Objective To evaluate the teased fibers and the consecutive internodes or segments of the same myeliated nerve fiber. Methods Measurements were performed on 1100 fibers (5522 internodal segment) from 11 control nerves as to detecting the anatomic features and pathologic abnormalities. Results Morphometric study on normal teased fibers revealed three results: (1) There is a certain variation of diameters (Ds) and length (IL) of the internodal segments along a fiber (1.0—2.5 ?m), and as a rule, the longest segment is not the thickest one, and the shortest segment is not the thinnest. Or even, sometimes the thinnest segment is the longest one in all internodal segments. (2) The relationship between IL and Ds is not linear, but shows a parabolic curve; thick segments are relatively shorter than the thinner segment. (3) The IL of segment along 10—20 mm of a fiber may be varied by maximally 50%; on the different IL, there is no casual distribution of internodal segments, but 2—4 (or even 6) segments of about equal length are grouped in a row. Conclusions On the classification of normal teased fibers, myelin of the internode may be ragular, irregular and variable in thickness among internodes.

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Objectives To investigate the normal value of myelinated fibre (MF) of healthy human sural nerve. Methods By morphometric analysis on biopsy of 41 normal subjects’sural nerve, the density of MF, and the distribution and proportion of short and long diameters of MF were measured. Results The number of MF for each body had arange from 5 057 to 10 104, and the density of MF showed 5 250～11 413 mm 2. The distribution on diameter size of MF was found double peaked.Conclusions Following the age increase, the density of MF in Chinese people is substantially decreased. As compared with the study of some western countries, the results show that the density value of MF in our study is significant higher than those from the western countrics ( P =0 002 4).

Objective To verify a uncommon neurodegenerative disease accompanying with dementia--frontal lobe dementia, or dementia of frontal lobe type (DFT). Methods A brain sample was obtained from a patient of 46-year-old male with progressive dementia. Conventional neurohistopathological examination and immunostaining for prion protein (PrP) and tau protein were performed, and clinical data were analysed. Results (1) It was shown having progressive neurological and psychical symptoms and a three month illness duration. (2) Atrophy was found in bilateral frontal gray matter in CT scan. Slow waves of high amplitude with long intermission of two second in whole course of electroencephalography examination were seen. (3) Brain weight was 1 050 g. The cerebral cortex was atrophied and restricted to frontal lobes. The temporal lobes were unaffected. (4) A severe loss of nerve cells from second frontal cortical layer with glioses was revealed, but pyramidal cells in this region remained intact. There were no positive findings on staining of Beilschowky and Gallyas methods. (5) No inclusions were seen in remaining nerve cells and gliocyte. (6) Immunohistochemistry revealed no significant changes on PrP and tau protein.Conclusion This is more typical a case of DFT , and now increasingly recognized. It suggests that a dementia of frontal lobe type should be considered when differential diagnosis of neurodegenerative disease with dementia have been made.

Objective To investigate clinical manifestation and pathological changes and the expressions of Emerin protein and STA gene of Emery-Dreifuss muscular dystrophy (EDMD).Methods The clinical features and STA gene detection from one patient with EDMD were analyzed retrospectively. Results The onset age of this patient was in early childhood. The four limbs were progressive muscle weakness and muscular atrophy. There were joint contractures and cardiac involvement in the early stage. The serum muscle enzymes increased slightly. The pathological changes in muscles showed that the sizes of muscle fibers were different, the fibers became spherical and some fibers were replaced by fat. Because of normal spinal anterior horn cells and sural nerves, neurogenic muscular atrophy might be ruled out. Emerin protein could not be tested in striated muscle and cardiac muscle. No mutation of STA gene was found in this case. Conclusions Emery-Dreifuss muscular dystrophy is one particular type of muscular dystrophy. It is characterized with joint contractures and cardiac involvement in the early stage. Emerin protein is deficient in EDMD. There is no mutation of STA gene in sporadic EDMD.

Objective 　To investigate the neuropathological changes of central nervous system in Guillain-Barré syndrom. Methods　Brain, spinal cord and sciatic nerve were obtained from 22 cases of Guillain-Barré syndrome. Eight cases were examined by general autopsy, 14 cases were examined by limited autopsy. HE, KB, Bielschowsky, Weil and Sudan Ⅲ　staining were carried out, the sections were observed by light microscopy. Results　1.Cerebral superficial veins congested, widening of the cortical sulci, narrowed gyri and mild cerebellar tonsillar hernia were present. 2. Majority of cerbral neurons presented an ischemic changes. Slightly loss of hippocampal pyramidal neurons were found. There was chromatolysis of motor neurons of brain stem. Lymphocytic infiltration around the small vessels occurred in the pons and medullary oblongata in 8 cases. Focal demyelination was noted in pons and frontal white matter in 2 cases. Loss of Purkinje cells and appearance of glial nodules were observed in molecular layer of cerebellum. 3. Swellin, central chromatolysis and eccentric nuclei of anterior horn cells appeared in 16 cases, which were pronounced in cervical and lumbal segment of spinal cord. Vaculated neuroplasma and lymphacytic infiltrition could be seen. 4. Segmental demyelination and lymphocytic infiltration were the main neuropathological changes observed in 20 cases. There were two other cases in which the axon were severely involved, which showed swelling and breakdown of axons and as well as axonal bulbs. Conclusions　1. Lymphocytic infiltration in brain stem and spinal cord were in continuousness of pathological changes of peripheral nerves. 2. Finding of glial nodules suggested that there was possibility of infection of neurotropic virus. 3. Occurence of focal demyelination in cerebrum and brain stem indicated that Guillain-Barré syndrome may have combined involvement of central and peripheral nervous system.

