Billard C, Gillet P, Signoret JL, Uicaut E, Bertrand P, Fardeau M, et al., 19921010 Billard C, Gillet P, Signoret JL, Uicaut E, Bertrand P, Fardeau M, et al. Cognitive functions in Duchenne Muscular Dystrophy: a reappraisal and comparison with spinal muscular atrophy. Neuromuscul Disord. 1992; 2(5-6):371-8.
Cognitive functions in Duchenne muscular dystrophy: a reappraisal and comparison with spinal muscular atrophy
Cross-sectional
Moderate risk of bias
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Duchenne muscular dystrophy (n=24): 12 to 16 years (mean 14 years); spinal muscular atrophy (n=17) types II and III): 12 to 16 years (mean 14 years). No control group. |
Assessment was made by a single-blinded evaluator. Three cognitive areas were assessed:
Language (MacCarthy intelligence scale for children, North syntactic screening test, Alouette test for reading age and the verbal scale of the Wechsler intelligence scale: information, similarities, arithmetic and vocabulary);
Visuospatial (the performance scale of the Wechsler intelligence scale: picture completion, picture arrangement, block design and object assembly);
Attention-memory (selective auditory response and Batterie d'Efficience Mnésique: global verbal and visual memory). |
Normal performance of children with spinal muscular atrophy.
Children with SMA had higher scores on Wechsler intelligence scale (normal level) than children with Duchenne muscular dystrophy (below normal).
Children with SMA had higher scores on the North syntactic screening test and on the global verbal and visual memory.
Only 61% of the participants with Duchenne muscular dystrophy had normal reading age compared to 100% of participants with SMA. |
Muscle strength/function and cranial nerve functions were not assessed and may have influenced results. Bias due to confounding.
No control group: spinal muscular atrophy was compared to Duchenne muscular dystrophy. Children with spinal muscular atrophy type I were not included. Education and social class were not controlled. Bias in selection of participants.
Sometimes the examiner helped the patient with hand manipulation. Tests with motor components and/or time limits were used. Bias in measurement of outcomes.
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Billard C, Gillet P, Barthez M-A, Hommet C, Bertrand P, 19981111 Billard C, Gillet P, Barthez M-A, Hommet C, Bertrand P. Reading ability and processing in Duchenne muscular dystrophy and spinal muscular atrophy. Dev Med Child Neurol. 1998;40(1):12-20.
Reading ability and processing in Duchenne muscular dystrophy and spinal muscular atrophy
Cross-sectional
Moderate risk of bias
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Duchenne muscular dystrophy (n=21): 8 to 13 years (mean 11 years); spinal muscular atrophy I and II (n=11): 6 boys, 8 to 13 years (mean 11 years). Control group (n=42): 22 boys, 6 to 14 years (mean 9 years). |
Wechsler intelligence scale: information, similarities, vocabulary and arithmetic (verbal tasks); picture completion, picture arrangement, object assembly and block design (non-verbal tasks): only children with spinal muscular atrophy and Duchenne muscular dystrophy were tested. Language: Batterie d'evaluation du langage; (word repetition, picture naming); Test de Vocabulaire Actif et Passif (word comprehension); North syntactic screening test. Reading ability: La pipe et le rat; reading aloud test. |
Normal performance of children with spinal muscular atrophy.
Children with spinal muscular atrophy and with Duchenne muscular dystrophy did not differ in Wechsler intelligence scale total scores. Children with Duchenne muscular dystrophy had lower scores in similarities and arithmetic (verbal tasks). Children with spinal muscular atrophy had significantly higher reading skills than children with Duchenne muscular dystrophy. |
Muscle strength/function and cranial nerve functions were not assessed and may have influenced results. Bias due to confounding.
Children with spinal muscular atrophy who were able to read but had severe motor impairment were excluded from the protocol. Education and social class were not controlled. Bias in selection of participants.
Wechsler intelligence scale was not applied in the control group. Bias due to missing data.
Tests with motor components and/or time limits were used. Bias in measurement of outcomes.
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Rivière,Lécuyer, 20021212 Rivière J, Lécuyer R. Spatial cognition in young children with spinal muscular atrophy. Dev Neuropsychol 2002;21:273-83.
Spatial cognition in young children with spinal muscular atrophy
Cross-sectional
Low risk of bias
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Spinal muscular atrophy type II (n=12, 7 boys, 2 years). 12 healthy children (1 year and 6 months), 12 healthy children (2 years), 12 healthy children (2 years and 6 months), 12 healthy children (3 years). |
A spatial location memory test was used. A toy was hidden in one of three cups aligned horizontally on a table. The tabletop was rotated 180 degrees and the child participated in three trials. The task was to find the hidden toy. |
Normal performance of children with spinal muscular atrophy.
