glycogen and Cerebrovascular-Disorders

Topics: Alkaline Phosphatase; Animals; Brain Edema; Cerebrovascular Disorders; Epilepsy; Glycogen; Guinea Pigs; Hematologic Diseases; Humans; Leukocytes; Neoplasms; Rabbits; Tuberculosis; Wounds and Injuries

Pompe disease (glycogen storage disease type 2 or acid maltase deficiency) is a rare autosomal recessive lysosomal storage disorder. Since the advent of ERT a lot has been learned about the phenotypic spectrum especially in the late onset patients. We describe in detail 44 patients diagnosed with late-onset Pompe disease (LOPD) at our neuromuscular department from 1985 to 2011 and compare them to patients with LOPD in the literature of the past 40 years. Study of the Munich LOPD group revealed varying musculoskeletal and cardio-cerebrovascular manifestation patterns. Several of these symptom patterns commonly appeared in conjunction with one another, highlighting the multisystem involvement of this condition. Common symptom patterns include: (i) Classic limb girdle and diaphragmatic weakness, (ii) rigid spine syndrome (RSS), scoliosis, and low body mass, and (iii) several cardio-cerebrovascular manifestation patterns. The most common presentation, limb girdle and diaphragmatic weakness, appeared in 78% (34/44) of our patients and over 80% of those in the literature. Sixteen percent (7/44) of our patients presented with rigid spine, scoliosis, and low body mass. Although scoliosis had a reported frequency of 33% in the general LOPD patient population, the literature only occasionally reported low body mass and RSS. Importantly, a multisystem extramuscular finding accompanied by cardio-cerebrovascular manifestations was found in 29% (13/44) of our LOPD patients; the literature showed an increasing prevalence of this latter finding. By examining the phenotype of patients with confirmed LOPD, we found a more subtle clinical multisystem involvement in LOPD. Whether patients presenting with the different symptom patterns respond differently to enzyme replacement therapy remains a key question for future research. © 2012 Wiley Periodicals, Inc.

Topics: Adolescent; Adult; Age of Onset; Aged; Cardiovascular Abnormalities; Cerebrovascular Disorders; Child; Female; Glycogen; Glycogen Storage Disease Type II; Humans; Male; Mallory Bodies; Middle Aged; Muscular Dystrophies; Muscular Dystrophies, Limb-Girdle; Musculoskeletal Abnormalities; Phenotype; Scoliosis

We examined the effects of intracerebroventricular (ICV) cannula implantation followed by the administration of morphine sulfate (MOR) and its metabolite, morphine-6-glucuronide (M6G), on the glycogen content of the brain, liver, and muscle. ICV cannulation resulted in nearly a 30% reduction in brain glycogen, and ICV MOR resulted in a 36% reduction in liver glycogen content compared to time-matched controls, but it had no additional effect on either the brain or muscle glycogen content. ICV M6G showed a more significant reduction, to 50% of liver glycogen, but it had no effect on either brain or muscle glycogen. Neither IV MOR nor M6G produced any significant alteration in tissue glycogen content. These results indicate that the stress response associated with neurosurgery, especially the placement of the ICV cannula, is associated with a decrement in brain glycogen. The activation of opioid receptors in the brain results in enhanced hepatic glycogenolysis but has no additional effect on the brain glycogen content.

Topics: Analgesics, Opioid; Animals; Brain Chemistry; Catheterization; Cerebrovascular Disorders; Glycogen; Liver; Male; Morphine; Morphine Derivatives; Muscle, Skeletal; Rats; Rats, Sprague-Dawley

