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Cerebellar mutism: Definitions, classification and grading of symptoms

Article  in  Child's Nervous System · September 2011

DOI: 10.1007/s00381-011-1509-7 · Source: PubMed

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Childs Nerv Syst
DOI 10.1007/s00381-011-1509-7
LETTER TO THE EDITOR
Cerebellar mutism: definitions, classification and grading
of symptoms
Thora Gudrunardottir & Astrid Sehested &
Marianne Juhler & Jacques Grill & Kjeld Schmiegelow
Received: 6 June 2011 / Accepted: 7 June 2011
# Springer-Verlag 2011
Dear editor,
We would like to thank Dr. Frassanito and Dr. Massimi
for their interesting and valuable comments on our
review article on cerebellar mutism, particularly in
sharing their own operative experience [1]. However,
their interpretation of the incidence of cerebellar mutism
syndrome, following surgery for cerebellar ependymoma
in von Hoff's article, seems to be based on a misunder-
standing regarding definitions of two different problems
related to the cerebellum in this article—“postoperative
cerebellar mutism” versus “postoperative cerebellar syn-
drome”. von Hoff et al. define the “cerebellar syndrome”
as a motoric triad of ataxia, nystagmus, and dysmetria,
grade it as mild, moderate, or severe as proposed by Riva
T. Gudrunardottir : A. Sehested : K. Schmiegelow (*)
Department of Pediatrics, University Hospital Rigshospitalet,
Copenhagen, Denmark
e-mail: kschmiegelow@rh.dk
M. Juhler
Department of Neurosurgery, University Hospital Rigshospitalet,
Copenhagen, Denmark
J. Grill
Department of Pediatric and Adolescent Oncology, Gustave
Roussy Institute,
Villejuif, France
K. Schmiegelow
Institute of Gynecology, Obstetrics and Pediatrics, the Faculty
of Medicine, University of Copenhagen,
Copenhagen, Denmark
et al. in 1991 [2] and refer to it as a general measure of
cerebellar damage. They do not relate it to the well-
recognized posterior fossa/cerebellar mutism syndrome as
such. Thus, a closer look at Table 1 reveals that the
incidence of postoperative cerebellar mutism was in fact 0
[3]. The same motoric cerebellar syndrome is described in
a group of patients as a measure of pre-operative clinical
status in another article by Callu et al from 2009. The
incidence of ataxia, nystagmus and dysmetria before
surgery was 31%, while the incidence of post-operative
mutism was 8% [4]. In both series, the telovelar approach
was used from year 2004 and onwards, but only in
patients whose tumor was located in the inferior part of
the vermis. This was recommended after recognizing the
role that splitting of the vermis plays in post-operative
intellectual impairment [5].
This discussion highlights the confusion that exists in
the literature regarding classification and definitions. We
have defined “cerebellar mutism” as muteness following
lesion of the cerebellum as opposed to the cerebrum or
the lower cranial nerves. It is further characterized by
delayed onset, limited duration, and (usually) long-term
linguistic sequelae [6]. A common synonym is “transient
cerebellar mutism” [7]. The “posterior fossa syndrome” is
a broad term that usually includes cerebellar mutism as its
main feature, but also encompasses motoric problems such
as ataxia, hypotonia, cranial nerve palsies together with
neurobehavioral abnormalities such as emotional lability,
poor oral intake, decreased spontaneous initiation of
movements, and impaired eye opening [8]. The “cerebellar
mutism syndrome” was initially used as a synonym for
isolated cerebellar mutism [9, 10], but has more recently in

Fig. 1 Schematic illustration of the relationship between the posterior
fossa syndrome, cerebellar mutism, and related syndromes. The
posterior fossa syndrome is made up of both linguistic, neuro-
behavioral, and motoric components. The cerebellar mutism syndrome
covers some of the most characteristic features of each category, while
the syndrome of mutism and subsequent dysarthria focuses on the
the largest prospective study to date been defined as the
constellation of mutism, ataxia, hypotonia, and irritability
[11]. It can be regarded as a term that covers easily
measurable highlights of the posterior fossa syndrome,
including both the neurobehavioral and motoric aspects, as
well as the mutism. The term “mutism and subsequent
dysarthria” has been used to specifically describe the
linguistic sequelae of cerebellar mutism [12]. Finally, there
is the “cerebellar syndrome” which describes ataxia,
dysmetria, and nystagmus, which were commonly
regarded as typical cerebellar signs long before the issue
of mutism and other aspects of the posterior fossa
syndrome came into play [2, 3]. They can usually be
observed both pre-and post-operatively, in contrast to the
other symptoms. Both the cerebellar syndrome [3] and the
cerebellar mutism syndrome [11] include ataxia in their
definition and have been graded as mild, moderate, or
severe in the literature, but they cannot be considered to be
one and the same and interpreted as such. Cerebellar
mutism is central to both the posterior fossa syndrome,
cerebellar mutism syndrome, and the syndrome of mutism
and subsequent dysarthria, but it is not part of the
cerebellar syndrome (Fig. 1). There are many more
overlapping terms in circulation, which underscores the
Childs Nerv Syst
linguistic part of the spectrum. At the core of these three post-
operative syndromes lies cerebellar mutism, also known as transient
cerebellar mutism. The cerebellar syndrome is a synonym for typical
motoric cerebellar signs that can be observed both pre- and post-
operatively. It does not include cerebellar mutism
need for a clear definition of the clinical picture that
cerebellar mutism is part of, and for standardized methods
of monitoring the acute and late sequelae of posterior fossa
surgery, that is now recognized to account for a large part
of the cognitive deficits encountered in this patient
population [13].
Not all patients with cerebellar mutism present with a
full-blown posterior fossa syndrome (Fig. 1 presents some
of the most commonly reported symptoms). Grading the
duration of mutism and degree of associated symptoms may
help in defining the true spectrum of this complication.
Indeed, the wide range in reported incidence of cerebellar
mutism is probably due to inhomogenous definitions and
variable appreciation of milder presentations of the poste-
rior fossa syndrome. Differences in the timing of patient
assessment may have a similar effect; thus, prospective
studies report a higher incidence than retrospective ones. In
their prospective study of the telovelar approach, Zaheer
and Wood report “posterior fossa syndrome symptoms” in 6
out of 20 patients—an incidence of 30%. However, only
two patients suffered from outright cerebellar mutism [14].
This illustrates how difficult it can be to compare different
surgical methods unless uniform measures are being used to
define the outcome.
Childs Nerv Syst
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