Some patients will increase the consistency of their saliva using the mentioned drugs, and their medical condition will even get worse. In these cases it is advised the added fluid supply, Acetylcystein (for example FluimucilÒ) and potentially inhalation as well as the suction of saliva.
In the US it was advised the transtympanic transaction of the Chorda tympani as an operative procedure. But the bilateral transaction cancels the sensation of taste in the fore two thirds of the tongue (28).
Further the irradiation of the parotid gland with 300-1000 cGy can reduce for several weeks the production of saliva by 50% (23).
Compulsive laughter and compulsive crying
The onsets like laughing, crying or grimacing, which can not be restrained willingly, is possible to eliminate using 0.6-1.5 g Levedopa (for example BrocadopaÒ) (33) or 50-75 mg Amitriptylin (for example SarotenÒ) (4,23). These autonomous phenomena can be explained with a lesion of dopaminergic neurons; accordingly the therapy - effect from Levedopa and Amitriptylin is understandable. Norris and his coworkers (19) have observed the amelioration with the help of Lithium.
Dysphagia and aspiration
Caused by dysfunction of the pharynx and dysmotility of the esophagus, the swallowing disorder, which leads to a frequent aspiration, is particularly encumbering. For the most part swallowing of fluids is first affected and later also hard food. Often even the reduced need in nutrients by immobilized patients is not covered because of this difficulty to ingest. The hard food should be transformed to puree or substituted by liquid and be rich in content.
Incapable to swallow, ALS patients are fed with the help of nasogastric alternatively nasoduodenal tube or parenteral central vein catheter. The gastrostomy tube feeding is less problematic and easier psychologically than parenteral application. Polyurethane feeding tubs (NutritubÒ) can be used several weeks. If using the thin hardly disturbing tube, the fluid homogeneous food is administered controlled with pumps, for example with FrentaÒ - feeding tub, liquid nourishment and powdered medications can be given through a large tub only six times per day. As food for the long-term nutrition is suited nutrient defined diets, so called formula-diets, for example NutrodripÒ-Standart or rich on dietary fiber tube feeding FresubinÒ plus. If a patient is bothered by the tube too much, percutaneous endoscopic gastrostomy can be arranged (34).
The literature describes different surgical procedures for the therapy of the Dyspahia: the Cricopharyngeal Myotomy for the relieving of the deglutition (11), the occlusion of the larynx against the chronic aspiration (15) and the esophagus incision for the nutrition (27). Newrick and Langton-Hewer criticize these procedures. We also do not implement them.
Dysarthria and anarthria
Dysarthria and anarthria are very serious phenomena in the disease’s flow. Possibly at an early stage should be started logopaedic therapy. As long articulatory musculature is not affected very much, it is possible to learn speech techniques, which should serve for a long understandability (5). In addition, this training supports mentally. It was given a try to narrow the velopharyngeal space with the help of the Teflon injection after which would be reduced “twang” of the ALS-patient (12). Specifically arranged micro computer systems (for example Gewa-apparats/ appliances, EPITECH, Hiddenhausen1
) and simple help in communication (blackboards for showing and so on) can provide limited help. All these resources should satisfy particular neuromuscular abilities, which is a very difficult goal to achieve in the case of the chronic progressive ALS.
Musculature adynamia, spasticity and Muscular cramps
The individually matched physiotherapy is helpful for giving a patient the feeling of physical achievement. Decreasing physical activity and immobilization raise paresis in consequence of the disease and lead to contractures and pain. Accordingly, a patient should be motivated to stay independent and active in his every day life as long as possible. Swimming is a very good exercise in the physiotherapy. Thereby limbs are given buoyancy with the help of a pneumatic tire.
The located myasthenia can be compensated with orthopaedic help like the Peroneus splints (25). The industry (for example Maddak Inc., which owns Servo Prax Ltd. D-4230 Wesel ) supplies many useful devices, for example a cutlery holder, button turnkey or a holder for playing cards. Or some of these devices can be produced at home with very simple instruments.
Only rarely Acetyl cholinesterase inhibitor (for example Pyridostigmin, Mestinon®) can help with achieving the increase of the musculature strength (17). Since the use of inhibitors leads to hypersalivation and diarrhea, using them is restricted.
Hiddenhausen is a town in Germany