• Clinical and Experimental Pediatrics
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• 제56권8호
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• pp.351-354
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• 2013

Isovaleric aciduria (IVA) is caused by an autosomal recessive deficiency of isovaleryl-CoA dehydrogenase (IVD). IVA presents either in the neonatal period as an acute episode of fulminant metabolic acidosis, which may lead to coma or death, or later as a "chronic intermittent form" that is associated with developmental delays, with or without recurrent acidotic episodes during periods of stress, such as infections. Here, we report the case of a 2-year old boy with IVA who presented with the chronic intermittent form. He was admitted to Asan Medical Center Children's Hospital with recurrent vomiting. Metabolic acidosis, hyperammonemia, elevated serum lactate and isovalerylcarnitine levels, and markedly increased urine isovalerylglycine concentration were noted. Sequence analysis of the IVD gene in the patient revealed the novel compound mutations-a missense mutation, c.986T>C (p.Met329Thr) and a frameshift mutation, c.1083del (p.Ile361fs$^*11$). Following stabilization during the acute phase, the patient has remained in a stable condition on a low-leucine diet.

• Journal of Korean Neurosurgical Society
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• 제51권2호
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• pp.86-90
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• 2012

Objective : Life-threatening hemispheric stroke is associated with a high mortality and morbidity. Decompressive hemicraniectomy has been regarded as an effective treatment option for refractory intracranial hypertension. Here, we reported the clinical course of 5 children with decompressive craniectomy and duroplasty after non-traumatic refractory intracranial hypertension. Methods : Four toddlers and one preschool-girl were included in this study; there were 3 boys and 2 girls with a mean age of 34.6 months (range 17-80). Decompressive craniectomy including duroplasty was performed in cases of dilatation of pupil size after intensified standard medical therapy had proven insufficient. All children had a Pediatric Glasgow Coma Scale score <8 at pre-operation state. The mean time-point of craniectomy after stroke attack was 12 hours (range 4-19). Results : During the long-term follow-up period (mean 47.6 months), no children died. One year later, when we checked their Glasgow Outcome Scale scores, only one toddler received a score of 4 (moderate disability). But the others had good recoveries although they had minor physical or mental deficits. According to the Pediatric Cerebral Performance Category Scale, 4 children received a score of 2 (mild disability). Conclusion : Despite our small cases, we suggest that decompressive hemicraniectomy and duroplasty is an acceptable and life-saving treatment for refractory intracranial hypertension after unilateral hemispheric stroke in toddlers and preschool children.

• Journal of Korean Neurosurgical Society
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• 제55권6호
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• pp.348-352
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• 2014

Objective : Management guidelines for single intracranial hematomas have been established, but the optimal management of multiple hematomas has little known. We present bilateral traumatic supratentorial hematomas that each has enough volume to be evacuated and discuss how to operate effectively it in a single anesthesia. Methods : In total, 203 patients underwent evacuation and/or decompressive craniectomies for acute intracranial hematomas over 5 years. Among them, only eight cases (3.9%) underwent operations for bilateral intracranial hematomas in a single session. Injury mechanism, initial Glasgow Coma Scale score, types of intracranial lesions, surgical methods, and Glasgow outcome scale were evaluated. Results : The most common injury mechanism was a fall (four cases). The types of intracranial lesions were epidural hematoma (EDH)/intracerebral hematoma (ICH) in five, EDH/EDH in one, EDH/subdural hematoma (SDH) in one, and ICH/SDH in one. All cases except one had an EDH. The EDH was addressed first in all cases. Then, the evacuation of the ICH was performed through a small craniotomy or burr hole. All patients except one survived. Conclusion : Bilateral intracranial hematomas that should be removed in a single-session operation are rare. Epidural hematomas almost always occur in these cases and should be removed first to prevent the hematoma from growing during the surgery. Then, the other hematoma, contralateral to the EDH, can be evacuated with a small craniotomy.

• Journal of Korean Neurosurgical Society
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• 제55권1호
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• pp.26-31
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• 2014

Objective : Early decompressive craniectomy (DC) has been used as the first stage treatment to prevent secondary injuries in cases of severe traumatic brain injury (TBI). Postoperative management is the major factor that influences outcome. The aim of this study is to investigate the effect of postoperative management, using intracranial pressure (ICP) monitoring and including consecutive DC on the other side, on the two-week mortality in severe TBI patients treated with early DC. Methods : Seventy-eight patients with severe TBI [Glasgow Coma Scale (GCS) score <9] underwent early DC were retrospectively investigated. Among 78 patients with early DC, 53 patients were managed by conventional medical treatments and the other, 25 patients were treated under the guidance of ICP monitoring, placed during early DC. In the ICP monitoring group, consecutive DC on the other side were performed on 11 patients due to a high ICP of greater than 30 mm Hg and failure to respond to any other medical treatments. Results : The two-week mortality rate was significantly different between two groups [50.9% (27 patients) and 24% (6 patients), respectively, p=0.025]. After adjusting for confounding factors, including sex, low GCS score, and pupillary abnormalities, ICP monitoring was associated with a 78% lower likelihood of 2-week mortality (p=0.021). Conclusion : ICP monitoring in conjunction with postoperative treatment, after early DC, is associated with a significantly reduced risk of death.

