Growth rate of non‐vestibular intracranial schwannomas

Growth rate of non‐vestibular intracranial schwannomas A group of nine patients with non‐vestibular intracranial neuromas (four jugular, four facial, one trigeminal) underwent an interval scanning management policy, with serial annual magnetic resonance (MR) imaging. Tumour volume was assessed by manual measurement of the tumour area by MR imaging. Tumour volume was assessed by manual measurement of the tumour area on MR imaging axial cuts. The mean tumour size at presentation was 4.6 cm³ (range 0.7–17.8 cm³). During a mean follow‐up of 36 months (range 22–50 months), five out of nine tumours grew significantly at a rate of more than 5% of their initial volume per year. Only those tumours growing at a rate of more than 20% initial volume per year exhibited symptom progression. During a 36‐month period of interval scanning, just over 50% of non‐vestibular intracranial neuromas exhibited significant growth. Symptom progression was found to be a strong indicator of a high growth rate. This proportion exhibiting growth is higher than that demonstrated by unilateral sporadic vestibular schwannomas, but less than in patients with neurofibromatosis II. Early treatment of non‐vestibular intracranial neuromas should therefore be considered.     

Deterioration in quality‐of‐life of late (10‐year) survivors of head and neck cancer

Objectives: To determine 10-year quality-of-life (QOL) in head and neck cancer patients and to examine the potential predictors of late QOL. Design: Prospective 10-year (QOL) assessment in a cohort of head and neck cancer patients. Setting: Tertiary referral head and neck cancer centre in Auckland, New Zealand. Participants: Two hundred patients diagnosed and were treated for head and neck cancer. Exclusion criteria were blindness, learning difficulties or inability to understand or read English. Main outcome measures: Quality-of-life at 10 years measured by Auckland QOL questionnaire, and analysed for associations with the following co-variates: age, gender; co-morbidities (alcohol intake and smoking), type and stage of disease; treatment modality; and QOL measures. Results: At 10 years following diagnosis, overall QOL (life satisfaction), decreased significantly by an average of 11% (95% CI: −5, −17) compared with before treatment, and by 15% when compared with years 1 and 2. Pre-treatment QOL significantly predicted late QOL, whilst QOL 1 year after treatment did not. None of the socio-demographic, disease- or treatment-related factors predicted long-term QOL on univariate analysis, but this may be due to the small sample size. Conclusions: This observed, late drop in the QOL of head and neck cancer patients requires further corroboration and investigation. Due to small sample sizes associated with long-term studies in head and neck cancer cohorts, studies of predictors of long-term QOL will only be likely to succeed if done as multi-centre studies. As there is some evidence to suggest that psychosocial interventions improve the QOL of head and neck cancer patients, it may be appropriate to consider screening for risk of a late deterioration in QOL in order to plan appropriate psycho-social intervention.     

Unusual case of spontaneous discharge of pus (infected cyst) through the dorsum of the tongue

Neck swellings are often a result of infections that spread from the teeth. This case highlights an unusual presentation of spontaneous pustular discharge from the anterior dorsum of the tongue.     

Possible antiosteoporotic mechanism of Cicer arietinum extract in ovariectomized rats

Objective: The present study aimed to throw the light on the anti-osteoprotic mechanism of Cicer arietinum extract (CAE) seeds against ovariectomized (OVX) rats. Methods: Seventy female rats were divided into two groups. The first group (14 rats/group) represented normal rats (Sham operated) while the second group (56 rats/group) underwent bilateral ovariectomy (OVX). After one week of recovery from ovariectomy surgery, the second group was randomly subdivided into 4 subgroups (14 rats/ each subgroup). The rats administered orally; distilled water (vehicle) (1^st subgroup), Cicer arietinum extract (CAE) (500 or 1000 mg/kg body weight/day) (2^nd and 3^rd subgroups), alendronate (6.5 mg/kg mg/kg body weight) as a positive control one time/week (4^rh subgroup), daily for 10 weeks. Results: The present study demonstrated that ovariectomy caused significant decrease in bone mineral; density (BMD) and content (BMC), Bone-specific alkaline phosphatase (BALP), calcium (Ca), phosphorus (P), parathyroid hormone (PTH) and calcitonin levels. Furthermore, ovariectomy induced significant elevation of tartrate-resistant acid phosphatase 5b (TRAP 5b) and receptor activator of nuclear factor (NF-kappa β) ligand (RANKL) concentration. Conversely, osteoprotegerin (OPG) and OPG/RANKL ratio were decreased following ovariectomy. The present work suggests that CAE has antiosteoporotic action against ovariectomy effects and its activity may results from its phytochemical and/or phytoestrogen contents. Conclusion: The ongoing study speculates that the CAE exerts its action through regulation of RANK/RANKL/OPG system. As, CAE not only promotes osteoblast differentiation, but also up-regulates OPG and downregulates RANKL secretion in osteoblasts, subsequently prevents bone loss and osteoporosis.     

