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References
Aarsen FK, Van Dongen HR, Paquier PF et al (2004) Long-term
sequelae in children after cerebellar astrocytoma surgery. Neurology
62(8):1311–1316
Adamaszek M, D’Agata F, Ferrucci R et al (2017) Consensus paper:
cerebellum and emotion. Cerebellum 16(2):552–576
Ahmadian N, van Baarsen K, van Zandvoort M, Robe PA (2019)
The cerebellar cognitive affective syndrome—a meta-analysis.
Cerebellum 18(5):941–950
Albazron FM, Bruss J, Jones RM etal (2019) Pediatric postoperative
cerebellar cognitive affective syndrome follows outow pathway
lesions. Neurology 93(16):e1561–e1571
Alexander MP, Gillingham S, Schweizer T et al (2012) Cognitive
impairments due to focal cerebellar injuries in adults. Cortex
48(8):980–990
Allin M, Matsumoto H, Santhouse AM et al (2001) Cognitive and
motor function and the size of the cerebellum in adolescents born
very pre-term. Brain 124(Pt 1):60–66
Annoni JM, Ptak R, Caldara-Schnetzer AS etal (2003) Decoupling of
autonomic and cognitive emotional reactions after cerebellar stroke.
Ann Neurol 53:654–658
Argyropoulos GPD, van Dun K, Adamaszek M etal (2020) The cer-
ebellar cognitive affective/Schmahmann syndrome: a task force
paper. Cerebellum 19(1):102–125
Berger A, Sadeh M, Tzur G etal (2005) Task switching after cerebellar
damage. Neuropsychology 19(3):362–370
Botez-Marquard T, Botez MI (1993) Cognitive behavior in heredode-
generative ataxias. Eur Neurol 33(5):351–357
Braga-Neto P, Pedroso JL, Alessi H etal (2011) Cerebellar cognitive
affective syndrome in Machado Joseph disease: core clinical features. Cerebellum 11:549–556
Buckner RL, Krienen FM, Castellanos A etal (2011) The organization
of the human cerebellum estimated by intrinsic functional connectivity. J Neurophysiol 106:2322–2345
Burk K, Bosch S, Globas C etal (2001) Executive dysfunction in spino-
cerebellar ataxia type 1. Eur Neurol 46(1):43–48
Catsman-Berrevoets CE, van Dongen HR, Zwetsloot CP (1992)
Transient loss of speech followed by dysarthria after removal of
posterior fossa tumour. Dev Med Child Neurol 34(12):1102–1109
Chheda M, Sherman J, Schmahmann JD (2002) Neurologic, psychiat-
ric and cognitive manifestations in cerebellar agenesis. Neurology
58(Suppl 3):356
Cooper FE, Grube M, Elsegood KJ etal (2010) The contribution of the
cerebellum to cognition in Spinocerebellar Ataxia Type 6. Behav
Neurol 23(1–2):3–15
D’Agata F, Caroppo P, Baudino B et al (2011) The recognition of
facial emotions in spinocerebellar ataxia patients. Cerebellum
10(3):600–610
Daly DD, Love JG (1958) Akinetic mutism. Neurology 8(3):238–242
Devos D, Schraen-Maschke S, Vuillaume I etal (2001) Clinical fea-
tures and genetic analysis of a new form of spinocerebellar ataxia.
Neurology 56(2):234–238
Dietze DD Jr, Mickle JP (1990) Cerebellar mutism after posterior fossa
surgery. Pediatr Neurosurg 16(1):25–31. discussion 31
Dimova PS, Bojinova VS, Milanov IG (2009) Transient mutism and
pathologic laughter in the course of cerebellitis. Pediatr Neurol
41(1):49–52
Exner C, Weniger G, Irle E (2004) Cerebellar lesions in the PICA but
not SCA territory impair cognition. Neurology 63(11):2132–2135
Gambardella A, Annesi G, Bono F etal (1998) CAG repeat length and
clinical features in three Italian families with spinocerebellar ataxia
type 2 (SCA2): early impairment of Wisconsin Card Sorting Test
and saccade velocity. J Neurol 245(10):647–652
Gardner RJ, Coleman LT, Mitchell LA etal (2001) Near-total absence
of the cerebellum. Neuropediatrics 32(2):62–68
Garrard P, Martin NH, Giunti P etal (2008) Cognitive and social cogni-
tive functioning in spinocerebellar ataxia: a preliminary characterization. J Neurol 255(3):398–405
Geschwind DH (1999) Focusing attention on cognitive impairment in
spinocerebellar ataxia. Arch Neurol 56(1):20–22
Gondim FA, Parks BJ, Cruz-Flores S (2001) “Fou rire prodromique” as
the presentation of pontine ischaemia secondary to vertebrobasilar
stenosis. J Neurol Neurosurg Psychiatry 71(6):802–804
Gorman MP (2010) Update on diagnosis, treatment, and prognosis
in opsoclonus-myoclonus-ataxia syndrome. Curr Opin Pediatr
22(6):745–750
Gottwald B, Wilde B, Mihajlovic Z etal (2004) Evidence for distinct
cognitive decits after focal cerebellar lesions. J Neurol Neurosurg
Psychiatry 75(11):1524–1531
Grill J, Viguier D, Kieffer V etal (2004) Critical risk factors for intel-
lectual impairment in children with posterior fossa tumors: the role
of cerebellar damage. J Neurosurg 101(2 Suppl):152–158
Gross-Tsur V, Ben-Bashat D, Shalev RS et al (2006) Evidence of a
developmental cerebello-cerebral disorder. Neuropsychologia
44(12):2569–2572
Gudrunardottir T, Morgan AT, Lux AL etal (2016) Consensus paper
on postoperative pediatric cerebellar mutism syndrome: the Iceland
Delphi results. Childs Nerv Syst 32(7):1195–1203
Guell X, Gabrieli JDE, Schmahmann JD (2018) Triple representa-
tion of language, working memory, social and emotion processing
in the cerebellum: convergent evidence from task and seed-based
resting-state fMRI analyses in a single large cohort. Neuroimage
15;172:437–449
Guell X, Hoche F, Schmahmann JD (2015) Metalinguistic decits in
patients with cerebellar dysfunction: empirical support for the dysmetria of thought theory. Cerebellum 14(1):50–58
Heilman KM, Leon SA, Burtis DB et al (2014) Affective communi-
cation decits associated with cerebellar degeneration. Neurocase
20(1):18–26
Herman-Bert A, Stevanin G, Netter JC etal (2000) Mapping of spino-
cerebellar ataxia 13 to chromosome 19q13.3-q13.4in a family with
autosomal dominant cerebellar ataxia and mental retardation. Am J
Hum Genet 67(1):229–235
Hoche F, Guell X, Sherman JC, Vangel MG, Schmahmann JD
(2016) Cerebellar contribution to social cognition. Cerebellum
15(6):732–743
Hoche F, Guell X, Vangel MG, Sherman JC, Schmahmann JD (2018)
The cerebellar cognitive affective/Schmahmann syndrome scale.
