Objective To evaluate the clinical,neuroimaging and electrophysiology features of 62 patients with multiple system atrophy(MSA).Methods Sixty-two cases with diagnosis of probable MSA were recruited in a retrospective studied.Clinical,neuroimage and external anal sphincter electromyography (EAS-EMG)data was retrospectively analyzed.Results In 62 cases(44 male and 18 female),the onset age was between 37 and 76.Among them,29 cases(46.8 % )were MSA-A,with orthostatie hypotension as the main clinical manifestation;24 cases(38.7 % )were MSA-C,with cerebellar ataxia ag the main chnical manifestation;9 cases(14.5 % )were MSA-P,with extrapyramidal symptoms as the main clinical manifestation.MRI showed that main lesion of MSA-A was in the cerebellum:that of MSA-C was in the cerebellum,pons and medulla;and that of MSA-P was in the putamen.Fifty-one cases did EAS-EMG and 46 cases showed neurogenie impairments.Nineteen cases were initially misdiagnosed with other diseases.Conclusions MSA is easy to be omitted or misdiagnosed at early stage.The diagnostie rate of MSA can be increased by the combination of clinical expressions,neuroimage,EAS-EMG and other necessary examinations.

Objective To investigate the clinical, electrophysiological and pathological features of critical illness polyneuropathy and myopathy (CIPNM). Methods The clinical outcomes, electromyogram Results as well as pathological features in nerves and muscles of 3 patients with CIPNM were investigated and analyzed. Results 3 patients were all provided with assisted respiration after tracheal intubation. 7～10 d after intubation, all the patients emerged muscle strength and tendon reflexes of extremities weakening; while 14 days after that, 2 patients appeared amyotrophy of extremities. Electromyogram showed that the conduction of many motor and sensory nerves for extremities decreased, while the amplitude of compound muscle action potential (CMAP) of part of motor nerves decreased. Biopsy for nerves revealed decreased medullated nerve fibers and regeneration phenomenon of auxiliary fibers; while that for muscles showed neuralgic damage and myopathy-like changes. Conclusion CIPNM can complicate after tracheal intubation. The electrophysiological and pathological examinations for nerves and muscles can be helpful for the diagnosis.

Objective To analyze the clinical,imaging and pathological features of cortical vein thrombosis (CoVT). Methods Ten patients with CoVT (4 patients with CoVT alone and 6 patients with CoVT and venous sinus thrombosis)treated at Navy General Hospital from January 2006 to Jun 2013 were collected retrospectively.Its etiology,imaging,and pathological features of brain tissue in 3 patients were analyzed. Results Of the 10 patients with CoVT,3 were female and 7 were male.Their mean age was 31 ± 15 years old.(1)Brain CT scan and MRI showed hemorrhagic cerebral infarction,and contrast-enhanced MRI showed scattered heterogeneous enhancement within lesions. DSA could find CoVT at different parts.(2)3 patients underwent brain biopsy because they were initially diagnosed as brain tumor. Pathological examination showed glial cell,endothelial proliferation and phagocytic reaction.Scattered spotty bleeding was observed,and significant expansion of small veins,A few of them had blood stasis and thrombosis. Conclusion CoVT is one kind of intracranial venous thrombosis. It is more common occurred in young and middle aged adults,and most of them were venous sinus thrombosis.It is caused by retrograde thrombosis and spread to cortical veins.CoVT is easily to be misdiagnosed as brain tumor.Combination of clinical and imaging findings is needed for accurate diagnosis.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biopsy, Fine-Needle ; adverse effects ; methods ; Child ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Hematoma ; etiology ; Humans ; Male ; Middle Aged ; Thyroid Neoplasms ; diagnosis ; pathology ; Thyroid Nodule ; pathology ; Young Adult

Metastatic epidural compression of the spinal cord is a significant source of morbidity in patients with systemic cancer. With improvment of oncotheray, survival period in the patients is improving and metastatic cord compression is en- countered increasingly often. Surgical management performed for early circumferential decompression for the spinal cord com- pression with spine instability, and spine reconstruction performed. Patients with radiosensitive tumours without spine instabili- ty, radiotherapy is an effective therapy. Spinal stereotactic radiosurgery and minimally invasive techniques, such as vertebro- plasty and kyphoplasty, percutaneous pedicle screw fixation, radiofrequency ablation are promising options for treatment of cer- tain selected patients with spinal metastases.
Decompression, Surgical ; Humans ; Minimally Invasive Surgical Procedures ; Spinal Cord Compression ; therapy ; Spinal Neoplasms ; secondary ; therapy

