1/74. Brainstem dysfunction in chiari malformation presenting as profound hypoglycemia: presentation of four cases, review of the literature, and conjecture as to mechanism.OBJECTIVE: We report four patients whose cases resulted in our observation that profound hypoglycemia resulting from intermittent hyperinsulinism plays a significant role in patients with brainstem dysfunction from Chiari I or II malformations who have intermittent autonomic dysfunction ("blue spells"). methods: The records of four children with severe brainstem dysfunction associated with hindbrain herniation (Chiari I or II malformation) were reviewed retrospectively. Each patient had severe lower cranial nerve dysfunction that required tracheotomy and feeding tube placement. After we found that profound hypoglycemia had occurred during a spell of autonomic dysfunction in one patient, the charts of the other three patients were reviewed for evidence of hypoglycemia. Now, whenever one of them has evidence of autonomic dysfunction, prospective studies of glucose and insulin levels are performed. Three of the patients had Chiari II malformation in association with myelomeningocele, and one patient had a Chiari I malformation resulting from Pfeiffer's syndrome. RESULTS: hypoglycemia occurred in these patients episodically, and usually when their shunts were functioning. The hypoglycemia was associated with hyperinsulinemia in each patient. The brainstem structures of these children (presumably the dorsal motor nuclei of the vagus) were extremely sensitive to changes in local or regional intracranial pressure. These changes were triggered by intermittent shunt failure, agitation from pain, abdominal distention from constipation, and retention of CO2. In patients with Chiari malformations, even mild increases in intracranial pressure lead to brainstem dysfunction. One possible explanation is that pressure on the deformed Xth cranial nerve nuclei may lead to insulin release and life-threatening hypoglycemia. Continuous-drip feeds are necessary to prevent this complication. CONCLUSION: patients with severe intermittent brainstem dysfunction after decompression of Chiari I or Chiari II malformations should have laboratory studies of glucose levels performed at the time of the episodes to rule out hypoglycemia.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/74. hemiplegia hypoglycaemia syndrome.We report the case of a 83-year-old man who presented to the emergency department with hypoglycaemia resembling a cerebrovascular accident. Hypoglycaemic hemiparesis is an under-recognized manifestation of hypoglycaemia. If not recognized and treated promptly, hypoglycaemia may cause irreversible central nervous system injury; it rarely results in death. It is imperative that emergency physicians consider hypoglycaemia in all patients with coma in spite of focal neurological deficit even when the findings seem to be explained initially by other aetiologies.- - - - - - - - - - ranking = 0.062738140817541keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/74. Hypoglycemic coma masquerading thyrotoxic storm.A 59-year-old woman was hospitalized in hypoglycemic coma. Although hypoglycemia was promptly reversed, she was in a somnolent, restless state with tachycardia, tremor, profuse sweating, and high body temperature. Thyrotoxic storm was highly suspected and vigorous antithyroid regimens gradually brought her up to normal mental and cardiovascular states in several days. However, profound generalized myopathy necessitated the maintenance with a respirator. One month later, an episode of angina pectoris was followed by generalized convulsion, coma, and death in a few days. neuroimaging study disclosed posterior leukoencephalopathy syndrome. This case is instructive in that hypoglycemic coma may masquerade the major symptomatology of thyrotoxic storm, and that profound myopathy and angiopathic or angiospastic processes of the brain and the heart may interfere with the outcome.- - - - - - - - - - ranking = 0.16666666666667keywords = brain (Clic here for more details about this article) |
4/74. 'Speaking in tongues'. Paradoxical fixation on a non-native language following anaesthesia.An intriguing case of transient language disturbance following anaesthesia is described which may throw some light on the way languages are stored in the brain. A review of the existing literature and its relevance to this unique case is discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = brain (Clic here for more details about this article) |
5/74. Hypoglycinaemia and psychomotor delay in a child with xeroderma pigmentosum.glycine is a nonessential amino acid that serves as both an inhibitory and an excitatory neurotransmitter. Hyperglycinaemia occurs in non-ketotic hyperglycinaemia, a primary defect in the glycine cleavage pathway, and as a secondary feature of several inborn errors of organic acid metabolism. However, specifically low levels of glycine have never been reported. Here we report a child with complementation group C xeroderma pigmentosum (XP) characterized by a splice donor mutation in the XPC gene, multiple skin cancers and specific and persistent hypoglycinaemia. He has cognitive delay, lack of speech, autistic features, hyperactivity and hypotonia, all unexplained by the diagnosis of XP group C, a non-neurological form of the disease. Treatment with oral glycine has improved his hyperactivity. Specific hypoglycinaemia could indicate a metabolic disorder producing neurological dysfunction. Whether it is related to or coincidental with the XP is unclear.- - - - - - - - - - ranking = 50.182808536268keywords = metabolic disorder (Clic here for more details about this article) |
6/74. Normal pressure hydrocephalus in diabetic patients with recurrent episodes of hypoglycemic coma.The pathophysiology of brain damage induced by severe hypoglycemia is still unknown. We experienced a case with type 1 diabetes and recurrent severe hypoglycemic coma who showed a central brain atrophy and an abnormal cerebrospinal fluid flow, suggesting normal pressure hydrocephalus. Following this case, the CSF flow was studied using 111In-DTPA cisternography in six consecutive diabetic patients admitted for repeated episodes of hypoglycemic coma. All the patients showed the central brain atrophy on computed tomography and four of them (67%) had the ventricular reflux, with delayed clearance of 111In-DTPA. Two patients with abnormal CSF flow showed cognitive dysfunction by WAIS or WAIS-R. In contrast, none of five randomly selected diabetic patients, without hypoglycemic coma showed abnormal CSF flow. Our results suggest the presence of normal pressure hydrocephalus in diabetic patients with recurrent hypoglycemic coma. It may associate with the cognitive dysfunction.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
