TOPICS IN DE KLINISCHE
NEUROPSYCHOLOGIE
Samenvatting
2022-2023
,Inhoud
1 Neuropsychologische symptomatologie ................................................................................................................... 9
1.1 Visuele waarneming.......................................................................................................................................... 9
1.1.1 Stoornissen in de visuele waarneming ...................................................................................................... 9
1. Visueel-velddefecten ......................................................................................................................................... 9
A. Casus met kwadrantanopsie en blindsight ............................................................................................. 10
B. Casus met corticale blindheid en blindsight ........................................................................................... 10
C. Andere basale visusstoornissen .............................................................................................................. 10
2. Lagere orde visuele stoornissen ...................................................................................................................... 10
A. cerebrale achromatopsie (V4 letsel) ....................................................................................................... 10
B. Cerebrale akinetopsie (bilaterale V5 letsel ............................................................................................. 10
3. Hogere orde visuele stoornissen/visuele agnosie ............................................................................................ 11
A. apperceptief versus associatief............................................................................................................... 11
Varianten hogere orde visuele perceptie ............................................................................................................. 12
A. Kleurenagnosie........................................................................................................................................ 12
B. Helderheidsagnosie................................................................................................................................. 12
C. Objectagnosie ......................................................................................................................................... 12
D. Optische afasie of objectanomie ............................................................................................................ 12
E. Pure alexie ............................................................................................................................................... 12
F. Prosopagnosie ......................................................................................................................................... 12
1.1.2 Visuele hallucinaties en illusies .............................................................................................................. 13
1.2 Ruimtelijke cognitie ........................................................................................................................................ 13
1.2.1 Stoornis in de ruimtelijke cognitie .......................................................................................................... 13
1. Perceptie .......................................................................................................................................................... 13
2. Aandacht ......................................................................................................................................................... 13
A. Simultaan-agnosie ................................................................................................................................... 13
B. Hemispatiaal neglect ............................................................................................................................... 14
3. Geheugen ........................................................................................................................................................ 16
A. Topografische desoriëntatie ................................................................................................................... 16
B. Topografische agnosie of landmark-agnosie .......................................................................................... 16
C. Topografische amnesia ........................................................................................................................... 16
D. links-rechts verwarring............................................................................................................................ 16
4. Praxis............................................................................................................................................................... 16
A. visuoconstructieve apraxie ..................................................................................................................... 16
B. optische ataxie ........................................................................................................................................ 17
1.3 Geheugen ........................................................................................................................................................ 17
1.3.1 Geheugenklachten ................................................................................................................................... 17
1.3.2 Stroonissen in het werkgeheugen ............................................................................................................ 17
1.3.3 Het amnestisch syndroom ....................................................................................................................... 17
1
, 1.3.4 Andere geheugen stoornissen.................................................................................................................. 18
Posttraumatische amnesie ................................................................................................................................... 18
Transient Global Amnesia (TGA) ....................................................................................................................... 18
Psychogene amnesie of fugue ............................................................................................................................. 18
andere selectieve gehuegenstoornissen ............................................................................................................... 18
1.4 Taal ................................................................................................................................................................. 19
1.4.1 Afasie ...................................................................................................................................................... 19
Kenmerken van afasie ......................................................................................................................................... 19
A. Woordvindingsproblemen en de productie van parafasieën ................................................................. 19
B. Stoornissen in de vloeiendheid ............................................................................................................... 20
C. Problemen met de zinsbouw .................................................................................................................. 20
D. Stoornissen in het auditief taalbegrip ..................................................................................................... 20
E. Stoornissen in het naspreken.................................................................................................................. 20
F. Stoornissen in het lezen en het schrijven ............................................................................................... 20
