CN. Chapter 24 - 31 Flashcards
(130 cards)
1
Q
In what ways does the work of pediatric neuropsychologist differ form working with grownups?
A
1) The individual in question is growing. And this must be taken into perspective in assessment and rehabilitation.
2) Way more multidisciplinary. Working with both health, social services, education personnel and family members. (often these tasks involve explaining the child’s case to these groups. This may not only yield empathetic understanding of ‘oddities’ in the child’s behavior, but may also be useful in generating creative behavioral manegement solutions).
2
Q
Is plastic reorganization more easily achieved in the younger ones (under 2 years)?
A
Not necessarily. Animal studies have shown some evidence, but it is not supported with research on human infants.
3
Q
An important note about adjustment:
A
“Adjustment will be facilitated if the paediatric neuropsychologist does not describe the child’s deficits, but integrates findings into an explanation of the neurological disorder in the context of the child’s world and his or her past and future.”
4
Q
What does PTA (often a good measure for severity of injury) stand for?
A
Posttraumatic amnesia.
5
Q
There are several types of acquired brain injury. Can you give examples of different Hypoxic-ischaemic injury?
A
- Near drowning
- Anaesthetic accidents
- Prolonged status epilepticus
- Cerebrovascular accidents
6
Q
Focal impairments to the frontal lobes in the teenage years generally lead to one of two behavioral phenotypes. Can you imagine what they might be?
A
1) irratability, anxiety, false euphoria, over sexualized behavior, poor social responses (My comment: So just executive function in general?)
2) Apathy, poor initiation, pseudo-depression.
7
Q
Some epileptic syndromes are associated with specific cognitive sequelae. What are these?
A
- Moderate to severe learning difficulties are observed in majority of children with Lennox-Gastaut and West’s syndrome.
- Epilepsy is a co-symptom of severe learning diffeculities (9 - 31%), autism (11-35%) and cerebral palsy (18-35%).
- Intellectual decline in epilepsy (not associated with other underlying conditions) is usually associated with anti-epileptic drug (AED) toxicity.
8
Q
What is Hydrocephalus?
A
In danish, also called: “vand i hovedet”. It is a secondary condition arising from a number of primary disorders. It is associated with:
- Gross and fine motor problems
- Greater impairment to language content than to structure
- List-learning problems
- Problems with focused attention
- Behavioral difficulties - children with shunted hydrocephalus have higher rates of behavior disorder, than those without.
(egen note: så alt er lort eller hvad?)
9
Q
What characterizes ADHD (Attention deficit hyperactivity disorders)?
A
A triad of inattention, overactivity & impulsivity. Symptoms persist into adolescence in two out of three cases.
10
Q
In what ways can ADHD be treated?
A
- Information provision
- Use of stimulant medication
- Family and school management advice
- Self-instructional training
11
Q
What is especially important in diagnosing ADHD?
A
Diagnosis must be based on the persistence of symptoms across settings.
12
Q
What is Specific learning difficulties?
A
The term is applied by educators to a number of ‘disorders’ (reading, writing, arithmetic and spelling) having some similarities in symptomatology to conditions seen in persons with an acquired brain injury. (p. 485)
13
Q
What does NVLD stand for, and if you know, what is it?
A
Nonverbal learning disability (NVLD). Is a postulated condition of multiple aetiology including neuropathological disorders. It’s characteristics are:
- Bilateral tactile-perceptual difficulties particularly affecting the left side
- Impaired visual recognition and discrimination
- Problems with visuospatial organization
(As the name sort of suggest, auditory verbal processing is preserved. As are simple motor skills, selective and sustained attention for auditory-verbal information, reading and spelling.) (p. 486).
14
Q
Glutamate is.. and mediates..?
A
An amino-acid. It mediates excitatory transmission in many central pathways. (Excessive release of glutamate may cause over-excitation of postsynaptic neurons)
15
Q
GABA is.. and mediates..?
A
An amino-acid. It is the principal inhibitory amino-acid transmitter in supraspinal pathways. The inhibitory efferent pathways from the basal ganglia and the cerebellum are GABAergic and GABA also mediates inhibitory effects of intra-cortical neurons.
16
Q
Acetylcholine is.. and mediates..?
A
Acetylcholine is the transmitter at skeletal neuromuscular junctions, parasympathetic effector junctions, and all autonomic ganglia. It mediates both excitatory and inhibitory effects in the central nervous system.
(Bonus: Cholinergic interneurons oppose the influence of dopamine in the basal ganglia, and manipulation of cholinergic function is an important strategy in the management of Parkinsons disease.)
17
Q
Dopamine is.. and is synthesized from?
A
Dopamine is a catecholamine synthesized from the amino-acid tyrosine. Tyrosine hydroxylase converts tyrosine to L-DOPA, which is then converted to dopamine by aromatic amoni-acid decarboxylase.
18
Q
The three principal dopaminergic pathways are?
A
- The nigrostriatal pathway (substantia nigra -> corpus striatum).
- The mesolimbic/mesocortical pathway (midbrain ventral tegmental area -> limbic structures and cerebral cortex).
