RESUMOS ORGANIZADOS PDF

RESUMOS ORGANIZADOS Clinical practice BEHAVIOURAL SYMPTOMS → latent variables that objectively can be assessed through test → can be subjectively observed by the patient and significant other SCIENTIST PRACTITIONER → combine clinical knowledge and skil...

RESUMOS ORGANIZADOS Clinical practice BEHAVIOURAL SYMPTOMS → latent variables that objectively can be assessed through test → can be subjectively observed by the patient and significant other SCIENTIST PRACTITIONER → combine clinical knowledge and skills with scientific attitude equal to medicine practice → model of evidence based research and clinical expertise and individual needs ASSESSMENT → after making the hypothesis → data collection (medical file, clinical interview, interview with informant, observations,test results, and questionnaires and result integration, and follows conclusion) REFERRAL VS. DIAGNOSTIC QUESTIONS r: from someone that refers the patient (GO, doctor, parent..) DQ: after reading medical file, history, clinical interview, tudo do assessment REPORTED COMPLAINTS VS. OBJECTIFIED DISORDERS RC: helps to formulate hypothesis and interpretation of impact OD: we can’t directly connect to the disorders HEALTH CONDITIONS body structure and functioning activity participation lead to : environmental factors and personal factors categorical hospital → focused in specific patient group Care → interview with scientific research More for neuropsychologist → focused in adults and elders, in collaboration with lot other fields besides diagnostics they focus on a short period treatment patient group → questions made are focused on diagnostic neuropsi assessment→ descrive the consequences of cognitive impairment and impact treatment → coping with cognitive disorders, bettering mood and return to the normal day activities (is needed a longer treatment plan they are taken to a specialized centre) Mental health care : neuropsychiatric model relationships between brain, cognition, emotion and behaviour RESUMOS ORGANIZADOS 1  diagnostic work up → neuropsychological testing and personality assessment/specific research into tje nature of psychiatric disorders- with structured interview DIAGNOSTIC → investigate the cause of cognitive symptom reported (brain damage or premorbid or current psychiatric disorders ?) specific treatment plan → last longer than …, pode tb ser admission and intensive observation Rehab care → multidisciplinary approach Can be inpatient or outpatient with the key theme the participation Aim : enable the person to do societal activities independently doesn’t aim for differential diagnosis - cause we already know that- but insight in progression and treatment see how the information is being perceived… coping→ do they need active coping or distractions? - know the different coping styles of each patient and apply them psico-education → cognitive rehab techniques or therapeutic intervention - cognitive behavioural therapy the loved ones are explicitly involved in the therapeutic progress Long term care → person who can’t live alone Mediative treatment and counselings, not seeking diagnostics, although if there is no clear diagnosis neuropsi examinations can be useful Forensic care → treatment can be voluntary or imposed Legal framework→ explanatory diagnosis or support treatment indication → appropriate care Risk assessment → extent of cognitive impairment or re-offending stats Not everyone is motivates, has real disorders and we need to try to see the truth of statements NEUROPSI EXAMINATION Clinical neuropsychologist They know the relation between brain abnormalities, cognition, emotional dysregulation and problematic behaviour, combines both clinical and neuropsychological - assessment, diagnose, treatment of cognitive dysfunctions (They aren’t brain researchers ) Complete hypothesis testing and diagnostic cycle is more or less the same as empirical cycle in scientific research referral questions → before examination patient file investigation extensive clinical interview: discuss complaints, limitations… dif symptoms and with significant others RESUMOS ORGANIZADOS 2  HYPOTHESIS (from the two above)→ specific tests and questionnaires (standart set of tests is used to measure key cognitive functions associated with neuropsi assessment → good for scientific research ) - → testing theories about cognitive functioning → testing hypothesis : multi informed, multi method, multi conceptual test selection and administration behavioural observations psychometric examination / interpretation observations ; conclusions; recommendations reporting if diagnostic is not finished and there is a lot of considerations it is better to tailor the set of tests to the individual → during neuropsi exam, psychosocial and personality factors take into account and can’t be considered seperatly from cognition → personality → affects the perception and presentation of complaints ; → personality plus somatic and psychiatric comorbidity can affect the development or course of symptoms It is really relevant because determine cause of brain damage less mortality rates ageing more interest in life qualitty RESUMOS ORGANIZADOS 3  Neuropsychologist → a scientist practioner Understand the relation between brain structures and cognitive functions ; assessment of brain dysfunction through testing Health-care psychologist RESUMOS ORGANIZADOS 4  General, mental health and psychological well being - not necessarily focused on brain-behaviour relation RELIABILITY VALIDITY test-retest : same response is administered to the same person at a different time and important for different examinators to apply in similar conditions is inter-rater reliability) validade : o teste mede que queremos medir facila → at first sight (achamos que mede) content → é representativo do que queremos medir ? (reconhecer fotografias é igual a reconhecer caras?) concept/construct → o resultado é indicativo da função cognitiva que queremos medir (face recognition mede memória facila) criterion validity → extent that it can predict a patient performance on an external criterion predictive “ → predict a behaviour (specific form of predictive “) ecological ”→ extent q prediz como uma pessoa funciona no seu ambiente normal concurrent “→ comparação com neuropsi teste e outro instrumento q mede o mesmo contruto Cut off point → balance between sensitivity and specificity Determine the number of patients with poor effort and at the same time prevent false positives to be wrongly accused of poor effort → if bellow it is insufficient mental effort Never rule out cognitive impairment, but performance invalidity there can’t be made any statement → low effort implies that we know the correct answers Confounding factors → variables that can interfere with or distort the result of the assessment → fatigue, stress or pre- existing conditions that affect cognitive performance influences performance not related with what the test is measuring can be less motivation ou distorted complaints → under-performance or over reporting Under performance When we do worse than what is expected/actually capable of doing, + symptoms are exaggerated → distorted can be due to lack of knowledge of the utility of the test/ no interest/ can’t see the correlation with the disorder lack os awareness of illness → anosognosia can it be beneficial?- aggravating or pretending a disorder RESUMOS ORGANIZADOS 5  We can’t accurately predict poor performance → dedicated performance testes, that appear difficult, like memory, but aren’t. Those rely on processes that are intact in brain damage patients- recognition of previously presented information. These are based on the unlikely performance in regular test Important words NIP - NL institute of psychologist AST - general standard of testing (responsability, integridity…) give the right to view the repport before it is issued… to correct some answers… to block the repport from being issued - guide lines and info about test choices and correct use of the instrument COTAN - dutch comity of testing and assessing NVM - dutch association of neuropsychology INS - international neuropsychology society ICF - international classification of functioning , disability and health Description of consequences of brain disease at 3 different levels : impairment- limitations- identify moderating factors, relevant to understand subjective complaints and problems, identifying the target for treatment or optimization environmental factos, body functions and structures activity and limitations participation restriction personal consequences Alcohol related cognitive disorders Alcohol (C2H5OH) Alcohol in the brain direct neurotoxic effect → damage axons and neurons indirect ''' → more calcium concentration damage the neurons after sudden withdrawal/ abstinence indirect effect of B1 deficiency indirect effect of comorbidities (hepatitis, multiple drug use, traumatic brain injury, cerebrovascular risk factors RESUMOS ORGANIZADOS 6  physically → greater risk of cardiovascular disease, different cancers and disease of gastrointestinal system (depending on the quantities and period of time) → most injuries and death are due to accidents - indirect consequences Too much alcohol → chronic inflammatory reactions 1/8 glasses per week → health damage and even alcohol addictions, can lead to clinically significant impairments or distress there are 11 main symptons - mild raging from 2/3 and moderate 3/4 and sever 6+ 1 Alcohol is often taken in larger amounts or over a longer period than was intended. 