VM Patients Research Articles

Stratification and characteristics of common venous malformation by anatomical location

PurposeVenous malformation (VM) has variable presentations. This study stratifies VM by its topographic distribution, types of tissues involved, proportion of affected patients undergoing active treatment and the types of treatment(s) administered within a closed population. MethodsConsecutive VM patients were identified from the prospectively maintained vascular anomalies database 1996–2015 at our national Vascular Anomalies Centre. Demographic data of the patients and the characteristics of their VM were obtained from the database and supplemented by review of medical records. ResultsAmong 1907 vascular anomalies patients, 316 (16.6%) had VM including 191 females and 125 males, aged 2–93 (mean, 34) years. Twelve (3.8%) patients had multiple VMs with four (1.3%) patients having a family history of VM. The lesion(s) was located in the head and neck (n = 152), lower limb (n = 80), upper limb (n = 61), trunk (n = 13), lower limb girdle (n = 11), perineal (n = 10), upper limb girdle (n = 6) and pelvic (n = 1) regions. Two anatomic sites were affected in 10 patients, and 3 sites in 6 patients. The lesion(s) affected the cutaneous/subcutaneous tissue (n = 211), muscle (n = 103), mucosa (n = 79), orbit (n = 14), parotid gland (n = 11), joint (n = 6), bone (n = 5), and intracranial vessels (n = 3). The management included observation (n = 133), surgery (n = 101), ethanol sclerotherapy (ES, n = 79) and low-dose aspirin (n = 32). Fifteen patients received both surgery and ES. ConclusionsVM affects different anatomic regions and tissues. Management of VM should be individualised for each patient who may require more than one treatment modality.

Vestibular migraine: A correlation study between clinical findings and vestibular evoked myogenic potentials (VEMPs)

ObjectivesVestibular migraine (VM) is one of the most common causes of episodic vertigo. Several studies have reported abnormal cervical vestibular evoked myogenic potentials (cVEMPs) in VM patients. Recent studies have investigated the diagnostic values of ocular vestibular myogenic evoked potentials (oVEMPs) in VM patients. However, they were few and rarely attempted to correlate the finding with the results of other vestibular tests. So this study aimed to investigate both cVEMPs and oVEMPs in VM patients and to correlate their results with other clinical findings. Subjects and methodsThe study group consisted of twenty adult patients with criteria of definite VM. The control group included age and gender matched twenty healthy subjects. For all subjects detailed history and basic audiologic evaluation were done. Vestibular evaluation included clinical office tests and VEMPs (cVEMPs and oVEMPs). Patients were examined during interictal period and in the absence of vertigo. ResultsTrue vertigo was experienced in 60% of patients, while 40% patients had sense of unsteadiness. Office tests for vestibulo-spinal reflex were abnormal in 5 patients (25%). Abnormal oVEMPs responses were more frequent (No.=19 “95%”) than cVEMPs (No=15 “75%”). The main abnormality was prolonged latency and/or decreased amplitude. Affection was more bilateral while absent response was less frequently encountered. Significant correlation was found between abnormal office tests and VEMPs abnormalities. ConclusionsBoth cVEMPs and oVEMPs abnormalities were detected in VM patients. Abnormal office tests were among patients with severe VEMPs affection during interictal period.

Clinical features of vestibular migraine in Egypt

BackgroundMany reports have documented a relationship between vestibular and migraine disorders. The term vestibular migraine appeared in an appendix of the third edition of IHS as a first step for new entities (Neuhauser et al., 2001). ObjectiveTo determine the prevalence of vestibular migraine in a dizziness clinic and to describe its clinical profile. MethodsRetrospective study was conducted in the period from January 2011 to December 2013. Charts of patients coming for Hearing and Balance Clinic were thoroughly reviewed. Patients fulfilling criteria of definite VM according to The International Classification of Headache Disorders, 3rd edition (beta version) were selected. A full neuro-otologic evaluation and Videonystagmography with bithermal caloric testing were included in their files and were analyzed. ResultsThe prevalence of vestibular migraine in a dizziness clinic was found to be 22%. Vertigo either isolated or combined with the sense of imbalance was found in over 60%. Phonophobia was the commonest aural symptom, followed by the sense of bilateral ear fullness and tinnitus. Positional nystagmus was frequently recorded in 60% of the patients. Caloric weakness was seldom seen (7.5%). Hearing loss was encountered in only 3.1%. None of the patients demonstrated abnormalities in ocular motor tests. History of motion sickness was highly statistically correlated to the presence of VNG abnormalities. ConclusionsVestibular migraine is one of the leading causes of dizziness in both adults and children. A high percentage of patients showed abnormalities in Videonystagmography during testing documenting vestibular system involvement in migraine patients during the dizziness attack. Good history taking and analysis of aural symptoms helps in the differential diagnosis. The profile of VM patients is outlined.