- The tuberoinfundibular pathway (hypothalamus -> pituitary stalk)
19
Q
Noradrenaline (norepinephrine) is.. and is synthesized from?
A
Noradrenaline is a catecholamine that is synthesized from tyrosine via the intermediate compounds L-DOPA and dopamine. The cell bodies of central noradrenergic neurons reside in nuclei in the pons and medulla.(Synaptically released noradrenaline is inactivated by re-uptake into terminals followed by re-storage or degradation by MAO).
20
Q
Can you name three noradrenergic pathways?
A
- The dorsal noradrenergic bundle (coeruleocortical pathway: locus coeruleus -> neocortex and hippocampus)
- The ventral noradrenergic bundle (tegmental nuclei -> hypothalamus)
- Descending noradrenergic pathways (tegmental nuclei -> spinal cord and medullary autonomic nuclei)
21
Q
5-Hydroxytrypyamine is also known as.. and is synthesized from? Where does the cell bodies of the neurons reside?
A
Serotonin, an indolamine synthesized from tryptophan via the intermediate precursor 5-hydroxytryptophan. Like noradrenaline, it is inactivated by by re-uptake into terminals followed by re-storage or degradation by MAO. Neurons reside in the raphe nuclei of the brainstem.
22
Q
5HTergic (Serotonin) pathways include?
A
- Ascending pathways from the dorsal and (overlapping) median raphe nuclei.
- Descending raphe-spinal pathways.
23
Q
Histamine is? and plays a role in?
A
Histamine is a monoamine. Histaminergic neurons are believed to play an important role in the maintenance of wakefulness and acid secretion in the stomach.
24
Q
What are neuropeptides?
A
They are large molecules made up of sequences of amino-acids. One of the postulated roles of neuropeptides is to modulate the action of co-localized ‘classical’ transmitters.
25
There are two main receptor 'superfamilies', distinguished by the effector mechanism to which they are coupled. Can you name and describe them?
Ionotopic (ion-channel-coupled). receptors: The transmitter or drug, binds directly to the site on an ion channel, and thereby regulates the movement of ions in or out.
Metabotropic (G-protein-coupled) receptors: The binding of the drug or transmitter, initiates a cascade of events within the postsynaptic cell (fx. alterring the permeability of the membrane to particular ionic species)
26
What are agonists compared to antagonists?
Transmitters and drugs that stimulate receptors and evoke physiological responses are referred to as agonists. Antagonists evoke no response of their own but prevent the action of agonists.
27
What is meant by the sustained-release preparations?
That you can provide a patient with a gradual release of the drug from particles during their passage through the gut, thus allowing for example a once-daily dosis.
28
Speaking about drugs.. what is meant by the term 'therapeutic range' and 'therapeutic index' ?
The therapeutic range is the concentration range in which therapeutic effects are obtained without intolerable side-effects.
Therapeutic index is the ratio of the maximum tolerated concentration to the minimum effective concentration.
29
During continued use, tolerance may occur to the drug. This may arise from?
A change in tissue sensitivity (neuroadaptation, pharmacodynamic tolerance) and/or a change in the rate of elimination of the drug.
30
Can you name the two major classes of antidepressants?
Monoamine oxidase inhibitors (MAOIs)
&
Monoamine uptake inhibitors (fx. SSRI)
31
How does 'MAOIs' work?
MAOI's block the enzyme MAO that normally destroys monoamine transmitters within presynaptic terminals. The increases the availability of the transmitter, and thus increases postsynaptic receptor stimulation. (MAOI's are seldom the first choice of antidepressant due to their potential for adverse effects. Though sometimes used in some anxiety disorders).
32
How does monoamine uptake inhibitors work?
Re-uptake into presynaptic terminals is the principal inactivation machanism for monoamine transmitters. Uptake blokade, therefore potentiates monoaminergic transmission.
33
Monoamine uptake inhibitors can be classified into which categories?
Tricyclic antidepressants (TCAs), Selective serotonin re-uptake inhibitors (SSRIs), Selectictive noradrenaline re-uptake inhibitors (NARIs) and Serotonin-noradrenerline re-uptake inhibitors (SNRIs).
(SSRIs and more recently, NARIs and SNRIs, have become more popular because they have fewer side-effects and are safer in overdose than TCAs).
34
How well does SSRIs and NARIs and SNRIs work?
In double-blind trials, only about 40% of patients attain full remission of depressive symptoms. So it should be noted, that a favorable response to treatment is not the same as complete remission of symptoms.
35
Presentation of other antidepressant drugs than the more traditional, are described at page 506.
It really is.
36
How is Lithium administered and what does it do?
Lithium is administered as a salt. It has numerous effects on monoaminergic transmission, including facilitation of tryptophan uptake, increased 5-HT and noradrenaline release and prevention of postsynaptic dopamine receptor super-sensitivity.
The relationship between these effects and clinical mood stabilization is uncertain. (p. 506).
37
Which disorders can benefit from Lithium?
