Comprehensive neuropsychological evaluation of children and adolescents with sickle cell anemia: a hospital-based sample

Nunes, Samantha; Argollo, Nayara; Mota, Marivania; Vieira, Camilo; Sena, Eduardo Pondé de

doi:10.1016/j.bjhh.2016.09.004

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Original Articles • Rev. Bras. Hematol. Hemoter. 39 (1) • Jan-Mar 2017 • https://doi.org/10.1016/j.bjhh.2016.09.004 linkcopy

Comprehensive neuropsychological evaluation of children and adolescents with sickle cell anemia: a hospital-based sample

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Background: Individuals with sickle cell anemia may suffer symptomatic or silent cerebral infarcts leading to neurocognitive complications. This study investigated the cognitive and intellectual performance of children and adolescents with sickle cell anemia.

Methods: The socioeconomic status, clinical aspects and behavioral profile of 15 young individuals with sickle cell anemia were evaluated. The Wechsler Intelligence Scale for Children, the Developmental Neuropsychological Assessment Test, and the Child Behavior Checklist were applied.

Results: Participants with a history of stroke had lower intelligence quotient (IQ) scores. Alterations were found in attention and executive functioning, language, verbal and visual memory, visuospatial processing and sensorimotor skills. These alterations were found both in the children and adolescents who had had a cerebral infarction and in those who apparently had not. In the majority of cases, there were learning difficulties, a history of repeating school years and a need for specialist educational support. The most common additional diagnoses in accordance with the Diagnostic and Statistical Manual of Mental Disorders IV were depressive disorder, anxiety disorder and somatic disorder, as well as conditions associated with physical and psychosocial repercussions of sickle cell anemia.

Conclusion: As sickle cell anemia is considered a progressive cerebral vasculopathy, it is a potential risk factor for neurocognitive and psychosocial development. Therefore, periodic neuropsychological and behavioral evaluations of children and adolescents with sickle cell anemia may represent a useful measure to reduce long-term biopsychosocial repercussions.

Keywords: Anemia, sickle cell; Intelligence tests; Neuropsychological tests; Behavioral disorders; Neurobehavioral manifestations

Estimated IQ ^a a WISC-III: Wechsler Intelligence Scale for Children.7		NEPSY-II ^b b NEPSY-II: a developmental neuropsychological assessment.8		CBCL ^c c CBCL: Child Behavior Checklist.9
Index	Classification	Percentile	Classification	T -score	Percentile	Classification
≤79	Low	≤25th	Low	≤64	≤85th	Normal
80-119	Average	26-75th	Average	65-69	86-96th	Borderline
≥120	High	76-99th	High	≥70	≥97th	Clinical