• Journal of Korean Neurosurgical Society
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• 제55권1호
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• pp.1-4
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• 2014

Objective : The case fatality rate of nonlesional intracerebral hemorrhage (n-ICH) was high and not changed. Knowing the causes is important to their prevention; however, the reasons have not been studied. The aims of this study were to determine the cause of death, to improve the clinical outcomes. Methods : We retrospectively analyzed consecutive cases of nonlesional intracerebral hemorrhage in a prospective stroke registry from January 2010 to December 2010. Results : Among 174 patients ($61.83{\pm}13.36$, 28-90 years), 29 patients (16.7%) died during hospitalization. Most common cause of death was initial neurological damage (41.4%, 12/29). Seventeen patients who survived the initial damage may then develop various potentially fatal complications. Except for death due to the initial neurological sequelae, death associated with immobilization (such as pneumonia or thromboembolic complication) was the most common in eight cases (8/17, 47.1%). However, death due to early rebleeding was not common and occurred in only 2 cases (2/17, 11.8%). Age, initial Glasgow Coma Scale, and diabetes mellitus were statistically significant factors influencing mortality (p<0.05). Conclusion : Mortality of n-ICH is still high. Initial neurological damage is the most important factor; however, non-neurological medical complications are a large part of case fatality. Most cases of death of patients who survived from the first bleeding were due to complications of immobilization. These findings have implications for clinical practice and planning of clinical trials. In addition, future conduct of a randomized study will be necessary in order to evaluate the benefits of early mobilization for prevention of immobilization related complications.

• Journal of Korean Neurosurgical Society
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• 제42권4호
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• pp.326-330
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• 2007

Objective : Stroke is the most prevalent disease involving the central nervous system. Since medical modalities are sometimes ineffective for the acute edema following massive infarction, surgical decompression may be an effective option when medical treatments fail. The present study was undertaken to assess the outcome and prognostic factors of decompressive surgery in life threatening acute, severe, brain infarction. Methods : We retrospectively analyzed twenty-six patients (17 males and 9 females; average age, 49.7yrs) who underwent decompressive surgery for severe cerebral or cerebellar infarction from January 2003 to December 2006. Surgical indication was based on the clinical signs such as neurological deterioration, pupillary reflex, and radiological findings. Clinical outcome was assessed by Glasgow Outcome Scale (GOS). Results : Of the 26 patients, 5 (19.2%) showed good recovery, 5 (19.2%) showed moderate disability, 2 (7.7%) severe disability, 6 (23.1%) persistent experienced vegetative state, and 8 (30.8%) death. In this study, the surgical decompression improved outcome for cerebellar infarction, but decompressive surgery did not show a good result for MCA infarction (30.8% overall mortality vs 100% mortality). The dominant-hemisphere infarcts showed worse prognosis, compared with nondominant-hemisphere infarcts (54.5% vs 70%). Poor prognostic factors were diabetes mellitus, dominant-hemisphere infarcts and low preoperative Glasgow Coma Scale (GCS) score. Conclusion : The patients who exhibit clinical deterioration despite aggressive medical management following severe cerebral infarction should be considered for decompressive surgery. For better outcome, prompt surgical treatment is mandatory. We recommend that patients with severe cerebral infarction should be referred to neurosurgical department primarily in emergency setting or as early as possible for such prompt surgical treatment.

• Journal of Korean Neurosurgical Society
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• 제44권5호
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• pp.295-302
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• 2008

Objective: Premorbid demographic backgrounds of injured individuals are likely to reflect more accurately the status of patients with traumatic brian injury (TBI) than clinical factors. However, the concrete study about the relationship between the demographic factors and neurocognitive function in TBI patients has not been reported. The object of this study was to evaluate the effect of premorbid demographic factors on the recovery of neurocognitive function following TBI. Methods: From July 1998 to February 2007, 293 patients (male: 228, female: 65) with a history of head injury, who had recovered from the acute phase, were selected from our hospital to include in this study. We analyzed the effect of premorbid demographic factors including age, sex, educational level and occupation on the recovery of neurocognitive function in each TBI subgroup as defined by Glasgow Coma Scale (GCS) score. Intelligence and memory are components of neurocognitive function, and the Korean Wechsler Intelligence Scale (K-WAIS) and the Korean memory assessment scale (K-MAS) were used in this study. The results were considered significant at p<0.05. Results: The higher level of education was a good prognostic factor for intelligence regardless of GCS score and younger age group showed a better result for memory with an exception of severe TBI group. In the severe TBI group, the meaningful effect of demographic factors was not noted by the cause of influence of severe brain injury. Conclusion: The demographic factors used in this study may be helpful for predicting the precise prognosis and developing an appropriate rehabilitation program for TBI patients.

