Original Paper
European
Neurology

Received: January 20. 1999
Accepted: August 5,1999

Eur Neurol 2000,43:47-49

Acute Brainstem Symptoms Associated
with Cervical Syringomyelia
Saskia H. Meves Thomas Postert Horst Przuntek Thomas BiJttner
Department of Neurology, St. Josef Hospital, Ruhr University of Bochum, Germany

Key Words
Syringomyelia • Brainstem symptoms
Pathomechanism

Abstract
Syringomyelia classically presents with slowly progressing dissociated sensory and upper and lower motor deficits. Atypical and acute manifestations have rarely been
described. We report here on 3 patients with syringomyelia, who had acute and atypical brainstem symptoms with regard to the underlying disease. These symptoms occurred after acute elevation of the intrathoracic
and intra-abdominal pressure, respectively, and remitted
subsequently. Vertebrobasiiar ischemia was initially suspected.
Copyright® 2000 S. Karger AG, Basel

Introduction

Syringomyelia. an abnormal cyst formation of the spinal cord, has hitherto been clinically defined as a chronic
progressive neurological disorder with typical motor and
sensory dysfunctions. During the last few years, attention
has been tbcussed on patients without these former characteristic features of syringomyelia. By means of MRI and

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CT, syringomyelia can be detected in earlier stages of the
disease, in patients with only small cyst formations and in
patients with mild or atypical symptoms [I]. Recent
reports also document acute and atypical presentations of
syringomyelia [2-5].
We report on 3 patients with acute brainstem symptoms which were attributable to a syringomyelia. These
manifestations were acutely caused or aggravated by the
same pathogenetic mechanism and were partially even
reproduced by another additional exposure to this specific
mechanism.
Case Reports
Patienl 1

A 73-year-old man came as an emergency patient because of sudden onset of rotatory vertigo, nystagmus with a rotatory component,
dysarthria and dysphagia. which lasted 4 h. After an initial rapid
remission of these symptoms, he experienced a second episode 24 h
later, which also resolved in the same time period. These symptoms
occurred initially after a severe cough attack. He had been suffering
from cervical syringomyelia for more than 30 years with typical
chronic progressive sensory and motor dysfunction. Cervical MRI
revealed a syrinx formation beginning at the level of the foramen
magnum and reaching downwards to Th2. MRI of the brain revealed
multiple small periventricuiar ischemic lesions of the medullary layer
without presentation of an acute ischemic lesion, espeeially in the
brainstem area, which could be related to the above-described symptoms. Furthermore, no syrinx formation was found in the brainstem.

Dr. S.H. Meves
Deparlment of Neurology. Si. Josef Hospital
Gudrunslrasse 5fi, D-44791 Bochum (Germany)
Tel. +49 2.14 5092022. Fax+49 2_14 5092414
E-Mail Saskia,M.Meves^jruhr-uni-hoehum.Je

Fig. 1. MRI of the cervical spine of patient
3. Syrinx formation from Cl to C3 (arrow),
a Sagittal section (T^^, technique), b Horizontal section (T:- technique).

EEG performed on dilTerent occasions was normal. The acoustic
evoked potentials could not be interpreted due to the patient's partial
deafness. In color-coded ultrasound, a right stenosis of the internal
carotid artery- of ?,0% was visualized, which was operated later on.
Three months later, he presented himsel!"again as an emergency case
after developing similar symptoms on getting out of bed. After another 24-hour initial remission, he developed an acute attack as he go!
out of bed. It took him an entire day to recover from this attack.
Patieiu 2
A 64-year-old man first came as an emergency patient because of
an acute worsening of a known slowly progressing gait disorder, He
showed signs of ataxia and horizontal nystagmus which had not been
previously described. In the elinieal examination and the electrophysiological studies, a polyneuropathy became apparent. Furthermore, on MRI. a syrinx formation was found in the cervical spinal
cord fromCl loC2. Isehemic lesions were not discovered in the cerebrum nor in the brainstem. EEG. extracranial color-coded duplexsonography and transcranial pw ultrasound were alt normal. One
year later, he eame again because of an acute exacerbation of liis
symptoms after bending down for a bowel movement. Before this
episode, he was able to walk using a cane. In this new situation, he
needed two helpers to stand up straight. It took him about a week to
recover from this episode.

Paticm i
A 72-year-old man was admitted to the hospital because of an
acute left nerve IX and XII palsy accompanied by severe pain in the
sensory dcrmatomes of C2 and C3. which appeared after sneezing.
Besides a disturbed temperature perception in theareaoftheC4dermatomeon the leit. no other neurological deticits were evident. MRI
revealed a not previously detected syrinx formation extending from
Cl to C3 {fig. 1). MRI of the brain was normal. EEG, extracranial
color-coded duplexsonography and acoustic and visual evoked potentials provided normal findings. EMG and ENG showed signs of a
diserete polyneuropathy, but the somatosensory evoked potentials
failed to demonstrate a central conduction disturbance. The symptoms improved over the next week. During the following year, he was
admitted twice to the hospital because of the reoccurrence of the
same symptoms after sneezing.

