The association between ALS and population density: A population based study.

We aimed to assess whether rural residence is associated with amyotrophic lateral sclerosis in the south-east of England using a population based register. Previous studies in different populations have produced contradictory findings. Residence defined by London borough or non-metropolitan district at time of diagnosis was recorded for each incident case in the South-East England ALS Register between 1995 and 2005. Each of the 26 boroughs or districts of the catchment area of the register was classified according to population density. Age- and sex-adjusted incidence of ALS was calculated for each region and the relationship with population density tested by linear regression, thereby controlling for the underlying population structure. We found that population density in region of residence at diagnosis explained 25% of the variance in ALS rates (r = 0.5, p < 0.01). Thus, in this cohort in the south-east of England, people with ALS were more likely to be resident in areas of high population density at diagnosis.

The sex ratio in amyotrophic lateral sclerosis: A population based study.

Replicable risk factors for ALS include increasing age, family history and being male. The male: female ratio has been reported as being between 1 and 3. We tested the hypothesis that the sex ratio changes with age in a population register covering the south-east of England. The sex ratio before and after the age of 51 years was compared using a Z-test for proportions. Kendall's tau was used to assess the relationship between age group and sex ratio using incidence and prevalence data. Publicly available data from Italian and Irish population registers were compared with results. There was a significant difference in the proportion of females with ALS between those in the younger group (30.11%) and those in the older group (43.66%) (p = 0.013). The adjusted male: female ratio dropped from 2.5 in the younger group to 1.4 in the older group using prevalence data (Kendall's tau = -0.73, p = 0.039). Similar ratios were found in the Italian but not the Irish registry. We concluded that sex ratios in ALS may change with age. Over-representation of younger patients in clinic registers may explain the variation in sex ratios between studies. Menopause may also play a role.

Replacement of mushroom cage gastrostomy tube using a modified technique to allow percutaneous replacement with an endoscopic tube in patients with amyotrophic lateral sclerosis.

Radiologic inserted gastrostomy (RIG) is the preferred method in our institution for enteral feeding in amyotrophic lateral sclerosis (ALS). Skin-level primary-placed mushroom cage gastrostomy tubes become tight with weight gain. We describe a minimally invasive radiologic technique for replacing mushroom gastrostomy tubes with endoscopic mushroom cage tubes in ALS. All patients with ALS who underwent replacement of a RIG tube were included. Patients were selected for a modified replacement when the tube length of the primary placed RIG tube was insufficient to allow like-for-like replacement. Replacement was performed under local anesthetic and fluoroscopic guidance according to a preset technique, with modification of an endoscopic mushroom cage gastrostomy tube to allow percutaneous placement. Assessment of the success, safety, and durability of the modified technique was undertaken. Over a 60-month period, 104 primary placement mushroom cage tubes in ALS were performed. A total of 20 (19.2%) of 104 patients had a replacement tube positioned, 10 (9.6%) of 104 with the modified technique (male n = 4, female n = 6, mean age 65.5 years, range 48-85 years). All tubes were successfully replaced using this modified technique, with two minor complications (superficial wound infection and minor hemorrhage). The mean length of time of tube durability was 158.5 days (range 6-471 days), with all but one patient dying with a functional tube in place. We have devised a modification to allow percutaneous replacement of mushroom cage gastrostomy feeding tubes with minimal compromise to ALS patients. This technique allows tube replacement under local anesthetic, without the need for sedation, an important consideration in ALS.

Mushroom-cage gastrostomy tube placement in patients with amyotrophic lateral sclerosis: a 5-year experience in 104 patients in a single institution.

To review our experience of placement of a mushroom-cage gastrostomy tube (Entristar, Tyco Healthcare, Mansfield, MA), using radiological guidance, in patients with amyotrophic lateral sclerosis (ALS). All procedures were performed under local anaesthesia without sedation. Complications were recorded as peri-procedural, early (<24 h), late (>24 h), major or minor. Deaths were recorded as related to the underlying ALS or secondary to radiological-inserted gastrostomy (RIG) placement. Replacement RIG tube rate was recorded. Over a 5-year period RIG tubes were placed in 104 patients with ALS (male n = 52, female n = 52), with a median age of 62 years (range 34-86 years). All procedures were technically successful. Of the RIG procedures, 21/104 (20.2%) were performed with respiratory support. The 30-day mortality rate was 7/104 (6.7%); no patient died as a result of the procedure. There were 23/104 (22.1%) complications overall; 20/104 (19.2%) were minor and 3/104 (2.9%) major, requiring surgery (n = 2) and radiological-guided abscess drainage (n = 1). A median interval between replacement RIG procedures in 20/104 (19.2%) patients was 141.5 days (range 43-537 days). A mushroom-cage RIG tube may be safely and effectively inserted in a 'one-step' radiological procedure and may replace endoscopic-inserted gastrostomy tubes in the nutritional management of ALS.

