Earth), pseudopsychopathic state, anosognosia, indifference, confabulation, and perseveration.
Clinically, it is important to recognize the symptoms because the diagnosis of frontal syndrome interferes with decisions making and has major implications for the patient’s autonomy. The frontal syndrome is prevalent because it can appear as a central entity (e.g., in traumatic brain injury, stroke, and tumors in the frontal lobe) or it can accompany other disorders such as adult forms of attention-deficit disorder (ADHD), addiction, schizophrenia, various forms of dementia (especially fronto-temporal dementia), and also to some degree normal aging. Classically the symptoms of the frontal syndrome can be grouped into three clusters:
1) Cognitive Deficits
2) Supervisory Deficits
3) Deficits of Initiation and Inhibition
links between the prefrontal cortex and the basal ganglia
- motor loop
- cognitive loop: DLFPC, caudate nucleus and dorsolmedial This loop is responsible for the initiation and termination of cognitive processes involved in focused attention on a task, planning, and loading or erasing the content of working memory. This loop initiates and terminates thoughts the same way the motor loop initiate and terminates movements.
- limbic loops that regulate emotional behaviors, emotional impulses, and in general drive-related behaviors: anterior cingulate cortex, the surrounding medial PFC, and the nucleus accumbens. While the motor loop receives dopamine from the substantia nigra, the cognitive and limbic loops receive dopamine from the ventral tegmental areas.
- trauma, tumors, infections (dental abscesses) and symptoms depend on the prefrontal region affected
- diagnosis requires symptoms form all three classes
- ADHD
- rarely finish their education (deficits of concentration, planning, organization), they cannot hold a job (they are unreliable, disorganized, inefficient, socially and emotionally impulsive) and cannot manage their finances, their health care, their family obligations. Typically, adult ADHD has comorbidities with anxiety and depression (as their deficits are regarded as flaws of character rather than a veritable mental dysfunction) and they are powerless in overcoming the challenges of school, work, and everyday life without psychiatric attention. Adult ADHD patients respond well to stimulants, SNRI’s, and recently to blood pressure medications. •schizophrenia
- often unable to separate and process accordingly task-relevant and task-irrelevant information. This process requires a normal flow of information between the prefrontal cortex, other association cortices, and the other areas of the brain. These types of deficits led to the formulation of disconnection syndromes that are frontal syndromes caused by remote lesions, such as strokes in the thalamus or the basal ganglia. Both clinical and recent MRI studies show that frontal lobe dysfunction can be caused by damage to areas outside the prefrontal cortex. The explanation for this phenomenon (called diaschisis) is the dynamic interruption of circuits established between the prefrontal cortex to other areas of the brain. The disruption of a single segment of these circuits is sufficient to compromise the complex functions of the entire prefrontal cortex.
DLPFC (cognitive):
- Working memory, problem solving, mental flexibility, symbolic thinking
- distracted on delayed responses task, cannot solve the Tower of Hanoi, persevernace on card sorting task, cannot explain proverbs/concepts
VMPFC (supervisory):
- impulse control, emotional stability, social incompetence
- losses on Iowa ganbling task, frequent mood swings, lack of empathy and inability to obey social rules
DLPFC/VMPFC (initiation/inhibition):
- motor perseverance, verbal perseverence, inability to function
- utilization behavior, poor word generation/echolalia, lack ofmotivation and organization to carry out simple daily activities
Frontotemporal Dementia
Frontotemporal dementia is thought to be at least as common as early-onset Alzheimer’s dementia (Alzheimer’s that develops before the age of 60). The prevalence may be approximately 15 per 100,000 persons aged between 45 and 64 years. Generally speaking, individuals tend to present with frontotemporal dementia at a younger age compared to individuals with Alzheimer’s. Variants of frontotemporal include a behavioral form where changes in personality and behavior are seen early on. They also tend as a group to have disordered initiation, goal-setting and planning. Another form of frontotemporal dementia has a greater impact on language. Individuals with this form exhibit profound anomia associated with progressive loss of conceptual knowledge of words . Patients with predominantly right temporal degeneration are associated with deficits in empathy and knowledge about people’s emotions.
MRI is the best imaging modality for visualizing atrophy. CTs are good if the person is unable to get an MRI. Functional imaging such as SPECT and PET scans are becoming more common. Most often these modalities are most helpful to confirm suspicion of dementia type such as frontotemporal dementia, where decreased metabolism in the frontal and temporal lobes might be seen, versus Alzheimer’s, where decreased metabolism in the parietal and posterior temporal areas may be seen initially.
General Anesthetics
Are used to induce states of:
1) analgesia
2) amnesia
3) loss of consciousness
4) skeletal muscle relaxation
5) inhibition of sensory and autonomic reflexes
2 important physiological effects:
1) hyperpolarize neurons
2) modulate synaptic transmission
Glioblastoma
This is a diffuse infiltrating astrocytoma with features of anaplastic astrocytoma (mitotic figures) but also necrosis and/or microvascular proliferation.