Angiotensin-converting enzyme (ACE) inhibitors can improve cardiac function independent of their blood pressure (BP)-lowering actions. We investigated the effect of chronic subantihypertensive ACE inhibitor treatment on functional and biochemical cardiac parameters in stroke-prone spontaneously hypertensive rats (SHRSP). Animals were treated in utero and subsequently to age 20 weeks with the ACE inhibitor perindopril (0.01 mg/kg/day). The contribution of endogenous bradykinin (BK) potentiation to the actions of the ACE inhibitor was assessed by cotreatment with the BK beta 2-receptor antagonist Hoe 140 (500 micrograms/kg/day subcutaneously, s.c.) from age 6 to 20 weeks and by measurement of myocardial prostacyclin and cyclic GMP concentrations. Chronic low-dose perindopril treatment had no effect on development of hypertension and left ventricular hypertrophy (LVH), but perindopril improved cardiac function, as demonstrated by increased LV pressure (LVP) (19.4%) and LVdp/dtmax (27.8%) but no change in heart rate (HR). The activities of lactate dehydrogenase (LDH) and creatine kinase (CK) as well as lactate concentrations in the coronary venous effluent were reduced by 39.3, 50, and 60.6%, respectively. Myocardial tissue concentrations of glycogen and the energy-rich phosphates ATP and CK were increased by 16.3, 33.1, and 28.2%, respectively. All ACE inhibitor-induced effects on cardiac function and metabolism were abolished by concomitant chronic BK receptor blockade. Cardiac prostacyclin concentrations were threefold elevated in perindopril-treated animals whereas cardiac cyclic GMP concentration remained unchanged as compared with that of controls. Our data demonstrate that chronic low-dose ACE inhibitor treatment can improve cardiac function and metabolism by potentiating endogenous BK.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: 6-Ketoprostaglandin F1 alpha; Angiotensin-Converting Enzyme Inhibitors; Animals; Blood Pressure; Bradykinin; Cerebrovascular Disorders; Coronary Circulation; Creatine Kinase; Cyclic GMP; Disease Models, Animal; Glycogen; Heart; Heart Rate; Hypertension; Hypertrophy, Left Ventricular; Indoles; L-Lactate Dehydrogenase; Myocardium; Perindopril; Rats; Rats, Inbred SHR

The effect of chronic low- and high-dose treatment with the angiotensin-converting enzyme (ACE) inhibitor ramipril (0.01 and 1 mg/kg per day) on the development of hypertension and left ventricular hypertrophy as well as on functional and biochemical alterations of the heart was studied in stroke-prone spontaneously hypertensive rats treated prenatally and subsequently up to the age of 20 weeks. The contribution of endogenous bradykinin potentiation to the ACE inhibitor actions was assessed by cotreatment of rats with the bradykinin B2-receptor antagonist Hoe 140 (500 micrograms/kg per day SC) from 6 to 20 weeks of age. High- but not low-dose ACE inhibitor treatment prevented the development of hypertension and left ventricular hypertrophy. Chronic bradykinin receptor blockade did not attenuate the antihypertensive and antihypertrophic actions of ramipril. High-dose ramipril treatment improved cardiac function, as demonstrated by an increase in left ventricular pressure (29.9%), dP/dtmax (34.9%), and coronary flow (22.1%), without a change in heart rate. The activities of lactate dehydrogenase and creatine kinase and lactate concentration in the coronary effluent were reduced by 39.3%, 55.5%, and 66.7%, respectively. Myocardial tissue concentrations of glycogen and the energy-rich phosphates ATP and creatine phosphate were increased by 31.3%, 39.9%, and 73.7%, respectively, whereas lactate was decreased by 20.8%. Chronic low-dose ACE inhibitor treatment led to a pattern of changes in cardiodynamics and cardiac metabolism similar to that observed with the high dose. All ACE inhibitor-induced effects on cardiac function and metabolism were abolished by chronic bradykinin receptor blockade.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adenosine Triphosphate; Administration, Oral; Animals; Bradykinin; Cerebrovascular Disorders; Coronary Circulation; Creatine Kinase; Dose-Response Relationship, Drug; Female; Glycogen; Heart; Hypertension; Hypertrophy, Left Ventricular; L-Lactate Dehydrogenase; Lactates; Male; Myocardium; Phosphocreatine; Pregnancy; Ramipril; Rats; Rats, Inbred SHR; Rats, Wistar; Ventricular Pressure

