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Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a rare immune-mediated inflammatory disease of central nervous system reported in recent years, and its specific biological marker is anti-GFAP autoantibody. In this paper, the etiology, pathogenesis, clinical manifestations, auxiliary examination and treatment of the disease are comprehensively expounded, so as to improve the understanding of clinicians, especially neurologists.
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To report a typical case of Morvan syndrome with positive anti-leucine rich glioma-inactivated 1(LGI1) and contactin-associated protein 2 (CASPR2) antibodies in serum and cerebrospinal fluid. A 39-years-old female initially presented weakness of extremeties. The main symptoms included paroxysmal limb pain, wheezing, itching, muscle twitching, epilepsy, hypomnesia, dysphoria, apathy, intractable insomnia, salivation and sweating. Tests of electrolytes found hypokalemia (2.7-3.1 mmol/L) and hyponatremia (130-136 mmol/L). Arterial blood gas analysis showed hypoxemia (oxygen saturation 50%-70%). Total thyroxine (TT4) was elevated to 207 nmol/L with positive thyroid peroxidase antibody (TPO-Ab) and thyroglobulin antibody (TG-Ab). LGI1and CASPR2 antibodies (CBA method) were positive in both serum and cerebrospinal fluid, and the remaining antibodies related to autoimmune encephalitis and paraneoplastic syndrome were negative. Head MRI was almost normal, while mild abnormalities were found in electroencephalogram. Electromyography showed slightly increased voltage of left quadriceps motor unit potential. After treated with corticosteroids, IVIG and mycophenolate mofetil, the patient completely improved. Cognitive function scores recovered from MoCA/MMSE (16/24) to MoCA/MMSE (26/29). Positivity of LGI1/CASPR2 antibodies both in serum/cerebrospinal fluid are rarely seen in patients with Morvan syndrome. Steroids and immunosuppressants are suggested for treatment as early as possible.
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Objective:To establish a tissue based assay and in-house cell based assay combined system to screen anti-metabotropic glutamate receptor 1 antibodies in a case of previously idiopathic encephalitis with prominent cerebellar ataxia and make the final diagnosis, and to summarize and analyze clinical characteristics and treatment response of the disease.Methods:A middle-aged woman admitted to Department of Neurology, People's Liberation Army General Hospical Accredited to the Sixth Medical Center in January 9, 2020, who presented with acute dizziness, unsteady gait and developed head titubation, repeated language and calculation impairment was reported. The patient′s serum and cerebrospinal fluid were firstly tested with commercial kits for conventional neural antibodies.Then samples were incubated with rat hippocampus, cerebellum and human embryonic kidney 293 cells transfected with metabotropic glutamate receptor 1 plasmid to screen extra antibodies by indirect immunofluorescence method. By reviewing literature, physical functions of metabotropic glutamate receptor 1 and clinical features of anti-metabotropic glutamate receptor 1 antibodies associated encephalitis were summarized.Results:The patient was neural antibodies negative with commercial kits. Further investigation showed neuropil staining pattern after her serum and cerebral spinal fluid were incubated with rat brain slices. The characteristic "Medusa head" staining pattern of Purkinje cells in cerebellum was also noticed. Along with her previous head titubation symptom, an in-house cell based assay using human embryonic kidney 293 cells transfected with metabotropic glutamate receptor 1 plasmid was developed and proved the existence of anti-metabotropic glutamate receptor 1 antibodies. The final diagnosis of anti-metabotropic glutamate receptor 1 antibodies associated encephalitis was made. One-year follow-up revealed her serum antibodies titers dramatically decreased and cerebrospinal fluid antibodies were negative after using steroids and intravenous immunoglobulin, but still left prominent cerebellum atrophy and severe ataxia.Conclusions:Anti-metabotropic glutamate receptor 1 antibodies may cause acute encephalitis. Cerebellar ataxia and head titubation are characteristic symptoms of metabotropic glutamate receptor 1 autoimmunity. The response to immunotherapies is limited and patients may have severe neurological deficits.
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Primary angiitis of the central nervous system is relatively rare. In recent years, research of the disease in neuroimaging, differential diagnosis, treatment and other aspects advanced greatly. This paper provided a systematic review of this disease entity in order to promote neurologists, neurosurgeons and radiologists′ experience in the diagnosis and treatment of the disease.