Participant number
NEPSY-II subtests	1	2	3	4	5	6	7	8	9	10	11	12	13	14	15	Low	Mean
Auditory attention total correct	<2	2	75	26	26	5	<2	50	50	25	75	26	<2	25	26	7/15	8/15
Auditory attention commission errors	<2	50	50	50	50	<2	<2	50	50	25	50	50	75	50	50	4/15	11/15
Auditory attention omission errors	<2	25	50	50	50	<2	<2	50	50	50	50	50	<2	25	50	6/15	9/15
Auditory attention inhibition errors	10	50	50	50	50	50	<2	50	50	50	50	50	50	50	50	2/15	13/15
Auditory response set total correct	<2	25	2	10	10	25	<2	75	50	25	25	10	NC	50	50	10/14	4/14
Auditory response set total commission errors	5	50	75	<2	25	10	<2	50	50	5	50	25	NC	50	50	7/14	7/14
Auditory response set total omission errors	<2	25	<2	<2	25	5	<2	75	50	25	25	25	NC	75	75	10/14	4/14
Auditory response set total inhibition errors	10	50	50	25	75	50	25	75	50	25	75	50	NC	25	50	5/14	9/14
Design fluency	5	2	NC	5	5	50	NC	NC	NC	10	75	5	<2	<2	10	9/11	2/11
Inhibition total errors	<2	10	25	50	5	<2	IN	75	75	<2	75	<2	<2	<2	5	10/14	4/14
Narrative memory free and cued recall	<2	<2	10	50	75	25	5	2	75	5	50	75	25	5	10	10/15	5/15
Narrative memory free recall	25	10	5	50	50	25	10	<2	75	10	50	75	5	25	25	10/15	5/15
Memory for faces	NC	NC	NC	75	75	75	NC	NC	NC	5	25	50	75	25	10	4/9	5/9
Memory for designs content	10	25	10	50	75	25	<2	25	50	10	25	5	<2	2	10	12/15	3/15
Memory for designs spatial	10	10	50	25	50	10	<2	75	10	25	25	2	5	<2	75	11/15	4/15
Memory for designs total	5	25	10	50	50	25	<2	25	25	25	25	5	<2	<2	10	13/1	2/15
Memory for designs delayed content	<2	50	25	P	75	25	<2	25	75	P	25	15	NC	10	25	9/12	3/12
Memory for designs delayed spatial	<2	50	10	25	75	25	2	75	75	P	25	10	25	10	75	9/14	5/14
Memory for designs delayed total	<2	75	10	10	75	10	<2	25	75	NC	10	10	10	25	75	10/14	4/14
List memory and list memory delayed	10	5	NC	25	25	25	NC	NC	NC	25	25	75	NC	50	75	7/10	3/10
Comprehension of instructions	10	10	2	10	25	19	<2	5	75	5	75	25	2	25	25	13/15	2/15
Phonological processing	10	<2	25	25	10	5	IN	2	50	5	50	<2	5	24	25	12/14	2/14
Speeded naming correct	10	5	2	<2	25	2	IN	50	50	<2	75	2	NC	<2	5	9/13	4/13
Speeded naming time	5	<2	25	<2	75	<2	IN	25	75	<2	50	<2	NC	50	75	8/13	5/13
Speeded naming errors	50	25	50	25	50	<2	IN	50	50	50	25	25	NC	25	50	6/13	7/13
Word generation semantic	2	75	25	10	50	2	<2	25	50	10	75	50	5	50	75	9/15	6/15
Word generation initial letter	<2	25	10	25	75	2	<2	75	25	<2	50	<2	NC	10	25	11/14	3/14
Arrows	<2	25	25	17	50	10	<2	25	10	25	50	50	<2	<2	50	11/15	4/15
Geometric puzzles	2	10	10	50	75	10	5	25	2	50	2	50	25	<2	25	11/15	4/15
Imitating hand positions	<2	50	NC	25	50	2	NC	NC	NC	50	10	10	<2	<2	75	7/11	4/11
Imitating hand positions (dominant hand)	75	25	NC	10	50	50	NC	NC	NC	50	10	5	<2	<2	75	6/11	5/11
Imitating hand positions (non-dominant hand)	<2	75	NC	50	75	<2	NC	NC	NC	50	25	25	<2	2	75	6/11	5/11
Visuomotor precision time	75	<2	NC	<2	50	25	NC	NC	NC	25	50	10	75	25	25	7/11	4/11
Visuomotor precision errors	<2	75	NC	75	75	<2	NC	NC	NC	75	50	25	<2	<2	50	5/11	6/11

Participant number
Characteristics	1	2	3	4	5	6	7	8	9	10	11	12	13	14	15
Gender	F	F	M	F	M	M	M	M	F	M	F	M	M	F	M
Age (years - mean: 10)	12	12	13	8	10	8	15	16	14	7	7	10	6	7	10
Right-handed	Yes	Yes	Yes	Yes	Yes	No	No	Yes	Yes	Yes	Yes	Yes	No	Yes	Yes
Type of school	Pub	Priv	Pub	Priv	Priv	Pub	Pub	Pub	Priv	Priv	Priv	Pub	Priv	Priv	Priv
Appropriate class for age	No	No	No	Yes	Yes	Yes	No	Yes	Yes	No	Yes	No	No	Yes	Yes
Learning difficulty	Yes	Yes	Yes	Yes	Yes	No	Yes	No	No	Yes	No	Yes	Yes	No	Yes
Mother's years of schooling	Miss	4	12	16	12	8	12	12	14	4	12	8	6	10	12
Socioeconomic level	B	B	B	A	B	C	C	B	A	B	B	B	B	B	C
Age at diagnosis (months - mean: 5)	36	4	0	4	0	0	9	4	12	0	0	0	0	0	1
Age at beginning of treatment (months - mean: 7)	36	9	0	15	1	0	11	4	12	6	1	2	6	0	2
Admissions to hospital	>10	>10	>10	>10	4	>10	3	>10	>10	>10	4	4	>10	>10	>10
Blood transfusions	>30	>30	>30	0	4	>30	>30	5	6	5	7	7	>30	>30	2
Hydroxyurea	Yes	Yes	No	Yes	Yes	Yes	Yes	No	Yes	Yes	No	Yes	Yes	No	No
Ischemic strokes	Yes	Yes	No	No	No	Yes	Yes	No	No	No	No	No	Yes	Yes	No
Hemisphere affected by strokes	Right	Right	None	None	None	Left	Left	None	None	None	None	None	Left	Right	None
Number of strokes	1	1	0	0	0	2	1	0	0	0	0	0	5	2	0
Silent infarcts	No	No	No	No	No	No	No	No	Yes	No	No	No	No	No	No
Estimated IQ (mean: 88.5)	77	77	91	94	100	71	56	91	103	85	123	91	80	83	106

