The medical records of six cases of nesidioblastosis were examined to determine the diagnostic approach, treatment, and neurologic sequelae. All six patients were male, and their ages at the onset of the disease ranged from one day to six months (mean 3.36 +/- 2.5 mo.). Initial clinical features were seizure, cyanosis, poor feeding, and apnea. Other subsequent symptoms were developmental delay, hyperactivity, and cold sweating. The Birth weight of the neonatal onset group was heavier than the postneonatal onset group (4.4 +/- 0.3 vs 3.26 +/- 0.04 kg). Before the diagnosis of hyperinsulinism, steroids of ACTH proved effective for seizure control. Initially, hyperinsulinemia (serum insulin greater than 10 microU/ml) was detected in four cases, but another two cases also showed hyperinsulinism by insulin/glucose(I/G) ratio greater than 0.3 during the fasting test. The glucagon response performed in 2 cases, showed normal and partial responses. Euglycemia was obtained by near total pancreatectomy (95% pancreatic resection)without malabsorption or persistent diabetes. In one case, nesidioblastoma coexisted with nesidioblastosis. Developmental delay was noted in three cases. In this group, the mean duration between symptom onset and operation was longer than the group without developmental delay (1.25 +/- 0.47 vs 0.38 +/- 0.19 yr).
Brain Damage, Chronic/*etiology ; Child, Preschool ; Developmental Disabilities/etiology ; Humans ; Hypoglycemia/blood/*etiology ; Infant ; Infant, Newborn ; Insulin/*blood ; Male ; Pancreatic Diseases/complications/*diagnosis/therapy ; Postoperative Complications/epidemiology

OBJECTIVETo summarize our experience in the management of adipsic central diabetes insipidus(ADI) accompanied with intracranial calcification.

METHODThe clinical data of one ADI patient accompanied with intracranial calcification who was treated in our hospital since December 2011 were retrospectively summarized.

RESULTSThe 24-hour urine volume was 800 ml. She didn't feel thirsty even with increased plasma sodium concentration(153 mmol/L) and blood osmotic pressure(333 mmol/L) . Combined water deprivation and vasopressin test revealed the diagnosis of central diabetes insipidus. The high intensity signal(on T1-weighted magnetic resonance imaging) in the posterior lobe of pituitary gland was found. Computed tomography showed calcifications in the bilateral basal ganglia.Serum cytomegalovirus IgG was positive. She was treated with desmopressin and asked for regular water intake regardless of the adipsia. The plasma sodium concentration was still below 150 mmol/L during the 4-month follow-up.

CONCLUSIONSRoutine adipsia evaluation and combined water deprivation and vasopressin test are critical for the diagnosis and treatment of ADI. Past insidious intracranial cytomegalovirus infection may explain the cause of ADI and calcification.

Brain Diseases ; complications ; Calcinosis ; complications ; Child ; Cytomegalovirus ; Diabetes Insipidus, Neurogenic ; complications ; diagnosis ; etiology ; Drinking ; Female ; Humans ; Sodium ; blood

We report on a 55-year-old man with alcoholic liver cirrhosis who presented with status epilepticus. Laboratory analysis showed markedly elevated blood ammonia. Brain magnetic resonance imaging (MRI) showed widespread cortical signal changes with restricted diffusion, involving both temporo-fronto-parietal cortex, while the perirolandic regions and occipital cortex were uniquely spared. A follow-up brain MRI demonstrated diffuse cortical atrophy with increased signals on T1-weighted images in both the basal ganglia and temporal lobe cortex, representing cortical laminar necrosis. We suggest that the brain lesions, in our case, represent a consequence of toxic effect of ammonia.
Ammonia/blood ; Atrophy/pathology ; Brain Diseases/blood/*diagnosis/*etiology ; Hepatic Encephalopathy/*complications ; Humans ; Liver Cirrhosis, Alcoholic/*complications ; Magnetic Resonance Imaging/*methods ; Male ; Middle Aged ; Necrosis/pathology ; Status Epilepticus/pathology

Adolescent ; Brain Neoplasms ; diagnosis ; Child ; Child, Preschool ; Endocrine System Diseases ; etiology ; Female ; Follicle Stimulating Hormone ; blood ; Humans ; Infant ; Luteinizing Hormone ; blood ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed

