VIM thalamic stimulation for tremor in a patient with IgM paraproteinaemic demyelinating neuropathy.

We demonstrate the effect of deep brain stimulation of the ventral intermediate thalamic nucleus on intractable action tremor, in a 72-year-old man suffering from neuropathy associated with monoclonal gammopathy.     

Antisocial personality disorder, HIV risk behavior and retention in methadone maintenance therapy

In a sample of 55 consecutive methadone maintenance admissions to our clinic, 42% were diagnosed with antisocial personality disorder (ASPD) using the National Institute of Mental Health Diagnostic Interview Schedule NIMH DIS. Individuals with ASPD exhibited greater risk for HIV infection as defined by more sexual contacts, needle use and equipment sharing. Data at 1 year follow-up were obtained on this group of patients. The objective was to compare the ASPD and non-ASPD groups with regards to demographics, drug abuse history, outcome and retention in treatment. There were no significant differences between the groups on any demographic or treatment outcome variables. Survival analysis indicated that there were no group differences in treatment retention. In conclusion, although there were no differences in treatment outcome between ASPD and non-ASPD groups it is possible that ASPD patients who drop out of treatment will be at higher risk for contracting and spreading HIV within the IV drug using population. These data also suggest that in this population the diagnosis of ASPD using primarily behavioral traits as measured in the NIMH-DIS-III, has little utility in predicting treatment outcome.     

A controlled evaluation of hypnobehavioural treatment for bulimia nervosa: Immediate pre-post treatment effects

The study reports the pre-post findings from a controlled comparative evaluation of treatments for bulimia nervosa. These pre-post results allow comparison of the hypnobehavioural and cognitive behavioural treatments with a waiting list control group and a comparison of the immediate effects of the two modalities. One hundred and thirty subjects were screened to enter the study. Seventy-eight subjects entered the investigation after being randomly allocated to either a waiting list control group, or to hypnobehavioural or cognitive behavioural groups. The treatments were delivered individually and matched in duration (8 weeks) and the number of sessions. Pre to posttreatment outcome indicated significant differences between the control group and the two treatments in reductions in bulimic behaviours and related eating pathology. The immediate effects of both treatments were equal. There were no differences at posttreatment between the treatments in abstinence from either bingeing or purging. The treatment effects were also similar to the immediate effects obtained by longer therapeutic approaches.     

Renal clearance and protein binding of melphalan in patients with cancer

Summary The renal clearance of melphalan and the fraction unbound in plasma were determined after intravenous infusion of 5 mg/m² over 5 min in nine patients with cancer to obtain information regarding the mechanism of renal handling of melphalan. Four of the patients underwent bone marrow transplantation and also received an IV dose of 220 mg/m². Total melphalan clearance after the 5 mg/m² dose ranged from 66.0 to 272 ml/min per m²; the percentage of the dose excreted unchanged in urine, from 2.5% to 92.8%; renal clearance, from 4.1 to 188 ml/min per m²; the fraction unbound in plasma, from 0.0598 to 0.460; and t_1/2, from 39.4 to 84.3 min. Unbound melphalan clearance and renal clearance calculated from the unbound fraction in plasma for each patient ranged from 441 to 3356 ml/min per m² and 15 to 961 ml/min per m² respectively and were not related to serum albumin, serum creatinine or creatinine clearance. The percentage of the dose exctreted and melphalan renal clearance were not related to urine flow. There was evidence of active secretion of melphalan in the kidney an possible reabsorption. There were no significant paired differences in melphalan disposition between the high- and low-dose studies. Highly variable renal clearance involving active secretion may contribute in part to large interpatient differences in the total plasma clearance of melphalan in patients with cancer.This study was supported by a grant from The Queen Elizabeth Hospital Research Foundation     

Concha bullosa and the nasal middle meatus obstructive syndrome

BACKGROUND: Concha bullosa (CB) is pneumatization of the middle turbinate and one of the most common anatomic variation of the sinonasal region. It is found in about 25% of the population. Middle meatus obstructive syndrome (MMOS) is, usually connected with CB. The main symptoms of this syndrome are headaches, impaired nasal breathing and hyposmia. Headache is the most common symptom and it may occur due to contact between a CB and other structures of the nasal cavity. CASE REPORT: We presented a case of 32 year-old-woman with headaches, located in the orbital and the left frontal region. The headaches were intermittent and corresponding to the nasal cycle. After neurologic and allergic examination, endoscopic nasal examination demonstrated a septal deviation to the right side and a large middle turbinate in the left side of the nasal cavity. Coronal computerized tomography (CT) of the paranasal sinuses demonstrated the septal deformation and pneumatization of the left middle turbinate. Diagnosis was confirmed by lidocaine test. In the functional endoscopic surgery (FESS), the lateral lamela of the anterior CB was removed. At the same time, the septoplasty was done. At the control examination, the patient was without symptoms. CONCLUSION: Although CB is the common anatomic variation of the nasal cavity, MMOS is rare. Headache (rhinogenic origin) is the most important symptom. Surgical treatment is the lateral resection of the CB in the FESS technique and the septoplasty.     

