Leigh's disease is a rare progressive neurological disorder that is characterized light microscopically by focal spongy necrosis in the brain and electron microscopically by mitochondriopathy. We report an autopsy case of Leigh's disease that showed abnormalities in the liver, kidney and skeletal muscle as well as the central nervous system. The patient was an 18-month-old girl who has carried a diagnosis of cerebral palsy ever since her birth to a 20-year-old mother. The baby was generally hypertonic and mentally retarded. She died of severe metabolic acidosis. Postmortem examination showed growth retardation, fatty liver, fatty kidney and soft brain. Brain section showed multifocal softenings in the brainstem, basal ganglia and periventricular areas. Microscopically increased capillaries with endothelial proliferation, vacuolar degeneration and mild gliosis were seen in the brain. The axons were relatively preserved. Liver and kidneys showed microvesicular fatty change. Myofiber degeneration of the skeletal muscle was also noted. Electron microscopic examination showed markedly increased mitochondria in the parenchymal cells of the brain, liver and kidney. The mitochondria showed round to ovoid ballooned appearance including electron-dense core-like structures and pseudoinclusions of glycogen granules.
Brain/pathology/ultrastructure ; Female ; Humans ; Infant ; Kidney/pathology/ultrastructure ; Leigh Disease/*pathology ; Liver/pathology/ultrastructure ; Mitochondrial Encephalomyopathies/pathology ; Muscles/pathology

Meningioangiomatosis is a rare benign hamartomatous lesion. We describe a case of meningioangiomatosis in an 18-year-old boy with a 15 year history of seizures. Computed tomography reveals an irregular calcification density along the right temporal gyrus. Microscopically, irregularly branched blood vessels, surrounded by a concentric arrangement of proliferating spindle cells, are extending into the gray matter from the meningeal surface. Ultrastructural and immunohistochemical examination failed to demonstrate features of meningothelial cell origin in this case. This is the first case of meningioangiomatosis published in Korea along with immunohistochemical and electron microscopic studies. The pathogenesis and previous reports of this lesion will be discussed.
Adolescent ; Brain Neoplasms/*pathology/radiography/ultrastructure ; Hemangioma/*pathology/radiography/ultrastructure ; Humans ; Immunohistochemistry ; Male ; Microscopy, Electron

OBJECTIVESTo investigate the findings of magnetic resonance (MR) imaging and histopathology in early postoperative normal brain, and to define the correlation between MR images and histopathology.

METHODSThirty-six New Zealand rabbits weighing 2.0 to 3.0 kg were divided into 10 groups according to different postoperative days: 1 to 10 days. A partial resection of the parietooccipital region was performed under usual aseptic conditions after the animals were anesthetized intravenously with 3% pentobarbital (30 mg/kg). MR imaging procedures consisted of pre- and postcontrast scanning and were carried out on postoperative days 1 to 10. Brain tissue samples were prepared for examination immediately after MR scanning. Histopathological examination was done under light both and electron microscopes. The findings of MR imaging were compared with histopathologic findings.

RESULTSSurgical margin contrast enhancement on MR images could be seen 24 hours after surgery. The degree of contrast enhancement increased gradually up to 5 days postoperation, and no remarkable changes were present from days 5 to 10. Disruption of the blood brain barrier (BBB) was the main cause of contrast enhancement during the first 3 postoperative days. After that period, the mechanism responsible for contrast enhancement was the formation of neovascularity and a broken BBB. An increase in the amount of neovascularity played a predominant role in contrast enhancement in normal postoperative brain tissue.

CONCLUSIONSThe features of enhanced MR images present at the surgical margin followed a typical time course during the early postoperative period. The role of neovascularity and BBB disruption in the formation of contrast enhancement at the surgical margin varies with time. Knowledge of the features of contrast enhancement in postoperative MR images of normal brain can help in differentiating benign changes from residual malignant glioma.

Animals ; Brain ; pathology ; surgery ; ultrastructure ; Dura Mater ; pathology ; ultrastructure ; Edema ; pathology ; Magnetic Resonance Imaging ; methods ; Microscopy, Electron ; Rabbits

OBJECTIVETo investigate pathological changes in the epileptogenic foci of children with intractable epilepsy and their clinical significance.

