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Case Study: Neuropsychological and Electroencephalogram Findings in Suspected Panayiotopoulos Syndrome

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Abstract

Panayiotopoulos syndrome (PS) is a common form of childhood epilepsy characterized primarily by autonomic seizures, which often last upwards of 20–30 min, thus meeting the criteria for Autonomic Status Epilepticus (ASE). While prognosis for PS is typically positive, subtle neuropsychological deficits have been reported, with one study demonstrating more marked changes following episodes of status epilepticus (SE). Despite the growing body of evidence supporting impairment in areas such as language, attention, and executive functions before seizures resolve, research suggests that these patients are rarely referred for neuropsychological evaluation. As a result, neuropsychologists may not be familiar with PS despite its frequency, and patients demonstrating these subtle deficits may not be receiving supports which allow them to fully engage in academic and extracurricular activities and maximize overall quality of life. Additionally, these patients often show internalizing symptoms which may also affect quality of life and academic performance if not addressed. This case study provides a brief overview of the existing literature on PS as well as an example of a school-age patient presenting with a neuropsychological profile and electroencephalogram (EEG) findings largely consistent with the existing literature. A brief discussion of interpreting inconsistent performance in the context of established attention difficulties and language delays — including failure of multiple embedded performance validity tests — is also included.

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Data Availability

Not applicable. All relevant test data is included in the Appendix.

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Corresponding author

Correspondence to D. J. Bernat.

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Appendix

Appendix

Tables 1–9: Test results and corresponding citations for performance on neuropsychological testing.

INTELLIGENCE

Wechsler Intelligence Scale for Children – 5.th Edition (WISC-V; Wechsler, 2014)

Index/Subtest

Standard Score

Scaled Score

Percentile

FSIQ

95

 

37

Verbal Comprehension

92

 

30

Similarities

 

6

9

Vocabulary

 

11

63

Visual Spatial

111

 

77

Block Design

 

11

63

Visual Puzzles

 

13

84

Fluid Reasoning

100

 

50

Matrix Reasoning

 

7

16

Figure Weights

 

13

84

Working Memory

79

 

8

Digit Span

 

5

5

Picture Span

 

8

25

Processing Speed

105

 

63

Coding

 

12

75

Symbol Search

 

10

50

LANGUAGE

Boston Naming Test – Second Edition (BNT; Kaplan et al., 2001)

Z-score

Percentile

-1.96

 ~ 2.5

MOTOR/VISUAL

Grooved Pegboard (Norms from Skogan et al., 2018).

Hand

Z-score

Percentile

Dominant (Right) Hand

 − 1.28

 ~ 10

Non-Dominant Hand

 − 0.61

 ~ 27

Beery-VMI (Beery & Beery, 2010)

Subtest

Standard Score

Percentile

Visual Motor Integration

96

39

Visual Perception

97

42

Motor Coordination

105

63

ATTENTION

Conners Kiddie Continuous Performance Test-Second Edition

(K-CPT 2; Conners, 2015)

Scale

T-Score

Description

Detectability

48

Average

Omissions

46

Average

Commissions

48

Average

Perseverations

46

Average

Hit Reaction Time (HRT)

61

Slow

HRT Standard Deviation

49

Average

Variability

51

Average

Hit Reaction Time Block Change

56

Average

Hit Reaction ISI Change

44

Average

MEMORY

Child and Adolescent Memory Profile (ChAMP; Sherman & Brooks, 2015)

Index

Standard Score

Percentile

Screening Index

74

4

Domain/Subtest

Scaled Score

 

Verbal Memory

 

 Lists Immediate

9

37

 Lists Delayed

7

16

 Lists Recognition

1

 < 1

Visual Memory

 

 Objects Immediate

2

 < 1

 Objects Delayed

10

50

ACADEMIC

Bracken Basic Concept Scale-3.rd Edition (BBCS-III; Bracken, 2006a, 2006b)

School Readiness Composite

Scaled Score

Percentile Rank

Expressive

82

12

Receptive

85

16

ADAPTIVE/BEHAVIORAL

Behavior Assessment System for Children – Third Edition Parent Rating Scales (BASC-3 PRS-C; Reynolds & Kamphaus, 2015)

Scale

T-Score

Hyperactivity

47

Aggression

42

Conduct Problems

45

Anxiety

46

Depression

45

Somatization

61*

Attention Problems

56

Atypicality

54

Withdrawal

51

Externalizing Problems

44

Internalizing Problems

51

Behavioral Symptom Index

49

Adaptability

43

Social Skills

52

Leadership

42

Functional Communication

37*

Activities of Daily Living

44

Adaptive Skills

43

  1. *Scores of 40–31 on measures of adaptive skills suggest at-risk symptoms
  2. *Scores of 60–69 on clinical scales suggest at-risk symptoms

Conners 3 – Parent form (Conners, 2008)

Conners 3 Content Scales

T-Score

Inattention

66*

Hyperactivity/Impulsivity

65*

Learning Problems

82**

Executive Functioning

59

Defiance/Aggression

42

Peer Relations

43

DSM-5 Symptom Scales

T-Score

ADHD Predominantly Inattentive Type

68*

ADHD Predominantly Hyperactive/Impulsive Type

61

Conduct Disorder

45

Oppositional-Defiant Disorder

43

Conners 3 Index Scores

T-Score

Conners 3 Global Index: Restless-Impulsive

60

Conners 3 Global Index: Emotional Lability

45

Conners 3 Global Index: Total Score

54

  1. *T-scores of 65–69 suggest elevated symptoms
  2. **T-scores of 70 or higher suggest very elevated symptoms

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Bernat, D.J., Albert, D. & Cass, J. Case Study: Neuropsychological and Electroencephalogram Findings in Suspected Panayiotopoulos Syndrome. J Pediatr Neuropsychol 9, 91–99 (2023). https://doi.org/10.1007/s40817-022-00136-y

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