In many cases the tumor cells line up around the necrosis and this feature is termed pseudopalisading necrosis. The microvascular proliferation is so abundant that there are loops upon loops of endothelial cells resembling renal glomeruli (glomeruloid vascular proliferation). This is due to the secretion of a vascular endothelial growth factor by the tumor. Glioblastomas commonly infiltrate through the white matter tracts of the corpus callosum into the other hemisphere resulting in a characteristic “butterfly appearance” grossly.
Glioblastomas often appear as ring-enhancing lesions on MRI scans due to central areas of necrosis and a ring-like enhancement due to breakdown of the blood brain barrier from the proliferating vessels. This “ring enhancing” appearance may also be seen with abscess formation, metastases, or primary CNS lymphoma. Rarely, acute multiple sclerosis can show a mass lesion with ring enhancement.
Because the neoplastic cells travel widely along white matter tracts, even when the scans look like the tumor has been totally removed by surgery (called a gross-total resection), there are microscopic cells left behind.
Glioblastoma has a bad prognosis, with most patients dying within 1-2 years after diagnosis. MGMT promotor methylation and IDH mutations are favorable prognostic features.
Global Aphasia
- A severe nonfluent aphasia with poor auditory comprehension and poor repetition. Conversational speech is nonfluent with slow, halting speech production. Utterances may be restricted to single words or phrases that are perseverative, such as I can see.
- Auditory comprehension is reduced to the extent that even single-word comprehension is significantly compromised. Comprehension is markedly impaired at the phrase or sentence level.
- Repetition is defective and even single words may not be repeated accurately.
- Naming is severely impaired.
- Lesion location. Lesions in global aphasia are extensive and typically involve the entire left perisylvian language zone. A common cause is embolic occlusion of the main stem of the middle cerebral artery.
- Prognosis. Global aphasia may evolve to Broca’s aphasia, or it may persist as global aphasia.
Huntington’s Disease
- a hereditary (autosomal dominant) disorder that affects the basal ganglia and the cerebral cortex. The onset of symptoms is usually in the 4th to 5th decades. The clinical findings are characterized by a triad of involuntary movements (chorea), dementia and psychiatric disturbances (especially depression). Movement abnormalities include chorea, dystonic posturing and dysarthria. Pathologically, Huntington’s disease is characterized by symmetrical degeneration of caudate nucleus and putamen, which may be seen on CT scan. There may also be degeneration of the frontal lobes. Microscopically, loss of neurons with gliosis is seen in caudate and putamen
- the CAG trinucleotide region is expanded in length in a gene that encodes a protein called huntingtin. Although the function of the huntingtin protein is unknown, it is essential, because targeted gene disruption in the mouse leads to a lethal phenotype. The normal huntingtin gene has less than 26 CAG repeats. With a repeat count of more than 40, there is full penetrance of the gene and the disease will be transmitted in an autosomal dominant fashion. The longer the CAG repeat region, the earlier and more severe the disease. There is a tendency of repeats to expand when transmitted from parent to child (called anticipation). The long polyglutamine region results in protein misfolding which impedes clearance. This toxic misfolded protein can be demonstrated in the nucleus of caudate neurons by immunohistochemistry
Humour
•mature defence mechanism •ability to playfully poke fun at yourself and the situation you are in
Idealisation
•immature defence mechanism •when one sees another individual as perfect and ignores any faults of that individual
Identification (with an aggressor)
•immature defence mechanism •occurs when one perceives another as a threat and tries to emulate their behavior in an unconscious effort to pacify them
Infant and Preschool Tests
Infant and preschool tests are generally considered valid as screening devices for developmental delays and disabilities
- Prior to Age 2 - little predictive validity
- E.g., Denver Development Screen Test, Bayley Scales, Fagan Test of Intelligence
Intellectualization
•immature defence mechanism •thinking or talking about an emotional subject in an unemotional way
Intelligence Testing
Intelligence Quotient (IQ) – is a general estimate of the functional capacity of a person.
IQ is not an absolute score, but a comparison among people.
Studies suggest that 70% is inherited.
- Previously used IQ formula: – Mental age (MA) method: • IQ = mental age/chronological age x 100 – **Now use standardized tests
- Verbal IQ; Performance IQ; Full scale IQ
- Wechsler Adult Intelligence Scale (WAIS-R) (ages >17)
- Wechsler Intelligence Scale for Children, fifth edition (WISC-V) (ages 6:0 – 16:11)
- Wechsler Preschool and Primary Scale of Intelligence (WPPSI-IV) (ages 2:6 – 7:7)
- Stanford-Binet Intelligence Scales, 5th edition (ages 2 – 85)
- Universal Nonverbal Intelligence Test 2 (UNIT2) (ages 5 – 21:11)
Intraparenchymal hemorrhage
Contusions can lead to bleeding into the brain, preferentially at the crests of gyri
Other causes of brain hemorrhage include hypertension, cerebral amyloid angiopathy, tumors and vascular malformations.