BACKGROUND: Pathological changes of the brain tissue in patients with dementia of frontal type(DFT) are still controversial. This paper brought forward the pathological alterative characteristics of brain tissue in DFT patients through one pathological case study of the brain tissue in one dead dementia patient.OBJECTIVE: To validate one uncommon neurodegenerative disease complicated with dementia, DFT.DESIGN: A case analysis.SETTING: Department of Neurology of the First Hospital of Jilin University METHODS: Brain anatomy, serials of histological staining and immunohistochemical staining for PrP, tau protein, etc. were performed after 3 hours since the death of one patient with progressive dementia.stainingfrontal lobes. EEG displayed a paroxysmal high-amplitude slow wave with and the brain atrophy was limited to frontal lobe and the temporal lobe loss of neurocyte companied with significant gliosis since the second layer; However, the pyramidal cell was relatively healthy. No abnormality was munohistochemical staining had negative reactions.CONCLUSION: This case was typical DFT. This type of dementia should be considered in future analysis of the neurodegenerative disease complicated with dementia.

Objective:To analyze the magnetic resonance imaging (MRI) features of parasellar cavernous haemangioma and improve the diagnosis and differential diagnosis accuracy.Methods:13 patients with parasellar cavernous haemangioma were collected.All the patients were diagnosed by MRI and confirmed by pathology.Based on the pathologic findings,the MRI features were discussed.Results:9 cases presented horizontal dumbbell.The lesions located in the parasellar were larger than the sella turcica.The main body position of the lesions were centered lateral to the parasellar and encasesed the intracavernous internal carotid artery (ICICA).While,1 cases were similar in size and shape.1 case was located in the sella turcica.2 cases was centered lateral to the ICICA.pituitary were detected obscurity in 7 cases and displaced in 6 cases,6 cases appeared extremely high homogeneous intensity on T2-weighted images:as bright as cerebrospinal fluid signal.Only 5 cases underwent three-dimensional arterial spin labelling perfusion imaging (3D-ASL).The lesions revealed marked hypoperfusion.The cases of misdiagnosis were 9,including 4 cpituitary adenomas and 5 meningiomas.Conclusions:The characteristics of MR images ofparasellar cavernous haemangioma were horizontal bottle gourd form,the main body position of the lesions were centered lateral to the parasellar and encasesed the (ICICA),and their extremely high homogeneous intensity on T2-weighted images:as bright as cerebrospinal fluid signal.In cases that are equivocal,3D-ASL were useful in differentiating cavernous haemangiomas from parasellar meningiomas,which could decrease mistaken diagnosis.Graspping the imaging feature and differential diagnosis were helpful for the diagnosis of this disease.

Objective To study the MRI findings of sporadic Creutzfeldt-Jakob disease and its clinical relations. Methods MRI of 10 cases CJD patients were examined 2-12 months after the onset. 6 cases were diagnosed using cerebral biopsy, 8 cases received CSF analysis for 14-3-3 protein, 8 cases showed special changes of electroencephalogram, PrP gene of 9 cases were analyzed. Results Symmetric bilateral high signals were observed in caudate nucleus and Putamen in T 2-weighted imaging and Flair imaging in 5 cases, but the pallidum and thalamus were normal. No changes were found in T1-weighted imaging. 2 cases showed brain atrophy, 1 case showed mild lacunar infarction,and the other 2 were normal. Conclusions Abnormal signals in basal ganglia of 4 patients of 129Met/Met homozygote occurred after 2.5 months averagely, they survived for 10.5 months at average. 1 patient of 129 Met/Val heterozygote showed abnormal signals in basal ganglia after 12 months, and survived for 16 months. The mean duration of patient with abnormal signals in basal ganglia (12.2 months) is longer than those without changes in basal ganglia (5.5 months). Symmetric high signal in bilateral caudate nucleus and Putamen is an important imaging feature of sCJD. It might be served as a diagnostic index in some circumstances.