DEMENTE-curs Actualizat STUDENTI Februarie 2014

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<ul><li><p>DIAGNOSTICUL PRIN PRISMA SPECTRULUI CLINICO-BIOLOGIC AL DEMENTELORProf. Dr. Ovidiu Bajenaru</p><p>UMF Carol Davila BucurestiSpitalul Universitar de Urgenta Bucuresti Clinica de Neurologie</p></li><li><p>Dementele provocarea viitoruluiNumrul persoanelor cu vrst peste 65 de ani se va dubla pn n 20301Numrul persoanelor suferind de boala Alzheimer se va dubla pn n 2050*Estimare a numrului de persoane suferind de dementa, 2005-2050*Persoane suferind de boala Alzheimer (milioane)An1. Green Paper on Demographic change, Brussels 2005;12(suppl 1):1-27*Sursa: Datamonitor, publicat Martie 2008</p></li><li><p>Lim, et al. J Am Geriatr Soc. 1999 May;47(5):564-9</p></li><li><p>DEMENTA reprezinta un sindrom clinic neurolgic caracterizat printr-o deteriorare cognitiva globala, care implica un declin fata de nivelul anterior de functionare si care asociaza o gama larga de simptome psihice, psihologice si comportamentale. </p></li><li><p>Tulburarile functiilor cognitive sunt uneori precedate si aproape ntotdeauna nsotite de:</p><p>- tulburari ale controlului emotional- modificari ale personalitatii- alte simptome psihiatrice:* apatie, depresie, tulburari psihotice* tulburari comportamentale</p></li><li><p>ETIOLOGIEdementele = un grup heterogen de afectiuni neurologice primare sau secundare asociate unor boli sistemice cu afectare a sistemului nervos central </p><p>formele cele mai ntlnite:- dementa din boala Alzheimer- dementele vasculare- dementa din -sinucleinopatii * dementele cu corpi Lewy * dementa asociata bolii Parkinson- formele mixte * boala Alzheimer asociata cu boala cerebro-vasculara * boala Alzheimer asociata cu dementa cu corpi Lewy- dementele fronto-temporale</p></li><li><p>Boli in care dementa este asociata cu semne clinice si de laborator ale altor afectiuni medicale:</p><p>A. Infectia HIV / SIDAB. Afectiuni endocrine: hipotiroidism, sd. Cushing, hipopituitarismC. Carente nutritionale: sd. Wernicke-Korsakov, degenerescenta combinata subacuta ( carenta de vit. B12 ), pelagraD. Meningoencefalite cronice: paralizia generala progresiva, sifilisul meningo-vascular, criptococcozaE. Degenerescenta hepato- lenticulara familiala ( b. Wilson ) si dobanditaF. Intoxicatii cronice ( inclusiv statusul dupa intoxicatie cu CO )G. Hipoglicemia sau hipoxia prelungitaH. Encefalita limbica paraneoplazicaI. Expunera la metale grele: As, Bi, Au, Mn, HgJ. Dementa dialitica ( rara in prezent, datorita evolutiei tehnologiilor de dializa )</p></li><li><p> Pacienta cu sd. demential encefalita limbica paraneoplazica asociata cu adenocarcinom pulmonar: debut neurologic inainte de diagnosticul oncologic </p></li><li><p>II. Boli in care dementa este asociata cu alte semne neurologice, dar fara alte afectiuni medicale evidente(1):</p><p>A. Invariabil asociate cu alte semne neurologice:1. Boala Huntington2. Scleroza multipla, boala Schilder, adreno-leucodistrofia si alte boli inrudite care afecteaza mielina SNC3. Lipidozele4. Epilepsia mioclonica5. B. Creutzfeldt- Jacob ( clasica si noua varianta ) b.Gerstmann-Strausler-Scheinker (dementele mioclonice, prionice)6. Degenerescenta cerebro-cerebeloasa7. Degenerescentele cortico-bazale DFT8. Dementa cu paraplegie spastica9. Paralizia supranucleara progresiva ( PSP ) DFT10. Boala Parkinson11. Boala difuza cu corpi Lewy 12. Scleroza laterala amiotrofica &amp; dementaDFT13. Complexul Parkinson-SLA-dementa14. Alte boli metabolice ereditare rare</p></li><li><p>II. Boli in care dementa este asociata cu alte semne neurologice, dar fara alte afectiuni medicale evidente (2):</p><p>B. Adesea asociate cu alte semne neurologice:1. AVC multiple ( ischemice/ hemoragice ) si b. Binswanger2. Tumorile ( primare/ secundare ) sau abcesele cerebrale*3a. Hematoamele intracraniene cronice*3b. Leziuni dupa