Cortical Activation and Motor Body Representations in a Patient with Subacute Sclerosing Panencephalitis

The current neuroimaging study investigated the sensorimotor maps during hand, feet and lips movements at one year after diagnosis of of subacute sclerosing panencephalitis (SSPE) in a 17 years-old patient. A lesion prediction algorithm showed that the posterior thalamic radiations, the splenium of the corpus callosum, the posterior and superior corona radiate, and the cingolum, showed a high lesion probability. Comparing the fMRI activations of the left and right hemisphere, we found that the representation of the left hand movement was more inferior/anterior and less represented than the representation of the right one; and the representation of the right foot movement was more superior, less represented than the representation of the left one and poorly activated at the predefined statistical threshold. The fMRI results are in line with the clinical report, describing an asymmetrical distribution of the periodic stereotyped myoclonic jerks, which mainly occurred for the left arm/hand and for the right leg/foot. This is the first fMRI study investigating the representation of the body parts in patients with SSPE. Results show that in SSPE the hyper-stimulation of the motor system (dedicated to the arm/hand and leg/foot more involved by the occurrence of the jerks) is accompanied by an under-activation of the corresponding motor representations in coincidence with voluntary movements.