Brain 141(1):248–270
Hoffmann M, Cases LB (2008) Etiology of frontal network syndromes
in isolated subtentorial stroke. Behav Neurol 20(3):101–105
Hoffmann M, Schmitt F (2004) Cognitive impairment in isolated sub-
tentorial stroke. Acta Neurol Scand 109(1):14–24
Hokkanen LS, Kauranen V, Roine RO etal (2006) Subtle cognitive de-
cits after cerebellar infarcts. Eur J Neurol 13(2):161–170
Horton LC, Frosch MP, Schmahmann JD (2011) Spinocerebellar ataxia
type 7 (SCA7): a 13-year longitudinal study of clinical course,
neuropathological features, and phenotype-genotype correlations.
In: 63rd annual meeting of the American Academy of Neurology,
Honolulu
Jawaid A, Rauf MA, Usman U et al (2008) Post-infarct cerebel-
lar cognitive affective syndrome: a case report. J Pak Med Assoc
58(7):415–417
Kalashnikova LA, Zueva YV, Pugacheva OV et al (2005) Cognitive
impairments in cerebellar infarcts. Neurosci Behav Physiol
35(8):773–779
Karatekin C, Lazareff JA, Asarnow RF (2000) Relevance of the cer-
ebellar hemispheres for executive functions. Pediatr Neurol
22(2):106–112
King M, Hernandez-Castillo CR, Poldrack RA etal (2019) Functional
boundaries in the human cerebellum revealed by a multi-domain
task battery. Nat Neurosci 22(8):1371–1378
75 The Cerebellar Cognitive Aective Syndrome andtheNeuropsychiatry oftheCerebellum
https://t.me/medicina_free
491
Kingma A, Mooij JJ, Metzemaekers JD etal (1994) Transient mutism
and speech disorders after posterior fossa surgery in children with
brain tumours. Acta Neurochir 131(1–2):74–79
Kirk EA, Howard VC, Scott CA (1995) Description of posterior fossa
syndrome in children after posterior fossa brain tumor surgery. J
Pediatr Oncol Nurs 12(4):181–187
Kish SJ, el-Awar M, Schut L etal (1988) Cognitive decits in olivopon-
tocerebellar atrophy: implications for the cholinergic hypothesis of
Alzheimer’s dementia. Ann Neurol 24(2):200–206
Koide R, Kobayashi S, Shimohata T etal (1999) A neurological dis-
ease caused by an expanded CAG trinucleotide repeat in the TATA-
binding protein gene: a new polyglutamine disease? Hum Mol
Genet 8(11):2047–2053
Kossorotoff M, Gonin-Flambois C, Gitiaux C etal (2010) A cognitive
and affective pattern in posterior fossa strokes in children: a case
series. Dev Med Child Neurol 52:626–631
Leggio MG, Silveri MC, Petrosini L etal (2000) Phonological grouping
is specically affected in cerebellar patients: a verbal uency study.
J Neurol Neurosurg Psychiatry 69(1):102–106
Levisohn L, Cronin-Golomb A, Schmahmann JD (2000)
Neuropsychological consequences of cerebellar tumour resection
in children: cerebellar cognitive affective syndrome in a paediatric
population. Brain 123(Pt 5):1041–1050
Lilja A, Hamalainen P, Kaitaranta E etal (2005) Cognitive impairment
in spinocerebellar ataxia type 8. J Neurol Sci 237(1–2):31–38
Limperopoulos C, Soul JS, Gauvreau K etal (2005) Late gestation cer-
ebellar growth is rapid and impeded by premature birth. Pediatrics
115(3):688–695
Limperopoulos C, Bassan H, Gauvreau K etal (2007) Does cerebel-
lar injury in premature infants contribute to the high prevalence of
long-term cognitive, learning, and behavioral disability in survi-
vors? Pediatrics 120(3):584–593
Malm J, Kristensen B, Karlsson T etal (1998) Cognitive impairment in
young adults with infratentorial infarcts. Neurology 51(2):433–440
Manes F, Villamil AR, Ameriso S etal (2009) “Real life” executive de-
cits in patients with focal vascular lesions affecting the cerebellum.
J Neurol Sci 283(1–2):95–98
Manto M, Mariën P (2015) Schmahmann’s syndrome—identication
of the third cornerstone of clinical ataxiology. Cerebellum Ataxias
2:2. https://doi.org/10.1186/s40673- 015- 0023- 1, eCollection 2015
Mantovan MC, Martinuzzi A, Squarzanti F etal (2006) Exploring men-
tal status in Friedreich’s ataxia: a combined neuropsychological,
behavioral and neuroimaging study. Eur J Neurol 13(8):827–835
Maryniak A, Roszkowski M (2005) Cognitive and affective distur-
bances in children after surgical treatment of cerebellar tumors.
Neurol Neurochir Pol 39(3):202–206
Nakamura K, Jeong SY, Uchihara T etal (2001) SCA17, a novel auto-
somal dominant cerebellar ataxia caused by an expanded polygluta-
mine in TATA-binding protein. Hum Mol Genet 10(14):1441–1448
Neau JP, Arroyo-Anllo E, Bonnaud V etal (2000) Neuropsychological
disturbances in cerebellar infarcts. Acta Neurol Scand
102(6):363–370
Orsi L, D’Agata F, Caroppo P et al (2011) Neuropsychological pic-
ture of 33 spinocerebellar ataxia cases. J Clin Exp Neuropsychol
33(3):315–325
Parvizi J, Anderson SW, Martin CO etal (2001) Pathological laughter
and crying: a link to the cerebellum. Brain 124(Pt 9):1708–1719
Parvizi J, Joseph J, Press DZ etal (2007) Pathological laughter and cry-
ing in patients with multiple system atrophy-cerebellar type. Mov
Disord 22(6):798–803
Pollack IF, Polinko P, Albright AL etal (1995) Mutism and pseudobul-
bar symptoms after resection of posterior fossa tumors in children:
incidence and pathophysiology. Neurosurgery 37(5):885–893
Ravizza SM, McCormick CA, Schlerf JE etal (2006) Cerebellar dam-
age produces selective decits in verbal working memory. Brain
129(Pt 2):306–320
Rekate HL, Grubb RL, Aram DM etal (1985) Muteness of cerebellar
origin. Arch Neurol 42(7):697–698
Richter S, Dimitrova A, Maschke M etal (2005) Degree of cerebellar
ataxia correlates with three-dimensional mri-based cerebellar volume in pure cerebellar degeneration. Eur Neurol 54(1):23–27
Richter S, Aslan B, Gerwig M etal (2007) Patients with chronic focal
cerebellar lesions show no cognitive abnormalities in a bedside test.
