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NEUROBEHAVIOR (BEHAVIORAL NEUROLOGY)
Divisi Neurology Kognitif dan Behavioral Neurology :pendekatan komprehensif multidiscipliner untui diagnosis dan management pasien dengan memory, fungsi eksekutif, atensi, bahasa, emosi, atau tingkah laku akibat penyakit, cedera atau gangguan perkembangan pada SSP.
Asesmen dan tatalaksana pada :
- Dementia (e.g., Alzheimer’s disease dan kelainan degeneratif lain, vascular dementia, frontotemporal dementia, dementia dengan parkinsonism) - Gangguan kognitif, emosi atau tingkah laku akibat bermacam sebab (epilepsy, multiple sclerosis, stroke, traumatic brain injury, brain tumor, kelainan sistemik dengan manifestasi SSP) - Gangguan perkembangan pada dewasa (Attention deficit disorder, gangguan belajar, mental retardation,autism).
Basic brain biologyOtak manusia terdiri atas:- Brain stem, hind brain, mid brain, cerebellum: organ yg paling awal, terhubung dg medula spinalis mengatur pergerakan, pernafasan dan jantung. - Limbic systemHypothalamus, hippocampus fungsi memori dan respon emosi. - Cerebral cortexthe distinguishing feature of human brains
Tahap pembentukan otak
1. Neural tube
2. Proliferasi
3. Migrasi
4. Mielinisasi
5. Sinaptogenesis
3 weeks
4 weeks 7 weeks
3 months 6 months
2 years
• Occipital - visual• Parietal – pergerakan,
orientasi, kalkulasi, pengenalan
• Temporal – suara dan bicara, aspek memori
• Frontal – berfikir, konseptualisasi, rencana.
Right HemisphereLeft Hemisphere
Specialities•Copying of designs, •Discrimination of shapes e.g. picking out a camouflaged object, •Understanding geometric properties, •Reading faces, •Music, •Global holistic processing, •Understanding of metaphors, •Expressing emotions, •Reading emotions.
•Language skills, •Skilled movement, •Analytical time sequence processing.
Shared•Sensations on both side of face, •Sound perceived by both ears, •Pain, •Hunger, •Position.
Emotions Negative emotions (fearful mournful feelings), Positive emotions
neurotransmitters Higher levels of norepinephrine Higher levels of dopamine
Grey Matter White Maatter ratio
More white-matter (longer axons) on rightmore grey-matter (cell bodies) on the left
Macam-macam ggn perkembangan :
1. Disfasia
2. Disleksia
3. Diskalkulia
4. Disgrafia
5. Dispraksia
6. GPPH
7. Autisme
1-6 Disebut sebagai Learning disability
Disfasia:Disfasia:
Tidak / terlambat bicaraTidak / terlambat bicara
12 bln ; ma…., pa….12 bln ; ma…., pa….
18 bln ; mama, papa18 bln ; mama, papa
24 bln ; mama makan24 bln ; mama makan
DISLEKSIA : kesulitan membacaDISLEKSIA : kesulitan membaca
VISUAL DYSLEXIAVISUAL DYSLEXIA
- ggn memori berat- ggn memori berat - tdk dpt membaca huruf atau baca huruf demi - tdk dpt membaca huruf atau baca huruf demi
hurufhuruf - terbalik huruf yang mirip : b –d, p –q.- terbalik huruf yang mirip : b –d, p –q.
DISCONNECTION DYSLEXIADISCONNECTION DYSLEXIA - - peralihan huruf atau kombinasi huruf ke bahasa, peralihan huruf atau kombinasi huruf ke bahasa,
buta buta huruf atau buta kata.huruf atau buta kata.
