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Post-transplant lymphoproliferative disorder (PTLD) is a solid organ or hematopoietic stem cells transplant associated syndrome, and central nervous system PTLD(CNS-PTLD) is extremely rare. A case of CNS-PTLD occurring after 24 years of kidney transplant was reported, and pathological examination proved it to be diffuse large B cell lymphoma. Cerebrospinal fluid next generation sequencing and pathological examination supported that Epstein-Barr virus infection was associated with it.
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Ocular opsoclonus is a rare disorder of the saccadic system, in which fixation is continuously interrupted by involuntary, chaotic, rapid and multi-directional saccadic eye movements, interrupting the gaze-holding function, leading to“dancing eyes”. Ocular opsoclonus has rarely been reported in China. A case of ocular opsoclonus associated with anti-N-methyl-D-aspartate receptor encephalitis is reported to improve awareness about the ocular sign. The patient′s condition reached the peak at the 5th week. He developed ocular opsoclonus on the basis of nonconvulsive status epilepticus, accompanied by frequent involuntary movements of the mouth and limbs. The ocular opsoclonus gradually relieved on the 7th week with the active treatment.
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Objective To analyze the clinical features and prognosis in adult Chinese patients with anti-γ-aminobutyric acid B receptor (GABA-BR) encephalitis.Methods We reviewed the clinical manifestations,cerebrospinal fluid (CSF) examinations,brain magnetic resonance imaging and prognosis of 12 patients who were diagnosed as anti-GABA-BR encephalitis in Capital Medical University Xuanwu Hospital from March 2013 to December 2015.Results The major clinical features of anti-GABA-BR encephalitis patients included seizures (12/12),cognitive disorder (10/12),psychiatric symptoms (10/12),sleep disorders (3/12),visual hallucination (2/12),involuntary movements (2/12),decreased consciousness (3/12),cerebellar signs (2/12),fever (2/12).GABA-BR-antibody was positive in CSF and serum of all the patients.Electroencephalogram revealed epileptic discharges in 4/12 patients.Brain MRI showed abnormal signal in up to 9/12 patients,located in the hippocampus,temporal lobes,thalamus and periventricular area.PET or SPECT indicated hypometabolism in 5/6 patients.After the average of fourteen months follow-up,9/12 patients had good prognosis,3 patients did not.Of 3 patients with poor outcome,2 had small cell lung cancer.Conclusions The predominant clinical features of the anti-GABA-BR encephalitis are seizures,cognitive disorder and psychiatric symptoms.The lesions are not only located in limbic system.Early diagnosis and immune modulation may provide a good outcome.
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Objective To observe the clinical characteristic and prognostic analysis of severe anti-N-methyl-D-aspartate receptor ( anti-NMDAR) encephalitis.Methods The clinical data of 9 patients with severe anti-NMDAR encephalitis were retrospective analyzed.Results Nine patients ( two male and seven females ) were taken care at ICU.They were 27.7 years old at average.The median course was 22.4 d and median length of stay in ICU was 50. 9 d.The mainly clinical characteristics were fever(7 cases), psychiatric behavioral symptoms(9 cases), seizures(9 cases) , decreased consciousness ( 8 cases ) , abnormal movement ( 7 cases ) , automatic instability ( 9 cases ) and hypoventilation(6 cases) .Three cases had ovarian teratoma.Anti-NMDAR antibodies in CSF of all patients were testing positive, while in serum were 6 cases.CSF-IgA in 7 cases were increased.The brain MRI indicated abnormal signals in temporal lobe, hippocampus in 3 patients.The EEG showed slowing wave abnormal in 5 patients.All patients were treated with combined first-line immunomodulatory therapies with steroids, intravenous immunoglobulin, or plasma exchange. Five patients were fullly recoveried, 4 patients were improved with residual symptoms. Conclusions Severe anti-NMDAR encephalitis may present progress quickly mental behavior change, epilepsy, and disturbance of consciousness, movement disorders, autonomic nerve dysfunction.Which accompanied with tumor is rare.Most patients with active immunotherapy can get good prognosis.
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@# Objective To evaluate the effect of contrast-enhanced transcranial Doppler (c-TCD) for detection of patent foramen ovale (PFO) comparing to transesophageal echocardiography (TEE). Methods 28 ischemic stroke patients with PFO detected with TEE were evaluated with TCD bubble study at rest and under Valsalva maneuver (VM). Results PFO was identified in 20 cases (71.43%) at rest by TCD bubble study. When VM was performed, PFO was detected in 24 cases (85.71%). There was no relevant in sensitivity of c-TCD with PFO size (P>0.05). Conclusion c-TCD is an excellent method for PFO identification with high sensibility while under VM. C-TCD can be used as a screening method for suspected PFO in patients with stroke test before TEE.
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AIM: To investigate the relationship between Alzheimer disease(AD) and alpha 2-macroglobulin(α2M) initially so as to provide evidence for the pathogenesis of late-onset AD.METHODS: The significance of α2M in the late-onset AD was revealed through pathology of α2M in AD and the polymorphism of α2M in AD patients.RESULTS: The association between AD and α2M region on the twelfth chromatosome was analyzed with the genic linkage, and pathological symptom of α2M in AD patients was supported by many evidences. Two polymorphism sites of α2M were located on the 12P12-13, which could increase more risks to AD patients.CONCLUSION: It is significant to investigate the frequency, mutation,polymorphism and expression of gene α2M for the pathogenesis of late-onset AD and its genic diagnosis.