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Objective To evaluate the injury of retinal microstructure using optical coherence tomography (OCT) and investigate the role of aquaporin 4 antibody (AQP4 Ab) in this injury process.Methods Forty patients with neuromyelitis optica spectrum disorders (NMOSD) were retrospectively studied,each of whom reported at least one episode of optic neuritis (ON),namely 59 ON eyes involved in all.All patients were divided into two subgroups based on AQP4 Ab tests including 25 patients (37 ON eyes) with AQP4 positive (Ab+/NMOSD group) and 15 patients (22 ON eyes) negative (Ab-/NMOSD group).In addition,10 healthy controls (20 eyes) matched for age and sex (HC group) were analyzed.Spectral domain optical coherence tomography (SD-OCT) was used to quantify peripapillary retinal nerve fiber layer (RNFL).Nonparametric test was used to compare differences between groups.Results Age distribution and gender ratio were comparable in three groups (P＞0.05).Visual acuity in ON eyes of Ab+/NMOSD group was worse than that of Ab-/NMOSD group (P=0.02).There were no significant differences between Ab+/NMOSD and Ab-/NMOSD in aspects of disease duration (2.6 vs.1.9 year),ON episodes (2 vs.1),longitudinal extensive transverse myelitis (LETM) ratio (48.0％ vs.66.7％),NMOSD specific intracranial lesions ratio (32.0％ vs.53.3％),positive autoimmune antibody ratio (52.0％ vs.20.0％) (P=0.13,0.08,0.25,0.18,0.06,respectively).The thickness of temporal,superior,nasal,inferior and average RNFL in ON eyes of both Ab+/ NMOSD and Ab-/NMOSD group were thinner than those in eyes of HC group (all P＜0.05).The thickness of superior and inferior RNFL in ON eyes of Ab+/NMOSD were 61.0 μm and 62.0 μm,which was thinner than those of Ab-/NMOSD 94.5 μm and 97.0 μm (P=0.03 and 0.01,respectively).Conclusions RNFL reflects the injury of retinal microstructure in NMOSD patients.AQP4 Ab seropositivity is correlated to the severity of RNFL damage,implying the potential role of AQP4 Ab in this pathological process.
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Objective To discover the significance of neurofilament light (NFL) chain of cerebrospinal fluid (CSF), an axonal injury biomarker, in diagnosis and prognosis prediction of neuromyelitis optica spectrum disorders(NMOSD).Methods Sixty-one NMOSD patients and 24 other patients such as neurosis, migraine and so on, with lumbar puncture were enrolled as NMOSD group and normal control (NC) group from in and out patients of Department of Neurology of Navy General Hospital from January 2014 to August 2016. The clinical and neuroimaging features of NMOSD group and CSF samples of both groups were collected,and the NFL levels of CSF were measured by enzyme linked immunosorbent assay. The CSF NFL levels in different subtypes of NMOSD patients were compared, and the influence factors of the NFL levels in CSF were calculated by multiple linear regression analysis. Results The NFL levels of CSF in NMOSD group (2 729.00(14 862.00) pg/ml) were significantly higher than that in NC group ((299.50(308.00) pg/ml, t=8.588, P=0.000;t test of NFL levels was performed after logarithmicly transforming based on 10). There were no statistically significant differences of CSF NFL levels among optic neuritis, longitudinally extensive transverse myelitis and neuromyelitis optica. In NMOSD group, age (b=0.017, P<0.01), Expanded Disability Status Scale score (b=0.078, P<0.05) and enhancement in gadolinium-magnetic resonance imaging (b=0.478, P<0.01) were correlated with the NFL levels of CSF, while gender, courses of diseases and aquaporin 4 antibody in serum were not related to the NFL levels. Conclusion The NFL levels of CSF are conducive to assess the severity and probable progress of NMOSD.
