MRI hypoperfusion as a determinant of cognitive impairment in adults with Moyamoya angiopathy.

INTRODUCTION: In Moyamoya angiopathy (MMA), mechanisms underlying cognitive impairment remain debated. We aimed to assess the association of cognitive impairment with the degree and the topography of cerebral hypoperfusion in MMA. METHODS: A retrospective analysis of neuropsychological and perfusion MRI data from adults with MMA was performed. Ischemic and haemorrhagic lesion masks were created to account for cerebral lesions in the analysis of cerebral perfusion. Whole brain volume of hypoperfused parenchyma was outlined on perfusion maps using different Tmax thresholds from 4 to 12 s. Regional analysis produced mean Tmax values at different regions of interest. Analyses compared perfusion ratios in patients with and without cognitive impairment, with multivariable logistic regression analysis to identify predictive factors. RESULTS: Cognitive impairment was found in 20/48 (41.7%) patients. Attention/processing speed and memory were equally impaired (24%) followed by executive domain (23%). After adjustment, especially for lesion volume, hypoperfused parenchyma volume outlined by Tmax > 4 s or Tmax > 5 s thresholds was an independent factor of cognitive impairment (OR for Tmax > 4 s = 1.06 [CI 95% 1.008-1.123]) as well as attention/processing speed (OR for Tmax > 4 s = 1.07 [CI 95% 1.003-1.133]) and executive domains (OR for Tmax > 5 s = 1.08 [CI 95% 1.004-1.158]). Regarding cognitive functions, patients with processing speed and flexibility impairment had higher frontal Tmax compared to other ROIs and to patients with normal test scores. DISCUSSION: Cerebral hypoperfusion emerged as an independent factor of cognitive impairment in MMA particularly in attention/processing speed and executive domains, with a strong contribution of frontal areas. CONCLUSION: Considering this association, revascularization surgery could improve cognitive impairment.

The Impact of Surgery on the Survival of Patients with Recurrent Glioblastoma.

OBJECTIVE: The purpose of this study was to investigate the possible benefit of repeat surgery on overall survival for patients with recurrent glioblastoma multiforme (GBM). METHODS: We performed a retrospective analysis of data from patients who presented with recurrent GBM over a 5-year period (n = 157), comparing baseline characteristics and survival for patients who had at least 1 new tumor resection followed by chemotherapy (reoperation group, n = 59) and those who received medical treatment only (no-reoperation group, n = 98) for recurrence. RESULTS: The baseline characteristics of the two groups differed in terms of WHO performance status (better in the reoperation group), mean age (60 years in the reoperation group vs. 65 years in the no-reoperation group), mean interval to recurrence (3 months later in the reoperation group than in the no-reoperation group) and more gross total resections in the reoperation group. Nevertheless, the patients in the reoperation group had a higher rate [32.8%] of sensorimotor deficits than those of the no-reoperation group [14.2]. There was no significant difference in sex; tumor localization, side, or extent; MGMT status; MIB-1 labeling index; or Karnofsky Performance Status [KPS] score. After adjustment for age, the WHO performance status, interval of recurrence, and extent of resection at the first operation, multivariate analysis showed that median survival was significantly better in the reoperation group than in the no-reoperation group (22.9 vs. 14.61 months, P < 0.05). After a total of 69 repeat operations in 59 patients (10 had 2 repeat surgeries), we noted 13 temporary and 20 permanent adverse postoperative events, yielding a permanent complication rate of 28.99% (20/69). There was also a statistically significant (P = 0.029, Student's t-test) decrease in the mean KPS score after reoperation (mean preoperative KPS score of 89.34 vs. mean postoperative score of 84.91). CONCLUSION: Our retrospective study suggests that repeat surgery may be beneficial for patients with GBM recurrence who have good functional status (WHO performance status 0 and 1), although the potential benefits must be weighed against the risk of permanent complications, which occurred in almost 30% of the patients who underwent repeat resection in this series.

Case Report: Successful Cerebral Revascularization and Cardiac Transplant in a 16-Year-Old Male With Syndromic BRCC3-Related Moyamoya Angiopathy.

