However, a large 2015 study showed that PEG insertion is safe in people with advanced ALS and low vital capacities, as long as they are on NIV during the procedure.There is weak evidence that PEG tubes improve survival. PEG insertion is usually performed with the intent of improving quality of life by sustaining nutrition and medication intake. This reduces the risk of weight loss and dehydration, and can decrease anxiety from extended mealtimes and decreased oral food intake. End-of-life care
Palliative care, which relieves symptoms and improves quality of life without treating the underlying disease, should begin shortly after someone is diagnosed with ALS. Early discussion of end-of-life issues gives people with ALS time to reflect on their preferences for end-of-life care and can help avoid unwanted interventions or procedures. Once they have been fully informed about all aspects of various life-prolonging measures, they can fill out advance directives indicating their attitude toward noninvasive ventilation, invasive ventilation, and feeding tubes. Late in the disease course, difficulty speaking due to muscle weakness (dysarthria) and cognitive dysfunction may impair their ability to communicate their wishes regarding care. Continued failure to solicit the preferences of the person with ALS may lead to unplanned and potentially unwanted emergency interventions, such as invasive ventilation.
Kayser–Fleischer rings (KF rings) are dark rings that appear to encircle the cornea of the eye. They are due to copper deposition in the Descemets membrane as a result of particular liver diseases. They are named after German ophthalmologists Bernhard Kayser and Bruno Fleischer who first described them in 1902 and 1903. Initially thought to be due to the accumulation of silver, they were first demonstrated to contain copper in 1934. Presentation
The rings, which consist of copper deposits where the cornea meets the sclera, in Descemets membrane, first appear as a crescent at the top of the cornea. Eventually, a second crescent forms below, at the "six oclock position", and ultimately completely encircles the cornea. Associations
Kayser–Fleischer rings are a sign of Wilsons disease, which involves abnormal copper handling by the liver resulting in copper accumulation in the body and is characterised by abnormalities of the basal ganglia of the brain, liver cirrhosis, splenomegaly, involuntary movements, muscle rigidity, psychiatric disturbances, dystonia and dysphagia. The combination of neurological symptoms, a low blood ceruloplasmin level and KF rings is diagnostic of Wilsons disease.Other causes of KF rings are cholestasis (obstruction of the bile ducts), primary biliary cirrhosis and "cryptogenic" cirrhosis (cirrhosis in which no cause can be identified). Diagnosis
As Kayser–Fleischer rings do not cause any symptoms, it is common for them to be identified during investigations for other medical conditions. In certain situations, they are actively sought; in that case, the early stages may be detected by slit lamp examination before they become visible to the naked eye.