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Abstract
We report the clinical features of 4 cases with positional or positioning down-beating nystagmus in a head-hanging or supine position without
any obvious central nervous system disorder. The 4 cases had some findings in common. There were no abnormal findings on neurological tests or
brain MRI. They did not have gaze nystagmus. Their nystagmus was observed only in a supine or head-hanging position and it was never observed
upon returning to a sitting position and never reversed. The nystagmus had no or little torsional component, had latency and tended to decrease
with time. The positional DBN (p-DBN) is known to be indicative of a central nervous system disorder. Recently there were some reports that
canalithiasis of the anterior semicircular canal (ASC) causes p-DBN and that patients who have p-DBN without obvious CNS dysfunction are
dealt with anterior semicircular canal (ASC) benign paroxysmal positional vertigo (BPPV). There are some doubts as to the validity of making a
diagnosis of ASC-BPPV in a case of p-DBN without CNS findings. It is hard to determine the cause of p-DBN in these cases.
# 2009 Elsevier Ireland Ltd. All rights reserved.
Keywords: BPPV; Down-beating; Nystagmus; ASC; Positional nystagmus; Positioning nystagmus
1. Introduction
Positional down-beat nystagmus (p-DBN) is a clinical sign
of central nervous system (CNS) involvement. It occurs with
lesions in the vestibulocerebellum, the craniocervical junction
and with drug intoxication [1]. Experimental extirpation of
the nodulus in the cat causes postural down-beat nystagmus
[2]. Occasionally, p-DBN is seen in patients without CNS
involvement [1]. The p-DBN with or without slight positional
vertigo in the head-hanging position, is a frequent, and often
the only clinical sign in neurological patients and the p-DBN
may spontaneously recover or persist. In these cases, a central
disorder is most commonly suspected, but examination of
these patients does not always disclose a CNS abnormality.
The cause of p-DBN is not clear yet.
* Corresponding author at: Nishishinjuku 6-7-1 Shinjuku-ku, Tokyo 1600023, Japan. Tel.: +81 3 3342 6111; fax: +81 3 3346 9275.
E-mail address: y-ogawa8@tokyo-med.ac.jp (Y. Ogawa).
0385-8146/$ see front matter # 2009 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.anl.2009.04.001
699
3. Discussion
DBN in primary gaze is a sign of central nervous system
dysfunction [5]. p-DBN has also been reported as a sign of
CNS dysfunction [1]. Brandt [1] reported p-DBN in the
Table 1
Clinical summary of patients with positional or positioning down beating nyastagmus and no CNS findings.
Patients number
1
2
3
4
Sex
f
m
f
f
Age
35
34
21
39
DBN
Symptom
Positioning test
Positioning test
(sitting position)
No
Supine, head left
Supine, head left, head right, head-hanging
Supine, head left, head right, head-hanging
Head-hanging
Head-hanging
Head-hanging
Head-hanging
Vomiting
No
No
No
No
Time course
Headache
+
+
+
8
2
9
3
months
weeks
months
months
700
Fig. 1. Electronystamograph of p-DBN in case 4, during the positioning nystagmus test. p-DBN was induced by the head-hanging maneuver and was accompanied
with leftward horizontal component. The evoked p-DBN gradually reduced. The vertical nystagmus was not observed upon returning to a sitting position.
Fig. 2. Diagram of the representation of the canalithiasis of the ASC during the positioning nystagmus test. (A) The location of otoliths in the ASC lumen in a
sitting position. (B) The patient is in a head-hanging position. The otoliths move in ampullofugal direction in the lumen of ASC by gravitational force, thus
producing the excitatory ampullofugal stimulation of the crista. (C) When the patient returns to a sitting position, the otoliths go out to the utriculus. Arrows
(solid line) indicate the head movement. A dotted line arrows indicate the direction of the otolith displacement.
5. Possible etiology
It is hard to determine the cause of p-DBN in these cases.
P-DBN may be caused by multiple sclerosis, ischemia,
intoxication, crania-cervical malformation or cerebella
degeneration. However, there was no identifiable etiology
in our 4 cases. Vascular insufficiency is not likely, since all 4
subjects were relatively young. The duration of their
nystagmus varies from 2 weeks to 10 months, and it might
be due to the diversity of the etiology. We speculate that the
possible cause of p-DBN in our 4 cases was a variant of
701
Acknowledgement
The authors are indebted to Prof. J. Patrick Barron of the
International Medical Communications Center of Tokyo
Medical University for his review of this manuscript.
References
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