2 There is a persistent desire or unsuccess, eforts to cut down or control alcohol use: effects. 3 A great deal of time is spent in activities necessary to obtain alcohol, use it or recover from ts 4 Craving or a strong desire to use alcohol. Recurrent alcohol use resulting in a failure to fulfil major role obligations at work, school 5 or home. Continued alcohol use despite having persistent or recurrent social or interpersonal 6 problems due to the effects of alcohol. Important social, occupational or recreational activities are given up or reduced 7 because of alcohol use. 8 Recurrent alcohol use in situations in which it is physically hazardous. Alcohol use is continued despite knowledge of having a persistent or recurrent physical 9 or psychological problem that is likely to have been caused or exacerbated by alcohol. Tolerance, as defined by either of the following A need for markedly increased amounts of alcohol to achieve intoxication or desired 10 effect; A markedly diminished effect with continued use of the same amount of alcohol. Withdrawal, as manifested by either of the following: 11 The characteristic alcohol withdrawal syndrome; Alcohol is used to relieve or avoid withdrawal symptoms. alcohol addiction or alcoholism is not stated on the DSM problematic A. use: drinking pattern resulting in physical complaints and/or psychological or social problems binge drinking: consuming large amounts in a short time (males more then 5 cups and women 4) Alcohol intoxication: disorientation, sexual/aggressive desinhibition, inability to make judgements, loss of consciousnes, shock, breathing problms … Alcohol withdrawal syndrome : sudden withdrawal after long-term alcohol abuse A use disorder (AUD)→ DSM criteria RESUMOS ORGANIZADOS 7  Alcohol related cognitive disorder Long term cognitive impairments associated with problematic alcohol use (alcohol related neurocognitive disorder) Alcohol related brain damage → implies explanation for cognitive impairment which is not always true → it can be used for people with cognitive impairments and alcohol problems WITHOUT brain damage Alcohol related cognitive disorder - impaired cognitive functions Executive functions Response inhibition Risk taking Decision making Abstract reasoning Mental flexibility Memory Learning & retrieval efficiency Not an amnestic syndrome Visuospatial functions Often with an executive component (e.g., Rey complex figure) Social cognition Affective prosody Perception of facial expressions Interpersonal interaction ACUTE MAJOR MILD NC COGNITIVE COGNITIVE DISORDER EFFECTS DISORDER CHRONIC WERNICKE KORSAKOFF ALCHOHOL CAUSE DRUNKENNESS EFFECT OF A. ENCEPALOPATHY SYNDROME DEMENTIA MISUSE RESUMOS ORGANIZADOS 8  ACUTE MAJOR MILD NC COGNITIVE COGNITIVE DISORDER EFFECTS DISORDER amnesic impairment in syndrome and every cognitive dependent, executive execution; domain desinhibition, visuoespatical acute confused visuospacial possible, no COGNITIVE numbness, and social mental state and social amnesic and no PROFILE decrease in cognitive delirium cognitive confabulations; reaction time, impairments impairments somatic confuse and lack of commorbidity illness insight frequent recovery possivle recovery chronic but normalises after after timetly possible until a chronic partial reovery PERSISTENCE a period of time treatment with year after (irreversible) depending on without A vitamin B1 abstinence aetiology injections diencephalon temporary, work diencephalon (anterior and wide spread on GABA, (thalamus, and medio-dorsal BRAIN prefrontal cortical and glutamate mammillary thalamus and ABNORMALITY volume loss subcortical neurotransmitter bodies) and mammillry abnormalities system cerebellum bodies) and cerebellum Acute cognitive effects What happens when we drink, the brain functions suboptimally, hampering cognitive function excessive drinking → RF for chronic changes, cortical atrophy due to white matter demyelination malnutrition and excessive drinking → severe vitamin defiency / adding to that comorbidity leads to dementia lcohol imitates the neurotransmitter GABA that stop the activity in the SN → less activity in the brain , the thinking, analysing are affected by the frontal lobe damage → motor skills diminish, slow and memory gaps → inibitory activity inthe cerebellum, hippocampus and cortex more alcohol → supression in breathing, coma and death Chronic effects of alcohol abuse Main effect : volume loss in frontal cortex (and major risk of alcohol relapse) both grey and white matter loss and increase of cerebrospinal fluid volume in areas of tissue loss less aparent volume loss in parietal cortex, hippothalamus, cerebellum and dienceephalon (thalamus) neurotoxicity → volume loss is more likely from demyelination then cell death → atrophy is reversible (from remyelination) RESUMOS ORGANIZADOS 9  Comorbid disorders 1. traumatic brain injury from alcohol related head injury (30/50%) 2. cardiovascular disease a. greater risk of diebetes type 2, cardiovascular disease → less cog functions 3. liver damage a. alcohol is break down in the liver b. cirrhosis(scarring) and hepatitis (inflammation) and failure → this last one can show perfect cognition one day and the next be completely dependent 4. malnutrition a. too much alcohol → less hunger cues → less food intake FAS - fetal alcohol syndrome → less social interaction and oversensitive , plus all the physical changes WERNICKE ENCEPHALOPATHY - preceeds KS Socially isolated life → higher risk of thiamine deficiency (B1) as a result of malnutrition leads do wernicke encephalopathy Acute neurological disorder ← severe B1 deficiency; it can be reversed in the first hours with B1 injections This plus malnutrition is characterized by 1 of the Caine criteria confusion/delirium motor problems (ataxia) eye movement disorder (nystagmus - rapid involuntary repetitive eye movement ) (ophthalmoplegia - eye muscle paralysis or weakness) people with WE also have respiratory infections, urinary tract, abdominal and meningitis or sepsis NEURO IMAGING - atrophy of mammilary bodies and thalamus in the Diencephalon KORSAKOFF SYNDROME It’s developed after delayed or inadequately treated Wernicke Encephalopathy, it is a neuropsychiatric disorder. The cognitive feature is disproportionate impairment in Episodic memory relative to other cognitive domains, and absence of illness insight. There are confabulations !false memories! AND PERSONALITY CHANGES WITH IRRITABILITY OR APATHY Criteria RESUMOS ORGANIZADOS 10  disproportional disorder in episodic memory with temporal gradient (anterrógrada e retrógrada) lack of illness insight confabulations Alcohol is not a KS cause- it’s the thiamine deficiency! It can occur in hyperemesis gravidarum (pregnancy puking), after bariatric surgery and cancer Memory affected by KS - amnesic syndrome Characterised by severe impairments in long-term episodic memory (anterograde and retrograde amnesia) anterrógrada (n consigo guardar nova info/ só guarda depois de apresentação repetida; dificuldade em por memório no espaço de tempo correto) Specifically in episodic memory (‘everyday memory’; ‘what, where and when’) Contextual confusion (target information related to wrong time and/or place) Increased (proactive) interference Can be established with any episodic memory test that includes Delayed recall Free recall vs recognition Verbal and nonverbal stimuli retrógrada (esquece memórias, memórias mais recentes esquecidas mais rapidamente)- with temporal gradient in autobiographical memory autobiographical memories : interview (personal semantic memory and episodic memory- specific life events) cue word tecnique - recall what comes to mind when i say summer diaries life narrative method retrieval problems and executive contextual memory: problems with placing memories in time increased sensititvity to interference - proactive: old memories disturb new ones retroactive : recent memories disturb older ones executive dysfunction (and other cognitive deficits associated with AUD also visuoespacial and executive functioning, social cognition and intelligence Memory not affected Unimpaired working memory (like phone numbers) RESUMOS ORGANIZADOS 11  Intact long-term memory for skills (procedural memory) Intact priming (previously presented information has an automatic beneficial effect on later performance) Intact emotional memory Flashbulb memories vivid recall of contextual details (‘flashbulb memories’), but less detailed and less consistent reports compared to HC