DISPHONEMIC DYSLEXIADISPHONEMIC DYSLEXIA - - ggn persepsi bunyi, krn ada huruf yg hilangggn persepsi bunyi, krn ada huruf yg hilang
VERBAL DYSLEXIAVERBAL DYSLEXIA - - bicara tdk lancarbicara tdk lancar
DISKALKULIA : kesulitan berhitungDISKALKULIA : kesulitan berhitung
1. Ideognostic dyscalculia1. Ideognostic dyscalculiaggn pemahaman abstraksi angkaggn pemahaman abstraksi angka7 berarti mewakili sesuatu berjumlah tujuh7 berarti mewakili sesuatu berjumlah tujuh
2.2. Verbal dyscalculiaVerbal dyscalculiaggn mengerjakan soal hitungan dengan kalimatggn mengerjakan soal hitungan dengan kalimat
3.3. Dysortographic dyscalculiaDysortographic dyscalculiaggn pemahaman tanda aritmatika, tanda + , - , : ggn pemahaman tanda aritmatika, tanda + , - , : , x, x
4. 4. Operational dyscalculiaOperational dyscalculiapemahaman aritmatika baik tapi ggn pemahaman aritmatika baik tapi ggn penghitungan yg komplekspenghitungan yg kompleks
DisgraphiaDisgraphia
Gangguan menulisGangguan menulis
Gangguan menggambarGangguan menggambar
DISPRAKSIADISPRAKSIA
1. Dyspraxia Ideomotoric1. Dyspraxia Ideomotoricggn praksis sederhana, kurang luwes; gsk gigi, makan pakai ggn praksis sederhana, kurang luwes; gsk gigi, makan pakai sendok, menggunting dllsendok, menggunting dll
22. Dyspraxia Ideasional. Dyspraxia Ideasionalggn dlm urut-urutan melakukan gerakan, bingung untuk mengawali ggn dlm urut-urutan melakukan gerakan, bingung untuk mengawali suatu aktifitassuatu aktifitas
3. 3. Dyspraxia KonstructionalDyspraxia Konstructionalggn gerakan kompleks yg berkaitan dgn bentuk, nyusun balok, ggn gerakan kompleks yg berkaitan dgn bentuk, nyusun balok, menggambar dll.menggambar dll.
4. 4. Dyspraxia oralDyspraxia oralggn gerakan motorik di mulut; ggn berbicara ( disartria)ggn gerakan motorik di mulut; ggn berbicara ( disartria)
GPPH / ADHDGPPH / ADHD
Perhatian : tidak bs konsentrasi, mudah Perhatian : tidak bs konsentrasi, mudah terpecah perhatiannya, bosan.terpecah perhatiannya, bosan.
Hiperaktif : tidak bisa diam, lari kesana Hiperaktif : tidak bisa diam, lari kesana kemari, kemari,
DSM III mendefinisikan GPPH dengan 3 DSM III mendefinisikan GPPH dengan 3 gejala utama :gejala utama :
– InatensiInatensi– ImpulsifitasImpulsifitas– HiperaktifHiperaktif
2 jenis GPPH : 2 jenis GPPH :
1. defisit atensi dan hiperaktifitas, ketiga 1. defisit atensi dan hiperaktifitas, ketiga gejala ada.gejala ada.
2. defisit atensi dan impulsifitas tanpa 2. defisit atensi dan impulsifitas tanpa hiperaktif hiperaktif
Atensi : kemampuan untuk memfokus Atensi : kemampuan untuk memfokus perhatian pada masalah yg dihadapiperhatian pada masalah yg dihadapi
atensi ada 2 aspek ;atensi ada 2 aspek ;
1. seleksi : lob. Frontal dan sirkit limbik1. seleksi : lob. Frontal dan sirkit limbik
2. sadar : R.A.S , talamik dan cortek 2. sadar : R.A.S , talamik dan cortek
DEDEMENTIAMENTIA– Sekumpulan gejala yg bisa disebabkan oleh Sekumpulan gejala yg bisa disebabkan oleh
60-7060-70 penyakit. penyakit.– Marked by progressive, irreversible declines Marked by progressive, irreversible declines
in in memory. memory.
visual-spatial relationshipsvisual-spatial relationships
performance of routine tasksperformance of routine tasks
language and communication skillslanguage and communication skills
abstract thinkingabstract thinking
ability to learn and carry out mathematical ability to learn and carry out mathematical calculations.calculations.