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Objective To explore the clinical characteristics of the neuromyelitis optica spectrum disorders (NMOSD) with the area postrema syndrome as the initial symptom.Methods A total of 14 cases were enrolled in the study with the diagnose of NMOSD and the area postrema syndrome as the initial symptom.All the clinical data and imaging profiles by the contrasted magnetic resonance imaging (MRI) of the head and spinal cord were collected and analyzed.Results The median age of onset was (38.1 ± 17.0)years old and the gender ratio of female to male was 10:4.The serum aquaporin-4 (AQP4)-IgG was positive in 11 subjects and several autoimmune antibodies was positive in 7 subjects.The lesions revealed by MRI of the head mainly located in the area postrema and ependymal periphery which often presented as the linear medullary lesion,while linear lesions over three pieces of vertebra were shown by MRI of the spinal cord which mainly in the grey matter and with aH shape around the spinal central canal.Misdiagnose happened in 11 subjects with seven of gastroesophageal reflux disease,two of neurogenic vomiting,one of spinal cord tuberculosis and one of stroke.Conclusions NMOSD should be considered in patients with unexplained intractable nausea,vomiting and/or hiccups lasted for 48 hours or above,especially in those with positive nervous signs.Contrasted MRI and serum AQP4-IgG need to be performed in the suspected patients.Early detection is crucial for patients with NMOSD.
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Objective To explore the prognostic factors for inability to walk independently in patients with multiple system atrophy (MSA).Methods A total of 123 patients with clinically confirmed MSA admitted to Navy General Hospital and Dongfang Hospital affiliated to the Second Clinical Medical College of Beijing University of Chinese Medicine , from February 2013 to February 2016, were retrospectively reviewed .Clinical data and all records were collected and all subjects were followed up by a telephone call in February 2016.The second milestone of activities of daily living scale (ADL), defined as inability to walk independently , was taken as the primary outcome .Eight possible prognostic factors were investigated and the survival analysis was performed with Cox proportional hazards model regression .Results Of all the MSA patients, 74 subjects were men and 49 were women with a sex radio of 1.51∶1(M∶F).Seventy cases were diagnosed with MSA-cerebellar type ( MSA-C ) and 53 with MSA-Parkinson type ( MSA-P ) (C∶P=1.32∶1).Mean age at the onset of first symptom was (53 ±8) years old.All patients had severe autonomic nervous dysfunction . At the last follow-up, 56 cases ( 45.5%) were unable to walk independently .The median survival time from the onset of MSA to inability to walk independently was 73 months.The age of onset ≥55 years (HR=1.969, 95%CI 1.095-3.542, P=0.024) and the interval time from disease onset to combined motor and autonomic involvement ≤3 years ( HR =2.308, 95%CI 1.158-4.600, P=0.017) were independent prognostic factors for inability to walk independently ,while gender, MSA clinical subtypes, initial symptoms, alcohol intake, smoking and toxic exposure were not indicators for independent walking (P>0.05).Conclusions The prognostic factors for inability to walk independently in patients with MSA are the age of onset ≥55 years and the interval time from disease onset to combined motor and autonomic involvement≤3 years.Although factors including gender , MSA clinical subtypes , initial symptoms , alcohol intake , smoking and toxic exposure are not the predictive factors for inability to walk independently in our MSA patients , their roles in the prognosis of MSA still need further investigation .
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To explore the clinical characteristics and imaging features of miliary tuberculomas in central nervous system (CNS).A total of 5 cases diagnosed with tuberculosis in CNS first diagnosed by neurologists in Navy General Hospital of PLA were enrolled in the study.All clinical and imaging data were collected and analyzed retrospectively.The main initial symptoms were fever and headache (4/5).Multiple diffused miliary lesions were shown by brain MRI,with maximum diameter ranged from 1-4 mm and ringshape or nodular enhancement after gadolinium injection.As mycobacterium tuberculosis could seldomly be found in serum and cerebrospinal fluid,contrast MRI remains the effective method for detecting miliary tuberculomas in CNS.