Mostly, manic-depressive illness (because of its mood stabilizing qualities perhaps). It is also sometimes used to help with management of impulsive agression.
38
How does Benzodiazepines work, and to who are they primarily administered?
Benzodiazepines bind to specific sites on the GABA(A) receptor-ionophore complex. GABA's inhibitory action is mediated by the opening of CL- channels in postsynaptic membranes. Benzodiazepines potentiate (translation: "effectivises or risen") GABA's channel-opening action.
Benzodiazepines are effective in relieving the symptoms of generalized anxiety disorder (sometimes also in minor surgery, in alcohol withdrawal and as night time sedatives).
39
How does beta-adrenoceptor antagonists work and to what therapeutic use?
It blocks the effects of noradrenaline mediated by beta-adrenoceptors, including tachycardia (high heart rate) and tremor. Thus maybe breaking the vicious cycle: nervous -> heart beating -> more nervous -> even more heart beating -> even more nervous.
So, they are helpful in managing situational anxiety, fx. public speeches.
(I use heartbeat here because the drug does not help with sweating which is mediated by other stuff, but in my humble opinion is maybe worse, since it is visible to other people, and thus makes the person more nervous).
40
What is the main mode of action with Anti-epileptic drugs?
To suppress neuronal excitability, either by enhancing inhibitory neurotransmission or by suppressing excitatory transmission. Even though mono-therapy is preferred, it is often necessary with combination therapy when medicating for epilepsi.
(For a list of the about 12 different drugs, see p. 510).
41
Drug treatment of Parkinson disease aims to? And what drug is used?
1. Restore dopaminergic function, or
2. Restore the balance og dopaminergic/cholinergic function in the basal ganglia.
L-DOPA is the most effective treatment for PD. (Me: ... And maybe deep brain stimulation?)
42
Can you descripe the side-effects to L-DOPA therapy?
Dyskinesias constitute a more serious problem for about 50% of patients during prolonged treatment. Behavioral side-effects include mood disturbances (depression or mania), insomnia, delirium and sometimes hypersexuality from limbic/hypothalamic dopaminergic hyperfunction.
43
What are the major neuropathological features of Alzheimer's disease? And how can it be treated medically?
1. Extracellualar plaques containing B-amyloid protein.
2. Intraneuronal neurofibrillary tangles
3. Neuronal loss
By using reversible competitive inhibitors of acetylcholinesterase ( Fx. Donepezil or Gallantamine)
44
What is meant by vascular disorders?
The term vascular disorders covers a range of conditions, including cerebrovascular disease (eg. stroke, vascular dementia) and specific blood vessel problems, such as aneurism and associated sub-arachnoid hemorrhage (SAH).
45
.. If one was to look at pre-stroke conditions, one might look into?
Hypertension (elevated blood pressure) or hypotension (Low blood pressure).
(A significant reduction in the probability of subsequent stroke or vascular dementia is achieved when hypertension has been successfully treated and blood pressure is reduced and controlled)
46
Describe what 'Cerebral Small Vessel Disease (SVD)' is?
It is a narrowing of penetrating arterioles which supply the deep white matter of the brain, leading to gradual ischemic damage. Diagnosis is confirmed by lesions in the white matter identified using MRI. (see p. 523 for more)
47
Try and describe what 'Transient Ischaemic Attacks (TIAs)' are.
TIAs are temporary (lasting less than 24 hours) neurological deficits arising from an ischemic episode, leading to a possible wide range of cognitive deficits. Early signs often include slowed speed of information processing and new learning.
48
What is a stroke? What is the neurological presentation?
A stroke is a disruption of vascular supply to the brain, of rapid onset with neurological symptoms persisting longer than 24 hours, caused by haemorrhage from an artery or from its occlusion through progressive narrowing of the vessel.
The neurological presentation can be extreme variable. When the stroke involves the language centers, aphasic problems often result. The 'classic' cognitive disturbance in right hemisphere stroke is perceptual deficit (i.e. left side of visual space does not exist).
49
What are the risk factors for Stroke?
Age, cardiac disease, smoking, prior TIAs (recall? Transient Ischaemic attacks), hypertension and diabetes.
50
What characterizes Vascular Dementia (VaD)? And how do diagnose it?
It is a generalised, significant reduction in cognitive function that is cerebrovascular in origin. (The term has replaced the older 'multi-infarct' dementia).
Diagnosis can be made, comparing the IQ figures obtained with the predicted pre-modbid IQ's for the patient generated using NART (National adult reading test) and comparing these to WAIS-III scores.
51
What characterizes Subarachnoid Haemorrhage (SAH)? How do they most often arise?
The term 'haemorrhagic stroke' is usually applied to bleedings into the substance of the brain, whereas SAH refers to a blood vessel rupturing and bleeding into the subarachnoid space. About 80% of SAHs arise from the rupture of an aneurysm. (85% aneurysm are found on the anterior cerebral artery distribution. The next common are middle cerebral artery aneurysms, the posterier cerebral circulation having the lowest occurrence. This often cause some personality changes (i.e. the aneterior cerebral artery distribution)).
