Skip to main content

Table 2 Patients’ symptoms before and after treatment with intrathecal baclofen

From: Treatment of motor and behavioural symptoms in three Lesch-Nyhan patients with intrathecal baclofen

Patient 1 2 3
Before ITB Dystonia: 22 23 38
UDRS total a
Aggression Yes No Yes
Self-injury b:    
Mouth/lip biting 2-3 2 1-2
Finger biting 10 20 3-4
Punching 10 0 2-3
Restraint Permanent whole body restraint Permanent finger protection None
Sleep c:    
Awakenings 7-8 5-6 5-6
Sleep hours 2 4 4
Date of ITB implantation and age (years) 29/04/2013 – 19 28/02/2014 – 39 19/03/2014 – 20
ITB dosage and follow-up duration (months) 380 μg /day – 16 270 μg /day – 6 550 μg /day – 5
After ITB Dystonia: 5 6.5 11
UDRS total a
Aggression No No No
Self-injury b:    
Mouth/lip biting 0-1 0 0
Finger biting 3 5 0
Punching 0 0 0
Restraint Occasional, right arm None None
Sleep c:    
Awakenings 1-2 1 0
Sleep hours 5 6 5
Notes Improved verbal communication - Persistent moderate nausea, daytime drowsiness
  1. Legend: a) Dystonia was scored using the UDRS scale [3]. Detailed scores are available in Table 3. b) Self-injury was scored by counting the daily episodes of different self-injurious behaviours, following interviews with caregivers. c) Quality of sleep was scored counting the number of awakenings per night and the average hours of uninterrupted sleep, following interviews with caregivers.