Article

The aims of this review were (1) to provide insight into the natural
course of upper-extremity (UE) impairments and UE activity limitations associated
with facioscapulohumeral dystrophy (FSHD) and limb-girdle muscular dystrophies
(LGMD), and (2) to provide an overview of outcome measures used to evaluate UE
function and activity in patients with FSHD and LGMD.
METHODS: Scientific
literature databases (PubMed, MEDLINE, EMBASE, CINAHL and Cochrane) were searched
for relevant publications. INCLUSION CRITERIA: (1) studies that included persons
with a diagnosis of FSHD or LGMD; and (2) studies that reported the natural
course of the UE functions and/or activity with outcome measures at these levels.
RESULTS: 247 publications were screened, of which 16 fulfilled the selection
criteria. Most studies used manual muscle testing (MMT) to evaluate UE function
and the Brooke Scale to evaluate UE mobility activities. The clinical picture of
UE impairments and limitations of UE activities in FSHD and LGMD patients was
highly variable. In general, FSHD and LGMD patients experience difficulty
elevating their upper extremities and the execution of tasks takes considerably
longer time. CONCLUSIONS: The clinical course of UE impairments and activity
limitations associated with FSHD and LGMD is difficult to predict due to its high
variability. Although measures like MMT and the Brooke Scale are often used,
there is a lack of more specific outcome measures to assess UE function and UE
capacity and performance in daily life. Measures such as 3D motion analysis and
electromyography (EMG) recordings are recommended to provide additional insight
in UE function. Questionnaires like the Abilhand are recommended to assess UE
capacity and accelerometry to assess UE performance in daily life. IMPLICATIONS FOR REHABILITATION: There is a need for specific outcome measures on the level of
UE activity. Both the level of capacity and performance should be assessed.
Possible outcome measures include 3D motion analysis to assess UE function,
questionnaires like the Abilhand to assess UE capacity and accelerometry to
assess performance of UE activities in daily life.

Langue : ANGLAIS