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Neuropsychological dysfunction in adults with cystic fibrosis
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Abstractsfrom the 17thAnnualMeeting
(LTL)and 6 right (RTL)left-languagedominanttemporallobectomypatientson the California VerbalLearningTest (CVLT)and Rey ComplexFigureTest (RCFT)were analyzed using Cheluneet al.’s (1993)reliable change index. Resultsreveal strong supportfor the FunctionalAdequacymodel (92% of predictionssupported)in that patients with higher pre-surgicalmemoryperformanceexperiencedreliablepost-surgicaldecrementswhile patients with lower pre-surgicalperformancedid not change. In contrast, the forecasting potentialof the FunctionalReservemodelwas poor (31% of predictionssupported)in that performance on memory measures hypothesizedto be mediated by the temporal lobe contralateralto thesurgicallobewasnot associatedwithpost-surgicalmemorychange.These resultsprovideadditionalsupportfor the FunctionalAdequacymodelas a valuableclinical neuropsychologicaltool for predictingpost-temporallobectomymemorychange. Luis, C. A., & Duara,R. The Common Form of Dl@se Lewy Body Disease: A Neuropsychological Case Study.
Recenthistologicalfindingshave demonstratedDiffuseLewy Body Disease(DLBD)to be an etiologicalfactorin 20–34%of dementiacasescomingto autopsy.To date,few reportsof the neuropsychologicalcorrelatesof DLBDhaveappearedin the literature.Infrequentuse of the appropriatestainingfor Lewy bodiesin post-mortemdiagnosis,nosologicalmisclassification,and/orlack of familiaritywith DBLD are possiblereasonsfor this paucity.This case report documentsthe neurobehavioralfindings in an autopsy confined case of DLBDcommonform.CM was a 76-year-old,right-handedCaucasianfemalewith a six-yearhistory of forgetfulness,depression,visual hallucinations,anxiety,and extrapyramidalsymptoms (bradykinesia,rigidity,widebasedand ataxicgait).The patientunderwentneuropsychological testing in years 3, 4, and 5 of her illness that revealed initial impairmentsin recent memory, visuospatialskills, and apraxia. Speech and language skills were intact. Serial testing revealed marked declinesin list learning,constructionalapraxia, informationprocessingspeed(SymbolDigitModalitiesTest),alternatingattention(TrailsB) and executive functioning.Retentivememoryand languageskills(i.e.,naming)showeda variabledecline. EEG revealed diffuseslowingwith intermittentfocal slowingin the right temporoparietal area. The MRI of the brain demonstrateddiffusecorticalatrophymore pronouncedin the medial-temporallobe along with accompanyingcistemal and ventriculardilatation.The findingsare discussedin relationto previouslypublishedcase descriptions. Maddrey,M. A., .Cullum,C. M., & Prestidge,C. Neuropsychological Dysfunction in Adults with Cystic Fibrosis. Cystic Fibrosis (CF), a systemic illness primarily involving the pulmonary system, is
associatedwith a varietyof neuromedicalrisk factors,includingchronicepisodichypoxia, poornutritionalstatus,diabetesand/orliverdisease.Withinthe lastthreedecades,the median survivalrate for patientswith CF has steadilyincreased,and currentlymore than one-third of patientswithCF are overthe ageof 16.Nevertheless,despitetheneuromedicalrisk factors associatedwith CF, no publishedstudiesto date have examinedcognitivefunctioningin adults with CF. The purpose of this study was to assess whether this unique population demonstratesevidenceof neuropsychologicalimpairmentin any of four cognitivedomains: attention,memory,visuospatialability,and abstraction/reasoning skills.Neuropsychological tests from each domain were administeredto 31 CF patients over the age of 17. Results indicatedthat comparedto age, gender,education-referencedtest norms,a percentageof CF subjects demonstratedneurocognitiveimpairment operationalizedas scores falling one standarddeviationor more below the mean. An analysisof frequencyof deficits demonstratedthat 23% of CF patientsshowedattentionaldeficits(DigitVigilanceTest);32% of subjectsdemonstratedmemory impairment(CVLT);61% showed deficits on a working
Abstracts@omthe 17thAnnualMeeting
119
memorycomponentof a computerizedvisuospatialtask (MicroCog;Tic Tac subtest);and 19%of the adultCF patientsdemonstratedimpairmenton a measureof abstractionh-easoning (WisconsinCard SortingTest).These preliminarydata suggestthat a significantportionof adultswith cysticfibrosisshowevidenceof neuropsychologicalimpairmentin one or more cognitivedomains,and meritsfurtherinvestigation. Mahalick,D. M., Hohn, G. E., Erlanger,D., Hunt, D., Schulder,M., & Cannel, P. W. Intracarotid Sodium Amytal Testing of Patients with Arteriovenous Ma~ormations: Its Utility a Function of Size and Shunt Value of the AVM.