BACKGROUND: Ischemic stroke is a complex condition influenced by many factors. Previous studies have demonstrated that inflammatory markers might play a role in such vascular diseases. Therefore the purpose of this study was to compare the expression of inflammatory markers in Korean ischemic stroke patients and to investigate their relationship to APOE polymorphism. METHODS: The patient group consisted of 275 patients with large artery atherosclerosis (LAA, n=169) and small artery occlusion (SAO, n=106). One hundred and nineteen age matched healthy subjects were recruited as the control group. Serum levels of three inflammatory markers (matrix metalloproteinase, MMP-9; tissue inhibitor of metalloproteinase-1, TIMP-1; and high-sensitivity C-reactive protein, hs-CRP) were measured in each patient by using commercially available kits. Comparison of clinical risk factors, inflammatory marker levels, and APOE genotypes between the stroke patient group and control group and between the two patient subgroups was assessed. RESULTS: Comparison of the stroke group to control group showed significantly elevated levels of circulating MMP-9 (P<0.01) and hs-CRP (P=0.01). Comparison between the individual subgroups revealed a significantly higher level of only TIMP-1 in the LAA subgroup compared to the SAO subgroup (P<0.01). There was no significant difference in inflammatory marker levels among each allele carrier. CONCLUSIONS: The present study revealed the obvious tendency of increased circulating inflammatory markers in the patients with acute ischemic attack, especially MMP-9 and hs-CRP. Our observations suggest that measurement of serum MMP-9, TIMP-1, and hs-CRP levels may be useful in the diagnosis of ischemic stroke patients.
Aged ; Apolipoproteins E/*genetics ; Biological Markers/blood ; Brain Ischemia/complications/*diagnosis ; C-Reactive Protein/analysis ; Carotid Artery Diseases/complications ; Female ; Genotype ; Humans ; Inflammation Mediators/*blood ; Korea ; Male ; Matrix Metalloproteinase 9/blood ; Middle Aged ; Polymorphism, Genetic ; Stroke/*diagnosis/etiology/immunology ; Tissue Inhibitor of Metalloproteinase-1/blood

A Dieulafoy lesion in the rectum is a very rare and it can cause massive lower gastrointestinal bleeding. An 83-year-old man visited our hospital. He had chronic constipation and had taken aspirin for about 10 years because of a previous brain infarction. He was admitted because of a recent brain stroke. On the third hospital day, he had massive hematochezia and suddenly developed hypovolemic shock. Abdominal computed tomography showed active arterial bleeding on the left side of the mid-rectum. Emergency sigmoidoscopy showed an exposed vessel with blood spurting from the rectal wall. The active bleeding was controlled successfully by an injection of epinephrine and two hemoclippings. On the fourth day after the procedure, he had massive recurrent hematochezia, and his vital signs were unstable. Doppler-guided hemorrhoidal artery band ligation was performed urgently at two sites. However, he rebled on the third postoperative day. Selective inferior mesenteric angiography revealed an arterial pseudoaneurysm in a branch of the superior rectal artery, as the cause of rectal bleeding, and this was embolized successfully. We report a rare case of life-threatening rectal bleeding caused by a Dieulafoy lesion combined with pseudoaneurysm of the superior rectal artery which was treated successfully with embolization.
Aged, 80 and over ; Aneurysm/radiography ; Angiography ; Aspirin/therapeutic use ; Brain Infarction/drug therapy/prevention & control ; Embolization, Therapeutic ; Gastrointestinal Hemorrhage/*diagnosis/etiology/therapy ; Hemorrhoids/*complications ; Humans ; Male ; Mesenteric Artery, Inferior/radiography ; Platelet Aggregation Inhibitors/therapeutic use ; Rectal Diseases/complications/diagnosis/therapy ; Rectum/blood supply ; Sigmoidoscopy ; Tomography, X-Ray Computed

A Dieulafoy lesion in the rectum is a very rare and it can cause massive lower gastrointestinal bleeding. An 83-year-old man visited our hospital. He had chronic constipation and had taken aspirin for about 10 years because of a previous brain infarction. He was admitted because of a recent brain stroke. On the third hospital day, he had massive hematochezia and suddenly developed hypovolemic shock. Abdominal computed tomography showed active arterial bleeding on the left side of the mid-rectum. Emergency sigmoidoscopy showed an exposed vessel with blood spurting from the rectal wall. The active bleeding was controlled successfully by an injection of epinephrine and two hemoclippings. On the fourth day after the procedure, he had massive recurrent hematochezia, and his vital signs were unstable. Doppler-guided hemorrhoidal artery band ligation was performed urgently at two sites. However, he rebled on the third postoperative day. Selective inferior mesenteric angiography revealed an arterial pseudoaneurysm in a branch of the superior rectal artery, as the cause of rectal bleeding, and this was embolized successfully. We report a rare case of life-threatening rectal bleeding caused by a Dieulafoy lesion combined with pseudoaneurysm of the superior rectal artery which was treated successfully with embolization.
Aged, 80 and over ; Aneurysm/radiography ; Angiography ; Aspirin/therapeutic use ; Brain Infarction/drug therapy/prevention & control ; Embolization, Therapeutic ; Gastrointestinal Hemorrhage/*diagnosis/etiology/therapy ; Hemorrhoids/*complications ; Humans ; Male ; Mesenteric Artery, Inferior/radiography ; Platelet Aggregation Inhibitors/therapeutic use ; Rectal Diseases/complications/diagnosis/therapy ; Rectum/blood supply ; Sigmoidoscopy ; Tomography, X-Ray Computed