POEMS syndrome presentation with progressive weakness in upper and lower limbs: A case report.

Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS) syndrome is a rare variant of plasma cell disorders with multiple systemic manifestations. A 50-year-old female patient presented with progressive weakness in her upper and lower limbs; tingling, numbness and burning in her feet; polyneuropathy (demyelinating in the majority of cases of POEMS syndrome); monoclonal plasma cell disorder (typicallyλ-restricted in cases of POEMS syndrome); sclerotic lesions on the spine and pelvis; organomegaly, including hepatomegaly, splenomegaly and lymphadenopathy; edema; pleural effusion; adrenal, thyroidal, pituitary, gonadal and pancreatic endocrinopathy; skin changes, including hyperpigmentation, dry skin and hypertrichosis; thrombocytosis; pulmonary hypertension; low vitamin B12 and weight loss. Following the diagnosis of POEMS syndrome, the patient was treated only with pain-alleviating corticosteroids. Respiratory failure-induced mortality occurred 24 months after the patient first experienced difficulty walking and numbness in her lower extremities. The present study suggests that abnormal symptoms in cases of POEMS syndrome should be further evaluated during the diagnosis and treatment.

[Comparison of brain effects of electroacupuncture at Zusanli (ST 36) and Guanyuan (CV 4) shown by fMRI in 21 healthy volunteers].

OBJECTIVE: To observe different brain effects of electroacupuncture (EA) stimulation of Guanyuan (CV 4) and Zusanli (ST 36) in the normal subjects by using functional magnetic resonance image (fMRI). METHODS: Twenty-one healthy volunteers were recruited in the present study. fMRI was used to investigate brain responses [blood-oxygenation-level-dependent (BOLD) signals] to EA stimulation of CV 4 and ST 36. A filiform silver needle was inserted into CV 4 or the left ST 36 randomly in two consecutive fMRI tests, and then manipulated with uniform reducing-reinforcing methods to induce "Deqi". fMRI scan was performed before needling, during needle retention, during EA stimulation, and post-EA. Volunteer's subjective needling sensations were recorded after EA. Data of fMRI were analyzed by using software SPM 2 and fMRI was mapped by Degree Centrality Measure method for whole brain correlation. The activation, deactivation, short-distance and long-distance functional connectivity maps of cerebral regions were investigated. RESULTS: The fullness and numbness feelings of the subjects were stronger during EA at ST 36 than at CV 4. EA at ST 36 or CV 4 induced apparent similar deactivation effects in the anterior cingulate and medial prefrontal cortices. The default mode of the brain at rest state was modified by needle retention and EA stimulation, respectively. The functional brain network was significantly changed after EA. The instant post-acupuncture effects (enhancement of the shortdistance functional connectivity) were mainly found in the ventral medial prefrontal cortex (VMPF) and ventral anterior cingulate cortex (vACC) in the limbic-paralimbic-neocortical network, and there were a little bit stronger signals in ST 36 than in CV 4. CONCLUSION" EA stimulation of CV 4 and ST 36 induces a similar modulation effect in the limbic-medial prefrontal network in healthy subjects.

[Effect of acupoints with different tissue on electroacupuncture sensation].

OBJECTIVE: To evaluate the influence on frequency, intensity and the amount of current flow produced by electroacupuncture sensation of acupoints with different distributions (meridian category, tissue type and nerve innervations). METHODS: Twenty-one healthy volunteers were enrolled. According to the different tissue of acupoints, paired acupoints were grouped: Zusanli (ST 36) vs Yanglingquan (GB 34) (different meridian categories), Neiguan (PC 6) vs Daling (PC 7) (different tissue types), Zusanli (ST 36) vs Shuidao (ST 28) (different nerve innervations), Guanyuan (CV 4) vs Zhongwan (CV 12) (different nerve innervations), and Zusanli (ST 36) vs Guanyuan (CV 4) (different meridian categories, tissue types and nerve innervations). The electric frequency was 15 Hz. The electric current was under the participant's tolerance without sharp pain; the electroacupuncture was applied for three times with interval, 90 seconds totally. Soreness, numbness, fullness, heaviness, tingling, pressure, dull pain, warmness, coolness and amount of current flow were recorded, and the frequency, intensity and current amount between the paired acupoints were compared. RESULTS: There were no significant differences in frequency of needling sensations between paired points (all P > 0.05). In the 9 electroacupuncture sensations, fullness, numbness and soreness were most commonly seen and obvious, heaviness, pressure and tingling came second, and dull pain, warmness, and coolness occurred at lower frequency. There were differences in the intensity of acupuncture sensation between paired acupoints in part of them, the soreness, fullness and heaviness at Zusanli (ST 36) were stronger than those at Shuidao (ST 28) (all P < 0.05 ); fullness and numbness at Zusanli (ST 36) were stronger than those at Guanyuan (CV 4) (both P < 0.01); fullness at Guanyuan (CV 4) was stronger than that at Zhongwan (CV 12) (P < 0.05 ); the current flow of electroacupuncture was similar between paired acupoints (all P > 0.05). CONCLUSION: In the situation that there is no obvious difference in amount of current flow caused by electroacu puncture sensation, the obvious correlations between frequency of electroacupuncture sensation and tissue properties of acupoints are not appeared, and the difference of sensation intensity between paired acupoints may be associated with different nerve innervations. The frequency and intensity of electroacupuncture sensation at one acupoint are stable at different times.

