[Loss of motivation and frontal dysfunction. Role of the white matter change]. / Démotivation et dysfonctionnements frontaux chez le sujet âgé: place de la leucoaraïose.

Since the recognition of white matter changes on computed tomography, researches were done to investigate a possible relation with ageing and cognition. This study examined whether computed tomography evidence of cerebrovascular disease in the form of white matter changes was associated with decreased implicit performance of frontal tests and with a loss of motivation in a group of 10 elderly volunteers with a mild cognitive impairment and in a group of 29 demented patients; 39 old patients (28 females: 82.4 +/- 7.1; 10 males: 75.5 +/- 11.3) cared in a psycho-geriatric day care hospital were enrolled for this essay. Motivation was evaluated with a specific scale: EAD. Patients were tested during the same period with MMSE for cognition, Cornell's scale for depression, Marin's scale for apathy. There were also assessed with a battery of frontal tests: BREF test. A brain scan was used to determinate the presence of leukoaraïosis. Table 1 give a description of the population according to the pathology. Cognitive disorder, but also apathy and loss motivation, frontal evaluation significantly differ in the two studied groups. The presence of a leukoaraïosis is associated with older people, a weaker cognitive status, a more important apathy or loss of motivation, and weaker results with frontal evaluation (table 2). Similar results were obtained considering only the frontal lesions (table 3). Age related changes of the white matter observed on computed tomography were associated with a decreased cognitive status. Leukoaraïosis is associated with loss of motivation and related with a poor results on frontal assessment. Loss of motivation is associated with certain frontal dysfunctions and with brain abnormal scan anomalies.

High-dose chemotherapy with hematopoietic stem cell transplantation in adults with bone marrow relapse of medulloblastoma: report of two cases.

Extraneural relapses of medulloblastoma are associated with a very poor outcome. We present two cases of young adults who developed bone marrow metastases after treatment of medulloblastoma. A very good response to a sequentially scheduled combination of carboplatin and etoposide was observed. Then, high-dose chemotherapy was delivered consisting of busulfan and thiotepa followed by infusion of autologous hematopoietic stem cells. Toxicity of the conditioning regimen was acceptable. The patients remained free of disease 20 and 27 months from the time of relapse, respectively. Further studies are needed to evaluate the impact of high-dose chemotherapy in terms of survival of such patients.

Recurrence of childhood acute lymphoblastic leukemia presenting as a tumor of the middle ear: a case report.

PURPOSE: Extramedullary relapse of childhood acute lymphoblastic leukemia most commonly occurs in the central nervous system or in the testes. Otologic involvement is very rare and has only been reported as an autopsy finding. PATIENT AND METHODS: We describe the case of a 5-year-old girl with CD10 positive acute lymphoblastic leukemia (ALL) who developed an isolated otologic relapse 18 months after the initial diagnosis of ALL. RESULTS: This otologic relapse presented as an atypical otitis media related to a mass of the middle ear. The leukemic infiltration of the middle ear was demonstrated by histologic examination. A cytogenetic change characterized by the occurrence of t(1;19)(q23;p13) was observed in the leukemic cells from the middle ear, and the t(1;19) molecular fusion transcript E2A-PBX1 was detected in the bone marrow by polymerase chain reaction. CONCLUSION: The ear is an exceedingly rare site of relapse in children with acute lymphoblastic leukemia. Molecular analysis demonstrates that such an extramedullary relapse can represent an early manifestation of systemic relapse.