• Journal of Korean Neurosurgical Society
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• v.39 no.6
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• pp.438-442
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• 2006

The sylvian aqueduct syndrome is a global rostral midbrain dysfunction induced by a transtentorial pressure gradient through the aquaeductus. Several months after ventriculoperitoneal shunt, a patient with hydrocephalus began experiencing a constellation of midbrain dysfunction symptoms, including bradykinesia, medial longitudinal fasciculus syndrome, third nerve palsy, and mutism. These were indicative of cerebral aqueduct syndrome. In addition, the patient showed posture-dependent underdrainage or overdrainage. All symptoms were resolved after distal catheter elongation and floating cranioplasty. We present a case of reversible parkinsonism, which developed in a patient with shunted hydrocephalus and aqueductal stenosis, and discuss the diagnosis and treatment of the sylvian aqueduct syndrome. We also review the literature to address problems of drainage and potential treatment modalities.

• Journal of Korean Neurosurgical Society
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• v.62 no.6
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• pp.643-648
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• 2019

Objective : Shunt-dependent hydrocephalus (SdHCP) is a well-known complication of aneurysmal subarachnoid hemorrhage (SAH). The risk factors for SdHCP have been widely investigated, but few risk scoring systems have been established to predict SdHCP. This study was performed to investigate the risk factors for SdHCP and devise a risk scoring system for use before aneurysm obliteration. Methods : We reviewed the data of 301 consecutive patients who underwent aneurysm obliteration following SAH from September 2007 to December 2016. The exclusion criteria for this study were previous aneurysm obliteration, previous major cerebral infarction, the presence of a cavum septum pellucidum, a midline shift of >10 mm on initial computed tomography (CT), and in-hospital mortality. We finally recruited 254 patients and analyzed the following data according to the presence or absence of SdHCP : age, sex, history of hypertension and diabetes mellitus, Hunt-Hess grade, Fisher grade, aneurysm size and location, type of treatment, bicaudate index on initial CT, intraventricular hemorrhage, cerebrospinal fluid drainage, vasospasm, and modified Rankin scale score at discharge. Results : In the multivariate analysis, acute HCP (bicaudate index of ${\geq}0.2$) (odds ratio [OR], 6.749; 95% confidence interval [CI], 2.843-16.021; p=0.000), Fisher grade of 4 (OR, 4.108; 95% CI, 1.044-16.169; p=0.043), and an age of ${\geq}50years$ (OR, 3.938; 95% CI, 1.375-11.275; p=0.011) were significantly associated with the occurrence of SdHCP. The risk scoring system using above parameters of acute HCP, Fisher grade, and age (AFA score) assigned 1 point to each (total score of 0-3 points). SdHCP occurred in 4.3% of patients with a score of 0, 8.5% with a score of 1, 25.5% with a score of 2, and 61.7% with a score of 3 (p=0.000). In the receiver operating characteristic curve analysis, the area under the curve (AUC) for the risk scoring system was 0.820 (p=0.080; 95% CI, 0.750-0.890). In the internal validation of the risk scoring system, the score reliably predicted SdHCP (AUC, 0.895; p=0.000; 95% CI, 0.847-0.943). Conclusion : Our results suggest that the herein-described AFA score is a useful tool for predicting SdHCP before aneurysm obliteration. Prospective validation is needed.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.25-28
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• 2016

Phelan-McDermid syndrome is a rare genetic disorder caused by the terminal or interstitial deletion of the chromosome 22q13.3. Patients with this syndrome usually have global developmental delay, hypotonia, and speech delays. Several putative genes such as the SHANK3, RAB, RABL2B, and IB2 are responsible for the neurological features. This study describes the clinical features and outcomes of Korean patients with Phelan-McDermid syndrome. Two patients showing global developmental delay, hypotonia, and speech delay were diagnosed with Phelan-McDermid syndrome via chromosome analysis, fluorescent in situ hybridization, and multiplex ligation-dependent probe amplification analysis. Brain magnetic resonance imaging of Patients 1 and 2 showed delayed myelination and severe communicating hydrocephalus, respectively. Electroencephalography in patient 2 showed high amplitude spike discharges from the left frontotemporoparietal area, but neither patient developed seizures. Kidney ultrasonography of both the patients revealed multicystic kidney disease and pelviectasis, respectively. Patient 2 experienced recurrent respiratory infections, and chest computed tomography findings demonstrated laryngotracheomalacia and bronchial narrowing. He subsequently died because of heart failure after a ventriculoperitoneal shunt operation at 5 months of age. Patient 1, who is currently 20 months old, has been undergoing rehabilitation therapy. However, global developmental delay was noted, as determines using the Korean Infant and Child Development test, the Denver developmental test, and the Bayley developmental test. This report describes the clinical features, outcomes, and molecular genetic characteristics of two Korean patients with Phelan-McDermid syndrome.