Hypoactive-hypoalert behavior ('psychic akinesia') in intracranial hypotension syndrome

Hypoactive-hypoalert behavior (HHB), also termed “psychic akinesia,” is characterized by loss of psychic autoactivation, apathy, and stereotyped activity that are reversed by external stimulation.1 Lesions in the brainstem, basal ganglia, or frontal lobes can underlie HHB.1 We report three patients presenting with HHB due to spontaneous intracranial hypotension (SIH) syndrome. Two men aged 49 and 57 years and a woman aged 60 years were referred by relatives because of a 2-, 5-, and 2-year history of a chronic state of decreased behavioral initiative, persistent somnolence, impaired attention, and stereotyped motor activity. The patients would lie inactive for whole days if left unstimulated, but would perform adequately whenever incited to. They were scarcely concerned about their ensuing family and job problems and did not complain of mood depression, but came to our observation only because urged to by the alarmed relatives. Neurologic examination showed diffuse deep tendon hyperreflexia variously combined with limitation of vertical and vergence eye movements, lateral gaze-evoked nystagmus, and right extensor plantar response. All patients displayed simple motor perseverations such as buttoning or unbuttoning the shirt, rubbing the face, blowing the nose, hand wringing, washing, rubbing or beating the legs or the trunk, or clearing the throat. They denied orthostatic headache. They fell asleep when left undisturbed but could be easily aroused by external stimulations. Neuropsychological tests showed normal findings …