Classificatie van afasiesyndromen ...................................................................................................................... 21
A. Broca afasie ............................................................................................................................................. 21
B. Wernicke afasie ....................................................................................................................................... 21
C. Conductie afasie ...................................................................................................................................... 21
D. Globale afasie .......................................................................................................................................... 21
E. Transcorticale afasie ............................................................................................................................... 22
F. Amnestische afasie ................................................................................................................................. 22
1.4.2 Verbale dyspraxie en dysartrie ................................................................................................................ 22
1.4.3 Andere taal-en spraakstoornissen bij kinderen ....................................................................................... 22
1.5 Aandacht en executieve functies ..................................................................................................................... 23
1.5.1 Stoornissen in de aandacht ...................................................................................................................... 23
A. vertraagde informatieverwerking ........................................................................................................... 23
B. stoornissen in de selectiviteit van de aandacht ...................................................................................... 23
C. stoornissen in de intensiteit van de aandacht ........................................................................................ 23
1.5.2 stoornissen in de executieve functies ...................................................................................................... 23
dysexecutief syndroom ....................................................................................................................................... 24
apatisch of amotivationeel syndroom.................................................................................................................. 24
pseudopsychopatisch syndroom .......................................................................................................................... 24
1.6 Emoties en sociale cognitie ............................................................................................................................. 24
1.6.1 Perceptie van sociaal relevante informatie .............................................................................................. 24
1.6.2 interpreteren van sociale situaties ........................................................................................................... 24
1.6.3 reguleren van eigen gedrag ..................................................................................................................... 25
1.6.4 casus R .................................................................................................................................................... 25
1.7 Actie en motoriek ............................................................................................................................................ 25
1.7.1 Hemiplegie en hemiparese ...................................................................................................................... 25
2
, 1.7.2 Cerebrale parese ...................................................................................................................................... 25
1.7.3 Developmental Coordination disorder .................................................................................................... 26
1.7.4 Apraxie .................................................................................................................................................... 26
Ideomotorische apraxie ....................................................................................................................................... 26
Ideationele apraxie .............................................................................................................................................. 26
1.7.5 Alien hand syndrome .............................................................................................................................. 27
1.7.6 Samenvatting........................................................................................................................................... 27
2 Diagnostiek (werkcollege) ...................................................................................................................................... 27
2.1 Les opname ..................................................................................................................................................... 27
2.1.1 Inleiding .................................................................................................................................................. 27
2.1.2 neuropsychologisch diagnostisch process ............................................................................................... 28
1. Vraagstelling ................................................................................................................................................... 28
D. Aanvraag voor neuropsychologisch onderzoek ...................................................................................... 28
2.Afname ............................................................................................................................................................. 29
A. factoren die testselectie beïnvloeden: onderzoeksvraag ....................................................................... 29
B. Factoren die de testselectie beïnvloeden: Patiëntenkenmerken ........................................................... 30
C. Factoren die de testselectie beïnvloeden: Psychometrische kenmerken van de test ........................... 30
D. Factoren die de testselectie beïnvloeden: Efficiëntie en inhoudsvaliditeit ............................................ 30
E. Factoren die de testselectie beïnvloeden: Praktische omstandigheden ................................................ 30
F. Testselectie ............................................................................................................................................. 31
3.Interpretatie ...................................................................................................................................................... 31
A. Testscore normaal of afwijkend: Vergelijkingsstandaard ....................................................................... 31
B. Validiteit van de testresultaten:.............................................................................................................. 31
3 Prematuriteit ............................................................................................................................................................ 32
3.1 Introduction ..................................................................................................................................................... 32
Small for gestational age ..................................................................................................................................... 33
3.2 Health conditions of the preterm infant .......................................................................................................... 34
3.2.1 Hyaline Membrane Disease – BronchoPulmonary Dysplasia ................................................................ 34