Participant number
Symptom	1	2	3	4	5	6	7	8	9	10	11	12	13	14	15	Absent	Present
Anxiety/depression	50	57	68	65	66	53	50	68	70	69	52	59	67	51	53	14/15	1/15
Alienated/depressed	51	54	78	64	66	58	53	82	54	62	52	62	66	52	66	13/15	2/15
Somatic complaints	59	59	76	61	50	61	61	76	70	70	68	57	67	50	76	11/15	5/15
Social problems	66	54	79	52	56	60	61	63	51	67	57	56	73	52	56	13/15	2/15
Atypical behavior	56	50	70	54	58	51	59	72	60	58	51	58	50	51	54	13/15	2/15
Attention problems	65	51	62	52	55	51	59	76	50	66	55	57	66	52	64	14/15	1/15
Disruptive behavior	57	51	60	52	51	53	54	60	54	51	59	51	57	50	50	15/15	0/15
Aggressive behavior	56	50	64	57	57	57	54	61	54	72	61	61	59	50	52	14/15	1/15
Depressive disorder	60	50	78	56	52	52	55	77	70	60	63	65	70	50	73	11/15	4/15
Anxiety disorder	55	55	66	67	65	55	53	62	60	72	59	68	70	50	51	13/15	2/15
Somatic disorder	60	59	64	61	50	61	60	73	62	77	61	57	65	50	70	12/15	3/15
Attention deficit hyperactivity disorder	60	50	59	52	56	50	57	62	51	60	60	62	56	52	56	15/15	0/15
Oppositional defiant disorder	51	51	66	55	51	51	55	55	50	70	67	58	62	50	52	14/15	1/15
Conduct disorder	63	51	64	50	51	65	53	58	51	54	56	54	54	50	50	15/15	0/15
Obsessive compulsive disorder	51	59	63	59	64	51	59	70	69	64	55	50	60	50	50	14/15	1/15
Post-traumatic stress disorder	51	53	76	63	59	51	54	79	56	74	61	62	62	50	51	12/15	3/15

Participant
Characteristics/functions	1	2	3	4	5	6	7	8	9	10	11	12	13	14	15	F
Number of episodes of CVA	1	1				2	1						5	2		6/15
Brain hemisphere affected by CVA	R	R				L	L						L	R		R = 3/15; L = 3/15
Silent infarct									x							1/15
Changed Doppler findings	x						x			x		x	x			5/15
Learning difficulty	x	x	x	x	x		x			x		x	x		x	10/15
IQ	x	x				x	x									4/15
Attention/executive functioning	x	x	x	x	x	x	x			x	x	x	x	x	x	13/15
Memory	x	x	x	x	x	x	x	x	x	x	x	x	x	x	x	15/15
Language	x	x	x	x	x	x	x	x	x	x	x	x	x	x	x	15/15
Visuospatial processing	x	x	x	x		x	x	x	x	x	x		x	x	x	13/15
Sensorimotor	x	x	NP	x		x	NP	NP	NP	x	x	x	x	x	x	10/11
Somatic complaints			x					x	x	x					x	5/15
Alienated/depressed			x					x								2/15
Anxiety/depression									x				x			2/15
Social problems			x													1/15
Attention problems								x								1/15
Atypical behavior			x					x								2/15
Aggressive behavior										x						1/15
Depressive disorder			x					x	x				x		x	5/15
Somatic disorder								x		x					x	3/15
Anxiety disorder										x			x			2/15
Oppositional defiant disorder										x						1/15
Conduct disorder																1/15
Post-traumatic stress disorder			x					x		x						3/15
Obsessive-compulsive disorder								x								1/15

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[TFN1] ^a
WISC-III: Wechsler Intelligence Scale for Children.⁷

[TFN2] ^b
NEPSY-II: a developmental neuropsychological assessment.⁸

[TFN3] ^c
CBCL: Child Behavior Checklist.⁹