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Objective To characterize the clinical,electrophysiology and neuropathological features of 4 cases with immune-mediated necrotizing myopathy (IMNM).Methods We retrospectively analyzed the clinical,electrophysiology,neuropathological characteristics of 4 IMNM patients with muscular and skin biopsy in our department during 4 years (from January 2011 to January 2014).Results Among these 4 patients,2 were men and 2 were women (aged 37 to 58 years) with disease duration ranging from 1 month to 60 months.Two patients were with acute onset and 2 with chronic onset.All 4 patients had proximal muscle weakness with three patients with cervical flexor muscle weakness and one with respiratory muscles weakness and noninvasive ventilator assisted respiration.One patient had interstitial lung disease.The anti-signal recognition particle antibodies were strong positive in all 4 patients.Muscle biopsy showed group necrotizing and regenerating fibers in one patient and few scattered necrotizing and regenerating fibers in the other 3 patients.Both muscle fiber hypertrophy and muscle fiber atrophy together with proliferation of connective tissue on endomysium could be viewed in all 4 patients.However,very few inflammatory cells were detectable in patients.One patient was treated with corticosteroids and the other three were treated with combination of corticosteroids and immunosuppressant drugs.Conclusions IMNM is characterized by heterogeneity at disease onset,severity and ilnvolvement of muscles with,however,similary pathological changes including the presence of numerous necrotic and regenerating fibers with little or none inflammation.Corticosteroid and/or immunosuppressant is effective for patients.
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Objective To summarize the clinical features,electrophysiology and neuropathological characteristics of peripheral nerves in patients with vasculitic neuropathy.Methods We retrospectively analyzed the clinical,electrophysiology and neuropathological characteristics of 15 patients with vasculitic neuropathy who underwent electrophysiology and sural nerve biopsy in our department from January 2009 to June 2013.Results There were 8 males and 7 females,aged from 38 to 82 years old,with a peripheral neuropathy course ranged from 0.5 month to 60 months.In the total of 15 patients,3 patients were diagnosed as nonsystemic vasculitic neuropathy,while the other 12 patients were diagnosed as systemic vasculitis neuropathy (SVN) including 5 cases of primary systemic vasculitis and 7 cases of secondary systemic vasculitis.In patients diagnosed as primary systemic vasculitis,there were 2 cases of Churg-Strass syndrome (CSS) and 3 cases of ANCA associated vasculitis.In patients diagnosed as secondary systemic vasculitis,there were 1 case of systemic lupus erythematosus (SLE),2 cases of sicca syndrome (SS),3 cases of rheumatoid arthritis (RA),1 case of Behcet' s disease associated with thyroid papillary carcinoma,1 case of hepatitis B and 1 case of RA-associated SS.For the pathological features of vasculitic neuropathy,type 1 lesion was found in 4 patients,type 2 lesion in 2 patients,and type 3 lesion in 9 patients.Axon degeneration was observed in 8 patients,while 7 patients manifested as axon degeneration associated with demyelination and local thickening of the perineurium was found in 2 patients.Conclusion Multiple mononeuropathy and asymmetric polyneuropathy are the common clinical presentations of vasculitic neuropathy.Electrodiagnostic testing almost always reveals the evidence of a predominantly axonal and sensorimotor process with associated demyelination presented in some cases.Sural nerve biopsy showes changes indicative of an axonopathy.
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Objective To explore the expression of nestin and Ki-67 in malignant peripheral nerve sheath tumors (MPNST) and its significance in the differential diagnosis. Methods Immunohistochemical technique (SP) was used to detect the expression of nestin and Ki-67 in 42 cases of MPNST and 24 cases of benign peripheral nerve tumor.Results Total expression of nestin was found in 95.2 ％(40/42) of MPNST.Strong expression of nestin was detected more frequent in MPNST compared to benign peripheral nerve tumors [40.5 ％(17/42) versus 4.2 ％(1/24),x2 =8.403,P =0.004].Ki-67 labeling index in MPNST varied from 1％-70 ％.However,greater than 3 ％ labeling index of Ki-67 staining was observed in 64.3 ％(27 / 42) of MPNST while none of the 24 benign tumors had nuclear staining exceeding 3 ％. The higher Ki-67 labeling index showed significant differences between the two groups (x2 =23.518,P =0.000).Conclusion Nestin and Ki-67 are useful markers in distinguishing MPNST from benign tumors.
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Objective To explore the feasibility of inherent depressive Fawn-Hooded (FH/Wjd)rats as a comorbidity model of mental disorder and irritable bowel syndrome (IBS) by comparing visceral sensitivity of FH/Wjd rats and Sprague-Dawley (SD) rats. Methods Depression trait of FH/Wjd rats was validated through forced swimming test (FST) and sucrose preference test (SPT).Visceral sensitivity to colorectal distention (CRD) under various pressures was assessed by abdominal withdrawal reflex (AWR). The expression of 5-HT in rats' colon,c-fos expression in colon and brain areas of infralimbic cortex (IL),prelimbic cortex (PrL) and rostral anterior cingulated cortex (rACC)was tested with immunohistochemistry. Results FST indicated that the immobility time of FH/Wjd rats was significantly longer than that in SD rats (t=-8. 931,P＜0. 01). SPT showed that the ratio of sucrose water in total liquid consumed was significantly lower in FH/Wjd rats than that of SD rats(t=4. 155,P=0. 01). At each CRD pressure gradient (20,40,60 and 80 mm Hg,1 mm Hg=0. 133kPa),AWR score was all significantly higher in FH/Wjd rats than that of SD rats (t=-2. 697,-3.464,-6.822 and -3. 976,P=0. 022,0.006,0.000 and 0.003). The expression level of 5-HT in FH/Wjd rats' control group was significantly higher than that of SD rats' control group (t=-11.371,P=0. 000). Compared with their own control group,5-HT expression increased both in FH/Wjd rats dilated groups and SD rats dilated groups,and the expression level in FH/Wjd rats was higher than that of SD rats (t= -3. 364,P= 0. 007). There was no significant difference of c-fos expression in colon and prefrontal brain areas between FH/Wjd rats' control group and SD rats'control group (colon:t=-0. 129,P= 0. 900;IL:t=-1. 316,P= 0. 218;PrL:t=1. 241,P = 0. 243;rACC:t=2. 151,P = 0. 057). Compared with their own control group,the expression of c-fos in colon and prefrontal brain areas significantly increased both in FH/Wjd rats dilated groups and SD rats dilated groups,and the expression level in FH/Wjd rats was higher than that of SD rats (colon: t=- 5.864,P= 0.000;IL:t=-2. 530,P = 0. 030;PrL:t=-7. 039,P= 0. 000;rACC:t=-6. 489,P=0. 000). Conclusions Inherent depressive FH/Wjd rats present visceral hypersensitivity. Meanwhile,the expression of 5-HT in colon is high,and with hyperreactivity to visceral noxious stimuli in colon and brain IL,PrL,and rACC areas. FH/Wjd rats might be a comorbidity animal model of mental disorder and IBS.