Lastly, SAH is more common in the younger population (50 - 55 years old) compared to stroke.
52
To read about different vascular surgeries, see p. 535.
535, yes.
53
How is the severity of head injury measured?
The severity of head injury is usually measured by: 1) depth of unconsciousness (usually measured immediately after resuscitation, using the Glasgow Coma Scale (GCS)), 2) length of post traumatic amnesia (PTA) and 3) length of unconsciousness and/or presence of neurological signs (e.g. for example from neuro-imageing techniques).
54
What are post concussion symptoms (PCS) and how many percent experience these (typically people who have had mild or moderate head injury) ?
Approximately 50% will experience post concussion symptoms: headaches, dizziness, fatigue, irritability, sleep disturbance, slowed thinking, depression and blurred vision.
55
What are the most common neuropsychological impairments following mild and moderate head injuries?
Speed of information processing and divided attention (measured by Paced Auditory Serial Addition Task (PASAT) and Stroop test).
56
What are the two main types of severe head injury?
Open head injury and closed head injury. (As the book nicely states, the latter is by far the most common form of head injury in peace time.. Yes).
57
Diffuse white matter damage is due to.. ?
The widespread rupturing of axons caused mainly by the brain impacting against itself as it moves within the skull.
58
Typically the majority of recovery occurs in the first.. ?
.. Two years after injury, such that significant difficulties evident at two years rarely resolve completely thereafter.
59
Short and long term emotional sequelae of severe head injury can include?
1) Post traumatic stress reactions (PTS) relating to the events which caused or followed the head injury. (of course unconsciousness and post traumatic amnesia can be "beneficial" in this regard)
2) Anger, frustration, depression and anxiety are common emotional responses to the disabilities that patients confront.
3) Major distress in adjusting to and coming to terms with the losses and changes arising as a result of a head injury e.g. break up a relationship or inability to return to work.
60
Can you name four important aspects of the process of rehabilitation?
1) Engaging the patient (and their family) in the rehabilitation process, 2) facilitating the setting of realistic rehabilitation targets and goals, 3) facilitating the achievement of targets and goals via co-ordinated interventions, and 4) evaluating, reviewing and modifying targets and goals over time.
(all four points are usually somewhat pragmatic in nature)
(Also, the use of groups for cognitive rehabilitation, education and emotional support should not be underestimated)
61
There are several general cognitive strategies, that are useful in helping brain injured patients. How many "ways" of helping can you think of?
- Developing habits, routines and over-learnt procedures (in order to minimise cognitive load, and provide structure)
- Developing a tidy living and working environment
- Taking many small breaks when impairments become apparent, rather than pushing on..
- Gradual return to premorbid activities and minimization of non-essential activity.
- Identifying specific times, or specific types of activity where fatigue, irritability, anxiety or frustration occur and helping with changes in these areas.
- Training the patient to allow ekstra time for tasks where speed of information or such is reduced.
- Using a diary.
62
Can you mention some strategies for managing memory?
- Using a dictaphone to record meetings, lectures etc.
- Using a watch with a alarm or a paging device as a cue to look in diary
- Using notes, calendars and lists as external cues and reminders.
- Systemising storage of household items with explicit labelling (especially important for safety of toxic substances for patients with visual agnosia).
- Developing internal memory (repetition, association, chunking, visualization of verbal material (or the opposite). This one is often hard for patients with memory and executive impairments.
- Using principles from behavioral psychology.
63
Can you mention some strategies for managing executive difficulties?
(Perhaps both for people suffering from problems with planning as well as people suffering from executive behavioral problems).
- Providing problem solving skill training to help develop an explicit and systematic approach to solving difficulties.
- Using an alarmed stopwatch to help patients monitor the amount of time taken on given tasks to help planning and temporal judgement impairments.
- Breaking tasks down into several steps, reducing disabilities from planning deficits.
- Introduce routines and cycles to reduce unnecessary decision-making.
and the more behavioral:
- Identifying a small range of people to provide behavioral feedback to help modify inappropriate behavior. (important to stress that the feedback should be immediate, concrete and supportive. Aimed at very specific behaviors only).
- Using video feedback to improve insight.
64
Alzheimers disease (AD), frontotemporal dementia (FTD) and its associated syndromes of semantic dementia and progressive aphasia result from degeneration principally of the ?
Neocortex.
65
Alzheimers Disease (AD) is the most common form of dementia. What are the earliest pathological changes?
The earliest pathological changes in AD are in medial temporal structures, and structural MR imaging shows atrophy of the hippocampi (hence the early memory problems, over the physical problems that only present much later). Over the course of the disease structural and functional changes become more widespread.
66
The most common language symptom in Alzheimers Disease is a problem in ...?
Word-finding, which is often most pronounced for Proper nouns, such as recall of people's names. This is often ascribed to impaired semantic memory.
(Quick tip: If you are tired, but still want to learn -> Watch Still Alice, were you follow 1) the development of early onset Alzheimers, but also how her Doctor does an assessment. Its not bad. (Congrats on the Academy award, Julianne Moore)).