Neurocase 13(1):25–36
Riva D, Giorgi C (2000) The cerebellum contributes to higher func-
tions during development: evidence from a series of children
surgically treated for posterior fossa tumours. Brain 123(Pt 5):
1051–1061
Roeske S, Filla I, Heim S etal (2013) Progressive cognitive dysfunction
in spinocerebellar ataxia type 3. Mov Disord 28(10):1435–1438
Ronning C, Sundet K, Due-Tonnessen B etal (2005) Persistent cogni-
tive dysfunction secondary to cerebellar injury in patients treated
for posterior fossa tumors in childhood. Pediatr Neurosurg 41(1):
15–21
Sadeh M, Cohen I (2001) Transient loss of speech after removal of
posterior fossa tumors—one aspect of a larger neuropsychological
entity: the cerebellar cognitive affective syndrome. Pediatr Hematol
Oncol 18(7):423–426
Schmahmann JD (1991) An emerging concept. The cerebellar contribu-
tion to higher function. Arch Neurol 48(11):1178–1187
Schmahmann JD (1998) Dysmetria of thought. Clinical consequences
of cerebellar dysfunction on cognition and affect. Trends Cogn Sci
2:362–370
Schmahmann JD (2010) The role of the cerebellum in cognition
and emotion: personal reections since 1982 on the dysmetria of
thought hypothesis, and its historical evolution from theory to therapy. Neuropsychol Rev 20(3):236–260
Schmahmann JD (2020) Pediatric post-operative cerebellar mutism
syndrome, cerebellar cognitive affective syndrome, and posterior
fossa syndrome: historical review and proposed resolution to guide
future study. Childs Nerv Syst 36(6):1205–1214
Schmahmann JD, Pandya DN (1997) The cerebrocerebellar system. In:
Schmahmann JD (ed) The cerebellum and cognition, International
review of neurobiology, vol 41. Academic Press, San Diego,
pp31–60
Schmahmann JD, Sherman JC (1997) Cerebellar cognitive affective
syndrome. In: Schmahmann JD (ed) Cerebellum and cognition,
International review of neurobiology, vol 41. Academic Press, San
Diego, pp433–440
Schmahmann JD, Sherman JC (1998) The cerebellar cognitive affective
syndrome. Brain 121(Pt 4):561–579
Schmahmann JD, Ko R, MacMore J (2004) The human basis pon-
tis: motor syndromes and topographic organization. Brain 127(Pt
6):1269–1291
Schmahmann JD, Weilburg JB, Sherman JC (2007) The neuropsy-
chiatry of the cerebellum—insights from the clinic. Cerebellum
6(3):254–267
Schweizer TA, Alexander MP, Susan Gillingham BA et al (2010)
Lateralized cerebellar contributions to word generation: a phonemic
and semantic uency study. Behav Neurol 23(1–2):31–37
Sokolovsky N, Cook A, Hunt H etal (2010) A preliminary characterisa-
tion of cognition and social cognition in spinocerebellar ataxia types
2, 1, and 7. Behav Neurol 23(1–2):17–29
Steinlin M, Zangger B, Boltshauser E (1998) Non-progressive congeni-
tal ataxia with or without cerebellar hypoplasia: a review of 34 subjects. Dev Med Child Neurol 40(3):148–154
Steinlin M, Imfeld S, Zulauf P etal (2003) Neuropsychological long-
term sequelae after posterior fossa tumour resection during childhood. Brain 126(Pt 9):1998–2008
Stoodley CJ, Schmahmann JD (2009a) The cerebellum and language:
evidence from patients with cerebellar degeneration. Brain Lang
110(3):149–153
492
https://t.me/medicina_free
J. D. Schmahmann
Stoodley CJ, Schmahmann JD (2009b) Functional topography in
the human cerebellum: a meta-analysis of neuroimaging studies.
NeuroImage 44(2):489–501
Stoodley CJ, Valera EM, Schmahmann JD (2012) Functional topogra-
phy of the cerebellum for motor and cognitive tasks: an fMRI study.
NeuroImage 59(2):1560–1570
Storey E, Forrest SM, Shaw JH et al (1999) Spinocerebellar ataxia
type 2: clinical features of a pedigree displaying prominent frontal-
executive dysfunction. Arch Neurol 56(1):43–50
Suenaga M, Kawai Y, Watanabe H etal (2008) Cognitive impairment
in spinocerebellar ataxia type 6. J Neurol Neurosurg Psychiatry
79(5):496–499
Tavano A, Grasso R, Gagliardi C etal (2007) Disorders of cognitive and
affective development in cerebellar malformations. Brain 130(Pt
10):2646–2660
Tedesco AM, Chiricozzi FR, Clausi S etal (2011) The cerebellar cogni-
tive prole. Brain 134:3672–3686
Tei H, Sakamoto Y (1997) Pontine infarction due to basilar artery stenosis
presenting as pathological laughter. Neuroradiology 39(3):190–191
Turkel SB, Shu Chen L, Nelson MD etal (2004) Case series: acute
mood symptoms associated with posterior fossa lesions in children.
J Neuropsychiatry Clin Neurosci 16(4):443–445
Turkel SB, Brumm VL, Mitchell WG etal (2006) Mood and behavioral
dysfunction with opsoclonus-myoclonus ataxia. J Neuropsychiatry
Clin Neurosci 18(2):239–241
van Dongen HR, Catsman-Berrevoets CE, van Mourik M (1994)
The syndrome of ‘cerebellar’ mutism and subsequent dysarthria.
Neurology 44(11):2040–2046
Van Overwalle F, Manto M, Cattaneo Z etal (2020) Consensus paper:
cerebellum and social cognition. Cerebellum 19(6):833–868
Vaquero E, Gomez CM, Quintero EA et al (2008) Differential
prefrontal- like decit in children after cerebellar astrocytoma and
medulloblastoma tumor. Behav Brain Funct 4:18
Wassmer E, Davies P, Whitehouse WP etal (2003) Clinical spectrum
associated with cerebellar hypoplasia. Pediatr Neurol 28(5):347–351
White M, Lalonde R, Botez-Marquard T (2000) Neuropsychologic and
neuropsychiatric characteristics of patients with Friedreich’s ataxia.
Acta Neurol Scand 102(4):222–226
Wisoff JH, Epstein FJ (1984) Pseudobulbar palsy after posterior fossa
operation in children. Neurosurgery 15(5):707–709
Wollmann T, Barroso J, Monton F etal (2002) Neuropsychological
test performance of patients with Friedreich’s ataxia. J Clin Exp
Neuropsychol 24(5):677–686
Zawacki TM, Grace J, Friedman JH etal (2002) Executive and emo-
tional dysfunction in Machado-Joseph disease. Mov Disord
17(5):1004–1010
Ziemus B, Baumann O, Luerding R et al (2007) Impaired working-
memory after cerebellar infarcts paralleled by changes in
BOLD signal of a cortico-cerebellar circuit. Neuropsychologia
45(9):2016–2024
Ataxia Scales fortheClinical Evaluation
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KatrinBürk
76
Abstract
The International Cooperative Ataxia Rating Scale
(ICARS), the Friedreich Ataxia Rating Scale (FARS), the
Scale for the Assessment and Rating of Ataxia (SARA),
the Brief Ataxia Rating Scale (BARS), and the Unied
Multiple System Atrophy Rating Scale (UMSARS) repre-
sent validated scales for the assessment of disease sever-
ity and progression in cerebellar disorders. Each scale has
its strengths and weaknesses. Extensive scales are cer-
tainly useful for thorough documentation of specic fea-
tures of certain phenotypes, but this gain of information is
not always essential for the purpose of a study. Therefore,
compact and manageable scales like SARA are often pre-
ferred to more complex scales in observational and thera-
peutic studies, although, for instance, oculomotor
assessment is missing in SARA. The chapter describes
the components and psychometric properties of each
scale and presents examples of their clinical application.
