PURPOSE: The purpose of this study was to identify the effect of ghrelin on memory impairment in a rat model of vascular dementia induced by chronic cerebral hypoperfusion. METHODS: Randomized controlled groups and the posttest design were used. We established the representative animal model of vascular dementia caused by bilateral common carotid artery occlusion and administered 80 µg/kg ghrelin intraperitoneally for 4 weeks. First, behavioral studies were performed to evaluate spatial memory. Second, we used molecular biology techniques to determine whether ghrelin ameliorates the damage to the structure and function of the white matter and hippocampus, which are crucial to learning and memory. RESULTS: Ghrelin improved the spatial memory impairment in the Y-maze and Morris water maze test. In the white matter, demyelination and atrophy of the corpus callosum were significantly decreased in the ghrelin-treated group. In the hippocampus, ghrelin increased the length of hippocampal microvessels and reduced the microvessels pathology. Further, we confirmed angiogenesis enhancement through the fact that ghrelin treatment increased vascular endothelial growth factor (VEGF)-related protein levels, which are the most powerful mediators of angiogenesis in the hippocampus. CONCLUSION: We found that ghrelin affected the damaged myelin sheaths and microvessels by increasing angiogenesis, which then led to neuroprotection and improved memory function. We suggest that further studies continue to accumulate evidence of the effect of ghrelin. Further, we believe that the development of therapeutic interventions that increase ghrelin may contribute to memory improvement in patients with vascular dementia.
Animals ; Atrophy ; Carotid Artery, Common ; Corpus Callosum ; Dementia ; Dementia, Vascular ; Demyelinating Diseases ; Ghrelin ; Hippocampus ; Humans ; Learning ; Memory Disorders ; Memory ; Microvessels ; Models, Animal ; Molecular Biology ; Myelin Sheath ; Neuroprotection ; Pathology ; Rats ; Spatial Memory ; Vascular Endothelial Growth Factor A ; Water ; White Matter

OBJECTIVETo investigate the clinical features of mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) in children.

METHODSThe clinical data of 8 children with MERS were retrospectively analyzed.

RESULTSThe mean age of onset was 5 years and 2 months (range 10 months to 12 years). The major clinical features included a history of prodromal infection, and among these children, 5 had pyrexia and 4 had vomiting. Of all the children, 6 were manifested as convulsion and 3 each were manifested as disturbance of consciousness and paroxysmal paropsia. Cranial diffusion-weighted magnetic resonance imaging (MRI) showed high signals in the splenium of the corpus callosum. Among these children, one child had symmetric and multiple long T1 and long T2 signals in the bilateral centrum semiovale and part of the temporal white matter. MRI reexamination performed after 5-30 days showed the disappearance of abnormal signals in all the children. The children were followed up for 3 months to 2 years, and no child experienced abnormal neurodevelopment.

CONCLUSIONSThe development of MERS in children is closely associated with infection. MERS is characterized by high signals in the splenium of the corpus callosum on cranial diffusion-weighted MRI. Most children have good prognosis.

Brain Diseases ; pathology ; Child ; Child, Preschool ; Corpus Callosum ; pathology ; Encephalitis ; pathology ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; methods ; Male ; Retrospective Studies

OBJECTIVETo investigate the characteristics of a new clinical-image syndrome-mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) of corpus callosum.

METHODThe clinical and imaging features of one pediatric patient with the diagnosis of MERS were analyzed and the clinical and radiologic data of 44 MERS cases which were reported all around the world were also analyzed.

RESULTThe underlying disease of the patient before the onset was respiratory mycoplasma infection. On the second day of the disease course, the patient presented symptoms of encephalopathy. Brain MRI indicated lesions in the splenium of corpus callosum, centrum semiovate and posterior periventricular white matter. And these lesions recovered completely within 3 weeks. Most of the 44 patients diagnosed with MERS were associated with infectious diseases and completely recovered within two weeks. Symptoms included consciousness disturbance, convulsions and dysarthria. In addition to the splenium, brain MRI also showed lesions in genu of corpus callosum, centrum semiovate and white matter of frontal lobe.

CONCLUSIONThe clinical presentations of MERS were sudden onset of symptoms of encephalopathy during acute inflammation. Brain MRI indicated a reversible lesion in the splenium of corpus callosum. Patients recover completely within a few days.