Isolation of Affect
•immature defence mechanism •separation of affect from ideation
Kaufman Assessment Battery for Children
Development of the K-ABC was based on Luria’s distinction between sequential and simultaneous processing
• Emphasis on nonverbal items, makes it – “culturally-fair” and appropriate for – language impaired
Leukodystrophies
- mutations in genes whose protein products are involved in the generation, turnover or maintenace of myelin
- unlike demyelinating diseases, these diseases present at an early age and are characterized by progressive deterioration of cerebral function
- diffuse, symmetric deterioration of white matter
- Krabbe disease, metabolic leukodystrophy and adrenoleukodystrophy
Levels of Consciousness
- Alert
- Lethargic (somnolent): drowsy and may fall asleep if not stimulated in some way, easily sidetracked
- Obtunded: difficult to arouse froma somnolent state and is frequently confused when awake, repeated stimulatoin to maintain consciousness, unproductive interactions with examiner
- Stuporous (semicoma): responds only to strong, generally noxious stimuli, when aroused patientis unable to interact with examiner
- Coma (deep coma): cannot be aroused by any type of stimulation, reflex motor responses may or may not be seen
Locked In State
- basal pontine storke could destroy the descending corticospinal and corticobulbar fibers bilaterally but leave the ARAS intact
- normal sleep-wake cycles but unable to communicate except with limited eye movements
Lysosomal Storage Diseases
- characterized by mutations in the genes encoding lysosomal enzymes - loss of function of these enzymes leads to accumulation of storgae material which may eventaully lad to cell death
- can be divided into catefores based on the nature of the accumulated material
- predominanlty grey matter (neuronal storage disease) or white matter (leukodystrophy) or both
- systemic disease may also be observed
- neuronal storage disease: accumulation of storage material within neurons usually leading to death of cell ex. Tay-Sachs, Niemann-Pick Disease, mucopolysaccharidoses and ceroid lipofuscinosis
- Leukodystrophies: mutations in genes whose protein products are involved in teh generation, turnover or mainteneance of myelin ex. Krabbe disease, metabolic leukodystrophy and adrenoleukodystophy
- Metachromatic leukodystrophy: an autosomal recessive lysosomal storage disorder
Medulloblastoma
This is an undifferentiated tumor with a high nuclear/cytoplasmic ratio (small blue cell tumor) and frequent mitoses.
Some may show rosettes with a fibrillary center (Homer-Wright Rosettes). By definition, medulloblastomas are found in the cerebellum.
Tumors with this undifferentiated morphologic appearance in other areas of the brain are called CNS embryonal tumors.
Medulloblastomas are extensively subdivided based on morphology and molecular characteristics and these features will impact prognosis.
Many medulloblastomas are very responsive to radiation treatment with a 75% five-year survival after treatment. Because of the tendency of these tumors to spread through the cerebrospinal fluid, radiation of the entire brain and spinal cord (the entire neuroaxis) is necessary.
Meningioma
These tumors show the differentiating features of meningothelial cells. Histologically they are characterized by benign appearing nuclei with indistinct cellular borders, whorl formation and psammoma bodies.
Grossly, they are well-circumscribed tumors that compress but generally do not invade the brain. They typically are attached to the dura.
Most meningiomas are slowly growing and have an excellent prognosis if completely excised.
Meningiomas with atypical features (WHO grade II, with mitoses, necrosis, increased cellularity and atypia) have a higher rate of recurrence.
Brain invasion signifies a greater tendency for recurrence after excision and are also considered WHO grade II tumors (atypical meningiomas).
An anaplastic meningioma (WHO grade III) is a highly aggressive tumor with the appearance of a high-grade sarcoma and very high mitotic rates.
Metachromatic Leukodystrophy
• an autosomal recessive lysosomal storage disorder caused by the deficiency of aryl-sulphatase A. Deficiency of this enzyme causes lysosomal storage of the sphingolipid cerebroside sulphate (a sulfatide), a lipid that is abundant in myelin. Storage of sulfatides in the lysosomes of oligodendroglial cells and Schwann cells is toxic resulting in demyelination of both the CNS and PNS. Within the brain and peripheral nerve, the storage material may also be identified in macrophages. Clinically the disease is heterogeneous, and there are three different types of metachromatic dystrophy which vary by age of onset. The name of the disease “metachromatic” is based on the brown color of the storage material when stained using a blue dye.
Metastatic
Metastatic tumors are high on the differential diagnosis of ring-enhancing lesions on MRI. The microscopic appearance is similar to the primary tumor: e.g. carcinoma, sarcoma, lymphomas. Lung, breast, skin (melanoma), gastrointestinal and kidney are the most common sites of origin.
