Atypical Takotsubo cardiomyopathy associated with neuromyelitis optica spectrum disorders: a case report / 中华神经科杂志

Recently, neuromyelitis optica spectrum disorders (NMOSD) appear to be a multi-organ disorder, however, the involvement of myocardium in NMOSD is extremely rare. In the present article, we present a young girl who manifested bilateral optic neuritis, area postrema syndrome, brainstem syndrome and transverse myelitis, as well as tachycardia, abnormal electrocardiograph, moderate elevation of myocardial biomarker and regional wall movement abnormalities, which confirmed the diagnosis of Takotsubo cardiomyopathy associated with NMOSD. The neurological deficits along with myocardial injury were recovered soon after the administration of intravenous methylprednisolone and intravenous immunogloblin. This is a rare case that should be paid attention and by which can not only broaden the symptom spectrum of NMOSD, but can also provide novel visions for further investigating the mechanism of organs damage.

Clinical and genetic analysis of a patient with Krabbe disease presented as peripheral neuropathy / 中华医学遗传学杂志

OBJECTIVE@#To explore the clinical, electrophysiological and imaging features of a patient with Krabbe disease caused by GALC mutation.@*METHODS@#A comprehensive analysis including clinical investigation and genetic testing was carried out.@*RESULTS@#The patient presented with peripheral neuropathy with electrophysiological anomaly suggestive of asymmetric demyelinating neuropathy. Brain imaging revealed leukoencephalopathy. Genetic analysis has identified compound heterozygous mutations in exons 5 and 11 of the GALC gene, namely c.461C>A and c.1244G>A.@*CONCLUSION@#Krabbe disease is a group of disorders featuring substantial phenotypic heterogeneity. Genetic and enzyme testing has become indispensable for accurate diagnosis for this disease.

Clinical Study onShugan Liqi Qingqiu Decoction in the Treatment of Allergic Rhinitis / 中国中医药信息杂志

Objective To evaluate the clinical efficacy and safety ofShugan Liqi Qingqiu Decoction in the treatment of allergic rhinitis.Methods Totally 74 patients were randomly divided into treatment group and control group in a 1:1 ratio. The treatment group received the prescription ofShuganLiqiQingqiu Decoction, and control group was given loratadine tablets orally, 7 days as a course of treatment and 3 courses successive. After treatment, the visual analogue scale (VAS), Rhinocoujunctivitis Quality of Life Questionaire (RQLQ), Specific IgE (SIgE) values and total effective rate were measured to evaluate the clinical efficacy, and the total number of cases of adverse reactions was statistically analyzed.Results After all the patients were researched, the total effective rate of treatment group was 86.49% (32/37) in the treatment group and 89.19% (33/37) in control group, without statistical difference (P>0.05). There was statistical difference in VAS and RQLQ between before and after treatment in the two groups (P0.05) in VAS and was statistical difference in RQLQ (P0.05) in SIgE in the treatment group, and was statistical difference (P0.05) in SIgE between the two groups after treatment. There was no adverse reaction except for 2 patients in the treatment group with mild diarrhea. Conclusion ShuganLiqiQingqiu Decoction has good clinical efficacy and safety for the treatment of allergic rhinitis.

IgG4-related spinal pachymeningitis.

The aim of this study is to study the clinical, laboratory, imaging pathology, and prognosis features of IgG4-related spinal pachymeningitis. We worked with a 55-year-old man suffering from IgG4-related spinal pachymeningitis who had the most widespread lesion in his dura mater. We also review previous related studies and discuss the clinical characteristics of this rare disease. In total, eight IgG4-related spinal pachymeningitis patients have been reported in the literature since 2009. They were mostly male patients, 51.7 ± 11.9 years old on average. Cervical and thoracic vertebrae were the most common sites for lesions. The most prominent symptom was varying numbness and weakness of the limbs and/or body associated with spinal cord compression. There was one patient (1/5) with elevated serum IgG4 levels and three patients (3/3) with increased cerebrospinal fluid (CSF) IgG4 index. Positive histopathologic findings are the strongest basis for a diagnosis. All the patients with IgG4-related spinal pachymeningitis responded well to glucocorticoid therapy. IgG4-related spinal pachymeningitis is an orphan disease that mainly occurs in cervical and thoracic vertebrae. Older males are the most susceptible group. Serum IgG4 levels were consistently normal in these cases, so analysis of CSF for IgG4 production (IgG4 index) could become a useful tool. Pathological findings remain the gold standard for diagnosis. Most patients responded favorably to glucocorticoid treatment.

Therapeutical Advances in Chronic Inflammatory Demyelinating Polyneuropathy (review) / 中国康复理论与实践

@#Chronic inflammatory demyelinating polyneuropathy (CIDP) is one of the acquired autoimmune peripheral neuropathy with various therapeutical methods. This article reviewed the therapeutical advances in CIDP.