Sensitivity to Fairness and Intentions of Others in the Ultimatum Game in Patients with Ventromedial Prefontal Lesions

This study aimed to examine the relationship between perspective-taking and impaired decision-making in patients with ventromedial prefrontal (VM) lesions, using the Ultimatum Game (UG). In the UG, two players split a sum of money and one player proposes a division while the other can accept or reject this. Eight patients with VM damage and 18 healthy controls participated as responders in a modified version of the UG, in which identical offers can generate different rejection rates depending on the other offers available to the proposer. Participants had to either accept or reject offers of 2:8 NIS (2NIS for them and 8 NIS for the proposer), which were paired with one of four different possible offers (5:5, 4:6, 2:8, 8:2). Results indicate that the controls more often rejected offers of 2:8 when the alternative was 4:6 (a greedy alternative) than when the alternative was 5:5 (fair alternative), whereas the VM patients showed the opposite pattern of decision-making. Additionally, the overall rejection rates were higher in patients as compared to controls. Furthermore, scores on a perspective-taking scale were negatively correlated with rejection rates in the patient group, suggesting that perspective-taking deficits may account for impaired decision-making in VM patients.

The Use of S100B and Tau Protein Concentrations in the Cerebrospinal Fluid for the Differential Diagnosis of Bacterial Meningitis: A Retrospective Analysis

Background: Patients with meningitis are often difficult to classify into bacterial (BM) or benign viral (VM) meningitis. To facilitate the differential diagnosis, S100B and Tau protein in the cerebrospinal fluid (CSF) were measured and compared with standard laboratory parameters. Methods: S100B_CSF, Tau_CSF, and routine parameters (CSF leukocyte count, protein_CSF, lactate_CSF, serum C-reactive protein, blood leukocyte count and body temperature) were analyzed in 33 patients with microbiologically confirmed BM and in 19 with VM. Their classification accuracy, sensitivity and specificity were studied by receiver operating characteristic (ROC) curves. Results: S100B_CSF concentrations were higher in BM than in VM patients (p = 0.03) and showed a promising accuracy (90%) for the differential diagnosis of BM versus VM. Its discriminative properties were comparable to routine parameters. Of all parameters, S100B_CSF showed the highest specificity (100%) with an optimal cut-off of 3.1 ng/ml. Tau_CSF concentrations were useless for the discrimination (p = 0.64). Conclusions: In contrast to Tau_CSF, S100B_CSF concentrations ≧3.1 ng/ml are promising to discriminate bacterial from viral meningitis.

Role of RANK, RANKL, OPG, and CXCR4 Tissue Markers in Predicting Bone Metastases in Breast Cancer Patients

BackgroundThe RANK/RANKL/OPG system is active in primary cancers such as breast, prostate, and also in their bone metastases. CXCR4 chemokine receptor is highly expressed in human breast cancer cells and is believed to facilitate the homing of tumor cells to organs such as bone that express high levels of its ligand SDF1. Our study aimed to investigate whether the analysis of these markers with an inexpensive and simple test can help to predict bone metastases in breast cancer patients. Patients and MethodsMarker expression was evaluated by immunohistochemical staining in paraffin-embedded tissue sections of primary breast cancers from 40 individuals: 20 patients with bone metastases (BM), 10 with visceral metastases (VM; considered together as the relapsed group), and 10 with no evidence of disease (NED). ResultsRANKL was not detected in tumor cells. OPG- and RANK-positive tumors are found with similar frequency in NED (20%) and in relapsed patients (23% and 17%, respectively). However, in the latter subgroup, only RANK positivity was always associated with bone relapse. The frequency of CXCR4-positive tumors was three-fold higher in relapsed (30%) than in NED (10%) patients and positivity was always linked to bone metastases. Considering NED and VM patients together versus BM patients, we observed that CXCR4 expression, alone (P = .008) or in combination with RANK (P < .001), identified patients destined to relapse to bone. ConclusionOur results provide the first clinical evidence to support a pivotal role of combined CXCR4 and RANK expression in predicting bone relapse.

