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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 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 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 The purpose of this study was to explore the risk factors of recurrence of atrial fibrillation (AF) after surgical radiofrequency ablation in patients undergoing cardiac valve surgery.Metbods Retrospective analysis was made in 255 cardiac valve patients undergoing concomitant radiofrequency modified Maze operation from April 2010 to December 2012 in Nanjing Drum Tower Hospital.Multivariate logistic regression analysis was used to screen independent risk factors for predicting recurrence of AF.Results Excluding 8 patients died after surgery,11 cases who were junctional rhythm after ablation or received pacemaker implantation and 60 lost case,176 patients were finished follow-up.107 cases maintained stable sinus rhythm (SR),whereas other 69 cases developed AF recurrence.Multivariate logistic regression analysis found larger left atrial size,higher B-type natriuretic peptide,lower preoperative heart rate and postoperative AF rhythm before discharge were independent risk factors for predicting AF recurrence.Conclusion Larger left atrial size,higher B-type natriuretic peptide and lower preoperative heart rate before surgery can impact outcome of surgical radiofrequency ablation in patients undergoing cardiac valve surgery after following 12(4-38)months.They can be used in clinical practice to choose more suitable cardiac valve patients with AF for surgical ablation.Postoperative appearance of AF rhythm before discharge indicated a poor prognosis,which should be interfered with drugs or electrical cardioversion.
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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 observe the electrophysiological changes, especially in the examination of external anal sphincter electromyography ( EAS-EMG), with those patients diagnosed as multiple system atrophy ( MSA), Parkinson's disease (PD) and late-onset spinocerebellar ataxia (LOSCA) and explore its clinical diagnostic value as well as differential diagnostic value for the three diseases. Methods The clinical data, cranial magnetic resonance imaging (MRI) data as well as results of EAS-EMG for 3 groups patients, including 88-cases MSA, 69-cases PD and 18-cases LOSCA, were analyzed retrospectively.Results EAS-EMG showed that 84 cases (95.5%) in MSA group had varying degrees of neurogenic injury. Meanwhile, mean motor unit potentials (MUPs) duration (( 12.92 ± 2.59)ms), mean MUPs amplitude ( ( 648.6 ± 251.0 ) μV ), and MUPs polyphasicity ( percentage of polyphasic MUPs; 42. 6% ±21.2% ) in MSA group were significantly different from those in PD ( ( 8. 99 ± 0. 47 ) ms, (470. 0 ±91.9) μV, 24.2% ±11.0%) and LOSCA groups ((9.04 ±0.62)ms, (493.1 ± 113.7)μV,22.0% ±12. 1%; Welch:94. 240,18. 093,26. 710,all P =0. 000). The spontaneous potentials and satellite potentials showed more common in MSA group, but not in other groups. Conclusions MSA and PD and LOSCA are easily mutually misdiagnosed because of some similar syndromes, but the method of EAS-EMG could be effective and helpful to enhance accurate diagnostic rate of MSA and its differential diagnosis with PD and LOSCA.
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Objective To explore the value of brain CT scanning for distinguishing tumor-like inflammatory demyelinating diseases (TIDD) from glioma or primary central nervous system lymphoma.Methods The brain CT features in 20 patients with TIDD(10 female,10 male;mean age (35.6±14.0)years;range,6-51 years)and 32 gliomas(16 female,16 male;mean age(42.0±19.8)years;range,12-75 years)and 6 lymphomas(3 female,3 male;mean age(53.8±11.8)years;range,32-68 years)were retrospectively reviewed and compared between brain tumors and TIDD.Results (1)Among the 38 primary brain tumors,there were 19 cases(50%,14 gliomas,5 lymphomas)with hyperdense lesions,10 cases(26.3%,9 gliomas,1 lymphomas)with isodense lesions,and 9 glionms (23.7%)with hypodense lesions.In contrast,the brain unenhanced CT manifestation of 20 TIDD all showed with hypodense lesions.(2)On unenhanced CT the lesions of 6 lymphomas all were hyperdense or isodense,like 90% of 20 high grade gliomas(WHO grade Ⅲ and Ⅳ),but this rate for grade Ⅱ was only 41.7%.(3)According to the shape of hyperdense lesions of the 19 primary brain tumors with,7 cages(6 gliomas,1 lymphomas)manifested with asymmetric hyperdense small-patches,4 cases(1 gliomas,3 lymphomas)with symmetric hyperdense large-patches,4 cases(3 gliomas,1 lymphomas)with diffused hyperdensed lesions,and 4 cases(4 gliomas)with ring-shaped hyperdensed lesions.Furthermore,4 primary brain tumors(4 lymphomas)underwent CT enhanced scanning and all the cases showed strong enhancement(3 cases with hyperdense lesions and 1 with isodense lesions on unenhanced CT),but only 3 cases of 7 TIDD showed mild enhancement in contrast.(4)By Spearman's relevant analysis,hyperdense and isodense on unenhanced CT was proved to have significant positive correlation between the grade of gliomas(r=0.435,P=0.013).Therefore,the frequency of hyperdense and isodense lesions in lymphomas and WHO grade Ⅲ and Ⅳ astrocytoma was higher in contrast with low grade astrocytoma.Conclusions Brain CT as a simple,economical and practical examination method has significant meaning for differentiating TIDD from glioma or PCNSL and could be used as an adjuvant method for MRI and magnetic resonance spectroscopy.Patients with hyperdense or isodense on unenhanced CT or strong enhancement could be excluded from TIDD.