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Objective:To investigate the clinicopathologic features of intraspinal ganglioneuroma.Meth-ods:We collected 1 2 cases of diagnosed ganlioneuroma arising from the spine and one case of ganlioneuro-ma arising from mediastinum as the control.Clinical and radiographic features were reviewed.The pathologi-cal parameters of these cases were analyzed with routine and immunohistochemical stainings of neural fiber (NF),S-100 protein(S-100),myelin based protein(MBP),peripheral myelin protein 22(PMP22),smooth mus-cle actin(SMA),glial fibrilary acidic protein(GFAP)and Ki-67.Results:The disease was likely to occur in pa-tients aged 30-40 years old and more common in female.These cases were all intral cervical spinal tumors and presented with radicular neuralgia and mass effects of cervical spinal cord compression.Ganglioneuro-mas which occasionally contained normal spindle shaped cells were composed of mature or degenarative ganglion cells and neoplastic Schwannian stroma.Ganglion cells appeared positive for NF.Schwannian stro-ma as well as satellite cells around ganglion showed immunoreactivity for S-100.more intense than neurofi-bro-stroma.Mature spindle shaped cells showed immumoreactivity for MBP.Ki-67 labeling indices were usual-ly 0-1%while in Schwannian stroma areas were 3%.No blood vessel endothelium proliferation was ob-served.Conclusion:Intraspinal ganglioneuromas are rare benign tumors(WHO grand I),causing radicular neuralgia.It is jmportant to distinguish ganglioneuroma with spinal root encircled from Schwannoma or neuro-fibroma in the same anatomic location.The optimal treatment is surgical total resection.
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We reported a rare case of protoplasmic astrocytoma presenting small muscle atrophy of the right hand as an initial sign.A 39-year-old male was admitted to hospital complaining of chronic muscle atrophy and subtle headache.Electromyography(EMG) showed brief small denervation and no signs of sensory-motor conduction impairment.CT and MRI revealed multiply expansive intracranial lesion in left hemisphere,which was highly suspected of cerebral echinococccus or Balo disease.The patient underwent surgical excision and pathological report was protoplasmic astrocytoma,with glial fibrillary acidic protein(GFAP,+++) of immunohistochemical method.We reviewed clinical features,radiological manifestations and pathology of protoplasmic astrocytoma with medical literature documents.
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Objective: To observe the pathological changes of the lens and anterior lens capsule of the patients with familial congenital aniridia, and discuss the histopathological etiology of the fragility of the anterior capsule and the significance of surgical project. Methods: Anterior lens capsules and lens specimens were obtained from aniridic patients during cataract surgery. The intraoperative behavior of each capsule was noted, after which the specimens were submitted for histopathologic evaluation and electron microscope examination. Results: The anterior lens capsule was extremely fragile and remarkably thin. Degenerative changes(degeneration, necrosis, loss) of the lens epithelium and discontinuity of the lens epithelium were found in some specimens. Proliferation and double layer of the epithelial cells in some area of the specimens can be seen also. Ply structure of the anterior capsule became thin or disappeared. Conclusion: Degenerative or proliferative changes of the lens epithelial cells were associated with the thinness and extreme intraperative fragility of the anterior lens capsules in familial aniridia with cataract. Greater awareness of anterior capsule fragility in some aniridic patients with cataract may reduce the risk of capsule complications and lead to safer surgical outcomes.
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Objective: To study the axonal effect and the expression of integrin α6β4 during Schwann cell(SC) differentiation and myelination. Methods: Schwann cells were dissociated from the sciatic nerve of neonatal Waster rats and neurons dissociated from spinal cord. Singal cultures and purified populations of SC were cocultured with NC. Four methods (contrast microscope, scanning electron microscopy(SEM), immunocytochemistry method and in situ hybridization ) were used. Results: The separately cultured Schwann cells showed MBP negetive by immunocytochemistry method. But cocultured SC were shown positive. SEM showed that Schwann cells' membrane loop progressively circumnavigated around the axon during myelination, which suggested that the non-myelinating SC(nMSC) transformed to myelinating SC (MSC). In situ hybridization showed integrin α6β4 positive signals only on the outer surface of the Schwann cell-axon unit in SC coculture with NC. Conclusion: The differentiation and maturation of SC depend on axon, and the activity of integrins is expressed by axon. Axonal contact induces the expression of α6β4 during SC myelination, which suggests that integrin α6β4 is an important mediator of interactions of myelinating SC with the basal limina.