The authors report a case of IgG4-related hypertrophic pachymeningitis that involved cerebral parenchyma. The mass was removed surgically. Histopathological studies showed diffuse infiltration of lymphoplasmacytic cells without evidence of Langerhans histiocytes or meningothelial cells. Immunoglobulin G4 was strongly positive on immunohistochemical staining. The Gd-enhanced lesion deep inside brain parenchyma was completely resolved after 3 months of oral corticosteroid medication. A nodular type of hypertrophic pachymeningitis that mimics a meningioma is rare. Nevertheless, preoperative presumption is very important, and immunohistochemical studies for IgG4 may be helpful in the differential diagnosis.
Eui Hyun Kim, Se Hoon Kim, Jin Mo Cho, Jung Yong Ahn, and Jong Hee Chang
So-Hyang Im, Chang Wan Oh, O-Ki Kwon, Byung-Kyu Cho, Young-Seob Chung, and Dae Hee Han
Object. Involuntary movement is an uncommon manifestation of a transient ischemic attack. It may be induced by cerebral hemodynamic insufficiency, which is associated with several cerebral ischemic diseases. The authors present three cases of limb shaking due to moyamoya disease (MMD) or radiation-induced middle cerebral artery stenosis, and three additional cases of choreic movement due to MMD. Neuroimaging studies and surgical outcomes in these patients were retrospectively analyzed to investigate the pathological mechanism underlying the symptoms and to provide guidance for the management of involuntary movement disorders in cases of ischemic cerebral disease.
Methods. The patient population included two children and four adults with ages at presentation ranging between 7 and 50 years. The initial presenting symptoms were involuntary movements in all six cases. A magnetic resonance imaging finding common in all cases was a small infarct in the frontal corona radiata, which did not extend to the cortex or basal ganglia. A perfusion defect in the frontoparietal cortical and subcortical regions was demonstrated by singlephoton emission computerized tomography in all patients. Improved hemodynamic circulation in the frontoparietal cortical and subcortical regions occurred in parallel with clinical improvement following indirect or direct bypass surgery.
Conclusions. Ischemic dysfunction of the frontal cortical and subcortical motor pathways rather than that of the basal ganglia was suspected to be the cause of the observed contralateral involuntary movements. Direct and indirect bypass surgery can be used effectively to treat involuntary movements in patients with cerebral ischemic diseases such as MMD and in those with stenosis of an intracranial major artery.
Mi Fa Jeon, Yoon Ha, Yoon Hee Cho, Bae Hwan Lee, Yong Gou Park, and Jin Woo Chang
Object. The purpose of this study was to investigate the spontaneous behavioral changes and the alteration of neuronal activities in the pedunculopontine nucleus (PPN) after ipsilateral subthalamic nucleus (STN) lesioning by kainic acid in a rat parkinsonian model created by lesioning with 6-hydroxydopamine (6-OHDA).
Methods. Assumptions about the mechanisms mediating the effects of lesioning of the nigrostriatal dopaminergic pathway by 6-OHDA and the effects of STN lesioning were examined behaviorally by means of apomorphine-induced rotational behavior and forepaw-adjusting steps. The authors subsequently investigated the alteration of neuronal activities in the PPN to compare them with the behavioral changes in rat parkinsonian models.
Conclusions. The results demonstrated that STN lesioning induced behavioral improvement in rat parkinsonian models. This result, which confirms previously held assumptions, may account for the therapeutic effect of STN stimulation in Parkinson disease. The alteration of the neuronal activities in the PPN units also indicates that the PPN units are responsible for the improvement in motor symptoms observed after STN lesioning in rat parkinsonian models.
