From: The infantile neuroaxonal dystrophy rating scale (INAD-RS)
Item name and score definitions | Comments, instructions | |
---|---|---|
1. | Gross Motor Skills | Consider aids of two small blocks, a small handheld bell, small spoon, stuffed animal or other bright toy to test visual tracking |
1. Hold head upright against gravity while sitting • Child cannot hold head erect for at least 3 s without support (Score = 0) • Child holds head erect for at least 3 s without support (Score = 1) • Child holds head erect and steady for at least 15 s without support (Score = 2) | • Typically performed on exam table | |
2. Roll over • Child does not roll front to back or back to front (Score = 0) • Child rolls front to back or back to front, but not both (Score = 1) • Child rolls front to back and back to front (Score = 2) | • Typically performed on exam table | |
3. Sit with support • Child cannot sit with support (Score = 0) • Child tenses muscles in an effort to maintain sitting position (Score = 1) • Child sits with slight support for at least 30 s (Score = 2) | • Typically performed on exam table | |
4. Sit without support • Child cannot sit without support (Score = 0) • Child sits without support for at least 5 s (Score = 1) • Child sits without proper support for at least 30 s (Score = 2) | • Typically performed on exam table | |
5. Stand aided • Child cannot stand aided (Score = 0) • Child can stand aided (Score = 1) • Child raises self to a standing position, using a chair or other convenient object for support | ||
6. Stand unaided a. Child cannot stand unaided (Score = 0) b. Child can stand alone for at least 3 s after you release his or her hands (Score = 1) c. Child comes to a standing position without using any support (Score = 2) | ||
7. Does head lag with dynamic change of position? a. Child cannot hold head when raised from supine to sitting by pulling on the arms (Score = 0) b. Child has head lag when raised from supine to sitting by pulling on the arms (Score = 1) c. Child has no head lag when raised from supine to sitting by pulling on the arms (Score = 2) | • Have child lie on table | |
8. Peripheral limb function: Hand a. Child has no hand grip and/or contractures (Score = 0) b. Child shows finger grip (pincer) (Score = 1) c. Child holds object in hand (Score = 2) | • Give child block or small toy | |
9. Peripheral limb function: Feet a. Child has contractures of both feet (Score = 0) b. Child has pes equinus or pes cavus without contracture (Score = 1) c. Child has no foot deformity (Score = 2) | ||
10. Crawling a. Child cannot crawl (Score = 0) b. Child moves from lying prone to being up on hands and knees (Score = 1) c. Child makes forward progress of at least 5 ft by crawling on hands and knees (Score = 2) | ||
11. Walk aided a. Child cannot walk with support (Score = 0) b. Child walks with support by a person and initiates multiple steps (Score = 1) c. Child walks independently while using or holding onto support (Score = 2) | ||
12. Walk unaided a. Child cannot walk without support (Score = 0) b. Child takes at least 3 steps without support, even if gait is stiff-legged and wobbly (Score = 1) c. Child takes at least 5 steps independently, displaying coordination and balance (Score = 2) | ||
2. | Fine Motor Skills | |
1) Reaches for objects • Child does not reach for an object (Score = 0) • Child extends one or both arms forward to reach object, but does not touch object (Score = 1) • Child extends one or both arms forward to reach object and touches object with any part of either hand (Score = 2) | • Ensure object is light (e.g. light-weight block, light-weight bell) | |
2) Grasps small objects • Child cannot pick up block (Score = 0) • Child picks up block using one or both hands (Score = 1) • Child uses pad of his or her thumb and any fingertip to grasp block (Score = 2) | ||
3) Picks up food or spoon • Child cannot pick up food pellet or spoon (Score = 0) • Child grasps food pellet or spoon, but does not bring it to his/her mouth (Score = 1) • Child grasps food pellet or spoon and brings it to his/her mouth (Score = 2) | • Use plastic spoon, put in child’s hand and see if brings to mouth | |
4) Rings bell • Child does not reach for bell (Score = 0) • Child extends one or both arms forward to reach bell and touches bell with any part of either hand (Score = 1) • Child picks up bell and attempts to ring bell (Score = 2) | ||
5) Transfer objects • Child does not grasp ring when handed (Score = 0) • Child uses at least one hand to grasp ring for at least 2 s (Score = 1) • Child grasps ring and transfers from hand to hand (Score = 2) | ||
6) Place one block on another • Child does not attempt to place one block on another (Score = 0) • Child attempts to place one block on another, but is unsuccessful (Score = 1) • Child is successful to place one block on another (Score = 2) | ||
3. | Bulbar Function | |
1) Swallows saliva • Child drools most of the time, requiring bib or several shirt changes per day (Score = 0) • Child drools occasionally (does not require a bib or a shirt change) (Score = 1) • Child does not drool (Score = 2) | • History based | |