67
A striking feature of AD patients presenting to a diagnostic clinic is often their .. ?
... Preserved social facade. Patients emotional warmth, social manner and facility in use of social platitudes in conversation frequently mask the magnitude of the underlying cognitive disorder. (Note that gross character change is not a hallmark of early and middle-stage Alzheimers disease and patients are regarded as the same person as before, albeit with acquired difficulties.
68
A diagnosis of probable Alzheimers disease is determined by.. ?
.. Historical reports of a gradual and progressive change in memory function together with demonstrations of significantly impaired episodic memory (encompassing recognition and cued recall as well as free recall) on neuropsychological testing. (Should be supported by medial temporal lobe atrophy).
69
Frontotemporal lobar degeneration (FTLD) is an umbrella term for ?
A collection of pathologically-linked clinical syndromes that result from circumscribed atrophy of the anterior hemispheres. (Nogen gange bruger de fandme mange ord på at sige: Frontallapperne har det skidt. Neuronerne dør).
70
Frontotemporal lobar degeneration (FTLD) includes which disorders?
Frontotemporal dementia (FTD), a disorder of behavior and executive function associated with bilateral atrophy of the frontal and anterior temporal lobes, and thus altered behavior.
Progressive non-fluent aphasia (PNFA), a disorder of expressive language associated with left perisylvian atrophy.
Semantic Dementia (SD), a disorder of conceptual understanding associated with bilateral atrophy of the inferior and middle temporal cortices.
(Unlike AD which increases in prevalence, all of these present most commonly in the 50s and 60s and can present even earlier)
71
Frontotemporal dementia is often misdiagnosed as AD. What separates the two?
Patientens with Semantic Dementia do not show the topographical memory loss that is so common in AD, evidenced by the fact patients who wander often follow the same route without becoming lost. Also, Frontotemporal Dementia patients' is relatively well at retaining information over a standard 20 - 30 minutes delay, suggesting that the problem is one of registration rather than retention per se.
72
What is often the most prominent (and first) characteristic of Sementic Dementia (SD)?
The initial presentation is most commonly of problems in word meaning. They also have difficulty understanding words and may ask what individual words mean (a feature that is not notable in AD or any other neurodegenerative disorder. Non-semantic aspects of language are well preserved - patients speak fluently).
73
Semantic Dementia (SD) is also commonly misdiagnosed as AD. What could be a difference though?
Patients who suffer from Sementic Dementia has well preserved day to day event memory. They find their way around without becoming lost, remember daily activities and keep track of time. They retain a degree of functional independence that would be unthinkable in a patient with classical amnesia.
74
In 'pure' semantic dementia cases the atrophy is relatively confined to the .. ?
Temporal Lobes.
75
Progressive non-fluent aphasia (PNFA) is distinct from Semantic Dementia in what ways? And brain wise, what might be different from Alzheimers Disease?
Unlike patients with semantic dementia, PNFA patients may exhibit a tip-of-the-tongue effect and show immense frustration, reflecting their awareness of the existence of the word that they cannot bring to mind. And so, PNFA may be associated with effortful speech, agrammatism and phonological disturbances, and bear resemblance to Broca's aphasia.
Compared to Alzheimers disease, there is often a striking hemispheric asymmetri of atrophy and specificity in the nature of linguistic breakdown.
76
The form of dementia most akin to Alzheimers disease is DLB, which stands for?
Dementia with Lewy bodies.
77
Dementia with Lewy bodies is characterized by?
The core defining features are cognitive impairment, together with mental fluctuations, visual hallucinations (often of people or animals) and parkinsonism. It is a disorder principally (altthough not exclusively) of the elderly.
- A salient and striking characteristic of DLB patients is fluctuations in their mental state (typically mentioned by relatives as the patient having 'good days and bad days' )
(Recognition of the disorder is crucial for patients' optimal management. DLB patients frequently sensitivity to traditional neuroleptic agents, which need to be avoided).
78
Is it generally accepted that Dementia with Lewy bodies and Parkinson's disease dementia are the same disease?
Yes, it is.
79
What characterizes Huntington's disease?
It is a genetic disorder caused by a trinucleotide (CAG) repeat mutation in the huntingtin gene on chromosome 4.
The cardinal features are involuntary (choreic and dystonic) movements, cognitive impairment and mood/behavioral changes (poor self care, loss of drive, self-centredness, poor temper control).
Patients show impoverished generation in verbal and design fluency tasks, poor sequencing, divided attention and mental set shifting. They typically make a high percentage of perseverative responses.
80
What characterizes Progressive supranuclear palsy (PSP) ?
It is a basal ganglia disorder, the hallmark of which is a progressive reduction in voluntary eye movements, culminating in total ophthalmoplegia (total inability to voluntary move the eyes). Whereas patients with Parkinsons disease have a flexed posture those with PSP, tend to hold their head in extension, and are prone to falling backwards.
81
What is Corticobasal degeneration?