Background: BRCC3/MTCP1 deletions are associated with a rare familial moyamoya angiopathy with extracranial manifestations. Case: We report the case of an adolescent male presenting with progressive and symptomatic moyamoya angiopathy and severe dilated cardiomyopathy caused by a hemizygous deletion of BRCC3/MTCP1. He was treated for renovascular hypertension by left kidney homograft and right nephrectomy in infancy and had other syndromic features, including cryptorchidism, growth hormone deficiency, and facial dysmorphism. Due to worsening of the neurological and cardiac condition, he was treated by a direct superficial temporal artery to middle cerebral artery bypass to enable successful cardiac transplant without cerebral damage. Conclusions: BRCC3-related moyamoya is a devastating disease with severe heart and brain complications. This case shows that aggressive management with cerebral revascularization to allow cardiac transplant is feasible and efficient despite end-stage heart failure.

Clinical long-term consequences of thoraco-lumbar spine fracture and osteosynthesis.

OBJECT: Although traumatic spine fractures can be treated by osteosynthesis, their long-term clinical, social, and familial consequences are less known. The aim of this study was to assess these global consequences to a very long-term (at least more than 12 years after the fracture). METHODS: Two groups, one composed of 30 patients operated for a thoracolumbar fracture by posterior fixation and one with 30 controls (who never had a spinal fracture) matched for age, sex, job and time of follow-up were studied. Patients and control subjects had to answer to 3 questionnaires: one about clinical, familial, and socio-professional changes, and 2 back pain (Dallas and Eifel) scales. RESULTS: The mean patient follow-up was 14.5 years (from 12 to 18 years, sd 2.3) - control subjects, 15 years. The majority (56%) of the fractures occurred at T12/L1 level. At last follow-up, the chronic low back pain concerned 20 (66,7%) patients versus 11 (36.7%) control subjects (p=0.03); more patients (13 patients - 43.3%) consumed analgesics than control (5 persons - 16.6%) subjects (p=0.04). A large majority (13 patients, 57%) had sick leaves that exceeded 6 months. The loss of wage due to traumatism or chronic low back pain was also significant (p=0.002) between patients and matched controls over the period. At follow-up, the mean Eifel score for the whole patients' cohort was significaty superior compared to control group (4.7 [sd 3.75] vs. 2.6 [sd 4.2], p=0.008). Dallas score was superior in the patient's group for the daily, work-leisure activities and sociability aspect (p<0.05). CONCLUSION: Chronic back pain, long sick leaves, changes in professional and familial life, the very long-term postoperative outcome of patients could be more difficult than expected in a majority of patients operated for thoracolumbar fracture. In order to facilitate the back to work and reduce these long-term consequences, we propose that guidelines about job resume in traumatic spinal fractures should be established along with early occupational medicine consultations. LEVEL OF EVIDENCE: III; retrospective case control study.

Reconstruction of Cranial Vault Defect with Polyetheretherketone Implants.

OBJECT: Reconstruction of a cranial vault defect is a frequent challenge in neurosurgery. Polyetheretherketone (PEEK) is used in many types of prostheses and has been employed for 10 years in our institution (University Hospital of Toulouse, France). The objectives of this study are to describe the benefits and drawbacks of reconstructing the cranial vault defect with a PEEK prosthesis. METHODS: Clinical data of the 37 patients who received a reconstruction with a custom-made PEEK prosthesis from 2007-2015 were retrospectively analysed. Operative technique, postoperative complications, and patient's satisfaction with the aesthetic result-on a scale ranging from 1 (very dissatisfied) to 5 (very satisfied)-were studied. RESULTS: Average follow-up was 4.3 years (from 2 months-9 years). The placement of the prosthesis was performed 195 days on average (from 0-1051 days, standard deviation 258 days) after the initial bone flap removal. One infection (2.7%), which required the removal of the prosthesis, was described. Six patients (16%) were reoperated by the maxillofacial surgery team to treat a lack of temporal projection related to muscle atrophy, using a fat cell autograft taken from the abdominal region. Overall, 30 patients (81%) answered the question about their aesthetic satisfaction, with good results on the satisfaction scale (average 4.5; from 3-5). CONCLUSION: The use of a PEEK prosthesis in cranial vault defect reconstruction is a reliable technique with a high patient satisfaction rate and few complications. Corrections of the temporal muscle atrophy by fat grafting may be performed in addition, without increasing the rate of complications.