Movement Disorders
Vol. 8, No. 2, 1993, pp. 198-2Oq
0 1993 Movement Disorder Society

Brief Report

Reflex Blepharospasm Associated with Bilateral Basal
Ganglia Lesion
R. Larumbe, J. Vaamonde, J. Artieda, J. L. Zubieta, and J. A. Obeso
Movement Disorders Unit and Clinical Neurophysiology Service, Department of Neurology and Neuroradiology
Unit, Department of Radiology, Clinica Universitaria, University of Navarra Medical School, Pamplona, Spain

Summary: A patient with a bilateral striatal lesion secondary to anoxia presented reflex blepharospasm associated with parkinsonism and dystonia in the
limbs. The blink reflex excitability curve was enhanced and the R-2 response
prolonged as in patients with essential blepharospasm. The findings in this
patient support the notion that blepharospasm may be secondary to basal
ganglia dysfunction through abnormal facilitation of reticular formation neurons controlling facial nucleus motoneuron excitability. Key Words: Blepharospasm-Blink reflex-Striatal lesion.

sociated risk factor was known from her history or
with relation to the surgical procedure. She was in a
coma for 3 days after surgery. On recovering consciousness, neurological examination revealed hypomimia, severe bradykinesia and rigidity in all four
limbs, and brief multifocal myoclonic jerks.
Occasionally blepharospasm occurred spontaneously. Any attempt to open the patient’s eyes passively or stretch any part of 00 with the eyes open
led to sustained spasms. Visual threatening also
provoked blepharospasm although much less frequently and with quick adaptation. In the following
weeks, generalized dystonia become apparent.
Computed tomographic brain scan and magnetic
resonance imaging (MRI) revealed bilateral symmetrical involvement of the basal ganglia affecting
the striatum (putamen and caudate) and lateral globus pallidus (Fig. 1). The brainstem and cerebellum
were intact. Routine blood tests and copper, ceruloplasmine, lactic and pyruvic acids, and lysosomal
enzymes were all normal.
Electromyographic (EMG) recording with surface electrodes over the 00 (Fig. 2) confirmed the
occurrence of blepharospasm elicited by rapid and
sustained stretching produced by movement of the
blunt end of a pin of either the inferior or superior
parts of the orbital section of the 00. The blink
reflex and its excitability cycle were studied by the

Blepharospasm is the involuntary spasmodic
closing of the eyelids. It generally occurs spontaneously but may be triggered by external stimuli such
as bright light, cold, air, and so forth (1). More
rarely, stretching the orbicularis oculi (00)muscle
may elicit blepharospasm (reflex bkphharospasm).
Spontaneous blepharospasm secondary to structural focal lesions has been described in quite a few
patients (2-13). Reflex blepharospasm has been described following large cerebral infarction (14),
within a family (15), and in more detail in a patient
with unknown cause (16). We report a patient with
bilateral infarction of the basal ganglia secondary to
anoxia during surgery who developed a dystonicparkinsonian syndrome associated with stretch reflex blepharospasm.
CASE REPORT

A 13-year-old girl with no known personal or familial history of neurological disease had cardiac
surgery for supravalvular aortic stienosis in 1989.
During surgery, blood pressure was relatively low
(systolic 40-60 mmHg) for about 2 h. No other asAddress correspondence and reprint request to Dr. J. A.
Obeso, Department of Neurology, Clinica Universitaria, Apdo.
192, 31080 Pamplona, Spain.

198

STRIATAL LESION AND BLEPHAROSPASM

199
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hh

R 00
9.5

Lo

29

o b
9.7

30

200 ms

FIG. 1. Cerebral magnetic resonance imaging (MRI) of the patient. A. T-l (coronal view at the level of the basal ganglia) showing hypoxic infarction (dark signal) of the putamen and head of
the caudate nucleus bilaterally and of the external globus pallidus
in the right hemisphere. B. Horizontal axial section (T2) showing
a similar distribution (high signal intensity) of the lesion.

