• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.8 no.2
• /
• pp.263-268
• /
• 2005

Erythropoietic protoporphyria is a genetic disorder due to a deficiency of ferrochelatase resulting in excessive accumulation and excretion of protoporphyrin. The predominant clinical feature is photosensitivity. Severe hepatic failure occurs in a small percentage of patients, and neurological symptoms are very rare. We report a case of erythropoietic protoporphyria associated with severe hepatic dysfunction and neurological symptoms. A 9-year-old girl presented with severe abdominal pain, nausea, weakness and pain of extremities, and urinary retention. Ultrasonogram and abdominal CT scanning revealed a diffuse infiltrated and enlarged liver. Liver biopsy showed deposition of dense dark brown pigment within the bile, hepatocytes and Kupffer cells. Plus, dense dark brown deposits gave a red birefringent under polarize light. Porphyrin studies demonstrated markedly elevated serum free erythrocyte protoporphyrin. This girl was diagnosed as erythropoietic protoporphyria with severe liver dysfunction and neurological symptoms.

• Korean Journal of Veterinary Research
• /
• v.56 no.2
• /
• pp.113-116
• /
• 2016

Our retrospective study reports the clinical findings and population characteristics of 81 surgically treated dogs for spinal cord compression. We compared the outcome of dogs with different grades of neurological dysfunction due to spinal cord compression and focused on the long-term outcome of surgical treatment, for which there are only a few recent records. We recorded a 13.6% recurrence, regardless of the degree of dysfunction. However, the degree of dysfunction negatively affected the recovery length.

• Molecules and Cells
• /
• v.45 no.11
• /
• pp.781-788
• /
• 2022

Proline plays a multifaceted role in protein synthesis, redox balance, cell fate regulation, brain development, and other cellular and physiological processes. Here, we focus our review on proline metabolism in neurons, highlighting the role of dysregulated proline metabolism in neuronal dysfunction and consequently neurological and psychiatric disorders. We will discuss the association between genetic and protein function of enzymes in the proline pathway and the development of neurological and psychiatric disorders. We will conclude with a potential mechanism of proline metabolism in neuronal function and mental health.

• Journal of Korean Neurosurgical Society
• /
• v.58 no.5
• /
• pp.432-441
• /
• 2015

Objective : To assess the impact of the complete resection of craniopharyngioma (CP) in adults on oncologic and functional outcomes. Methods : We retrospectively analyzed 82 patients with CP who were surgically treated by the same neurosurgeon at our institution between January 1994 and December 2012. Results : Gross total resection (GTR) was achieved in 71 patients (86.6%), near total resection (NTR) in 7 patients (8.5%), and subtotal resection (STR) in 3 patients (3.7%). The disease-specific overall survival rate was 100% with the exclusion of 2 surgery-related mortalities. The overall recurrence rate was 12.2% (10 of 82 patients), however the recurrence rate according to extent of resection (EOR) was 9.9% (7 of 71 patients) after GTR, 14.3% (1 of 7 patients) after NTR, and 66.7% (2 of 3 patients) after STR. The overall recurrence-free survival (RFS) rates at 5 and 10 years were 87.0% and 76.8%, respectively. Postoperatively, most patients (86.3%) needed hormone replacement for at least 1 hypothalamic-pituitary axis. Vision improved in 56.4% of the patients with preoperative abnormal vision, but deteriorated in 27.4% of patients. Hypothalamic dysfunction developed in 32.9% of patients. There were no significant differences in the risks of pituitary dysfunction, visual deterioration, or hypothalamic dysfunction between the groups with complete vs. incomplete removal. The overall rate of postoperative complications was 22.0%, which did not differ between groups (p=0.053). Conclusion : The complete removal of a CP at first surgery can provide a chance for a cure with acceptable morbidity and mortality risks.

• Annals of Clinical Neurophysiology
• /
• v.25 no.1
• /
• pp.1-9
• /
• 2023

Breathing is controlled by complex interactions between the central and peripheral nervous systems in conjunction with the respiratory system. Neurological diseases predispose patients to nocturnal desaturation and pneumonia due to respiratory dysfunction, which increases mortality, daytime sleepiness and fatigue, and reduces the quality of life. Respiratory function tests are required to identify respiratory function decline and to consider compensatory management. This review summarizes the characteristics of several respiratory function tests and their applications to neurological diseases.