Geographical clustering of amyotrophic lateral sclerosis in South-East England: a population study.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a degenerative disease of motor neurons that causes progressive paralysis and eventually results in death from respiratory failure. Environmental factors that trigger ALS might result in a pattern of geographical clustering of cases. We tested this hypothesis using the South-East England ALS population register, which covers south-east London, Kent and parts of neighbouring counties. METHODS: The register's catchment area was divided into postcode districts and sectors. The expected rates of ALS (adjusted for age and sex) were compared with the observed rates using a standardised residuals method and the SaTScan programme. RESULTS: There were 406 cases of ALS identified in the catchment area during the study period. Four of the 126 postcode districts, all in Greater London, had residuals >2.5 SDs from the mean. Similarly, there were 15 postcode sectors (out of 420) that had residuals >1.96 SDs from the mean. Nine of these were in Greater London. SaTScan identified an area that had a 5.61-km radius in which the relative risk of ALS was 1.70 (p = 0.012). This area overlapped with the postcode districts and some of the sectors identified using the residuals method. CONCLUSIONS: These findings suggest an excess of ALS cases in some postcode districts in south-east England.

Management of respiration in MND/ALS patients: an evidence based review.

This systematic review comprises an objective appraisal of the evidence in regard to the management of respiration in patients with motor neuron disease (MND/ALS). Studies were identified through computerised searches of 32 databases. Internet searches of websites of drug companies and MND/ALS research web sites, 'snow balling' and hand searches were also employed to locate any unpublished study or other 'grey literature' on respiration and MND/ALS. Since management of MND/ALS involves a number of health professionals and care workers, searches were made across multiple disciplines. No time frame was imposed on the search in order to increase the probability of identifying all relevant studies, although there was a final limit of March 2005. Recommendations for patient and carer-based guidelines for the clinical management of respiration for MND/ALS patients are suggested on the basis of qualitative analyses of the available evidence. However, these recommendations are based on current evidence of best practice, which largely comprises observational research and clinical opinion. There is a clear need for further evidence, in particular randomised and non-randomised controlled trials on the effects of non-invasive ventilation and additional larger scale cohort studies on the issues of initial assessment of respiratory symptoms, and management and timing of interventions.

Survival of patients with ALS following institution of enteral feeding is related to pre-procedure oximetry: a retrospective review of 98 patients in a single centre.

A retrospective review was carried out on the influence of pre-procedure respiratory assessment on survival of patients with amyotrophic lateral sclerosis (ALS) requiring nutritional support with either a gastrostomy or a nasogastric feeding tube. Over a five-year period 98 patients (49 male, 49 female; median age 61 years, range 26-86 years) with ALS were referred for enteral feeding with either radiological inserted gastrostomy (RIG), percutaneous endoscopic gastrostomy (PEG) or nasogastric tube (NG). Case notes review was performed to record patient age, sex, pre-procedure respiratory assessment, method of enteral feeding and survival post-procedure. Kaplan-Meier survival curves were constructed for each group, with Cox regression analyses performed in order to establish the effect of each variable on outcome. Median survival (with 95% confidence intervals) following RIG, PEG and NG was 6.31 months (4.58-8.04 months), 7.13 months (4.81-9.45 months) and 0.95 months (0.00-2.77 months), respectively. The survival advantage between RIG and PEG was not statistically significant (p = 0.50), but for NG versus RIG and PEG groups combined, there was a significant difference (p = 0.03). For patients with normal overnight oximetry, median survival was 8.54 months (3.88-13.21 months), compared to 4.80 months (1.20-8.39 months) in the abnormal oximetry group (p = 0.03; relative risk 1.97). It is concluded that RIG and PEG are equivalent in terms of post-procedure survival. Abnormal oximetry prior to the procedure is a significant indicator of post-procedure survival.

Comparison of two percutaneous radiological gastrostomy tubes in the nutritional management of ALS patients.

Patient care and minimizing complications post gastrostomy have to date received little attention in ALS patients. We compare the complications associated with pigtail and mushroom type percutaneous radiological gastrostomy tubes in this patient group. Patients requiring PRG received either Wills-Oglesby or the skin level Entristar. Retrospective review of the clinical notes was performed capturing demographic data, peristomal infection, tube displacement, tube failure, nutritional status, site of disease onset, and survival. Thirty-five patients (Group 1) had the Wills-Oglesby tube of which 14 (40%) tubes required replacement. The Entristar tube was inserted in 29 patients (Group 2) where 8 (28%) required replacement (NS). The incidence of infection was significantly lower with the Entristar tube, (p<0.001). The mean time to tube removal in Group 2 was 223 days (SD 147; range 71-494 days) due to 'buried bumper syndrome'. We conclude that the Entristar skin level gastrostomy tube is associated with a reduction in peristomal infection, tube failure and blockage compared with the Wills-Oglesby tube.