The clinical treatment of joint contracture due to immobilization remains difficult. The pathological changes of muscle tissue caused by immobilization-induced joint contracture include disuse skeletal muscle atrophy and skeletal muscle tissue fibrosis. The proteolytic pathways involved in disuse muscle atrophy include the ubiquitin-proteasome-dependent pathway, caspase system pathway, matrix metalloproteinase pathway, Ca-dependent pathway and autophagy-lysosomal pathway. The important biological processes involved in skeletal muscle fibrosis include intermuscular connective tissue thickening caused by transforming growth factor-β1 and an anaerobic environment within the skeletal muscle leading to the induction of hypoxia-inducible factor-1α. This article reviews the progress made in understanding the pathological processes involved in immobilization-induced muscle contracture and the currently available treatments. Understanding the mechanisms involved in immobilization-induced contracture of muscle tissue should facilitate the development of more effective treatment measures for the different mechanisms in the future.
Atrophy ; Autophagy ; Calcium ; metabolism ; Caspases ; metabolism ; Connective Tissue ; metabolism ; pathology ; Contracture ; etiology ; metabolism ; pathology ; therapy ; Fibrosis ; Humans ; Immobilization ; adverse effects ; Joints ; Lysosomes ; metabolism ; Matrix Metalloproteinases ; metabolism ; Muscle, Skeletal ; metabolism ; pathology ; Proteasome Endopeptidase Complex ; metabolism ; Proteolysis ; Signal Transduction ; physiology ; Transforming Growth Factor beta1 ; metabolism ; Ubiquitin ; metabolism

In the present study we explored the different patterns of volumetric atrophy in hippocampal subregions of patients with left and right mesial temporal lobe epilepsy with hippocampal sclerosis (TLE-HS). Meanwhile, the memory impairment patterns in Chinese-speaking TLE-HS patients and potential influencing factors were also determined. TLE-HS patients (21 left and 17 right) and 21 healthy controls were recruited to complete T2-weighted imaging and verbal/nonverbal memory assessment. The results showed that both left and right TLE-HS patients had overall reduced hippocampal subregion volumes on the sclerotic side, and cornu ammonis sectors (CA1) exhibited maximum atrophy. The verbal memory of left TLE-HS patients was significantly impaired (P < 0.001) and was not associated with the volumes of the left hippocampal subregions. Verbal or nonverbal memory impairment was not found in the patients with right TLE-HS. These results suggested that the atrophy of hippocampal subregion volumes cannot account for the verbal memory impairment, which might be related to the functional network.
Adult ; Asian Continental Ancestry Group ; Atrophy ; pathology ; Epilepsy, Temporal Lobe ; complications ; pathology ; Female ; Functional Laterality ; Hippocampus ; pathology ; Humans ; Male ; Memory Disorders ; etiology ; pathology ; Sclerosis ; pathology ; Young Adult

Spinal and bulbar muscular atrophy (SBMA) is a rare X-linked motor neuron disease with significant phenotypic viability. Here, we present a genetically identified SBMA family without bulbar paralysis or androgen insensitivity. All four male patients presented with progressive lower motor neuron paralysis in all limbs, with distal extremities more dominant. None of them had bulbar palsy or androgen insensitivity. A consistently mild elevated blood creatine phosphokinase (CPK) levels were detected in all patients and the EMG showed a chronic neurogenic damage. Muscle biopsy of propositus indicated a typical neurogenic amyotrophy. Genetic testing for SMA of mutation in SMN1 was negative, while for SBMA of androgen receptor showed the increased CAG repeat in exon 1, suggesting that although bulbar symptoms and androgen insensitivity are characteristic symptoms of SBMA, they are not obligatory for the diagnosis. In adult males with a chronic motor neuron syndrome without upper motor neuron signs, even in absence of the classical features of androgen insensitivity or bulbar findings, genetic testing for SBMA should be strongly considered.
Adult ; Bulbo-Spinal Atrophy, X-Linked ; complications ; diagnosis ; genetics ; Creatine Kinase ; blood ; Genetic Testing ; Humans ; Male ; Motor Neurons ; pathology ; Muscular Atrophy ; etiology ; Mutation ; genetics ; Paralysis ; diagnosis ; etiology ; Pedigree ; Receptors, Androgen ; genetics

