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Objective:To summarize the clinical, pathological and muscle magnetic resonance imaging (MRI) features of human immunodeficiency virus (HIV)-associated nemaline myopathy (NM; HIV-NM).Methods:The present patient was a 23-year-old man with HIV infection who developed progressive proximal weakness and atrophy for more than 10 months. He was admitted to the Department of Neurology of Beijing Ditan Hospital in early June 2021. Electromyography showed myogenic findings. The serum creatine kinase was 202.4 U/L. CD 4+ count was 585×10 6/L. Serum monoclonal immunoglobulin (M protein) was negative. The patient underwent MRI examination of bilateral thigh muscles, biopsy of left biceps brachii and gene detection. The clinical, pathological and muscle MRI changes of HIV-NM were summarized based on the literature review. Results:MRI examination of bilateral thigh muscles showed edema changes. Muscle biopsy showed nemaline structures in some muscle fibers, accompanied by fiber atrophy and regeneration. No gene mutation related to clinical phenotype was found by second-generation sequencing. After intravenous immunoglobulin combined with prednisone, the patient′s weakness symptoms were significantly improved. A total of 17 cases of HIV-NM (including the present case) were identified in the literature, who were aged (33.7±9.1) years. Fifteen were males and two were females. All patients developed proximal limb weakness. Creatine kinase was normal or slightly elevated. Serum monoclonal protein was positive in 3 cases (3/7). Immunosuppressive therapy was effective.Conclusions:The main clinical characteristics of HIV-NM are progressive proximal limb weakness and muscle atrophy. The muscle pathology shows a large number of nemaline structures in atrophic muscle fibers. Muscle edema can be seen on muscle MRI. This is the first report of HIV-NM in China, which may be a special subtype of immune myopathy.
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The clinical manifestations,serum and cerebrospinal fluid (CSF) results,EEG and imaging findings of 4 cases of encephalitis with positive leucine-rich glioma inactivated protein 1 (LGI1)antibody admited from March 2017 to January 2019 were retrospectively analyzed.All 4 patients were males with a mean age of (42±17) years.Two patients had faciobrachial dystonic seizure(FBDS)as the main symptom,and 3 patients had epilepsy.There were normal blood sodium and no memory deficits in all 4 patients.The LGI1 antibody was positive in the serum and negative in the CSE.The longest diagnosis time was 5 months.The head MRIs were normal in 3 patients;and abnormal signals in the right basal ganglia were found in one case,and the lesicl became smaller after the treatment.Chest CT,abdominal CT,and pelvic CT did not detect tumors.All patients were treated with gamma globulin and glucocorticoids,they all had good prognosis.LGI1 antibody-positive encephalitis is mainly characterized by epilepsy. ldentifying FBDS would be helpful for early diagnosis.Timely treatments often result in good clinical prognosis.
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Objective: To study correlation between heart rate variability (HRV) and arteriosclerosis in patients with essential hypertension (EH).Methods: A total of 139 EH patients from our hospital were enrolled as EH group.According to carotid-femoral pulse wave velocity (PWV), they were divided into normal subgroup (n=65, PWV<9 m/s) and abnormal subgroup (n=74, PWV≥9 m/s).Another 60 healthy adults undergoing physical examination in our hospital during the same period were selected as healthy control group.HRV indexes, PWV, carotid intima-media thickness (IMT) and number of carotid plaques were compared among all groups.Correlation among above indexes were analyzed in EH patients.Results: Compared with healthy control group, there was significant reduction in standard deviation of normal to normal RR intervals in all 5 min segments of the entire recording[SDANN, (142.3±21.4)ms vs.(119.3±16.5)ms], significant rise in low frequency[LF, (341.7±37.9)Hz vs.(695.4±43.3) Hz], high frequency[HF, (519.1±33.6) Hz vs.(636.8±39.5)Hz], LF/HF[(0.6±0.3) vs.(1.0±0.2)], PWV[(7.44±0.82) m/s vs.(11.13±0.65) m/s]and IMT[(1.03±0.24)mm vs.(1.74±0.41)mm]in EH group, P<0.01 all;in EH group, compared with normal PWV subgroup, there was significant reduction in SDANN, and significant rise in LF, HF, LF/HF, IMT and mean number of carotid plaques in abnormal PWV subgroup, P<0.05 or <0.01;Pearson correlation analysis indicated that in EH patients, PWV and IMT were significant inversely correlated with SDANN (r=-0.713,-0.699, P<0.01 both), significant positively correlated with LF, HF, LF/HF and carotid plaques (r=0.506~0.935, P<0.01 all), and PWV was significant positively correlated with IMT (r=0.883, P=0.001).Conclusion: In EH patients, SDANN, LF, HF, LF/HF of HRV are significantly correlated with arteriosclerotic extent.Besides lowering blood pressure , HRV etc.related with artery pathological changs should also be treated.
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Objective To analyze the clinical characteristics of cerebral syphilitic gunma.Method The clinical characteristics,brain CT and MRI results and prognosis of 7 patients with cerebral syphilitic gumma admitted in Beijing Ditan Hospital from June 2012 to October 2016 were retrospectively analyzed.Results There were 5 females and 2 males in 7 patients.The symptoms or signs at onset were headache (n =5),increased white blood cells in cerebrospinal fluid (n =6) and increased protein contents in cerebrospinal fluid (n =6).Total 10 lesions were detected in 7 cases,including 7 in cerebral lobe,and 3 in cerebellum,corpus callosum or intercrural space.The brain MRI showed nodular reinforcement in 5 cases,irregular circular reinforcement in 2 cases and obvious edema around the lesions in 6 cases.The patients were given intravenous infusion of 400 million units common penicillin,q.4h for 2 wks,sequential intramuscular injection of 240 million units long-term penicillin q.w for 3 wks.Among 7 patients,1 case died,1 case showed symptomatic improvement and 5 cases obtained complete improvement with the significant imaging improvement.Conclusion Headache is the most common symptom at onset of cerebral syphilitic gumma,and the lesions mostly are located in the cerebral lobes with nodular enhancement in brain imaging,and the prognosis is generally good.