[MIM 157 600, 614 508, 616 059]

(Isolated congenital mirror movements, congenital contralateral familial syncinesis , isolated congenital contralateral syncinesis)

Prevalence estimated at < 1 / 10-6.  Autosomal dominant transmission with variable penetrance, some cases of autosomal recessive inheritance. In about 35 % of the cases: mutation of the RAD51, DCC or DNAL4 gene. Pathology of the lateralization of the movements without clinical signs or other symptoms in its isolated form. Can be associated with syndromes such as Gorlin, Kallman, Klippel-Feil, Joubert, Moebius, Seckel, Wildervanck (see these topics) and some congenital hemiplegias. This pathology appears to be due to a defect in crossing of the motor pathways at the connection between the two cerebral hemispheres during movement or to an abnormality in programming of the movements of the hands. Clinically: the initiation of a voluntary movement with one hand results in the unintentional initiation of the same movement by the other hand.

The severity is evaluated as follows:

- 1: barely visible but repeated mirror movements

- 2: mirror movements that are either small and sustained or strong and rare

- 3: important and repeated mirror movements

- 4: mirror movements similar to or more powerful than the initial contralateral movement.

No treatment: avoid tasks where a bimanual activity is necessary.

Anesthetic implications:

none for the isolated forms; According to the others anomalies in the forms associated with a syndrome

References : 

-         Franz EA, Chiaroni-Clarke R, Woodrow S et al.
Congenital mirror movements: phenotypes associated with DCC and RAD51 mutations.
J Neurol Sci 2015; 351: 140-5.

-        Sag E, Gocmen R, Yildiz FG, Ozturk Z, Temucin C, Teksam O, Utine E.
Congenital mirror movements in Gorlin syndrome: a case report with DTI and functional MRI features.
Pediatrics 2016; 137:e20151771

Updated: January 2019