on behalf of the DECIMAL InvestigatorsBackground and Purpose-There is no effective medical treatment of malignant middle cerebral artery (MCA) infarction.The purpose of this clinical trial was to assess the efficacy of early decompressive craniectomy in patients with malignant MCA infarction. Methods-We conducted in France a multicenter, randomized trial involving patients between 18 and 55 years of age with malignant MCA infarction to compare functional outcomes with or without decompressive craniectomy. A sequential, single-blind, triangular design was used to compare the rate of development of moderate disability (modified Rankin scale score Յ3) at 6 months' follow-up (primary outcome) between the 2 treatment groups. Results-After randomization of 38 patients, the data safety monitoring committee recommended stopping the trial because of slow recruitment and organizing a pooled analysis of individual data from this trial and the 2 other ongoing European trials of decompressive craniectomy in malignant MCA infarction. Among the 38 patients randomized, the proportion of patients with a modified Rankin scale score Յ3 at the 6-month and 1-year follow-up was 25% and 50%, respectively, in the surgery group compared with 5.6% and 22.2%, respectively, in the no-surgery group (Pϭ0.18 and Pϭ0.10, respectively). There was a 52.8% absolute reduction of death after craniectomy compared with medical therapy only (PϽ0.0001). Conclusions-In this trial, early decompressive craniectomy increased by more than half the number of patients with moderate disability and very significantly reduced (by more than half) the mortality rate compared with that after medical therapy.

Background: The spectrum of cognitive alterations associated with CADASIL, a model of pure vascular dementia, has not been thoroughly evaluated. Objectives: The aims of this study were: (i) to describe the cognitive profile in CADASIL patients according to age; (ii) to compare the profile of patients with dementia with that of patients without dementia; and (iii) to determine the association between alterations in performance in different cognitive domains. Methods: Forty two consecutive individuals with CADASIL (35-73 years old) were investigated. Cognitive skills were analysed in five domains (executive functions, reasoning, attention, memory, visuospatial abilities) according to age and compared between patients with and without dementia. Associations between cognitive performance and stroke were tested. Results: The youngest patients presented with attention (69%), memory (70%), and executive disturbances (100%). Visuospatial abilities and reasoning deteriorated with age, mainly after the age of 60. About one quarter of patients had dementia, and 75% of these were .60 years of age. Age .60 years was associated with a Rankin score .3 and a significant deficit in all cognitive domains. No association was found between dementia and the number of ischaemic attacks. Episodic memory disorder was characterised by difficulties in retrieval rather than impairment of the encoding process. Conclusion: Cognitive decline in CADASIL is dominated by early impairment of executive functions. Skills in other cognitive domains deteriorate with age and are found to be diffusely impaired in patients with dementia. The relative preservation of the encoding process in episodic memory impairment, even in individuals with dementia, is noteworthy.

The results suggest that apathy is common in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), appears in association with cognitive impairment, global functional disability, and severe neuropsychiatric symptoms during the course of the disease, and can occur separately from depression. Apathy has an independent impact on the overall quality of life in CADASIL.

Malignant middle cerebral artery (MCA) infarction is a devastating condition leading to early death in nearly 80% of cases due to the rapid rise of intracranial pressure despite maximum medical management of the ischaemic brain oedema. 1 Decompressive craniectomy (DC) has been proposed to prevent brain herniation in malignant MCA infarction, but it remains controversial in the absence of randomised controlled trials and because of the fear of a severe residual disability after surgery. 1-4 We present herein the results of a quality of life assessment using patient and proxy versions of the Stroke Impact Scale (SIS) in eight patients 12-30 months after craniectomy for malignant MCA infarcts.

CADASIL is an inherited small-artery disease of the brain due to mutations of the Notch3 gene on chromosome 19. It is characterized by strokes, migraine with aura, and severe mood disturbances during mid adulthood and leads progressively to subcortical dementia. The precise onset of the cognitive decline in CADASIL remains unknown. We report here the cognitive evaluation of 8 non-demented symptomatic patients with CADASIL from 35 to 66 years of age. Altered performances were found in all subjects with the Wisconsin Card-Sorting Test (WCST), in 5/8 with the Trail-Making Test, and in 3/8 with copying of Rey’s figure. Altered performances with codes and similarities of the WAIS-R, the Wechsler Memory Scale, Raven’s Progressive Matrices, and the category and letter fluency task were observed less frequently (n ≤2). The score obtained with the WCST was not significantly correlated with the severity of the white-matter or basal ganglia signal abnormalities at MRI examination. Our data show that: (1) symptomatic CADASIL patients, although non-demented, can present with a subtle cognitive impairment; (2) tasks involving the frontal lobes are found most frequently altered, and (3) this subtle cognitive deficit can develop in the absence of major vascular events and does not appear to be correlated with the severity of brain lesions as seen at MRI examination.