Keywords
Ataxia · Scale · ICARS · FARS · SARA · BARS ·
UMSARS · Progression
76.1 Introduction
Several clinical scales have been validated for the assessment of disease severity and progression in cerebellar disease. There are several quality characteristics for clinical
scales: The interrater reliability covers the variation of the
ratings on the same subject between independent investiga-
K. Bürk (*)
Department of Neurology, Philipps University of Marburg,
Marburg, Germany
Swissmedic, Swiss Agency for Therapeutic Products,
Berne, Switzerland
e-mail: katrin.buerk@swissmedic.ch
tors while test–retest reliability describes the concordance
between the ratings of an individual rater at different time
points. The quality of interrater and test–retest reliability
are depicted as intraclass correlation coefcients (ICC) with
numerical values above >0.8 being considered reliable.
Internal consistency corresponds to the score consistency
across the various items of a scale: all items that are thought
to refer to the same theoretical construct should not differ
signicantly in their scoring. The quality of internal consistency is expressed as Cronbach’s α. It is assessed by com-
paring the scores referring to the same theoretical construct.
Numerical values of 0.8 or above correspond to a good internal consistency. Validity describes the extent to which a
scale actually measures what it claims to measure: Internal
construct validity of a scale is determined by a principal
component analysis that evaluates whether the number of
factors and the loadings of the variables measured on them
correspond to what is expected on the basis of the proposed
theoretical concept. Furthermore, the factor analysis
assesses the variability of observed variables with respect to
unobserved variables (the so-called ‘factors’) by investigating whether these factors mainly account for associated variations in a series of observed variables. Linearity of a scale
and particularly of the differences between ratings is essential to capture a wide spectrum of clinical severity. A scale
should also not have signicant oor (when data cannot take
a value lower than some particular number) or ceiling effects
(all scores are localized in the high end of the distribution).
Last, but not least, the acquisition of a scale should not be
complicated and time-consuming.
The most common clinical rating scales are the
International Cooperative Ataxia Rating Scale (ICARS),
the Friedreich Ataxia Rating Scale (FARS), the Scale for
the Assessment and Rating of Ataxia (SARA), the Brief
Ataxia Rating Scale (BARS), and the Unied Multiple
System Atrophy Rating Scale (UMSARS). Each scale has
its strength and weaknesses, and the selection of the clinical
scale to use depends on the respective clinical trial’s focus. In
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
D. L. Gruol et al. (eds.), Essentials of Cerebellum and Cerebellar Disorders, https://doi.org/10.1007/978-3-031-15070-8_76
493
494
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K. Bürk
the following, each scale will be shortly introduced and its
psychometric properties will be presented.
76.2 International Cooperative Ataxia
Rating Scale (ICARS)
ICARS hypothesizes that all of its 19 items are grouped into
four multi-item subscales reecting core cerebellar features
(see Table 76.1) (Trouillas et al. 1997). A maximum total
score of 100 indicates most severe symptoms. ICARS rating
takes about 20min (Schmitz-Hübsch etal. 2006b). Cano rst
established reliability and validity of total ICARS scores and
the subscale ‘posture and gait’ in FA (Cano etal. 2005).
Video-based ICARS rating in patients with spinocerebellar ataxia (SCA), FA and controls yielded high interrater reliability for ICARS sum and subscores as well as a good
test–retest (live vs. videotaped rating) reliability (Storey
etal. 2004). In a large cohort of SCA patients, ICARS interrater reliability, test–retest reliability and internal consistency were found to be good (Schmitz-Hübsch etal. 2006b).
Testing for validity (with ataxia disease stages or Barthel
indices serving as external criteria) yielded that several
ICARS items were redundant and overlapping. Furthermore,
rating scores appeared determined by four different factors
that did not coincide with ICARS subscales. Therefore,
ICARS subscore structure has to be questioned. Schoch evaluated reliability, criterion-related validity, and internal construct validity in focal cerebellar lesions and degenerative
ataxia (Schoch et al. 2007). Test–retest reliability for total
ICARS scores was good while it was highly variable for
ICARS subscores. Internal consistency for all 19 ICARS
items and criterion-related validity were good. However,
progression over time was only captured by subscore analysis in focal cerebellar lesions, but not in degenerative ataxia.
A cross-sectional analysis of internal consistency, factor
structure, and correlation with UPDRS-III scores in multiple
system atrophy (MSA), Parkinson’s Disease, and controls
(Tison etal. 2002; Goetz etal. 2008) questioned the applicability of ICARS in MSA due to interference of basal ganglia
symptoms. To date, ICARS has been applied in a number of
clinical and therapeutic trials (Ristori etal. 2010; Di Prospero
et al. 2007). In 2018, a Brazilian Portuguese version of
ICARS has been validated in SCA patients (Maggi et al.
2018).
76.3 Friedreich Ataxia Rating Scale (FARS)
The original FARS scale had been developed in a small
cohort of 14 FA patients in 2005 (Subramony etal. 2005).
The scale has four parts including a neurological examination, a disability staging, patient-reported activities of
daily living (ADL), and timed performance measures (25feet walk, 9-hole peg test, and PATA rate) (see Tables 76.2
and 76.3). In the initial validation study, ADL measures
and timed activities were signicantly correlated with the
FARS examination total scores and most of its subscores
(Subramony etal. 2005). Interrater reliability was excellent for the FARS neurological examination total and its
Table 76.1 International Cooperative Ataxia Rating Scale (ICARS) (Trouillas etal. 1997)
Total maximum score: 100
I. Posture and gait disturbances (maximum score 34)
1. Walking capacities (maximum score 8)
2. Gait speed (maximum score 4)
3. Standing capacities and eyes open (maximum score 6)
4. Spread of feet in natural position without support, eyes open (maximum score 4)
5. Body sway with feet together, eyes open (maximum score 4)
6. Body sway with feet together, eyes closed (maximum score 4)
7. Quality of sitting position (maximum score 4)
II. Kinetic functions (maximum score 52)
8. Knee-tibia test for each side (decomposition of movement and intention tremor) for each side (maximum score 4)
9. Action tremor in the heel-to-knee test for each side (maximum score 4)
10. Finger-to-nose test: decomposition and dysmetria for each side (maximum score 4)
11. Finger-to-nose test: intention tremor of the nger for each side (maximum score 4)
12. Finger–nger test (action tremor and/or instability) for each side (maximum score 4)
13. Pronation–supination alternating movements for each side (maximum score 4)
14. Drawing of the Archimedes’ spiral on a predrawn pattern r the dominant hand (maximum score 4)
III. Speech disorders (maximum score 8)
15. Dysarthria: uency of speech (maximum score 4)
16. Dysarthria: clarity of speech (maximum score 4)
IV. Oculomotor disorders (maximum score 6)
17. Gaze-evoked nystagmus (maximum score 3)
18. Abnormalities of the ocular pursuit (maximum score 2)
19. Dysmetria of the saccade (maximum score 1)
a
Each side of the body is assessed separately. The sum of both sides is added to the total score
a
a
a
a
a
a
a
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subsccores with the exception of bulbar and peripheral
scores that were less consistent between examiners. FARS
timed performance tests actually showed less rater bias
and good interrater reliability (Subramony etal. 2005). In
2006, two items related to stance without visual control
were added to the FARS examination protocol (FARSn).