Minimally Conscious State
•The definition of minimally conscious state is not completely codified. The minimally conscious state is defined as severely altered consciousness in which minimal but definite, sustained and/or reproducible behavioral evidence of awareness of self or environment is demonstrated. The person may have periods where they can communicate or respond to commands, such as moving a finger when asked. A person may enter a minimally conscious state after being in a coma orvegetative state. In some cases a minimally conscious state is a stage on the route to recovery, but in others it’s permanent. To make the diagnosis of MCS, limited but clearly discernible evidence of self or of environmental awareness must be demonstrated on a reproducible or sustained basis by one or more of the following behaviors:
- Follow simple commands (eg touch your nose, look up).
- Gestural or verbal yes/no responses (regardless of accuracy).
- Intelligible verbalization.
- Purposeful behavior that is not due to reflexive activity.
- Appropriate smiling or crying in response to emotional but not to neutral topics or stimuli
- Reaching for objects demonstrating a clear relationship between object location and direction of reach
- Touching or holding objects appropriately in relation to the size and shape of the object
- Pursuit eye movement or sustained fixation that occurs in direct response to moving or salient stimuli.
Minimum Alveolar Concentration
- Finally, volatile general anesthetics are administered based on a concept of the minimal alveolar concentration (MAC). MAC is a concept used to compare the potency of volatile anesthetics. MAC concentrations are reported based on the concentration of vapors delivered to the lungs that prevents motor responses in 50% of subjects in response to a painful stimulus. Things that may alter MAC include age (i.e., 6%/decade), hypotension, pregnancy, metabolic acidosis, hypoxemia, body temperature and other medications/medications)…. Most importantly is to understand that the Anesthesia achieved depends upon the brain partial pressure. A good rule to remember is that the partial pressure in the Brain will be equal to the Alveolar partial pressure (i.e., Pbrain = Palveolar). The partial pressure of the alveolar (PA) is a balance between delivery of the medication to the alveolus and uptake of the medication into the blood and the body. Factors that influence uptake include the solubility (i.e., blood:gas partition coefficient), cardiac output and alveolus-venous pressure gradient. Remember the anesthesia one achieves is related to Pbrain = Palveolar ; So, more anesthetic dissolves in the blood, the less remains in the alveoli. More molecules of a soluble gas are required to saturate the liquid phase before increasing partial pressure. Therefore, the lower the blood:gas partition coefficient the faster the onset. (i.e., blood:gas partition coefficients = desflurane 0.45, nitrous oxide 0.47, sevoflurane 0.65, isoflurane 1.8 – speed of onset desflurane>nitrous oxide>sevoflurane>isoflurane).
- Some Rules to go by:
- Greater systemic uptake of anesthetic leads to lower mixed venous anesthetic concentrations and to a slower induction of anesthesia. (It also leads to greater alveolar-venous partial pressure gradients).
- Lower solubility in the blood compared to alveolar gas (lower blood/gas partition coefficient) leads to more rapid induction of anesthesia.
- Lower cardiac output leads to more rapid induction of anesthesia.
Minnesota Multiphasic Personality Inventory 2 (MMPI 2)
- The MMPI is used to screen for personality and psychosocial disorders in adults (i.e., over age 18) and adolescents age 14 to 18. It is also frequently administered as part of a neuropsychological test battery to evaluate cognitive functioning.
- The MMPI-2 is designed with 10 clinical scales which assess 10 major categories of abnormal human behavior, and four validity scales, which assess the person’s general test-taking attitude and whether they answered the items on the test in a truthful and accurate manner.
Assess test-taking attitudes to determine if the tests are valid
- High L – favorable light
- High F – carelessness (eccentric)
- High K – clinical defensiveness (faking good)
- Score on K is used to correct scores on several clinical scales
Mixed Transcortical Aphasia
- Also called the “isolation syndrome” because the perisylvian language zone is disconnected from extrasylvian regions.
- It is a nonfluent aphasia with poor comprehension but relatively preserved repetition. Conversational speech is similar to that found in global aphasia, in that meaningful verbal expression is severely limited or is absent altogether. Stereotyped utterances are common, as is echolalia – the inappropriate, and somewhat irrepressible, repetition of what others say.
- Auditory comprehension is markedly impaired, often even at the single-word level. Repetition of phrases and complete sentences is preserved, although repetition typically occurs without comprehension.
- Naming is significantly impaired.
- Lesion location. MTcA is seen in association with diffuse or multifocal lesions that result in anatomic isolation of the perisylvian language zone from surrounding cortical areas.
- Prognosis. Variable recovery occurs in MTcA; patients in whom the cause is vascular have the best chance of some recovery of language.
