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.