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Objective To explore the clinical characteristics of infratentorial primary angiitis in central nervous system (PACNS).Methods A total of 5 cases diagnosed as infratentorial PACNS in the neurology department of Navy General Hospital of PLA in 2015 were enrolled in the study.The clinical,imaging and pathological data were collected and analyzed.Results All the 5 cases were male with the median onset age of thirty-four.Five cases presented with dizziness,two with headache,three with walking unstable,two with facial numbness and one with dysarthria.Rising pressure of cerebrospinal fluid (CSF) (190-245 cmH2O,1 cmH2O =0.098 kPa) was found in 4 cases by the lumbar puncture,mildly increased number of leukocyte in 2 cases [(12-28) × 106/L],increased CSF protein in 3 cases(540-979 mg/L) and increased IgG index in 3 cases (0.84-1.45).Pons lesions were revealed by magnetic resonance imaging(MRI) in 4 cases,brachium pontis lesions in 2 cases,cerebellum lesions in 2 cases,one with midbrain lesion in 1 case,unilateral lesions in 4 cases and bilateral lesion in 1 case.Different degree of edema and mass effect were shown in all lesions by MRI.Patch like enhancement was found by contrast MRI in 5 cases and meningeal enhancement in 2 cases.Elevation of choline (Cho) peak was found by magnetic resonance spectroscopy (MRS) in 4 cases,reduction of N-acetyl aspartate (NAA) peak in 3 cases,appearance of lactate peak in 1 case and lipid peak in another case.Arterial spin labeling (ASL) was performed in 4 cases and no hyperperfusion was found.Susceptibility weighted imaging(SWI) was performed in 3 cases and microhemorrhage in the lesions was found in 2 cases and normal in 1 case.Magnetic resonance arteriography(MRA) was performed in 1 case and no stenosis was found.Digital subtraction arteriography(DSA) was performed in 1 case and multiple stenosis of the intracranial arteries was showed.Two cases had taken the stereotactic brain biopsy and the histopathologic diagnosis was angiitis.Five cases were treated with methylprednisolone and cyclophosphamide was added on in 1 case.Good prognosis was found in all cases.Conclusions Infratentorial PACNS mostly attacks middle-aged males.The lesions tend to locate in unilateral pons,brachium pontis,cerebellum and midbrain.Hemorrhage or microhemorrhage in lesions is often found by SWI and no hyperperfusion is shown by ASL,which would be useful to distinguish PACNS from malignant tumors.Given the limitations of brain biopsy in clinical practice,clinical and imaging features would be helpful to diagnose PACNS.
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Objective To improve differential diagnosis of tumefactive demyelinating lesions (TDL) and primary central nervous system lymphoma (PCNSL) by analyzing the clinicopathological features of the diseases.Methods The clinical features,neuroimaging findings and pathological characteristics of 4 patients with pathologically proven TDL and 9 patients with pathologically proven PCNSL were retrospectively analyzed.Computer tomography and magnetic resonance imaging were used for neuroimaging studies.The hematoxylin and eosin staining,Luxol Fast Blue staining and immunohistochemistry were used for pathological studies.Results (1) The features of lesions on brain imaging scan:CT in TDL patients showed low density.Enhanced MRI demonstrations were different in different courses:3 cases with ring enhancement,1 case with spotty strengthen;5 PCNSL cases showed hyperdensity in CT,1 case showed isodensity,and 3 cases low-density.MRI showed enhancement of uniform enhancement in PCNSL patients.(2) The features of lesions on pathology:the plaques of lesions in TDL patients were characterized by massive demyelination with relatively axonal preservation associated with prominent astrocytosis and profound infiltrates composed.Typical pathological features in PCNSL cases were that tumor cells around blood vessels showed the cuff-like arrangement.Due to use of hormones and other causes,pathological demonstrations of a part of PCNSL cases were atypical,which were easily confused with TDL.There were 4 cases with more than one biopsy for diagnosis.Conclusions (1) PCNSL with low or equal density in CT needs to be differentiated with TDL.(2) The pathological features of some cases of PCNSL after hormone therapy were similar to TDL.It is better not to use hormone before definite diagnosis.(3) The pathology of PCNSL may be related to the progression of the disease.Some of patients need to be re-biopsied.It is important to combine clinical imaging and pathology for diagnosis of the disease,and attention should be paid to followup.
