ATL1
-related spastic paraplegia SPG3A is a pure form of hereditary spastic paraplegia. Rare complex phenotypes have been described, but few data concerning cognitive evaluation or molecular imaging ...of these patients are available. We relate a retrospective collection of patients with SPG3A from the Neurology Department of Nancy University Hospital, France. For each patient were carried out a
18
F-FDG PET (positron emission tomography), a electromyography (EMG), a sudoscan®, a cerebral and spinal cord MRI (magnetic resonance imaging) with measurement of cervical and thoracic surfaces, a neuropsychological assessment. The present report outlines standardised clinical and paraclinical data of five patients from two east-France families carrying the same missense pathogenic variation, NM_015915.4(ATL1): c.1483C > T p.(Arg495Trp) in
ATL1
. Mean age at onset was 14 ± 15.01 years. Semi-quantitatively and in comparison to healthy age-matched subjects, PET scans showed a significant cerebellar and upper or mild temporal hypometabolism in all four adult patients and hypometabolism of the prefrontal cortex or precuneus in three of them. Sudoscan® showed signs of small fibre neuropathy in three patients. Cervical and thoracic patients’ spinal cords were significantly thinner than matched-control, respectively 71 ± 6.59mm
2
(
p
= 0.01) and 35.64 ± 4.35mm
2
(
p
= 0.015). Two patients presented with a dysexecutive syndrome. While adding new clinical and paraclinical signs associated with
ATL1
pathogenic variations, we insist here on the variable penetrance and expressivity. We report small fibre neuropathy, cerebellar hypometabolism and dysexecutive syndromes associated with SPG3A. These cognitive impairments and PET findings may be related to a cortico-cerebellar bundle axonopathy described in the cerebellar cognitive affective syndrome (CCAS).
Breakthroughs in 3D Sequential technology Brunet, L.; Fenouillet-Beranger, C.; Batude, P. ...
2018 IEEE International Electron Devices Meeting (IEDM),
2018-Dec.
Conference Proceeding
The 3D sequential integration, of active devices requires to limit the thermal budget of top tier processing to low temperature (LT) (i.e. \mathrm{T}_{\text{TOP}}=500^{\circ}\mathrm{C}) in order to ...ensure the stability of the bottom devices. Here we present breakthrough in six areas that were previously considered as potential showstoppers for 3D sequential integration from either a manufacturability, reliability, performance or cost point of view. Our experimental data demonstrate the ability to obtain 1) low-resistance poly-Si gate for the top FETs, 2) Full LT RSD epitaxy including surface preparation, 3) Stability of intermediate BEOL between tiers (iBEOL) with standard ULK/Cu technology, 4) Stable bonding above ULK, 5) Efficient contamination containment for wafers with Cu/ULK iBEOL enabling their re-introduction in FEOL for top FET processing 6) Smart Cut™ process above a CMOS wafer.