DementiaDementia
Reversible:Reversible:– D= D= Drugs, DeliriumDrugs, Delirium– E=E= Emotions (such as depression) and Emotions (such as depression) and
Endocrine DisordersEndocrine Disorders– M=M= Metabolic DisturbancesMetabolic Disturbances– E=E= Eye and Ear ImpairmentsEye and Ear Impairments– N=N= Nutritional DisordersNutritional Disorders– T=T= Tumors, Toxicity, Trauma to HeadTumors, Toxicity, Trauma to Head– I=I= Infectious DisordersInfectious Disorders– AA Alcohol, Arteriosclerosis Alcohol, Arteriosclerosis (Dick-Mulheke- Overview of (Dick-Mulheke- Overview of
Alzheimer's Disease)Alzheimer's Disease)
DementiaDementiaIrreversible:Irreversible:– Alzheimer’sAlzheimer’s– Lewy Body DementiaLewy Body Dementia– Pick’s Disease (Frontotemperal Dementia)Pick’s Disease (Frontotemperal Dementia)– Parkinson’sParkinson’s– Heady InjuryHeady Injury– Huntington’s DiseaseHuntington’s Disease– Jacob-Cruzefeldt DiseaseJacob-Cruzefeldt Disease
ETIOLOGIETIOLOGIPenyakit AlzheimerPenyakit Alzheimer 50%50%Demensia VaskulerDemensia Vaskuler 10%10%PseudodemensiaPseudodemensia 8% 8%Demensia alkoholikDemensia alkoholik 7% 7%Tumor intrakranialTumor intrakranial 5% 5%NPHNPH 5% 5%IntoksikasiIntoksikasi 3% 3%HuntingtonHuntington 2% 2%Penyakit lainPenyakit lain 10%10%
Adams RD, 1997Adams RD, 1997
Baldereschi et al., 1998
Progression of normal aging to dementiaProgression of normal aging to dementia
NormalNormal Brain AgingBrain AgingCognitionCognition
stable orstable orProdromal Prodromal reversrevers MCI MCI reversible reversibleDementiaDementia impairment impairment
otherother Alzheimer’s Alzheimer’s vascular vascularDementia dementiaDementia dementia diseasedisease dementia dementia
Golomb J,Kluger A,Ferris SH, 1999Golomb J,Kluger A,Ferris SH, 1999
MCI / VCIstable or reversible
impairment
other dementia
Alzheimerdementia
vasculardementia
•Alzheimer's DiseaseAlzheimer's Disease4,000,000 4,000,000 orang di orang di U.S.U.S.Perubahan pada Alzheimer’s Disease:Perubahan pada Alzheimer’s Disease:
Diminished blood flowDiminished blood flow
Neurofibrillary TanglesNeurofibrillary Tangles
Neuritic PlaquesNeuritic Plaques
Degeneration of hippocampus, cerebral Degeneration of hippocampus, cerebral cortex, hypothalamus, and brain stemcortex, hypothalamus, and brain stem
Neurofibrillary TanglesNeurofibrillary TanglesIntracellular inclusion bodiesIntracellular inclusion bodies: filamen : filamen helical berpasangan helical berpasangan double-helix.double-helix.HHyperphosphorylated microtubuleyperphosphorylated microtubule yg yg berhub,. Dg protein, disebut berhub,. Dg protein, disebut tautau. . Kerusakan nKerusakan neuronal microtubuleseuronal microtubules..
Amyloid Plaques Amyloid Plaques
Struktur Struktur extracellular extracellular di di hippocampus hippocampus dan dan neocortex.neocortex.
Padat dan Padat dan insoluble structures.insoluble structures.
Inti Inti beta-amyloid protein beta-amyloid protein yg dikelilingi yg dikelilingi axons axons dan dan dendritdendrit abnormal abnormal..
Teori Etiologi Teori Etiologi Alzheimer'sAlzheimer'sPerubahan Neurotransmitter: Perubahan Neurotransmitter: Acetycholine Acetycholine ↓↓,penting untk fungsi kognitif,penting untk fungsi kognitif..