We compared the predictive ability of the various neuroimaging tools and determined the most cost-effective, non-invasive Alzheimer's disease (AD) prediction model in mild cognitive impairment (MCI) individuals. Thirty-two MCI subjects were evaluated at baseline with [18F]-fluorodeoxyglucose positron emission tomography (FDG-PET), MRI, diffusion tensor imaging (DTI), and neuropsychological tests, and then followed up for 2 yr. After a follow up period, 12 MCI subjects converted to AD (MCIc) and 20 did not (MCInc). Of the voxel-based statistical comparisons of baseline neuroimaging data, the MCIc showed reduced cerebral glucose metabolism (CMgl) in the temporo-parietal, posterior cingulate, precuneus, and frontal regions, and gray matter (GM) density in multiple cortical areas including the frontal, temporal and parietal regions compared to the MCInc, whereas regional fractional anisotropy derived from DTI were not significantly different between the two groups. The MCIc also had lower Mini-Mental State Examination (MMSE) score than the MCInc. Through a series of model selection steps, the MMSE combined with CMgl model was selected as a final model (classification accuracy 93.8%). In conclusion, the combination of MMSE with regional CMgl measurement based on FDG-PET is probably the most efficient, non-invasive method to predict AD in MCI individuals after a two-year follow-up period.
Aged ; Alzheimer Disease/complications/*diagnosis ; Atrophy/pathology ; Biomarkers/blood ; Brain/*pathology ; Diffusion Tensor Imaging/methods ; Female ; Glucose/*metabolism ; Gray Matter/*pathology ; Humans ; Male ; Mild Cognitive Impairment/*diagnosis/etiology ; Neuroimaging/methods ; Positron-Emission Tomography/methods ; Reproducibility of Results ; Sensitivity and Specificity ; Severity of Illness Index ; White Matter/*pathology

OBJECTIVETo investigate the clinicopathologic features of adult-onset autoimmune enteropathy (AIE).

METHODSA case of adult-onset AIE was described along with a literature review.

RESULTSA 41-year-old male patient was admitted for intractable diarrhea for more than three months despite of any dietary restriction or anti-inflammatory therapy. Fat globule was observed by stool examination and Sudan III staining of the stool was positive. Enteroclysis showed weak movement and few plica of small intestine, while colonoscopy appeared normal. Small bowel biopsies revealed villus atrophy and increased crypt apoptotic bodies and lymphocytic infiltration in deep crypt. Although without significant surface intro-epithelial lymphocytosis, there were a large number of monocytes, lymphocytes, plasmacytes and neutrophilic granulocytes infiltrating in the lamina propria. Morphologically, the colonic mucous was similar to the small intestine although cryptitis and crypt abscess were significant in the former. Serum IgG anti-goblet cell antibody was demonstrated by indirect immunofluorescence. Other causes of diarrhea were excluded on the base of medical history, histopathology and other accessory examinations before the diagnosis of AIE was made. The patient had a complete remission after steroid treatment without recurrence for eight months during the follow-up even after steroid withdrawal for five months.

CONCLUSIONSAIE is exceedingly rare and timely diagnosis is important for successful therapy. Histological differential diagnoses should include ulcerative colitis, celiac disease, lymphocytic colitis, etc. The final diagnosis should be based on histological examination combined with the patient history, clinical manifestation, endoscopy finding and serological testing.

Atrophy ; Biopsy ; Celiac Disease ; pathology ; Colon ; pathology ; Colonoscopy ; Diagnosis, Differential ; Diarrhea ; etiology ; Humans ; Intestinal Mucosa ; pathology ; Intestine, Small ; pathology ; Lymphocytes ; Lymphocytosis ; pathology ; Polyendocrinopathies, Autoimmune ; pathology

PURPOSE: To evaluate optic disc pallor using ImageJ in traumatic optic neuropathy (TON). METHODS: This study examined unilateral TON patients. The optic disc was divided into 4 quadrants (temporal, superior, nasal, and inferior), consistent with the quadrants on optical coherence tomography (OCT) retinal nerve fiber layer (RNFL) thickness maps. Optic disc photography was performed and disc pallor was quantified using gray scale photographic images imported into ImageJ software. The correlation between optic disc pallor and RNFL thickness was examined in each quadrant. RESULTS: A total of 35 patients (31 male, 4 female) were enrolled in the study. The mean participant age was 34.8 +/- 15.0 years (range, 5 to 63 years). Overall RNFL thickness decreased in 6 patients, with thinning most often occurring in the inferior quadrant (28 of 35 eyes). There was a significant correlation between optic disc pallor and RNFL thickness (superior, rho = -0.358, p = 0.04; inferior, rho = -0.345, p = 0.04; nasal, rho = -0.417, p = 0.01; temporal, rho = -0.390, p = 0.02). The highest level of correspondence between disc pallor and RNFL thickness values outside of the normative 95th percentiles was 39.3% and occurred in the inferior quadrant. CONCLUSIONS: Optic disc pallor in TON was quantified with ImageJ and was significantly correlated with RNFL thickness abnormalities. Thus, ImageJ evaluations of disc pallor may be useful for evaluating RNFL thinning, as verified by OCT RNFL analyses.
Adolescent ; Adult ; Child ; Child, Preschool ; Colorimetry/methods/standards ; Diagnosis, Computer-Assisted/*methods/standards ; Female ; Humans ; Male ; Middle Aged ; Optic Atrophy/etiology/*pathology ; Optic Nerve Diseases/etiology/*pathology ; Optic Nerve Injuries/*pathology ; Photography/*methods/standards ; Reproducibility of Results ; Software ; Tomography, Optical Coherence/*methods/standards ; Trauma Severity Indices ; Young Adult

