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Cerebral ptosis with contralateral arteriovenous malformation A report of two cases.

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BRIEF COMMUNICATIONS
Cerebral Ptosis with
Contralateral Arteriovenous
Mafformation: A Report
of Two Cases
Daniel H. Lowenstein, MD,’ Thomas K. Koch, MD,t
and Michael S. Edwards, MDSt
~~~~
We describe two patients with chronic unilateral ptosis
and contralateral hemispheric arteriovenous malformations. Both patients were brought to our attention after
a subacute or acute neurological deterioration. Analysis
of prior photographs showed the ptosis progressed up to
the time of presentation and significantly resolved following surgical removal of the arteriovenous malformation. T h e partially reversible and chronic nature of the
ptosis in these two patients represents a particularly unusual form of cerebral ptosis.
Lowenstein DH, Koch TK, Edwards MS: Cerebral
ptosis with contralateral arteriovenous
malformation: a report of two cases.
Ann Neurol 21:404-407, 1987
Cerebral ptoszs refers to deficient tone of the levator
palpebrae superioris caused by a supranuclear lesion
[8]. Stimulation and lesion experiments 13, 41 suggest
the existence of cortical control of eyelid muscle tone,
but the neuroanatomical pathways remain poorly defined. Distinct clinical examples in which the cerebral
lesion has n o direct effect on the oculomotor complex
o r infranuclear pathways are rare. Descriptions of
chronic ptosis contralateral to a cerebral lesion are
rarer still.
In this report we describe two young patients with
chronic unilateral ptosis probably caused by arteriovenous malformations (AVM) in the frontal lobe. In
both instances, clinical and radiographic observations
strongly supported the notion that the cerebral lesions
exerted a direct supranuclear effect o n levator tone.
Though this association appears to be extremely uncommon, it suggests that patients with certain forms of
congenital o r acquired unilateral ptosis should undergo
imaging studies to rule out hemispheric pathology.
From the Departments of *Neurology, tpediatrics, and SNeurosurgery, School of Medicine, University of California, San Fratcisco, CA.
Received June 18, 1986, and in revised form Aug 14. Accepted for
publication Aug 1.5, 1986.
Address correspondence to Dr Lowenstein, Room 794-M, Department of Neurology, School of Medicine, University of California,
San Francisco, CA 94143.
404
Case Reports
Patient 1
A 14-year-old boy was admitted in October 1985 for excision of an AVM of the right frontal lobe. He had had an
uncomplicated birth and neonatal history, but photographs at
3 months of age (Fig 1A) suggested isolated weakness of the
left levator palpebrae superioris. At 6 months of age he
began having focal seizures, first involving the left face and
eventually the left extremities. An electroencephalogram
(EEG) was reportedly normal. Phenobarbital provided partial
control until age 2 % years, at which time the seizures remitted and medications were withdrawn. H e remained seizure-free for the next ten years. Developmental milestones
were entirely normal. However, he had mild left-sided weakness, and the unilateral ptosis remained constant (Fig 1B).
Seizures recurred at 12 years of age. These were characterized by focal twitching of the left face, which often became
generalized and resulted in Todd’s paralysis of the left side.
The seizures responded partially to a combination of phenytoin and phenobarbital. A computed tomographic (CT) scan
in June 1985 revealed a contrast-enhancing calcified lesion in
the right posterior frontal lobe without evidence of edema or
mass effect. A magnetic resonance imaging (MRI) scan in
July 1985 showed a lesion in the same location, with varying
signal intensities (Fig 2). A cerebral angiogram showed no
evidence of abnormal vasculature or vascular blush.
Up to this point the patient’s ptosis had remained unchanged. However, his mother clearly recalls that 5 hours
after the angiogram the left-sided ptosis suddenly worsened,
and there was a mild droop of the right lid as well. This
gradually abated over the next 2 to 3 days, but not to
baseline (Fig IC). In the month before admission he began
complaining of bifrontal headaches, and the left ptosis increased further.
Findings from general physical examination and mental
status testing at the time of admission were unremarkable.
Cranial nerve examination was notable only for the loss of
tone in the left levator palpebrae superioris. The inferior
tarsus appeared uninvolved. There was no evidence of pupillary or extraocular muscle abnormalities or facial paresis.
Mild clumsiness and weakness were noted in the left upper
and lower extremities without reflex asymmetry. Sensory
testing was normal.
The patient underwent a right frontal craniotomy and resection of an angiographically occult AVM. The golfballsized mass lay approximately 1 cm below the cortex in the
region of the precentral gyrus and posterior middle frontal
gyrus. Surrounding the partially thrombosed vessels was
hemosiderin-stained white matter and clear evidence of recent hemorrhage. The mass was removed in toto, and the
patient made an uneventful recovery, other than having
one brief focal seizure o n the second postoperative day.