Dong Gyu Kim, Je G. Chi, Sung Hye Park, Kee Hyun Chang, Sun Ho Lee, Hee-Won Jung, Hyun Jib Kim, Byung-Kyu Cho, Kil Soo Choi, and Dae Hee Han
✓ A retrospective analysis of seven patients with intraventricular neurocytoma is presented. Patient age at diagnosis ranged from 15 to 38 years (mean 24.6 years) and the male:female ratio was 6:1. Raised intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. An isodense mass with multiple intratumoral cysts and homogeneous contrast enhancement was the characteristic computerized tomography finding. The lesions commonly involved the lateral ventricle with or without extension to the third ventricle. Cerebral angiography showed homogeneous vascular staining in five patients. Magnetic resonance images revealed a mass isointense with the cerebral cortex on both T1 and T2-weighted images. Gadolinium-diethylenetriaminepenta-acetic acid-enhanced images showed homogeneous enhancement. Total removal of the tumor was possible in four patients. Pathologically, six cases were initially diagnosed as oligodendroglioma and the remaining case as ependymoma. However, immunohistochemical studies demonstrated strong positivity for neuron-specific enolase in all seven cases and for synaptophysin in five cases. On electron microscopy, three cases showed well-defined neurosecretory granules and 10-nm microtubules in their cytoplasm and cytoplasmic processes. One patient developed a recurrent tumor 18 months after surgery. The remaining six patients are free of recurrent tumors at 2 to 62 months after surgery. It is suggested that neurocytoma must be included in the differential diagnosis of intraventricular lesions, and that electron microscopic and immunohistochemical studies should be undertaken.
Kee-Yong Ha, Eung-Ha Kim, Young-Hoon Kim, Hae-Dong Jang, Hyung-Youl Park, Chang-Hee Cho, Ryu-Kyoung Cho, and Sang-Il Kim
The most catastrophic symptom of proximal junctional failure (PJF) following long instrumented fusion surgery for adult spinal deformity (ASD) is neurological deficits. Although previous reports have shown that PJF usually developed during the early postoperative period, some patients showed late neurological deficits. The aim of this study was to report the incidence, characteristics, and surgical outcomes of PJF with late neurological deficits.
Patients surgically treated for ASD at a single institution were retrospectively reviewed. Among them, the patients requiring revision surgery for newly developed neurological deficits at least 6 months after the initial surgery were included. Patient demographic, radiographic, surgical, and clinical data were investigated. Neurological status was assessed using the Frankel grading system.
PJF with late neurological deficits developed in 18 of 385 patients (4.7%). The mean age at the onset of neurological deficits was 72.0 ± 6.0 years, and the median time from the initial surgery was 4.5 years. The most common pathology of PJF was adjacent disc degeneration and subsequent canal stenosis (11 patients). Five patients showed disc degeneration with aseptic bone destruction. Fractures at the upper instrumented vertebra (UIV), UIV + 1, and UIV + 2 occurred in 2, 3, and 2 patients, respectively. Ossification of the yellow ligament, which had not been found at the first surgery, was identified in 6 patients. Eight patients showed improvement of their neurological deficits and 10 patients showed no improvement by the final follow-up. Perioperative major complications occurred in 8 of 18 patients.
The incidence of PJF with late neurological deficits following ASD surgery was 4.7% in this cohort. The patients showed several morphological features. After revision surgery, perioperative complications were common and the prognosis for improved neurological status was not favorable.
Seung-Ki Kim, Kyu-Chang Wang, Yong-Seung Hwang, Ki Joong Kim, Jong Hee Chae, In-One Kim, and Byung-Kyu Cho
Ideal epilepsy surgery would eliminate seizures without causing any functional deficits. The aim of the present study was to assess seizure outcomes and complications after epilepsy surgery in children with intractable epilepsy.
Data obtained in 134 children (75 boys and 59 girls) age 17 years or younger who underwent epilepsy surgery at Seoul National University Children's Hospital between 1993 and 2005 were retrospectively reviewed. Epilepsy surgery included temporal resection (59 cases), extratemporal resection (56 cases), functional hemispherectomy (7 cases), callosotomy (9 cases), multiple subpial transection (1 case), and disconnection of a hamartoma (2 cases). The mean follow-up duration was 62.3 months (range 12–168 months).