7/74. myocardial infarction with acute insulin poisoning--a case report.A 36-year-old woman without overt coronary risk factors was admitted to hospital with coma about 9 hours after mass self-injection of insulin (1,500 units). Laboratory investigation revealed severe hypoglycemia and hyperinsulinemia. During the treatment of her hypoglycemia, circulatory collapse occurred. The ECG, echocardiogram, and elevation in troponin t suggested a diagnosis of myocardial infarction. Although the patient became apallic and developed systemic spasticity due to hypoglycemic brain damage, her hemodynamics improved with supportive care alone. coronary angiography and myocardial scintigraphy performed later demonstrated a broad area of myocardial damage despite intact coronary artery circulation. The authors hypothesize that temporary coronary arterial narrowing or coronary arterial vasospasm induced by severe hyperinsulinemia contributed to the pathogenesis of the myocardial infarction. The possibility of myocardial infarction should be considered in patients with acute insulin poisoning.- - - - - - - - - - ranking = 0.16666666666667keywords = brain (Clic here for more details about this article) |
8/74. Hyperinsulinemic hypoglycemia associated with possible hypopituitarism in a patient with acquired immunodeficiency syndrome.OBJECTIVE: To describe endocrine dysfunctions associated with human immunodeficiency virus (hiv) infection. methods: We present a case report, discuss the laboratory findings, and suggest potential contributing factors in this complex milieu. RESULTS: A 44-year-old man infected with hiv was admitted with loss of consciousness after a grand mal seizure and was found to have a prolonged course of hypoglycemia in conjunction with increased serum levels of insulin and C peptide. Iatrogenic hypoglycemia was excluded by laboratory evaluations. Hypothalamic-pituitary dysfunction was suspected because the serum growth hormone and cortisol concentrations during hypoglycemia were low. Radiologic evaluation of the brain revealed no structural abnormalities involving the hypothalamic-pituitary area. A normal cortisol response to adrenocorticotropic hormone stimulation (cosyntropin, 250 mg) was documented on two occasions. The cause of the severe hypoglycemic episode and the possible hypothalamic-pituitary-adrenal axis dysfunction in this patient is unknown. CONCLUSION: Despite the complexity of endocrine disorders in hiv-infected patients, the course of the disease in this patient underscores the possibility that some of the endocrinologic changes may be reversible.- - - - - - - - - - ranking = 0.16666666666667keywords = brain (Clic here for more details about this article) |
9/74. Hereditary gingival fibromatosis and growth retardation.OBJECTIVE: To describe two patients with hereditary gingival fibromatosis (HGF) and growth hormone deficiency and to review the literature on HGF and related endocrine abnormalities. methods: We present case reports of two patients (first cousins)-an 8-year-old girl and a 13-year-old boy-with an existing diagnosis of HGF, who were assessed because of presumed growth failure. Both patients underwent growth hormone stimulation testing and more in-depth endocrine evaluation, including measurement of morning cortisol, adrenocorticotropic hormone (ACTH), and prolactin levels as well as thyroid function tests. An ACTH stimulation test was also performed. Radiologic evaluation included assessment of bone age and magnetic resonance imaging of the brain. RESULTS: In addition to HGF, both patients had short stature, subnormal growth velocity, and delayed bone age but no abnormalities on magnetic resonance imaging of the brain. serum prolactin levels and results of thyroid function tests were normal. Subnormal growth hormone response was noted during hypoglycemia and pharmacologic stimuli with clonidine and levodopa. The female patient, who also had recurrent hypoglycemic episodes, had a suboptimal cortisol and ACTH response during hypoglycemia. On the ACTH stimulation test, she showed an inadequate cortisol response at 30 minutes but a normal response at 60 minutes. The male patient had normal morning cortisol and ACTH levels plus a normal response to ACTH stimulation. Both patients are responding well to treatment with growth hormone. The girl is also receiving cortisol replacement and has had no further episodes of hypoglycemia. CONCLUSION: Although HGF has been described as an isolated finding, it can occur as part of a syndrome, including infrequent endocrine abnormalities such as growth hormone insufficiency. The cause of the growth hormone deficiency remains unclear in these two patients. We believe that patients with HGF should be monitored carefully for a prolonged period for growth as well as other endocrine abnormalities.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
10/74. Profound postoperative hypoglycemia in a malnourished patient.PURPOSE: To present an unusual case of profound postoperative hypoglycemia resulting in irreversible brain damage in a malnourished patient. CLINICAL FEATURES: A 56-yr-old malnourished woman underwent laparotomy for intestinal obstruction under general anesthesia. Five hours postoperatively she was found to be unresponsive with an immeasureably low blood glucose level. This event was not associated with hyperinsulinemia. Predisposing factors like diabetes mellitus, pheochromocytoma, insulin secreting tumours, adrenal or pitutary deficiency were absent. She was treated with iv dextrose and hydrocortisone with blood glucose levels stabilizing fairly rapidly. However, she unfortunately had sustained irreversible cerebral damage and is left with significant neurological disability. CONCLUSION: Severe postoperative hypoglycemia has several well documented causes. Although hypoglycemia does occur to a moderate degree in malnutrition, it has not been reported to be so severe as to cause cerebral damage in the postoperative setting.- - - - - - - - - - ranking = 0.16666666666667keywords = brain (Clic here for more details about this article) |
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