[Neuropsychological and scintigraphic aspects of frontotemporal dementia preceding amyotrophic lateral sclerosis]. / Aspects neuropsychologiques et scintigraphiques des démences fronto-temporales précédant l'atteinte du motoneurone.

Between 1993 and 2001, we observed fifteen patients (ten men and five women, mean age 63 years) with frontotemporal dementia (FTD) which preceded signs of amyotrophic lateral sclerosis (ALS) which developed 21 months later. Mean disease duration in the fourteen deceased patients was 38 months. FTD associated with ALS is characterized by rapid course, predominance of disinhibited forms (orbito-basal), presence of aphasia with neologisms, and semantic memory disorders. Performed in all patients, single-photon emission computed tomography demonstrated a bifrontal pattern of low uptake, sometimes associated with low uptake in the anterior temporal region. In one patient, neuropathology revealed neuron atrophy and loss in the frontotemporal region, the anterior horns, and the hypoglossal nucleus. Ubiquitin-positive inclusions were visible in the dentate gyrus of the hippocampus and in the anterior horns. The dementia/ALS association is classically described is uncommon. It belongs to the FTD group since the Lund and Manchester consensus. Approximately 15 p.100 of patient with FTD can be expected to develop ALS. About 250 cases have been reported in the literature, half of them in the Pacific area where the incidence of ALS is high (55/100,000 inhabitants versus 1/100,000 in the rest of the world). Intermediary forms of FTD, semantic dementia, and progressive non-fluent aphasia are discussed since several cases of non-fluent progressive aphasia associated with ALS are reported in the literature. The links between these two degenerative diseases are discussed.

[Congenital oculomotor apraxia with corpus callosum agenesis and subtentorial atrophy]. / Apraxie oculomotrice congénitale avec agénésie du corps calleux et atrophie sous-tentorielle.

A 7 year-old child with congenital oculomotor apraxia (Cogan's syndrome) presented with mainly voluntary horizontal saccadic movements. Fixation of a visual target could be obtained only by a sudden movement of the head preceded by eyelid blinking. Electro-oculography confirmed the predominant disturbance of saccadic movements (delay in onset, hypometria). CT scan showed agenesis of the corpus callosum and atrophy of the vermis together with enlargement of the 4th ventricle and pre-pontine cisternae. The role of these anomalies in the pathogenesis of this rare congenital syndrome of good prognosis is discussed.

[Diagnosis of hematomas in the Retzius space during anticoagulant therapy. Prospective study (11 cases)]. / Diagnostic de l'hématome de l'espace de Retzius sous traitement anticoagulant. Etude prospective (onze cas).

During a three-year, prospective study carried out at the Saint Jacques Hospital, Nantes, France, in a group of 33 patients with echographically identified intra-abdominal hematoma, 11 cases of Retzius' space hematoma were detected in patients under anticoagulant treatment (heparin or antivitamin K). This suggests that the frequency of this complication is greater than supposed. The authors emphasize two aspects of Retzius' space hematoma: on one hand, clinical symptomatology, especially involving the urinary system (transient oliguria or oligoanuria in 7 of the 11 cases), on the other, ultrasonic diagnosis (slip-page of the intraperitoneal contents along the posterior surface of the hematoma, rupture sign, denoting diffusion of the hematoma immediately below the Douglas' arch). Ordinarily, the clinical course of this complication of rectus sheath hematoma is spontaneously favorable.