METHODSThirty children with intractable epilepsy were included in the study. The epileptogenic foci were surgically resected and pathological changes in the obtained specimens were observed under a light microscope (LM) and a transmission electron microscope (TEM).

RESULTSUnder the LM, cortical dysplasia was found in 14 cases (47%), hippocampal sclerosis in 11 cases (37%), dysembryoplastic neuroepithelial tumor in 1 case (3%), ganglioglioma in 1 case (3%), and encephalomalacia in 3 cases (10%). The TEM observation revealed pathological changes in the ultrastructure of the hippocampus and extra-hippocampal cortex, such as changes in the number of synapses and synaptic structure, decrease in neurons and karyopyknosis, swelling and degeneration of astrocytes, and changes in mitochondrial structures.

CONCLUSIONSPathological changes in the hippocampus and extra-hippocampal cortex, especially synaptic remodeling, may be the morphological basis for spontaneous recurrent seizures in children with intractable epilepsy. The pathological changes and epileptiform activity are related to an imbalance between excitatory and inhibitory neurotransmission.

Adolescent ; Brain ; pathology ; ultrastructure ; Cerebral Cortex ; pathology ; ultrastructure ; Child ; Child, Preschool ; Epilepsy ; pathology ; surgery ; Female ; Hippocampus ; pathology ; ultrastructure ; Humans ; Infant ; Intelligence ; Male ; Microscopy, Electron, Transmission

OBJECTIVETo document ultrastructural changes of brain, spinal cord, skeletal muscle, jejunum and lung of EV71 infection mouse model, and to explore the myotropism and pathogenesis of EV71 in nervous system.

METHODSTen-day-old suckling mice were infected with EV71 strain via the intraperitoneal route. Mice with paralysis were scarified on day 4 post infection and the brain, spinal cord, skeletal muscle, jejunum and lung were sampled for transmission electron microscopy and light microscopy.

RESULTSLesions in brain were generally mild with inner chamber swelling in some of mitochondria. Myelin sheaths of medullated fibers were split with vacuolated changes. The Nissl bodies in anterior motor neurons disappeared along with mitochondria swelling, rough endoplasmic reticulum swelling and degranulation. Cytoplasm of anterior motor neurons showed cribriform appearance accompanied by neuronophagia. The bands of skeletal muscle in the infected group disappeared with degeneration and karyopyknosis in myocytes, in addition to mitochondrial swelling. Microvilli of epithelium in jejunum became loosely arranged along with formation of spiral medullary sheath structure and mitochondria swelling. Interstitial pneumonia was observed in lungs with type II pneumocyte proliferation and evacuation of the multilamellar bodies.

CONCLUSIONSEV71 infection causes severe myositis in the mouse model suggesting a strong myotropism of EV71 virus. The presence of lesions of various degrees in central nervous system and changes in anterior motor neurons may be associated with limb paralysis.

Animals ; Brain ; ultrastructure ; virology ; Disease Models, Animal ; Enterovirus A, Human ; Enterovirus Infections ; pathology ; virology ; Jejunum ; ultrastructure ; virology ; Lung ; ultrastructure ; virology ; Mice ; Mice, Inbred BALB C ; Microscopy, Electron, Transmission ; Muscle, Skeletal ; ultrastructure ; virology ; Spinal Cord ; ultrastructure ; virology

OBJECTIVETo probe the changes of fast component of brainstem auditory evoked potentials (FC-BAEP), slow component of brainstem auditory evoked potentials (SC-BAEP) and the mitochondrial ultrastructures of the neurons in the brainstem in the rat pups with hyperbilirubinemia.

METHODS7 days old SD rat pups were randomly divided into control group (C, 17 rat pups) and two test groups (T1, 17 rat pups and T2, 17 rat pups). Bilirubin solutions (2 g/L) were injected into the abdominal cavity of the rat pups in the group T1 and T2 at the postnatal day 7 and 10. Six hours after the second injection, seven rat pups of each group were randomly selected to test serum bilirubin concentration via a micro-gauge. FC-BAEP and SC-BAEP were examined with an evoked potential recorder in the rest rat pups of each group at postnatal day 17 and 20. At the postnatal day 20, the endocardial perfusion was performed in these rat pups for the fixation of the brain, and then the brains were taken out. The cochlear nuclei were used for observation via electron microscope.

