Unconsciousness or unresponsiveness in Akinetic Mutism? Insights from a
multimodal longitudinal exploration
Abstract
The clinical assessment of patients with disorders of consciousness
(DoC) relies on the observation of behavioral responses to standardized
sensory stimulation. However, several medical comorbidities may directly
impair the production of reproducible and appropriate responses, thus
reducing the sensitivity of behavior-based diagnoses. One of these is
Akinetic Mutism (AM), a rare neurological syndrome characterized by the
inability to initiate volitional motor responses, sometimes associated
with clinical presentations overlapping with those of DoC. Here we
describe the case of a patient with large bilateral mesial frontal
lesions showing a prolonged behavioral unresponsiveness and a severe
disorganization of electroencephalographic (EEG) background, compatible
with a vegetative state/unresponsive wakefulness syndrome (VS/UWS). By
applying an unprecedented battery of multimodal longitudinal
measurements encompassing spontaneous EEG, evoked potentials,
event-related potentials, transcranial magnetic stimulation-evoked
potentials, and structural and functional MRI, we provide (i) a
demonstration of the preservation of consciousness despite
unresponsiveness in the context of a complete AM, (ii) a plausible
neurophysiological explanation of behavioral unresponsiveness and of its
subsequent recovery during rehabilitation stay and (iii) novel insights
into the relationships between DoC, AM and parkinsonism. The present
case provides proof-of-principle evidence supporting the clinical
utility of a multimodal hierarchical workflow combining conventional and
advanced techniques to detect covert signs of consciousness in
unresponsive patients.