Developmentally Delayed Children and Adults


General intellectual functioning

Raw score

Scaled score

Description

Peabody Picture Vocabulary Test-4

111

SS = 43

Impaired

WAIS-III
   
 Vocabulary

9

3

Impaired

 Similarities

5

2

Impaired

 Comprehension

5

3

Impaired

 Digit span

14

8

Low average

 Block design

12

4

Borderline

 Matrix reasoning

6

5

Borderline







































Attention and concentration

Raw score

T score

Description

WAIS-III digit span

14

ss = 8

Low average

Forward

7
   

Backward

3
   

Trails A (seconds)

53″

22

Impaired

Errors

0
   










































































Language

Raw score

T score

Description

Phonemic fluency: F(2), A(2), S(2)

6

12

Impaired

Animal naming

9

15

Impaired

Stroop color

140

z = −6.49

Impaired

Errors/self-corrections

1/2
   

Stroop word

116

z = −10.60

Impaired

Errors/self-corrections

0/2
   

Boston Naming Test

35,0,4

25

Impaired

Language screening

13/24
   

WRAT-3 reading (grade Eq = 2)

24

SS = 49

Impaired

WRAT-3 spelling (grade Eq = 2)

22

SS = 57

Impaired

WRAT-3 arithmetic (grade Eq = 2)

22

SS = 50

Impaired

WJ-III word attack (grade Eq = 1.7)

6

SS = 62

Impaired












































Visuospatial skills

Raw score

T score

Description

WAIS-III block design

12

ss = 4

Borderline

WMS-III visual reproduction discrim

7/7
 
Average

Rey-O complex figure copy

14.5
 
Impaired

Beery VMI (age Eq = 5.6)

15

<45

Impaired

Beery visual perception (age Eq = 6.2)

18

<45

Impaired

Beery motor coordination (age Eq = 5.6)

16

<45

Impaired








































































































Verbal memory

Raw score

Z score

Description

WMS-III
     

 Logical memory I (5,3,8)

16

ss = 3

Impaired

 Logical memory II (4,7)

11

ss = 6

Low average

 Recognition (8,10)

18/30
 
Borderline

 CVLT-II total = (6,7,8,12,11)

44

T = 38

Low average

 List A Trial 1

6

−1.0

Low average

 Trial 2

7

−1.5

Borderline

 Trial 3

8

−2.0

Impaired

 Trial 4

12

−0.5

Average

 Trial 5

11

−1.5

Borderline

 List B

7

0.0

Average

 List A short delay free recall

10

−0.5

Average

 List A short delay cued recall

7

−2.5

Impaired

 List A long delay free recall

11

−0.5

Average

 List A long delay cued recall

6

−3.0

Impaired

 Recognition hits

16

0.0

Average

 Recognition false positives

2

−0.5

Average

 Total recognition discriminability

3.4

0.0

Average



































































































Nonverbal memory

Raw score

T score

Description

WMS-III
     

 Visual reproduction I

57

ss = 3

Impaired

 Visual reproduction II

0

ss = 1

Impaired

 Recognition

38/48

ss = 4

Borderline

 BVMT-R total = (2,2,4)

8

<20

Impaired

 Trial 1

2

25

Impaired

 Trial 2

2

<20

Impaired

 Trial 3

4

<20

Impaired

 Learning

2

40

Low average

 Delay

4

<20

Impaired

 Percent retention

100
 
Average

 Recognition

6, 1fp
 
Low average

 Rey-O complex figure
     

 Immediate recall

3.5

<20

Impaired

 Delayed recall

2.0

<20

Impaired

 Recognition

18/24

28

Impaired

 Whole figure recognition

no
   






















































Executive functions

Raw score

T score

Description

WAIS-III similarities

5

ss = 2

Impaired

WAIS-III comprehension

5

ss = 3

Impaired

WAIS-III matrix reasoning

6

ss = 5

Borderline

Phonemic fluency: F(2), A(2), S(2)

6

12

Impaired

Trails B (s)

300″

2

Impaired

Errors

1
   

Stroop interference

218

z = −3.68

Impaired

Errors/self-corrections

0/14
   



Qualitatively, her verbal skills were slightly stronger than indicated by her impaired performance on formal measures of language functioning because she was able to converse with the examiner, and her comprehension of simple questions and basic task instructions was adequate. In contrast, however, she demonstrated considerable difficulty when required to appreciate the broad or abstract concepts and context of conversational speech. Generally, she demonstrated qualitatively weaker complex comprehension of the gestalt of speech, thus indicating weaknesses in right hemisphere contributions to complex language information processing. She demonstrated significant strengths on measures of simple auditory attention, indicating that she was able to attend to information at an almost age-appropriate level. On measures of verbal memory, she demonstrated poor learning and memory for contextual verbal information, which was likely negatively impacted by her inability to appreciate semantic and verbal contextual and gestalt cues, once again likely reflecting weaknesses in right hemisphere contributions to language. In contrast, she demonstrated a significant strength on a task of verbal list learning and memory, which was felt to be indicative of adequate left mesial temporal functioning. On measures of visual memory, she demonstrated impaired learning and memory for all visual information, even relatively simple visual information, which was felt to be indicative of impaired right mesial temporal functioning. Furthermore, her performances on a number of other measures of neuropsychological functioning implicated right hemisphere involvement in her seizures. Specifically, she demonstrated difficulty with visual perception and visual-motor integration. Qualitatively, she was unable to appreciate the gestalt of both verbal and visual information, which also suggested right hemisphere involvement in her seizures.

