Symposium for Patients & Caregivers

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Symposium for Patients & Caregivers. Cognitive Impact of HH (and what can we do about it). Jennifer V. Wethe, Ph.D.* Clinical Neuropsychologist Hook Rehabilitation Outpatient Services Community Hospital Network Indianapolis, Indiana *Formerly with Barrow Neurological Institute/SJHMC. - PowerPoint PPT Presentation

Text of Symposium for Patients & Caregivers

  • Symposium for Patients & Caregivers

  • Cognitive Impact of HH(and what can we do about it)

    Jennifer V. Wethe, Ph.D.*

    Clinical NeuropsychologistHook Rehabilitation Outpatient ServicesCommunity Hospital NetworkIndianapolis, Indiana*Formerly with Barrow Neurological Institute/SJHMC

  • OutlineCognitive functioning in individuals with epilepsy and HH

    Cognitive outcome of neurosurgical interventions for HH

    Interventions for cognitive difficulties

    Working with schools

  • Cognitive Functioning

  • Cognitive Functioning in EpilepsyEpilepsy is associated with impaired/abnormal cognitive functioningHigh rates of mental retardation (MR) in patients with childhood-onset epilepsyIncreased risk of MR if intractible seizures with onset during the first 2 years of life, especially if daily seizuresRefractory epilepsy is associated with cognitive decline, particularly in childrenBjornes et al., (2001), Dodrill (2004), Herman & Seidenberg (2007), Vasoncellos et al., 2001

  • Clinical Syndrome of HH with EpilepsyNeuropsychological/Behavioral FeaturesWide range of intellectual functioningNear normalDeclining after a period of relatively normal developmentMental Retardation & clear developmental delayBehavioral DisturbanceRage reactions, irritabilityImpulse control, ODD, CD, attention/ADHD, aggression, anxiety & compulsive behavior, depression, mood instabilityAutistic or Aspergers-like interpersonal skills & affectWeissenberger et al, 2001; Prigatano et al, 2006; Veendrick-Meekes (2007)Not all elements occur in every patient

  • Cognitive Functioning in HH PatientsBerkovic et al 19884 pediatric/adult patients with follow-upAll had cognitive deficits with 3 showing deterioration over timeFrattali et al 2001All 8 children displayed cognitive deficits, ranging from mild to severeGelastic/CPS seizure frequency and severity correlated with broad cognitive ability scoresRelative weakness in long term retrieval and information processing speedRelative strength in visual processing

  • Cognitive Functioning in HH PatientsHarvey et al 200329 patients aged 4-2372.4% of patients in series had intellectual disabilityMullatti 200314 patients whose HH was discovered at age 16 or later. No or minimal seizure difficultiesCompared to series of younger patients: Fewer learning difficulties, although 2/14 had moderate to severe learning difficulties and were in residential care; 6 had mild learning difficultiesMore patients with normal IQ, although they may not show typical patterns of cognitive functioningFewer behavior problems

  • Cognitive Functioning in HH PatientsQuiske et al 200613 juvenile and adult patientsIQ ranged from moderate MR to good54% had below average IQMemory impaired in most patients-both verbal & visualImpairments in attention, executive systems functioning and visuospatial abilities was commonRegis et al 200627 patients aged 3 to 50Mental retardation in 30% and low average IQ in an additional 26% of patientsDifficulties with sustained attention, impulsivity, disinhibition

  • Cognitive Functioning in HHPrigatano et al 200849 HH patients aged 5-55. Three patterns were identifiedPattern 1: (near normal) average or above average IQ with no significant verbal-nonverbal split (17 patients; 35%)Pattern 2: (transitional) Notable disparity between verbal and visuospatial skills -- One at least 1 SD below mean with other score normal (9 patients; 18%)Pattern 3a: Mentally retarded, but testable (16 patients; 33%))Pattern 3b: Mentally retarded, untestable (7 patients; 14%)Wethe, Prigatano et al32 pediatric & adult patients evaluated prior to surgeryMean pre-surgical IQ in the low average (mildly impaired) rangeMildly to moderately impaired new learning and memoryMildly to moderately impaired speed of processingSeverely impaired mental flexibility (e.g., multi-tasking)Low average basic language and motor abilities

  • Cognitive Functioning in HH:(Pre-surgical) SummaryHighly variable, ranging from essentially or near normal to profoundly impairedHigh proportion of mental retardationAbnormalities in cognitive functioning even in patients with normal IQAttention, memory, visuospatial skills, speed, mental flexibilityIndividuals with later onset of seizure disorder (e.g., late adolescent or adulthood) and less disabling seizures tend to have better cognitive functioning

  • Surgical Outcome

  • SurgerySurgical advances in the treatment of HH have been shown to improve seizure outcome, but little is known about cognitive and behavioral outcome.HH is located deep within the brain and neuroanatomical structures important for memory may be placed at risk by the surgical approach.