FARSn has ve subscales A to E with a maximum score of
125 points indicating most severe symptomatology (see
Table 76.2) (Lynch et al. 2006). The administration of
FARSn takes about 30 min. To further improve FARS
Table 76.2 Friedreich
Ataxia Rating Scale (FARS).
A detailed description of the
two FARS examination
scales is depicted in
Table76.3
Part 1. FARS neurological examination
• Two versions FARSn (maximum score 125) and mFARS (maximum
score 93)
Part 2. FARS Disability Staging (FDS) (maximum score 5)
• 0 no
• 1 minimal
• 2 mild
• 3 moderate
• 4 severe
• 5 total disability
Part 3. Patient-reported activities of daily living (ADL) (maximum score 36)
• 9 items with a maximum score of 4 per item
Part 4. Timed performance measures
• 25-feet walk
• 9-hole peg test
• PATA rate
scale properties, the overall number of items of FARSn
was reduced by deleting the two bulbar items facial and
tongue atrophy (subscale A) as well as the complete subscore peripheral nervous system (subscale D) thereby creating the novel modied FARS (mFARS) with a maximum
total score of 93 points (Patel etal. 2016) (see Table76.3).
FARSn and mFARS scale properties have been compared
in a cross-sectional analysis of baseline data from
Friedreich Ataxia Clinical Outcome Measures (FACOMS) (clinicaltrials.gov NCT03090789), a registry of
Table 76.3 FARS part 1 neurological examination
FARSn (maximum total score 125) mFARS (maximum total score 93)
A: Bulbar (maximum score 11)
• A1 Facial atrophy (maximum score 3)
• A2 Tongue atrophy (maximum score 3)
A: Bulbar (maximum score 5)
• A3 cough (maximum score 2)
• A4 speech (maximum score 3)
• A3 cough (maximum score 2)
• A4 speech (maximum score 3)
B: Upper limb coordination (maximum score 36)
• B1 nger–nger (maximum score 3)
• B2 nose–nger (maximum score 3)
• B3 dysmetria (maximum score 4)
• B4 rapid movements (maximum score 3)
• B5 nger taps (maximum score 4)
C: Lower limb coordination (maximum score 16)
• C1 heel–shin slide (maximum score 4)
• C2 heel–shin tap (maximum score 4)
D: Peripheral nervous system (maximum score 26)
• D1 Muscle atrophy (maximum score 2)
• D2 Muscle weakness (maximum score 5)
• D3 Vibratory sense (maximum score 2)
• D4 Position sense (maximum score 2)
• D5 Deep tendon reexes (maximum score 2)
a
a
a
a
a
a
a
a
a
a
a
a
E: Upright stability (maximum score 36)
• E1 sitting position (maximum score 4)
• E2A stance, feet apart with eyes open (maximum score 4)
• E2B stance, feet apart with eyes closed (maximum score 4)
• E3A stance, feet together with eyes open (maximum score 4)
• E3B stance, feet together with eyes closed (maximum score 4)
• E4 tandem stance (maximum score 4)
• E5 stance dominant foot (maximum score 4)
• E6 tandem walk (maximum score 3)
• E7 gait (maximum score 5)
a
Each side is assessed separately. The sum of both sides is added to the total score
B: Upper limb coordination (maximum score 36)
• B1 nger–nger (maximum score 3)
• B2 nose–nger (maximum score 3)
• B3 dysmetria (maximum score 4)
• B4 rapid movements (maximum score 3)
• B5 nger taps (maximum score 4)
C: Lower limb coordination (maximum score 16)
• C1 heel–shin slide (maximum score 4)
• C2 heel–shin tap (maximum score 4)
E: Upright stability (maximum score 36)
• E1 sitting position (maximum score 4)
• E2A stance, feet apart with eyes open (maximum score 4)
• E2B stance, feet apart with eyes closed (maximum score 4)
• E3A stance, feet together with eyes open (maximum score 4)
• E3B stance, feet together with eyes closed (maximum score 4)
• E4 tandem stance (maximum score 4)
• E5 stance dominant foot (maximum score 4)
• E6 tandem walk (maximum score 3)
• E7 gait (maximum score 5)
a
a
a
a
a
a
a
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1011 FA patients representing a broad range of FA stages
(Rummey etal. 2019). Mean-corrected item-total correlations were 0.58 for subscale mA (mFARS), 0.67 for subscale B, 0.84 for subscale C, 0.60 for subscale E, and 0.40
for subscale A (FARSn) and 0.42 for D (FARSn). Internal
consistency and robustness against random errors for subscales B, C, and E were good (Cronbach’s α 0.87-0.91).
With respect to the bulbar subscale, the deletion of items
A1 and A2 improved Cronbach’s α moderately from 0.53
for A (FARSn) to 0.65 for mA (mFARS)). For both total
scales, FARSn and mFARS, Cronbach’s α was excellent
(0.92 both). Subscale intercorrelations were within 0.49
and 0.93 for both mFARS and FARSn. Validity of mFARS
was demonstrated by single- item- own subscale correlations (all coefcients >0.31). It is to note that lowest coefcients were found for items E3B (stance, feet together,
eyes closed), E4 (tandem stance), and E5 (stance on the
dominant foot) (Rummey etal. 2019). With respect to the
items deleted from the bulbar subscore in mFARS, weak
single-item-own subscale correlations were evident (A1
0.22, and A2 0.35). For subscale D, the correlation coefcients were highly variable (0.2–0.73) thereby questioning the underlying construct for the subscale peripheral
nervous system. There was evidence for ceiling effects in
subscales C and E (reecting non- of ambulatory patients).
The deletion of A1 and A2 from FARSn reduced ceiling
effects for subscale mA, but they are still apparent for
mFARS.Principal component analysis for mFARS yielded
three components with eigenvalues >1 and one additional
with an eigenvalue of 0.83. Factor analysis using four factors resulted in a separation of independent, clinically
meaningful factors. These four factors explained 70% of
the variance in the overall construct of mFARS thereby
supporting the four subscale structure of mFARS
(Rummey etal. 2019). Test–retest reliability was good to
excellent for mFARS total and subscales B, C, and E (ICC
all >0.95), while subscale A was less robust (ICC 0.38)
(Tai etal. 2021). Subscale and total scores for FARSn and
mFARS correlated with FDS and disease duration
(Rummey etal. 2019). With respect to the monitoring of
progression, FARS and mFARS scores increased by 2.11
and 1.91, respectively, in the rst year of follow-up.