The overall seizure-free rate was 69% (93 of 134 cases). The seizure-free rate was significantly higher in children who underwent temporal resection than in those in whom extratemporal resection was performed (88 vs 55%, p < 0.05). The most frequent causes of treatment failure were related to the absence of structural abnormality demonstrated on magnetic resonance imaging, development-associated disease, widespread disease documented by postoperative electroencephalography, and limited resection due to the presence of functional cortex. There were no postoperative deaths. Visual field defects were the most common complication after temporal resection (22% [13 of 59 cases]), whereas hemiparesis (mostly transient) was the most common morbidity after extratemporal resection (18% [10 of 56 cases]).
Epilepsy surgery is an effective and safe therapeutic modality in childhood. In children with extratemporal epilepsy, more careful interpretation of clinical and investigative data is needed to achieve favorable seizure outcome.
Eun Suk Park, Do Hoon Kwon, Jun Bum Park, Do Hee Lee, Young Hyun Cho, Jeong Hoon Kim, and Chang Jin Kim
Brain metastases from hepatocellular carcinoma (HCC) are rare, and the evidence of the effectiveness of Gamma Knife surgery (GKS) in this disease is lacking. The authors report their institutional experience with GKS in patients with brain metastases from HCCs.
The authors retrospectively reviewed the medical records of 73 consecutive patients who had a combined total of 141 brain metastases arising from HCCs and were treated with GKS. Sixty-four (87.7%) patients were male, and the mean age of the patients was 52.5 years (range 30–79 years). The mean tumor volume was 7.35 cm3 (range 0.19–33.7 cm3). The median margin dose prescribed was 23 Gy (range 15–32 Gy). Univariate and multivariate survival analyses were performed to identify possible prognostic factors of outcomes.
The estimated rate of local tumor control was 79.6% at 3 months after GKS. The median overall survival time after GKS was 16 weeks. The actuarial survival rates were 76.7%, 58.9%, and 26.0% at 4, 12, and 24 weeks after GKS, respectively. In the univariate analysis, an age of ≤ 65 years, Child-Pugh Class A (pertaining to liver function), high Karnofsky Performance Scale score (≥ 70), and low Radiation Therapy Oncology Group recursive partitioning analysis class (I or II) were positively associated with the survival times of patients. No statistically significant variable was identified in the multivariate analysis.
Although survival was extremely poor in patients with brain metastases from HCCs, GKS showed acceptable local tumor control at 3 months after the treatment. The authors suggest that GKS represents a noninvasive approach that may provide a valuable option for treating patients with brain metastases from HCCs.
Ji Hoon Phi, Byung-Kyu Cho, Kyu-Chang Wang, Ji Yeoun Lee, Yong Seung Hwang, Ki Joong Kim, Jong-Hee Chae, In-One Kim, Sung-Hye Park, and Seung-Ki Kim
The long-term surgical outcome of pediatric patients with epilepsy accompanied by focal cortical dysplasia (FCD) is not clear. The authors report on the long-term surgical outcomes of children with FCD, based on longitudinal analyses.
The authors retrospectively analyzed the records of 41 children who underwent epilepsy surgery for pathologically proven FCD. Twenty of these patients were male and 21 were female. The median age at surgery was 9 years (range 1–17 years).
The actuarial seizure-free rates were 49, 44, and 33% in the 1st, 2nd, and 5th years after surgery, respectively. There was no seizure recurrence after 3 years. Three patients with initial failure of seizure control experienced late remission of seizures (the so-called running-down phenomenon). Eventually, 19 patients (46%) were seizure free at their last follow-up visit. Absence of a lesion on MR imaging and incomplete resection were significantly associated with seizure-control failure. Concordance of presurgical evaluation data was a marginally significant variable for seizure control in patients with lesional epilepsy. Three patients with seizure-control failure became seizure free as a result of the running-down phenomenon. The actuarial rate of antiepileptic drug discontinuation was 91% in the 5th year in the seizure-free patients.
The seizure-free rate after surgery in children with FCD was 49% in the 1st year; however, it declined thereafter. The running-down phenomenon could be an important mechanism of seizure alleviation for patients with FCD during long-term follow-up. Because a complete resection of FCD has a strong prognostic implication for seizure control, a better method to define the extent of FCD is required to assist with resection, especially in nonlesional epilepsy.