OBJECTIVESTo compare the paravertebral muscle (such as multifidus, erector spinae, psoas muscle) changes between the patients with degenerative lumbar instability and normal person by MRI and to observe the degeneration of paravertebral muscles. To analyze the relationship between paravertebral muscle degeneration and lumbar curvature of degenerative lumbar instability.

METHODSSixty patients with degenerative lumbar instability were retrospectively enrolled from December 2011 to July 2013 as degeneration group, meanwhile 60 health persons with no degenerative lumbar instability were selected as control group. No significant differences were found in the gender, age and body mass index between the two groups. The cross-sectional area(CSA) and percentage of fat infiltration area (FIA) of the paravertebral muscles at the L4-S1 levels were measured using T2-weighted axial MRI and Image J soft ware. And the lumbar curvature(expressed as lumbar lordosis angle) of all the patients in lumbar X-ray were measured in the two groups. The measured data were analyzed with independent samples t-test.

RESULTSThe difference of multifidus cross-sectional area and the percentage of fat infiltration in the patients of degenerative lumbar instability at the L4-L5, L5-S1 level, compared with the control group, was statistically significant (t = 2.768, t = 6.216, P < 0.05). Between the two groups, the percentage of fatty infiltration in erector spinae showed significant differences (t = 5.862, P < 0.05). The cross-sectional area of erector spinae and the degeneration of the psoas muscle between the two groups was not statistically significant. The lumbar lordsis angle in the patients with degenerative lumbar instability was (43.9 ± 15.6)°, which was higher than the (39.3 ± 14.2)° in control group (t = 2.915, P < 0.05).

CONCLUSIONSCompared with the control group, patients with degenerative lumbar instability exists erector spinae and multifidus muscle degeneration, and erector spinae is more obvious. The degeneration among psoas muscle, erector spinae and multifidus muscle are inconsistent, which may be related to the increasing of the lumbar lordosis angle in the patients with degenerative lumbar instability.

Aged ; Case-Control Studies ; Female ; Humans ; Joint Instability ; diagnosis ; etiology ; pathology ; Lumbosacral Region ; physiopathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Muscle, Skeletal ; pathology ; Muscular Atrophy ; complications ; diagnosis ; pathology

OBJECTIVETo identify the affect of chronic low back pain on multifidus muscle atrophy and fatty infiltration.

METHODSFrom March 2010 to August 2013, a retrospective study were carried out in the department of orthopedics of patients with low back pain. Finally 31 cases were selected to this study including 19 males and 12 females with an average age of 36.4 years ranging from 23 to 55 years. The main symptoms of these patients were repeated back pain. Duration was more than 1 year. X-ray, CT, MRI showed no obvious abnormalities. The changes of net cross-sectional area of multifidus and T2 signal ratio of the same patient were measured at different time by MRI. VAS and Oswestry disability scores were recorded in two MRI examination. Correlation between these change of multifidus net area and T2 signal ratio in two times measurement and duration of low back pain, VAS, Oswestry disability scores were analyzed to find the affection of low back pain on paraspinal multifidus muscle.

RESULTSThe net multifidus cross-sectional area in same case by the second follow-up MRI is significantly smaller than that of the first follow-up, T2 signal ratio at second was significantly higher than that of the first (P < 0.05). The net cross sectional area of multifidus muscles reduced rate were positively correlated with VAS scores, duration and of Oswestry disabilitry scores (P < 0.001). The rate of increase in T2 signal ratio was not correlated with VAS scores,duration and the Oswestry disability scores (P > 0.05).