RESULTSSix hours after the injection of bilirubin solution at the postnatal day 10, the serum bilirubin concentrations of the rat pups in group T1 and T2 were increased significantly. Except for II-IV inter-peak latency(IPL), all the peak latency(PL) and IPL of FC-BAEP evoked via three sound stimulating rates (10/s, 40/s,80/s) at the postnatal day 17 prolonged significantly in the rat pups of group T1 and T2, and the PL in group T2 were much longer than that in group T1. Except for II-IV IPL of FC-BAEP evoked via sound stimulating rates of 10/s and 40/s, all the PL and IPL at the postnatal day 20 prolonged significantly in the rat pups of group T1 and T2. The PL of SC-BAEP evoked via sound stimulating rate of 10/s at the postnatal day 17 and 20 in the rat pups of group T1 and T2 prolonged significantly, and the PL at the postnatal day 17 in group T2 were much longer than that of group T1. The changes of mitochondria of the neurons in the cochlear nuclei at the postnatal day 20 in the rat pups of group T1 and T2 were characterized by swell, the slurred membranes, the broken crista and so on.

CONCLUSIONThere were the abnormal changes of FC-BAEP, SC-BAEP and the mitochondrial ultrastructures of the neurons in the brainstem in the rat pups with hyperbilirubinemia. The PL and IPL of FC-BAEP and SC-BAEP could be taken as the objective and sensitive indexes for early monitoring the bilirubin-induced hearing loss and brain injury.

Animals ; Animals, Newborn ; Brain Stem ; pathology ; Evoked Potentials, Auditory, Brain Stem ; physiology ; Hearing Loss ; etiology ; physiopathology ; Hyperbilirubinemia ; complications ; physiopathology ; Male ; Mitochondria ; ultrastructure ; Neurons ; ultrastructure ; Rats ; Rats, Sprague-Dawley

OBJECTIVETo explore the effects of taurine on the ultrastructure and P2X7 receptor protein expression in brain following traumatic brain injury (TBI) in rats.

METHODSForty male SD rats, were divided randomly into four groups that were sham-operated group, TBI group, TBI plus low-dose taurine group and TBI plus high-dose taurine group. The TBI model was established by Marmarou's method, the expression of P2X7 receptor protein in parietal cortex and hippocampus was detected by the immunohistochemical method, the ultrastructure of parietal cortex were observed by transmission electron microscope.

RESULTSCompared with sham-operated group, the positive expression cells of P2X7 receptor protein in parietal cortex and hippocampus of TBI group were significantly increased (P < 0.01). Compared with TBI group, the positive expression cells of P2X7 receptor protein in parietal cortex and hippocampus of TBI plus low-dose taurine group and TBI plus high-dose taurine group were significantly decreased (P <0.01 or P <0.05). Compared with TBI plus low-dose taurine group, the positive expression cells of P2X7 receptor protein in parietal cortex and hippocampus of TBI plus high-dose taurine group were significantly decreased (P < 0.05 or P < 0.01). The pathological damage of parietal cortex in the TBI plus high-dose taurine group was obviously lightened.

CONCLUSIONTaurine exerts the neuroprotective effect on TBI in rats, the protective mechanism might be associated with down-regulating the expression of P2X7 receptor protein in parietal cortex and hippocampus.

Animals ; Brain ; metabolism ; ultrastructure ; Brain Injuries ; metabolism ; pathology ; Male ; Rats ; Rats, Sprague-Dawley ; Receptors, Purinergic P2X7 ; metabolism ; Taurine ; pharmacology

OBJECTIVETo explore the culture conditions of human neural stem cells and to investigate the ultrastructure of neurospheres.

METHODSThe cells from the embryonic human cortices were mechanically dissociated. N2 medium was adapted to culture and expand the cells. The cells were identified by immunocytochemistry and EM was applied to examine the ultrastructure of neurospheres.

RESULTSThe neural stem cells from human embryonic brains were successfully cultured and formed typical neurospheres in suspension, and most of the cells expressed vimentin, which was a marker for neural progenitor cells, and the cells could differentiate into neurons, astrocytes and oligodendrocytes. In vitro myelin formation in neurospheres were observed at an early stage of culture.

