OBJECT: It seemed worthwhile to estimate nonlinear measures of the electroencephalogram (EEG) in schizophrenic patients, because nonlinear measures might serve as indicators of the specific brain function in schizophrenia. METHOD: Previous studies which estimated the chaoticity in the brain of schizophrenia with nonlinear methods recorded the EEGs at limited electrodes, so we tried to record EEGs from 16 channels for nonlinear analysis in 19 patients with Schizophrenia and 8 healthy control subjects. We employed a new method to calculate the nonlinear invariant measures. For limited noisy data, this algorithm was strikingly faster and more accurate than previous ones. RESULTS: Our results showed that the schizophrenic patients had lower values of the largest positive Lyapunov exponent at the left inferior frontal and anterior temporal head regions compared with normal controls. CONCLUSIONS: These results suggest that the nonlinear analysis of the EEGs such as the estimation of the largest positive Lyapunov exponent seems to be a useful tool in analyzing EEG data to explore the neurodynamics of the brain of schizophrenic patients.
Brain ; Electrodes ; Electroencephalography* ; Head ; Humans ; Nonlinear Dynamics* ; Schizophrenia*

Churg-Strauss syndrome (CSS) is a systemic necrotizing vasculitis of the small and medium vessels, associated with extravascular eosinophilic granulomas, peripheral eosinophilia, and asthma. The exact etiology of CSS is unknown. This syndrome commonly affects the lungs, peripheral nerves, skin, heart, and gastrointestinal tract, but rarely the central nervous system. Subarachnoid and intracerebral hemorrhage in CSS patients is extremely rare; however, clinicians should consider that CSS may be a cause of intracranial hemorrhage and its high rate of mortality and morbidity. The authors report on two cases of subarachnoid and intracerebral hemorrhage with CSS and discuss a brief review of CSS.
Asthma ; Central Nervous System ; Cerebral Hemorrhage ; Churg-Strauss Syndrome ; Eosinophilia ; Eosinophilic Granuloma ; Gastrointestinal Tract ; Heart ; Humans ; Intracranial Hemorrhages ; Lung ; Peripheral Nerves ; Skin ; Subarachnoid Hemorrhage ; Vasculitis

No abstract available.
Brain ; Brain Injuries ; Bruxism

Anterograde amnesia in Behcet's disease is a rare occurrence. A 50-year-old man presented with anterograde amnesia. He had been suffering multiple oral aphthous ulcers and genital ulcers with erythema nodosum. A neurological examination revealed prominent anterograde memory disturbance. Brain MRI revealed high signal intensity lesions involving the anterior thalamus, posterior part of the basal ganglia and the mesial temporal lobe. We report a rare case of Behcet's disease manifesting severe anterograde amnesia resulting from thalamic and mesial temporal lesions.
Amnesia, Anterograde* ; Basal Ganglia ; Brain ; Erythema Nodosum ; Humans ; Magnetic Resonance Imaging ; Memory ; Middle Aged ; Neurologic Examination ; Stomatitis, Aphthous ; Temporal Lobe ; Thalamus ; Ulcer

BACKGROUND: Ductal adenocarcinoma (DAC) of the prostate is an uncommon histologic subtype whose prognostic factors and immunoprofile have not been fully defined. METHODS: To define its prognostic factors and immunoprofile, the clinicopathological features, including biochemical recurrence (BCR), of 61 cases of DAC were analyzed. Immunohistochemistry was performed on tissue microarray constructs to assess the expression of prostate cancer-related and mammalian target of rapamycin (mTOR) signaling-related proteins. RESULTS: During the median follow-up period of 19.3 months, BCR occurred in 26 cases (42.6%). DAC demonstrated a wide expression range of prostate cancer-related proteins, including nine cases (14.8%) that were totally negative for pan-cytokeratin (PanCK) immunostaining. The mTOR signaling-related proteins also showed diverse expression. On univariate analysis, BCR was associated with high preoperative serum levels of prostate-specific antigen (PSA), large tumor volume, predominant ductal component, high Gleason score (GS), comedo-necrosis, high tumor stage (pT), lymphovascular invasion, and positive surgical margin. High expressions of phospho-mTOR (p-mTOR) as well as low expressions of PSA, phospho-S6 ribosomal protein (pS6) and PanCK were associated with BCR. On multivariable analysis, GS, pT, and immunohistochemical expressions of PanCK and p-mTOR remained independent prognostic factors for BCR. CONCLUSIONS: These results suggest GS, pT, and immunohistochemical expressions of PanCK and p-mTOR as independent prognostic factors for BCR in DAC. Since DAC showed diverse expression of prostate cancer–related proteins, this should be recognized in interpreting the immunoprofile of DAC. The diverse expression of mTOR-related proteins implicates their potential utility as predictive markers for mTOR targeted therapy.
Adenocarcinoma* ; Carcinoma, Ductal ; Follow-Up Studies ; Immunohistochemistry ; Neoplasm Grading ; Prognosis ; Prostate* ; Prostate-Specific Antigen ; Prostatic Neoplasms ; Recurrence ; Ribosomal Proteins ; Sirolimus ; Tumor Burden

Menkes disease is an X-linked recessive copper transport disorder characterized by neurological deterioration, connective-tissue damage, and abnormal hair growth. It is caused by the mutation of the ATP7A gene. This report describes a four-month-old boy with neurological symptoms typical of Menkes disease plus unusual liver involvement. He developed seizures at three months of age and exhibited hypotonia, cephalhematoma, a sagging face, redundant and hypopigmented skin, and abnormal hair growth. In addition, he had unexplained hepatomegaly and high hepatic transaminase. We confirmed the diagnosis of Menkes disease by mutation analysis of the ATP7A gene. To exclude other possible causes for the hepatic abnormalities, a liver biopsy was performed, revealing intracytoplasmic cholestasis, focal spotty necrosis, and minimal lobular activity. The patient's liver involvement may be an underestimated complication of Menkes disease.
Biopsy ; Cholestasis ; Copper ; Hair ; Hepatomegaly ; Infant ; Infant, Newborn ; Liver ; Menkes Kinky Hair Syndrome ; Muscle Hypotonia ; Necrosis ; Seizures ; Skin ; Spasms, Infantile