Bacterial Infections ; complications ; Brain ; diagnostic imaging ; pathology ; Child ; Child, Preschool ; Corpus Callosum ; diagnostic imaging ; pathology ; Diffusion Magnetic Resonance Imaging ; Electroencephalography ; Encephalitis ; diagnostic imaging ; pathology ; Female ; Humans ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; Radiography ; Virus Diseases ; complications

Child ; Corpus Callosum ; pathology ; Encephalitis, Herpes Simplex ; pathology ; Humans ; Magnetic Resonance Imaging ; methods ; Male

BACKGROUND AND PURPOSE: Involvement of the corpus callosum (CC) is reported to be a consistent feature of amyotrophic lateral sclerosis (ALS). We examined the CC pathology using diffusion tensor tractography analysis to identify precisely which fiber bundles are involved in ALS. METHODS: Diffusion tensor imaging was performed in 14 sporadic ALS patients and 16 age-matched healthy controls. Whole brain tractography was performed using the multiple-region of interest (ROI) approach, and CC fiber bundles were extracted in two ways based on functional and structural relevance: (i) cortical ROI selection based on Brodmann areas (BAs), and (ii) the sulcal-gyral pattern of cortical gray matter using FreeSurfer software, respectively. RESULTS: The mean fractional anisotropy (FA) values of the CC fibers interconnecting the primary motor (BA4), supplementary motor (BA6), and dorsolateral prefrontal cortex (BA9/46) were significantly lower in ALS patients than in controls, whereas those of the primary sensory cortex (BA1, BA2, BA3), Broca's area (BA44/45), and the orbitofrontal cortex (BA11/47) did not differ significantly between the two groups. The FreeSurfer ROI approach revealed a very similar pattern of abnormalities. In addition, a significant correlation was found between the mean FA value of the CC fibers interconnecting the primary motor area and disease severity, as assessed using the revised Amyotrophic Lateral Sclerosis Functional Rating Scale, and the clinical extent of upper motor neuron signs. CONCLUSIONS: Our findings suggest that there is some degree of selectivity or a gradient in the CC pathology in ALS. The CC fibers interconnecting the primary motor and dorsolateral prefrontal cortices may be preferentially involved in ALS.
Amyotrophic Lateral Sclerosis* ; Anisotropy ; Brain ; Corpus Callosum* ; Diffusion Tensor Imaging ; Diffusion* ; Humans ; Motor Neuron Disease ; Motor Neurons ; Pathology ; Prefrontal Cortex

Agenesis of Corpus Callosum ; diagnostic imaging ; genetics ; pathology ; Brain ; diagnostic imaging ; pathology ; Chromosomes, Human, Pair 15 ; Humans ; Infant, Newborn ; Magnetic Resonance Angiography ; Male ; Radiography ; Translocation, Genetic ; genetics

OBJECTIVETo detect potential mutation of Doublecortin (DCX) gene in a patient featuring X-linked subcortical laminar heterotopia (X-SCLH) and epilepsy.

METHODSMutation of the DCX gene was screened by PCR and direct sequencing. Pathogenicity of the mutation was analyzed with a PolyPhen-2 software.

RESULTSA de novo missense mutation c.971T>C (p.Phe324Ser) was discovered.

CONCLUSIONA diagnostic method for X-SCLH has been established, which may facilitate diagnosis and genetic counseling of patients featuring this disease.

Agenesis of Corpus Callosum ; diagnosis ; genetics ; Base Sequence ; Brain ; pathology ; Child ; Classical Lissencephalies and Subcortical Band Heterotopias ; diagnosis ; genetics ; Electroencephalography ; Epilepsy ; diagnosis ; genetics ; Exons ; Female ; Humans ; Magnetic Resonance Imaging ; Microtubule-Associated Proteins ; genetics ; Mutation ; Neuropeptides ; genetics

OBJECTIVETo evaluate the changes in subcortical white matter and corpus callosum volumes in patients with type 2 diabetes mellitus (T2DM).

METHODSHigh resolution three-dimensional T1-weighted fast spoiled gradient recalled echo magnetic resonance images were obtained from 16 T2DM patients and 16 normal controls, and 11 T2DM patients also received the same magnetic resonance imaging scans after insulin therapy for 1 year. Volumetric analysis was performed and analysis of covariance and paired t test were applied.