Non-memory domains affected Executive function: shifting and updating most affected Social cognition: Theory of Mind: mentalising, the ability to infer thoughts (cognitive) and feelings (affective) of others Emotion perception Relevant for successful social interaction Apathy “a lack of motivation that is not attributable to intellectual impairment, emotional distress, or diminished level of consciousness” Three dimensions of apathy Behavioural activation (eg. Immediately doing something after having planned this) Social motivation (eg. Starting a conversation with others) Emotional sensitivity (eg. Feeling bad when someone close to you was informed to be seriously ill) Neuropsychiatric symptoms provoked and spontaneous confabulations are a hall mark - unintentionally recall non-existent memories (its different to lie) confabulations reflected filling in memory gaps, if I don’t know the answer temporal confusion, strategic retrieval deficit, executive memory dysfunction (impaired reality monitoring) impaired reality monitoring - old memories may become activated on the basis of a question, despite the fact that these have no relationship with ongoing reality increased irritability, agitation, desinhibition or apathy → which can be severe Brain abnormalities in Korsakoff Syndrome RESUMOS ORGANIZADOS 12  diencephalon → prominently affected / may appear in the MRI in acute phase → thalamus and mammillary bodies damage to the anterior thalamus → not only in alcohol use but also with those who recovered from WE prefrontal and hippocampus atrophy → present cause the long term alcohol consumption in non alcoholic KS patients → damage is limited to the diencephalon memory and executive deficit central to KS appear to be directly related to the diencephalon lesions Mammillary bodies connect to hippocampus with thalamus and fornix, referred to as the diencephalic-hippocampus memory circuit- essential for encoding and storing new memories Thalamus is connected with pre-frontal cortex, making it an important hub for executive functions CONFABULATIONS Provoked: incorrect response to a question or situation in which a person feels compelled to say something Spontaneous: occur without a trigger, patient acts accordingly Not just “filling in memory gaps” due to amnesia, but result of temporal confusion strategic retrieval deficit, and impaired reality monitoring(“executive-memory dysfunction”) Spontaneous confabulations may deminish over time, provoked confabulations still present in chronic phase Effects of Alcohol on the adolescent brain Drinkers lower volumes of global grey and white matter Also in specific brain regions But only in the 2 ‘older’ cohort No dose-response relation Explanations: Direct neurotoxicity? Effect of intoxications (drunk/hangover)? Cause or consequence? (risky behaviour) genetic predisposition. pre existing mental health… RESUMOS ORGANIZADOS 13  Effects possibly stronger for cannabis than alcohol But many study limitations so far Small and heterogeneous samples Substanse (mis)use very heterogeneous Large methodological differences No clear dose-response relationship Effects of sociodemographic factors not always taken into account Alcohol dementia determining the aetology of the dementia syndrome is complicated and may be the result of somatic/psychiatric comorbidity, social factors/addiction history alcohol dementia is NOT due to pathophysiology, it is progressive neuro degeneration solely as a result of Alcohol abuse RESUMOS ORGANIZADOS 14  CARE TRAJECTORY IN ARCD Hospital → suspected Wernicke encephalopathy? Addiction care → suspected ARCD? Multidisciplinary diagnostic work-up Cognitive / neuropsychological assessment (after 6 weeks of abstinence) Neurological examination (incl. MRI) Somatic assessment incl. vitamine status Psychiatric evaluation (co-morbidity) Abstinence of all other substances Korsakoff’s syndrome→ Sheltered living/nursing home Alcohol-related cognitive disorder →Assisted Living/outpatient treatment No or mild cogn. disorder→ Regular addiction care Vascular cognitive impairment Cerebrovascular diseases include diferent clinal syndromes ans disorders (e.g small vessel disease, cerebral infarcts and haemorrhages, strokes or cva) stroke → acutte loss of function in body part and or speaking (and loss of strengh, sensory disorders or loss of coordination/control of movements) cognitive impairment affects functional recovery, quality of life and social participation after stroke Cognitive disorders with a cerebrovascular aetiology Vascular Cognitive Impairment (VCI) all froms of cognitive disorder associated with cerebrovascular disease aetiology large vessel disease RESUMOS ORGANIZADOS 15  small vessel disease VCI covers the entire spectrum from mild cognitive disorders to vascular dementia (covers the cognitive consequences its a spectrum going from no impairments a vascular dementia sever cognitive impairment due to stroke : without using dementia cause dementia is thought as progressive - and is not the cause epidemiology NL→ 40,000 persons per year risk: ageing and incidence greater due to age 15% with strokes are between 18-50 yo people with cerebral haemorrhage are younger than with cerebral infarct VCI MILD : vascular risk factors lacunar infractions MAJOR - vascular dementia post-stroke dementia PSD subcortical ischaemic vascular dementia multi infarct corticale dementie mixed dementia RESUMOS ORGANIZADOS 16 RESUMOS ORGANIZADOS 17  Large vessel : afeta partes enormes no cérebro - too much white matter Small vessel: cerevral microbleeds ; white matter hyperintensities (branco a mais); lacunes - pontos lacunas pretas Causes: prevalence of vascular risk factors YA- smoking and depression MA- hipertension, hearing loss, smoke, depression and diabetes OA- hiper tension and hearring loss and diabetes are more proeminent Vascular risk relates to accelerated cognitive decline plus obesity and groing of sedentary lifestle make ageing related changes more pronounced, modifying can prevent delays up to 50% of all dementia cases Cardiovascular risk factors make aging related changes more pronounced RESUMOS ORGANIZADOS 18  E.g. hypertension, alcohol usage, obesity, smoking, physical inactivity, diabetes Modifying these might prevent or delay up to nearly half of all dementia cases Executive functioning and (psycho)motor speed are most prominently affected by SVD (working episodic memory speed and ) cerebral perfusion → flow of the blood, which is more in individuals that work out (there is better Executive functioning and Psychomotor speed) STROKE - cva Cerebral infarct blood clot - embolism- closes an artery or the bloodstream becimes obstructed by local stenosis of a blood vessels acute symptoms: drooping of the face, loss of functioning of the body, difficulty speaking less comon is decreased vision (double), balance, impaired coordination, sever headache and fainting neurological symptoms: one side of the body usually Its done a CT scan without contrast, and the place of the stroke is demernid with ct angiography - with contrast Patients who reach the hospital within a few hours of clearly defined symptom onset may be eligible for intravenous thrombolytic therapy, in which drugs are administered to dissolve the clot. A proportion of patients are eligible for endo-vascular treatment, in which microcatheters are inserted into the brain from the groin, and the blood clot is removed mechanically. Ischemic stroke its the 80% of the cases of strokes(trombus → anoxia (without blood)) blood flow is blocked → tissue damage EMBOLIC : blood clot or debris (embolus), can cause damage to large arteries or distal branches ; focal neurological defecits clotted material that breaks off from elsewhere in the body LACUNAR : small deep penetration artery is blocked (plaques) due to diabetes → small area damage (lacunae); small affecting deep structures (deep in the brain) ; 1/3 of patients why this type of stroke have cognitive impairment described as decrease in mental capacity RESUMOS ORGANIZADOS 19  cognitive impairments rage from: attention, info processin speed, executive functions - also memoru, language and visuospatialfunctions also social cognition - emotion recognition and theory of the mind ATHERO-THROMBOTIC : forms in brain artery, trombrus (blood clot) in a large artery with a caroitid; severe symptom formation of blood clot (trombotic stroke) in brain artery ischaemic core → destined to die tissue penumbra → area located around the core and salvagable Diaschisis is a sudden change of function in a portion of the brain connected to a distant, but damaged, brain area. Risk factors: Hypertension, hypercholesterolaemia, type 2 diabetes, obesity, arterioscle-rosis, and atrial fibrillation a Haemorrhagic stroke more or less 20% of the strokes (rupture) - brain vessel rupture, loss of consciousness, confusion… due to high pressure intracranial Intracerebral haemorrhage (within brain) Lobar: located in one of the cerebral lobes Non-lobar: located in basal ganglia, thalamus, cerebellum