3.2.2 Retinopathy Of Prematurity .................................................................................................................... 35
3.2.3 Infections................................................................................................................................................. 35
3.2.4 Periventricular white matter disease/Periventricular leukomalacia ........................................................ 36
3.2.5 Intraventricular hemorrhage .................................................................................................................... 36
Posthemorrhagic hydrocephalus ......................................................................................................................... 37
Treatment of IVH and PHH ................................................................................................................................ 37
3.3 Follow-Up of children born preterm ............................................................................................................... 38
3.4 Developmental problems ................................................................................................................................ 38
3.4.1 In general................................................................................................................................................. 38
3.4.2 Predictive factors..................................................................................................................................... 39
Head circumference ............................................................................................................................................ 39
3
, Neuroimaging ..................................................................................................................................................... 39
Prognostic factors cerebral palsy and motor impairment .................................................................................... 39
3.5 Preterms and development domains................................................................................................................ 40
3.5.1 Motor Development ................................................................................................................................ 40
A. Cerebral Palsy (CP) .................................................................................................................................. 40
B. Developmental coordination disorder (DCD ) ........................................................................................ 40
Visual problems .................................................................................................................................................. 41
A. Ocular problems ...................................................................................................................................... 41
B. Visual perceptual problems .................................................................................................................... 41
3.5.2 Cognitive development ........................................................................................................................... 41
A. executive dysfunction ............................................................................................................................. 41
3.5.3 Behavioral problems ............................................................................................................................... 43
A. ASD .......................................................................................................................................................... 43
B. ADHD ....................................................................................................................................................... 43
C. Psychiatric disorders ............................................................................................................................... 43
3.6 Late preterms................................................................................................................................................... 44
3.6.1 Does early intervention make a difference? ............................................................................................ 44
4 Cerebrale parese (hersenverlamming)..................................................................................................................... 45
4.1 Definitie .......................................................................................................................................................... 45
4.2 Incidentie/prevalentie ...................................................................................................................................... 45
4.3 Classificatie ..................................................................................................................................................... 46
4.3.1 Naar topografie (aangetaste ledematen) .................................................................................................. 46
Topografie US ..................................................................................................................................................... 46
4.3.2 Naar klinisch beeld van de neuromotore dysfunctie ............................................................................... 47
Neurologische niveau.......................................................................................................................................... 47
A. Spastische CP........................................................................................................................................... 47
B. Dyskinesie ............................................................................................................................................... 47
C. Atactische CP........................................................................................................................................... 48
4.3.3 Naar ernst van de motorische stoornis .................................................................................................... 48
GMFCS E & R .................................................................................................................................................... 48
MACS: manual ability classification .................................................................................................................. 49
CFCS: Communication Function Classification System .................................................................................... 49
EDACS: eating and drinking classification system ............................................................................................ 50
VFCS: Visual function classification scale ......................................................................................................... 50
4.3.4 Naar timing van het letsel ....................................................................................................................... 50
4.4 Oorzaken ......................................................................................................................................................... 51
4.4.1 Prenatale oorzaken .................................................................................................................................. 51
4.4.2 Pre/perinatale oorzaken Prematuriteit ..................................................................................................... 51
4.4.3 Perinatale oorzaken van CP .................................................................................................................... 51
4
, 4.4.4 Postnataal ................................................................................................................................................ 52
4.5 Comorbiditeit .................................................................................................................................................. 52
