Biomedical subjects
Publications and source records attributed to M L Royère.
A left-handed man presented with a partial hemispheric disconnection syndrome of traumatic origin composed by a left hand unilateral agraphia, a left hand tactile anomia, a left visual field anomia, a left visual field alexia and a partial left ear extinction for verbal material. MRI of the brain showed lesion involving the trunk of the corpus callosum.
A 42 year-old man with intraventricular haemorrhage developed a transient Korsakoff's syndrome. A small arteriovenous malformation was identified on the inner side of the left parietal lobe. Cognitive disorders are very frequent after surgery on anterior communicating artery aneurysms, more rarely when the haemorrhage interest the axial anatomic loci implicating in the process of amnesic syndromes. This case illustrates well the possibility of transient Korsakoff's syndrome after pure intraventricular haemorrhage.
In 3 hypertensive patients, aged 57 to 66, profound behavioral and personality changes occurred rather abruptly, characterized by total loss of spontaneous activity and initiative, apathetic behavior, passivity, lack of drive and motivation, loss of interest for any of previous occupations and hobbies, and total flatness of affect. Neurological examination was normal or only showed mild extra-pyramidal signs. Neuropsychological evaluation was only remarkable for mild intellectual impairment suggestive of frontal lobe dysfunction. None of the 3 patients fulfilled criteria for dementia or severe depression. This neurobehavioral syndrome has been coined "athymhormic syndrome" (Habib & Poncet, 1988), a term emphasizing the specific defect in drive ("horme") and affect ("thumos"). Electrical and clinical heart examination was unremarkable. Blood pressure was always found within normal limits during hospitalization, including 24-hour monitoring in one case. However, all patients were known as hypertensive in the past, with repeated bouts of high blood pressure (up to 270 mmHg systolic in one case). X-ray CT-scan was usually normal or showed non-specific white matter changes (so-called "leukoaraiosis"). In all 3 cases, a brain MRI scan showed multiple small infarcts mainly involving deep subcortical structures (caudate nuclei and/or adjacent periventricular white matter) of both hemispheres, consistent with the definition of lacunes.(ABSTRACT TRUNCATED AT 250 WORDS)
We report a case of neurosarcoidosis characterized by central nervous system involvement 6 years after the onset of the disease. Our case is remarkable for the gradual development of an amnesic syndrome. Although neurological examinations gave normal results, the semeiological profile of the cognitive disorder suggests the presence of a hypothalamic sarcoid lesion involving the basal forebrain.
A 49 year-old women with no medical history suddenly presented bilateral striato-capsular infarct causing frontal-like behavioural disturbances associating inertia with loss of drive, interest and affect, and preservation of intellectual function ("athymhormic syndrome" or "loss of psychic self-activation"). Ischaemic lesions mainly affected right globus pallidus and left lentiform nucleus with no involvement of the caput of the nuclei caudati. Such changes were close to anoxic lesions known to cause the same symptomatology. Infarction was attributed to a Moyamoya disease on angiographic data. Bilateral involvement of basal ganglia, arterial borderzones or both, may explain the frequent occurrence of neuropsychological disturbances in Moyamoya disease.