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Objective To explore the clinical characteristics of vestibular migraine in patients from the clinic and ward of the neurological department in comprehensive hospitals.Methods A total of 226 patients diagnosed as vestibular migraine were enrolled in the study.Clinical data were collected and analyzed,including the medical history,clinical symptoms and signs,as well as the result of diagnostic examinations.Results The mean age of the patients at the visit was 51.7 years old,with the male to female ratio of 1:1.48.The occurrence of vertigo and migraine varied in order,with 53.1％ (120/226) patients presented migraine several years before vertigo.The duration time of vertigo ranged from seconds to days,with 1.8％ (4/226) patients presented no headache during the whole course.Several punctate long T2 or high FLARE (fluid attented inversion recovery) signals scattered at the centrum ovale.Conclusions Vestibular migraine has complex mechanism and presents multiple clinical manifestations with certain regularities of the onset.Differential diagnosis should be made from the similar diseases.
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Objective To accumulate clinical experience and to directe clinical work.Methods A total of 208 patients with psychogenic dizziness from department of neurology of Navy General Hospital of PLA were included in the study.Self-rating anxiety scale (SAS),self-rating depression scale (SDS) and Bech-Rafaelsen mania rating scale (BRMS) were used for the evaluation.Results Among all the patients aged from 17 to 77 (the average age:52),152 were female and 56 were male.There were 3 types according to different clinical features and therapy prognosis:anxiety and depression type (176 cases,84.6％),hysteria type(18 cases,8.7％) and mania type(14 cases,6.7％).The drugs increasing the concentration of excitatory had a good therapeutic efficacy on anxiety and depression type.Alluding cure had notable effect on hysteria type and mood stabilizer had notable effect on mania type.Conclusions Women tended to have psychogenic dizziness.It can be divided into three types:anxiety and depression type,hysteria type and mania type.Clinical symptoms and laboratory examination of each type have their own characteristics,and treatment strategies are also different.
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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.
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Objective To explore the clinical features,neuroimaging and histopathological findings in patients with idiopathic hypertrophic cranial pachymeningitis (IHCP) with a nodular space occupying effect.Methods Four IHCP cases with a nodular space occupying effect diagnosed in our hospital were retrospectively studied.Results All the 4 patients were men with a mean onset age of 40.25 (33 ～ 50) years old.They all had long disease duration and relapses.The common symptoms of IHCP were chronic headache,multiple cranial nerve palsies and epileptic seizures.CT and MRI of the brain revealed prominent dural partial thickening,which indicated a mass or nodular space occupying effect that mimicked intracranial tumour-like meningioma.The histopathological findings of dura in 2 cases revealed connective tissue proliferation,scattered neutrophile granulocytes and plasmacytes infiltration.Combination therapy of corticosteroid or/and immunosuppressive drugs was effective for the IHCP patients.Conclusions IHCP patients with a nodular space occupying effect usually onset with chronic headache and are often recurrent.The combination therapy of corticosteroid or/and immunosuppressive drugs is effective.The image of the brain presents prominent dural partial thickening,indicating a mass or nodular space occupying effect,which often lead to confusion with intracranial tumours or granulomatosis.
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Objective To analyse the clinical features,imaging characteristics diversity of deep cerebral veins thrombosis (DCVT).Methods From 2004 to 2013,6 patients diagnosed as DCVT were recorded and a retrospective review of the cases were undertaken for the purpose of this analysis.Results Among the 6 patients with DCVT,4 were male and 2 were female,aged from 28 to 69 years old.The disease duration of 4 cases ranged from 2 to 7 days,remnants were 20 days and 3 months respectively.The first symptoms of 4 cases were headache,1 was feeblemindedness,and the other was hemiplegia.The secondary symptoms were disturbance of consciousness,apathy,diplopia and non-infectious fever.Non-contrast computed tomography showed low signal in the bilateral thalamus in four patients,high signal in the transverse sinus and straight sinus in one patient and high signal in torcular in one patient.Abnormal signal was found in bilateral thalamus on magnetic resonance imaging in all patients and some of them had abnormal signal in the mesencephalon or basal ganglia.The patients were definitely diagnosed as DCVT by magnetic resonance venography (MRV) or digital subtraction angiography (DSA).Among them,2 patients were confirmed by brain biopsy.Four patients were followed up with good outcome and 2 were lost to follow-up.Conclusions The clinical manifestations of DCVT are not specific.For acute-onset DCVT patients,the first symptoms are always headache and vomiting,while the main symptoms are declined cognition and slow reaction for chronic-onset ones.Along with the progress,the main symptoms of DCVT are disturbance of consciousness,psychiatric symptoms and intracranial hypertension.Changes in the bilateral thalamus and basal ganglia are especially main characteristics which are easily misdiagnosed as brain tumor according to the images.DCVT can be definitely diagnosed by no signal of deep cerebral veins on MRV or DSA.