traumatisme cranio-cerebrale ( de regula tipuri de leziuni insotite de diferite forme de sangerare cerebrala )4. Boala difuza cu corpi Lewy5. Hidrocefaliile comunicante normotensive sau hidrocefaliile obstructive*6. Leucoencefalita multifocala progresiva ( LEMP )7. Boala Marchiafava Bignami8. Granulomatozele si vasculitele cerebrale9. Encefalitele virale</p></li><li><p>III. Boli in care de obicei dementa este singura manifestare evidenta a unei afectiuni neurologice sau medicale:</p><p>A. Boala AlzheimerB. Unele cazuri de SIDAC. Dementele fronto-temporale ( inclusiv sd. afazice primare progresive) si cele de lob frontal F. Boli degenerative nespecificate</p></li><li><p>CRITERII DE DIAGNOSTICDSM IV TR ( nu mai este de actualitate, incepand cu mai 2013):Criteriile diagnosticului de demen, indiferent de cauza:</p><p>1. Dezvoltarea mai multor deficite cognitive, dintre care obligatoriu:Afectarea memoriei (scderea capacitii de a nva informaii noi sau de a evoca informaii nvate anterior)</p><p>si,</p><p>Cel puin una dintre urmtoarele:</p><p>Afazie (tulburare de limbaj)Apraxie (afectarea abilitii de a executa activiti motorii ntr-o anume secven i care servesc unui scop, n lipsa afectrii funciei motorii)Agnozie (incapacitatea de a recunoate sau identifica obiecte n lipsa afectrii funciilor senzoriale)Perturbarea funcionrii excutive (planificare, organizare, secvenializare, abstractizare).</p></li><li><p>CRITERII DE DIAGNOSTIC2. Deficitele cognitive menionate mai sus reprezint un declin fa de nivelul anterior de funcionare i cauzeaz, fiecare, afectarea semnificativ a funcionrii sociale sau ocupaionale</p><p>3. Deficitele cognitive menionate mai sus nu apar exclusiv n cursul unui episod de delirium</p><p>4. Criterii de diagnostic specifice se adaug pentru stabilirea diferitelor etiologii ale demenei</p><p>5. Afectarea memoriei trebuie obligatoriu s fie prezent ns uneori poate s nu fie simptomul predominant ( f-ctie de forma etiopatogenica ! ) va disparea in DSM5</p><p>6. Pentru a se putea stabili diagnosticul de demen, deliriumul* i orice alt tip de tulburare confuzional trebuie exclus prin diagnostic diferenial.</p></li><li><p>NEUROCOGNITIVE DISORDERSA proposal from the DSM-5 Neurocognitive Disorders Work GroupNeurocognitive Disorders - a new category to replace the DSM-IV Category of Delirium, Dementia, Amnestic, and Other Geriatric Cognitive Disorders</p><p>The defining characteristics of these disorders are that their core or primary deficits are in cognition and that these deficits represent a decline from a previously attained level of cognitive functioning</p><p>This section includes three broadly defined syndromes:(1) Delirium(2) Major Neurocognitive Disorder(3) Mild Neurocognitive Disorder</p></li><li><p>Neurocognitive Disorder - new reccomended criteria by APA -Terminology for the cognitive domains has been updated to reflect current usage in neuropsychology and neurology</p><p>Evidence of significant cognitive decline from a previous level of performance in one or more of the domains based on:Complex attention (sustained attention, divided attention, selective attention, processing speed)Executive ability (planning, decision-making, working memory, responding to feedback/error correction, overriding habits, mental flexibility)Learning and memory (immediate memory, recent memory [including free recall, cued recall, and recognition memory])Language (expressive language [including naming, fluency, grammar and syntax] and receptive language)Visuoconstructional-perceptual ability (construction and visual perception)Social cognition (recognition of emotions, theory of mind, behavioral regulation)</p></li><li><p>Major Neurocognitive Disorder </p><p>A. Evidence of significant cognitive decline from a previous level of performance in one or more of the domains outlined above based on:1. Concerns of the patient, a knowledgeable informant or the clinician that there has been a significant decline in cognitive functionAND2. Clear decline in neurocognitive performance, typically 2 or more standard deviations below appropriate norms on formal testing, or equivalent clinical evaluation.