Table 1 Clinical features and muscle CoQ10 concentrations in 13 patients

Patient no.Age, y/sexAge at onsetAtaxia/cerebellar atrophySeizureDevelopmental motor delayMental retardationOther symptomsMuscle CoQ10, μg/g
* Siblings.
124 /F2 mo+Generalized tonic-clonic+12.8
28 /FBirth++Muscle weakness9.9
39 /F6 y+Psychomotor/ pseudoabsences2.9
411 /M9 y+Myoclonus5.9
59 /M10 mo+++Hemiplegia12.1
612 /F2 y+Generalized tonic-clonic++Spastic paraparesis, muscle weakness11.0
76 /M2 mo+Partial motor+8.2
810 /M5 y+Pyramidal signs12.6
935 /F3 y+++Spastic tetraparesis9.2
1012 /FBirth+++Muscle weakness, ophthalmoparesis12.2
1111 /M9 y+2.9
12*27 /F2 y+Pyramidal signs, distal weakness8.7
13*30 /F8 y+Pyramidal signs, distal weakness, pes cavus14.8