Search

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 …

MRI showed a cortically-based partially cystic and markedly enhancing mass in the uncus of the right temporal lobe in a patient with long standing refractory partial complex seizures. Histopathological examination revealed a pleomorphic xanthoastrocytoma, a rare, usually benign tumour thought to originate from subpial astrocytes.

Bit-mapped multicomponent CNV complex and reaction time (RT) were recorded and measured in 24 presenile patients with initial symptoms of very mild to moderately severe primary mental deterioration without depression, and in 10 age-matched controls. All patients underwent CT and MRI examinations, EEG spectral analysis and a battery of psychometric test. Significant group differences were obtained for measures of some post-S1 ERP and CNV components, particularly of the post-S1 N1b, P300 and early and late pre-S2 CNV. P300 with increased latency, no significant CNV activity, very prolonged RTs, EEG slowing down and diffuse brain atrophy were observed in the majority of patients with probable presenile Alzheimer's dementia. These results suggest that CNV/RT and EEG activity changes similar to those observed in our patients may constitute a valuable clue for the study of brain dysfunction in the early stage of presenile idiopathic cognitive impairment.

MRI findings of bilateral central macrogyria allowed the diagnosis of a congenital variant of Foix-Chavany-Marie syndrome in four patients aged between 13 and 32 years, with facio-pharyngo-glosso-masticatory central diplegia, mental retardation and seizures.