Narcolepsy
This is a strange disorder defined by a variable combination of excessive daytime sleepiness, often accompanied by irresistible sleep attacks; cataplexy, a sudden loss of muscle tone during wakefulness, usually triggered by emotion; sleep paralysis, a complete loss of muscle tone during sleep-wake transitions; and hallucinations during sleep-wake transitions. Narcoleptic patients usually have abnormal sleep at night; they typically slip almost immediately into REM and may have many awakenings. In just the last few years the major probable cause of narcolepsy has been figured out. The small group of peptidergic neurons in the tuberal hypothalamus, near the columns of the fornix, that release orexin (aka hypocretin) are selectively lost in most of the narcoleptic brains examined to date, perhaps through an autoimmune mechanism. Loss of input to histaminergic neurons and to REM-control circuitry could explain the symptoms of narcolepsy, and the development of orexin agonists may provide new treatments.
Negative reinforcement
Event that, when removed after a response, increases likelihood of that response occurring again - escape
Negative reinforcement
•a response is followed by the removal of or decrease in the intensity of a stimulus •and averse stimulus an organism tries to “escape” (Alarm clock, seat belt buzzer)
Neurofibromatosis Type 1
- an autosomal dominant disorder with a frequency of 1 in 3000. In half of the cases it is inherited, with the other half being new mutations. The NF1 gene is very large, with approximately 250,000 base pairs and thus there is a high number of spontaneous mutations. The NF1 gene encodes a protein called neurofibromin. This protein is found in many tissues and is thought to be a tumor suppressor gene that normally acts by converting RAS (an oncogene) from an active to an inactive form. RAS plays a key role in promoting cellular growth. A decreased cellular brake on RAS because of a mutated NF1 gene may explain the occurrence of multiple tumors in NF1.
- The clinical phenotype does not correlate with the type or location of the mutation, and the clinical course is highly variable. This is an example of variable expressivity, in which there are differing manifestations in people with the same genotype. Persons with NF1 may develop neurofibromas (both solitary and plexiform) of peripheral nerves, gliomas of the optic nerve, pheochromocytomas, Lisch nodules (pigmented nodules in the iris) and multiple cafe-au-lait spots on the skin.
- Neurofibromas are commonly found in the skin. They occur as elevated dermal lesions which microscopically consist of haphazard Schwann cells and fibroblasts. Growth may produce a nodule, or over time, a pedunculated mass. A patient with a simple neurofibroma of the skin may or may not have neurofibromatosis. If a neurofibroma expands a nerve to produce a complicated growth, it is termed a plexiform neurofibroma. Massive diffuse and plexiform neurofibromas of the skin may produce large tumors. Multiple skin tumors and/ or plexiform neurofibromas are consistent with a diagnosis of neurofibromatosis. Microscopically, plexiform neurofibromas consist of diffusely proliferating Schwann cells and fibroblasts, often in association with a myxoid (mucus-like) matrix. Because Neurofibromas incorporate the parent nerve, residual axons may be present within the substance of the tumor. Because these tumors grow within the nerve, tumor removal requires sacrifice of the nerve. Neurofibromas can undergo malignant degeneration and this is more likely to occur in larger tumors. Therefore, large neurofibromas that are resected should be examined for evidence of malignancy (numerous mitotic figures and high cellularity). These are termed malignant peripheral nerve sheath tumors.
Neurofibromatosis Type 2
- (central form of NF) is also an autosomal dominant disorder with a frequency of 1 in 50,000 to 100,000. Like NF1, this disease may be inherited or be a result of a new mutation. The NF2 gene produces a protein known as merlin which has a structural similarity to cytoskeletal proteins and is widely distributed in tissues throughout the body. This protein functions as a tumor suppressor that mediates contact inhibition of growth through signals from the extracellular matrix.
- Patients with NF2 develop vestibular schwannomas (typically bilateral), multiple meningiomas and spinal cord ependymomas. The schwannoma is a well circumscribed, encapsulated, benign tumor of Schwann cells attached to a peripheral or cranial nerve. Bilateral vestibular schwannomas are the hallmark of NF2. (Schwannomas are also common tumors throughout the peripheral nervous system and in the cranial nerves in individuals without gene mutations.) Vestibular schwannomas are located at cerebellopontine angle of the brainstem, attached to the vestibular portion of the eighth nerve. Microscopically, these tumors have a biphasic pattern with spindled cells palisading in compact zones (Antoni A) and microcystic zones with a loose meshwork of cells (Antoni B).
Norm referencd Tests
One of the most useful ways to compare an individual’s performance on a test to the test scores of others.
- Norms are average scores computed for a large representative sample of the population.
- Focus on validity and reliability of test
- An appropriate normative sample answers these:
– How large is the sample?
– When and Where was the sample gathered?
– How were individuals identified and selected?
