The purpose of this animal experiment was to evaluate the changes of bone mineral density in paralyzed limbs, and to assess the effects of electrically stimulating muscle contraction upon bone mineral density (BMD) in paralyzed limbs during the four week period immediately following spinal cord injury (SCI). Ten rabbits were used for the study, spinal cords were totally transected at the T11 spine level. The paralyzed quadriceps femoris of one limb was contracted by electrical stimulation for 60-minutes daily, while the other side was not stimulated as a control. The BMD of each lower limb was measured by Dual Photon Absorptiometry before and four weeks after acute SCI. BMD of both limbs decreased in all rabbits four weeks after SCI. The decrease in BMD for stimulated and non-stimulated limbs was 6.130 +/- 3.212% and 9.098 +/- 3.831%, respectively during the four-week period after SCI. The BMD of stimulated limbs decreased significantly less than that of the non-stimulated limbs. Electrically induced muscular contraction reduced bone mineral loss in the paralyzed limb during the early stage of SCI in the rabbit.
Animal ; Bone Density* ; Electric Stimulation Therapy* ; Hindlimb* ; Male ; Paralysis/therapy* ; Paralysis/metabolism* ; Rabbits

Congenital adrenal hyperplasia is an uncommon diagnosis in routine clinical practice. 21-hydroxylase deficiency, which is its most common subtype, may be diagnosed at birth in a female infant by virilisation or by features of salt wasting in both genders. However, other uncommon subtypes of this condition such as 17-alpha-hydroxylase deficiency, 11-beta-hydroxylase deficiency may present much later in adolescence or adulthood. A high index of suspicion is necessary when evaluating children with hypertension, hypokalaemia, metabolic alkalosis or sexual infantilism.
Adolescent ; Adrenal Hyperplasia, Congenital ; diagnosis ; genetics ; Alkalosis ; diagnosis ; Diagnosis, Differential ; Female ; Humans ; Hypertension ; diagnosis ; Hypokalemia ; diagnosis ; Models, Biological ; Paralysis ; diagnosis ; Sexual Infantilism ; diagnosis ; Steroid 21-Hydroxylase ; metabolism

Hypokalemic periodic paralysis (HOPP) is a rare disease characterized by reversible attacks of muscle weakness accompanied by episodic hypokalemia. Recent molecular work has revealed that the majority of familial HOPP is due to mutations in a skeletal muscle voltage-dependent calcium-channel: the dihydropyridine receptor. We report a 13-yr old boy with HOPP from a family in which 6 members are affected in three generations. Genetic examination identified a nucleotide 3705 C to G mutation in exon 30 of the calcium channel gene, CACNA1S. This mutation predicts a codon change from arginine to glycine at the amino acid position #1239 (R1239G). Among the three known mutations of the CACNA1S gene, the R1239G mutation was rarely reported. This boy and the other family members who did not respond to acetazolamide, showed a marked improvement of the paralytic symptoms after spironolactone treatment.
Acetazolamide/pharmacology ; Adolescent ; Arginine/chemistry ; Calcium Channels/chemistry/*genetics ; Codon ; Exons ; Family Health ; Female ; Glycine/chemistry ; Humans ; Hypokalemia/metabolism ; Hypokalemic Periodic Paralysis/*diagnosis/*genetics ; Korea ; Male ; Muscle, Skeletal/metabolism ; Mutation ; Pedigree ; Protein Structure, Tertiary ; Sequence Analysis, DNA ; Spironolactone/pharmacology

OBJECTIVETo study the transfer of paralytic shellfish toxins (PST) using four simulated marine food chains: dinoflagellate Alexandrium tamarense --> Artemia Artemia salina --> Mysid shrimp Neomysis awatschensis; A. tamarense --> N. awatschensis; A. tamarense --> A. salina --> Perch Lateolabrax japonicus; and A. tamarense --> L. japonicus.

METHODSThe ingestion of A. tamarense, a producer of PST, by L. japonicus, N. awatschensis, and A. salina was first confirmed by microscopic observation of A. tamarense cells in the intestine samples of the three different organisms, and by the analysis of Chl.a levels in the samples. Toxin accumulation in L. japonicus and N. awatschensis directly from the feeding on A. tamarense or indirectly through the vector of A. salina was then studied. The toxicity of samples was measured using the AOAC mouse bioassay method, and the toxin content and profile of A. tamarense were analyzed by the HPLC method.

