Vessel-based brain-shift compensation using elastic registration driven by a patient-specific finite element model

International audienceDuring brain tumor surgery, planning and guidance are based on pre-operative images which do not account for brain-shift.However, this shift is a major source of error in neuro-navigation systems and affects the accuracy of the procedure. The vascular tree is extracted from pre-operative Magnetic Resonance Angiography and from intra-operative Doppler ultrasound images, which provides sparse information on brain deformations.The pre-operative images are then updated based on an elastic registration of the blood vessels, driven by a patient-specific biomechanical model.This biomechanical model is used to extrapolate the deformation to the surrounding soft tissues.Quantitative results on a single surgical case are provided, with an evaluation of the execution time for each processing step.Our method is proved to be efficient to compensate for brain deformation while being compatible with a surgical process

Vessel-based brain-shift compensation using elastic registration driven by a patient-specific finite element model

International audienceDuring brain tumor surgery, planning and guidance are based on pre-operative images which do not account for brain-shift.However, this shift is a major source of error in neuro-navigation systems and affects the accuracy of the procedure. The vascular tree is extracted from pre-operative Magnetic Resonance Angiography and from intra-operative Doppler ultrasound images, which provides sparse information on brain deformations.The pre-operative images are then updated based on an elastic registration of the blood vessels, driven by a patient-specific biomechanical model.This biomechanical model is used to extrapolate the deformation to the surrounding soft tissues.Quantitative results on a single surgical case are provided, with an evaluation of the execution time for each processing step.Our method is proved to be efficient to compensate for brain deformation while being compatible with a surgical process

Vessel-based brain-shift compensation using elastic registration driven by a patient-specific finite element model

International audienceDuring brain tumor surgery, planning and guidance are based on pre-operative images which do not account for brain-shift.However, this shift is a major source of error in neuro-navigation systems and affects the accuracy of the procedure. The vascular tree is extracted from pre-operative Magnetic Resonance Angiography and from intra-operative Doppler ultrasound images, which provides sparse information on brain deformations.The pre-operative images are then updated based on an elastic registration of the blood vessels, driven by a patient-specific biomechanical model.This biomechanical model is used to extrapolate the deformation to the surrounding soft tissues.Quantitative results on a single surgical case are provided, with an evaluation of the execution time for each processing step.Our method is proved to be efficient to compensate for brain deformation while being compatible with a surgical process

Correction to: Variant of Rett Syndrome and CDKL5 gene: clinical and autonomic description of 10 cases

No abstract available

Variant of Rett Syndrome and CDKL5 gene: clinical and autonomic description of 10 cases

Rett syndrome (RTT) is a severe neurodevelopmental disorder affecting almost exclusively females. The Hanefeld variant, or early-onset seizure variant, has been associated with mutations in CDKL5 gene. AIMS: In recent years more than 60 patients with mutations in the CDKL5 gene have been described in the literature, but the cardiorespiratory phenotype has not been reported. Our aim is to describe clinical and autonomic features of these girls. METHODS: 10 girls with CDKL5 mutations and a diagnosis of Hanefeld variant have been evaluated on axiological and clinical aspects. In all subjects an evaluation of the autonomic system was performed using the Neuroscope. RESULTS: Common features were gaze avoidance, repetitive head movements and hand stereotypies. The autonomic evaluation disclosed eight cases with the Forceful breather cardiorespiratory phenotype and two cases with the Apneustic breather phenotype. CONCLUSIONS: The clinical picture remains within the RTT spectrum but some symptoms are more pronounced in addition to the very early onset of seizures. The cardiorespiratory phenotype was dominated by Forceful breathers, while Feeble breathers were not found, differently from the general Rett population, suggesting a specific behavioral and cardiorespiratory phenotype of the RTT the Hanefeld variant

Variant of Rett Syndrome and CDKL5 Gene: Clinical and Autonomic Description of 10 Cases

No abstract available

Clinical and genetic characteristics of late-onset Huntington's disease

Background: The frequency of late-onset Huntington's disease (>59 years) is assumed to be low and the clinical course milder. However, previous literature on late-onset disease is scarce and inconclusive. Objective: Our aim is to study clinical characteristics of late-onset compared to common-onset HD patients in a large cohort of HD patients from the Registry database. Methods: Participants with late- and common-onset (30–50 years)were compared for first clinical symptoms, disease progression, CAG repeat size and family history. Participants with a missing CAG repeat size, a repeat size of ≤35 or a UHDRS motor score of ≤5 were excluded. Results: Of 6007 eligible participants, 687 had late-onset (11.4%) and 3216 (53.5%) common-onset HD. Late-onset (n = 577) had significantly more gait and balance problems as first symptom compared to common-onset (n = 2408) (P <.001). Overall motor and cognitive performance (P <.001) were worse, however only disease motor progression was slower (coefficient, −0.58; SE 0.16; P <.001) compared to the common-onset group. Repeat size was significantly lower in the late-onset (n = 40.8; SD 1.6) compared to common-onset (n = 44.4; SD 2.8) (P <.001). Fewer late-onset patients (n = 451) had a positive family history compared to common-onset (n = 2940) (P <.001). Conclusions: Late-onset patients present more frequently with gait and balance problems as first symptom, and disease progression is not milder compared to common-onset HD patients apart from motor progression. The family history is likely to be negative, which might make diagnosing HD more difficult in this population. However, the balance and gait problems might be helpful in diagnosing HD in elderly patients