Neurological examination 10 days following operation revealed anisocoria with the left pupil 0.5 mm smaller than the
right, although both pupils dilated in the dark. The direct and
consensual light reflexes were brisk bilaterally. Lid measurements showed an upper lid margin of 3 mm on the right and
6 mm on the left, and palpebral fissures of 10 mm on the
right and 8 mm on the left. No abnormalities of the inferior
lids were seen. Mild left-sided weakness was again observed.
Over the next 6 months the patient remained free of sei-
A
B
C
D
Fig I . Photographs of Patient 1 demonstrating progressive ldt
ptosis between the ages of 3 months (A),7 years (B),and 14
years (C). Marked resolution of the ptosis was evident 6 months
afer removal of an occult arteriovenous malformation (0).
zures and headaches. Neurological examination in April
1986 again revealed mild anisocoria without abnormal extraocular movements and marked resolution of the ptosis compared with that seen during the month before surgery
(Fig 1D).
Patient 2
A 26-year-old man arrived at the emergency room with
lethargy, headache, and right hemiparesis. Thirty minutes
earlier he had been lifting weights at a health club. As he was
leaving the facility he suddenly complained of a severe headache and lost consciousness transiently. Cranial nerve examination revealed marked ptosis of the right levator and mild
ptosis of the left (Fig 3D). The pupils were symmetrical and
extraocular movements were full. Motor examination
showed a dense right hemiparesis with hyperreflexia and an
extensor plantar response. Sensation was decreased on the
right side.
Fig 2. Tz-weighted spin echo magnetic resonance imaging (MRI)
scan of Patient 1 showing a discrete lesion in the right posterior
frontal region at the level of the roof of the lateral ventricle. The
center region has a heterogeneous signal intensity and is surrounded by a rim of signal void, most likely due t o hemosiderin.
Although a cerebral angiogram failed to demonstrate abnormal
vascularity in this area, the MRI characteristics were thought t o
be typical of an arteriovenous malformation.
Inspection of the photograph on the patient’s driver’s
license revealed a preexisting right ptosis. Photographs later
provided by family members showed that his lid asymmetry
was not present in infancy (Fig 3A), but was evident at least
as early as age 10 years (Fig 3B). Furthermore, the patient’s
ptosis on admission was markedly worse than that shown in a
photograph taken one year before (Fig 3C).
A CT scan of the head was immediately obtained and
showed a left subcortical frontal hematoma with blood dissecting into the ventricular system. A three-vessel cerebral
angiogram revealed a left parasagittal AVM fed by single
vessels from the callosal and pericallosal marginal arteries
(Fig 4).
The patient was observed closely over the next 10 days,
and had mild resolution of both his right ptosis and hemiparesis. H e then underwent a left craniotomy and resection
of the hematoma and AVM. One week following operation
(17 days after his presentation), his ptosis had returned to
that seen 1 year earlier (Fig 3Ej.
The Table lists the palpebral fissure measurements of the
two patients based upon sequential photographs and examinations. In both cases the ptosis gradually worsened, was
most prominent during the apparent subacute (Patient 1j or
acute (Patient 2) hemorrhagic manifestations of the AVM,
and eventually returned to baseline following operation.
Brief Communication: Lowenstein et al: Cerebral Ptosis
405
A
B
C
D
E
Fig 3 . Photographs of Patient 2. There was no apparent lid
asymmetry at the age of6 months (Aj, but right ptosis was
evident as early as 10 years of age (B), and again at 25 years
(C). The ptosis worsened considerablj during the hemorrhage of
a left hemisphere arteriovenous malformution (D),and partially
resolved after operation 17 days later (E).
Discussion
The first patient’s unilateral ptosis, which had been
apparent from infancy, was initially considered to be
congenital. However, there was an abrupt increase in
ptosis following his angiogram, as well as gradual worsening over the next month. His increasing headaches
406 Annals of Neurology Vol 2 1 No 4 April 1987
Fig 4. Lateral view from the lejit carotid angiogram of Patient 2.
The study demonstrated a l&t parasagittal arteriovenous malformution supplied by vessels from the callosal and pericallosal marginal arteries.
suggested a new subacute intracranial process. The
signs of both old and recent hemorrhage surrounding
the AVM at the time of surgery raise the possibility
that the patient’s increasing ptosis was due to new disruption of efferent fibers from the posterior frontal
cortex. Removal of the AVM perhaps allowed for resolution of the hemorrhage and edema in the region,
and thus partial restoration of function.
The second patient had acquired ptosis that was
present by 10 years of age and gradually worsened,
with an acute asymmetrical change in levator tone at
the time of hemorrhage. Since there were no pupil or
oculomotor nerve abnormalities, it seems highly unlikely that this was due to mass effect on the oculomotor nerve. Resolution of the ptosis to baseline, as in
the first patient, followed removal of the AVM and
subsiding edema.