4.5.1 Intellectuele mogelijkheden .................................................................................................................... 53
4.5.2 Voeding ................................................................................................................................................... 54
4.5.3 Epilepsie .................................................................................................................................................. 55
4.6 Behandeling .................................................................................................................................................... 55
4.6.1 Behandeling van primaire problemen neurologisch................................................................................ 55
4.6.2 Managment of spasticity ......................................................................................................................... 55
Orale medicatie ................................................................................................................................................... 55
Botuline toxine .................................................................................................................................................... 56
Intrathecale Baclofen pomp ................................................................................................................................ 56
Selective Dorsal Rhizotomy................................................................................................................................ 56
4.6.3 Orthopedische heelkunde ........................................................................................................................ 57
5 Niet-aangeboren hersenletsel (werkcollege) ........................................................................................................... 58
5.1 Beroerte/cerebrovasculair accident (CVA) ..................................................................................................... 58
5.1.1 Ischemisch hersenfarct ............................................................................................................................ 58
5.1.2 hersenbloeding ........................................................................................................................................ 59
5.1.3 neuropsychologische gevolgen ............................................................................................................... 59
5.1.4 Beroerte in de hersenslagaders ................................................................................................................ 60
Beroerte in de voorste hersenslagader arteria cerebri anterior (ACA) ................................................................ 60
Beroerte in de linker middelste hersenslagader arteria cerebri media (MCA) .................................................... 60
Beroerte in de rechter middelste hersenslagader arteria cerebri media (MCA) .................................................. 60
Unilaterale beroerte in de achterste hersenslagader arteria cerebri posterior (PCA) .......................................... 60
Bilaterale beroerte in de achterste hersenslagader arteria cerebri posterior (PCA) ............................................ 61
5.1.5 Andere syndromen .................................................................................................................................. 61
5.1.6 Andere gevolgen met impact op revalidatie............................................................................................ 61
5.1.7 Multidisciplinair team ............................................................................................................................. 61
5.2 Traumatische hersenletsel ............................................................................................................................... 61
5.2.1 Klinisch beeld ......................................................................................................................................... 62
5.2.2 Primaire schade na een TBI .................................................................................................................... 63
Diffuus axonaal letsel (DAI) ............................................................................................................................... 63
Coup-contrecoupletsel ........................................................................................................................................ 63
5.2.3 Secundaire schade na een TBI ................................................................................................................ 63
5.2.4 Neuropsychologische gevolgen .............................................................................................................. 64
Middelzwaar tot ernstig hersenletsel ................................................................................................................... 64
Licht traumatisch hersenletsel ............................................................................................................................. 65
Whiplash-gerelateerde stoornissen...................................................................................................................... 65
6 Cerebrale visuele inperking .................................................................................................................................... 66
6.1 Definitions....................................................................................................................................................... 66
6.2 Prevalence and causes of visual impairment ................................................................................................... 66
5
, 6.3 Impact on development ................................................................................................................................... 66
6.3.1 Impact on motor function ........................................................................................................................ 66
6.3.2 Impact on other developmental domains ................................................................................................ 68
6.4 CVI .................................................................................................................................................................. 68
6.4.1 Definition and pathophysiology .............................................................................................................. 68
Visual pathway and lesions ................................................................................................................................. 68
Visual field impaired: visual fields ..................................................................................................................... 69
A. Hemianopia: lack of vision on one side................................................................................................... 69
B. Lower visual field impairment................................................................................................................. 69
C. Central visual field impairment ............................................................................................................... 69
6.4.2 Screening for CVI ................................................................................................................................... 70
Visual limitations caused by cognitive visual dysfunction ................................................................................. 70
Causes of CVI ..................................................................................................................................................... 71
6.4.3 Assessment of CVI.................................................................................................................................. 72