or brain stem Subarachnoid haemorrhage (not within brain) Bleeding in the subarachnoid space, space between meninges - sub-aracnoid space death for most cases 85% is because aneurysm rupture intracanial also called as aneurysmal SAH ASAH →sudden severe headache, plus nausea plus neck stiffness → hippertension → cause change in wall of BV bleeding outside of the brain→ related to C amyloid angiopathy CAA Transient Ischaemic Attack (TIA) Recovery < 24 hours. TIA: Brief ischaemic event Focal symptoms: Problem is specific to a certain area of the brain Abnormalities often also appear on acute brain scan Increased risk of having a stroke 25% are reffered as lucanar infact - small penetration arteries Many people still report cognitive problems 3 months after a TIA RESUMOS ORGANIZADOS 20  Impairments on neuropsychological assessment 29 to 68% have mild cognitive impairments, and 8-22% have severe the prevalence of cognitive impairments depende on the method of measurement, timing of examiation and age a neuropsychological assessment is most suitable to detect the subtle cognitive impairments that may have a negative impact Type of motor or cognitive dysfunction depends on location of stroke: Supply area of anterior cerebral artery ACA 80% OF THE STROKES OCCUR HERE largest cerebral artery and supplies blood to frontal temporal and parietal areas of brain and deep structures Supplies blood to the dorsal and medial parts of the frontal and parietal lobes. Impairments are found in: Language - broca por ser frontal Executive function Social cognition Behaviour and emotions personality changes, emotional disorders, neurological symptoms, behavioural disorders, cognitive disorders Supply area of middle cerebral artery Supplies blood to the frontal, temporal and parietal lobes, and deep brain structures (eg thalamus). Accounts for approximately 80% of all stroke cases. Common consequences: Memory disorders Aphasia Apraxia Hemispatial neglect Extinction RESUMOS ORGANIZADOS 21  Supply area of posterior cerebral artery Supplies blood to the occipital and temporal lobes. Common consequences: Hemianopsia or quandrantanopsia and visual agnosia (e.g., object agnosia, proposagnosia) MAJOR STROKE 2/3 of patients have cognitive impairments - significant predictor of limited quality of life and poor functional outcome may be present in all domains , depending on the location and severity (the brain is interrelated so even tho is affects one part it affects a lot more) attention, information processinh and difuse processing speed, memoru (anterograde amnesia) and executive functions, remembering new info, switching between dif tasks (it difficults the initiative or keeping track of a sequence of actions) and aphasia (persists in long term ) time pressure - absorve a lot of ifo in small gap of time- is difficult THALAMIC STROKE can present the symptoms nor dirrected related to stroke (unusual combinations of speech, sensory and motor symptoms, confabulations, acute memory impairment) thalamus is the switching station - crucial connectinh role between dif brain regions- therefore the stroke depends in the precise location within the thalamus ACUTE AND CHRONIC EFFECTS cognitive impairment may be difuse and severe, consciousness can be decreased and often it may be difuse and severe, consciousness can be decreased and often patients feel disoriented. This can be a direct consequence of the haemorrhage or infarct, damage in specific brain regions, but also of complicationg such as increased pressure and haematoma. About 50% experience cognitive impairment in the first 3 weeks. Cognitive impairment also occurs in the long term, with rates ranging from about 40% to three-quarters of patients. In cerebral haemorrhages, cognitive disorders are mainly related to the severity and size of the haemorrhage, rather than its location. In contrast, type and severity of cognitive disorders after cerebral infarcts are often linked to the specific location in blood supply areas of the brain. Cognitive recovery 49% cognitive impairments in acute phase → 31% in the follow up (language suffers decreases less) RESUMOS ORGANIZADOS 22  ‘Unimpaired’ patients still unimpaired after six months Other group generally shows progress Dynamic recovery and not ‘demented’ Dependent on affected domain may be linked to location of recovery Determinants Schooling/age → ‘cognitive reserve’? Lesion volume less important than location → small strategic infarcts sometimes accompanied by dementia Mood disorders post-stroke Depressive symptoms occur in one third of stroke survivors Frequency is highest within the first year after stroke event Associated with poor recovery and long-term outcomes Suggested underlying mechanisms: biological hypothesis reactive hypothesis vascular depression hypothesis DEMENTIA SUBTYPES Alzheimer 50-75% Lewy Body 10-25% Fronto-temperal 10-15% VASCULAR 20%-30% Cortical v. subcortical profile No specific neuropsychological pattern Course often fluctuates Strategic infarct or multi-infarct may contribute RSK FACTOR : cardiac arrhythmias, high blood pressure and diabetes Most important cognitive deficits executive deficits - at the level of thought, behaviour, emotion additional deficits: mental processing speed RESUMOS ORGANIZADOS 23  memory visuoconstruction language concentrating information processing and executive functions → more severe than alzeihmeirs its not needed to be progressive to be labelled , emphasis in the stepwise decline Alzheimer disease - n fui you can have alzeihmer disease without dementia and like wise DISTINCTION: clinical syndrome - dementia - and aetiology subtyoe - cause for the clinical syndrome MAJOR NEUROCOGNITIVE DISORDER DEMENTIA → neurodegenerative disease that causes dementia (clinical syndrome, group of the, ) (you can have alzeihmer disease without dementia and like wise) Progressive cognitive decline with regard to previous performance level in one or more cognitive domains memory or executive functioning, or language of information processing - most proeminent is the memory loss Presence of interference in daily activities support needed to perform complex (or instrumental) activities of daily living (iADL), such as internet banking, using a smartphone, administration, medication intake, cooking, etc. No delirium present No other psychiatric disorder present that could explain the cognitive disorders such as depression, schizophrenia can lead to depression, apathy and anxiety final stage : overall cognitive loss, and the person is completly dependent RESUMOS ORGANIZADOS 24  SUBTYPES DEMENTIA Dementia = syndrome (descriptive), says nothing about underlying disease / pathology Underlying cause/aetiology must therefore be specified: ‘Major neurocognitive disorder due to…’ (DSM-5) Alzheimer’s disease Frontotemporal lobe degeneration Lewy body dementia Vascular aetiology Traumatic brain injury Alcohol and drug use RESUMOS ORGANIZADOS 25  HIV infection Prion disease Parkinson’s disease Huntington’s disease PRE-DEMENTIA STAGE MILD COGNITIVE IMPAIRMENT MCI (mild cognitive impairment): pre-dementia stage of cognitive decline Memory (or other domain) impairment (established on neuropsychological tests) Cognitive complaints (subjective) No significant limitations in daily life functioning (= no dementia!) About 25-50% of MCI patients develop dementia in the next 2-5 years (often dementia due to Alzheimer’s disease) Research focuses on predictors of conversion to dementia and early treatment to delay onset of dementia (or even stop the disease) MILD COGNITIVE DISORDER Cognitive decline with regard to previous performance level in one or more cognitive domains Dementia doesn’t develop overnight, is gradual, beginning with mild cognitive impairment MCI No interference with daily activities no dementia; no delirium; no other psychiatric disorder present that could explain the cognitive disorders such as depression, schizophrenia ‘Mild NCD due to...’ : Alzheimer’s disease Parkinson’s disease Vascular dementia Main differences between MCI and dementia : the ability to funcion independently is still relatively intact in the MCI DIAGNOSTIC CRITERIA modest cognitive decline concerns about mild decline compared with previous level of functioning, expressed by th individual or reliable informant modest impairment in cognitive performance (1-2 standart deviations from the mean) documented by the objective cognitive assessment there is no overt interference with independence in everyday activities, although these may require more time and effort, accommodation or compensatory strategies cognitive problems do not exclusively occur in the context of a delirium and cannot be explained better by another mental disorder RESUMOS ORGANIZADOS 26  can be atributed too underlying Alzheimers disease pathology with different levels of certainty; likelihood is due to : cognitive profile that is typical for early Alzheimers disease : prominent impairment in episodic memory - amnesic MCI- with insidious onset and a progressive course positive biomarker reflecting the Alzeihmers disease pathophysiological process Amnesic MCi can be considered a prodromal stage of Alzheimer , but can also be he precursor of oher forms of demenia there are people with MCI that do not have an underlying neurodegenerative disease → never develop dementia the changes of developing dementia in 5-10 years after MCI diagnosis is 50% NORMAL AGING - MCI- DEMENTIA CONTINUUN continuum of cognitive (dys)function : MCI, mild demetia, moderate dementia, and severe dementia ALZHEIMER’S DISEASE First patient described in 1911 by Alois Alzheimer “Cortical dementia” 50-60% of all dementias Progressive and degenerative (poor prognosis) Structural and functional abnormalities in the brain Disease (one of the causes of dementia syndrome) insidious onset and gradually progressive course - over months to years, not hours or days cognitive impairment involves minimum of 2 of the following: memory, executive functions, language or visuospatial functions grey matter volume loss, starting with medio-temporal lobe followed by posterior and frontal cortical regions and eventual global cortical atrophy; the structure and volume of the hippocampus is affected first RESUMOS ORGANIZADOS 27  no evidence of a substancial concominant cerebrovascular, neurological or psychiatric disease that could explain the cognitive symptoms level of certainty can be increased by : evidence of progressive decline on subsequent evaluations based on information from informants and cognitive testing in the context of either formal neuropsychological evaluation o standardised mental status examinations evidence of the Alzheimers disease pathophysiological process obtained through neuroimaging or cerebrospinal fluid analysis evidence of a causative A disease genetic mutation can only be confirmed post-mortem by autopsy neuropathology examination modern biomarker research with structural imaging(CT AND MRI), molecular imaging (amyloid and tau PET imaging) and cerebropsinal fluid (CSF) → help make in vivo assessment pathophysiology ABNORAML ACCUMULATION AND DEPOSIT OF EXTRACELLULAR PLAQUES OF THE AMYLOID-BETA PROTEIN -senile plaques- AND INTRACELLULAR TANGLES OF THE PROTEIN TAU - nruofibrillary tangles- LEAD TO DEGENERATION OF NERVE CELLS AND BRAIN ATROPHY ULTIMATLY (plaquets are amyloids or accumulations in the neurons ) shrinkage of the brain - explains the memory problems, it happens in the medial temporal lobe and hippocampus (grey matter) increase of cerebrospinal fluid around hippocampus DIAGNOSIS Clinical diagnosis on basis of exclusion Supporting diagnostic evidence: MRI/SPECT/PET- quite limited, there is nothing to treat, no medication Protein abnormalities in cerebrospinal fluid (CSF or liquor) - AMYLOID BETA - if is low is indicative Neuropsychological assessment RESUMOS ORGANIZADOS 28  Differential diagnosis often difficult: vascular dementia, depression, frontotemporal dementia, etc. The cognitive profile of Alzheimer's dementia Memory impairment present from the start (with temporal gradient- cant be encoded nor retrieved) Decline in abstract thinking (intelligence) Impaired orientation in time, place, person Other cortical disorders: aphasia, agnosia, apraxia, executive dysfunction Progressive course MEDIAL TEMPORAL LOBE ATROPHY older stages dont affect only memory but all domains lower then 7 is indicating the coping images, the 3 dimensional is gone, shown some neglect VAT- visual association test → associating new memories(macaco e depois macaco com pincel e ela n se lembra de ver um pincel sequer) Cognitive Course over time In principle, all cognitive domains can be impaired Amnesic MCI - predementia stage RESUMOS ORGANIZADOS 29  progressiveepisodic memory(anterrógrada) deficits are the 1st signs : impaired learning and retrieving of recently learned information deficit in memory consolidation by neuronal loss of the hippocampus word finding problems Mild dementia problems in other cognitive functions - language production, orientation in time and place, planning and performing activities (most profound cause by deficits in mental flexibility and divided attention ) language may be gradual decline in semantic knowledge and relationships -increasing atrophy in medial temporal lobe reduced orientation in time and place - episodic memory deficits primeiros sinais de dificuldade em executar atividades do dia a dia Deficits in abstract reasoning (intellectual functioning) Executive dysfunction Reduced insight semantic memory, languagem working memoru and executive functions… Moderate dementia even more extensive cognitive impairments: episodic memory is deteorating, language production, orientation in time and place worsens and visuoconstruction as well as apraxia mais dependente Language disorders (production, comprehension) Increasing retrograde amnesia with temporal gradient Apraxia Visuospatial deficits → pca: posterior variant of Alzeihmers dementia - LATER ON Severe dementia severe cognitive loss and the person is completely dependent on their environment; confusion, disturbed behavior, and difficulty in communicating (persevations and intrusions), mustism and severe mental retardation Social cognitive deficits Behavioural problems (disinhibition, apathy) Profile of memory impairment Impairments in episodic memory tasks ▪ Specifically in the encoding of contextual information ▪ Poor delayed recall ▪ Rapid forgetting Semantic memory (general knowledge) in early stages (relatively) intact ▪ However: semantic priming impaired later in the course RESUMOS ORGANIZADOS 30  (in subcortical dementia, motoric priming is impaired) ▪ Eventually all functions are affected Working memory ▪ relatively intact on span tasks (phonological loop and visuospatial sketchpad) ▪ impaired on associative (‘binding’) and executive aspects (dual tasks) of WM Meta-memory in early stages intact; later severely impaired (knowledge of memory missing ) Implicit knowledge often intact ▪ Skills unimpaired (already learned) but apraxia can be present ▪ Implicit learning intact, but ‘structured learning” required - need to show repetedly… Pathophysiology Three hypotheses: cholinergic hypothesis [obsolete: it is a consequence] treatment with acetylcholinesterase inhibitors (such as rivastigmine: Exelon® and galantamine: Reminyl ®) does not cure the disease (treats just the symptoms) and may have considerable side effects - not good… aducanumab - fda approved but not in the eu, twice a week injections, way too expensive, dangerous… amyloid cascade hypothesis (under debate) widely accepted abnormal accumulation of the amyloid beta protein is the primary driver of alzheimer based in people with mutation in the amyloid precursor protein APP gene they show overproduction of amyloid beta and all eventually develop A. APP is a precursor of amyloid-beta protein and when is broken down in abnormal proportions it leads to overproduction of beta-amyloid THE HYPOTHESIS- the accumulation of amyloid beta plaques triggers the propagation of tau pathology → sinapse loss leading to neuronal death, cognitive decline and ultimatly dementia RESUMOS ORGANIZADOS 31  amyloid beta can be present as 20/30 years before the onset of dementia, and those with amyloid beta plaques and tau pathology are in higher risk the presence of this amyloid IS NOT EQUIVALENT to having A disease nor developing dementia (1/3older people have the pathology and do have the plaques) idea that amyloid deposition plays a key role : there is therapies designed to reduce the quantity although they are inconclusive (probably due to intervention in people in late stages in which the damage is thought to be irreversible) OR amyloid deposition is not sufficient to develop this disease, multiple pathophysiologiccal processes play a role in the development vascular hypothesis: mixed pathology cerebrovascular disease plays an important role in the development of the disease → vascular risk factors, such as hypertensions… lead to reduced blood flow and oxigen deficiency in the brain → goes to lead to stiffening of the arterial walls but also to a metabolic reaction that causes an overproduction of amyloid beta and ultimatly neurodegeneration gain strong support: because high blood pressure and vascular damage are a predictor of cognitive decline and dementia POSTERIOR CORTICAL ATROPHY Atypical form of Alzheimer's dementia Atrophy is occipitoparietal in particular Characterised by visuospatial and visioconstruction disorders: Agnosia Hemispatial neglect Spatial cognition More common in younger patients pattern of neurodegeneration starts in theparietal and occipital lobe and spreads to occipitotemporal lobe (vs. Alzheimer which the medio-temporal lobe is spared for a long time ) RESUMOS ORGANIZADOS 32  BRAIN CHANGES - COGNITIVE AGEING RESUMOS ORGANIZADOS 33  METHODOLOGICAL CHALLENGES - COGNITIVE AGEING Cross-sectional research Longitudinal research Less decline than previously assumed, but decline does set in with advancing agE EXECUTIVE FUNCTION