The AVM in Patient 1 was located below the precentral gyrus and posterior aspect of the middle frontal
gyrus; in Patient 2 it occupied a region of white matter
below the posterior part of the superior frontal gyrus
and lateral to the cingulate gyrus. Both locations correspond well to cortical regions shown to exert control
over levator tone. Bilateral eye opening has been produced by stimulating, among other places, the second
and third frontal convolution anterior to the motor
strip 171, the rostral aspect of the cingulate gyrus 1111,
Sequential Measurements of Palpebral Fissure Ratios in Two Patients with Contralateral Arteriwenous Malformations
Patient 1
Patient 2
4s
Ratio” (lefdright)
Age
Ratio (righdleft)
3 mo
0.93
0.81
0.73
0.50
6 mo
10 yr
25 yr
26 yr (acute event)
26 yr (17 days post event)
1.0
0.92
0.87
7 Yr
14 yr (1 mo pre-op)
14 yr (10 days post-op)
14 yr (6 mo post-op)
0.80
0.69
0.87
“Ratio of maximal width of palpebral fissures.
and the first frontal convolution just above the frontal
eye fields 121. In the latter experiments, stimulation
caused a bilateral increase in levator tone, especially
contralaterally, whereas lesions in the same region
cause mild, symmetrical ptosis 11). Penfield and Rasmussen 110) later produced eye opening in humans by
stimulating the prefrontal and precentral cortex.
The rare clinical reports of cerebral ptosis have been
reviewed by Caplan 131 and Miller [SJ. Most early
reports included hemispheric lesions in which mass
effect on the oculomotor nuclei or nerves could not be
excluded. More recent reports have described patients
who developed ptosis after acute hemispheric vascular
events E3, 5, 6, 91. There are only a few examples in
the literature of patients with chronic unilateral ptosis
ascribed to hemispheric lesions. Wilbrand and Saenger
C131, in a review of 25 cases of putative “cortical”
ptosis published at the turn of the century, mention
three patients with focal epilepsy and ptosis ascribed to
hemispheric lesions. Subsequently, Cogan 141 published a photograph of a middle-aged man with left
ptosis and a contralateral temporal-occipital lobe lesion
that was not further characterized. Walsh and Hoyt
112) described a 37-year-old woman with acquired unilateral ptosis and seizures that always began with motor
movements on the same side as the ptosis. No cause
was given, although an EEG demonstrated an abnormality in the contralateral temporal lobe.
Out two patients’ diagnoses were recognized because of acute or subacute neurological events. However, we believe that their long-standing unilateral
ptosis may have been a manifestation of an occult
AVM. Certainly the benign syndrome of congenital
maldevelopment of the levator muscle accounts for the
majority of infants born with unilateral ptosis. However, in infants who develop other signs of neurological dysfunction, as in our first patient, prompt radiographic studies to rule out a focal hemispheric lesion
are indicated. The case of our second patient suggests
that young patients with acquired unilateral ptosis
which remains unexplained after standard clinical examination should undergo hemispheric imaging studies
as well.
~~
The authors thank Drs William F. Hoyt and Michael J. Aminoff for
their critique of the manuscript, and Georg Emlein for his translation
of German references.
References
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F, Bruckner A (eds): Kurzes Handbuch der Ophthalmologie.
Berlin, J Springer, 1931, Vol6, p 531
2. Brown T G Reflex orientation of the optical axes and the influence upon it of the cerebral cortex (abstract). Zentralbl
Gesamte Ophthalmol9:159, 1923
3. Caplan LR: Ptosis. J Neurol Neurosurg Psychiatry 37:l-7,
1974
4. Cogan DG: Neurology of the Ocular Muscles, ed 2. Springfield,
IL, T h o r n , 1956, pp 139-142
5. Krohel GB, Griffin J F Cortical blepharoptosis. Am J Ophthalmol85:632-634, 1978
6. Lepore FE: Bilateral cerebral ptosis (abstract). Neurology
36(suppl 1):251, 1986
7. Leyton ASF, Sherrington CS: Observations of the excitable cortex of the chimpanzee,orang-utang, and gorilla QJ Exp Physiol
11~135-222, 1917
8. Miller NR: Walsh and Hoyr‘s Clinical Neuro-ophthalmology,
ed 4. Baltimore, Williams & Wilkins, 1985, Vol2, pp 933-938
9. Nutt JG: Lid abnormalities secondary to cerebral hemisphere
lesions. Ann Neurol 1:149-151, 1977
10. Penfield W, Rasmussen T: The Cerebral Cortex of Man. New
York, Hafner, 1968, pp 51-52
11. Smith WK: The functional significance of the rostral cingular
cortex as revealed by its responses to electrical excitation. J
Neurophysiol8241-255, 1945
12. Walsh FB, Hoyt W.Clinical Neuro-Ophthalmology,ed 3. Baltimore, Williams & Wilkins, 1969, Vol l, p 298
13. Wilbrand H, Saenger A: Die Beziehungen des Nervensystems
zu den Lidern. In: Die Neurologie des Auges. Wiesbaden, JF
Bergman, 1900, Vol8, pp 96-103
Brief Communication: Lowenstein e t al: Cerebral Ptosis
407
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