» Articles » PMID: 20074952

The Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND): Test Development and Reliability

Overview
Specialty Neurology
Date 2010 Jan 16
PMID 20074952
Citations 130
Authors
Affiliations
Soon will be listed here.
Abstract

The motor skills of patients with spinal muscular atrophy, type I (SMA-I) are very limited. It is difficult to quantify the motor abilities of these patients and as a result there is currently no validated measure of motor function that can be utilized as an outcome measure in clinical trials of SMA-I. We have developed the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders ("CHOP INTEND") to evaluate the motor skills of patients with SMA-I. The test was developed following the evaluation of 26 infants with SMA-I mean age 11.5 months (1.4-37.9 months) with the Test of Infant Motor Performance and The Children's Hospital of Philadelphia Test of Strength in SMA, a newly devised motor assessment for SMA. Items for the CHOP INTEND were selected by an expert panel based on item mean and standard deviation, item frequency distribution, and Chronbach's alpha. Intra-rater reliability of the resulting test was established by test-retest of 9 infants with SMA-I over a 2 month period; Intraclass correlation coefficient (ICC) (3,1)=0.96. Interrater reliability was by video analysis of a mixed group of infants with neuromuscular disease by 4 evaluators; ICC (3,4)=0.98 and in a group of 8 typically developing infants by 5 evaluators ICC (3,5)=0.93. The face validity of the CHOP INTEND is supported by the use of an expert panel in item selection; however, further validation is needed. The CHOP INTEND is a reliable measure of motor skills in patients with SMA-I and neuromuscular disorders presenting in infancy.

Citing Articles

Multi-omics profiling in spinal muscular atrophy (SMA): investigating lipid and metabolic alterations through longitudinal CSF analysis of Nusinersen-treated patients.

Zandl-Lang M, Zullig T, Holzer M, Eichmann T, Darnhofer B, Schwerin-Nagel A J Neurol. 2025; 272(3):183.

PMID: 39904776 PMC: 11794407. DOI: 10.1007/s00415-025-12909-4.


Safety and Efficacy of Nusinersen Focusing on Renal and Hematological Parameters in Spinal Muscular Atrophy.

Senol H, Yildiz G, Polat A, Aydin A, Hiz A, Soylu A Brain Behav. 2025; 15(1):e70221.

PMID: 39829133 PMC: 11743982. DOI: 10.1002/brb3.70221.


Transitioning From Nusinersen to Risdiplam for Spinal Muscular Atrophy in Clinical Practice: A Single-Center Experience.

Bekircan-Kurt C, Subramanian S, Chagat S, MacKenzie S, Iammarino M, Reash N Muscle Nerve. 2025; 71(3):414-421.

PMID: 39744899 PMC: 11799403. DOI: 10.1002/mus.28329.


Case Report: Atypical motor development in a patient with the mosaic form of Down syndrome and spinal muscular atrophy type 2- long-term observation.

Gajewska E, Flicinski J, Sobieska M, Michalska J, Zarowski M, Steinborn B Front Genet. 2024; 15:1483903.

PMID: 39649095 PMC: 11621056. DOI: 10.3389/fgene.2024.1483903.


High Expression of SMN circ4-2b-3 in SMA I Children Treated with Nusinersen is Associated with Improved Motor Outcomes.

Guerra M, Marini A, Pagliarini V, Pitolli C, Coratti G, Bonvissuto D Mol Neurobiol. 2024; .

PMID: 39592557 DOI: 10.1007/s12035-024-04605-7.


References
1.
Finkel R, Hynan L, Glanzman A, Owens H, Nelson L, Cone S . The test of infant motor performance: reliability in spinal muscular atrophy type I. Pediatr Phys Ther. 2008; 20(3):242-6. DOI: 10.1097/PEP.0b013e318181ae96. View

2.
Zerres K, Rudnik-Schoneborn S, Forrest E, Lusakowska A, Borkowska J, Hausmanowa-Petrusewicz I . A collaborative study on the natural history of childhood and juvenile onset proximal spinal muscular atrophy (type II and III SMA): 569 patients. J Neurol Sci. 1997; 146(1):67-72. DOI: 10.1016/s0022-510x(96)00284-5. View

3.
Ludvigsson P, Olafsson E, Hauser W . Spinal muscular atrophy. Incidence in Iceland. Neuroepidemiology. 1999; 18(5):265-9. DOI: 10.1159/000026221. View

4.
Lefebvre S, Burglen L, Reboullet S, Clermont O, Burlet P, Viollet L . Identification and characterization of a spinal muscular atrophy-determining gene. Cell. 1995; 80(1):155-65. DOI: 10.1016/0092-8674(95)90460-3. View

5.
Iannaccone S . Modern management of spinal muscular atrophy. J Child Neurol. 2007; 22(8):974-8. DOI: 10.1177/0883073807305670. View