Hammersmith Infant Neurological Examination

Instrument Name: Hammersmith Infant Neurological Examination

Abbreviation: HINE

Points for Consideration:

More information needed. Appears to have been used successfully (responsivity) in one clinical trial.

Description of Tool:

The HINE is a ClinRO designed to assess multiple aspects of neurological function in infants between 3 and 24 months of age.

Minimum Qualification Required by COA Administrator: MA or BA

Year: 1999

Objective of Development:

To assess infants from 2-24 months of age

Population of Development: Age range (therapeutic indication):

3-24 months (Child Development Disorders, Pervasive)

Pediatric Population(s) in which COA has been used:

Congenital, Hereditary and neonatal diseases and Abnormalities; Nervous system disorders; Virus diseases

COA type:

Number of Items 37 items

Mode of Administration:

Data Collection Mode:

Time for Completion: Not reported

Response Scales: Varies by item: 3 to 5-point categorical responses based on examination

Summary of Scoring:

Available Scores:
Global Score ranging from 0 to 78
Score by domains
Score by items

Weighting:
No

Score Interpretation:
Lower score = Higher impairment

Evidence of Literature Review: None identified

Evidence of Instrument Review: Yes

Evidence of Clinical or Expert Input: Yes

Evidence of concept elicitation in target patient population: None identified

Evidence of a Saturation Grid: None identified

Evidence for Selection of Data Collection Method: None identified

Recall/Observation Period:

Evidence for Selection of Reponse Options: Yes

Evidence of cognitive interviewing of draft instrument in target patient population: None identified

Evidence of Preliminary Scoring of Items and Domains: None identified

Evidence related to respondent and administrator burden: None identified

Evidence of a Conceptual Framework: None identified

Evidence of an item-tracking matrix: None identified

Evidence related to item selection: None identiifed

Evidence of re-testing the final version: None identified

Internal consistency (Cronbach's alpha): None Identified

Test-retest Reliability (ICC):

Not applicable

Inter-rater/ inter-interviewer reliability (kappa):

Inter-observer reliability:

Haataja 1999
Correlation Coefficient:
The maximum difference between observers was ≤1
Intra-observer correlation coefficient was close to 1
Population/Disease: Infants; n= 22 (Age for this cohort Not stated but of the full cohort n=135, 92 were assessed at the age of 12 months (range, 11.5 to 13.5 months; mean, 12.2 months) and 43 were assessed at the age of 18 months (range, 17 to 19.5 months; mean, 18.2 months))

Evidence of test-retest or inter-rater reliability: Yes

Concurrent validity (convergent, divergent):

None identified

Known-group validity:

None identified

Evidence of Translatability Assessment: None identified

Evidence related to missing data: None identified

Evidence for Selection of Recall Period: None identified

Evidence of Administration Instructions and Training Provided: Yes

Evidence of concurrent validity: None identified

Evidence of known-groups validity: None identified

Evidence of ability to detect change over time: None identified

Ability to detect change (Responsiveness):

None identified

Evidence of responder thresholds: None identified

Haataja L, Mercuri E, Regev R. Optimality score for the neurologic examination of the infant at 12 and 18 months of age. J Pediatr. 1999 Aug;135(2 Pt 1):153-61

None identified

Yes

Spiranza (EMA, 2017)
Results: Study 1: At the final analysis, a statistically significant greater percentage of patients achieved the definition of a motor milestone responder in the Spinraza group (51%) compared to the sham-control group (0%) (p<0.0001). Statistically significant effects on event-free survival, overall survival, the proportion of patients achieving the definition of a motor milestone responder, and the percentage of patients with at least a 4-point improvement from baseline in Children’s Hospital of Philadelphia Infant Test for Neuromuscular Disease (CHOP INTEND) score were observed in patients in the Spinraza group compared to those in the sham-control group (Table 2). In the efficacy set, 18 patients (25%) in the Spinraza group and 12 patients (32%) in the sham-control group required permanent ventilation. Of these patients, 6 (33%) in the Spinraza group and 0 (0%) in the sham-control group met the protocol-defined criteria for a motor-milestone responder. New data 02/05/2019: Study CS11 (SHINE): Improvements in motor function were observed among patients continuing Spinraza from Study CS3B, as well as those who initiated Spinraza in Study CS11 (Figure 3), with the greatest benefit observed in those with earlier treatment initiation... Further improvement in mean total motor milestone (HINE-Section 2) (2.1; SD 4.36; n=22) and CHOP INTEND (4.68; SD 3.993, n=22) scores were observed from baseline to Study Day 304 in Study CS11... Improvement in mean total motor milestone (HINE-Section 2) (1.2; SD 1.8; n=12) and CHOP INTEND (3.58; SD 7.051, n=12) scores were observed from baseline to Study Day 304 in Study CS11... At this time, 13 out of 20 patients (65%) had met the primary endpoint with a sustained improvement in mean motor milestone achievement over time. Study 3: Patients achieved milestones unexpected in Type I or II SMA and more consistent with Normal development. At the interim analysis, all 25 (100%) patients had achieved the WHO motor milestone of sitting without support, 22 (88%) patients were walking with assistance... All patients had the ability to suck and swallow at last assessment, with 22 (88%) infants achieving a maximal score on the HINE Section 1... A comparison of motor milestone achievement among the patients with symptomatic infantile-onset SMA and pre-symptomatic SMA is shown in Figure 3. [Please see Table 2 and Figure 3 in the subworksheet "Spinraza_EMA_HINE"] Spiranza (FDA, 2016) Results: Of the 82 patients who were eligible for the interim analysis, a statistically significantly greater percentage of patients achieved the definition of a motor milestone responder in the SPINRAZA group (40%) compared to the sham-control group (0%). Results from the final analysis were consistent with those from the interim analysis (Table 3). Fifty-one percent of patients in the SPINRAZA group achieved the definition of a motor milestone responder compared to 0% of patients in the sham-control group. Figure 1 is a descriptive display of the distribution of net change from baseline in the total motor milestone score for Section 2 of the HINE for patients in the final efficacy set who did Not die or withdraw from the study. (See Table 3 and Figure 1) [Please see Table 3 and Figure 1 in the subworksheet "Spinraza_FDA_HINE"]

Original Language: English for the UK

Translations:
Finnish for Finland
French for France
German for Germany
Greek for Greece
Italian for Italy
Polish for Poland
Portuguese for Brazil
Portuguese for Portugal
Spanish for Spain
Ukrainian for Ukraine

Haataja L; Mercuri E; Regev R; Cowan F; Rutherford M; Dubowitz V; Dubowitz L

Hammersmith Neurological Examinations team
E-mail: info@hammersmith-neuro-exam.com

*Free access
*Other: please visit the Hammersmith Neurological Examinations website (http://hammersmith-neuro-exam.com/)

List of items available in Haataja et al. (1999)
Review copy available here: http://hammersmith-neuro-exam.com/recording-scoring-proformas/

None identified