There was no difference between groups. All groups showed normal spatial location performance. Locomotor impairment does not appear to be a risk factor for slowing down the development of spatial perception. |
This study evaluated only children with one type of spinal muscular atrophy (type II). Diagnosis was not genetically confirmed. Bias in selection of participants.
This study explored a very specific developmental phase. Each trial ended when the child had searched for the hidden toy (correct versus incorrect location). Therefore, only Chi-square tests were possible. Bias in measurement of outcomes.
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von Gontard A, Zerres K, Backes M, Laufersweiler-Plass C, Wendland C, Melchers P, et al., 200277 von Gontard A, Zerres K, Backes M, Laufersweiler-Plass C, Wendland C, Melchers P, et al. Intelligence and cognitive function in children and adolescents with spinal muscular atrophy. Neuromuscul Disord. 2002; 12(2):130-6.
Intelligence and cognitive function in children and adolescents with spinal muscular atrophy
Cross-sectional
Low risk of bias
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96 children and adolescents with Spinal muscular atrophy: 18 with type I, 58 with type II and 20 with type III (6 to 18 years); 45 non-affected siblings: mean age 11 years; 59 healthy children without affected siblings: mean age 11 years. |
Raven colored and standard progressive matrices and Kaufman assessment battery for children(6 to 11 years): spatial perception, memory, executive function, arithmetic, reading comprehension.
Wechsler intelligence scale (12 to 18 years): reading comprehension, arithmetic, executive function, vocabulary, memory.
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Normal/above normal performance of children with spinal muscular atrophy.
Children and adolescents with spinal muscular atrophy have normal intelligence. Adolescents with spinal muscular atrophy showed higher intelligence than controls, probably due to environmentally mediated factors (strategies to compensate for their physical handicap). There were no differences between the performances of types I, II and III. |
Of a sample of 261 patients, 128 died (mostly type I) and six were excluded due to language difficulties. Some patients classified as type I were borderline between types I and II. Only 83 patients underwent genetic testing. Bias in selection of participants.
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Rivière,Lécuyer, 20031313 Rivière J, Lécuyer R. The C-not-B error: a comparative study. Cogn Develop. 2003;18(3):285-97.
The C-not-B error: a comparative study
Cross-sectional
Moderate risk of bias |
14 children with spinal muscular type II, aged 1 to 3 years (mean 2 years, 6 boys) and 14 controls (7 boys). |
A spatial location memory test involving invisible displacements of an object was used. A toy was hidden under one of three cloths. The child had to find the hidden toy. The misplacement of the hidden toy is known as the C-not-B error. |
Normal/above normal performance of children with spinal muscular atrophy.
The performance of children with spinal muscular atrophy was better than the performance of the control group. Inhibitory mechanisms present in children with spinal muscular atrophy are not as well-developed in healthy children, who tend to show more impulsive responses. |
Reaching and grasping are critical for this task. Therefore, muscle strength may have influenced movement inhibition. Bias due to confounding.
This study evaluated only children with one type of spinal muscular atrophy (type II). Diagnosis was not genetically confirmed. Bias in selection of participants.
Each trial ended when the child had searched for the hidden toy (correct versus incorrect location). Therefore, only Chi-square tests were possible. Bias in measurement of outcomes.
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Chung, Wong, Ip, 20041515 Chung BHY, Wong VCN, Ip P. Spinal muscular atrophy: survival pattern and functional status. Pediatrics 2004;114(5):548-53.
Spinal muscular atrophy: survival pattern and functional status
Cohort study
Moderate risk of bias
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Children and adults with spinal muscular atrophy (n=83), aged 1 to 40 years (22: type I, 9 boys; 26: type II, 9 boys; 16 type IIIa, 9 boys; 19 type IIIb, 7 boys) |
Parents/caregivers were interviewed for the scoring of the functional independence measure for children. The questionnaire consisted of three domains: selfcare, mobility and cognition. The cognition domain evaluated comprehension, expression, social interaction, problem solving, and memory and the maximum score was 35. This domain aimed to assess the real-life performance in daily activities, which required executive function.
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The performance of children with spinal muscular atrophy was below normal.
The percentage of individuals who had achieved functional cognitive independence were SMA II: 60%; SMA IIIa: 78%; spinal muscular atrophy IIIb: 90%. The cognition scores of participants with SMA types III and II were higher than the scores of participants with type I. |
Education and social class were not controlled. Bias due to confounding.
