Oromandibular dystonia and hormonal factors: twelve years follow‐up of a case report

Summary Oromandibular dystonia (OMD) is a focal neurological movement disorder characterized by involuntary sustained and often painful muscle contraction, usually producing repetitive movements or abnormal positions of the mouth, jaw and/or tongue. We report on a 30‐year‐old woman affected with OMD with a 12‐year follow‐up. Focal dystonia involved an involuntary activity of the lateral pterygoid muscles causing forceful jaw displacement in the maximal protrusive position. These episodes initially occurred during jaw function and increased up to an open‐lock with bilateral pre‐auricular pain. Dystonic spasms were absent during sleep and were reduced temporarily by sensory tricks. Treatment with botulinum toxin type A (BTX) was performed during three different sessions over a 1‐year period. Electromyographic‐guided BTX injections into the lateral pterygoid muscles were given with cannula electrodes. Botox reduced the involuntary activity of the muscles. Recurrence and exacerbation of dystonic symptoms occurred during the two pregnancies and completely disappeared immediately after both deliveries with prolonged symptom‐free periods. During the last 8 years, the patient had a slight relapse of symptoms during flu attacks, periods of stress and during menses. The temporal pattern of these symptoms indicates a possible relationship between OMD and hormonal factors.     

Hospital specific aspects predominantly determine primary failure of hemodialysis arteriovenous fistulas

BACKGROUND: Primary failure of the arteriovenous fistula (AVF) is a major problem affecting native hemodialysis access use. A multicenter guideline implementation program, Care Improvement by Multidisciplinary approach for Increase of Native vascular access Obtainment (CIMINO), was designed to identify modifiable and nonmodifiable factors involved in the early functionality of the AVF. METHODS: Physicians and dialysis staff in 11 centers in the Netherlands (N = 1092 prevalent vascular accesses) were strongly and repeatedly advised to adhere to current guidelines. It was advised to always perform a standard preoperative duplex examination and physicians were encouraged to attempt salvaging procedures for failing and failed fistulae. Specially appointed access nurses prospectively registered all created vascular accesses in an internet-linked database. Primary failure (PF) was defined as a complication of the AVF before the first successful cannulation for hemodialysis treatment. Modifiable and nonmodifiable factors were related to risk of primary failure using logistic regression models. We restricted the analyses to the first AVF of each patient that was placed during the observation period. RESULTS: Between May 2004 and May 2006, an AVF was created in 395 patients. Primary failure occurred in one third (131 cases). Factors related to an increased risk of primary failure were female gender (odds ratio (OR): 1.73, 95% confidence interval (CI): 1.01-2.94), renal replacement therapy prior to AVF placement (OR: 1.19 per year on RRT, CI: 1.05-1.34), diabetes mellitus (OR: 3.08, CI: 1.53-6.20), and AVF placement at the wrist (compared with elbow) (OR: 1.86, CI: 1.03-3.36). Primary failure rate among the participating centers varied from 8% to 50%. Compared to the two centers with the lowest primary failure rates, six centers had a significantly higher primary failure rate. Adjustment for risk factors and surgery-related factors did not materially change the center-related findings. CONCLUSION: In conclusion, we have identified location of AVF placement as a modifiable factor influencing primary failure risk. More importantly, this study shows that the probability of primary failure is strongly related to the center of access creation, suggesting an important role for the vascular surgeon's skills and decisions.     

Multigenetic lesions in infant acute leukaemias: correlations with ALL-1 gene status

The large majority of childhood B-precursor cell acute lymphoblastic leukaemia cases present IgH and TCRδ gene rearrangements. These rearrangements have been widely used as specific markers for monitoring minimal residual disease. However, their prognostic value still remains unclear. In order to determine whether IgH and TCRδ gene rearrangements have any influence on relapse and event-free survival (EFS), we analysed the clinical impact of these genetic characteristics in 51 B-precursor acute lymphoblastic leukaemia patients. 46/51 patients (90.2%) showed IgH gene rearrangements by Southern blot and/or polymerase chain reaction (PCR) analysis. No statistically significant associations were found between IgH gene rearrangement pattern and age, sex, WBC count, immunophenotype, risk factor, relapse or EFS. 27/41 patients (66%) showed Vδ₂Dδ₃ recombination by Southern blot and/or PCR analysis. At a median follow-up of 53 months the estimated 5-year EFS probability was 78 ± 3% for the whole group. The EFS probability among patients with a Vδ₂Dδ₃ recombination pattern in the TCRδ locus was 90 ± 3%, whereas for patients without Vδ₂Dδ₃ recombination was 39 ± 13% ( P  < 0.005).
IgH rearrangement patterns do not appear to influence relapse or EFS probability. However, TCRδ gene rearrangement patterns have a relevant impact on the relapse rate and the EFS probability. Patients with Vδ₂Dδ₃ recombination have better clinical outcome than patients without this recombination, independent of any other prognostic factors.     

Spinal arteriovenous fistula

A 62-year-old male suddenly developed a severe paraparesis after epidural anaesthesia. He recovered gradually over the next few months. He had an acute relapse one year later and a selective spinal angiography showed a dural T8 arteriovenous fistula with large draining veins. Intravascular embolisation of the fistula produced immediate and sustained clinical improvement. The mechanism commonly held responsible for neurological disturbances in spinal dural arteriovenous fistulas is cord hypoxia secondary to venous hypertension. The 20-ml of local anaesthetic solution injected into a narrow spinal canal with osteophytosis may have caused further venous engorgement, cord hypoxia and acute neurological deficit.     

Chronic uridine treatment reduces the level of [3H]spiperone-labelled dopamine receptors and enhances their turnover rate in striatum of young rats: relationship to dopamine-dependent behaviours

The effect was studied of chronic uridine treatment on the recovery of striatal D-2 dopamine (DA) receptors after their irreversible blockade by N-ethoxycarbonyl-2-ethoxy-1,2-dihydroquinoline (EEDQ) in young (40 days old) and adult (14 months old) male rats using [³H]spiperone as radioligand. Chronic uridine treatment (15 mg kg^-1 day^-1, i.p., 14 days) causes a reduction of [³H]spiperone binding sites in striatum of young rats. This treatment also produces an increase in the rate of recovery of striatal [³H]spiperone-labelled DA receptors in young, but not in adult rats. Catalepsy and exploratory locomotor activity, two behaviours associated with blockade versus activation of DA receptors, were evaluated in the same rats. The behavioural recovery from the EEDC^induced syndrome is more rapid in the young rats treated with uridine than in the saline-treated group. The behavioural recovery in old rats was not affected by chronic uridine treatment. Thus, in young rats the pyrimidine nucleoside uridine may modulate the steady state and the turnover rate of striatal D-2 DA receptors.