2) Swallows pureed food • Child cannot eat pureed food (Score = 0) • Child can occasionally eat pureed food (Score = 1) • Child can eat pureed food with no problem (Score = 2) | • History based | |
3) Swallows solid food (including soft foods) • Child cannot eat solid food (Score = 0) • Child can occasionally eat solid food (Score = 1) • Child can eat solid food with no problem (Score = 2) | • History based | |
4) Bite strength • Absent (Score = 0) • Weak (Score = 1) • Strong (Score = 2) | • Can place gloved hand in mouth to assess bite strength • Alternatively can be history based by asking parents / caregivers | |
5) Nourishes liquids by syringe or tube feeding • Syringe feeding or tube feeding only (Score = 0) • Syringe feeding or tube feeding most of the time or occasional (Score = 1) • No syringe or tube feeding (Score = 2) | ||
6) Tube feeding • Permanent (Score = 0) • Occasional (Score = 1) • Never (Score = 2) | ||
7) Upper Airway • Tracheotomy or CPAP support (Score = 0) • Child has sleep apnea (Score = 1) • Child has normal sleep respiration (score = 2) | ||
4. | Ocular | |
1. Nystagmus • Child has nystagmus most of the time (Score = 0) • Child has occasional nystagmus (Score = 1) • Child has no nystagmus (Score = 2) | • Observe at rest, some have constant nystagmus, some only brief when shifting gaze | |
2. Strabismusa • Severe (Score = 0) • Moderate (Score = 1) • Mild/No Strabismus (Score = 2) | • Severe Strabismus: Constant exotropia • Moderate Strabismus: Exotropia > 50% of the exam before dissociation, or Exotropia < 50% of the exam before dissociation • Mild Strabismus: No exotropia unless dissociated, recovers in > 5 s; no exotropia unless dissociated, recovers in 1–5 s; or no exotropia unless dissociated, recovers in < 1 s (phoria) | |
3. Tracks human face • Child does not track human face (score = 0) • Child fixes gaze on a person for at least 2 s (score = 1) • Child turns head to follow a person through the room (score = 2) | • Asses if they track face from around 1 ft. away | |
4. Tracks object • Child does not track an object (score = 0) • Child’s eyes follow an object that is moved horizontally or vertically (Score = 1) • Child’s eyes follow an object that is moved in a circular motion (Score = 2) | • Use small toy | |
5. Optic atrophy/temporal pallor • Child has severe optic atrophy/temporal pallor (Score = 0) • Child has moderate optic atrophy/temporal pallor (Score = 1) • Child has mild or no optic atrophy/temporal pallor (Score = 2) | • Either by way of indirect fundoscopy or slit-lamp exam by pediatric ophthalmologist | |
5. | Temporo-frontal | |
1) Interacts with parents or examiner • Child does not interact with parent or examiner (Score = 0) • Child clearly responds to the person’s voice (Score = 1) • Child actively participates in at least one play routine (Score = 2) | • Helpful to have parents elicit emotional response to parental voice or movement | |
2) Responds to verbal commands • Child does not respond to verbal commands (Score = 0) • Child stops reaching for objects in response to “no,” but does not respond in an appropriate manner to other requests (Score = 1) • Child responds in an appropriate manner to at least one spoken request more complex than “no” (does not need to complete task) (Score = 2) | ||
3) Repeats simple sounds • Child does not repeat simple sounds (Score = 0) • Child repeats a single vocalization only (Score = 1) • Child repeats two different, distinct vocalizations (Score = 2) | ||
4) Smiles • Child does not smile nor vocalize mood (Score = 0) • Child expresses at least one mood (Score = 1) • Child’s mood or focus can change in response to speaker’s attention (Score = 2) | ||
5) What is the child’s affect? • Sad, distressed or crying a lot (Score = 0) • Neutral affect (Score = 1) • Happy, ebullient, or cooperative (Score = 2) | ||
6) Speaks individual words • Child does not speak individual words (Score = 0) • Child imitates at least one word, even if imitation consists of vowels only (Score = 1) • Child uses at least one word to make wants known (Score = 2) | ||
7) Puts words together • Child does not use words (Score = 0) • Child uses at least one word to make wants known (Score = 1) • Child produces at least one utterance that includes two or more words (Score = 2) | ||
8) Point to objects in a book • Child does not attempt to point to an object in a book (Score = 0) • Child points to object in a book, but does not identify object that was named (Score = 1) • Child points to object in a book that was named (Score = 2) | ||
6. | Autonomic Nervous System | |
1) Constipation • Child has fewer than 2 bowel movements per week and is dependent on a laxative (Score = 0) • Child has 2 or more bowel movements per week and is dependent on a laxative (Score = 1) • Child has 2 or more bowel movements per week without a laxative (Score = 2) | • History based | |
2) Urinary • Indwelling catheter or dependent upon catherization (Score = 0) • Catherization no more than once per day (Score = 1) • No catherization required (Score = 2) | • History based |