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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