Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 23-year-old male was referred to our hospital for evaluation of new-onset seizures. Signs and symptoms of neuroglycopenia, including weakness, dizziness, and confusion, appeared during fasting and resolved promptly with intravenous dextrose administration. Insulin, proinsulin, and C-peptide levels were consistent with a diagnosis of insulinoma. Screening tests for multiple endocrine neoplasia type 1 and surreptitious sulfonylurea uses were negative. Preoperative localization of the insulinoma by transabdominal ultrasonography, computed tomography, and indium-111 octreoscanning were unsuccessful. Endoscopic ultrasonography (EUS) identified a 6- to 7-mm tumor at the juncture of the head and body of the pancreas. Surgical exploration confirmed the preoperative localization, and an 8-mm tumor was simply enucleated. The patient has been free of symptoms for 18 months since surgery. This report describes the utility of EUS to localize a solitary pancreatic insulinoma and provides a comparison of EUS and other preoperative localization techniques.
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PMID:Endoscopic ultrasound localization of a pancreatic insulinoma: case report and review of the localization techniques. 986 68

Ultimately traceable to neural glucose deprivation, symptoms of hypoglycemia include neurogenic (autonomic) and neuroglycopenic symptoms. Neurogenic symptoms (tremulousness, palpitations, anxiety, sweating, hunger, paresthesias) are the results of the perception of physiologic changes caused by the autonomic nervous system's response to hypoglycemia. Neuroglycopenic symptoms (confusion, sensation of warmth, weakness or fatigue, severe cognitive failure, seizure, coma) are the results of brain glucose deprivation itself. Glycemic thresholds for symptoms of hypoglycemia shift to lower plasma glucose concentrations following recent episodes of hypoglycemia, leading to the syndrome of hypoglycemia unawareness--loss of the warning symptoms of developing hypoglycemia. Thus, patients with recurrent hypoglycemia (e.g., those with tightly controlled diabetes or with an insulinoma) often tolerate abnormally low plasma glucose concentrations without symptoms.
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PMID:Symptoms of hypoglycemia, thresholds for their occurrence, and hypoglycemia unawareness. 1050 Sep 27

A six-year-old, spayed female, cocker spaniel was presented for hypoglycemic seizures. Hypoglycemia with concomitant hyperinsulinemia suggested an insulin-secreting tumor. Pancreatic masses were resected, and insulinoma was diagnosed. Six weeks later, the dog presented in hyperinsulinemic-hypoglycemic crisis (HHC). The dog was initially stabilized with intravenous dextrose boluses and infusions, but seizure activity recurred and persisted. A glucagon constant-rate infusion (GCRI) was initiated, and neurological signs quickly resolved. Dextrose was withdrawn over 24 hours, and euglycemia was maintained by GCRI alone. Despite aggressive medical management including the use of prednisone, diazoxide, bovine somatotropin, and streptozocin, the dog presented on two subsequent occasions in HHC and both times was rapidly stabilized with GCRI alone. In this dog, GCRI was a fast, safe, and effective method of achieving and maintaining euglycemia despite intractable hyperinsulinism. The clinical use of GCRI merits further investigation for management of HHC in veterinary species.
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PMID:Glucagon constant-rate infusion: a novel strategy for the management of hyperinsulinemic-hypoglycemic crisis in the dog. 1066 3

We report on a patient with clinical, electroencephalographic and radiological features of temporal lobe epilepsy, whose seizures were initially controlled with antiepileptic treatment. Five years after the onset of seizures, he developed more frequent and prolonged episodes of confusion, which were misinterpreted as secondary to temporal lobe epilepsy. Further investigation revealed the presence of hypoglycaemia and the eventual diagnosis of metastatic insulinoma.
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PMID:Seizure recurrence in temporal lobe epilepsy. 1183 54

Digestive neuro-endocrine tumours have a broad and initially misleading clinical spectrum. Tumours from the duodenopancreatic area should be distinguished from digestive carcinoid tumours. In the first group, insulinomas, gastrinomas, and non-functioning tumours are the most frequent. Insulinoma is responsible for hypoglycaemic symptoms (coma, confusion, seizure, psychiatric disorders) associated with adrenergic response (sweat, tachycardia, palpitations). Gastrinoma is responsible for the Zollinger-Ellison syndrome, which associates peptic ulcers in the oesophagus, stomach, and duodenum without Helicobacter pylori infection, and chronic volumogenic diarrhoea. Non-functioning tumours are recognised fortuitously or at a late stage, when large tumour mass contrasts with often unaltered general condition. Carcinoid tumours are mainly located in the appendix, the rectum, and the small bowel. In the 2 first conditions, the diagnosis is most often made on a resection specimen after uneventful appendectomy or polypectomy; in the latter, the carcinoid syndrome is frequent, combining cutaneous flushing, motor diarrhoea, tricuspid valve insufficiency and bronchospasm.
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PMID:[Clinical spectrum of digestive neuroendocrine tumors]. 1192 15