RESULTSBoth A. salina and N. awatschensis could ingest A. tamarense cells. However, the ingestion capability of A. salina exceeded that of N. awatschensis. After the exposure to the culture of A. tamarense (2000 cells x mL(-1)) for 70 minutes, the content of Chl.a in A. salina and N. awatschensis reached 0.87 and 0.024 microg x mg(-1), respectively. Besides, A. tamarense cells existed in the intestines of L. japonicus, N. awatschensis and A. salina by microscopic observation. Therefore, the three organisms could ingest A. tamarense cells directly. A. salina could accumulate high content of PST, and the toxicity of A. salina in samples collected on days 1, 4, and 5 of the experiment was 2.18, 2.6, and 2.1 MU x g(-1), respectively. All extracts from the samples could lead to death of tested mice within 7 minutes, and the toxin content in artemia sample collected on the 1st day was estimated to be 1.65 x 10(-5) microg STX equal/individual. Toxin accumulation in L. japonicus and N. awatschensis directly from the feeding on A. tamarense or indirectly from the vector of A. salina was also studied. The mice injected with extracts from L. japonicus and N. awatschensis samples that accumulated PST either directly or indirectly showed PST intoxication symptoms, indicating that low levels of PST existed in these samples.

CONCLUSIONParalytic shellfish toxins can be transferred to L. japonicus, N. awatschensis, and A. salina from A. tamarense directly or indirectly via the food chains.

Animals ; Artemia ; drug effects ; Cell Count ; Chlorophyll ; metabolism ; Eukaryota ; drug effects ; Feeding Behavior ; drug effects ; Fishes ; Food Chain ; Hydrolysis ; Marine Toxins ; analysis ; metabolism ; toxicity ; Mice ; Models, Biological ; Mollusca ; chemistry ; Paralysis ; chemically induced

OBJECTIVETo study the apoptosis of facial motor neurons and the expression of apoptosis-related genes, Bcl-2 and Bax, in the animal model of viral facial paralysis.

METHODSTotal of 84 Balb/c mice were divided into viral inoculation group and nerve transaction group. The animals were executed 1, 3, 7, 10, 15, 20 and 30 days after being operated respectively. The histopathological features of facial neurons in brain stem were observed by HE and Nissl stain. The changes of facial neuronal apoptosis were observed by TUNEL. The changes of expression of Bcl-2 and Bax genes in facial neurons were observed by immunohistochemistry staining.

RESULTSAfter nerve transection, increased apoptotic cells were found in homolateral facial motor nucleus and the peak appeared at 10 and 15 days. The level of Bcl-2 expression in neurons declined while the expression of Bax increased gradually. Correspondingly, the ratio of Bcl-2/Bax declined. In the viral inoculation group, no visible change of apoptosis and Bax expression, but the level of Bcl-2 and the ratio of Bcl-2/Bax increased gradually.

CONCLUSIONSComparing to axotomy, facial motor nucleus in HSV-1 infective animal model are free of apoptosis. Both the mild form of lesion and the ability to block apoptosis of HSV-1 are likely to be involved into the phenomenon. Bcl-2 and Bax might interfere with the apoptotic response.

Animals ; Apoptosis ; Facial Paralysis ; pathology ; virology ; Female ; Herpesvirus 1, Human ; pathogenicity ; Mice ; Mice, Inbred BALB C ; Neurons ; pathology ; Proto-Oncogene Proteins ; metabolism ; Proto-Oncogene Proteins c-bcl-2 ; bcl-2-Associated X Protein ; metabolism

BACKGROUND: Total oxygen consumption has been found to be reduced under deep neuromuscular blockade due to a lower rate of metabolism of skeletal muscles. However, the magnitude of this effect in individual muscles has not been investigated. Thus the aim of this study was to compare the oxygenation of paralyzed versus non-paralyzed forearm muscle under tourniquet-provoked ischemia. METHODS: After ethics approval and written informed consent, 30 patients scheduled for elective hand and wrist surgery were included. Ischemia was provoked by inflation of bilateral upper arm tourniquets and muscle relaxation was achieved via intravenous administration of rocuronium 0.9 mg/kg. Bilateral tourniquets were applied to both upper arms before induction of anesthesia and near infrared spectrometry (NIRS) electrodes applied on both forearms. Muscular ischemia in an isolated (= non-paralyzed, NP) as well as a paralyzed forearm (P) was created by sequential inflation of both tourniquets before and after intravenous administration of rocuronium. Muscle oxygen saturations (SmO2) of NIRS in both forearms and their changes were determined and compared. RESULTS: Data of 30 patients (15 male, 15 female; 41.8 +/- 14.7 years) were analyzed. The speed of SmO2 decrease (50% decrease of SmO2 from baseline (median [percentiles]: NP 210 s [180/480s] vs. P 180 [180/300]) as well as the maximum decrease in SmO2 (minimum SmO2 in % (median [percentiles]: NP 20 [19/24] vs. P 21 [19/28]) were not significantly affected by neuromuscular paralysis. CONCLUSIONS: No significant effect of muscle relaxation on NIRS-assessed muscle oxygenation under tourniquet-induced ischemia was found in human forearm muscles.
Administration, Intravenous ; Anesthesia ; Arm ; Electrodes ; Ethics ; Female ; Forearm* ; Hand ; Humans ; Inflation, Economic ; Informed Consent ; Ischemia ; Male ; Metabolism ; Muscle Relaxation* ; Muscle, Skeletal* ; Muscles ; Neuromuscular Blockade ; Oxygen Consumption ; Oxygen* ; Paralysis ; Prospective Studies* ; Spectroscopy, Near-Infrared ; Spectrum Analysis ; Tourniquets ; Wrist