The intracarotidsodium amytal (ISA) examination,developedby Jun Wada, has gained increasedpopularityover the last decadeas a meansof evaluatinglateralizationof language and memoryfunctionsin variouscandidatesfor neurosurgicalprocedures.Despitethe fact that this techniqueis employedmostcommonlywith epilepsypatients,it also has achieved widespreaduse in patients with brain tumors and arteriovenousmalformations(AVMS), particularlythose occupyingeloquentareas of cortex. It has been our experiencethat this procedureis very helpfulin somecases, but useIess in others,becauseof the uniquehemodynamicpropertiesof theAVM.AVMSare congenital vascular anomalieswhich profoundlyaffect the brain’s cerebrovascularsystem. Recent literaturesuggeststhat theAVMcan significantlyalterpatternsof neurobehavioralfunctioning, and anecdotally,localizationof these functions.In the AVM patient, the Wada test becomesa vitalstepin the assessmentof lateralizationof importanthighercorticalfunctions, with implicationsfor subsequentintervention. In an ongoinginvestigation,we have determinedthat the size of the AVMand its shunt valuehas significantbearingon the overallutilityand the decisionto proceedwith intracarotid sodiumamytalexamination.Informationprovidedby this initialinvestigationwillhelp establishguidelinesdelineatingwhen the ISA procedureis indicatedand contraindicatedin AVMpatients. Mende,J. M., Ho, M. R., & Bennet~ T. L. Hippocampal Mechanisms in P300 and Attention in an Amnestic Individual.
This case studyinvestigatedthe role of the event-relatedpotentialP300 componentin Mike S., an amnesticindividual,with significanthippocampaldamageas featuredin “Livingwith Amnesia:The Hippocampusand Memory”of The Brain(2ndcd.) series.Consistentwithhis amnesticsyndrome,Mike’s scoreson the MemoryAssessmentScalesfall at or below the first percentileon verbal memory,visualmemory,and global memory.Except on tests of learningand memory(TPT,CVLT),Mike performsin the normalrange in all tests of the expandedHalstead-ReitanNeuropsychologicalTest Battery.The P3b is stronglylinked to attentionand occurswith focusedattentionto a targetstimulus.The P3a is a subcomponent that is elicitedby novelor unexpectedstimuliand habituatesrapidly.To investigatethe role of the hippocampusin attentionand gatingprocesses,Mike’sP3a and P3b componentswere measuredin auditory and visual continuousperformancetasks with either intramodalor crossmodaldistracters.Behavioralperformanceon the attentiontasksrangedfrom 80-100% responseaccuracy,confirminghis ability to attend normally,as seen in his neuropsychological profile. However, the P3b component,which is considered to be reflective of attention,wasverylowor nearlyabsentfor someof the attendedstimuli.The P3acomponent also showed little habituationto the unexpectedstimuli, and was sometimesgreater in amplitudethantheP3b.Althoughlimitedin its conclusionsas a case study,this apparentlack of consistentP300 data in light of the attentionalbehaviormanifestedmay supportthe role of hippocampalgeneratorsin the P300 waveforms.Anotherimplicationfrom this data seen
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Abstractsfrom the 17thAnnualMeeting
(LTL)and 6 right (RTL)left-languagedominanttemporallobectomypatientson the California VerbalLearningTest (CVLT)and Rey ComplexFigureTest (RCFT)were analyzed using Cheluneet al.’s (1993)reliable change index. Resultsreveal strong supportfor the FunctionalAdequacymodel (92% of predictionssupported)in that patients with higher pre-surgicalmemoryperformanceexperiencedreliablepost-surgicaldecrementswhile patients with lower pre-surgicalperformancedid not change. In contrast, the forecasting potentialof the FunctionalReservemodelwas poor (31% of predictionssupported)in that performance on memory measures hypothesizedto be mediated by the temporal lobe contralateralto thesurgicallobewasnot associatedwithpost-surgicalmemorychange.These resultsprovideadditionalsupportfor the FunctionalAdequacymodelas a valuableclinical neuropsychologicaltool for predictingpost-temporallobectomymemorychange. Luis, C. A., & Duara,R. The Common Form of Dl@se Lewy Body Disease: A Neuropsychological Case Study.