Topics: Acid Phosphatase; Adult; Aged; Alkaline Phosphatase; Brain; Cerebral Hemorrhage; Cerebrovascular Disorders; Electron Transport Complex IV; Female; Glycogen; Histocytochemistry; Humans; Ischemia; Ischemic Attack, Transient; Leukocytes; Male; Middle Aged; Peroxidases

A cerebral ischemia was produced by unilateral ligation of the common carotid artery in the neck of Mongolian gerbils (Meriones unguiculatus), which are frequently characterized by deficiencies in the circulus of Willis. Concentrations of glucose, lactate, pyruvate and glycogen were measured in the hemisphere on the side of occlusion and in the contralateral control hemisphere of animals sacrificed after 5, 15 and 30 min, as well as after 1,3,5 and 9 hrs of carotid clamping. Significant decrease of glucose, and increase in lactate and pyruvate concentration were found in the hemisphere ipsilateral to occlusion; the extent of the changes was proportional to the duration of the ischemia. After an initial fall, an increase in the glycogen content occurred in the later stages of ischemia. Glycogen, glucose, lactate and pyruvate were determined also at 1, 5, 20 hrs and 1 week intervals following release of an occlusion lasting for 1 hr. Return to normal values of glucose and pyruvate was seen at 1 hr after release. The lactate and glycogen levels were significantly raised on the occluded side after 20 hrs release. An increased level of glycogen was observed as long as 1 week after a 1-hr carotid occlusion.

Topics: Animals; Brain; Carbohydrate Metabolism; Carotid Arteries; Cerebrovascular Disorders; Gerbillinae; Glucose; Glycogen; Ischemia; Lactates; Ligation; Pyruvates; Time Factors

Topics: Acid-Base Equilibrium; Adenosine Diphosphate; Adenosine Monophosphate; Adenosine Triphosphate; Animals; Brain; Carbon Dioxide; Cerebrovascular Disorders; Electroencephalography; Energy Metabolism; Glucose; Glycogen; Hydrogen-Ion Concentration; Infarction; Lactates; Male; Oxygen; Phosphocreatine; Pyruvates; Rats; Rats, Inbred Strains; Time Factors; Water-Electrolyte Balance

Topics: Acid Phosphatase; Animals; Brain; Cerebral Cortex; Cerebrovascular Disorders; Disease Models, Animal; Glucuronidase; Glycogen; Hemiplegia; Hydrolases; Hypoxia; Hypoxia, Brain; Ischemic Attack, Transient; Lysosomes; Male; Mitochondria; Rats

Topics: Animals; Brain; Brain Chemistry; Cerebrovascular Disorders; Female; Glucosyltransferases; Glycogen; Ischemia; Male; Phosphorylases; Rats; Time Factors

Topics: Adenosine Triphosphate; Anesthetics; Brain; Brain Edema; Cerebral Cortex; Cerebrovascular Circulation; Cerebrovascular Disorders; Coma; Consciousness; Diabetic Coma; Glucose; Glycogen; Hepatic Encephalopathy; Humans; Hypoglycemia; Lactates; Oxygen Consumption; Seizures; Sleep

Topics: Adolescent; Adult; Aged; Biopsy; Cell Nucleus; Cerebrovascular Disorders; Cytoplasm; Female; Glycogen; Humans; Lysosomes; Male; Microscopy, Electron; Middle Aged; Mitochondria, Muscle; Muscles; Muscular Atrophy; Neoplasms, Nerve Tissue; Sarcoplasmic Reticulum

Topics: Adenosine Triphosphate; Animals; Animals, Newborn; Blood Glucose; Brain; Cerebrovascular Disorders; Glucose; Glycogen; Hexosephosphates; Hypoxia, Brain; Ischemia; Lactates; Mice; Phosphocreatine