Purpose: Tyrosine kinase inhibitors (TKI) targeting vascular endothelial growth factor receptor (VEGFR) signaling pathways have been used for metastatic clear cell renal cell carcinoma (mCCRCC), but resistance to the drug develops in most patients. We aimed to explore the underlying mechanism of the TKI resistance with regard to programmed death-ligand 1 (PD-L1) and to investigate signaling pathway associated with the resistant mechanism. Materials and Methods: To determine the mechanism of resistance, 10 mCCRCC patients from whom tumor tissues were harvested at both the pretreatment and the TKI-resistant post-treatment period were included as the discovery cohort, and their global gene expression profiles were compared. A TKI-resistant renal cancer cell line was established by long-term treatment with sunitinib. Results: Among differentially expressed genes in the discovery cohort, increased PD-L1 expression in post-treatment tissues was noted in four patients. Pathway analysis showed that PD-L1 expression was positively correlated with the mammalian target of rapamycin (mTOR) signaling pathway. The TKI-resistant renal cancer cells showed increased expression of PD-L1 and mTOR signaling proteins and demonstrated aggressive tumoral behaviour. Treatment with mTOR inhibitors down-regulated PD-L1 expression and suppressed aggressive tumoral behaviour, which was reversed with stimulation of the mTOR pathway. Conclusion These results showed that PD-L1 expression may be increased in a subset of VEGFR-TKI–resistant mCCRCC patients via the mTOR pathway.

PURPOSE:Temporal lobe epilepsy(TLE) is now recognized as a distinct syndrome in adults. The seizure evolution in adult patients is well characterized, manifesting initially with an aura, behavioral arrest, automatism, and secondary generalized tonic-clonic seizures. In contrast, relatively few studies are available for the pediatric age group. In the present study, we investigated children undergoing temporal lobectomy for refractory seizures and correlated the pathologic findings with clinical presentations. METHODS:The records of the pediatric patients admitted at the Seoul National Children's Hospital for epilepsy surgery between January 1995 and December 2005 were reviewed. Then, eighteen patients were included in this study. The clinical records were reviewed in terms of the patient profiles imaging findings, surgical techniques, and pathologic findings. The seizure outcomes were described according to the Engel's classification. RESULTS:The postsurgical outcomes were favorable. Lateral temporal epilepsy was more common in childhood than in adulthood. Dual pathology was commonly found. Arm dystonia or tonic arm elevation have a lateralizing value. Head turning may have a lateralizing value based upon a time sequence. The brain MRI was less predictable for pathologic findings. The ictal EEG cannot always have a localizing value. Delta beginning in the ictal rhythm may suggest lateral lobe epilepsy. Anterior temporal beginning of the ictal location may suggest mesial temporal lobe epilepsy. Ganglioglioma tends to cause rhythmic beta activities at the beginning of the ictal event. CONCLUSION:TLE in childhood shows more complex and atypical clinical manifestations and have more variable etiologies. No single presurgical investigation can be a good predictable value to localization or lateralization.
Adult ; Arm ; Automatism ; Brain ; Child ; Classification ; Dystonia ; Electroencephalography ; Epilepsy ; Epilepsy, Temporal Lobe* ; Ganglioglioma ; Head ; Humans ; Magnetic Resonance Imaging ; Pathology ; Seizures ; Seoul ; Temporal Lobe*

PURPOSE: Pompe disease is one of the glycogen storage diseases caused by a deficiency of acid alpha-glycosidase. This enzyme defect results in lysosomal glycogen accumulation in many tissues and shows a various spectrum of clinical features from early infantile hypotonia to mild weakness. For the investigation of the clinical characteristics of Pompe disease, we reviewed 6 cases of childhood Pompe disease diagnosed by muscle biopsy and acid alpha-glycosidase assay. METHODS: We reviewed the medical records of 6 childhood Pompe disease patients in Seoul National University Children's Hospital, retrospectively from January 2001 to October 2006. RESULTS: The age of the symptom onset was 1 month to 11 years(mean 2.2 years) and the diagnosis was made at the age of 8 months to 14 years(mean 5.3 years). The patients showed delayed motor development, motor weakness, hypotonia, cardiomegaly, hypertrophic cardiomyopathy, hepatomegaly, recurrent pulmonary infections but the severity was very diverse. Liver transaminase and CK levels were elevated in all of the patients. Their muscle biopsy showed the characteristic accumulation of purple colored glycogen granules and the degeneration of myofibers. CONCLUSION: Childhood Pompe disease had various clinical features and severities depending on the age of onset, organ involvement and the rate of progression. Enzyme replacement therapy may modify the disease courses, so we need to diagnose earlier for the treatment at an optimal time.
Age of Onset ; Biopsy ; Cardiomegaly ; Cardiomyopathy, Hypertrophic ; Diagnosis ; Early Diagnosis ; Enzyme Replacement Therapy ; Glycogen ; Glycogen Storage Disease ; Glycogen Storage Disease Type II* ; Hepatomegaly ; Humans ; Liver ; Medical Records ; Muscle Hypotonia ; Retrospective Studies ; Seoul

No abstract available.
Dysgeusia ; Infarction