THEORY- COGNITIVE AGEING In young adults less brain activation needed, only in one hemisphere In older adults, more activation needed, also more bilateral To maintain the same behavioural performance Hemispheric Asymmetry Reduction in OLDer adults SCAOFFOLDING THEORY OF COGNITIVE AGING (STAC) COMPENSATORY MECHANISMS RESUMOS ORGANIZADOS 34  COGNITIVE RESERVE - COMPENSATORY MECHANISMS Cognitive reserve (CR) is a concept coined by Stern (2009) Protective against effects of brain pathology (compensatory mechanism) Higher cognitive reserve – slower cognitive decline CR ~ “cognitive scaffolding” : education, social activity, cognitively demanding lifestyle Parkinson disease spectrum - n fui Second most common neurodegenerative disorder after Alzheimer, so it is a complex disease and includes motor and non-motor symptoms. Chronic progressive nature(onset gradually after 50yo.), increasing the symptoms in severity and the number of them. Cannot be prevented, cured or get progression delayed, only symptomatic treatment is available Vascular parkinsonism is a 2º form of Parkinson disease PD in which the parkinsonism symptoms occur due to cerebrovascular damage or abnormalities We have atypical parkinsonsonisms : Multiple system atrophy -MSA - cereberall part Progressive supernuclear paralysis -PSP corticobasal degeneration CBD Lewy body dementi DLB -not motor problems but cognitive → these have some clininal similarities to PD, but also differenes, including faster progression, limited response to medication and prominent cognitive decline at a relatively early stage of disease RESUMOS ORGANIZADOS 35  tremor is only 50% of the cases in PD drug induced- is movement disorders associetad with the side effect of certain drugs Epidemiology 2/3 have PD and the other have atypical from 58,000 NL men are most common (3,7/1000 men vs. 2,5/1000 women ) onset is typically in between 50/70, 65+ people (1% 60+ people have Parkinson), insidence increases when u get older , but earlu onset is possible ( PDD > AD Hallucinations (early) in LBD > PDD > AD Motor impariment in PDD > LBD > AD Memory impairment in AD > PDD and LBD Visuospatial impairment in PDD and LBD > AD Noradrenergic+cholinergic deficits in LBD > PDD Dopamine deficits in PDD > LBD and AD RESUMOS ORGANIZADOS 47 RESUMOS ORGANIZADOS 48 RESUMOS ORGANIZADOS 49  Epilepsy - n fui Epilepsy is one of the worlds oldest described condition- also called as falling disease. There are different typs which we categorize as absences, tonic-clonic or focal seizures. Epilepsy causes cognitive dysfunction Severity is very difficult to measure in epilepsy, the frequency can lead to brain damage because is a state of anoxia and leads to cognitive dysfunction. The whole brain is envolved and in the EEG we can see activity in the whole brain, the activity up down aggressive is the attack RESUMOS ORGANIZADOS 50  Epilepsy: definition is a chronic brain disorder characterised primarily by seizures where ther is abnormal and excessive eletrical activity in the brain that leads to changes in the fuctioning or behaviour of the patient → epileptic seizure (the focus can differ→ causing temporary disturbances, consciousness, motor skills, cognitive functioninh or sensory functions) “epilepsy is having seizures” → differences are the common characteristics epilepsy has nothing to do with the eeg or mri, they support the visualization of the seizure 2005: conceptual definition - ILAE – Seizure: transient signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain - saw the excessive activity in the eeg and synchronous becausse it was in the whole brain – Epilepsy: disorder of the brain characterized by an enduring predisposition to generate epileptic seizures, and by the neurologic, cognitive, psychological and social consequences is not attacks is seizures if they occur repreatedly and are no observable seizures but theres continuous epileptic activites on the EEG → epilepsy 2014: operational definition 1. At least two unprovoked seizures > 24h apart 2. One unprovoked seizure and a probability of another unproked siezure (next 10 years) 3. Diagnosis of an epilepsy syndrome unprovoced means that is not due to stop consuming alcohol, a lot of people after drinking a lot can have seizures from stoping the intake epilepsy is more than the sum of seizures: it leads to cognitive, behavioural, emotional and or psychosocial problems in patients RESUMOS ORGANIZADOS 51  ictal → seizure , variation between patients is very high (location of the focal point of the seizure, and so the symptoms are different)but within the patient is not (the focus is always the same in one patient) the symptoms are due to the focus of the seizure Basic mechanisms epileptogenesis unknown why they exist -the basis is unknown, we know why is doing that but sequence of events that converts normal brain to epileptic brain 2 essential epileptogenic processes seizure: as a clinical manifestation of abnormal and excessive excitation and synchronization of a population of neurons → changes of the normal balance between inhibition and excitations, also in balance → epilepsy as the tendency of recurrent unprovoked seizures neuronal network defect (condition): sequence of changes that convert a normal neuronal network to a hyperexcitated network can be the cause for the seizure but also the product of the seizures → changes in the functioning of one or more neuronal cells can have significant changes in normal functioning neurons nearby 1 seizure is not epilepsy, we need to wait for the other to be considered epilepsy RESUMOS ORGANIZADOS 52  Aetiology Can be divided into six subgroups if epilepsy is diagnosed, an mri scan is often performed first to determine if there is a structural brain abnormality or lesion that causes the epilepsy 1 gene analysis, blood tests and cerebrospinal fluid analysis, it can be investigated wether the cause of the epilepsy is respectively genetic 2 infectious 3 metabolic 4 immunologic 5 epilepsy can be cause by a combination of these factors for a lot of patients the aetiology of their epilepsy cannot be established 6 1. structural aetiology : there is an abnormality in the brain structure that is visible on neuroimaging of which can be reasonably be assumed that it is the cause of the patients seizures a. These structural brain abnormalities can be congenital or acquired. For example, a child can be born with structural brain abnormalities as a result of a malformation of cortical development, an abnormal development of the cerebral cortex during pregnancy. b. In the case of cortical dysplasia, for instance, neurons in the cerebral cortex are developed and organised in such an aberrant way that the neuronal excitation in the brain deviates signifi-cantly, making the prospect of developing epileptic seizures very high. c. Well-known types of epilepsy with a structural aetiology are mesial temporal lobe epilepsy with hippocampal sclerosis, the so-called 'post-stroke' epilepsy, and post- traumatic epilepsy after traumatic brain injury. 2. genetic aetiology: epileptic seizures are the result of a known or assumed genetic mutation. This gene mutation may be hereditary or de novo. Epilepsy with a genetic cause is very diverse and, in most cases, the underlying genes are not yet known. RESUMOS ORGANIZADOS 53  a. A well-known type of genetic epilepsy is Dravet syndrome (see Table 151). About 85% of patients with Dravet syndrome have a mutation or deletion in the sCiA gene. As a result of this gone mutation, the functioning of specific sodium channels in some neurons is reduced or non-functional, leading (eventually) to more epileptic discharges in the brain whereby epileptic seizures can arise. b. Epilepsy can have both genetic and a structural cause. An example of this is tuberous sclerosis. This is a genetic disorder in which there is an accumulation of abnormal cells on the surface of the brain (tubers) that can then be the cause of the epilepsy 3. Epileptic seizures can be the result of an infection. Often, seizures only occur after the patient has had an infection and not immediately during the infection. This is often seen in, for example, encephalitis and meningitis. a. Epilepsy can also be a consequence of an infectioh such as HIV, tuberculosis, or cerebral malaria, especially in non-Western countries. 4. /5 range of metabolic disorders and autoimmune diseases. An example of this last category is an autoimmune enceph-alitis. 'Autoimmune diseases' is a term to describe approximately 20 separate immune diseases of the brain in which the brain becomes inflamed due to a pathological disruption of the immune system. This leads, among other things, to epileptic seizures. a. In general, epilepsy is rarely caused by a metabolic disease. It is likely that most epilepsies based on a metabolic disorder also have a genetic basis. 