A 14-year-old spayed female domestic shorthair cat was evaluated for weakness, lethargy, decreased appetite, diarrhea, weight loss, and seizures. On physical examination, the cat appeared disoriented and had an inconsistent menace response. An insulinoma was diagnosed on the basis of normal serum insulin activity in conjunction with profound hypoglycemia and histologic examination (with immunohistochemical staining for chromogranin A and insulin) of a pancreatic mass that was removed surgically. Blood glucose concentration was within reference limits after surgery. However, neurologic abnormalities persisted, and the cat was euthanatized. Chronic hypoglycemia, associated with insulinomas, can cause irreversible neuronal changes in cats; therefore, rapid diagnosis and treatment of hypoglycemic conditions are of critical importance.
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PMID:Hypoglycemia and irreversible neurologic complications in a cat with insulinoma. 1450 96

An Afghan hound was presented with recurring episodes of weakness progressing to seizures after exercise. Insulinoma was diagnosed by demonstrating excessive insulin secretion in the presence of hypoglycemia. A pancreatic mass was identified and removed during an exploratory laparotomy. Surgical and pathological findings, diagnosis, and clinical management are discussed.
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PMID:Pancreatic insulin-secreting neoplasia in a 9-year-old Afghan hound. 1466 55

Insulinoma tumors are often difficult to detect, as the symptoms largely precede occurrence of a visualized tumor. We report the case of an insulinoma patient with long delayed diagnosis and marked adaptation to extreme hypoglycemia. The patient with a 7-yr history of seizures was found to have plasma glucose concentration during a starvation test as low as 16 mg/dl, with no clinically significant symptoms and concomitant normal plasma insulin levels: 10-30 microIU/ml. All attempts to localize a tumor with repeated abdominal ultrasound examinations or computed tomography scanning were unsuccessful. The patient did not tolerate the introduced oral treatment with diazoxide. Once it had become technically available, endoscopic ultrasonography of the pancreas was performed. It revealed a 10 mm tumor in the pancreatic head. The tumor was subsequently removed surgically. During the operation plasma insulin concentration rose almost 15-fold, which confirmed the insulin-secreting character of the growth. Microscopic examination revealed benign insulinoma, with partially trabecular structure. One month after the operation the patient had normal plasma glucose values of 60-120 mg/dl, but she constantly complained of excessive thirst, which occurred soon after the operation and slowly subsided in the following weeks. In conclusion, the present report demonstrates that insulinoma should be considered and searched for in every case of hypoglycemia associated with normal insulin levels. It also confirms the essential role of endoscopic ultrasonography in the diagnosis of insulin-secreting tumors.
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PMID:Diagnostic difficulties in long-standing insulinoma with near-normal plasma insulin levels. 1588 65

Clinical history and inappropriate insulin secretion during hypoglycemic episodes permit the diagnosis of hyperinsulinism. We report 2 cases of factitious hyperinsulinism leading to partial pancreatectomy. Case 1 was an 8-year-old girl who presented with severe hypoglycemia and elevated insulin and C-peptide levels. Catheterization of pancreatic veins was performed to localize the excess insulin secretion. Insulinoma was suspected, and partial pancreatectomy was performed. Ten days after surgery, severe hypoglycemia recurred with severely elevated plasma insulin levels (x100) but very low C-peptide plasma levels, suggesting factitious hyperinsulinemia. Hypoglycemic episodes before surgery were provoked by oral sulfonamides; postoperative episodes were caused by parenteral insulin. Falsified prescriptions for sulfonamides and insulin by the mother, a nurse, were found. Case 2 was a 6-month-old girl who presented with seizures and hypoglycemia but had a symptom-free interval of many months afterward. At 2 years of age, repeated hypoglycemic seizures and elevated insulin plasma levels suggested congenital hyperinsulinism. C-peptide plasma level, measured once, was normal, but blood sampling was performed 15 minutes after a hypoglycemic episode. Partial pancreatectomy was performed. Two weeks after surgery, hypoglycemic seizures recurred, and the patient was admitted for pancreatic vein catheterization. This investigation was performed during hypoglycemia and revealed high insulin levels and undetectable C-peptide levels, suggesting factitious hypoglycemia. Insulin/C-peptide ratio analysis is crucial to assess factitious hypoglycemia, although sulfonamide-induced hypoglycemia is not thereby detected. One percent (2 of 250) of all cases of hyperinsulinemic hypoglycemia in our unit have been identified as Munchausen syndrome by proxy. Atypical disease history should raise the question of factitious hypoglycemia.
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PMID:Factitious hyperinsulinism leading to pancreatectomy: severe forms of Munchausen syndrome by proxy. 1599 15

Several nuclear and cytoplasmic proteins in metazoans are modified by O-linked N-acetylglucosamine (O-GlcNAc). This modification is dynamic and reversible similar to phosphorylation and is catalyzed by the O-linked GlcNAc transferase (OGT). Hyperglycemia has been shown to increase O-GlcNAc levels in pancreatic beta cells, which appears to interfere with beta-cell function. To obtain a better understanding of the role of O-linked GlcNAc modification in beta cells, we have isolated OGT interacting proteins from a cDNA library made from the mouse insulinoma MIN6 cell line. We describe here the identification of Ataxin-10, encoded by the SCA10 (spinocerebellar ataxia type 10) gene as an OGT interacting protein. Mutations in the SCA10 gene cause progressive cerebellar ataxias and seizures. We demonstrate that SCA10 interacts with OGT in vivo and is modified by O-linked glycosylation in MIN6 cells, suggesting a novel role for the Ataxin-10 protein in pancreatic beta cells.
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PMID:Ataxin-10 interacts with O-GlcNAc transferase OGT in pancreatic beta cells. 1618 53


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