Cognition............................................................................................................................................................. 73
How to adapt the environment of the child ......................................................................................................... 77
1.1.1.1 Neuropsychology ................................................................................................................................ 79
1.1.1.2 Visuomotor.......................................................................................................................................... 79
6.5 Treatment ........................................................................................................................................................ 79
6.5.1 1. Physiotherapy ...................................................................................................................................... 79
6.5.2 2. Education/health care .......................................................................................................................... 79
7 Autisme Spectrum Stoornis .................................................................................................................................... 79
7.1 Definitie .......................................................................................................................................................... 79
7.2 Epidemiology .................................................................................................................................................. 79
7.3 Neurobiology .................................................................................................................................................. 79
7.3.1 Head circumference: macrocrania........................................................................................................... 79
7.3.2 ASD: brain structure and function across the lifespan ............................................................................ 80
7.4 Medical problems............................................................................................................................................ 80
7.4.1 Clinical history & General pediatric and neurological examination ....................................................... 80
Physical examination .......................................................................................................................................... 81
Genetics............................................................................................................................................................... 81
A. Fragile X syndroom.................................................................................................................................. 83
B. Williams syndroom ................................................................................................................................. 83
C. Velocardiofaciaal syndroom.................................................................................................................... 83
D. Foetaal alcohol syndroom ....................................................................................................................... 83
E. Angelman syndroom/ Happy puppet...................................................................................................... 84
F. Tubereuze sclerose (huid en hersenen) .................................................................................................. 84
Epilepsy............................................................................................................................................................... 84
Sleep problems .................................................................................................................................................... 85
6
, A. Sleep Hygiene: Sleep Environment ......................................................................................................... 86
B. Treatment of sleep problems: Melatonin ............................................................................................... 86
C. Causes of Insomnia: Neurobiological ...................................................................................................... 86
D. Causes of Insomnia Behavioral/Emotional ............................................................................................. 86
E. Causes of Insomnia Medical/physical ..................................................................................................... 87
Feeding problems in ASD ................................................................................................................................... 87
Treatment ....................................................................................................................................................... 87
Probleem van betekenisverlening & sociale communicatie ........................................................................... 88
Gastro-intestinal problems .................................................................................................................................. 89
Voiding problems................................................................................................................................................ 89
Dentals problems................................................................................................................................................. 89
Behavioural problems other than ASD ............................................................................................................... 89
7.5 Motor evaluation ............................................................................................................................................. 90
8 Neuromusculaire aandoeningen .............................................................................................................................. 90
8.1 Introductie ....................................................................................................................................................... 90
8.2 Neuromuscular disorders ................................................................................................................................ 91
8.2.1 Anterior horn cell (motor neuron) ........................................................................................................... 91
Spinal muscular atrophy (SMA) ......................................................................................................................... 91
8.2.2 peripheral nerve....................................................................................................................................... 92
Charcot-Marie-Tooth (CMT) .............................................................................................................................. 92
Guillain-Barré syndrome (GBS) ......................................................................................................................... 92
8.2.3 neuromuscular junction ........................................................................................................................... 93
myasthenia gravis (MG)...................................................................................................................................... 93
congenital myasthenia syndrome (CMS) ............................................................................................................ 93
8.2.4 Muscle ..................................................................................................................................................... 93
Duchenne muscular dystrophy (DMD) ............................................................................................................... 93
Myotonic dystrophy type 1 (Steinert’s disease) .................................................................................................. 95