While her impaired performances on some of the neuropsychological tasks may have been negatively impacted by her extremely low level of general intellectual functioning, her relative strength in visual abstract reasoning indicated relatively adequate nonverbal reasoning skills and supported the idea that she was able to process visuospatial information at a level commensurate with her overall level of intellectual functioning. Thus, her qualitative weaknesses on the current evaluation likely represented true neurological weaknesses in right hemisphere functioning, rather than simply impairments secondary to her extremely low level of general intellectual functioning. Overall, the results of the neuropsychological evaluation suggested a specific involvement of right mesial temporal areas along with general involvement of the right hemisphere, with relatively spared right frontal lobe functioning. These findings were felt to indicate an area of epileptogenic focus in right mesial temporal structures, consistent with findings from a brain MRI, PET imaging, and inpatient video EEG monitoring.

Although G.L.’s performance was in the extremely low range across most neuropsychological domains, her strong performance on a measure of verbal learning and memory suggested adequate left mesial temporal functioning. While her quantitative performances on measures of language functioning were also extremely low, there was some qualitative and behavioral evidence for relatively good functioning of left hemisphere language areas which were not reflected by neuropsychological measures that were insensitive to performances in the extremely low range. Furthermore, she demonstrated a relative strength (both qualitatively and quantitatively) in her visuospatial reasoning skills, which indicated that she can appreciate visuospatial information appropriately and has relatively good functioning of frontal lobe structures, bilaterally. Thus, her extremely low performances on measures of visual learning and memory, combined with dramatically disrupted processing of visual and verbal gestalt and contextual information, were felt to provide evidence for right mesial temporal and generalized right hemisphere inefficiencies, with relatively spared right frontal lobe functioning. It was recommended that if G.L. became a candidate for right anterior temporal resection, Wada testing be conducted to determine the ability of her relatively stronger left mesial temporal lobe to support memory postsurgically. However, the neuropsychological team noted that it would be important that the Wada procedure be thoroughly explained to G.L. at a developmentally appropriate level, to ensure that she understood what the procedure involved, as her cooperation would be essential to the validity of the results. Furthermore, when G.L. was asked about her understanding of the neuropsychological evaluation and the possibility of undergoing resection surgery, she indicated limited understanding of the process. Thus, an additional recommendation of the evaluation was that the surgical procedure and possible sequelae of the surgical intervention be explained to her at a developmentally appropriate level given her extremely low level of general cognitive functioning in order to ensure her cooperation and compliance with pre- and postsurgical management.



Case 2


B.R. was a 55-year-old mildly intellectually disabled female with left anterior temporal lobe epilepsy who had been seizure-free for 2 years following a standard left anterior temporal lobectomy and continued medication management on a relatively low level of lamotrigine. She was referred for a neuropsychological evaluation to assess her postoperative level of neurocognitive and behavioral functioning. In particular, her mother reported that B.R. had a tendency to talk about events that did not really happen, so she had concerns that B.R. was either lying or was experiencing medication-related hallucinations. Her mother also expressed concern that B.R.’s memory had worsened since the surgery.

B.R. had undergone two prior presurgical neuropsychological evaluations. The results of the first presurgical neuropsychological evaluation indicated, “This woman demonstrates intellectual abilities which fall within the range of mild intellectual disability overall with visual-spatial abilities just lightly stronger than those in the verbal area. Her level of academic knowledge is just slightly below her level of general intelligence, and academic achievement may never have been especially strong. The battery of neuropsychological tests which was administered resulted in the identification of moderate impairment in brain functions with findings implicating both cerebral hemispheres about equally. This impairment is expected to have a substantial impact upon functioning in daily life…In terms of predictors for seizure relief following resection surgery for epilepsy, it was noted that only one of the four predictors of likely relief from seizures arising from this battery of tests were within a favorable range. While this is not a positive result, it should be noted that a full prognostic statement of likely relief from seizures following resection surgery must take into account other critical clinical and EEG data.” The results of the second presurgical neuropsychological evaluation indicated, “The patient is a 53-year-old right-handed female with moderate intellectual disability. This is conferred on the current IQ assessment. The majority of neuropsychological measures completed are consistent with her overall level of cognitive function. Interestingly, an area of particular strength was seen for repeat trials of verbal learning and memory…The patient interacts at a higher level than would be anticipated based on her IQ…Oral and cognitive presentation is reflective of generalized cortical dysfunction with preservation of left temporal lobe capacity. These findings are consistent with her history of bilateral seizures and significant illness during critical development.” It was concluded that improved seizure control would probably positively improve her life in terms of her personal safety and functional ability, with some risks to her verbal memory that would likely not be apparent in her day-to-day functional capacity.