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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.
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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.
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Objective To analyze diagnostic evaluation of urethral sphincter electromyography (US-EMGs) for patients with multiple system atrophy (MSA).Methods Totally 15 patients who were diagnosed as MSA were examined as treatment group while 17 non-MSA patients were examined as controls.US-EMGs were performed in the both groups.Spontaneous activities when relax,parameters of motor unit potentials(MUPs) mean duration and amplitude,percentage of polyphasic ware,satellite potential,recruitment potentials and amplitude when strong contraction were recorded and analyzed.Results USEMGs changes of various abnormalities were found in 13 cases (86.7％) in MSA group.There were significant differences of electromyographic findings between the MSA group and control group including MUPs mean duration[(12.79 ±3.18)ms vs (9.49 ± 1.51)ms] and amplitude[(828.53 ±459.89) μV vs (378.76 ± 152.26) μV] as well as recruitment potentials [(11.47 ± 21.55) ％ vs (8.23 ± 10.74) ％] and amplitude [(2.19 ± 1.24) mV vs (0.75 ± 0.42) mV] when strong contraction (all P values ＜ 0.05).Conclusions There is certain value of US-EMGs for the diagnosis of MSA.It could be used as a routine electrophysiological method for the patients who are suspected of MSA.It could be a supplement of externalanal sphincter electromyography.
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Objective To compare the imaging characteristics of multiple sclerosis (MS) and neuromyelitis optica (NMO) for better diagnosis and differential diagnosis.Methods The brain and spinal MRI images of 60 MS and 48 NMO cases were retrospectively reviewed.The imaging characteristics including the predilection site,morphological features,enhancement manifestations were summarized.All data was analyzed by using t test and Chi square test with SPSS 13.0.Results (1) The three top predilection sites of brain in head MRI of MS patients were periventricular white matter (34 cases in 60),subcortical white matter (27 cases in 60),brain stem (23 cases in 60).MS lesions also were found in basal ganglia,cerebellum,corpus callosum and thalamus,as well as cortex (9 cases in 60).By contrast,brain lesions were observed in 59.4％ (19/32) of NMO patients,and the three top predilection sites of NMO by turns were brain stem (13 cases in 19),periventricular white matter (12 cases in 19),subcortical white matter (7 cases in 19).Furthermore,the lesions surrounding third ventricle (6 cases in 19) and the tegmentum of brain stem near peri-aqueduct (8 cases in 19) in NMO were not found in patients of MS.The involvement of brain stem and thalamus was more frequent in NMO than in MS (x2 =5.267,6.004,P ＜0.05,respectively).(2) The lesions of spinal cord in MS patients were typically oval,peripheral,and asymmetric,but in NMO patients they were longitudinally extensive and centrally located.The mean number of involved vertebral segments in NMO patients was significantly more than that in MS patients (7.3 vs 2.2,t =-9.288,P ＜ 0.01).Furthermore,the number of spinal cord lesions in MS patients was remarkably more than that in NMO (2.0 vs 1.3,t =4.565,P ＜0.01).The ratios of occurrence of spinal cord swelling and distension of NMO patients was 58.3％ (28/48),which was significantly higher than 21.9％ in MS (7/32,x2 =10.370,P ＜0.01).(3)The enhancement pattern in MS was circular (7 cases in 42),oval (6 cases in 42) and irregular (4 cases in 42),while in NMO was mainly sheet-shaped with mild enhancement (5/11).The lesions of spinal cord showed in MS mainly manifested as oval enhancement (16 cases in 26) and linear enhancement (8 cases in 26),while in NMO lesions manifested as strand or mild linear enhancement (26 cases in 35).Conclusions NMO has several distinct imaging characteristics,which are helpful for differentiation from MS.