  • Outcome of GK SurgeryRegis et al 200627 patients at least 3 years post GKS59% had dramatic behavioral and cognitive improvement and many had developmental learning acceleration at school but details not providedNo complaints of worsening cognitive abilities or short-term memory complaintMathieu et al 20109 patients aged 12-57Quality of life and verbal memory improved

  • Outcome of Interstitial Radiotherapy Quiske et al 2007

    14 adolescent and adult patients did not demonstrate any significant cognitive changes 3 months following interstitial radiotherapy

  • Outcome of Radiofrequency ThermocoagulationKameyama et al 2009

    25 patients aged 2-36 years56% MR pre-surgery

    Intellectual improvement and resolution of behavior disorder

  • Outcome of TC surgeryHarvey et al 200329 patients aged 4-2314 patients had early short-term memory impairment. This persisted in 4 patients, 2 of which had undergone prior surgeryNg et al 200626 patients (no formal post-op testing)Subjective report of improved cognitive functioning in 65% of patientsTransient post-operative memory impairment in 58%, persisted in 8% (2 patients)Anderson and Rosenfeld 20104 of the patientsImprovement in perceptual/visuospatial functioning patients showed decline in memory

  • Outcome of TC and Endoscopic Resection: Barrow SeriesPediatric and adult patients (3-39 yo; mean 12 yo) with refractory epilepsy11 TC; 20 Endoscopic, 1 combinedMostly sessile Type II HH (within 3rd ventricle)Early onset of epilepsy (most within 1st months, all by age 5)Mean follow-up interval was nearly 2 years (range 5 47 months)

  • Raw or RangePercentagesDemographics Mean (SD)Age at surgery (years)12.2 (7.0)3.3-39.3Mos. btwn surgery & post-eval23.4 (12.0)5.1-47.2Sex (male/female)20/1262.5/37.5Handedness (right/left)22/1068.8/31.3Precocious Puberty (Yes/No)9/2328.1/71.9HH Characteristics Pre-surgeryType (I/II/III/IV)1/24/4/33.1/75/12.5/9.4Side of attachment (L/R/b)13/10/940.6/31.3/28.1Pre-surgery Volume (cm3)4.45 (8.62)0.13-38.25Seizure Characteristics Pre-surgeryAge of onset (months)10.0 (15.1)1-60Duration of epilepsy (months)136.3 (83.7)38-459Seizure types (Gel only / multiple)6/2618.8/81.3# of AEDs2.1 (0.76)1-4Surgery CharacteristicsTC/Endoscopic/Combined11/20/134.4/62.5/3.1% Resection83.7 (18.0)40-100Thalamic Infarct 618.8

    HH-NBOSDemographic & Clinical Characteristics

  • HH-NBOS Methods Pre and Post Assessment Measures Cognitive ScreeningBNIS or BNIS-CIntelligence TestingWAIS-III, WISC-III/IV, WPPSI-IIIProcessing SpeedDigit Symbol-CodingTMTAttentionDigit SpanLanguageAnimal FluencyMemoryRAVLT, CVLT-CBVMT-RMotorHalstead FTTBehaviorCBCL

  • Outcome of TC and Endoscopic Surgery: Barrow Series

    68.8% were seizure free at post-op assessment

    Patients were taking significantly fewer AEDs with 25+% not taking any AEDs

    Seizure freedom not necessary for cognitive gains

  • Cognitive Outcome of TC and Endoscopic Surgery: Barrow SeriesPerformance on key and summary measures of intellectual functioning was improvedFSIQ (12): 83 91.3 (Range -1 to 18)Performance on measures of attention and speed was improvedNo clear pattern for memory outcome (no overall decline)List Learning (17): 32.2 29.9 (Range -29 to 28)List delayed recall (14): 30.4 24.4 (Range -25 to 17)Trend toward decline on delayed verbal recall (n.s.)Some patients improved their memory performance while others clearly declinedPatients with MRI Type III HH may be at greater risk of memory decline than patients with MRI Type II HHVerbal Fluency and nondominant hand finger tapping improved

  • Outcome of TC and Endoscopic Surgery: Barrow SeriesYounger patients and those with shorter duration of epilepsy were more likely to improve their intellectual functioningPatients with mental retardation at pre-surgery were more likely to have improved their intellectual functioning post-surgeryLower intellectual functioning and shorter duration of epilepsy at time of surgery was associated greater gains in intellectual functioning at post-surgical follow-upComplete seizure cessation not necessary for cognitive gains

  • Cognitive Outcome Post Neurosurgical Intervention: Key PointsHH with refractory epilepsy is associated with cognitive decline (epileptic encephalopathy). Successful neurosurgical intervention can halt and even reverse the cognitive and behavioral decline.Complete seizure cessation may not be necessary for improvements to be observed. Temporary and permanent surgical complications are a risk with the invasive approaches and may negatively impact cognitive functioning (e.g., memory is an area of particular risk, although some patients experience improved memory functioning with successful surgery) Early intervention is important. Greatest gains with shortest duration of epilepsy.

  • Interventions

  • Professional AssistanceCognitive RehabilitationSpeech therapyaddress cognitive skills (e.g., attention, memory, problem solving) and compensationsOccupational therapyAddress activities of dai