8.3 Brein en gedrag bij jongens met Duchenne Musculaire Dystrofie ................................................................. 99
8.4 Neuromusculaire aandoeningen .................................................................................................................... 100
9 Epilepsie ................................................................................................................................................................ 100
9.1 Definition and introduction ........................................................................................................................... 100
9.2 Diagnosis of epilepsy .................................................................................................................................... 102
9.2.1 Seizure description ................................................................................................................................ 102
9.2.2 Seizure type ........................................................................................................................................... 102
9.2.3 Epilepsy syndrome ................................................................................................................................ 103
West syndrome.................................................................................................................................................. 103
Lennox Gastaut Syndrome ................................................................................................................................ 104
Landau-Kleffner syndrome ............................................................................................................................... 104
9.3 Childhood and epilepsy................................................................................................................................. 104
7
, 9.3.1 Childhood Absence Epilepsy ................................................................................................................ 104
9.3.2 Benign Epilepsy with Centrotemporal Spikes ...................................................................................... 105
9.3.3 Epilepsy and cerebral palsy................................................................................................................... 105
9.4 Diagnoses and brainscans ............................................................................................................................. 105
9.4.1 Diagnosis of epilepsy ............................................................................................................................ 106
9.5 Treatment ...................................................................................................................................................... 107
9.5.1 Anti-epileptic drug treatment: ............................................................................................................... 107
9.5.2 Epilepsy surgery.................................................................................................................................... 108
9.5.3 Other treatments .................................................................................................................................... 108
9.6 Comorbidities ................................................................................................................................................ 108
9.6.1 Factors Affecting Cognitive Function in Epilepsy................................................................................ 110
Seizure related variables ................................................................................................................................... 110
Treatment related variables ............................................................................................................................... 111
Non–SeizureRelated Variables ......................................................................................................................... 111
9.7 Practical guidelines ....................................................................................................................................... 111
10 Neurodegeneratieve aandoeningen (werkcollege) ............................................................................................ 113
10.1 Neurocognitieve stoornis .............................................................................................................................. 113
10.1.1 Classificatie volgens DSM-5................................................................................................................. 113
Normale veroudering ........................................................................................................................................ 113
Delirium ............................................................................................................................................................ 113
Beperkte of uitgebreide NCS ............................................................................................................................ 113
Etiologie (oorzaak)............................................................................................................................................ 113
A. Ziekte van Alzheimer ............................................................................................................................. 113
B. Vasculaire dementie ............................................................................................................................. 115
C. Frontotemporale dementie .................................................................................................................. 116
D. Lewy body dementie ............................................................................................................................. 117
8
, 1 Neuropsychologische symptomatologie
1.1 Visuele waarneming
• Komt frequent voor
• Varieert tussen totaal blind, tot gedeeltelijk
• Varieert problemen van zien van bewegingen, kleur, visuele illusies
1.1.1 Stoornissen in de visuele waarneming
• 3 categorieën
• Sommige stoornissen komen niet enkel voor bij aangeworven of neurogeneratieve aandoending.
• Sommige komen congenitaal (aangeboren) voor, zonder sprake van hersenaandoening
1. Alles is in orde ➔ Na optische chiasma
2. Problee thv de gaat info naar de
retina/nervus opticus. Is thalamus om dan naar
juist achter het oog. Dit de primaire visuele
kan leiden tot blindheid. cortex te gaan. Dan heb
Blindheid beperkt tot 1 je hier problemen aan de
oog (monoculaire contralaterale HH
blindheid) 5. Letsel hebt RH, uitval
➔ Uitval van beide in linker visueel veld, en omgekeerd.
visuele velden 6. Soms een kleiner deel van de HH dat niet meer goed
3. Chiasma opticum: banen kruisen. Niet meer per oog functioneert, dan heb je een kwadrant
georganiseerd, maar hier per visueel veld 7. Centrale gedeelte van visueel veld, blijft intact.
gegroepeerd. Blauw + blauw, rood + rood Maar je hebt wel nog uitval (hemianopsie). Dit is
Dit kan leiden tot bitemporale H: er is een letsel aan een letsel in primair visuele cortex. Vaak na een
de mediale zijde van het chiasma (R: rood, L blauwe beroerte
baan die dan is aangetast; buitenste gedeelte van
visueel veld, valt weg)
4. Enkel uitval langs de laterale kant van het chiasma.
Uitval aan 1 oog, en enkel maar het binnenste
gedeelte van visueel veld
Voorbeelden: tumor vd
hypofyse. Tumor duwt op lk oog krijgt info van
optisch chiasma, uitval van de 2 visuele velden
buitenste visuele velden Info van R visueel veld
-> wordt verwerkt in
de L occipitale cortex
(Blauw) en omgekeerd
a) sulcus calcarinus: letsel boven en onder deze sulcus, dan krijg
homonieme hemianopsie
b) Letsel onder sulcus calcarinus, bij de gyrus. Uitval bovenste
gezichtsveld (L -> R, B-> O). dit is klein stukje, mensen zijn zich daar vaak
niet van bewust
c) Onder de sulcus calacarinus: uitval in het bovenste gezicht kwadrant.
Groter letsel, dus grotere uitval dan in b)
9
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