Mutism and auditory agnosia due to bilateral insular damage--role of the insula in human communication.

We report a case of transient mutism and persistent auditory agnosia due to two successive ischemic infarcts mainly involving the insular cortex on both hemispheres. During the 'mutic' period, which lasted about 1 month, the patient did not respond to any auditory stimuli and made no effort to communicate. On follow-up examinations, language competences had re-appeared almost intact, but a massive auditory agnosia for non-verbal sounds was observed. From close inspection of lesion site, as determined with brain resonance imaging, and from a study of auditory evoked potentials, it is concluded that bilateral insular damage was crucial to both expressive and receptive components of the syndrome. The role of the insula in verbal and non-verbal communication is discussed in the light of anatomical descriptions of the pattern of connectivity of the insular cortex.

[Changes in personality and hypertension. The "athymhormic" syndrome]. / Modifications de la personnalité et hypertension artérielle. Le "syndrome athymhormique".

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)

[Athymhormic syndrome caused by bilateral striato-capsular infarction. Moyamoya disease in adults]. / Syndrome athymhormique par infarctus striatocapsulaire bilatéral. Maladie de Moyamoya de l'adulte.

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.

[Post-traumatic interhemispheric disconnection syndrome]. / Syndrome de déconnexion interhémisphérique post-traumatique.

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.

[Isolated memory disorders disclosing antiphospholipid antibody syndrome]. / Troubles mnésiques isolés révélant un syndrome des anticorps anti-phospholipides.

Most of the neurological complications of the antiphospholipid syndrome are consequences of arterial or venous cerebral thromboses. We report the case of a 46-year-old woman presenting with the main signs of antiphospholipid syndrome: Raynaud's phenomenon, livedo, leg ulcers, repeated miscarriages, presence of a circulating anticoagulant and of antiphospholipid antibodies, who developed an isolated amnestic syndrome with a peculiar pattern; 1) almost complete sparing of the ability to learn new skills and of short-term recall; 2) deterioration, followed by disappearance of recent memories after a sufficient delay; 3) progressive alteration of increasingly old memories including knowledge memory suggested a gradual alteration of mnestic traces. The lack of neuroradiological signs of multiple infarcts suggests a direct intervention of antibodies in this patient's memorisation mechanisms.

[Behavior and mental activity disorders after carbon monoxide poisoning. Bilateral pallidal lesions]. / Troubles du comportement et de l'activité mentale après intoxication oxycarbonée. Lésions pallidales bilatérales.

Two patients who had suffered severe carbon monoxide intoxication showed lasting neuropsychologic sequelae: 1) deep inertia involving the whole behaviour and expressed by an almost complete lack of activity if not induced by someone else; a mental gap when the patients were left to themselves and a tendency to give up the mental activity when stimuli ceased; an apparent affective indifference connected with a lack of spontaneous expression of the affects; 2) pseudo-obsessionnal activities: coprolalia with sexual themes in one patient, obsessive collecting and tidying up activities in the other one. Neurologic examination was normal, particularly no parkinsonian syndrome was present. CT scans showed bilateral pallidal low density areas. Both patients had moderate intellectual impairment in psychometric tests and amnestic disorders. There is a possible relationship between memory and cognitive impairment and mental inertia. The onset of pseudo-obsessional signs following basal ganglia lesions is emphasized. The central semeiological fact seems to be a disorder of the initiation and the carrying on of any external action as well as mental activity itself. This may be related with the activity disorders represented at a more elementary level by motion impairment in parkinsonian akinesia.

[Amnesic syndrome in sarcoidosis]. / Syndrome amnésique au cours d'une sarcoïdose.

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.

[Transient Korsakoff's syndrome after intraventricular hemorrhage]. / Syndrome de Korsakoff transitoire au décours d'une hémorragie intraventriculaire.

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.

A pure reversible amnesic syndrome following tuberculous meningoencephalitis.

The authors report the case of a 33-year-old right-handed man who developed a pure classical amnesic syndrome during the course of a probable tuberculous meningitis. This amnesic syndrome occurred 3 months after the onset of the illness, after the patient had stopped the treatment and it regressed completely after the antituberculous treatment had been recommenced. The neuroradiological investigations suggested that both the mamillary bodies and the medial temporal lobe regions were involved in this amnesic syndrome.