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Table 1 The infantile neuroaxonal rating scale with comments and instructions

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