Perubahan Sintesis Protein: Perubahan Sintesis Protein: Beta amyloidBeta amyloid (penyebab plaque), (penyebab plaque), TauTau((neurofibrillaryneurofibrillary tanglestangles))..
Teori Genetik: Teori Genetik: ApoE4 ApoE4 pdpd chromosone 19 chromosone 19: : late-late-onsetonset, , Chromosome 21Chromosome 21: : early-onset.early-onset.
Teori Metabolik: Berkurangnya metabolisme Teori Metabolik: Berkurangnya metabolisme glukosa scr dramatisglukosa scr dramatis..
Teori Teori CalciumCalcium: terlalu banyak kalsium : terlalu banyak kalsium membunuh sel membunuh sel neuron mati neuron mati
Theories Regarding Causes of Theories Regarding Causes of Alzheimer'sAlzheimer's
EnvironmentalEnvironmental: : AluminumAluminum, , Zinc—Zinc—pada otak pada otak saat autopsisaat autopsi..
ViralViralTrauma Kepala: meningkatkan konsentrasi Trauma Kepala: meningkatkan konsentrasi B-amyloid B-amyloid proteinprotein
Edukasi Rendah Edukasi Rendah kurang mampu kurang mampu mengkompensasi defisit kognitifmengkompensasi defisit kognitif
Defisiensi Defisiensi EstrogenEstrogen
Early Life Experience---Early Life Experience---kehilangan ortu sebelum 16 kehilangan ortu sebelum 16 tahuntahun
Gauthier at all, 1997
Diagnosis Alzheimer’sDiagnosis Alzheimer’s DementiaDementia Memory ImpairmentMemory Impairment
Multiple cognitive deficits :Multiple cognitive deficits :– AphasiaAphasia::
– Expressive—Expressive—tidak mampu mengucapkan kata2tidak mampu mengucapkan kata2
– Receptive—Receptive—tidak memahami bahasa lisan maupun tulisantidak memahami bahasa lisan maupun tulisan
– Apraxia—Apraxia—tidak mampu melakukan gerakan tidak mampu melakukan gerakan kompleks, atau tidak mampu melakukan aktivitas kompleks, atau tidak mampu melakukan aktivitas dibawah perintah.dibawah perintah.
– Agnosia---Agnosia---tidak mampu mengenali objek dengan tidak mampu mengenali objek dengan melihat, menyentuh, merasa, mencium atau melihat, menyentuh, merasa, mencium atau mendengarmendengar
Diagnostic TestsDiagnostic Tests
Neurological ExamNeurological Exam
Brain Imaging—shrinkage, atrophy of Brain Imaging—shrinkage, atrophy of brain (CT or MRI)brain (CT or MRI)
Blood WorkBlood Work
Median Scores on Mini-Mental State ExaminationMedian Scores on Mini-Mental State Examination by Age and Educational Levelby Age and Educational Level
Age (years)Age (years)Educational levelEducational level
44thth grade grade 88thth grade grade High schoolHigh school CollegeCollege
18 to 2418 to 24 2222 2727 2929 2929
25 to 2925 to 29 2525 2727 2929 2929
30 to 3430 to 34 2525 2626 2929 2929
35 to 3935 to 39 2323 2626 2828 2929
40 to 4440 to 44 2323 2727 2828 2929
45 to 4945 to 49 2323 2626 2828 2929
50 to 5450 to 54 2323 2727 2828 2929
55 to 5955 to 59 2323 2626 2828 2929
60 to 6460 to 64 2323 2626 2828 2929
65 to 6965 to 69 2222 2626 2828 2929
70 to 7470 to 74 2222 2525 2727 2828
75 to 7975 to 79 2121 2525 2727 2828
80 to 8480 to 84 2020 2525 2525 2727
8484 1919 2323 2626 2727
Reprinted with permisssion from Crum RM, Anthony JC, Basset SS, Folstein MF. Population-based norms for the mini-mental state examination by age and educational level. JAMA 1993 ; 18 : 2386-91
Stages of Alzheimer’s DiseaseStages of Alzheimer’s Disease
Mild StageMild Stage– Memory LossMemory Loss– Symptoms:Symptoms:
Confusion About PlaceConfusion About Place
Loss of SpontaneityLoss of Spontaneity
Loss of InitiativeLoss of Initiative
Mood/Personality ChangesMood/Personality Changes
Poor JudgmentPoor Judgment
Takes Longer to Perform Routine choresTakes Longer to Perform Routine chores
Trouble Handling Money, Paying BillsTrouble Handling Money, Paying Bills
Stages of Alzheimer’s DiseaseStages of Alzheimer’s Disease
Moderate StageModerate StageImpairments in:Impairments in:
languagelanguage
motor abilitymotor ability
object recognitionobject recognition
increasing memory loss and confusionincreasing memory loss and confusion
Stages of Alzheimer’s DiseaseStages of Alzheimer’s DiseaseModerate StageModerate Stage– Symptoms:Symptoms:
PProblems recognizing family members, close friends.roblems recognizing family members, close friends.