CONCLUSIONSHuman neural stem cells can be cultured from embryonic brains, can form the typical neurospheres in suspension in vitro and have the ability of myelinating, and may be potential source for transplantation in treating myelin disorders.

Brain ; cytology ; Cells, Cultured ; Culture Media ; Female ; Humans ; Immunohistochemistry ; Male ; Microscopy, Electron ; Myelin Sheath ; pathology ; ultrastructure ; Neurons ; cytology ; pathology ; ultrastructure ; Sensitivity and Specificity ; Stem Cell Transplantation ; Stem Cells ; physiology ; ultrastructure

OBJECTIVETo observe the ultrastructure changes of cerebral cortex neuron and endothelial cell in hypoxia preconditioning mice and the effects of Tongxinluo (TXL, Chinese traditional medilihe) on them.

METHODSMice were randomly divided into 4 groups: control group, hypoxia group, hypoxia preconditioning (HP) group and Tongxinluo (TXL) group. The hypoxia preconditioning mice were exposed by repetitive hypoxia for 5 runs. The animal's tolerance time of each hypoxia run was recorded. The ultrastructure change of cerebral neuron and endothelial cell were studied by electron microscope.

RESULTSThe hypoxic tolerance time in HP and TXL groups were significantly increased run by run. Compared with HP group, the tolerance time of TXL group were increased in every run. The ultrastructure of cerebral neuron and endothelial cell in hypoxia group changed obviously, mitochondrion and endoplasmic reticulum destroyed. However they were slighter in HP group than those in hypoxia group. The change in TXL group had no obvious differentce with control group and were slighter than those in HP group.

CONCLUSIONHypoxia preconditioning shows that organism has a strong self-repairing ability. Tongxinluo self-repairing; could increase self-repairing ability and adaptive ability of mice to hypoxia obviously.

Adaptation, Physiological ; Animals ; Cerebral Cortex ; pathology ; Drugs, Chinese Herbal ; pharmacology ; Endothelial Cells ; ultrastructure ; Hypoxia ; physiopathology ; Hypoxia, Brain ; physiopathology ; Ischemic Preconditioning ; methods ; Male ; Mice ; Neurons ; drug effects ; ultrastructure

OBJECTIVETo preliminarily investigate the long-term structural and functional injuries of mitochondria in rat brain caused by sepsis.

METHODSWistar rats were randomly assigned into sepsis and control groups. A rat model of sepsis was prepared by an intraperitoneal injection of 10 mg/kg lipopolysaccharide (LPS) of gram-negative bacteria, and the survival assay was performed. Eight rats in the sepsis group were sacrificed at 12, 24, 48, or 72 hours after LPS injection, while rats in the control group were sacrificed after an intraperitoneal injection of an equal volume of normal saline. Mitochondria were extracted from rat brain tissue. Mitochondrial membrane potential (MMP) and mitochondrial swelling level were determined by flow cytometry, and the activities of electron transport chain complexes (I-V) were measured using enzyme assay kits. Hematoxylin-eosin (HE) staining and electron microscopy were used to observe morphological changes in brain tissue and mitochondria.

RESULTSThe sepsis group had a significantly lower survival rate than the control group (P<0.01). The MMP and activities of electron transport chain complexes (I-V) in the sepsis group, which were significantly lower than those in the control group (P<0.05), were reduced to the lowest levels at 48 hours and partially recovered at 72 hours. The mitochondrial swelling level in the sepsis group, which was significantly higher than that in the control group (P<0.05), increased to the peak level at 48 hours and partially recovered at 72 hours. Hematoxylin and Eosin staining revealed substantial damages in the structure of brain tissue, and electron microscopy showed mitochondrial swelling, and vacuolization in a few mitochondria.

CONCLUSIONSIn the rat model of LPS-induced sepsis, both structural and functional injuries are found in cerebral mitochondria, and achieve the peak levels probably at around 48 hours.

Animals ; Brain ; pathology ; physiopathology ; ultrastructure ; Lipopolysaccharides ; toxicity ; Male ; Membrane Potential, Mitochondrial ; Mitochondria ; physiology ; ultrastructure ; Rats ; Rats, Wistar ; Sepsis ; chemically induced ; mortality ; physiopathology