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Table 1 Moser-Raymond Severity Scoring System: The scoring system used in this analysis to determine the clinical status of patients with ALD was previously developed by Moser and Raymond [17]

From: Chitotriosidase as a biomarker of cerebral adrenoleukodystrophy

Hearing/auditory processing problems 1

1

Aphasia/apraxia

1

Loss of communication

3

Vision impairment/fields cut

1

Cortical blindness

2

Swallowing difficulty or other central nervous system dysfunction

2

Tube feeding

2

Running difficulties/hyper-reflexia

1

Walking difficulties/spasticity/spastic gait (no assistance)

1

Spastic gait (needs assistance)

2

Wheelchair required

2

No voluntary movement

3

Episodes of incontinency

1

Total incontinency

2

Nonfebrile seizures

1

Possible Total

25

  1. A score for each patient was established at baseline (prior to transplantation) and at 1 year following transplantation. The difference (delta) is presented as the clinical neurologic progression to one year after transplant in Figures 3 and 4.