Reproducibility of progression was found to improve with
increasing observational intervals (Patel et al. 2016).
FARSn has been successfully applied in large observational
studies such as FA-COMS or European Friedreich’s
Ataxia Consortium for Translational Studies (EFACTS)
(clinicaltrials.gov NCT02069509). Many clinical trials
have successfully used FARSn (Di Prospero etal. 2007;
Lynch etal. 2012). Meanwhile, mFARS has been approved
by the US Food and Drug Administration (FDA) as outcome measure in clinical trials.
76.4 Scale fortheAssessment andRating
ofAtaxia (SARA)
SARA has eight items with a total score of up to 40 points
(most severe ataxia) (Schmitz-Hübsch et al. 2006a)
(Table76.4). The time needed to complete varies between 4
and 40min (Schmitz-Hübsch etal. 2006a; Yabe etal. 2008).
SARA has primarily been validated in SCA (SchmitzHübsch etal. 2006a, 2010). In SCA, it did not show major
ceiling effects. Interrater variability was signicant and better than for ICARS with signicant ICCs for all single items.
Test–retest reliability and internal consistency were excellent (Cronbach’s α 0.94). Factorial analysis yielded a single
factor that determined all items and accounted for 80% of the
variance. So, in contrast to ICARS and FARS), SARA
appears to assess a common underlying construct—namely
ataxia. Linearity was demonstrated by the linear relation
between SARA total ratings and the differences between
individual ratings. When comparing SARA to ICARS scores,
‘disease stages,’ ‘Barthel indices,’ and part IV of the Unied
Huntington’s Disease Rating Scale (UHDRS-IV), scores
were found to be correlated to UHDRS-IV and Barthel indices and to increase with disease stages (Huntington-StudyGroup 1996; Schmitz-Hübsch et al. 2008a). A weak
correlation between SARA scores and disease duration was
attributed to differences in the individual progression rate of
various SCA genotypes. However, SARA does not reliably
capture all symptoms at onset and SARA does not assess
oculomotor movements. Concerning longitudinal assessment, SARA clearly displays decline after 12 months.
Standardized response means were good: A 2-arm trial that
aims to decrease progression by 50% would require a sample
size of 250 patients with various SCAs, 57 with SCA2, 70
with SCA1, and 75 with SCA3 per group (Schmitz-Hübsch
etal. 2008b; Tezenas du Montcel etal. 2012). SARA has also
been validated in a variety of cerebellar disorders including
sporadic ataxia, FA, MSA, focal cerebellar lesions and multiple sclerosis (Weyer et al. 2007; Yabe et al. 2008; Bürk
etal. 2009; Winser etal. 2017). In young children with early
onset ataxia, for SARA items gait/posture interrater reliabil-
Table 76.4 Scale for the Assessment and Rating of Ataxia (SARA)
Total maximum score: 40
• Gait (maximum score 8)
• Stance (maximum score 6)
• Sitting (maximum score 4)
• Speech disturbance (maximum score 6)
• Finger chase (maximum score 4)
• Nose–nger test (maximum score 4)
• Fast alternating hand movements (maximum score 4)
• Heel–shin slide (maximum score 4)
a
As rated independently for both sides; mean of both sides is considered
for total scores
a
a
a
a
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ity and external convergent validity were good. Due to the
inuence of myopathy on SARA gait/posture scores, discriminant validity was considered incomplete in this specic
cohort (Brandsma etal. 2017). SARA has also served as outcome measure in clinical trials and longitudinal observational registries such as the Prospective Study of individuals
at Risk for SCA1, SCA2, SCA3, SCA6, and SCA7 (RISCA)
(Clinical Trials.gov #NCT01037777).
76.5 Brief Ataxia Rating Scale (BARS)
The Brief Ataxia Rating Scale (BARS) was developed by a
multistep process starting with a supplemented version of
ICARS, the ‘Modied ICARS’ (MICARS). However, this
augmented complexity weakened not only the practicability
but also the quality of the novel scale. Five MICARS items
that correlated best with the overall MICARS scores (r 0.92–
0.952) and corresponded to the clinical domains of ataxia of
gait, upper and lower limbs, as well as speech and oculomotor dysfunction were nally selected to form the novel Brief
Ataxia Rating Scale (BARS) (Table 76.5). The time to
administer BARS is with 3–5min slightly shorter than for
SARA (Camargos etal. 2016). The maximum score of 30
points reects most severe ataxia. Internal consistency
(Cronbach’s α 0.86–0.90 as tested in three cohorts) and inter-
rater reliability (ICC 0.91) of BARS were excellent
(Schmahmann etal. 2009). In the original validation study,
though construct validity and interrater reliability were found
to be slightly superior for SARA as compared to BARS
(Cronbach’s α 0.89-0.92, ICC 0.93) despite the fact that
SARA scores had retrospectively been derived from
MICARS subscores (Schmahmann etal. 2009). Subsequent
validation of the Brazilian Portuguese BARS in 35 adult
patients with cerebellar disorders of various etiologies
yielded good correlation with SARA scores (r=0.91); internal consistency and interrater reliability measures for the
Brazilian Portuguese version were in the same range or even
superior to the original version (Cronbach’s α 0.972, ICC
0.94) (Camargos etal. 2016). Excellent external validity was
Table 76.5 Brief Ataxia Rating Scale (BARS)
Maximum score: 30
• Walking capacity (maximum score 8)
• Heel-to-shin test for decomposition of movement (maximum
• Finger-to-nose test for decomposition and dysmetria
• Dysarthria assessed by clarity of speech (maximum score 4)
• Abnormalities of the ocular pursuit (maximum score 2)
a
Each side of the body is assessed separately. The sum of both sides is
added to the total score
a
score 4)
(maximum score 4)
a
also established for BARS in SCA7 subjects by demonstrating signicant correlation of BARS and SARA scores
(r=0.9714, p<0.0001). BARS scores were also positively
correlated to CAG expansion size in SCA7 (Velázquez-Pérez
etal. 2015). In contrast to SARA, BARS considers oculomo-
tor features. While an early version of SARA had also
covered eye movement abnormalities, this item was deleted
during validation as it was found to impair the scale’s overall
validity (Schmitz-Hübsch etal. 2006a). The impact of the
oculomotor item will have to be assessed in larger, multicenter studies involving many investigators.
Compactness of BARS proposes its use especially in the
pediatric population. Overall, BARS scores signicantly correlate with SARA (r=0.68) and ICARS (r=0.77) scores in
healthy children (Brandsma etal. 2014). Intrarater and interrater reliability as well as test–retest reliability were also
substantial in the healthy pediatric population (Brandsma
etal. 2014). Interestingly, both were even superior in ataxic
children (Brandsma et al. 2017). The authors themselves
accounted the differences in ICCs to differing analytic methods between studies (Brandsma etal. 2017). An alternative
explanation though could correspond to the difculty of
judging movements as within or without the normal range
for the respective age cohort in the pediatric population. In a
multiple regression model, BARS scores were signicantly
predicted by the severity of the most prominent movement
syndrome in children with complex symptomatology with
no respect whether the most prominent movement disorder
corresponded to ataxia or not (Brandsma etal. 2017). Ataxia
scores measured on SARA, ICARS, and BARS were found
to be inversely correlated to age with 89% of healthy subjects younger than 16years achieving SARA, ICARS, and
BARS scores above 0 (Kuiper etal. 2018). These ndings
are likely to result from the late maturation of the developing
cerebellar motor system. Scores considered as normal in the
adult population on BARS cannot be expected prior to the
age of 11years (Brandsma etal. 2014).