From Normal Gait to Loss of Ambulation in 6 Months: a Novel Presentation of SCA17

Spinocerebellar ataxias are a group of rare and heterogeneous autosomal dominant disorders characterized by progressive ataxia and other features. Spinocerebellar ataxia 17 (SCA17) is one of the 32 subtypes described to date and is secondary to CAG/CAA repeat expansion in the gene coding for the TATA-box binding protein (TBP). SCA17 is clinically heterogeneous and typically presents with slowly evolving ataxia, dysarthria, dementia, depression, and other movement disorders such as chorea. More than 41 CAG/CAA repeats are considered diagnostic of SCA17, with more than 49 being associated with full penetrance. We report one patient presenting with isolated rapidly evolving ataxia who was found to have 44 CAG/CAA repeats in the TBP gene. This suggests that, while SCA17 typically slowly progresses over years, its repertoire of presentations should be expanded to include rapidly progressive isolated ataxia resembling paraneoplastic disorders or prion disease.     

Analysis of CYP2D6 genotype and response to tetrabenazine

Tetrabenazine is effective in the treatment of the chorea associated with Huntington disease and other hyperkinetic movement disorders. Following oral administration, tetrabenazine is hepatically transformed into 2 active metabolites that are CYP2D6 substrates. There are 4 CYP2D6 genotypes: poor metabolizers, intermediate metabolizers, extensive metabolizers, and ultrarapid metabolizers. CYP2D6 genotyping was performed on sequential subjects treated with tetrabenazine, but results were not known at the time of titration. Duration of titration to a stable dose, total daily dose, response rating scores, and adverse events were retrospectively collected and subsequently analyzed. Of 127 patients, the majority (n = 100) were categorized as extensive metabolizers, 14 as intermediate metabolizers, 11 as poor metabolizers, and 2 as ultrarapid metabolizers. Ultrarapid metabolizer patients needed a longer titration (8 vs 3.3, 4.4, and 3 weeks, respectively; P < .01) to achieve optimal benefit and required a higher average daily dose than the other patients, but this difference did not reach statistical significance. The treatment response was less robust in the intermediate metabolizer group when compared with the extensive metabolizer patients (P = .013), but there were no statistically significant differences between the various groups with regard to adverse effects. Our findings demonstrate that, aside from the need for a longer titration in the ultrarapid metabolizers, there are no distinguishing features of patients with various CYP2D6 genotypes, and therefore the current recommendation to systematically genotype all patients prescribed more than 50 mg/day of tetrabenazine should be reconsidered. © 2012 Movement Disorder Society     

Evaluation of the policy of secondary prevention against rheumatic fever among Egyptian children

Objective

We evaluated the effectiveness of long acting penicillin (LAP) as a 2-weekly regimen in winter and a 3-weekly regimen in summer for prevention of streptococcal colonization and also studied the common complaints of patients during the period of compliance for LAP administration. We also attempted to determine the incidence of relapses or recurrence of rheumatic fever (RF) after the onset of first episode of RF.

Patients & methods

210 rheumatic patients with good compliance to LAP (for at least one year) were included in the study. Demographic, clinical information, patients'' complaints and echocardiographic data of rheumatic patients were collected both retrospectively and prospectively. Anti-streptolysin O titre (ASOT) and throat swab culture were done at the end of the study (on Day 14 in the 2-weekly regimen and on Day 21 in the 3-weekly regimen).

Results

The age of onset of rheumatic fever was mostly between 5 and 15 years and the youngest patient was 2 years old. Subclinical carditis (SCC) was present in 79 (37%) of all the patients in the study population. Only 7 patients (3.3%) had a relapse within 2 years of the acute episode of RF. At the end of the study, ASOT was found to be high only in 11 patients (5.2%) and throat swab cultures were found negative in all patients

Conclusion

LAP regimen is fairly effective in eradicating streptococcal colonization. The incidence of relapse of RF within 2 years of the acute episode of RF is relatively low.