• The Journal of the Society of Stroke on Korean Medicine
• /
• v.10 no.1
• /
• pp.68-73
• /
• 2009

Type 2 diabetes mellitus (T2DM) is a progressive disorder caused by a combination of insulin resistance and 𝛽 cell dysfunction. Sogal(消渴) is a traditional Korean medical term referring to a condition pertaining 3 major symptoms - thirst, polyphasia, polyuria. Sogal has been reported to have similar characteristics with DM. This case report demonstrates a patient with T2DM complaining of typical Sogal symptoms. We diagnosed him as So-yang person Sogal and treated him with acupuncture and herbal medicine.

• Sleep Medicine and Psychophysiology
• /
• v.2 no.2
• /
• pp.115-128
• /
• 1995

Present study reviewed various standardized neuropsychological assessment methods for children that are widely used in Korean child-psychiatric clinic settings to evaluate neurological problems, especially soft neurological signs that could not be identified by neurological techniques like CT, MRI. The characteristics of those neuropsychological test responses in children with psychiatric disorders that neurological factors are thought to play more important role than psychological factors in their etiology were examined. It is more important and required to establish the developmental norms for interpreting the results of neuropsychological tests and for identifying the signs of brain damage in case of children than adults. There are many difficulties in diagnosing brain dysfunction and conducting research for neurological problems in psychiatric disordered children due to lack of the standardized Korean version of neuropsyhological test for children, Therefore, several issues on developing the Korean version of neuropsyhological tests for children were discussed.

• Journal of Ginseng Research
• /
• v.45 no.3
• /
• pp.371-379
• /
• 2021

Mitochondrial dysfunction contributes to the pathogenesis and prognosis of many common disorders, including neurodegeneration, stroke, myocardial infarction, tumor, and metabolic diseases. Ginsenosides, the major bioactive constituents of Panax ginseng (P. ginseng), have been reported to play beneficial roles in the molecular pathophysiology of these diseases by targeting mitochondrial dysfunction. In this review, we first introduce the types of ginsenosides and basic mitochondrial functions. Then, recent findings are summarized on different ginsenosides targeting mitochondria and their key signaling pathways for the treatment of multiple diseases, including neurological disorders, cancer, heart disease, hyperglycemia, and inflammation are summarized. This review may explain the common targets of ginsenosides against multiple diseases and provide new insights into the underlying mechanisms, facilitating research on the clinical application of P. ginseng.

• Journal of Korean Neurosurgical Society
• /
• v.54 no.4
• /
• pp.296-301
• /
• 2013

Objective : We conducted a retrospective study examining the outcomes of intracerebral hemorrhage (ICH) in patients with chronic kidney disease (CKD) to identify parameters associated with prognosis. Methods : From January 2001 to June 2008, we treated 32 ICH patients (21 men, 11 women; mean age, 62 years) with CKD. We surveyed patients age, sex, underlying disease, neurological status using Glasgow Coma Scale (GCS), ICH volume, hematoma location, accompanying intraventricular hemorrhage, anti-platelet agents, initial and 3rd day systolic blood pressure (SBP), clinical outcome using the modified Rankin Scale (mRS) and complications. The severity of renal functions was categorized using a modified glomerular filtration rate (mGFR). Multifactorial effects were identified by regression analysis. Results : The mean GCS score on admission was $9.4{\pm}4.4$ and the mean mRS was $4.3{\pm}1.8$. The overall clinical outcomes showed a significant relationship on initial neurological status, hematoma volume, and mGFR. Also, the outcomes of patients with a severe renal dysfunction were significantly different from those with mild/moderate renal dysfunction (p<0.05). Particularly, initial hematoma volume and sBP on the 3rd day after ICH onset were related with mortality (p<0.05). However, the other factors showed no correlation with clinical outcome. Conclusion : Neurological outcome was based on initial neurological status, renal function and the volume of the hematoma. In addition, hematoma volume and uncontrolled blood pressure were significantly related to mortality. Hence, the severity of renal function, initial neurological status, hematoma volume, and uncontrolled blood pressure emerged as significant prognostic factors in ICH patients with CKD.

• Journal of the Korean neurological association
• /
• v.36 no.4
• /
• pp.375-377
• /
• 2018