48

Eur Neurol 2000;43:47-49

Discussion
The pathogenetic origin and the progressive enlargement of syringomyelias have been discussed controversely in the literature. Gardner [6] assumed that the intracranial arterial pulse wave, transmitted by a gentle waterhammer effect, presses ventricular fluid through the central canal initiating a dissection and cyst formation. Williams [7] proposed that venous pressure changes cause an
obstruction of the normal draitiage of cerebrospinal fluid
through the spinal subarachnoid space leading to an
enlargement of a preexisting syrinx. Such pressure
changes are encountered during postural changes and Valsalva maneuvers. Hall et al. [8] supported Williams' theory that transmission of thoracic pressures to the subarachnoid space might possibly lead to a compression of the
syrinx and by that to an enlargement of the lesion. Oldfield et al. [9] suggested that coughing or sneezing could
explain the occasional progression of neurological deficits
in syringomyelia. The increase in intrathecal pressure
during coughing, sneezing or severe straining might affect
the cord surface. In our cases, the development of acute
symptoms was preceded by a sudden increase in intrathoracic and intra-abdoininal pressure during sneezing,
coughing and straining, respectively. In 1 patient, repetitive sneezing even caused identical paroxysmal symptoms. Exercise and Valsalva maneuvers can also lead to
acute presentations in syringomyelia [5].
Formerly, the diagnosis of syringomyelia was primarily based on its typical clinical presentations of varied
degrees of slowly progressive motor and sensor\' deficits.
The development of new diagnostic procedures such as
MRI necessitates a new approach to syringomyeiia. Especially patients with stnall cyst formations and without

Meves/Postert/Przuntek/Buttner

characteristic signs have been overlooked in previous
studies conceming the clinical features of syringomyelia
[1]. There have been more reports on acute symptoms,
which could even be the t1rst manifestation of syringomyelia, as in one of our cases. Furthermore, a collection of
atypical symptoms of syringomyelia has accumulated.
Reports exist on acute respirator)' failure [2], bladder disturbances [1,3] and brainstem symptoms [4], such as we
encountered in our patients. Typical symptoms, such as
dissociated sensory disturbances, can even be found missing in older patients with syringomyelia [10]. Due to the
relapsing course ofthe disease and perivcntricular hypodensities on MRl in elderly patients, the misleading diagnosis of eneephalomyclitis disseminata [10] or of recurring cerebrovascular ischemia, as in our patients, can be
made. Because of the age of our patients, we initially
assumed their having ischemia in the vertebrobasilar
region. In the ensuing diagnostic assessment of these
patients, no eerebrovascular ischemic event could be
related to their symptoms. However, we cannot definitely
exclude the existence of microlacunes due to small vessel
disease, which could not been demonstrated on MRl.
Nevertheless, the cause of their symptoms was seen in the
cervical syringomyelia mimicking vertebrobasilar ischemia.
Even though MRl faeilitates the diagnosis of syringomyelia, syringobulbia might still be ditTieult to be detected on MRl [4] and could mimick other diseases. Anwer and Fisher [4] presented 3 cases with symptoms ofthe
brainstem, whieh they explained by an extension of a eervieal syrinx which would not be revealed by MRl. Zagcr

et al. [5] related acute brain symptoms in syringomyelia to
an isehemie or mechanical distortion ofthe brainstem by
means of an acute expansion of an existing cervieal
syrinx. In our 3 patients with cervieal syringomyelia,
acute presentations manifested after Valsalva maneuvers
and time-limited affection ofthe brainstem due to extension ofthe syrinx was assumed. Low flow ischemic events
in relation to Valsalva maneuvers must also be eonsidered
as a potential cause of the symptoms, but one would
expeet a more general reaction of the brain than those
localized symptoms. An Arnold-Chiari malformation,
which eould also have produced the acute deficits, was
missing in all 3 patients. Symptoms in these patients
remained transient as the edema, caused by mechanical
compression, receded. A surgical intervention was refrained from because of the only transient worsening of
the symptoms and the only slowly progressive courses of
the disease.
Even though syringomyelia has been known for years
for its variability in presentation due to its location and
extension, new aspects must be considered regarding its
clinical presentations. New diagnostic procedures enable
a mueh higher degree of insight. Cases with acute presentations also allow a better view of the pathomeehanic origin of syringomyelias. On the other hand, syringomyelia
may be the cause of acute neurological symptoms referring to the brainstem. which could be misdiagnosed as
vertebrobasilar stroke. Acute brainstem symptoms should
therefore take syringomyelia as a differential diagnosis
into account, and MRl should include the upper cervical
myelon to exclude a cervical syrinx.

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Eur Neurol 2000;43:47-49

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