RESULTSA lower volume was demonstrated in T2DM patients than that in the normal controls in bilateral caudal middle frontal lobe, bilateral pars opercularis, right inferior parietal lobe, right inferior temporal lobe, right middle temporal lobe, and left transverse temporal lobe (all P<0.05). After insulin therapy for 1 year, the increased subcortical white matter of brain regions were demonstrated in left medial orbitofrontal lobe, right caudal middle frontal lobe, right inferior parietal lobe, right lingual lobe, right inferior temporal lobe, right middle temporal lobe, left transverse temporal lobe, left entorhinal lobe and left insular lobe at the follow-up level in T2DM patients compared with baseline level (P<0.05). The decreased volumes of subcortical white matter were observed in the bilateral paracentral lobe, right superior forntal lobe and right precuneus lobe in T2DM patients on insulin therapy for 1 year (P<0.05). A decreased trend of subcortical white matter was involved in corpus callosum (P>0.05) and a significant decreased volume was demonstrated in the middle posterior part of corpus callosum after insulin therapy for 1 year(P<0.05).

CONCLUSIONST2DM patients have decreased subcortical white matter of multiple brain regions, and the atrophy of subcortical white matter can be improved by the insulin therapy. The volume analysis of subcortical white matter is a simple and effective tool for the evaluation of white matter damage in T2DM patients.

Aged ; Case-Control Studies ; Corpus Callosum ; pathology ; Diabetes Mellitus, Type 2 ; pathology ; Female ; Humans ; Image Processing, Computer-Assisted ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Plethysmography

OBJECTIVETo analyze the phenotype and genotype of CMTX1 patients with episodic transient reversible white matter involvement, and delineate the features of brain MRI in the episode and the possible mechanisms.

METHODThree Chinese probands and their family members were sequenced in the coding regions of GJB1. With the other 16 reported CMTX1 patients with episodic transient reversible white matter involvement, the clinical feature of the episodic central nervous system symptoms and the genotypes were reviewed.

RESULTMissense mutations in GJB1 were identified in all 3 probands. In 19 patients with transient reversible white matter involvement, the episodes were manifested as weakness of the limbs, dysarthria, and dysphagia, without disturbance of consciousness or seizures. The episodes lasted for 13 hours (10 min-72 hours) with complete remission in all patients; There were multiple episodes in 9 patients. During the episode, brain MRI showed symmetrical high signals in T2 weighted, Flair and DWI images in periventricular white matter, with predominance in posterior region including splenium of corpus callosum. These changes in imaging were most prominent during or within 1 week after the clinical episode.Significant improvements occurred within 1 month, with complete remission within 4-6 months.No specific locations of mutant amino acids in GJB1 protein were found in these patients with episodic transient reversible white matter involvement.

CONCLUSIONEpisodic transient reversible white matter involvement may present in a small number of patients with CMTX1. Transient edema of oligodendrocytes due to the dysfunction of gap junction may be involved in the pathogenesis. There is no correlation between the location of the mutant amino acids in GJB1 and the occurrence of the episodes.

Adolescent ; Brain ; diagnostic imaging ; pathology ; Brain Diseases ; diagnostic imaging ; etiology ; pathology ; Central Nervous System ; pathology ; Charcot-Marie-Tooth Disease ; complications ; genetics ; pathology ; Child ; Connexins ; genetics ; Corpus Callosum ; pathology ; Genetic Linkage ; Humans ; Magnetic Resonance Imaging ; Male ; Mutation, Missense ; Pedigree ; Phenotype ; Radiography

Aicardi syndrome is a rare neurodevelopmental disease characterised by congenital chorioretinal lacunae, corpus callosum dysgenesis, seizures, polymicrogyria, cerebral callosum, chorioretinopathy and electroencephalogram abnormality. We present a case of Aicardi syndrome with callosal hypogenesis in a 4.5-month-old baby who presented with infantile spasms. Ophthalmoscopy revealed chorioretinal lacunae. The clinical and magnetic resonance imaging features were diagnostic of Aicardi syndrome.
Agenesis of Corpus Callosum ; diagnosis ; Aicardi Syndrome ; diagnosis ; Brain ; diagnostic imaging ; pathology ; Choroid ; abnormalities ; Cornea ; physiopathology ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; methods ; Malformations of Cortical Development ; diagnosis ; Ophthalmoscopy ; methods ; Radiography ; Retina ; abnormalities ; Spasms, Infantile ; diagnosis