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2014 ; 13
(2
): 248-68
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Consensus paper: management of degenerative cerebellar disorders
#MMPMID24222635
Ilg W
; Bastian AJ
; Boesch S
; Burciu RG
; Celnik P
; Claaßen J
; Feil K
; Kalla R
; Miyai I
; Nachbauer W
; Schöls L
; Strupp M
; Synofzik M
; Teufel J
; Timmann D
Cerebellum
2014[Apr]; 13
(2
): 248-68
PMID24222635
show ga
Treatment of motor symptoms of degenerative cerebellar ataxia remains difficult.
Yet there are recent developments that are likely to lead to significant
improvements in the future. Most desirable would be a causative treatment of the
underlying cerebellar disease. This is currently available only for a very small
subset of cerebellar ataxias with known metabolic dysfunction. However,
increasing knowledge of the pathophysiology of hereditary ataxia should lead to
an increasing number of medically sensible drug trials. In this paper, data from
recent drug trials in patients with recessive and dominant cerebellar ataxias
will be summarized. There is consensus that up to date, no medication has been
proven effective. Aminopyridines and acetazolamide are the only exception, which
are beneficial in patients with episodic ataxia type 2. Aminopyridines are also
effective in a subset of patients presenting with downbeat nystagmus. As such,
all authors agreed that the mainstays of treatment of degenerative cerebellar
ataxia are currently physiotherapy, occupational therapy, and speech therapy. For
many years, well-controlled rehabilitation studies in patients with cerebellar
ataxia were lacking. Data of recently published studies show that coordinative
training improves motor function in both adult and juvenile patients with
cerebellar degeneration. Given the well-known contribution of the cerebellum to
motor learning, possible mechanisms underlying improvement will be outlined.
There is consensus that evidence-based guidelines for the physiotherapy of
degenerative cerebellar ataxia need to be developed. Future developments in
physiotherapeutical interventions will be discussed including application of
non-invasive brain stimulation.