It should be considered that such a clinical observation is only meaningful when the child has already developed the basic motor functions. Especially with DEB this is not always the case.

Furthermore, before starting the observation the child should be inspected to note the degree of blistering and skin injury. In the case of severe acute blistering on the soles of the feet, knees, abdomen or back it should be considered whether the examination can be performed.

In the initial interviews with parents and the examinations of children with EB aged between 1.5 and 4 years it appears that many of these children show delayed motor development. The parental questionnaire focusing on early motor development on p. 18 can be used to discover whether the reasons are EB specific. This information can be very useful for therapists in interpreting the assessment. For example when the child first walked unsupported at 2.5 years, this must be taken into consideration when observing the vestibular reaction at 4 years.

Blistering may have an influence on performance, especially on tasks carried out in the prone position (e. g. prone extension) or on all fours (e. g. tonic neck reflex test).

Performance of tasks for the vestibular system (e. g. Schilder’s arm-extension test) may also be affected by blisters on the soles of the feet.

Similarly some of the additional items such as standing on one foot or hopping may cause problems. Some relief may be achieved by performing the tasks on a gymnastic mat, but then it must be remembered that the soft supporting surface requires a higher level of vestibular performance than a hard floor. It may be wise to leave out some tasks to prevent blistering and pain. Hopping, for example can put a lot of strain on many children.

When assessing tonic postural or support reactions any contractures and imbalance of muscle function in the knees, hips, trunk and neck must be taken into consideration.

The full pronation and supination may be affected by contractures and must be considered when assessing diadochokinesia.

Checking the slow motions of arms to shoulders and the return to full extension, which is one of the criteria, may be limited by the restricted elbow extension in children with EB.

Children with mutilations, pseudosyndactyly and contractures in the hands as a result of RDEB-HS (recessive DEB Hallopeau-Siemens) have very severe limitations of finger movement (cf. Laimer et al. 2003).

The testing of the thumb–finger opposition is often impossible.

Foot deformities and webbing of the toes, especially the big toe, have a large influence on stability while standing or walking and on equilibrium.

There may be complications involving the eyes in children with JEB and DEB. When testing eye movements, following a moving object may be affected by scarring (cf. Laimer et al. 2003).

Inflammation and hypersensitivity to light may also be present.

Some children with DEB und JEB may have microstomia (small mouth) and adhesions of the tongue and gums (cf. Laimer et al. 2003) so that observation of the tongue and lip movements may be restricted.

For tasks with the ball an inflatable ball should be used to prevent injury.

Localisation of touch on the hands: The therapist touches different places on the hand with a light paintbrush. The child has his/her eyes closed and should point to the touched place.

To achieve standard values the item ‘Finger localisation’ from the Miller Assessment for Preschoolers (MAP)¹ may be used.

Two-point discrimination on the hands (meaningful from the age of approximately 6–7 years): Using a two-point discrimination tester (see Fig. 3.2) the therapist touches the fingers or palm of the hand while the child has his/her eyes closed. The child should be able to feel this and say whether it is one or two points.

The standard value on the tip of the index finger is 2–5 mm, whereas on the proximal phalanx it is 6–10 mm (cf. Waldner-Nilsson 2009).

Texture discrimination with different surfaces: The child is given small blocks with different surfaces in each hand and, with his/her eyes shut, must say whether the two are the same or different.

Stereognosis: The child feels typical everyday objects in a bag, such as a pencil, marble, spoon or building block, with his/her eyes shut and either names the object or points to the same object on the table. From the age of 5 years, shapes such as a square, triangle, star or moon may be used to take into account the child’s increasing abilities.