</p><p>B. The cognitive deficits are sufficient to interfere with independence (i.e., requiring assistance at a minimum with instrumental ADL [more complex tasks such as paying bills or managing medications]).</p><p>C. The cognitive deficits do not occur exclusively in the context of a delirium.</p><p>D. The cognitive deficits are not wholly or primarily attributable to another Axis I disorder (e.g., Major Depressive Disorder, Schizophrenia)</p></li><li><p>Mild Neurocognitive DisorderA.Evidence of minor cognitive decline from a previous level of performance in one or more of the domains outlined above based on:1. Concerns of the patient, a knowledgeable informant or the clinician that there has been a mild decline in cognitive functionAND2. Mild decline in neurocognitive performance, typically between 1 and 2 standard deviations below appropriate norms on formal testing, or equivalent clinical evaluation.</p><p>B.The cognitive deficits are insufficient to interfere with independence (i.e., instrumental ADL [more complex tasks such as paying bills or managing medications] are preserved), but greater effort, compensatory strategies, or accommodation may be required to maintain independence.</p><p>C. The cognitive deficits do not occur exclusively in the context of a delirium.</p><p>D. The cognitive deficits are not wholly or primarily attributable to another Axis I disorder (e.g., Major Depressive Disorder, Schizophrenia).</p></li><li><p>The distinction between Major and Mild disorders is primarily one of severity, with the threshold for Major Neurocognitive Disorder encompassing a greater degree of cognitive impairment and hence a loss of independence in instrumental activities of daily living</p><p>In most progressive disorders such as the neurodegenerative disorders and some forms of vascular cognitive impairment, Minor and Major may be earlier and later stages of the same disorderS 24 - 35 Major Neurocognitive DisorderS 24 Major Neurocognitive DisorderS 25 Major Neurocognitive Disorder Associated with Alzheimer's DiseaseS 26 Major Neurocognitive Disorder Associated with Vascular DiseaseS27 Major Neurocognitive Disorder Associated with Fronto-Temporal Lobar DegenerationS 28 Major Neurocogntive Disorder Associated with Traumatic Brain InjuryS 29 Major Neurocognitive Disorder Associated with Lewy Body DiseaseS 30 Major Neurocognitive Disorder Associated with Parkinson's DiseaseS 31 Major Neurocognitive Disorder Associated with HIV InfectionS 32 Major Neurocognitive Disorder Associated with Substance UseS 33 Major Neurocognitive Disorder Associated with Huntington's DiseaseS 34 Major Neurocognitive Disorder Associated with Prion DiseaseS 35 Other Specified Major Neurocogntive DisorderS 12 - 23 Mild Neurocognitive DisorderS 12 Mild Neurocognitive DisorderS 13 Mild Neurocognitive Disorder Associated with Alzheimer's DiseaseS 14 Mild Neurocognitive Disorder Associated with Vascular DiseaseS 15 Mild Neurocognitive Disorder Associated with Fronto-Temporal Lobar DegenerationS 16 Mild Neurocognitive Disorder Associated with Traumatic Brain InjuryS 17 Mild Neurocognitive Disorder Associated with Lewy Body DiseaseS 18 Mild Neurocognitive Disorder Associated with Parkinson's DiseaseS 19 Mild Neurocognitive Disorder Associated with HIV InfectionS 20 Mild Neurocognitive Disorder Associated with Substance UseS 21 Mild Neurocognitive Disorder Associated with Huntington's DiseaseS 22 Mild Neurocognitive Disorder Associated with Prion DiseaseS 23 Other Specified Mild Neurocogntive DisorderDSM-5 Neurocognitive Criteria, Draft 1/7/10</p></li><li><p>M E M O R I