Corticobasal degeneration (CBD), like PSP, falls within the rubric of 'parkinson-plus' disorders. The striking abnormality in CBD is limb apraxia of ideomotor type: the patient has the idea of the required action but cannot implement it effectively. Buccofacial apraxia develops with progression of disease, causing difficulty with speech.
82
What is there to say about dematia as a global, undifferentiated impairment?
That it needs to be abandoned. It is more appropriate to regard the 'dementias' as representing constellations of deficit, which form distinct performance profiles, corresponding to the topographical distribution of degenerative change within the brain. (p. 581)
83
Multiple Sclerosis (MS) is characterized by?
The demyelinating of the central nervous system. The defining pathological feature being the demyelinated plaque - a lesion characterized by the loss of myelin, the relative preservation of axons, and the presence of astrocytic scars. Majority of plaques (about 75%) are observed in white matter.
The cause it not yet fully understood, though it is thought to be related to an autoimune process or maybe a slow acting virus.
84
How is Multiple Sclerosis diagnosed?
MRI data are considered preferable to other paraclinical tests; however, addtional test can be used when clear-cut MRI findings are not present. Also, seperation in time should be evident, as reflected by the onset of new MRI lesions or increased level of disability over the course of at least one year.
85
What are the most commonly affected domains in Multiple Sclerosis (MS) ?
Problems with encoding and/or retrieving both verbal and visual information. Working memory is also commonly impaired.
Perhaps most prominent: MS patients show greatest difficulty on tasks requiring rapid and complex information processing, such as attentional shifting or rapid visual scanning.
86
What sorts of verbal/linguistic deficits could one suspekt in multiple sclerosis?
Aphaias are rare in MS. Deficits in verbal fluency are common (e.g. processing speed).
87
What emotional disorders are related to Multiple Sclerosis?
Very common in MS with lifetime prevalence around 50% is major depression. Accurate screening and diagnosis is extremely important, given the increased risk of suicide in MS; the latter may be the cause of death in up to 15% of MS clinic patients. (This is historically undertreated in MS)
The other one is Anxiety. Possibly even more common than depression. There is often a decline in distress associated with more definitive diagnostic statements by treatment professionals. Best predictors of anxiety include being a female, a co-morbid diagnosis of depression, and limited social support.
88
Can you name two other demyelinating diseases (other than MS) and what characterizes them?
Marchiafava-Bignami disease:
Most commenly associated with chronic alcoholism. Characterized by focal demyelination in the medial zone of corpus callosum.
Central Pontine Myelinolysis (CPM)
Involves the destruction of the myelin sheaths in the central portion of the basis pontis, and is most often found in young and middle-aged adults.
89
Ontaining an accurate history of metabolic imbalance may be a ... ?
Complex undertaking. Determining the date of onset is frequently ambiguous. Because the endocrine system and metabolism in general is subject to degeneration due to age, subclinical syndromes may exist for years before being identified, which may be associated with poorer response to therapeutic intervention.
90
Which cognitive domains are most often effected in neuroendokrine dysregulation?
The cognitive domains of attention, memory, and psychomotor speed are most commonly affected, although the effects are subtle. It is thus important to use very sensitive measures of cognitive efficiency and working memory.
91
The endocrine system produces its effects on bodily and mental functions by means of various hormones secreted by specific endocrine glands. Can you name to general categories of hormonal actions?
1. Those that affect the developing brain are known as Organizational Effects. (for instance congenital hypothyroidism can produce permanent functional and structural changes by altering the pattern of thyroid receptors present in brain substrate), and
2. Activational effects. These on the other hand, are the result of a chemical being present at any given moment.
92
Insert the X's.
The hypothalamus is the principal autonomic center of the brain, acting on the X and X nervous system to maintain X and to prepare the body for emergencies.
Acting on the 1. sympathetic and 2. parasympathetic..
| Maintain 3. Homeostasis.
93
The hypothalamus also mediates drives for ?
Hunger and thirst, sexual activity, and aggression.
94
How many trophic hormones can you name?
Growth hormone (GH), Adrenocorticotropin Hormone (ACTH), Thyroid Stimulating Hormone (TSH), the gonadotropins known as Follicle Stimulating Hormone (FSH) and Luteinizing Hormone (LH) and Prolactin.
95
From where are the trophic hormones secreted?
The anterior pituitary gland.
96
Commonly, hypothalamic dysfunction may result from either .. ?
Hypersecretion of a hormone as a result of a tumor (compression on the pituitary gland) and/or hyposecretion of other hormones secondary to the tumor's mass effect on the pituitary gland and hypothalamus.
97
What might patient complain about if the pituitary gland is not functioning normally?
Low libido, impotence, lack of menstruation, and visual changes.
98
What is happening to patient who suffer from Androgen (a gonadal hormone) Insensitivity (AI) ?
Patients with Androgen Insensitivity (AI) are genetic males who produce androgens but manifest partial to total insensitivity of androgen receptors. Depending on the degree of insensitivity they are born either with external female genitalia (and no female reproductive organs) called total AI and are raised as girls, or ambiguous genitalia-partial AI, and are raised as either boys or girls.