Developmentally, B.R. and her husband lived with her parents. She was able to bathe and dress herself and do a few chores around the house, such as taking care of her cat, doing some of the cleaning, and folding the laundry, all of which she could do successfully with minimal supervision or guidance. She did not do the shopping or cooking, nor did she manage any finances. She was not able to manage her medications independently and relied on her mother to remind her to take her medication.

Postsurgically, B.R. was unable to provide subjective information about her cognitive difficulties. It had been well documented that she was mildly intellectually disabled and, per medical records, had limited insight into her cognitive limitations. Her mother reported that B.R.’s memory was poor prior to her undergoing the left anteromedial temporal resection but had seemed worse since the surgery. Additionally, she reported that B.R. had a tendency to confabulate and displayed some paranoid ideation. For example, 1 day B.R. could not find her cat and assumed someone had stolen it; in fact, the cat had simply wandered away for a short time. Another time, she found that money was gone from her husband’s wallet and assumed someone had stolen it, but he had just put the money in his pocket. Of note, medical records prior to surgery indicated that B.R. had a tendency to talk about memory for events that did not really happen even back then. Thus, it appeared that her tendency to confabulate was not new since the surgery. Further consistent with presurgical reports, B.R. had always been easily confused and forgetful about upcoming appointments and had often misplaced items. However, her mother felt that those problems had worsened since surgery. Her mother also reported that B.R. had begun to make literal paraphasic errors in conversation, such as saying that she wanted to go to the “feel good” store instead of the Goodwill store. This was reportedly new since she had undergone surgery. Her mother expressed concerns that these perceived cognitive changes were being caused by her long-term medication regimen of lamotrigine.

The postoperative neuropsychological test results are given in Table 13.2. Consistent with previous presurgical evaluations, the postsurgical neuropsychological evaluation measured B.R.’s overall level of intellectual functioning to be mildly intellectually disabled. On testing, B.R. performed in the impaired range on most tests of neuropsychological functioning, consistent with her performances on two previous presurgical evaluations. She once again demonstrated a relative strength in her verbal learning and memory abilities, as her performances were largely borderline to low average across those tasks. Compared with the most recent previous presurgical neuropsychological evaluation, however, she demonstrated a very mild decline in her verbal list learning capacity and retention, which was not surprising given that left mesial temporal structures that support verbal learning and memory were resected. Once again, her visual learning and memory was not as good as her verbal learning and memory. Interestingly, she also demonstrated a mild decline in her visual learning and memory abilities compared with presurgical performances. Overall, the findings of the postsurgical neuropsychological evaluation were consistent with a woman who suffered generalized cortical dysfunction and who, as a result, had mild intellectual disability and significant developmental delays. As had been demonstrated on previous presurgical neuropsychological evaluations, B.R. had a few areas of relative cognitive strength, including her verbal learning and memory ability and some aspects of social and executive skills. Her verbal learning and memory abilities continued to be a relative strength for her, despite showing a mild decline from presurgical levels. It was concluded that she was a woman who had clearly led a life that had been meaningful to her, and she continued to have the capacity to participate in her life at a level that gave her a sense of accomplishment and kept her feeling happy. Given that she was now seizure-free, she was leading a healthier life, and it was likely that she would continue to lead a happy and productive life for the foreseeable future, with very minimal to no change in her cognitive abilities since the surgery. Overall, it was felt that the neurosurgical resection to treat her refractory epilepsy was successful. Furthermore, there was no evidence to suggest that her antiepileptic medication regimen was negatively impacting her cognitive functioning or causing a significant change in her psychiatric or cognitive functioning.


Table 13.2
Neuropsychological test results in Case 2, a postsurgical evaluation of a 55-year-old mildly intellectually disabled female with left anterior temporal lobe epilepsy who is seizure-free following a standard left anterior temporal lobectomy










































































General intellectual functioning

Raw score

Scaled score

Description

WAIS-IV (standard norming)
     

 Verbal comprehension subtests
     

  Similarities

11

4

Impaired

  Vocabulary

10

3

Impaired

  Information

3

3

Impaired

 Comprehension

8

3

Impaired

 Perceptual reasoning subtests
     

  Block design

20

6

Low average

  Matrix reasoning

5

4

Impaired

  Visual puzzles

6

5

Borderline

 Working memory subtests
     

  Digit span

13

3

Impaired

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Sep 24, 2016 | Posted by in NEUROLOGY | Comments Off on Developmentally Delayed Children and Adults

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