Deep brain stimulation in a young child with GNAO1 mutation - Feasible and helpful.

Background: GNAO1 is an emerging disorder characterized with hypotonia, developmental delay, epilepsy, and movement disorder, which can be potentially life threatening during acute exacerbation. In the USA, deep brain stimulation (DBS) has been licensed for treating children with chronic, treatment-resistant primary dystonia, who are 7 years old or older. Case Description: A 4-year-old girl diagnosed to have GNAO1-related dyskinesia and severe global developmental delay. She had severe dyskinesia precipitated by intercurrent infection, requiring prolonged intensive care for heavy sedation and related complications. Her dyskinesia improved dramatically after DBS implantation. Technical difficulties and precautions of DBS in preschool children were discussed. Conclusion: DBS should be considered early in the treatment of drug-resistant movement disorders in young children with GNAO1, especially after dyskinetic crisis, as they tend to recur. Presurgical counseling to parents and close monitoring of complications is also important in the process.

Blessing or burden? Long-term maintenance, complications and clinical outcome of intrathecal baclofen pumps.

AIM: The intrathecal baclofen pump is an effective treatment for spasticity. However, long-term results have reported patients' dissatisfaction and perception of disability. Potential causes include a frequent need for baclofen pump refill and risks of complications. The aim of the present study was to evaluate the long-term maintenance, complications and clinical outcome of intrathecal baclofen pumps. PATIENTS AND METHODS: We conducted a 16-year retrospective cohort study of patients with spasticity treated with an intrathecal baclofen pump at a university hospital from 2000 to 2016. The primary outcome was the rate of infection per puncture for baclofen pump refill. Secondary outcomes included the incidence of other complications, such as running out of baclofen causing symptomatic withdrawal symptoms, pump mechanical failure, pump battery end of life and the need for pump replacement. The clinical outcome was assessed by the Modified Ashworth Scale (mAS). RESULTS: In total, 340 follow-up episodes with pump refill procedures were recorded. The average interval between each pump refill was 57.3 days (±15.4 days). The average duration of admission for each pump refill was 4 h and 49 min (from 2 h 23 min to 10 h). There were two events with established infection after puncture for the refill, giving rise to an infection rate per puncture of 0.6 percent (2/340).For the long-term clinical outcome, at an average follow-up period of 7.6 years, the postoperative mAS for spasticity was 2.0 ± 0.756, which was significantly better than the preoperative mAS at 3.75 ± 0.462 (P = 0.001). CONCLUSION: Long-term aftercare with baclofen pump refill was safe, with an infection rate of 0.6 per cent per puncture for each refill. Long-term intrathecal baclofen pump was effective in the treatment of spasticity with persistent significant improvement in the spasticity scale.

Early Cerebral Infarction after Aneurysmal Subarachnoid Hemorrhage.

Aneurysmal subarachnoid hemorrhage (SAH) is a serious disease with high case fatality and morbidity. Early cerebral infarction has been suggested as a risk factor for poor outcome. We aimed to assess the pattern of early and delayed cerebral infarction after aneurysmal subarachnoid hemorrhage. We prospectively enrolled consecutive aneurysmal subarachnoid hemorrhage (SAH) patients presenting to an academic neurosurgical referral center (Prince of Wales Hospital, the Chinese University of Hong Kong) in Hong Kong. Cerebral infarction occurred in 24 (48 %) patients, in which 14 (28 %) had early cerebral infarction and 14 (28 %) had delayed cerebral infarction. Early anterior cerebral infarction occurred in a similar proportion of anterior and posterior circulation aneurysms (24 % vs. 21 %), whereas posterior circulation aneurysm patients had a higher proportion of early posterior cerebral infarction compared with anterior circulation aneurysm patients (18 % vs. 2 %). In conclusion, early cerebral infarction was common and different from delayed cerebral infarction.

Intracranial aneurysm size responsible for spontaneous subarachnoid haemorrhage.

INTRODUCTION: It has been theorised that the relationship between smaller body size and smaller ruptured intracranial aneurysms in Asians indirectly supports the treatment of small, unruptured intracranial aneurysms. There has also been uncertainty regarding whether the progress that has been made in neuroimaging allows for better detection of smaller ruptured intracranial saccular aneurysms. Therefore, we conducted this systemic review of ruptured intracranial saccular aneurysm sizes according to region and time. MATERIAL AND METHODS: Computerised MEDLINE and PubMed searches of the literature for population-based studies of ruptured intracranial saccular aneurysms were carried out from 1 January 1980 to 1 March 2011. Statistical analyses were generated using SPSS for Windows, Version 15.0 (SPSS Inc., Chicago, IL) and Comprehensive MetaAnalysis 2.0 for Windows (Biostat, Englewood, NJ). The results of the meta-analyses are presented with 95% confidence intervals (CIs). RESULTS: Six eligible population- or hospital-based studies were analysed. The percentage of ruptured intracranial aneurysms measuring less than 5 mm was 28.4% (95% CI: 18.1% to 41.6%, I(2) = 98%). The percentage of ruptured intracranial aneurysms measuring less than 10 mm was 76.7% (95% CI: 69.2% to 82.9%, I2 = 89%). A higher proportion of patients with ruptured intracranial aneurysms of less than 5 mm was found in Asia compared to other regions. Similarly, a higher proportion of patients with ruptured intracranial aneurysms of less than 10 mm was found in Asia compared to other regions. CONCLUSIONS: The present findings suggest that ruptured intracranial aneurysms are smaller in Asians and should be confirmed in future prospective international multi-centre registries to assess ethnicity. Whether these findings support treating smaller unruptured intracranial aneurysms in Asians should be investigated.