99
Behavioally, what are androgens associated with?
Sexual drive end increased aggression in both genders. And also, optimal levels of androgens in both genders are necessary, for normal visuospatial functioning.
100
Where are the adrenal glands located?
At superior poles of the kidneys.
101
Each adrenal gland is divided into two distinct portions. What are they and what to they secrete?
The adrenal medulla, which secretes the hormones epinephrine and norepinephrine and is functionally related to the sympathetic nervous system, and,
The adrenal cortex, which secretes the hormones known as corticosteroids, of which cortisol is of principle interrest in regard to cognitive functions.
(The adrenal glands, also produce small amounts of certain androgens).
102
Cortisol acts to.. ?
Increase blood glucose concentrations, which in turn mobilizes available energy stores, and helps the body to maintain homeostasis through its regulatory effects on protein, carbohydrate, and lipid metabolism.
103
What happens, chemically, in the hypothalamic-pituitary-adrenal axis?
Corticotropin releasing hormone (CRH) is produced by hypothalamic neurons both according to a circadian pattern and in response to physiologic stress. CRH regulates the release of adrenocorticotropin hormone (ACTH) from the pituitary. ACTH stimulates the adrenal glands, which in turn produce cortisol. Cortisol completes the feedback loop by its effect on the hypothalamus and other structures.
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Cushing's syndrome is characterized by?
The clinical manifestation of increased concentrations of cortisol due to any number of disorders. Typical features associated with hypercortisolism include truncal (fedme omkring midten af kroppen) obesity, hirsutism and baldness, osteoporosis (increased bone weekness), hypertension, and generalized muscular weakness.
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What are the psychiatric symptoms in more than half of all cases of hypercortisolism?
Depression, anxiety, irritability, affective lability, decreased libido and psychosis. (However! Note: Patients with major depression may have elevated cortisol levels with no evidence of endocrinopathy.
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As oppose to hypercortisolism (elevated levels, Cushing's syndrome) hypocortisolism is a case of adrenocortical insufficiency. What disease is this linked to? What are clinical features often associated with hypocortisolism?
Addison's disease in 75% of all cases.
Clinical features of hypocortisolism include pigmentation of the skin and mucous membranes, nausea, vomitting, weightloss, muscle weakness and fatigue. Psychiatric symptoms include depression, confusion, anhedonia, and schizophrenic behaviors.
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What role does dehydroepiandrosterone (DHEA) have?
In healthy subjects, DHEA appears to enhance general well-being, manifested as increased energy, deeper sleep, improved mood, greater relaxation and better stress handling capacity.
Numerous investigations have recently found that this hormone decrease with normal aging and a correlation with age-related immune system decline.
(Might explain part of the curious part of the curious case of Benjamin Button)
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What role does Melatonin play?
In addition to its possible role in the seasonal regulation of human sexual behavior and its role in regulating body temperature, the pineal gland has received scrutiny because of its synthesis of the hormone melatonin. Melatonin is secreted cyclically, with low levels associated daylight, and increases peaking toward midnight and than gradually returning to baseline by morning. (It is fx. sold for jetlag treatment).
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Growth Hormone (GH) has received a lot of interrest, because it potentially can ..
.. Stave off the effects of aging on the body and on cognition. For individuals with GH deficiency, GH replacement significantly improves cognition, especially in attention and memory. (Children with GH deficiency have notable cognitive deficits as well, which improve with replacement therapy).
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Epilepsy refers to.. ?
.. a group of conditions that have recurrent seizures as a symptom and consequently THE EPILEPSIES, is the term recommended. An epileptic seizure is a transient, abnormal electrical discharge from a set of neurons in the brain; the region involved will shape the behavioral manifestation of the attack. Seizures may involve motor, sensory, psychic or autonomic disturbances, alone or in combination.
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Most epileptic seizures can be classified as either?
Partial or generalized.
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What characterizes a partial seizure?
Partial seizures begin with epileptic activity in a localised brain region. Any area of the cortex can be the epileptogenic zone but the temporal lobes are the most susceptible (60 - 70%).
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What characterizes a generalised seizure?
In generalised seizures the epileptogenic activity involves the whole cortex from the outset. Consciousness is lost and accordingly there is no awareness of the event. Absence seizure, involve only a brief arrest of consciousness in the order of a few seconds (often in children), whereas Atypical absences refers to attacks of longer duration that may involve some body jerking.
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The temporal lobes, are the most common place of partial epileptic seizures. What are the next most common?
The frontal lobes are the next most common site of partial epilepsy. Complex partial seizures of frontal origin are characteristically short, lasting less than a minute and in contrast to temporal lobe seizures recovery is generally quick.
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Seizures arising in the parietal regions may present with.. ?
Tingling, numbness and burning sensations; illusions of size (macropsia, micropsia), shape and sound. May present with visual perceptual phenomenon (flashing lights, colored shapes) often restricted to a hemi-field.
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What are the generalized Tonic Clonic seizures? And what is a tonic seizure?