6. The last category is unknown origin as for a significant percentage of patients, the aetiology of their epilepsy cannot be established. COGNITIVE FUNCTIONS INTELLIGENCE within normal range only specific epilepsies are associated with lowered intelligence quotient (children with generalised epilepsy- both christalized and fluid intelligence are in average 5/10 QI points lower; also patient with epilepsy due to a structural brain abnormality or lesions) intellectual decline - not the 1 feature of epilepsy exception : seve epilepsy syndrome in which a epileptic encephalopathy leads to intellectual decline associated mainly with infantile and childhood epilepsy syndromes in adults , this decline has been demostrated in adult-onset epilepsy and comorbid pathology, such as cardiovascular disease cognition profile significant decline in fluid intelligence and info processing speed as a result of depletion of cognitive reserve capacity due to the accumulation of pathogenic effects of ageing, epilepsu and comorbid diseases RESUMOS ORGANIZADOS 54  severity : related to functionl connectivity within and betweem 3 ajor brain networks decreased MEMORY Most scientific research on cognitive impairment in epilepsy has been focused on memory. Temporal lobe epilepsy, the most common type of focal epilepsy, is mainly associated with impairments in episodic memory. hippocampal atrophy or mesiotem-poral sclerosis: Difficulties in encoding and retrieving new information , which are often seen in and identified as the cause of temporal lobe epilepsy. The lateralisation of the focus of the epilepsy in the right or left brain hemisphere seems to influence the nature of the memory impairment. Patients with a left mesiotemporal epilepsy → impairment in verbal memory. This modality-specific memory impairment is seen less frequently in people with left temporal lobe epilepsy from an early age. the difference is due that in the young and still plastic brain of patients with an early-onset epilepsy, a reorganisation of brain functions can take place and alternative memory networks are formed. Despite it being well established that the hippocampus and other mesiotem-poral structures have a key role in encoding and retrieval, impairment in episodic memory is not restricted to patients with (mesio) temporal epilepsy. Although research findings are less unequivocal, patients with other types of focal or gen- eralised epilepsies also tend to score lower on memory tests than healthy peers. Reasoning from network theory, this is understandable; after all, memory is ogranised in networks that include not only temporal but also other brain structure Within episodic memory, autobiographical memory occupies a special place. Theu have problems in remembering personal events, people and/or places in their lifes, more than people with neurological diseases. Compared with healthy people, patients with focal elepsy appear to be able to recall fewer specific memories, and those memories are less detailed. There is evidence that suggests that the severity of autobiographical memory impairment increases with the number if seizures experienced. Seizure fredom would sometimes lead to improvement in autobiographical memory. semantic memory (memory for facts and knowledge) may be affected in patients with both temporal lobe and frontal lobe epilepsy. This is often accompanied by impairment in episodic memory INFO PROCESSING SPEED 'slowed information processing speed' in the context of epilepsy, : difference between psychomotor speed and central (or cognitive) information processing speed. Psychomotor speed is measured with tasks that mainly rely on (partly) automated 'peripheral' information processing, while central information processing speed also calls on attention, memory and/or executive functioning when processing information. RESUMOS ORGANIZADOS 55  It has been frequently shown that in people with epilepsy there is a slowed information processing speed visible on reaction time tasks, psychomotor speed tasks, information processing speed tasks with and without a motor component, divided attention tasks and/or selective attention tests. This 'mental slowing' was long attributed to the use of anti-epileptic drugs. The 'first-generation anti-epileptic drugs' such as phenobarbital, phenytoin, carbamazepine and valproate are particularly associated with slowing. The fact that epilepsy itself can also lead to mental slowing is evidenced by studies in which mental slowing was determined before anti-epileptic drugs were prescribed Therefore, in patients with epilepsy who are treated with (multiple) anti-epileptic drugs, there is potentially an accumulative effect of both epilepsy and anti-epileptic drugs on information processing speed. Slowing of information processing speed is described in all types of epilepsy and cannot be unequivocally attributed to specific structural abnormalities in the brain. has been shown to be associated with a reduced efficient organisation within and between three major brain networks; the visual perception network, the (dorsal and ventral) attention networks and a 'subcortical network' ATTENTION AND EXECUTIVE FUNCTIONS executive dysfunctions are mainly expected in frontal epilepsy. Impairments in executive function and attention are quite common in other types of epilepsy as well. For example, several studies indicated that patients with temporal lobe epilepsy perform similarly on executive function tasks as patients with a frontal localised epilepsy. In frontal lobe epilepsy, impairments are consistently found in several executive and attentional functions, among which working memory, problem-solving abilities and cognitive control functions The executive dysfunctions described in temporal lobe epilepsy are associated with impaired integrity of brain structures that are part of large fronto temporal brain networks. In generalised epilepsies, impairments in executive functions are described with regard to mental flexibility, inhibi-tion/self-regulation, planning and working memory. Impairments in alertness, responsiveness, selective attention and sustained attention are also well-known in several types of epilepsy VERBAL FUNCTION AND LANGUAGE SKILLS mostlyhas been performed in children. This may be because impairment in this cognitive domain can significantly impair learning and adversely affect children's academic performance and socio-emotional functioning. It may also be related to the fact that specific epilepsy types and syndromes that interfere with language tend to begin in childhood or adolescence (e.g. Landau-Kleffner syndrome and Rolandic epilepsy, see Table 15.1). Nevertheless, in daily clinical practice, language problems are one of the most common (second after memory) complaints in adults with epilepsy. RESUMOS ORGANIZADOS 56  Impairment in verbal function and language is often relatively mild in nature. Naming and word-finding problems are the most studied and also most consistently demonstrated, especially in patients with (left) temporal lobe epilepsy. Receptive language impairments are rarely observed SOCIAL COGNITION Adults with epilepsy generally have fewer social contacts, are more likely to be lonely, and also experience more psychosocial problems than their healthy peers (Stewart et al., 2016). Impairment in social cognition might underlie this. Patients with epilepsy appear less able to recognise basic facial emotions. This applies to both patients with focal (temporal or frontal) and generalised epilepsy (Edwards et al., 2017). In addition to emotion recognition, mentalising or Theory of Mind (ToM) is also globally impaired in patients with frontal, temporal or generalised epilepsy Adults with epilepsy score lower on tests invoking ToM skills developed early in life (false- belief tasks), but also appear to be impaired in later developed mentalising abilities (measured by faux-pas tasks or strange stories). Such impairments are not demonstrated in patients with a posterior epileptic focus, Although there are several hypotheses that an impaired ToM is actuady derived from impairments in executive functioning, research findings rescing this matter are inconclusive. In temporal lobe epilepsy, a young age at onset seems to be associated with more severe ToM impairment. This could have a neurological basis (interference of epilepsy with neurocognitive developmen),or be (also) related to environmental factors. That is, children with epilepsy are more likely to grow up (over)protected and find it harder to connect with peers because of their epilepsy. As a result, they risk falling behind in learning social skills and these are not caught up later in life EPILEPSY AND DEMENTIA Patients with epilepsy have up to three times a higher risk of developing dementia than those without epilepsy This holds particularly for older people with epilepsy that developed later in life (late-onset epilepsy). Whether or not a patient develops dementia does not seem to be related to the lifetime number of epileptic seizures. Also the opposite is true: people with vascular or Alzheimer's dementia are significantly more likely to develop epilepsy than those without dementia. The