CONCLUSIONChronic low back pain is one of the most important reasons of paraspinal multifidus muscle atrophy and fatty. The duration, VAS and Oswestry disability scores of chronic low back pain were positively correlated with the multifidus muscle atrophy.

Adult ; Chronic Disease ; Female ; Humans ; Low Back Pain ; complications ; Male ; Middle Aged ; Muscular Atrophy ; diagnostic imaging ; etiology ; Paraspinal Muscles ; diagnostic imaging ; Radiography ; Retrospective Studies ; Young Adult

We report on a 55-year-old man with alcoholic liver cirrhosis who presented with status epilepticus. Laboratory analysis showed markedly elevated blood ammonia. Brain magnetic resonance imaging (MRI) showed widespread cortical signal changes with restricted diffusion, involving both temporo-fronto-parietal cortex, while the perirolandic regions and occipital cortex were uniquely spared. A follow-up brain MRI demonstrated diffuse cortical atrophy with increased signals on T1-weighted images in both the basal ganglia and temporal lobe cortex, representing cortical laminar necrosis. We suggest that the brain lesions, in our case, represent a consequence of toxic effect of ammonia.
Ammonia/blood ; Atrophy/pathology ; Brain Diseases/blood/*diagnosis/*etiology ; Hepatic Encephalopathy/*complications ; Humans ; Liver Cirrhosis, Alcoholic/*complications ; Magnetic Resonance Imaging/*methods ; Male ; Middle Aged ; Necrosis/pathology ; Status Epilepticus/pathology

OBJECTIVETo explore the effect of human umbilical cord blood mononuclear cells (UCBMC) promoting nerve behavior function and brain tissue recovery of neonatal SD rat with hypoxic ischemic brain injury (HIBI).

METHODA modified newborn rat model that had a combined hypoxic and ischemic brain injury as described by Rice-Vannucci was used, early nervous reflex, the Morris water maze and walking track analysis were used to evaluate nervous behavioral function, and brain MRI, HE staining to evaluate brain damage recovery.

RESULTNewborn rat Rice-Vannucci model showed significant brain atrophy, obvious hemiplegia of contralateral limbs,e.g right step length [(7.67 ± 0.46) cm vs. (8.22 ± 0.50) cm, F = 1.494] and toe distance [(0.93 ± 0.06) cm vs. (1.12 ± 0.55) cm, F = 0.186] were significantly reduced compared with left side, learning and memory ability was significantly impaired compared with normal control group (P < 0.01); Cliff aversion [(8.44 ± 2.38) s vs.(14.22 ± 5.07) s, t = 4.618] and negative geotaxis reflex time [(7.26 ± 2.00) s vs. (11.76 ± 3.73) s, t = 4.755] on postnatal 14 days of HIBI+ transplantation group were significantly reduced compared with HIBI+NaCl group (P < 0.01) ; the Morris water maze experiment showed escape latency [ (23.11 ± 6.64) s vs. (34.04 ± 12.95) s, t = 3.356] and swimming distance [ (9.12 ± 1.21) cm vs.(12.70 ± 1.53) cm, t = 17.095] of HIBI+transplantation group were significantly reduced compared with those of HIBI+NaCl group (P < 0.01) ; the residual brain volume on postnatal 10 d [ (75.37 ± 4.53)% vs. (67.17 ± 4.08)%, t = -6.017] and 67 d [ (69.05 ± 3.58)% vs.(60.83 ± 3.69)%, t = -7.148]of HIBI+ transplantation group were significantly larger than those of HIBI+NaCl group (P < 0.01); After human UCBMC transplantation, left cortical edema significantly reduced and nerve cell necrosis of HIBI+ transplantation group is not obvious compared with HIBI+NaCl group.

CONCLUSIONHuman UCBMC intraperitoneal transplantation significantly promoted recovery of injured brain cells and neurobehavioral function development.

Animals ; Animals, Newborn ; Atrophy ; etiology ; pathology ; Brain ; diagnostic imaging ; pathology ; Cerebral Cortex ; pathology ; Cord Blood Stem Cell Transplantation ; methods ; Disease Models, Animal ; Female ; Fetal Blood ; cytology ; Humans ; Hypoxia-Ischemia, Brain ; complications ; pathology ; therapy ; Learning Disorders ; etiology ; prevention & control ; Leukocytes, Mononuclear ; cytology ; transplantation ; Magnetic Resonance Imaging ; Male ; Maze Learning ; Neurons ; pathology ; Psychomotor Performance ; Radiography ; Rats ; Rats, Sprague-Dawley ; Transplantation, Heterologous