[Electroacupuncture at Guanyuan (CV 4) and Zhongwan (CV 12) modulates functional connectivity of the brain network in healthy volunteers].

OBJECTIVE: To observe the specific brain effects of electroacupuncture (EA) stimulation of Guanyuan (CV 4) and Zhongwan (CV 12). METHODS: Twenty-one healthy volunteers were recruited in the present study. Two silver filiform needles were separately inserted into Guanyuan (OV 4) or Zhongwan (CV 12), and manipulated with uniform reducing-reinforcing method to induce "Deqi". fMRI scan was performed before needling, during needle retention, EA stimulation, and post-EA. Data of fMRI was analyzed by using software SPM 2. The volunteer subjective needling sensations were recorded. The activation, deactivation, short-distance and long-distance functional connectivity maps of different cerebral regions were analyzed by using whole brain correlation analysis. RESULTS: Comparison between the two acupoints showed that fullness feeling was stronger in CV 4 than in CV 12. EA at CV 4 and CV 12 induced a similar stronger and prevalent deactivation in the ventral medial prefrontal cortex and the anterior cingulated cortex (ACO). The deactivation of the ACC was stronger in the CV 4 group than in the CV 12 group. The default BOLD mode of the brain at rest was modified by needle retention and EA, respectively. The short-distance functional connection brain network was significantly changed after EA. Interestingly, the ventral medial prefrontal cortex and anteroinferior portion of the anterior cingulate cortex in the limbic-paralimbic-neocortical network (LPNN) were involved in the instant post-effects of EA. Relatively smaller differences in the brain functional activity and short-distance functional connectivity were found between these two acupoints. CONCLUSION: EA of CV 4 and CV 12 can modulate short-distance functional connectivity of the LPNN, and have fewer differences in inducing needling sensation and deactivation of ACC, etc.

[A genetic and clinical study in a family with familial hypercholesterolemia].

OBJECTIVE: To investigate the low density lipoprotein receptor (LDLR) gene and apolipoprotein (Apo) B gene mutation in a Chinese family with familial hypercholesterolemia (FH) and give the kindreds clinical check-ups. METHODS: After physical examination, the kindreds underwent ECG and ultrasound checks. Blood samples were tested for lipid profiles. The promoter and all eighteen exons of LDLR gene were investigated by using PCR and agarose gel electrophoresis in combination with DNA sequence analysis. The results were compared with the normal sequences in GenBank and FH database (www.ucl.ac.uk/fh) to find mutations. In addition, the apolipoprotein B100 gene for known mutations (R3500Q, R3531C, R3501W and R3480W) that cause familial defective ApoB100 (FDB) was also tested using the same method. RESULTS: A novel homozygous G > A mutation at the 1581 bp of exon 10 was detected in the proband and his siblings. It caused a substitution of amino acid Glu to Gly at codon 496. A novel heterozygous G > A mutation at the 1581 bp of exon 10 was detected in his parents. No mutations of R3500Q, R3531C, R3501W and R3480W of ApoB100 were observed. ECGs were normal. Atherosclerosis were found in all family members by ultrasound checks. CONCLUSIONS: The homozygous G > A mutation at the 1581 bp of exon 10 was first determined in our country. The change of amino acid Glu to Gly is responsible for FH of the family. The type of the gene mutation was not found in the FH database (www. ucl.ac.uk/fh). It's a new type of LDLR mutation.

Study on syndrome element characteristics and its correlation with coronary angiography in 324 patients with coronary heart disease.

OBJECTIVE: Recently, a new traditional Chinese medicine differentiation theory "Syndrome Element (SE)" has been raised. In this study, the main syndrome element types and their correlations with the results of coronary angiography (CAG) in patients with coronary heart disease (CHD) were investigated. METHODS: Epidemiology cross-sectional study method was employed and 324 patients with CHD were enrolled, and their syndrome element types as well as the CAG results were analyzed. The correlations among syndrome element types, Gensini score, and the number of abnormal branches were also analyzed based on the distribution characteristics of syndrome element and coronary angiography results in the 324 cases. RESULTS: According to their occurrence frequency in 324 CHD patients, the top eight major heart syndrome elements were Xin () blood stasis (85.8%), Xin qi deficiency (79.6%), Xin heat blockage (41.1%), Xin phlegm with turbid fluid (38.0%), Xin qi stagnation (24.7%), Xin yang deficiency (18.9%), Xin yin deficiency (17.5%) and Xin cold coagulation (4.4%), respectively, which suggested that Xin blood stasis and Xin qi deficiency were the two most common syndrome elements. Also, as coronary artery Gensini score increased, the changing trend of the syndrome element was "Xin yang deficiency with blood stasis" to "Xin phlegm obstruction with heat blockage" to "Xin yin deficiency with blood stasis" to "Xin qi deficiency with blood stasis" to "Xin cold coagulation with phlegm and turbid fluid, "Xin cold coagulation with blood stasis" to "Xin deficiency of qi, yin and yang". As the number of abnormal branches increased, the syndrome element changing trend was "simultaneous occurrence of cold and heat syndrome" to "Xin qi and yang deficiency with blood stasis" to "Xin retention of phlegm with turbid fluid" to "Xin cold coagulation in the heart meridian", "Xin deficiency of both qi and yin". The result of this study shows that Xin qi deficiency and Xin blood stasis were the major syndrome elements in patients with CHD. CONCLUSION: As the severity and extent of coronary artery lesion increased, there were some apparent correlations among syndrome elements, Gensini score and number of abnormal coronary artery branches.