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