FIG. 3. Blink reflex of the patient after electrical stimulation of
the right supraorbital nerve. The latency of the R1 (9.5 and 9.7
ms) and R2 (29 and 30 ms) components is normal but the duration
of the R2 is prolonged bilaterally. R, right orbicularis oculi. L
00. left orbicularis oculi.

DISCUSSION
standard method (17,lS). The right supraorbital
nerve was electrically stimulated with supramaxima1 intensity, giving a single stimulus (control) or
paired stimuli separated by 20, 50, 100, 150, 200,
300, and 500 ms. Ten unconditioned and conditioned stimuli were given for each point of the excitability curve. Single stimulation evoked a normal
R-1 response. The R-2 component had normal latency bilaterally but prolonged duration (260 ms in
the patient vs. 30 to 50 ms for normal controls) (Fig.
3). The excitability of the blink reflex was also increased. Thus, the R-2 response was recovered by
50% for an interstimulus interval of 147 ms [normal
values for 50% recovery, 920 to 1,200 ms (17-19;
authors’ personal experience)].

It

J

t

I t

t

J

I

2seC

’

FIG. 2. Electromyographic (EMG) recording from the right orbicularis oculi (R 00) muscle. Sustained stretch of the muscle
(starting and ending point indicated by upgoing and downgoing
arrows, respectively) elicited a long-lasting spasm (upper half)
and short stretches provoked phasic EMG responses (lower
half).

Blepharospasm secondary to lesion of the putamen bilaterally and upper brainstem has been previously documented (2-13). Among those reports,
there are a few patients (5-7) whose blepharospasm
was noticed to increase following physical attempts
by the examiner to open their eyes, but this contingency was not further explored. Reflex blepharospasm was not the only clinical manifestation of
striatal infarction in our patient. However, there
was no clinical or radiological evidence of lesion
outside the lenticular nucleus. It should be admitted
nevertheless that anoxia may have damaged other
neuronal groups, i.e., the brainstem reticular formation, which could be primarily involved in the origin
of blepharospasm. With this limitation in mind, this
is the first case of reflex blepharospasm, to our
knowledge, associated with a known pathological
basis and with physiological documentation.
The clinical and electrophysiological features of
the patient here described are identical to the patient reported by Obeso et al. (16). Reflex blepharospasm may be one extreme of the clinical spectrum of blepharospasm and may have a common
pathophysiological basis regardless of the cause. Indeed, the capability to aggravate or trigger blepharospasm by external stimuli is well recognized
(1,12), and enhanced excitability of the blink reflex
is a well-established characteristic of patients with
essential blepharospasm and other forms of focal
dystonia (18-20).
The R2 response of the blink reflex is mediated
by a polysynaptic pathway that goes from the sensory nuclei of the trigeminal nerve through the
bulbo-pontino reticular formation before targeting

Movement Disorders, Vol. 8 , N o . 2, 1993

R . LARUMBE ET AL.

200

onto the facial nucleus (21). Blepharospasm and increased excitability of the R2 response have also
been described in patients with Parkinson’s disease
(17). Ballard et al. (22) also observed blepharospasm in patients with parkinsonism secondary to
MPTP intoxication. All these data suggest that the
basal ganglia exerts a tonic control of the excitability of the blink reflex. Regardless of the cause and
associated clinical features, the R-1response of the
blink reflex is normal, and the R-2 is enhanced in
patients with blepharospasm. This dissociation indicates a discrete effect of the basal ganglia on
brainstem neurons controlling the excitability of the
R-2 response. In conclusion, this observation supports the evidence from patients with idiopathic
blepharospasm (18-20) suggesting that abnormal
functioning of reticular formation neurons may be
the ultimate pathophysiological basis of blepharospasm.
Acknowledgment: Ms. M. Mar Lopez and Mrs. Carol
Elsden edited the article for publication.

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