Recenthistologicalfindingshave demonstratedDiffuseLewy Body Disease(DLBD)to be an etiologicalfactorin 20–34%of dementiacasescomingto autopsy.To date,few reportsof the neuropsychologicalcorrelatesof DLBDhaveappearedin the literature.Infrequentuse of the appropriatestainingfor Lewy bodiesin post-mortemdiagnosis,nosologicalmisclassification,and/orlack of familiaritywith DBLD are possiblereasonsfor this paucity.This case report documentsthe neurobehavioralfindings in an autopsy confined case of DLBDcommonform.CM was a 76-year-old,right-handedCaucasianfemalewith a six-yearhistory of forgetfulness,depression,visual hallucinations,anxiety,and extrapyramidalsymptoms (bradykinesia,rigidity,widebasedand ataxicgait).The patientunderwentneuropsychological testing in years 3, 4, and 5 of her illness that revealed initial impairmentsin recent memory, visuospatialskills, and apraxia. Speech and language skills were intact. Serial testing revealed marked declinesin list learning,constructionalapraxia, informationprocessingspeed(SymbolDigitModalitiesTest),alternatingattention(TrailsB) and executive functioning.Retentivememoryand languageskills(i.e.,naming)showeda variabledecline. EEG revealed diffuseslowingwith intermittentfocal slowingin the right temporoparietal area. The MRI of the brain demonstrateddiffusecorticalatrophymore pronouncedin the medial-temporallobe along with accompanyingcistemal and ventriculardilatation.The findingsare discussedin relationto previouslypublishedcase descriptions. Maddrey,M. A., .Cullum,C. M., & Prestidge,C. Neuropsychological Dysfunction in Adults with Cystic Fibrosis. Cystic Fibrosis (CF), a systemic illness primarily involving the pulmonary system, is
associatedwith a varietyof neuromedicalrisk factors,includingchronicepisodichypoxia, poornutritionalstatus,diabetesand/orliverdisease.Withinthe lastthreedecades,the median survivalrate for patientswith CF has steadilyincreased,and currentlymore than one-third of patientswithCF are overthe ageof 16.Nevertheless,despitetheneuromedicalrisk factors associatedwith CF, no publishedstudiesto date have examinedcognitivefunctioningin adults with CF. The purpose of this study was to assess whether this unique population demonstratesevidenceof neuropsychologicalimpairmentin any of four cognitivedomains: attention,memory,visuospatialability,and abstraction/reasoning skills.Neuropsychological tests from each domain were administeredto 31 CF patients over the age of 17. Results indicatedthat comparedto age, gender,education-referencedtest norms,a percentageof CF subjects demonstratedneurocognitiveimpairment operationalizedas scores falling one standarddeviationor more below the mean. An analysisof frequencyof deficits demonstratedthat 23% of CF patientsshowedattentionaldeficits(DigitVigilanceTest);32% of subjectsdemonstratedmemory impairment(CVLT);61% showed deficits on a working
Abstracts@omthe 17thAnnualMeeting
119
memorycomponentof a computerizedvisuospatialtask (MicroCog;Tic Tac subtest);and 19%of the adultCF patientsdemonstratedimpairmenton a measureof abstractionh-easoning (WisconsinCard SortingTest).These preliminarydata suggestthat a significantportionof adultswith cysticfibrosisshowevidenceof neuropsychologicalimpairmentin one or more cognitivedomains,and meritsfurtherinvestigation. Mahalick,D. M., Hohn, G. E., Erlanger,D., Hunt, D., Schulder,M., & Cannel, P. W. Intracarotid Sodium Amytal Testing of Patients with Arteriovenous Ma~ormations: Its Utility a Function of Size and Shunt Value of the AVM.