Generalised tonic clonic seizures can last several minutes and are biphasic. The attack begins with muscle contradiction and stiffness. The person will fall if standing and muscle tightening around the chest may result in a vocalisation as air is expelled from the lungs (tonic phase). Last a few minutes!
Tonic seizures are brief. Suddenly all muscles stiffen, the person becomes rigid and will fall if standing. Injury is common. Last only seconds.
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Note that 70 - 80% of people with epilepsy will become seizure free and about 50% will be able to discontinue their anti-epileptic medication.
.. Yes.
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The causes of epilepsy are varied and several. Can you name some possible causes?
- Epilepsy may be genetically determined, though the mode of inheritance is unclear and is likely to be multigenetic.
- Congenital malformations of the brain may cause epilepsy. Such as cortical dysgenesis (brain abnormalities that have developed as the result of faulty neuronal migration during embryo-genesis)
- The incidence of epilepsy is high following cerebral infections and often seizures are difficult to control.
- Epilepsy may be caused by head injuries. The risk is high when the post-traumatic amnesia exceeds 24 hours.
- Metabolic disorders.
(see more on page 641).
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An EEG trace represents the summation of synchronized.. ?
.. excitatory or inhibitory postsynaptic potentials. (An EEG should never be used in isolation to diagnose epilepsi. About 10% of patients with epilepsy will have normal EEGs).
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Obtaining MRIs scans are actually recommended as best practise in the majority of patients with epilepsy. But what are the most common abnormalities seen?
The most common abnormalities identified in epilepsy are hippocampal sclerosis, malformations of cortical development, vascular malfunctions, tumours and acquired cortical damage.
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Which medication forms the mainstay of epileptic treatment?
Anti-epileptic drugs (AEDs). About 70% of patients developing epilepsy may expect to become seizure free with AED therapy. The majority will achieve control with a single drug and 10 - 15 % with two drugs.
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There are two major types of surgery for epilepsy: Resective and Functional. What are they?
Resective surgery aims to remove the epileptogenic brain region in order to stop seizures.
The aim of functional surgery is palliative. It is only considered when resective surgery has been ruled out. The surgical plan is to disconnect the epileptogenic region or to prevent the spread of epileptic discharges to other regions (one example: Corpus callosotomy).
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Does epilepsi cause any memory problems?
Deficits of new learning have been the subject of most investigations and are the most common cognitive impairment in temporal lobe epilepsy. Memory impairment is most extensive in cases with known bilateral temporal lobe damage, with the amnesic syndrome being being the most severe of cases.
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Treating epilepsy with surgery such as Hemispherectomy (half the bran removed) or corpus callosotomy (on people with severe life threatening seizures including drop attacks) , is almost always only done on..
Children.
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What are pre- and postictal mood disturbances in epilepsy?
Pre-ictal mood disturbance may last hours and sometimes days but will resolve following a seizure. Post-ictal mood disturbance often follows seizure clusters and can persist for days. Depression is most commonly encountered and may be severe and involve suicidal ideation.
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What factors contribute to adverse mood change in epilepsy?
- Unpredictability of ongoing seizures.
- Intrusive and embarassing aspects of seizures, such as bizarre behaviors and incontinence.
- Risk of injury (particularly for those with drop attacks)
- Fear of death
- Teasing and bullying at school.
- Failure of surgical treatment which may occur in approximately in a third of cases.
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What is Non-epileptic attack disorder (NEAD) ?
NEAD, Non-epileptic attack disorder, is a term used to describe transient episodes of altered movement, sensation or experience resembling epilepsy but caused by a psychological process, and not associated with any abnormal activity in the brain.
Features suggestive of NEAD include gradual onset, long duration (over 5 minutes), asynchronous limb flailing, directed actions especially aggression, reactivity to external stimulation and identifiable environmental/psychological triggers.
NEAD is recognised as a heterogeneous grouping that may be a manifestation of an anxiety disorder or a dissociative disorder.
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Differentiating between epileptic and non-epileptic seizures is .. ?
Difficult. No characteristics are exclusive to epilepsy and no features occur only in non-epileptic attacks. Incontinence and injury do not reliably distinguish epileptic from non-epileptic seizures.
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Neuropsychological assessment of epilepsi, generally involve?
1. Have a description of the seizure(s). In partial epilepsy this may provide pointers to area of cerebral disturbance.
2. Measurement of mood is recommended; high rates of anxiety and depression will influence the interpretation of the test results.
3. Test selection (influenced by the referral question & also shaped by the results of EEG and/or MRI).
4. Documentation of drugs taken, including dosages.
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What would one look at, in an evaluation of a candidate for resective surgery?
1. Assess for cognitive disturbance concordant with known lesion and electrophysiological disturbance. With temporal cases, attention will be on memory skills; with frontal cases, attention to executive functioning.
2. Assess for cognitive competency in other areas; a good surgical candidate will be the individual who functions well on other tests.
3. Pre-operative counselling: Higher risks of neuropsychological deficits (as fx. older age of operation) needs to be discussed with patients.