Repetitive statements and/or movements.Repetitive statements and/or movements.
Restless, especially in late afternoon and at night.Restless, especially in late afternoon and at night.
Occasional muscle twitches or jerking.Occasional muscle twitches or jerking.
Perceptual motor problems.Perceptual motor problems.
Problems organizing thoughts, thinking logically.Problems organizing thoughts, thinking logically.
Can’t find right words, makes up stories.Can’t find right words, makes up stories.
Problems reading and writing.Problems reading and writing.
May be suspicious, irritable, fidgety, teary or silly.May be suspicious, irritable, fidgety, teary or silly.
Stages of Alzheimer’s DiseaseStages of Alzheimer’s Disease
Severe StageSevere Stage– Symptoms:Symptoms:
Loses weight even with good diet.Loses weight even with good diet.
Little capacity for self-care.Little capacity for self-care.
Can’t communicate with words.Can’t communicate with words.
May put everything in mouth or touch everything.May put everything in mouth or touch everything.
Can’t control bladder or bowel.Can’t control bladder or bowel.
May have difficult with seizures, swallowing, skin May have difficult with seizures, swallowing, skin breakdown, infections.breakdown, infections.
Stages of Alzheimer’s DiseaseStages of Alzheimer’s DiseaseTerminal StageTerminal Stage– Symptoms:Symptoms:
Loss of ability to ambulate.Loss of ability to ambulate.
Loss of ability to sit.Loss of ability to sit.
Loss of ability to smile.Loss of ability to smile.
Loss of ability to hold up head.Loss of ability to hold up head.
Loss of ability to swallow. Loss of ability to swallow.
Stages of Alzheimer’s DiseaseStages of Alzheimer’s DiseaseStage IV--Terminal StageStage IV--Terminal Stage– Symptoms:Symptoms:
Loss of ability to ambulate.Loss of ability to ambulate.Loss of ability to sit.Loss of ability to sit.Loss of ability to smile.Loss of ability to smile.Loss of ability to hold up head.Loss of ability to hold up head.Loss of ability to swallow. Loss of ability to swallow.
Management of Challenging Behaviors in Dementia—Mahoney, Volicer, Hurley. Management of Challenging Behaviors in Dementia—Mahoney, Volicer, Hurley.
Health Professionals Press:2000. Baltimore, MdHealth Professionals Press:2000. Baltimore, Md..
Potential agents that can be of Potential agents that can be of benefit for Alzheimer’s diseasebenefit for Alzheimer’s disease
Reversible inhibitors of the enzyme Reversible inhibitors of the enzyme acetylcholinesterase (donepezil, tacrine, acetylcholinesterase (donepezil, tacrine, rivastigmine)rivastigmine)?Vitamin E 2000 IU units per day - 6 ?Vitamin E 2000 IU units per day - 6 month delay in disease progressionmonth delay in disease progression?Selegiline 5 mg twice a day - 4 month ?Selegiline 5 mg twice a day - 4 month delay in disease progressiondelay in disease progression?Gingko biloba – 40 mg tid?Gingko biloba – 40 mg tid