The treatment of ALS

Amyotrophic lateral sclerosis (ALS) is a progressive

degenerative neurological disorder.
Both upper and lower motor neurons are in regression
which causes uncontrolled movements, stiff muscles and
muscle weakness.
- The world-wide incidence ALS is around 12/100.00 in
most populations.
- The survival time after diagnose is 2-4 years for 8090% of the patients.
- Currently there is no truly effective medicine available

Riluzole:
A cause of ALS may be an abnormal glutamate metabolism, a drug that modulates
the glutamatergic system is Riluzole (2-amino-6-(trifluoromethoxy)benzothiazole),
and is thereby a treatment for neurological diseases. The favorable effect of Riluzole
on survival time seems to depend on the site of onset of disease.

Percutaneous endoscopic
gastrostomy
A low body mass index (BMI) is
common and a significant problem at
patients diagnosed with ALS. Weight
loss is a significant negative
prognostic factor to survival, caused
by swallowing difficulties in ALS.
To maintain body weight, a placement
of a percutaneous endoscopic
gastrostomy (PEG) tube is the most
effective intervention. The great
benefit of this adjustment is that
patients who cannot swallow food still
receive their nutrition and fluids. Due
to the PEG tube the survival time of
patients will be extended.

Non-invasive ventilation
A frequently occurred problem at ALS
patients is hypercapnia. Hypercapnia is a
high level of carbon dioxide in blood.
Research proved that hypercapnia
significantly reduced the survival of ALS
patients. Fortunately there is a solution for
respiratory problems, non-invasive
ventilation (NIV). NIV can prolong the
survival of patients diagnosed with ALS.

G. Bensimon, L. Lacomblez, V. Meininger, and the ALS/Riluzole Study Group, A Controlled Trial of Riluzole in Amyotrophic Lateral Sclerosis, The New England Journal of Medicine, March 1994
Zoccolella S, Beghi E, Palagano G, Fraddosio A, Guerra V, Samarelli V, Lepore V, Simone IL, Lamberti P, Serlenga L, Logroscino G; SLAP registry. Riluzole and amyotrophic lateral sclerosis survival: a population-based study in southern Italy. European Journal of neurology,
March 2007
Jeffrey Rosenfeld, Amy Ellis, Nutrition and Dietary Supplements in Motor Neuron Disease, Physical Medicine and Rehabilitation Clinics of North America, August 2008
Desport JC, Preux PM, Truong TC, Vallat JM, Sautereau D, Couratier P, Nutritional status is a prognostic factor for survival in ALS patients. Neurology, September 1999
Ahmed RM, Mioshi E, Caga J, Shibata M, Zoing M, Bartley L, Piguet O, Hodges JR, Kiernan MC, Body mass index delineates ALS from FTD: implications for metabolic health. Journal of Neurology, September 2014
Wikipedia, search: hypercapnia (25 February 2015)
K.p Czudaj, S. Suchi, B. Schnhofer, Physiologische Atmungsparameter und der Effekt der nicht invasiven Beatmung (NIV) bei Patienten mit amyotropher Lateralsklerose (ALS), (Physiological parameters of breathing and the impact of non-invasive ventilation (NIV) on
patients with amyotrophic lateral sclerosis (ALS)), Pneumologie, 2009