Congenital hydrocephalus in the northeast of Brazil: epidemiological aspects, prenatal diagnosis, and treatment

Purpose

Congenital hydrocephalus (CH) has higher proportions in developing countries such as Brazil, reaching rates of 3.16:1,000 newborns. Early diagnosis is essential and can be done during the second trimester of pregnancy, highlighting the importance of regular prenatal care. Our objective is to describe the epidemiological profile of children with CH in the state of Bahia.

Methods

Consecutive medical records of patients with CH, aged less than 2years and operated at a pediatric reference hospital between September 2009 and September 2012, were reviewed.

Results

One hundred twenty-one children underwent ventricular peritoneal shunt (VP shunt) due to CH. As for prenatal care, 38 % of pregnant women did it regularly. Males predominated with 56 % of cases with a median age of 3 months, and 68 % were from the countryside area. The most frequent clinical manifestations were bulging fontanelle (37 %) and increased head circumference (30 %). There were 13 (11 %) reports of complications associated with VP shunts. There were nine deaths (7 %), and respiratory complications were the main cause.

Conclusions

Public health measures are the key to increase mothers’ knowledge about the importance of regular prenatal monitoring. There was a predominance of male children, with a median age of 3 months, with the majority coming from the countryside.     

Intracranial atheromatous disease treatment with the Wingspan stent system: Evaluation of clinical,procedural outcome and restenosis rate in a single-center series of 21 consecutive patients with acute and mid-term results

Background

Intracranial atherosclerosis may be the underlying pathology in up to 15% of ischemic strokes, but may account for about 40% of strokes in some populations. After an ischemic event determined by intracranial atherosclerosis, patients have a 12% annual risk of stroke recurrence, mostly during the first year.

Objective

To evaluate procedural safety, clinical outcome and restenosis rate of Wingspan stent placement.

Methods

Twenty-one caucasoid patients were enrolled. Target patients were affected by high-grade, symptomatic, intracranial atherosclerotic lesions, were on antithrombotic therapy and at high stroke risk. All patients were treated with the Wingspan stent system.

Results

Technical success resulted 100%, with all target lesions being reduced to < 50%. No stroke or death were observed at 30.The mean percent of stenosis was reduced from a middle value of 84% to a middle value of 17% after stent placement. Medium follow-up was 19.5 months (range 6–36 months). No stroke or death occurred in any patient. None of the patients presented a <50% stent patency rate at follow-up.

Conclusions

The short-term results and follow up analysis provide evidence demonstrating the safety of the Wingspan system when used in high-risk patient population. Due to concerns regarding long-term stent patency and ischemic events occurrence emerged from clinical trials such as the SAMMPRIS, intracranial angioplasty and stent with the Wingspan system should be considered only for high risk patients in which it may be considered the only viable therapeutic option.     

Vitamin D deficiency in myotonic dystrophy type 1

Myotonic dystrophy type 1 (DM1) is a multisystemic disorder affecting, among others, the endocrine system, with derangement of steroid hormones functions. Vitamin D is a steroid recognized for its role in calcium homeostasis. In addition, vitamin D influences muscle metabolism by genomic and non-genomic actions, including stimulation of the insulin-like-growth-factor 1 (IGF1), a major regulator of muscle trophism. To verify the presence of vitamin D deficit in DM1 and its possible consequences, serum 25-hydroxyvitamin D (25(OH)D), calcium, parathormone (PTH), and IGF1 levels were measured in 32 DM1 patients and in 32 age-matched controls. Bone mineral density (BMD) and proximal muscle strength were also measured by DXA and a handheld dynamometer, respectively. In DM1 patients, 25(OH)D levels were reduced compared to controls, and a significant decrease of IGF1 was also found. 25(OH)D levels inversely correlated with CTG expansion size, while IGF1 levels and muscle strength directly correlated with levels of 25(OH)D lower than 20 and 10 ng/ml, respectively. A significantly higher percentage of DM1 patients presented hyperparathyroidism as compared to controls. Calcium levels and BMD were comparable between the two groups. Oral administration of cholecalciferol in 11 DM1 patients with severe vitamin D deficiency induced a normal increase of circulating 25(OH)D, ruling out defects in intestinal absorption or hepatic hydroxylation. DM1 patients show a reduction of circulating 25(OH)D, which correlates with genotype and may influence IGF1 levels and proximal muscle strength. Oral supplementation with vitamin D should be considered in DM1 and might mitigate muscle weakness.     

Asymmetry and freezing of gait in parkinsonian patients

It has been hypothesized that freezing of gait (FOG) in parkinsonian patients (PD) might be triggered by a breakdown in the normal symmetry of gait. In this study, we evaluated the relationship between asymmetry of gait and FOG and the effects of intensive treadmill treatment on asymmetry. We studied 30 patients with (FOG+) and 30 without (FOG?) freezing in “on” stage. Patients underwent a 4-week rehabilitation treatment using a treadmill with auditory and visual cues and were evaluated at enrolment and at the end of rehabilitation. Outcome measures were gait speed, stride length, asymmetry of gait, Six-minute walking test (6MWT), Unified Parkinson’s Disease Rating Scale (UPDRS) II–III, Berg Balance Scale, Timed Up and Go Test, comfortable-fast gait speeds, freezing of gait questionnaire (FOGQ). At enrolment, no differences in gait parameters were observed between the two groups, which differed only in UPDRS_II and BBS. Both FOG+ and FOG? patients spent more time on the left foot (time on left/time on right foot 1.37, p = 0.002, 1.18, p = 0.016, respectively). Rehabilitation determined a homogeneous improvement in both groups of patients for all variables except UPDRS_II and balance, for which a better improvement was observed in FOG+ patients. The improvement in FOGQ in FOG+ patients was significantly correlated to the improvement in asymmetry of gait (Spearman R = 0.46, p = 0.013). Our data support a direct involvement of the asymmetry of gait in the development of FOG in PD. Treadmill training is effective in improving gait and balance in PD FOG+ patients and this might be related to a reduction of asymmetric gait.     

Validation of a low-cost virtual reality system for training street-crossing. A comparative study in healthy,neglected and non-neglected stroke individuals

Unilateral spatial neglect is a common consequence of stroke that directly affects the performance of activities of daily living. This impairment is traditionally assessed with paper-and-pencil tests that can lack correspondence to real life and are easily compensated. Virtual reality can immerse patients in more ecological scenarios, thus providing therapists with new tools to assess and train the effects of this impairment in simulated real tasks. This paper presents the clinical validation and convergent validity of a low-cost virtual reality system for training street-crossing in stroke patients with and without neglect. The performance of neglect patients was significantly worse than the performance of non-neglect and healthy participants. In addition, several correlations between the scores in the system and in the traditional scales were detected.