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Objective To summarize the clinical features, neuroimaging findings and pathological characteristics of 26 patients with tumor-like inflammatory demyelinating diseases (TIDD) confirmed by histopathology for better diagnosis and differential diagnosis. Methods The clinical features, neuroimaging findings and pathological characteristics of 26 patients (14 male, 12 female) with pathologically proven TIDD(24 brain-type and 2 spinal cord-type ) were retrospectively analysed. Results The mean onset age was 6-69 (36.7±13.8) years. Twenty-one patients had good prognosis with a median followed-up duration of 51.0 months. Two patients were died of post-operative complication and pulmonary infection respectively and the remaining 3 patients were lost to followed up. The TIDD patients almost showed monophasic clinical setting. Headache, indifference accompanied with hypomnesis were the commonest initial symptoms. The positive or abnormol rates of cerebrospinal fluid oligoclonal bands (OCB) and myelin basic protein (MBP)in TIDD patients were high. The involvements of bilateral and multi-lesions were commonest in TIDD (61.5%, 65.4% respectively). Twenty-two patients with CT unenhanced scanning showed hypodense lesions. Long T1 and long T2 signal intensity was showed on MRI and most cases apeared round-like lesion in shape. Acccording to the shape of enhancement of the 23 patients performed with contrast agents, 11 were shown with open-ring enhancement, 4 cases (including 2 accompanied with open-ring enhancement) with complete ring enhancement, 3 with asymmetrical dotted enhancement, 2 with diffused even enhancement,and no enhancement was seen in the other 6. Furthermore, 14 cases with DWI and 12 with FLAIR all appeared hyperdensity. The typical pathological changes were demyelinating, perivascular inflammatory cells infiltration and reactive gliosis. Occationally, the Creutzfeuldt cells were also found in brain tissue of some patients. Conclusions TIDD is a distinct demyelinating disease entity. In spite of being apt to be confused with the neoplasm in brain and spinal cord. TIDD has its own-features, for example, OCB is frequently positive in patients with TIDD and the level of MBP may be significantly increased. Furthermore, the involvements of bilateral and multi-lesions are the common in TIDD, and most cases showed open-ring enhancement or complete rim enhancement on MRI. In addition, all cases present hypodense lesions on unenhanced CT and patients with hyperdense seemed not to be considered as TIDD.
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Objective To study the clinical and neuroimaging features of gliomatosis cerebri(GC)so as to enhance the accuracy of diagnosis. Methods The clinical manifestations and cranial MRI characteristics of 10 cases of GC confirmed by neuropathology of brain biopsy were analyzed. Results Main clinical features of the 10 patients were not so severe and varied with headache, dizziness, dementia,aphasia, epilepsy, etc. Cranial MRI scan showed diffuse long T2 signals in frontal lobes, temporal lobes,parietal lobes, occipital lobes bilaterally, as well as corpus collosum, thalamus, paraventricular white matter, caudate nucleus and putamen. Brain stem and cerebellum were also involved. The borders of the lesions were unclear and no obvious mass effect was found. Contrast enhancement was positive only in 3 patients after Gd-DTPA injection. The lesions were always bilateral without cystic formation or hemorrhage.DWI signals of 8 cases were in accordance with pathological grades. Cho/Cr and Cho/NAA ratio increased in all 7 cases with MRS imaging. Cho/Cr in tumor area versus normal area was 1.28 ±0. 15 vs 0. 92 ±0. 17(t=4.201,P=0.0012,95% CI:0.17-0.57).The value of Cho/NAA was 3.21 ± 1.19 vs 0.61 ±0.18(t = 5.716,P=0.0001,95 % CI:1.61-3.59).Biopsy pathological examination showed diffuse infiltrative growth of astroglioma cells of 8 cases with low-grade while 2 cases with high-grade. Conclusions Mismatch between relatively slight clinical manifestations and heavy diffuse imaging features are important in the diagnosis of GC, with the aid of brain biopsy. MRS would contribute to diagnosis and DWI signal may be correlated with GC grade and help judging prognosis.
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Objective To investigate the clinical manifestions ,neuropathology and imaging in the patients with MELAS type of mitochondrial encephalomyopathy for exploring the diagnostic method of the disease. Methods Systemic study was performed on the clinical features,imaging of four MELAS patients. Muscle biopsy and 2 brain biopsies of 3 cases were examined. Results The main clinical features were characterized by intolerance to exercise,recurrent headache and vomit,focal or generalized seizures,dementia,stroke like episodes,sensorineural deafness, hypertrophic cardiomyopathy,endocrine dysfunction,short stature,lactic acidosis and so on. Electromyography showed myopathic damage. CT showed calcification in basal ganglia. CT showed multiple low density lesion primarily in gray matter of occipital,parietal and temporal cortex,which was expressed by the abnormal longer T 1 and T 2 weighted signals on MRI.Muscle biopsy showed red ragged fiber and abnormal mitochondria. Brain biopsy showed laminar necrosis of cortex,astrocytosis,diffused microvascular proliferation and calcification. Four cases were diagnosed as MELAS type.Conclusion According to clinical manifestations and neuroimage features,MELAS is possibly early defined in combination with muscle or/and brain biopsy.