483

sions [2] or more severe spinal cord
lesions, which also interrupted connections between the brain stem and
the spinal cord neurons. In our patient there must have been some influence of supraspinal centres upon
spinal cord neurons, because the patient could still walk.
The use of a three-way stopcock
during emptying and refilling is
mandatory in the type of pump described. For safety reasons this pump
type should be filled by the surgeon
himself or under his supervision during surgery or by the attending
physician after surgery. Further, we
would advise an observation period
of at least 1 h after a baclofen bolus
or filling of a pump to reduce the
risk of missing an acute overdose.

References
1. Delhaas EM, Brouwers JRBJ (1991) Intrathecal baclofen overdose: report of
7 events in 5 patients and review of the
literature. Int J Clin Pharmacol Ther
Toxicol 29:274-280
2. Kofler M, Saltuari L, Schmutzhard E,
Berek K, Baumgartner H, Russegger L,
Aichner F (1992) Electrophysiological
findings in a case of severe intrathecal
baclofen overdose. Electroencephalogr
Clin Neurophysiol 83:83-86
3. Mtiller-Schwefe G, Penn RD (1989)
Physostigmine in the treatment of intrathecal baclofen overdose. J Neurosurg 71:273-275
4. Ashworth B (1964) Preliminary trial of
carisoprodol in multiple sclerosis. Practitioner 192:540-542
5. Sallerin-Caute B, Lazorthes Y, Monsarrat B, Cros J, Bastide R (1991) CSF baclofen levels after intrathecal administration in severe spasticity, Eur J Clin
Pharmacol 40:363-365
J. Dressnandt (g:~) - T. R. T611e
A. Konstanzer • B. Conrad
Department of Neurology,
Technical University, M6hlstrasse 28,
D-81675 Munich, Germany
Tel.: +49-89-41404660
Fax: +49-89-41404867
F. X. WeinzierI
Department of Neurosurgery,
Technical University, Munich, Germany

D. G. Nabavi
P. Zunker
T. Mumme
D. Georgiadis

Cheiro-oral syndrome
due to severe stenosis
of the middle cerebral artery
Received: 6 December 1995
Accepted: 26 January 1996

Sirs: The cheiro-oral syndrome
(COS), a pure sensory disorder restricted to the corner of the mouth
and the hand on the same side, has
been attributed exclusively to lesions
of the central somatosensory pathways [4, 9, 10, 15] visualized on
imaging studies [7] or at autopsy
[14]. We describe a patient with relapsing-remitting COS due to highgrade stenosis of the contralateral
middle cerebral artery (MCA) and
without any other abnormality of the
arterial system.
A 57-year-old woman was admitted to hospital with an 1-year history
of repeated paraesthesias at the right
corner of the mouth and the ulnar
site of the ipsilateral hand. During
the 2 months before admission, the
frequency of attacks increased from
once monthly to once weekly. She
had moderate arterial hypertension,
which was well controlled with an
angiotensin-converting enzyme inhibitor. There was no history of
smoking, hyperlipidaemia, diabetes
mellitus or any other cardiovascular
disease.
Neurological examination disclosed only a mild hypaesthetic area
at the right corner of the mouth and
in the right hand, while general examination was normal. After exclusion of intracranial bleeding by computed tomography of the brain, intravenous heparin was administered
with a target partial thromboplastin
time of 60-80 s. Four hours after initiation of anticoagulation, the sensory symptoms disappeared. Examination of the basal intracranial arteries by means of transcranial Doppler

sonography (TCD) revealed an extreme increase in blood flow velocity in the left MCA (mean flow
about 245 cm/s) together with lowfrequency components due to vessel
wall covibrations. The diagnosis of a
severe left-sided MCA stenosis was
made and later confirmed by magnetic resonance angiography and intra-arterial digital subtraction angiography (Fig. 1). High-resolution magnetic resonance imaging of the brain
6 days after admission, including
contrast-enhanced scans, did not disclose any pathological findings.
Electrocardiogram, transthoracic
echocardiography and coagulation
tests were normal. Oral anticoagulation with phenprocoumon (Marcumar) was initiated 8 days after admission, and heparin was discontinued when the international normalized ratio reached target values
(2.5-4.0).
Recent studies have suggested
that TCD monitoring for microembolic signals (MES) provides information on the anticoagulation efficacy [5, 13] and is potentially of
prognostic significance in patients
with extracranial occlusive disease
[12]. In our case, TCD monitoring
for MES was performed on admis-

Fig. ! Magnetic resonance angiography of
the basal brain-supplying arteries showed
> 70% narrowing (---~)of the proximal left
middle cerebral artery (MCA). Note that
all basal arteries appear symmetrical, thus
excluding an artificial loss of MCA flow
signal due to erroneous plane section