In adults, BARS has been used to assess cerebellar ataxia
symptoms in primary orthostatic tremor patients and healthy
age-matched controls (Thompson etal. 2020). Interestingly,
the mean BARS score in the control group was 1.14 (SD
1.42, median 1). Healthy adults obtained scores above 0in
the categories walking capacity, nger-to-nose test, and ocu-
lomotor function (Thompson et al. 2020). In a population
based study on alcoholic cerebellar degeneration, there was
evidence for an association of BARS scores with years of
drinking, the amount of alcohol consumed, and binge drinking (Del Brutto etal. 2016). Interestingly, BARS scores were
not correlated to the extent of cerebellar atrophy. This suggests that BARS scores also capture sensory ataxia due to
alcohol-induced peripheral neuropathy.
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76.6 Unied Multiple System Atrophy
Rating Scale (UMSARS)
Multiple System Atrophy (MSA) is a rapidly progressive
neurodegenerative disorder characterized by Parkinsonian,
cerebellar, and autonomic features. The Unied Multiple
System Atrophy Rating Scale (UMSARS) represents a semiquantitative multidimensional measuring tool aimed to capture the various aspects of the highly polymorphic clinical
syndrome of MSA.This specic scale is based on the knowledge from earlier scales for akinetic and cerebellar syndromes such as the Hoehn and Yahr Scale (H&Y), the
Schwab and England Scale (Schwab and England 1969),
ICARS, and the Composite Autonomic Symptom Scale
(Wenning et al. 2004). UMSARS maximum total score of
points indicates most severe dysfunction. UMSARS has four
subscales (Table 76.6). Rating for all four subscales takes
about 15min (Palma etal. 2021). According to its primary
purpose, UMSARS was initially validated in MSA of the
cerebellar (MSA-C) as well as the striatonigral (MSA-P)
type (Wenning et al. 2004). Cronbach’s α coefcient as a
measure of internal consistency was 0.84 for UMSARS I and
0.90 for UMSARS II subscales. Further analyses also yielded
low to mostly high correlation of single UMSARS I items
with UMSARS I total subscores with the exception of the
item orthostatic symptoms. For UMSARS II, there was also
a moderate to high correlation of most single-item scores
with the total UMSARS II subscore with the exception of the
item ocular motor dysfunction (Wenning etal. 2004). Due to
its rather descriptive character, UMSARS III was not found
to correlate with scores on UMSARS I, II, and IV (Wenning
etal. 2004). Interrater reliability for the total subscale scores
was excellent with ICCs of 0.88 for UMSARS I and 0.93 for
UMSARS II (Wenning etal. 2004). On the single-item level,
interrater agreement for UMSARS I and II single items varied between substantial and excellent with the exception of
orthostatic hypotension (UMSARS I) and ocular motor dysfunction, increased tone, rapid alternating movements of
hands, and nger tapping (all UMSARS II) (Wenning etal.
2004). Intrarater reliability was found to be good for all, but
oculomotor items (Krismer et al. 2012). Criterion-related
validity was conrmed by testing UMSARS subscores across
a simple three-point severity scale and several quantitative
timed tests (Wenning etal. 2004). Discriminant validity as an
aspect of construct validity was assessed by comparing
UMSARS I and II scores to the mental dysfunction subscale
of the UPDRS while convergent validity was analyzed by
comparing UMSARS I, II, and IV scores to UPDRS, ICARS,
H&Y, and Schwab and England Scale (Wenning etal. 2004).
In a comparative study of UMSARS and ICARS in SCA3
subjects, UMSARS II was granted similar validity for the
assessment of SCA3 as for ICARS. The authors also dis-
Table 76.6 Unied Multiple System Atrophy Rating Scale (UMSARS)
UMSARS I Historical review: Patient reported functional disability
(12 items, maximum score 48):
Questions 1–8 referring to motor symptoms (8 items with a
maximum score of 4)
Questions 9–12 referring to autonomic symptoms (4 items with a
maximum score of 4):
1. Speech
2. Swallowing
3. Handwriting
4. Cutting food and handling utensils
5. Dressing
6. Hygiene
7. Walking
8. Falling
9. Orthostatic symptoms
10. Urinary function
11. Sexual function
12. Bowel function
UMSARS II Motor examination scale: Clinician assessed motor
impairment (14 items, maximum score 56)
1. Facial expression (maximum score 4)
2. Speech (maximum score 4)
3. Ocular motor dysfunction (maximum score 4)
4. Tremor at rest (in the most affected limb) (maximum score 4)
5. Action tremor (in the most affected limb) (maximum score 4)
6. Increased tone (in the most affected limb) (maximum score 4)
7. Rapid alternating movements of hands (in the most affected
limb) (maximum score 4)
8. Finger taps (in the most affected limb) (maximum score 4)
9. Leg agility (maximum score 4)
10. Heel–knee–shin test (maximum score 4)
11. Arising from chair (maximum score 4)
12. Posture (maximum score 4)
13. Body sway (maximum score 4)
14. Gait (maximum score 4)
UMSARS III Autonomic examination
1. Blood pressure and heart rate in the supine and standing
position
UMSARS IV Global disability scale (maximum score 5)
1. Completely independent
2. Not completely independent
3. More dependent
4. Very dependent
5. Totally dependent and helpless. Bedridden
a
a
a
a
a
a
a
cussed potential superiority of UMSARS in complex syndromes such as SCA3 since UMSARS also captures
extracerebellar features such as dystonia (D’Abreu et al.
2007). The change of UMSARS scores over time was sig-
nicantly correlated to the progression on other motor scales,
such as H&Y, Schwab and England Scale, or ICARS (Geser
etal. 2006). The annual increase varies from 3.1 to 6.7 for
UMSARS I and 3.5 to 9.6 for UMSARS II scores between
studies (Geser etal. 2005, 2006; May etal. 2007; Levin etal.
2019; Low et al. 2014, 2015; Wenning et al. 2013). The
annual difference in UMSARS I and II scores seemingly
receded in the second year follow-up of the prospective multicenter registry study European Multiple System Atrophy
Study Group (EMSA-SG) (Wenning etal. 2013). In another
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large prospective study, a similar mitigation was most evident in patients who had started with motor symptoms and/
or orthostatic hypotension (Foubert-Samier etal. 2020). An
alternative explanation to the apparent attenuation of disease
progression, however, could correspond to insufcient psychometric properties: Palma actually found evidence for
reduced sensitivity of UMSARS in more advanced MSA
stages pointing to ceiling effects (Palma etal. 2021). The
minimally important differences (MID) were estimated in a
cohort of MSA-P patients as 1.5 points for both, UMSARS I
and II, and 3.5 points for the total scale (Krismer etal. 2016).
