Huntington's disease (HD) is one of a group of neurodegenerative diseases caused by an expanded trinucleotide (CAG) repeat coding for an extended polyglutamine tract. The disease is inherited in an autosomal dominant manner, with onset of motor, cognitive, and psychiatric symptoms typically occurring in midlife, followed by unremitting progression and… (More)
Huntington's disease (HD) is a genetically transmitted neurodegenerative disorder. The neuropathology in HD is a selective neuronal cell death in several brain regions including cortex. Although changes in synaptic plasticity were shown within the hippocampus and striatum of HD transgenic mice, there are no studies considering neocortical synaptic… (More)
BACKGROUND Huntington's disease (HD) is caused by a tandem repeat expansion and involves progressive cognitive decline, psychiatric abnormalities and motor deficits. Disease onset and progression in HD mice can be substantially delayed by a housing environment with enhanced sensorimotor and cognitive stimulation. However, the proposed benefits of… (More)
PURPOSE Selective dorsal rhizotomy (SDR) is a surgical technique used to treat spasticity in children secondary to cerebral palsy (CP). METHOD We report, to the best of our knowledge for the first time, the case of a child who underwent SDR for the management of spasticity secondary to Leigh syndrome. RESULT SDR resulted in excellent functional outcome… (More)
PURPOSE Selective dorsal rhizotomy (SDR) is a neurosurgical procedure used to treat spasticity in children with cerebral palsy (CP). The vast majority of studies to date suggest SDR is particularly effective in reducing lower limb spasticity in spastic diplegia with long-lasting effect. METHOD We report, to the best of our knowledge for the first time,… (More)
We report the case of a 14-month-old infant presenting with unresponsiveness and seizure following thoracic surgery. Imaging showed full territory left middle cerebral artery infarct and signs of raised intracranial pressure (ICP) that required emergency decompressive craniectomy (DC). The child made a good functional recovery. We discuss the case.
We report a case of accidental intracranial insertion of a foreign body through the nose resulting in dural tear and subsequent CSF leak. We also describe the surgical approach to remove it and a new method to seal the dural defect.
We report a case of an interventricular ependymoma which was surgically removed but also required bilateral transection of the fornices resulting in memory impairment which gradually improved. Functional MR images using a memory paradigm showed that recovery correlated with cortical plasticity involving activation of the orbitofrontal cortex and the middle… (More)