Awake Craniotomy: A Case Series of Anaesthetic Management using a Combination of Scalp Block, Dexmedetomidine and Remifentanil in Hospital Universiti Sains Malaysia.

Awake craniotomy is a brain surgery in patients who are kept awake when it is indicated for certain intracranial pathologies. The anaesthetic management strategy is very important to achieve the goals of the surgery. We describe a series of our first four cases performed under a combination of scalp block and conscious sedation. Scalp block was performed using a mixture of ropivacaine 0.7% and adrenaline 5 5µg/ ml administered to the nerves that innervate the scalp. Conscious sedation was achieved with a combination of two recently available drugs in our country, dexmedetomidine (selective α 2-agonist) and remifentanil (ultra-short acting opioid). Remifentanil was delivered in a target controlled infusion (TCI) mode.

Mutations in SLC39A14 disrupt manganese homeostasis and cause childhood-onset parkinsonism-dystonia.

Although manganese is an essential trace metal, little is known about its transport and homeostatic regulation. Here we have identified a cohort of patients with a novel autosomal recessive manganese transporter defect caused by mutations in SLC39A14. Excessive accumulation of manganese in these patients results in rapidly progressive childhood-onset parkinsonism-dystonia with distinctive brain magnetic resonance imaging appearances and neurodegenerative features on post-mortem examination. We show that mutations in SLC39A14 impair manganese transport in vitro and lead to manganese dyshomeostasis and altered locomotor activity in zebrafish with CRISPR-induced slc39a14 null mutations. Chelation with disodium calcium edetate lowers blood manganese levels in patients and can lead to striking clinical improvement. Our results demonstrate that SLC39A14 functions as a pivotal manganese transporter in vertebrates.