It is, however, unknown so far whether these values also
apply for the cerebellar variant of MSA.In MSA, group size
estimates to detect a 30% reduction in annual UMSARS II
decline at 80% power vary from 129 (Wenning etal. 2013) to
203 subjects (May etal. 2007) per study group. In order to
optimize UMSARS scale properties, Palma analyzed all
UMSARS items with respect to their sensitivity to change.
The items orthostatic hypotension (UMSARS I), bowel
function, and tremor at rest (both UMSARS II) were found
less suitable to detect change while dressing and hygiene
(UMSARS I) and posture and gait (UMSARS II) were eligible due to their high standardized effects. Based on these
analyses, a modied UMSARS version composed of only 11
items from UMSARS I and II with a maximum score of 44
was proposed (Palma etal. 2021). It is of interest that most
UMSARS items related to cerebellar function were considered as meaningful due to their high standarized effects. On
the modied UMSARS version, the annual increase was
13% as compared to 11% on the original UMSARS.
However, this abbreviated UMSARS scale still awaits validation. UMSARS has been applied in clinical trials for MSA
(Low etal. 2014; Levin etal. 2019; Rascol etal. 2021). The
European Medicines Agency (EMA) as well as the US FDA
have ofcially acknowledged UMSARS as a valid outcome
measure in clinical trials for MSA.
Conict of Interest The author does not have any potential conict of
interest.
References
Brandsma R, Lawerman TF, Kuiper MJ et al (2017) Reliability and
discriminant validity of ataxia rating scales in early onset ataxia.
Dev Med Child Neurol 59(4):427–432. https://doi.org/10.1111/
dmcn.13291
Brandsma R, Spits AH, Kuiper MJ etal (2014) Ataxia rating scales
are age-dependent in healthy children. Dev Med Child Neurol
56(6):556–563. https://doi.org/10.1111/dmcn.12369
Bürk K, Mälzig U, Wolf S etal (2009) Comparison of three clinical rat-
ing scales in Friedreich ataxia (FRDA). Mov Disord 24(12):1779–
1784. https://doi.org/10.1002/mds.22660
Camargos S, Cardoso F, Maciel R etal (2016) Brief ataxia rating scale:
a reliable tool to rate ataxia in a short timeframe. Mov Disord Clin
Pract 3(6):621–623. https://doi.org/10.1002/mdc3.12364
Cano SJ, Hobart JC, Hart PE et al (2005) International Cooperative
Ataxia Rating Scale (ICARS): appropriate for studies of Friedreich’s
ataxia? Mov Disord 20(12):1585–1591. https://doi.org/10.1002/
mds.20651
D’Abreu A, Franca M Jr, Lopes-Cendes I et al (2007) The interna-
tional cooperative ataxia rating scale in Machado–Joseph disease.
Comparison with the unied multiple system atrophy rating scale.
Mov Disord 22:1976–1979
Del Brutto OH, Mera RM, Sullivan LJ etal (2016) Population-based
study of alcoholic cerebellar degeneration: the Atahualpa Project. J
Neurol Sci 367:356–360. https://doi.org/10.1016/j.jns.2016.06.051
Di Prospero NA, Baker A, Jeffries N etal (2007) Neurological effects
of high-dose idebenone in patients with Friedreich’s ataxia: a randomised, placebo-controlled trial. Lancet Neurol 6(10):878–886.
https://doi.org/10.1016/S1474- 4422(07)70220- X
Foubert-Samier A, Pavy-Le Traon A, Guillet F et al (2020) Disease
progression and prognostic factors in multiple system atrophy: a
prospective cohort study. Neurobiol Dis 139:104813. https://doi.
org/10.1016/j.nbd.2020.104813
Geser F, Seppi K, Stampfer-Kountchev M etal (2005) The European
Multiple System Atrophy-Study Group (EMSA-SG). J Neural
Transm 112:1677–1686
Geser F, Wenning GK, Seppi K et al (2006) Progression of multiple
system atrophy (MSA): a prospective natural history study by the
European MSA Study Group (EMSA SG). Mov Disord 21:179–186
Goetz CG, Tilley BC, Shaftman SR etal (2008) Movement Disorder
Society-sponsored revision of the Unied Parkinson’s Disease
Rating Scale (MDS-UPDRS): scale presentation and clinimetric testing results. Mov Disord 23(15):2129–2170. https://doi.
org/10.1002/mds.22340
Huntington-Study-Group (1996) Unied Huntington’s Disease Rating
Scale: reliability and consistency. Mov Disord 11(2):136–142.
https://doi.org/10.1002/mds.870110204
Krismer F, Seppi K, Tison F et al (2012) The Unied Multiple
System Atrophy Rating Scale: intrarater reliability. Mov Disord
27(13):1683–1685. https://doi.org/10.1002/mds.25181
Krismer F, Seppi K, Wenning GK et al (2016) Minimally clinically
important decline in the parkinsonian variant of multiple system
atrophy. Mov Disord 31(10):1577–1581. https://doi.org/10.1002/
mds.26743
Kuiper MJ, Brandsma R, Vrijenhoek L (2018) Physiological move-
ment disorder-like features during typical motor development.
Eur J Paediatr Neurol 22(4):595–601. https://doi.org/10.1016/j.
ejpn.2018.03.010
Levin J, Maaß S, Schuberth M et al (2019) Safety and ef-
cacy of epigallocatechin gallate in multiple system atrophy
(PROMESA): a randomised, double-blind, placebo-controlled
trial. Lancet Neurol 18(8):724–735. https://doi.org/10.1016/
S1474- 4422(19)30141- 3
Low PA, Reich SG, Jankovic J etal (2015) Natural history of multiple
system atrophy in the USA: a prospective cohort study. Lancet Neurol
14(7):710–719. https://doi.org/10.1016/S1474- 4422(15)00058- 7
Low PA, Robertson D, Gilman S etal (2014) Efcacy and safety of
rifampicin for multiple system atrophy: a randomised, double-blind,
placebo-controlled trial. Lancet Neurol 13(3):268–275. https://doi.
org/10.1016/S1474- 4422(13)70301- 6
Lynch DR, Farmer JM, Tsou AY etal (2006) Measuring Friedreich
ataxia: complementary features of examination and performance
measures. Neurology 66:1711–1716
Lynch DR, Willi SM, Wilson RB et al (2012) A0001 in Friedreich
ataxia: biochemical characterization and effects in a clinical trial.
Mov Disord 27(8):1026–1033. https://doi.org/10.1002/mds.25058
Maggi FA, Braga-Neto P, Chien HF etal (2018) Cross-cultural adapta-
tion and validation of the International Cooperative Ataxia Rating
Scale (ICARS) to Brazilian Portuguese. Arq Neuropsiquiatr
76(10):674–684. https://doi.org/10.1590/0004- 282X20180098
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