• Journal of Korean Neurosurgical Society
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• 제63권5호
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• pp.631-639
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• 2020

Objective : Traditionally, staged surgery has been preferred in the treatment of compound comminuted depressed fracture (FCCD) after traumatic brain injury (TBI) and involves the removal of primarily damaged bone and subsequent cranioplasty. The main reason for delayed cranioplasty was to reduce the risk of infection-related complications. Here, the author performed immediate reconstruction using a titanium mesh in consecutive patients with FCCD after TBI, reported the surgical results, and reviewed previous studies. Methods : Nineteen consecutive patients who underwent single-stage reconstruction with titanium mesh for FCCD of the skull from April 2014 to June 2018 were retrospectively analyzed. The demographic and radiological characteristics of the patients with FCCD were investigated. The characteristics associated with surgery and outcome were also evaluated. Results : The frequency of TBI in men (94.7%) was significantly higher than that in women. Most FCCDs (73.7%) occurred during work, the rest were caused by traffic accidents. The mean interval between TBI and surgery was 7.0±3.9 hours. The median Glasgow coma scale score was 15 (range, 8-15) at admission and 15 (range, 10-15) at discharge. FCCD was frequently located in the frontal (57.9%) and parietal (31.6%) bones than in other regions. Of the patients with FCCDs in the frontal bone, 62.5% had paranasal sinus injury. There were five patients with fractures of orbital bone, and they were easily reconstructed using titanium mesh. These patients were cosmetically satisfied. Postoperatively, antibiotics were used for an average of 12.6 days. The mean hospital stay was 17.6±7.5 days (range, 8-33). There was no postoperative seizure or complications, such as infection. Conclusion : Immediate bony fragments replacement and reconstruction with reconstruction titanium mesh for FCCD did not increase infectious sequelae, even though FCCD involved sinus. This suggests that immediate single-stage reconstruction with titanium mesh for FCCD is a suitable surgical option with potential benefits in terms of cost-effectiveness, safety, and cosmetic and psychological outcomes.

• Journal of Korean Neurosurgical Society
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• 제40권1호
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• pp.11-15
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• 2006

Objective : Acute cerebral infarction is often accompanied by transtentorial herniation which can be fatal. The aim of this study is to determine the timing of surgical intervention and prognostic factors in patients who present with acute cerebral infarction. Methods : We reviewed retrospectively 23 patients with acute cerebral infarction, who received decompressive craniectomy or conservative treatment from January 2002 to December 2004. We divided patients into two groups according to the treatment modalities [Group 1 : conservative treatment, Group 2 : decompressive craniectomy]. In all patients, the outcome was quantified with Glasgow Outcome Scale and Barthel Index. Results : Of the 23 patients, 11 underwent decompressive craniectomy. With decompressive craniectomy at the time of loss of pupillary light reflex, we were able to prevent death secondary to severe brain edema in all cases. Preoperative Glasgow Coma Scale and loss of pupillary light reflex were significant to the clinical outcome statistically. With conservative treatment, 9 of the 12 patients died secondary to transtentorial herniation. The clinical outcomes of remaining 3 patients were poor. Conclusion : This study confirms the value of life-saving procedure of decompressive craniectomy after acute cerebral infarction. We propose that the loss of pupillary light reflex should be considered one of the most important factors to determine the timing of the decompressive craniectomy.

• Journal of Korean Neurosurgical Society
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• 제30권3호
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• pp.325-333
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• 2001

Objective : To clarify the benefits and therapeutic effects of intra-arterial papaverine infusion on the symptomatic cerebral vasospasm, we analyzed the results of treatment in 32 patients retrospectively. Methods : A total of 510 patients underwent surgical clipping or endovascular intra-aneurysmal treatment for ruptured intracranial aneurysm between May, 1996 and June, 1999. The delayed ischemic deficit(DID) was developed in 90 of 510 patients. Of these 90 patients, 32 developed symptomatic vasospasm inspite of using modest "3H therapy". The brain CT scan was taken before the intra-arterial infusion of papaverine. The 32 patients underwent 42 intra-arterial papaverine infusion. The symptomatic vasospasm was divided into three groups : deterioration of mental status(Group 1), appearance of a focal neurologic deficit(Group 2), or both(Group 3). We measured Glasgow Coma Scale(GCS), arterial diameters, and cerebral circulation time(CCT) at the time of pre- and postangioplasty. Results : The number of patients in group 1, 2 and 3 were 26, 7, 9 respectively. Eighteen cases showed improvement of GCS more than 2 scores, 16 more than 1, and 8 showed no change of GCS. Average cerebral circulation time(CCT) was decreased ranging from 0.0%-67.5%, and arterial diameters were increased in 21 cases ranging from 1 to 4 folds. Conclusion : Intra-arterial papaverine infusion seemed to have therapeutic effects on symptomatic vasospasm by improving the neurological signs and increasing the arterial diameter. We suggest that intra-arterial papaverine infusion would be an useful adjunctive therapeutic modality in symptomatic vasospasm.