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The European Journal of Health Economics (2021) 22:1151–1166
The economic burden ofamyotrophic lateral sclerosis: asystematic
Received: 14 July 2020 / Accepted: 27 May 2021 / Published online: 18 June 2021
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2021
Objectives This systematic review aimed to comprehensively collect and summarise the current body of knowledge regard-
ing the cost-of-illness of amyotrophic lateral sclerosis, to identify cost-driving factors of the disease and to consider the
development of costs over the course of disease. Further, the review sought to assess the methodological quality of the
Methods A systematic review was performed using the databases MEDLINE, Embase, Cochrane Library and PsycINFO.
Studies examining the economic burden of amyotrophic lateral sclerosis on a patient or national level written in English or
German published from the year 2001 onwards were included. Additional searches were conducted. Study characteristics
and results were extracted and compared.
Results In summary, 20 studies were included in this review. Most studies investigated costs per patient, amounting to total
costs between €9741€ to €114,605. Six studies conﬁrmed a rise in costs with disease progression, peaking close to the death
of a patient. National costs for amyotrophic lateral sclerosis varied between €149 million and €1329 million.
Conclusion Most of these studies suggest the economic burden of amyotrophic lateral sclerosis to be considerable. However,
further research is needed to establish a cost-eﬀective health policy in consideration of disease severities.
Keywords Amyotrophic lateral sclerosis· Motor neuron disease· Cost-of-illness· Economic burden· Systematic review·
Some years ago, a popular social media campaign raised
the awareness of amyotrophic lateral sclerosis (ALS) sig-
niﬁcantly. Millions of people got inspired to take part in
this social media phenomenon called ‘Ice Bucket Challenge’
by pouring a bucket of ice water on their heads. Besides
increasing awareness for this progressive neurodegenerative
disease another primary goal of the Ice Bucket Challenge
was to raise money for its research . To date, the disease is
incurable and will eventually lead to a patient’s death [2–4].
ALS is a rare disease with global incidence rates ranging
from 0.5 to 3.6 cases per 100,000 persons . The incidence
is increasing with advancing age, peaking at about 60 to
75years. The disease is very rare under 30years and almost
not prevalent in under 20years olds [5–7]. The mean age is
at 56 to 58years [2, 8, 9]. Due to a short survival period of
about 2 to 4years after diagnosis, the global prevalence of
the disease is quite low amounting from 1.1 to 8.2 cases per
100,000 persons . With a proportion of 1.5:1, the condi-
tion slightly aﬀects men more often than women though at a
declining rate [5, 6]. ALS is characterized by the simultane-
ous appearance of signs of lower and upper motor neuron
degeneration. With disease progression the patients typically
experience atrophies and paralyses which can either begin at
the upper or the lower extremities [2, 3, 11]. Furthermore,
in the remaining cases patients suﬀer from a bulbar course,
where they ﬁrst experience articulation problems and dys-
phagia . Regardless of where the symptoms ﬁrst occur,
in the terminal phase most patients suﬀer from a generalized
paralysis as well as a respiratory insuﬃciency [2, 9]. The
treatments commonly aim to attenuate the symptoms and
halt the deterioration of the disease. For many years, Riluzol
treatment has been the only widely approved drug therapy
with positive eﬀects to prolong the survival with ALS .
* K. Achtert
1 Institute forApplied Health Services Research (inav GmbH),