[Psychological and behavioral disorders with good outcome in neurosarcoidosis]. / Troubles psychocomportementaux d'évolution favorable au cours d'une neurosarcoïdose.

INTRODUCTION: Neurological involvement is observed in 5% of cases of sarcoidosis and includes impairment of the central nervous system, the meninges, and the cranial and peripheral nerves. Besides neurological defects, cognitive impairment may be encountered ranging from isolated memory defect to dementia. EXEGESIS: We report a case of neurosarcoidosis occurring in a 40-year-old woman, a native of Reunion Island, with initial meningeal and hypophyseal involvement. Three years later, while treated with low dose prednisolone and methotrexate, she presented a paranoid state associated with cognitive impairment of frontal type and severe behavioral disturbances. After 2 years of high dose steroid treatment associated with hydroxychloroquine, her behavioral status improved, allowing social and familial reinsertion. CONCLUSION: In our observation, sarcoidosis was revealed through a central neurological impairment, with chronic meningitis, facial nerve palsy, and, finally, through psychiatric symptoms and severe behavioral disturbances. A slow favorable outcome was obtained using high dose methylprednisolone and hydroxychloroquine with total regression of behavioral disturbances but with persisting cognitive alteration.

[Characterization of gestural disorders in primary progressive apraxia: diagnostic and nosographic contribution]. / Caractérisation du trouble gestuel dans l'apraxie progressive primaire: apport diagnostique et nosographique.

Five cases are reported of patients with so-called primary progressive apraxia, defined as a slowly worsening disturbance of gestural abilities, without other major cognitive changes during a long period, in relation to degenerative cortical atrophy. All five cases, as other cases in the literature, share the following common features: 1) asymmetrical onset of upper limb clumsiness, more often involving the left side, later involving the contralateral side and lower limbs; 2) after a variable delay, the occurrence of symptoms suggesting subcortical involvement (akinesia, limb stiffness, various kinds of movement disorders, dystonia, paresis of vertical gaze); 3) diffuse cortical atrophy typically more pronounced in the superior parietal cortex opposite to the first side affected. The unusual nature of apraxia in all these cases is pointed out and referred to as Luria's "kinesthaesic apraxia", ascribed to a loss of "selectivity" of distal elementary movements. This pattern of symptoms and their specific outcome could represent a distinct entity.

[Ambulatory autonomy and visual motion perception in a case of almost total cortical blindness]. / Autonomie déambulatoire et perception visuelle du mouvement dans un cas de cécité corticale quasi totale.

A 37-year-old man experienced cortical blindness following a bilateral stroke in the territory of the posterior cerebral arteries. Four years later, the measurement of visual field defects (Goldmann perimeter) showed persistence of bilateral blindness with a 2-degree preservation of macular vision and a perifoveal sparing between 10 to 30 degrees of eccentricity in the left inferior quadrant. Despite this visual impairment, the subject was able to perform visually-guided locomotion. Moreover he consciously perceived visual motion in the blind parts of his visual field. CT and MRI showed a lesion involving most of the striated cortex. The visual cortex located in the internal occipito-parital regions was relatively spared. The contribution of this structure to extra-striated vision of motion is discussed.

[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.