A</p><p>DECLARATIVA( EXPLICITA )NON-DECLARATIVA / PROCEDURALA( IMPLICITA )FapteEvenimenteCOMPORTAMENTALA(abilitati, obiceiuri)De IDENTIFICARE(DETECTARE)De INVATAREASOCIATIVADE BAZADe INVATARENON-ASOCIATIVARASPUNSURIEMOTIONALERASPUNS MUSCULARSCHELETICHIPOCAMPLOB T MEDIALDIENCEFALSTRIATCORTEX MOTORCEREBELNEOCORTEXSISTEM LIMBICCEREBELCAIREFLEXE</p></li><li><p>MILD COGNITIVE IMPAIRMENT ( MCI )</p><p>Criterii clinice pentru MCI:( Mayo Alzheimer Disease Center, Petersen et al., 1999, modificate in 2001 )</p><p>1. Acuze privind tulburari cognitive (de obicei de memorie), de preferat sesizate si de o persoana din anturaj</p><p>2. Tulburari cognitive (de obicei de memorie) obiective, in raport cu varsta si nivelul de instruire</p><p>3. Functiile cognitive generale intacte in cea mai mare masura</p><p>4. Conservarea in esenta a activitatilor din viata cotidiana</p><p>5. Absenta dementei</p></li><li><p>Domeniu unicDomenii multipleDomeniu unicDomenii multipleMCI amnesticaMCI non-amnesticaClasificare clinicaSUBTIPURI de MCIDegenerativaVascularaPsihiatricaConditii medicaleETIOLOGIE</p><p>B. AlzheimerDepresieB. AlzheimerVaDDepresieDFTDLBVaD</p></li><li><p>Examenul clinic general - obligatoriu- semne care s orienteze ctre diagnosticul unei afeciuni generale care se nsoete de demen ( tumor malign, afeciune metabolic, SIDA, hipotiroidism, anemie sever, etc.)</p><p>Examenul neurologic obligatoriu- poate decela semne neurologice specifice care s orienteze diagnosticul ctre boli neurologice primare care se asociaz cu demen (boala Wilson, boala Creutzfeldt-Jacob, etc.). </p><p>* Ex. : examenul neurologic este foarte important pentru a deosebi o demen de tip Alzheimer de o demen vascular.</p></li><li><p>Examenul psihiatric obligatoriu</p><p> poate depista tulburri non-cognitive: - simptome psihiatrice ( adeseori prezente din primele stadii evolutive ):* depresia * fenomene psihotice* stri confuzionale* episoade obsesive* anxietate* iritabilitate* dezinhibiie* agitaie</p><p> scop: asigurarea unui management optim al bolii</p></li><li><p>Examenul neuropsihologic - obligatoriu n caz de demen usoar sau probabil</p><p>- teste pentru aprecierea deficitului cognitiv - scale specifice pentru evaluarea depresiei (depresia poate mima o demen sau se poate asocia unei demene). </p></li><li><p>Analize de laborator</p><p> - obligatoriu cele uzuale ( hemoleucogram, uree, creatinin, VSH, glicemie, transaminaze,etc.) </p><p> se recomand: ionograma investigarea funciei tiroidiene nivelul seric de vitamin B12 </p><p>- pot fi necesare analize specifice:* teste serologice pentru boli infecioase ( HIV, sifilis, borelioz, encefalita herpetic, etc.)* teste imunologice (diagnosticul vasculitelor, a lupusului sistemic, etc.)* probe toxicologice (intoxicaii cu metale grele)* teste genetice (identificarea bolii Alzheimer precoce familiale, DFT, CADASIL, etc.)* alte determinari (ex. homocistein) * alte teste specifice</p></li><li><p>Examenul lichidului cefalorahidian </p><p>- n cazuri selecionate de diagnostic diferenial</p><p>- in boala Alzheimer: * peptidul A42 are un nivel sczut * proteina tau &amp; proteina tau fosforilata - nivel crescut vs. non-demeni de aceeai vrst ( grad de recomandare de nivel B )</p><p> - in cazul suspiciunii de boal Creutzfeldt-Jakob:* proteina 14-3-3 este important pentru diagnostic ( grad de recomandare de nivel B )</p></li><li><p> Investigaiile neuroimagistice </p><p> - Rolul principal: * excluderea alte patologii cerebrale * sprijinirea diagnosticul tipului de demen neurodegenerativ n boala Alzheimer, atrofia cerebral predominant la nivelul hipocampului i a lobului T n DFT atrofia cerebral predominant la nivelul lobilor F i T n demena vascular: evidenierea leziunilor vasculare i a tipului acestora, etc.</p><p> NB. Sunt ns i situaii n care simptomatologia este clinic ev...</p></li></ul>

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