Objective To improve the diagnostic ability of leukoencephalopathy with cerebral calcifications and cysts (LCC),a rare central nervous system disease.Methods The clinical manifestations,neuroimages and neuropathological features of a 19-year-old male patient were analyzed.A total of 20 cases from 14 literatures were reviewed.Results The patient was admitted with right limb weakness,cognitive decline,headache and blurred eyesight.Head CT scan showed multiple calcifications,cysts formation and leukoencephalopathy.Brain MRI showed several cysts in bilateral hemisphere,basal ganglia,thalamus and paraventricular areas.A mural nodule was noted inside one of the cyst,which was enhanced on the contrasted MRI.The wall of the cysts was partially enhanced,but not with the fluid inside the cysts.The corresponding CT calcifications foci showed on T1 and T2 with either both hyperintensity or both hypointensity,which was also partial enhanced.Extensive leukoencephalopathy was formed around the cysts and the ventricles.But neither Cho nor NAA changed a lot on MRS.Amplitude diagram of SWI series exhibited multiple round small dark signals all over the affected areas with mixed signals showed in the phase diagram,which indicated both calcifications and microbleedings at the lesions.Neuropathological examinations found no tumor cells in the operated cyst,and showed angiomatous small blood cells were dominant in the cyst wall.Hyaline degenerations,microcalcifications and hemosiderin deposition were observed.No obvious demyelination was discovered,while gliosis,numerous Rosenthal fibers and fibrinoid vascular necrosis were found around the lesions.The clinical,neuroimaging and pathological features of this patient were in accordance with the cases reported in the literatures.Conclusions Neuroimaging is the most important method for the diagnosis of LCC.As small vessel lesions are probably closely related to the pathophysiology of LCC,SWI could be recommended to further reveal the etiology of LCC.

Objective To report the clinical and pathological characteristics of one patient with glycogen storage disease Ⅳ (Anderson disease). Methods The patient was received detailed clinical examinations, ultrasound, electromyography, head MRI and muscle biopsy. Results The onset of the 22 years old male patient was 7yrs. The main symptoms were intolerance and fatigue in proximal limbs muscular movement, cardiopalmus by chance. Abdominal ultrasound examinations showed cirrhosis, portal hypertension, splenomegaly. Echocardiogram showed left ventricular myohypertrophia, mild mitral and tricuspid valve insufficiency. Electrophysiology study revealed widespread myogenic changes. Cranial MRI, MRA and MRS were normal. Muscle biopsy showed basophilic intracytoplasmic material in a lot of fibers deposits, which was intensively PAS-positive material and partially resistant to diastase digestion. In the electron microscope, the storage material consisted of filamentous and finely granular material. Conclusions There was the first case of glycogen storage disease Ⅳ reported in our country, mainly involved skeletal muscle, liver, spleen and cardiac muscle.

Objective To investigate the pathological changes and pathogenesis of the MELAS syndrome (mitochondrial encephalopathy lactic acidosis strok-like episodes ) by using the method of immunohistochemical staining in the brain biopsy specimens with anti-mitochondrial antibody (AMA).Methods We performed immunohistochemical staining in 3 confirmed MELAS patients' paraffin-imbed brain biopsy specimens.Results Small vessel proliferation and the uneven thickness of the wall were found in the 3 MELAS patients.A lot of brown deposits was shown in the wall of small vessels and also noted in neurons.Conclusions The main pathological change in the MELAS brain biopsy immunohistochemical staining with AMA was the small vessel proliferation,indicating that abnormal mitochondria accumulated in the vascular smooth muscle,endothelial cell and neurons of the lesion sites.This finding was consistent with the electron microscopic discovery and valuable for the diagnosis of MELAS.

Objective To investigate the special expression and diagnostic value of anti-mitochondrial antibody (AMA) in the muscle tissues of patients with mitochondria encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Methods All cases including 10 cases of MELAS, which were diagnosed definitely by clinical, pathological and DNA analysis, and 2 cases of normal control and 3 cases of necrotic muscle fibers, underwent muscle biopsies in order to carry out AMA staining. At the same time the original MGT staining was observed. Results A large number of ragged brown fibers (RBF) were observed under light microscopy with AMA immunohistochemical staining in the muscle tissues of patients with MELAS. But no RBF were observed in control groups. Conclusion RBF might be the specific performance of paraplastic mitochondrial, which was similar to the ragged red fibers, so RBF might be a pathological index in evaluating abnormal mitochondrial function and in diagnosing mitochondriopathy.

Objective To compare the clinical characteristics of multiple sclerosis ( MS) and neuromyelitis optica (NMO) for better diagnosis and differential diagnosis of them. Methods The characteristics of 40 MS and 38 NMO cases were retrospectively studied on clinic manifestations, electroneurophysiology,some laboratory indices, imaging characteristics and so on. Results The ratios of male to female were 1: 1. 35 and 1:4. 43 respectively in patients with MS and NMO, so patients with NMO were more likely to be female as compared with MS ( P < 0. 05 ). The mean onset age was ( 35. 5 ±13. 9 ) years in MS patients and (30. 6 ± 15. 6) years in NMO patients, but no significant difference was found (P>0. 05). The cases of visual acuity ≤0.1 in patients NMO was 13, which of MS was merely 1. The cases of visual acuity less than 0. 5 after treatment in NMO patients was 19, which in MS was only 1. The cases of cognitive impairment in NMO was 3, which of MS was 10. The cases of cerebrospinal fluid oligoclonal bands in MS was 16, which in NMO patients was 9. The lesions of spinal cord shown in MRI of MS patients were typically oval, peripheral and asymmetric, but those in NMO patients extended longitudinally and converged centrally. The mean number of involved vertebral segments in NMO patients was significantly greater than that in MS patients ( 6. 6 vs 2. 2, P < 0. 01). Furthermore, the number of spinal cord lesions in MS patients was alse remarkably greater than that in NMO patients (2. 0vs1.2, P <0. 01). Conclusions NMO may be a distinct clinical entity, which is likely to be differentiated from MS by its tendency to affect women, younger age at onset, and other features clinical manifestations, electroneurophysiology, laboratory parameters, neuroimaging show..