484

sion, 1, 2 and 4 days after initiation
o f intravenous heparin and 3 months
after discharge from hospital, when
the patient was stabilized on oral anticoagulants. A pulsed ultrasound
machine with a 2 M H z bigate probe
was used, able to harvest simultaneously echos from spatially separated
vessel portions (Pioneer 4040, EME,
Oberlingen, Germany). A proximal
(depth: 58 mm) and distal segment
(depth: 44 m m ) o f the left M C A
were simultaneously monitored for
45 min. Sample volume was kept
low (between 6 and 8 mm) throughout the examinations. All monitoring
sessions were recorded on DAT tapes
and evaluated by three independent
observers blinded to the patient's
clinical course. MES were detected
according to criteria described
elsewere [3]. In the initial examination (observer 1 : 4 MES; observer 2
and 3 : 3 MES) M E S were detected
only in the poststenotic M C A segment, but neither in the prestenotic
channel nor not in the following
examinations (all three observers in
agreement) while the patient was
free o f s y m p t o m s on anticoagulants.
The large somatotopic thalamic
and cortical representation of the
sensory fibres from the mouth and
hand together with the close proximity in the brain stem and the thalamic
nuclei [11] favour the simultaneous
occurrence o f sensory disturbances at
these sites. A higher hypoxic vulnerability o f these sensory fibres has
been assumed to explain this particular distribution o f symptoms [6],
which is also seen in the hyperventilation syndrome [2], caused by hypocapnia-induced vasoconstriction
leading to ischaemia [8]. In our case,
both the detection of MES distal to
the M C A stenosis while the patient
was symptomatic and their cessation
parallel to the regression of symptoms argue for an embolic rather
than a h a e m o d y n a m i c induction of
cerebral ischaemia, in accordance
with current concepts [1]. The cessation of MES after initiation of intravenous heparin, which has already
been reported by Siebler et al. [13],
further underpins this hypothesis.

Use of the bigate probe in our patient
identified the M C A stenosis as the
active embolic source, since emboli
of a more proximal origin (e.g. heart
or internal carotid artery) would have
been detected in both channels.
In conclusion, we have described
a patient with repetitive COS due to
severe M C A stenosis. T C D monitoring in this case provided information
o f potential clinical relevance.

References
1. Adams HP, Gross CE (1981) Embolism distal to the stenosis of the middle
cerebral artery. Stroke 12:228-229
2. Blau JN, Wiles CM (1983) Unilateral
somatic symptoms due to hyperventilation. BJM 286:1108
3. Consensus Committee of the ninth international cerebral hemodynamic
symposium (1995) Basic identification
criteria of microembolic signals. Stroke
26:1123
4 Garcin R, Lapresle J (1960) Deuxi~me
observation personelle de syndrome
sensitif de type thalamique et ~t topographie chairo-orale par 16sion localis6e du thalamus. Rev Neurol (Paris)
103:474--481
5. Georgiadis D, Hill M, Zunker P, St6gbauer F, Ringelstein EB (1994) Anticoagulation monitoring with transcranial
Doppler (letter). Lancet 344: 13731374
6. Holter JT, Tijsen C (1988) Cheiro-oral
syndrome: does it have a specific localizing value. Eur Neurol 28:326-330
7. Kawakami Y, Chikama M, Tanimoto
T, Shimamura Y (1989) Radiological
studies of the cheiro-oral syndrome.
J Neurol 236:177-181
8. Kugelberg E (1948) Activation of human nerves by hyperventilation and
hypocalcemia: neurological mechanism
of symptoms of irritation in tetany.
Arch Neurol Psychiatry 60:153-164
9. Omae T, Tsuchiya T, Yamaguchi T
(1992) Cheiro-oral syndrome due to
lesions in the corona radiata. Stroke
23:599-601
10. Ono S, Inoue K (1985) Cheiro-oral
syndrome following midbrain haemorrhage. J Neurol 232:304-306
11. Penfield W, Boldrey E (1937) Somatic
motor and sensory representation in the
cerebral cortex of man as studied by
electrical stimulation. Brain 60: 389443
12. Siebler M, Sitzer M, Rose G, Benfeldt
D, Steinmetz H (1993) Silent cerebral
embolism caused by neurologically
symptomatic high-grade carotid stenosis. Event rates before and after carotid
endarterectomy. Brain 116:1005 1015

13. Siebler M, Nachtmann A, Sitzer M,
Steinmetz H (1994) Anticoagulation
monitoring and cerebral microemboli
detection (letter). Lancet 344:555
14. Sittig O (1914) Klinische Beitr~ige zur
Lehre vonder Lokalisation der sensiblen Rindenzentren. Prag Med
Wochenschr 45:548-550
15. Yasuda Y, Akiguchi I, Ishikawa M,
Kameyama M (1988) Bilateral cheirooral syndrome following pontine
haemorrhage. J Neurol 235:489-490
D. G. Nabavi (N~) - p. Zunker
T. Mumme - D. Georgiadis
Department of Neurology,
University of MOnster,
Albert-Schweitzer-Strasse 33,
D-48129 MOnster, Germany
Tel.: 49 251 838172
Fax: 49 251 838181

Marc Sanson
Charles Duyckaerts
Jean-Luc Thibault
Jean-Yves Delattre

Sarcoidosis presenting as
late-onset dementia
Received: 12 September 1995
Received in revised form:
8 December 1995
Accepted: 17 December 1995

Sirs: There is neurological involvement in about 5% of patients with
sarcoidosis, and neurological symptoms are the presenting manifestation
of the disease in half the cases [8, 9].
Lesions m a y be found in the leptomeninges, particularly in the skull
base, peripheral (including cranial)
nerves, muscles and in the central
nervous system, particularly the hypothalamus. In exceptional cases,
sarcoidosis m a y cause a progressive
dementia [4], mimicking more comm o n degenerative or vascular processes. We report here a case in
which neurosarcoidosis was responsible for slowly progressive dementia
with widespread involvement of the
central nervous system.