– What was the composition of the sample (i.e. age, gender, sex, ethnicity, SES, education level, etc.)? Intelligence
Objective Tests
– Utilize simple stimuli
– Have a restricted range of possible responses (provided options)
– Scored mechanistically using a standardized scoring key
– Requires no clinical experience to score
– Some tests require specific training or a particular license to administer
Oligodendroglioma
Tumors that show the differentiating features of oligodendroglial cells are most commonly found in the cerebral hemispheres of adults.
Microscopically they are characterized by monotonous cells with round nuclei and clear cytoplasm (fried egg appearance), fine capillaries in chicken wire distribution and calcification.
Oligodendrogliomas are diffusely infiltrating tumors similar to the diffuse astrocytomas. On imaging, they are difficult to distinguish from low grade astrocytomas. They tend to be calcified but a small proportion of diffuse astrocytomas can also have calcifications.
A characteristic feature of oligodendrogliomas is loss of chromosomes 1p and 19q. Similar to grade astrocytomas, oligodendrogliomas are usually IDH mutant. Most are WHO grade II, but higher grades are also seen.
Operant conditioning
•striated muscles an volition •reinforcement INCREASES BEHAVIOR *positive: event that when presented after a response, increases likelihood of that response again *negative: event that, when removed after a response, increases likelihood of that response run again - escape •punishment *consequences that reduce the strength of behavior
Oppositional Defiant Disorder
•conflict with authority Diagnostic Criteria •6 on this with 4 symptoms Treatment •psychotherapy •psychopharmacology -aggression: clonidine, guanfacine –hyperactivity/inattention: clonidine and guanfacine or stimulants - methylphenidate and amphetamine salts or atomoxetine -anxiety and/or depressive symptoms: alpha 2 agonists or atomoxetine
Orbital Frontal Cortex
encodes value to both internal and external characteristics
Other Aphasia Syndromes
Many cortical lesions extend to subcortical regions, but aphasia can also result from deep lesions that appear to spare the cerebral cortex. Subcortical aphasias include thalamic aphasia and aphasia associated with damage to the basal ganglia and surrounding white matter pathways. Language impairment is one of the symptoms of generalized cognitive decline associated with dementia. In the case of Alzheimer’s disease, language deterioration typically follows a progressive course that begins with anomic aphasia, proceeds to transcortical sensory aphasia, and then to Wernicke’s aphasia, and ultimately becomes global aphasia. In some patients, progressive language deterioration occurs without significant dementia and is referred to as primary progressive aphasia (PPA).
Other Projective Tests
• Sentence Completion Test
– Semi-structured: provided with “stems” to complete
– Discovering attitudes, beliefs, motivations or other mental states
• Projective Drawings (e.g. Draw-A-Person test)
– Provided with a sheet of paper asked to draw a house, tree, person, family, etc.
– Limited validity (pathology or poor artist?)
paraphasia
incorrect word choice and sound substitution errors
Parasomnias
- Parasomnias are undesirable physical or behavioral phenomena that occur predominantly during sleep.
- disorders of arousals, such as sleepwalking or night terrors;
-classic disorders of arousal include sleepwalking, sleep terrors, and confusional arousals. These behaviors are more common in children and adolescents and are characterized as events occurring as partial arousals from the deeper stages of SWS. Sleep terrors start with a piercing scream or fright with significant sympathetic nervous system output. Patients have tachycardia, pupillary dilation, and sweating and appear wide-eyed and inconsolable. They occur during the deepest stages of SWS (stage III/IV) and therefore, unlike nightmares, often cannot remember the arousal episode. Confusional arousals occur following sudden awakening and may be accompanied with disorientation and the patient striking out.
- sleep-wake transition disorders, such as sleep talking; rhythmic movement disorder (e.g., head banging);
- REM parasomnias, such as REM sleep behavior disorder (RBD)
-malfunction of parts of the brainstem responsible for suppressing muscle tone during REM sleep can cause patients (usually older males) to thrash around in bed, sometimes seemingly acting out their dreams. Occasionally this is associated with a pontine lesion, but typically the cause is unknown.
Passive Aggression
•immature defence mechanism •occcurs when aggressive feelings to thoughts are disavowed, so one may engage in other behaviors as an outlet for the aggressive cognitions
Physical Findings in Comatose Patients - Posturing
- Either spontaneously or in response to stimuli, comatose patients may assume postures that are correlated with the level of the damage. The names decorticate and decerebrate have come to be associated with two of these postures.
- decorticate posturing usually means damage in the rostral midbrain or above
- decerebrate posturing usually means damage below the rostral midbrain.
- A transition from decorticate to decerebrate can mean something like compression moving down the brainstem.
Physical Findings in Comatose Patients - Pupils
- Lots of things can cause changes in behavior of the pupils. There are metabolic, pharmacological (i.e., opiates, excess acethylcholine) or diencephalic damage that causes a bilateral, small pupils that remain reactive to light.
- When both pupils are dilated and fixed (do not react to light) it could be a sign of diffuse damage, global hypoxia, barbiturates or atropine.