OBJECTIVETo study the effects of inducible NOS inhibitor aminoguanidine on the expression of NOS in facial nerve and surrounding tissue of traumatic facial paralysis rats.

METHODSA small dose of aminoguanidine were intraperitoneally injected into rats before and after facial paralysis. The facial nerve and surrounding tissues were cut at different time point. Immunohistochemical ABC method was used to study the changes of NOS expression in facial nerve and surrounding tissues.

RESULTSThe inducible NOS immunoreactivity was obvious inhibited in the facial nerve and surrounding tissues in aminoguanidine group.

CONCLUSIONAminoguanidine chronic treatment can obvious inhibit the inducible NOS expression in the facial nerve and surrounding tissues. Aminoguanidine can improve the regeneration of facial nerve and the recovery of traumatic tissues.

Animals ; Facial Nerve ; enzymology ; Facial Nerve Injuries ; enzymology ; Facial Paralysis ; enzymology ; Guanidines ; pharmacology ; Male ; Muscles ; enzymology ; Nerve Regeneration ; Nitric Oxide Synthase ; antagonists & inhibitors ; metabolism ; Nitric Oxide Synthase Type II ; Random Allocation ; Rats ; Rats, Sprague-Dawley

Ataxia ; genetics ; physiopathology ; Calcium Channels ; genetics ; physiology ; Epilepsy ; genetics ; physiopathology ; Genetic Diseases, Inborn ; genetics ; physiopathology ; Humans ; Hypokalemic Periodic Paralysis ; genetics ; physiopathology ; Malignant Hyperthermia ; genetics ; physiopathology ; Migraine with Aura ; genetics ; physiopathology ; Myopathy, Central Core ; genetics ; physiopathology ; Ryanodine ; metabolism ; Spinocerebellar Ataxias ; genetics ; physiopathology

OBJECTIVECombine olfactory ensheathing glia (OEG) implantation with ex vivo non-viral vector-based neurotrophin-3 (NT-3) gene therapy in attempting to enhance regeneration after thoracic spinal cord injury (SCI).

METHODSPrimary OEG were transfected with cationic liposome-mediated recombinant plasmid pcDNA3.1(+)-NT3 and subsequently implanted into adult Wistar rats directly after the thoracic spinal cord (T9) contusion by the New York University impactor. The animals in 3 different groups received 4x10(5) OEG transfected with pcDNA3.1(+)-NT3 or pcDNA3.1(+) plasmids, or the OEGs without any plasmid transfection, respectively; the fourth group was untreated group, in which no OEG was implanted.

RESULTSNT-3 production was seen increased both ex vivo and in vivo in pcDNA3.1(+)-NT3 transfected OEGs. Three months after implantation of NT-3-transfected OEGs, behavioral analysis revealed that the hindlimb function of SCI rats was improved. All spinal cords were filled with regenerated neurofilament-positive axons. Retrograde tracing revealed enhanced regenerative axonal sprouting.

CONCLUSIONNon-viral vector-mediated genetic engineering of OEG was safe and more effective in producing NT-3 and promoting axonal outgrowth followed by enhancing SCI recovery in rats.

Animals ; Animals, Newborn ; Brain Tissue Transplantation ; methods ; Cells, Cultured ; DNA, Recombinant ; therapeutic use ; Disease Models, Animal ; Female ; Gene Transfer Techniques ; Genetic Therapy ; methods ; Genetic Vectors ; genetics ; Graft Survival ; genetics ; Growth Cones ; metabolism ; ultrastructure ; Nerve Regeneration ; genetics ; Neuroglia ; metabolism ; transplantation ; Neurotrophin 3 ; biosynthesis ; genetics ; Olfactory Bulb ; cytology ; transplantation ; Paralysis ; metabolism ; physiopathology ; therapy ; Plasmids ; genetics ; Rats ; Rats, Wistar ; Recovery of Function ; genetics ; Spinal Cord Injuries ; metabolism ; physiopathology ; therapy ; Treatment Outcome ; Up-Regulation ; genetics