The intracarotidsodium amytal (ISA) examination,developedby Jun Wada, has gained increasedpopularityover the last decadeas a meansof evaluatinglateralizationof language and memoryfunctionsin variouscandidatesfor neurosurgicalprocedures.Despitethe fact that this techniqueis employedmostcommonlywith epilepsypatients,it also has achieved widespreaduse in patients with brain tumors and arteriovenousmalformations(AVMS), particularlythose occupyingeloquentareas of cortex. It has been our experiencethat this procedureis very helpfulin somecases, but useIess in others,becauseof the uniquehemodynamicpropertiesof theAVM.AVMSare congenital vascular anomalieswhich profoundlyaffect the brain’s cerebrovascularsystem. Recent literaturesuggeststhat theAVMcan significantlyalterpatternsof neurobehavioralfunctioning, and anecdotally,localizationof these functions.In the AVM patient, the Wada test becomesa vitalstepin the assessmentof lateralizationof importanthighercorticalfunctions, with implicationsfor subsequentintervention. In an ongoinginvestigation,we have determinedthat the size of the AVMand its shunt valuehas significantbearingon the overallutilityand the decisionto proceedwith intracarotid sodiumamytalexamination.Informationprovidedby this initialinvestigationwillhelp establishguidelinesdelineatingwhen the ISA procedureis indicatedand contraindicatedin AVMpatients. Mende,J. M., Ho, M. R., & Bennet~ T. L. Hippocampal Mechanisms in P300 and Attention in an Amnestic Individual.
This case studyinvestigatedthe role of the event-relatedpotentialP300 componentin Mike S., an amnesticindividual,with significanthippocampaldamageas featuredin “Livingwith Amnesia:The Hippocampusand Memory”of The Brain(2ndcd.) series.Consistentwithhis amnesticsyndrome,Mike’s scoreson the MemoryAssessmentScalesfall at or below the first percentileon verbal memory,visualmemory,and global memory.Except on tests of learningand memory(TPT,CVLT),Mike performsin the normalrange in all tests of the expandedHalstead-ReitanNeuropsychologicalTest Battery.The P3b is stronglylinked to attentionand occurswith focusedattentionto a targetstimulus.The P3a is a subcomponent that is elicitedby novelor unexpectedstimuliand habituatesrapidly.To investigatethe role of the hippocampusin attentionand gatingprocesses,Mike’sP3a and P3b componentswere measuredin auditory and visual continuousperformancetasks with either intramodalor crossmodaldistracters.Behavioralperformanceon the attentiontasksrangedfrom 80-100% responseaccuracy,confirminghis ability to attend normally,as seen in his neuropsychological profile. However, the P3b component,which is considered to be reflective of attention,wasverylowor nearlyabsentfor someof the attendedstimuli.The P3acomponent also showed little habituationto the unexpectedstimuli, and was sometimesgreater in amplitudethantheP3b.Althoughlimitedin its conclusionsas a case study,this apparentlack of consistentP300 data in light of the attentionalbehaviormanifestedmay supportthe role of hippocampalgeneratorsin the P300 waveforms.Anotherimplicationfrom this data seen
——