- One might expect various kinds of midbrain damage affecting parts of the pupillary reflex arc to have an effect on pupil size (e.g., uncal herniation can push unilaterally on CN III and cause one pupil to be dilated and fixed).
- In addition, bilateral pontine damage that causes coma typically results in tiny but still-reactive pupils. This apparently results from damage to descending sympathetic neurons (as in Horner’s syndrome) combined with disruption of normally present inhibitory inputs to the Edinger-Westphal nuclei.
- Whereas bilateral midbrain damage can result in pupils that are in a mid-position and fixed.
Physical Findings in Comatose Patients - Respiration
- There are a few repiratory patterns in coma that are diagnostic for the location of damage.
- Cheyne-Stokes respiration, is a waxing and waning breathing pattern with a period of 10–20 seconds, that indicates bilateral diencephalic damage, cardiac failure, or acidosis.
- Damage to the pontine respiratory centers (e.g., parabrachial nucleus) causes a pattern of respiration called apneustic breathing characterized by deep, gasping inspiration with a pause at full inspiration followed by a brief exhale.
- Upper medullary damage causes a respiratory pattern called ataxic breathing that is irregular and has periods or disorganized sequences of inhales and exhales interleaved with periods of apnea but some automaticity is still there because the pre-Botzinger cells are still intact. Finally, when the damage spreads to the preBotzinger complex of the medulla respiration ceases (respiratory failure).
Pilocytic Astrocytoma
WHO Grade I
These tumors commonly occur in the cerebellar hemispheres of children. Grossly, the pilocytic astrocytoma is a wellcircumscribed, homogeneously enhancing nodule associated with cyst formation. Microscopically there are pilocytic (hair-like or elongated) regions with eosinophilic inclusions termed Rosenthal fibers. The solid areas alternate with cystic regions that contain fluid. Mitotic figures are rare. The circumscription of pilocytic astrocytomas allows complete resection and therefore these tumors have an excellent prognosis.
Positive reinforcement
Event that, when presented after a response, increases likelihood of that response occurring again
Primary reinforcer
•appetitive stimulus - food, water, physical contact
Production of Language
We must make the distinction between speech and language production. Speech production involves moving the muscles, bones, and cartilage involved in articulation, primarily in the lower airway, larynx, pharynx, and mouth. Speech is the motor act that carries the linguistic message. Disorders of motor control for speech are distinct from language impairments, but can co-occur with aphasia.
Projection
•immature defence mechanism •unwanted wishes/feelings displaced onto another person
Projective Tests
- Use ambiguous stimuli and have a wide range of possible responses.
- Response meaning is interpreted (content analyzed) by clinical correlation between collected cases of responses and personal characteristics (psychopathologies).
- Tests are only scored by experienced clinicians using consensual standards
- Origins in psychoanalysis
Transcortical Motor Aphasia
- A nonfluent aphasia characterized by relatively good auditory comprehension and preserved repetition. Conversational speech is nonfluent, and patients make little attempt to produce speech spontaneously. In the acute stage, patients may be mute. When utterances are produced, usually after a long delay, they tend to be of reduced length (typically less than four words) and grammatical complexity.
- Auditory comprehension is good for most conversational interaction, but there may be difficulty with complex syntax or multi-step commands.
- Repetition is preserved in striking contrast to the virtual absence of spontaneous conversational speech.
- Naming can be relatively preserved.
- Lesion location. Lesions producing TcMA involve extrasylvian regions of the left frontal lobe. TcMA has also been described following dorsolateral frontal lesions located anterior or superior to Broca’s area. This is another lesion that causes the language area to be isolated.
- Prognosis. TcMA may essentially resolve, or it may persist as relatively mild anomic aphasia.
Transcortical Sensory Aphasia
- A fluent aphasia with impaired comprehension and preserved repetition. Conversational speech is fluent with relatively normal utterance length but semantic paraphasias, word-finding difficulties, and circumlocutions are common.
- Auditory comprehension is significantly impaired, although usually not to the extent seen in Wernicke’s aphasia.
- Repetition is surprisingly preserved, sometimes even for long complex utterances and unfamiliar words.
- Naming is severely impaired.
- Lesion location. TcSA most commonly results from extrasylvian lesions involving the temporo-parietal-occipital region, typically located posterior and deep to Wernicke’s area. This means that sensory information does not reach language areas.
- Prognosis TcSA due to stroke may evolve to anomic aphasia. TcSA associated with dementing disease may progress to Wernicke’s aphasia over time and ultimately to global aphasia.
Wernicke’s Aphasia
- A fluent aphasia with poor auditory comprehension and repetition. Conversational speech is fluent, easily articulated speech of relatively normal utterance length. Abundant semantic and phonemic paraphasias are present, as well as some neologisms. Utterances may be empty due to a lack of content words (nouns, verbs), an excess of grammatical words (e.g., articles, prepositions), and overuse of imprecise words (it, thing). Utterances may violate the rules of syntax and grammar, resulting in paragrammatic speech (e.g., “We need the thing in the over the house”). Verbal output may be excessive and rapid, with an apparent lack of inhibition of the flow of speech.