361 Neuropsychological Performance in Patients with Alcohol Dependence

A decision-making instrument known as the ‘gambling task’ was used, which has been shown to be sensitive to the decision-making impairment of patients with bilateral lesions of the ventromedial prefrontal cortex (VM). Three groups of subjects were tested, substance dependent individuals (SD) (n=41), normal controls (n=40), and VM patients (n=5). All SD met the DSM-IV criteria for dependence, with either alcohol or stimulants (metamphetamine or cocaine) as the primary substance of choice. The results revealed a significant impairment in the performance of SD relative to normal controls. A significantly high proportion of SD (61 vs. only 32.5% of normal controls) performed within the range of the VM patients, while the rest performed within the range of normal controls. General demographic factors such as age, sex, and level of education could not explain these differences in performance. As well, differences in performance were not explained by intelligence (IQ), memory, or performance on standard executive function/frontal lobe tests. Performance on the gambling task was best predicted by a combination of factors, including duration of abstinence, years of abuse, relapses and times in treatment, and the ability to hold gainful employment. The results support the hypothesis that impairment in decision-making linked to a dysfunctional VM cortex is associated with at least a sub-group of SD.

Abstract P1-13-04: Immunohistochemical Evaluation of RANK/RANKL/OPG Axis and CXCR4 in Primary Breast Cancer and Their Role in Bone Metastasization

Abstract Background: The RANK/RANKL/OPG axis plays an important role in the bone metastasization process. CXCL12 is overexpressed in bone and, like its receptor, CXCR4, is a determinant of organ tropism. The objective of the present study was to evaluate whether the expression of these markers in primary breast cancer can predict the onset of bone metastases. Methods: Marker expression was evaluated by immunohistochemical staining in paraffin-embedded sections of primary breast cancers from 40 individuals: 10 patients (median age 64 years, range 48-78) showed no evidence of disease (NED — control group) at a median of 9.8 (range 6.9-11.5) years, while 30 (median age 67 years, range 42-87) had relapsed. In the latter group, 10 (median age 66 years, range 42-87) had visceral metastases (VM — control group) and 20 (median age 69 years, range 42-87) had bone metastases (BM — case group), both confirmed radiologically. The 3 patient subgroups (NED, visceral lesions, bone lesions) were matched for age classes (≥60 years, &amp;gt; 60 years). The study design was reviewed and approved by the local ethics committee. Results: In the overall series, 17.5% of tumors were positive for RANK, 22.5% for OPG and 25% for CXCR4. None of the patients expressed RANKL. RANK and OPG resulted equally expressed in 20% of NED patients, while CXCR4 was expressed in only 10% of this group. OPG was expressed in 20% of VM patients, whereas RANK or CXCR4 were not detected. RANK and OPG positivity was present in 25% of BM patients, while CXCR4 was expressed in 45% of cases. CXCR4 was the only marker with a significantly higher frequency of expression in bone metastases than in visceral lesions (p=0.013). Taking into consideration all the patients without bone involvement (NED plus VM patients), it was observed that CXCR4 expression, alone and in combination with RANK, significantly discriminated between BM patients and the control group (p=0.008 and P&amp;lt;0.001, respectively). ER-positivity and HER2-negative status were observed in 80% and 95% of BM patients, respectively, but neither discriminated between cases and controls. Conclusions: In our study, ER-positivity and HER2- negativity identified a high percentage of bone relapse patients, but also highlighted patients who did not have bone involvement. RANK and CXCR4 expression, on the other hand, would appear to be a more accurate predictor of bone relapse. Such evidence could help to identify patients with a high probability of developing bone metastases, which can be treated with bisphosphonates or other bone-targeted therapies, such as denosumab, in an attempt to prevent distant metastases. Our results are currently being validated in a larger series of breast cancer patients. Citation Information: Cancer Res 2010;70(24 Suppl):Abstract nr P1-13-04.

Who is at risk for ongoing dizziness and psychological strain after a vestibular disorder?

