ABSTRACT
OBJECTIVE: Hypothalamic hamartomas are congenital lesions that typically present with gelastic seizures, refractory epilepsy, neurodevelopmental delay, and severe cognitive impairment. Surgical procedures have been reported to be effective in removing the hamartomas, however, they are associated with significant morbidity. Therefore, it is not considered a safe therapeutic modality. Image-guided robotic radiosurgery (CyberKnife® Radiosurgery System) has been shown to provide good outcomes without lasting complications. METHODS: This series of cases describes the clinical, radiological, radiotherapeutic, and postsurgical outcomes of five patients with epileptic encephalopathies secondary to hypothalamic hamartomas who were treated with CyberKnife®. RESULTS: All patients exhibited refractory epilepsy with gelastic seizures and were unsuitable candidates for surgical resection The prescribed dose ranged between 16 and 25 Gy, delivered in a single fraction for four patients and five fractions for one patient while adhering strictly to visual pathway constraints. After radiosurgery, four patients maintained seizure control (one with an Engel class Ia, three with an Engel class 1d), and another presented sporadic, nondisabling gelastic seizures (with an Engel class IIa). After 24-26 months of follow-up, in three patients, their intelligence quotient scores increased. No complications were reported. SIGNIFICANCE: This report suggests that Cyberknife may be a good option for treating hypothalamic hamartoma, particularly in cases where other noninvasive alternatives are unavailable. Nevertheless, additional studies are essential in order to evaluate the effectiveness of the technique in these cases.
ABSTRACT
Created Out of Mind is an interdisciplinary project, comprised of individuals from arts, social sciences, music, biomedical sciences, humanities and operational disciplines. Collaboratively we are working to shape perceptions of dementias through the arts and sciences, from a position within the Wellcome Collection. The Collection is a public building, above objects and archives, with a porous relationship between research, museum artefacts, and the public. This pre-planning framework will act as an introduction to Created Out of Mind. The framework explains the rationale and aims of the project, outlines our focus for the project, and explores a number of challenges we have encountered by virtue of working in this way.
Subject(s)
Anesthesia, Conduction/methods , Anesthesia, Local/methods , Phacoemulsification , HumansABSTRACT
BACKGROUND: Midazolam and propofol are sedative agents commonly administered to patients with brain injury. We compared plasma concentrations of glial cell S100beta protein and nitric oxide (NO) between patients who received midazolam and those who received propofol sedation after severe brain injury, and investigated the association between S100beta and NO concentrations and neurological outcome. DESIGN: 28 patients with severe head injury (Glasgow Coma Score <9) who required sedation and ventilation were randomly assigned to receive midazolam (n =15) or propofol (n = 13) based sedation. Blood samples were drawn daily for 5 days for estimation of S100beta and NO concentrations. Neurological outcome was assessed 3 months later as good (Glasgow Outcome Score [GOS], 4-5) or poor (GOS, 1-3). RESULTS: A good neurological outcome was observed in 8/15 patients (53%) in the midazolam group and 7/13 patients (54%) in the propofol group. Patients with a poor outcome had higher serum S100beta concentrations on ICU admission and on Days 1-4 in the ICU than those with a good outcome (mean [SD] on Day 1, 0.99 [0.81] v 0.41 [0.4] microg/L; Day 2, 0.80 [0.81] v 0.41 [0.24] microg/L; Day 3, 0.52 [0.55] v 0.24 [0.25] microg/L; and Day 4, 0.54 [0.43] v 0.24 [0.35] microg/L; P<0.05). There was no significant difference on Day 5. Plasma NO concentrations were not associated with outcome. In subgroup analysis, there was no difference in S100beta and NO concentrations between patients with a good outcome versus those with a poor outcome in either the midazolam or propofol group. CONCLUSIONS: Plasma concentrations of markers of neurological injury in patients with severe head injury were similar in those who received midazolam sedation and those who received propofol. Patients who had a poor neurological outcome at 3 months had consistently higher serum S100beta concentrations during the initial 4 days after injury than patients who had a good outcome.