- Auditory comprehension is severely impaired, even at the singleword level in many cases. Difficulty with complex syntax or multi-step commands is always present. Poor self-monitoring of their own speech may make patients surprisingly unaware of their inability to produce meaningful speech, and result in failure to attempt self-correction.
- Repetition is significantly defective; patients may not be able to repeat even single words.
- Naming attempts are often paraphasic, and severe anomia is the rule.
- Lesion location. Wernicke’s aphasia is typically associated with large posterior perisylvian lesions encompassing Wernicke’s area and often extending superiorly into the inferior parietal region.
- Prognosis. If auditory comprehension improves over time, the profile becomes more consistent with conduction aphasia. Other cases may evolve in the direction of TcSA.
Diffuse astrocytoma
Anaplastic Astrocytoma
Glioblastoma with microvascular proliferation
Glioblastoma with pseudopalisading necrosis
Glioblastoma crossing the corpus callosum
Glioblastoma
Pilocytic Astrocytoma
Pilocytic Astrocytoma
Pilocytic Astrocytoma with Rosenthal fibers
Oligodendroglioma
Oligodendroglioma
Oligodendroglioma chicken wire formation
Oligodendroglioma Calcification
Ependymoma
Ependymoma withtrue rosettes
Ependymomam with perivascualr rosettes
Medulloblastoma
Medulloblastoma undifferentiated blue cell appearance
Medulloblastoma Homer Wright rosettes
Medulloblastoma subarachnoid spread
Meningioma
Meningioma
Meningioma whorls
Meningioma whorls
Meninigioma psamomma bodies
Meningioma with brain invasion (WHO Grade II)
Metastatic Carcinoma
Metastatic Cancer
Metastatic Cancer
Neurofibrillary tangles - Alzheimer’s
neuroitic plaque and neurofibrillary tangle - Alzheimer’s
neuritic plaque - Alzheminer’s
neuritic processes - fragments of axona and dendrites with tau
amyloid core - beta amyloid peptide
amyloid beta peptide
frontotemporal lobar degeneration - Pick’s
Normal vs. Parkinson’s
Lewy Body - alpha synuclein
Lewy Body in Cortex - Lewy Body Dementia
ALS corticospinal tract degeneration
ALS - motor neuron degeneration
ALS - atrophy of ventral roots
ALS - neuronal inclusion
misfolded TDP-43
spongiform change
spongiform change
prion
- There is a normal endogenous prion protein
- Infectious prion protein differs from normal by a conformational change (alpha helix to a beta pleated sheet).
- The abnormal protein can covert other prion proteins into the abnormal conformation (propagation)
- Aggregation and deposition damages brain tissue
spongiform change and amyloid plaques in new variant CJD
Bacterial Meningitis
Bacterial Meningitis
Bacterial Meningitis
polymorphonuclear lymphocytes - abscess
abscess
abscess
abscess
morphology of an abscess
viral encephalitis
viral encephalitis - microglial nodule
herpes encephalitis - prediliction for temporal lobe
herpes encephalitis
herpes encephalitis - perivascualr mononuclear cells, encephalitis and brain necrosis
herpes encephalitis - Cowdry Type A intranuclear inclusion
herpes encephalitis - long term sequelae
HIV encephalitis
HIV encephalitis - microglial nodule
HIV encephalitis - perivascular macrophages, multinucleated giant cells
HIV encephalitis - multinucleated giant cells site of HIV
fungal infection - cryptococcus
CNS Lymphoma - ring enhancing lesion
toxoplasmosis
primary CNS Lymphoma
toxoplasmosis
normal vs. Tay Sachs anterior horn motor neurons
metachromatic leukodystrophy
metachromatic leukodystrophy
metachromatic leukodystrophy - brown is stored material
Trinucleotide Repeat Expansion Disorder - Huntington’s frontal lobe atrophy
normal vs. Huntington’s
Huntington’s Disease
Huntington’s Disease - atrophy of caudate corresponds to severity of movement disorder
Huntington’s Disease - loss of neurons and gliosis, huntingtin protein
plexiform neurofibroma
plexiform neurofibroma
neurofibromatosis 1
cutaneous neurofibromas
neurofibroma vs. malignant peripheral nerve sheath tumor
schwannoma
schwannoma
schwannoma
neurofibromatosis 1
neurofibromatosis 2
multiple meningiomas
vestibular schwannomas and trigeminal schwannoma
contusions
old contusion
diffuse axonal injury
diffuse axonal injury - axonal spheroids
CTE - tau positive tangles
traumatic dissection of vertebral artery