Vascular thoracic outlet syndrome

Abstract The surgical treatment of 30 cases of vascular thoracic outlet syndrome (TOS) in 25 patients is presented. Patients included 17 women and 8 men with average age of 26.1 years. The causes of compression were cervical rib (n = 16), soft tissue anomalies (n = 12), and scar tissue after clavicle fracture (n = 2). Ten subclavian artery aneurysms containing intraluminal thrombus as well as one subclavian artery occlusion were found. All such cases had multiple distal arterial embolization. Presenting features of cases with arterial TOS included: hand ischemia (n = 11), transient ischemic attack (TIA) (n = 1), and claudication or vasomotor phenomena during the arm hyperabduction (n = 11). Two patients with venous TOS developed hand edema during arm hyperabduction, and five other patients had axillary-subclavian venous thrombosis. In all cases decompressive procedures using a combined supraclavicular and infraclavicular approach were performed. Decompression was achieved by cervical rib excision (n = 12), combined cervical and first rib excision (n = 4), and first rib excision (n = 14). In all cases division of all soft tissue elements was also accomplished. Associated vascular procedures included resection and replacement of 10 subclavian artery aneurysms, one subclavian-axillary and one axillary-brachial bypass, as well as nine brachial embolectomies. All five cases with axillary-subclavian vein thrombosis before decompression were treated with anticoagulant therapy. The mean follow-up period was 3 years and 2 months (range 1 to 6 years). Two pleural entry injuries and two transient brachial plexus injuries were noted. All reconstructed arteries were patent during the follow-up period. Complete resolution of symptoms with a return to full activity was noticed in all cases with arterial TOS and in two cases with venous TOS without axillary-subclavian vein thrombosis. In cases with axillary-subclavian vein thrombosis relief of symptoms was mild, and there were limitations on daily activity. Vascular TOS is seen less frequently than the neurogenic form; however, in most cases it requires surgical treatment. We prefer a combined supraclavicular and infraclavicular approach because it offers complete exposure of the subclavian artery, cervical and first ribs, and all soft tissue anomalies. Electronic Publication     

Repeated treatment of vestibular schwannomas after gamma knife radiosurgery

Purpose  When gamma knife radiosurgery (GKS) does not achieve control of the growth of a tumour, the need to repeat treatment is considered. The results and risks of repeat treatment of patients with a vestibular schwannoma were reviewed to assess its efficacy and safety. Methods  Between 1992 and 2001, we treated 351 patients with a vestibular schwannoma by GKS, control of the growth of the tumour was not achieved in 32. 26 patients underwntrepeat GKS and five patients had an open microsurgical operation and one stereotactic aspiration of a tumour cyst. Results  Twenty-four of 26 patients were followed up after the repeat GKS for a median of 43 months. 15 tumours became smaller, seven remained unchanged and two enlarged. After the second GKS one patient’s hearing deteriorated, one developed facial weakness and three facial spasms. One patient required insertion of ventriculo-peritoneal drainage. An operation to radically resect the tumour was performed in five patients after the first GKS and for a subtotal removal in one after repeated GKS. Conclusions  In the small proportion of patients (9%) in whom initial GKS does not control the growth of a vestibular schwannoma, most can be controlled by further GKS with a very low risk of a complications.     

Iron status in patients with pyruvate kinase deficiency: neonatal hyperferritinaemia associated with a novel frameshift deletion in the PKLR gene (p.Arg518fs), and low hepcidin to ferritin ratios

Pyruvate kinase (PK) deficiency is an iron‐loading anaemia characterized by chronic haemolysis, ineffective erythropoiesis and a requirement for blood transfusion in most cases. We studied 11 patients from 10 unrelated families and found nine different disease‐causing PKLR mutations. Two of these mutations ‐ the point mutation c.878A>T (p.Asp293Val) and the frameshift deletion c.1553delG (p.(Arg518Leufs*12)) ‐ have not been previously described in the literature. This frameshift deletion was associated with an unusually severe phenotype involving neonatal hyperferritinaemia that is not typical of PK deficiency. No disease‐causing mutations in genes associated with haemochromatosis could be found. Inappropriately low levels of hepcidin with respect to iron loading were detected in all PK‐deficient patients with increased ferritin, confirming the predominant effect of accelerated erythropoiesis on hepcidin production. Although the levels of a putative hepcidin suppressor, growth differentiation factor‐15, were increased in PK‐deficient patients, no negative correlation with hepcidin was found. This result indicates the existence of another as‐yet unidentified erythroid regulator of hepcidin synthesis in PK deficiency.     

A model of the viscoelastic behavior of flowable resin composites prior to setting

Objective

The aim of this study is to develop fractional derivative models for the assessment of viscoelastic properties related to handling characteristics of dental resin composites belonging to two classes: flowable (Revolution Formula 2 and Filtek Ultimate) and posterior “bulk-fill” flowable base (Smart Dentin Replace).

Methods

Rheological measurements on all materials tested in this study were performed using dynamic oscillatory rheometer at temperature of 23 °C. A parallel plates module with a diameter of 20 mm was used to measure the properties of the resin composites tested. We developed two models to describe the obtained data: the generalized Newton model and the generalized Zener model (the so-called three parameter model). Both models contain fractional derivatives of Riemann–Liouville type. By determining the parameters of the model we were able to fit experimental data with high accuracy.

Results

Our results show that flowable “bulk-fill” resin-composite material (SDR) has distinct properties as compared to other two flowable resin composite materials (Revolution Formula 2 and Filtek Ultimate). Thus, previously found SDR properties as “bulk-fill” flowable base results in the fact that it is described by generalized Zener model (i.e., it has properties of solid like viscoelastic material).

Significance

Our model may be used to predict behavior of tested composites in different flow conditions. The SDR has initially small almost constant complex viscosity showing that it has good self-leveling property.