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.

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

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 analyze the clinical,imaging and pathological features of cortical vein thrombosis (CoVT).Methods Three cases of cortical vein thrombosis were diagnosed in our hospital from February 2010 to October 2012.We reviewed and summarized their clinical manifestation,radiological feature and pathological characteristics.Results All patients were young with acute onset.The main clinical manifestations included headache,epilepsy or limbs weakness.Two cases had intracranial hypertension.One case had decreased activity of protein S.One had increased plasma homocysteine.Brain computed tomography scan showed hypodensity lesion with some hyperdensity inside.Cranial routine magnetic resonance imaging showed long signal in T1-weighed image and T2-weighed image,with occasional short T1 signal inside.Contrast-enhanced magnetic resonance imaging revealed heterogeneous enhancement.All of 3 cases underwent brain biopsy because of the suspected diagnosis of brain tumor.Brain pathology showed the local necrosis and hemorrhage,dilated small vein with congestion or thrombosis.Neuronal degeneration,hyperplasia of gliocyte,hyperplasia of endotheliocyte in small blood vessels with reaction of histiocytes was also displayed.Duration from initial visit to final diagnosis was from 14 days to 2 months.Conclusions CoVT has various clinical and radiological manifestations and it is easy to misdiagnose as brain tumor.Careful analysis of clinical and imaging data could improve its diagnostic accuracy.Brain biopsy would also be helpful for diagnosis.

OBJECTIVETo evaluate a surgical technique and outcome and illustrate its important of open reduction and internal fixation for fractures of posterior malleolus.

METHODSFrom Jan. 2004 to Jan. 2008, 25 cases were treated with open reduction and internal fixation involving 17 males and 8 females with an average age of 34 years (ranged from 21 to 67 years old). According to Lauge-Hansen classification: 13 cases of type pronation-external rotation (IV), 7 and 5 cases of type supination-external rotation (III and IV). Twenty cases were treated with screw, 5 cases were treated with gypsum. To evaluate the outcome of operation with X-ray.

RESULTSAfter seven months to three years followed-up, clinic therapeutic effect of each patient was evaluated according to the Leeds scoring system, the results were excellent in 21 cases and good in 2. As compared with the uninjured side on the evaluation of the activity range of ankle joint, there were no significant difference in back-extend and plantar flexion.

CONCLUSIONIn order to obtain ankle joint satisfactory result, open reduction and rigid internal fixation must be adopted in the fracture of posterior malleolus.

Adult ; Aged ; Ankle Injuries ; surgery ; Female ; Fracture Fixation, Internal ; Fracture Healing ; physiology ; Fractures, Bone ; surgery ; Humans ; Male ; Middle Aged ; Range of Motion, Articular ; Rotation ; Treatment Outcome

Computed tomography (CT) is considered the most sensitive method for the detection of intraocular foreign bodies (IOFBs).The purpose of this study was to evaluate a new method of 3-dimentional (3D) localization of IOFBs that takes advantage of the anatomical structure of the optic nerve and to assess the clinical outcomes using this new method.Twenty-two trauma patients with IOFBs or suspected IOFBs admitted to our hospital were scanned with multislice CT (MSCT) between July and December 2003.All scanning was performed with a 16-row spiral CT in axial plane using a sequential scanning protocol.During the scanning,the eyeball of the patient was kept stable and was not allowed to rotate internally or externally.Section collimation was set at 16 mm × 0.75 mm.Table feed was 12 mm.Reconstruction index was 0.75 mm.After scanning,the reconstructed images were loaded into a workstation to create the multiplanar reconstruction images with the aid of the 3D software.We compared the localization results with the operative findings.Multiplanar reconstruction images showed IOFBs in all 22 patients.IOFBs occurred in the eyeball of 14 patients,in the wall of the eyeball of 5 patients and in the posterior orbits of 3 patients.Different surgical procedures were designed according to the localization by this new method and all IOFBs were successfully removed.All of these foreign bodies were metallic and the localization of IOFB using MSCT was consistent with that found by operative findings.It was suggested that MSCT is a simple and effective imaging modality for the localization of IOFBs.In our study,we localized the IOFBs more quickly and accurately by taking advantage of the fixed position of the intraocular segment of the optic nerve,and determined the necessary surgical parameters.

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.

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 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.