exact cause of the increased risk of dementia in epilepsy, and vice versa, is not known. There is empirical evidence that cognitive impairment manifests earlier in patients with dementia and epilepsy than in people suffering from dementia alone. Also, cognitive decline seems to have a more progressive course in people with both epilepsy and dementia. This suggests shared risk factors or an overlapping underlying pathology in RESUMOS ORGANIZADOS 57  epilepsy and dementia (see further Sen et al., 2018). PSYCHIATRIC DISORDERS can occur berofe (pre-ictal; ictus =seizure), during (peri-ictal) and after (post-ictal) before and during : a lot of anxiety is experienced post: mood symptoms and agitation are often reported psychiatric and or emotional symptoms patients experience between their seizures are more chronic in nature and may include depression or various anxiety disorrdes - generalised anxiety disorder, social phobia and panic disordder incidence is higher for those with epilepsy than in those without , suicide is also more significant explained: common pathophysiology underlying both epilepsy and anxiety/modd disorder - although consistent empiral findings are lacking others that contriute to the onset or maintenance of anxiety or mood symptoms : reduced self-confidende, lower self-esteem, reduced quality of lide, fear of seizures, feelings of vulnerability, stigma, perceived loss of control and less acceptance of disease prevalence of ADHD and autism is higher in patients with epilepsy Clinical epilepsy factors such as early seizure onset, a high seizure frequency, large amount of epileptiform EEG activity as well as frontal involvement in seizures are associated with symptoms that resemble the clinical symptoms of ADHD more freuent complaints of physical complaints withou a known medical aetiology psychogenic non-epileptic seizures- functional neurological symptom disorder not caused by epilepsy nor any other neurological or somatic disease EEG shows no epileptiform psychotrauma is one of the main risks factor for developing PNES personality disorders, depression, chronic anxiety and intellectual disability are also well known risks factors PSYCHOSOCIAL SEQUELAE people with this disorder experience psychosocial problems more often then others healthy completing education, difficulty finding a job and engaging in social contacts dirext consequences of their condition (not being allowed to drive a car, until they did not have a seizure in 12 months ) constant anti-epileptic drugs and dealing with precepts (number of hours of sleep, alcohol consumption…) more likely to experience external locus of control, accompanied with passive attitude and denial and avoidant coping, less use is made of problem-solving strategies in vicious circle this leads to an increase in psychosocial problems and reduced well-being, sometimes even social isolation. The perceived unpredictability of seizures further fuels feelings of uncontrollability and insecurity. RESUMOS ORGANIZADOS 58  COGNITIVE IMPAIRMENT IN EPILEPSY high disease burden : determined by the degree of impairment in cognitive, emotional and behavioural functioning resilience is not only related to seizures, but mostly to the degree of cognitive impairment cognitive impairment - less efficient processing handling reduces belief that one can cope with the difficulties they face, mainly the consequences of their epilepsy lower self esteem, increased emotional problems, intense worying → reduces quality of life AETIOLOGY FOR COGNITIVE IMPAIRMENT which factors have considered long-term effects on cognitive functioninh changes in behavioural, cognitive and emotional functioning are traditionallu thought to result from specific clinical features of epilepsy of which seizure frequency is historically the most focused on 1st hypothesis each epileptic seizure induces alterations in brain structure (not entirelly true- inconclusive and mild direct correlation between seizures and persistent neuropsychologuical dysfunction ) the only patient that we can see the link betweem the epilepsy and worsening of neuropsychological impairment → early-onset chronic intractable epilepsy, patients with multiple status epilepticus or suffering from developmental or epileptic encephalopathy -< in the contex of a syndromal epilepsy 2nd hypothesis not seizure but the pathophysiology of the specific type of epilepsy is predictive for the cognitive impairment in a patient focal epilepsy → impairments in specigic cognitive functions generalised epilepsies → lead to diffuse and widespread cognitive impaiment there are incongruenties 1. a lot of patients with focal epilepsy have diffuse cognitive impairments 2. relatively few similarities in neuropsychological profiles between patient with the same type of epilepsy 3. there is much overlap in neuropsychological profiles between different types of epilepsy complementary to modern neuroimaging studies detecting more extended rather than localised abnoramlities in brain structure and connectivity within several epilepsy syndromes this supports the reframing of both epilepsy and neuropsychological impairment as the product of diseased networks in the network theory RESUMOS ORGANIZADOS 59  NETWORK THEORY- epilepsy is regarded as a network disease, characterised by the rapid hypersynchrony of brain networks interconnections between and within brain regions - functional connectivity- in patients with epilepsy differ from healthy peers in path length, number of network nodes and efficiency the disruptions cause epileptic seizures, but also cognitive functioninh, emotion or behaviour brain networks close to the epileptogenic point are the most disturbed, but abnormalities can extend far beyond that zone this means that there can be both focal and widespred cognitive impaiment, eben in the absence of structural brain abnrmalities from the network theory - cognitive impairment is not a consequence of epilepsy but rather should be considered a comobidity of epilepsy TAXONOMY OF COGNITION IN EPILEPSY - cognitive phenotype based 1. generalised cognitive impairment (18% of patients studied) significant impairment affecting all cognitive domains. 2. focal, relatively mild cognitive impairment (31%), specifically in language, memory and/or executive functioning and speed of information processing. 3. No cognitive impairment (51%: cognitive performance intact and comparable to healthy controls. In their study group of patients with temporal lobe epilepsy, the taxonomy of cognitive (dys)function only partially overlapped with syndrome-specific expectations based on the type of epilepsy and total number of experienced (ifetime) Generalized seizures. More relevant in the prediction of cognitive (dys) function was the degree of efficient organisation of efficient organization of brain networks, and patient-specific factors like the degree reserve and socio- economic status Epidemiology Most frequent chronical neurological disease Prevalence: 0,7 – 1% à 100.000 No difference in gender Incidence: 20 tot 70 / 100.000 – Varies strongly with age – Excluding febrile seizures: developing because of fever, and can cause epilepsy further on, Course – Children incidence is higher: 15 years follow up à 71% remission at least 5 years ;12% refractory (no drugs are efficient) – Adults: 20 years follow up à 70% seizure free; refractory 20-30%; average of 20 years RESUMOS ORGANIZADOS 60  More risks for psychosocial problems; – Cognitive – School performances – Behavioural and emotional problems – Social difficulties Methodological bias!! Diagnostic steps Seizure → first aid or general practitioner “..do nothing..” stats epileptic - one person that has a seizure after seizure after seizure, can only stop with medication Neurologist general hospital – Clinical interview, clinical description of seizures – Diagnostic investigations: ElectroEncephaloGraphy (standard, after sleep deprivation, videomonitoring, MEG, et cetera) Neuroimaging techniques (MRI, PET, SPECT, CT, et cetera). Treatment; -Neurologist, general practitioner -Specialized epilepsy centers: medical treatment insufficient psychosocial problems CLASSIFICATION OF EPILEPTIC SEIZURES AND EPILEPSY there are a lot of diagnoses… and its important to provide correct insight into the disease prognosis and possible death risks, as well as the likelihood of the presence of comorbidity classification : 1. seizure types are determined (always 1st, done on the basis of seizure symptoms- observable characteristics) 2. on this base the type of epilepsy is ascertained 3. determined wether the seizures types and the type of epilepsy are characteristic of a specific epilepsy syndrome !!! the inventory of comorbid problems on a neurological, psychiatric, behavioural and neuro-psychological level → can contribute to aetiological diagnosis and tools for guidance and treatment comorbid factors can vary in type and severity, from subtle learning difficulties to intellectual disability and from psychiatric characteristics with