There was no control group. Some participants were not genetically tested for spinal muscular atrophy. Bias in selection of participants.
Intervention discontinuation was likely to be related to prognostic factors: higher mortality among more severe cases (probably type I). Bias due to missing data.
Only one domain of the functional independence measure for children evaluated cognition and no other scales were used. Bias in measurement of outcomes.
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Rivière, Lécuyer, Hickmann, 20091414 Rivière J, Lécuyer R, Hickmann M. Early locomotion and the development of spatial language: evidence from young children with motor impairments. Eur J Develop Psychol. 2009;6(5):548-66.
Early locomotion and the development of spatial language: evidence from young children with motor impairments
Cross-sectional
Moderate risk of bias |
12 children with spinal muscular atrophy type II (2 to 3 years, mean age 3 years) and 12 controls. |
The acquisition of language (understanding and production of specifically spatial terms) was evaluated in a series of 22 pictures. The understanding and production of the words in, on, under, above, in front of, behind, near, far, in the upper region, in the lower region were tested (in French). |
Normal/above normal performance of children with spinal muscular atrophy.
There was no difference between the two groups on the comprehension task.
In the production task, the SMA group had better performance than controls when producing the words for the relations in front of and behind. |
Only one type of SMA was evaluated. Participants were not genetically tested for SMA. Bias in selection of participants.
This study explored a very specific developmental phase and some tasks were too difficult for both groups. Bias in measurement of outcomes. Chi-square tests compared the percentage of correct answers but no data about variability were presented. Bias in selection of the reported result.
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Barja S, Muñoz C, Cancino N, Núnez A, Ubilla M, Sylleros R, et al., 20131616 Barja S, Muñoz C, Cancino N, Núnez A, Ubilla M, Sylleros R, et al. Estimulación audiovisual en niños con limitación grave de la motricidad: mejora su calidad de vida? Rev Neurol. 2013;57(3):103-11.
Estimulación audiovisual en niños con limitación grave de la motricidade: ?mejora su calidad de vida?
Case series
Serious risk of bias
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3 children with spinal muscular atrophy type I; 2 with neuropathy; 3 with myopathy. All children were classified as V in the Gross Motor Function Classification System |
Audiovisual stimuli were used for evaluation/training (4 weeks, twice a day). Attention was assessed by Tobii P10 with the software Catch-me (gaze viewer, eye-tracking device). Animation figures were based on the country's flag and other images while the sounds were based on farm animals. Blood pressure, heart/respiratory rates, peripheral oxygen saturation, facial expressions, ocular and extremity movements and presence of crying were also evaluated. |
The performance of children with spinal muscular atrophy was below normal.
There was attention improvement. Patients had difficulty following the objects and showing other communicative responses. Five patients improved their eye-tracking performance. Stimuli were well tolerated, with no change in baseline variables. |
The study did not perform standardized ophthalmologic evaluation. Bias due to confounding.
The study did not include a healthy control group; the sample was heterogeneous and small. Bias in selection of participants.
Only qualitative data were available. Bias in measurement of outcomes.
Some patients were not evaluated or followed. Bias due to missing data.
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Polido GJ, Barbosa AF, Morimoto CH, Caromano FA, Favero FM, Zanoteli E, et al., 201766 Polido GJ, Barbosa AF, Morimoto CH, Caromano FA, Favero FM, Zanoteli E, et al. Matching pairs difficulty in children with spinal muscular atrophy type I. Neuromuscul Disord. 2017;27(5):419-27.
Matching pairs difficulty in children with spinal muscular atrophy type I
Cross-sectional
Low risk of bias
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12 children with spinal muscular atrophy type I (3 to 9 years)
12 healthy children (3 to 9 years) |
Matching pairs in four tasks of increasing complexity (task 1: matching pairs of same figures; task 2: matching a figure with its corresponding color; task 3: matching letters in uppercase and lowercase; task 4: matching arabic and written numerals). The task involved attention, spatial location processing and executive function.
An eye-tracking device (Tobii P10) and a notebook were used. Responses were detected by the tracker when children gazed at the stimulus for two seconds. |
The performance of children with spinal muscular atrophy was below normal. There were no differences between groups in number of correct answers on task 1 (which had few pairs). On the other three tasks, children with spinal muscular atrophy had poorer performance than controls (less accuracy and longer times). Children with spinal muscular atrophy had more difficulty when the number of stimuli and complexity of the task were increased. |
The study did not perform standardized ophthalmologic evaluation. Some children may have performed poorly because they were not familiar with the equipment. Bias due to confounding.
The study evaluated only one type of spinal muscular atrophy. Bias in selection of participants.
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