Patients with vestibular vertigo syndromes often suffer from anxiety and depression, whereas patients with psychiatric disorders often experience subjective unsteadiness, dizziness, or vertigo. Thus, it has been hypothesized that the vestibular system may be interlinked with the emotion processing systems. The aim of the current study was to evaluate this hypothesis by correlating vestibular and psychiatric symptoms with the course of the disease over 1 year. This interdisciplinary, prospective, longitudinal study included a total of 68 patients with acute vestibular vertigo syndromes. Four subgroups of patients with benign paroxysmal positioning vertigo (BPPV, n=19), acute vestibular neuritis (VN, n=14), vestibular migraine (VM, n=27), or Menière's disease (MD, n=8) were compared. All patients underwent neurological and neuro-otological examinations and filled out standardized self-report inventories including the Vertigo Symptom Scale (VSS), the Vertigo Handicap Questionnaire (VHQ) and the Symptom Checklist 90R (GSI, SCL-90R) at five different times (T0–T4) in the course of 1 year. VM patients experienced significantly more “vertigo and related symptoms” (VSS-VER), “somatic anxiety and autonomic arousal” (VSS-AA), and “vertigo induced handicap” (VHQ) than all other patients (P<0.001−P=0.006). Patients with a positive history of psychiatric disorders had significantly more emotional distress (GSI, SCL-90R), regardless of the specific phenomenology of the four diagnostic subgroups. Finally, fluctuations of vestibular excitability correlated positively with the extent of subjectively perceived vertigo. VM patients are significantly more handicapped by vertigo and related symptoms. They show significantly elevated fluctuations of vestibular excitability, which correlate with the (subjective) severity of vertigo symptoms.

Assessment of the response to chemotherapy in gestational trophoblastic neoplasia with vaginal metastases

This study is designed to survey gestational trophoblastic neoplasia with vaginal metastases (GTN + VM) manifestations, prognosis and chemotherapy response in order to consider appropriate chemotherapy regimen for these patients. There have been just a few studies about treatment of GTN + VM. Patients with Stage III GTN with or without vaginal metastases who had referred to Vali-e-Asr Hospital during 1996-2006 have been selected to take part in this study and the size of metastases was measured. Then response and resistance to single and combination chemotherapy regimens have been compared in these two groups. The data were processed using SPSS system (release 10). Statistical analysis was done with Chi(2) to determine factors associated with complete clinical response. The level of significance was assigned at P< .05. Forty-eight patients with stage III (with pulmonary metastases) GTN patients have been selected, 13 with vaginal metastases and 35 without vaginal metastases. Incidence of vaginal metastases in stage III was 26%. Metastases were mainly in distal part of vagina and suburethra. Chief complaint was severe hemorrhage in 25% of patients and was controlled by vaginal packing with just one exception. Group of vaginal metastases showed 66.6% resistance to first-line chemotherapy, compared to 28.6% in patients with no vaginal metastases (P-value=0.010). Median of chemotherapy courses in low-risk vaginal metastatic patients was 5 (mean=5.2), compared to three courses in the group without vaginal metastases. Resistance to single chemotherapy was significantly higher in GTN + VM versus GTN without VM patients and resistance was higher especially in patients with metastases with more than 3 cm (in diameter). Vaginal metastasis with more than 3 cm in diameter is an important prognostic factor in GTN patients. There have higher risks for severe hemorrhage and resistance to single agent chemotherapy, so in these patients combination chemotherapy, with no regard to staging and scoring, would be a better choice.

Moral Belief Attribution: a Reply to Roskies

I here defend my earlier doubts that VM patients serve as counterexamples to motivational internalism by highlighting the difficulties of belief attribution in light of holism about the mental and by suggesting that a better understanding of the role of emotions in the self-attribution of moral belief places my earlier Davidsonian “theory of mind” argument in a clearer light.

Decision-making deficits, linked to a dysfunctional ventromedial prefrontal cortex, revealed in alcohol and stimulant abusers

A decision-making instrument known as the ‘gambling task’ was used, which has been shown to be sensitive to the decision-making impairment of patients with bilateral lesions of the ventromedial prefrontal cortex (VM). Three groups of subjects were tested, substance dependent individuals (SD) ( n=41), normal controls ( n=40), and VM patients ( n=5). All SD met the DSM-IV criteria for dependence, with either alcohol or stimulants (metamphetamine or cocaine) as the primary substance of choice. The results revealed a significant impairment in the performance of SD relative to normal controls. A significantly high proportion of SD (61 vs. only 32.5% of normal controls) performed within the range of the VM patients, while the rest performed within the range of normal controls. General demographic factors such as age, sex, and level of education could not explain these differences in performance. As well, differences in performance were not explained by intelligence (IQ), memory, or performance on standard executive function/frontal lobe tests. Performance on the gambling task was best predicted by a combination of factors, including duration of abstinence, years of abuse, relapses and times in treatment, and the ability to hold gainful employment. The results support the hypothesis that impairment in decision-making linked to a dysfunctional VM cortex is associated with at least a sub-group of SD.