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Midbrain Infarction Presenting With Monocular Elevation Palsy and Ptosis: Topographic Lesion Analysis

Choi, Yun-Ju MD; Lee, Seung-Han MD, PhD; Park, Man-Seok MD, PhD; Kim, Byeong C. MD, PhD; Kim, Myeong-Kyu MD, PhD

Journal of Neuro-Ophthalmology: June 2015 - Volume 35 - Issue 2 - p 175–178
doi: 10.1097/WNO.0000000000000208
Clinical Observation
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Abstract: A combination of monocular elevation palsy and ptosis is usually characteristic of an extra-axial lesion of the superior branch of the third nerve. We report an unusual case of monocular elevation palsy and ipsilateral ptosis due to midbrain infarction involving the third nerve fascicle. In addition, we conducted a review of the literature of similar cases and produced an overlay image of the magnetic resonance scans from these reports. The overlapping regions primarily were located in the midbrain between the red nucleus and cerebral peduncle. This correlated with involvement of the lateral portion of the third nerve fascicle containing fibers to the superior rectus and levator palpebrae.

Department of Neurology (Y-JC, S-HL, M-SP, B-CK, M-KK), Chonnam National University Medical School, Gwangju, Korea; and Department of Neurology (Y-JC), Presbyterian Medical Center, Seonam University College of Medicine, Jeonju, Korea.

Address correspondence to Seung-Han Lee, MD, PhD, Department of Neurology, Chonnam National University Medical School, 8 Hak-dong, Dong-gu, Gwangju 501-757, Korea; E-mail: nrshlee@chonnam.ac.kr

The authors report no conflicts of interest.

A partial third nerve palsy may due to involvement of the fascicular portion of the nerve within the brainstem (1,2). Reports following midbrain stroke include pupil-sparing third nerve palsy, isolated adduction palsy, isolated ptosis and mydriasis, and monocular elevation palsy with ipsilateral ptosis (3–8). We describe a patient with findings mimicking a superior division third nerve palsy and review similar reported cases with associated magnetic resonance imaging (MRI) abnormalities.

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CASE REPORT

A 75-year-old woman presented with a 2-day history of right ptosis and diplopia. She had a 13-year history of diabetes mellitus and hypertension. There was no ocular injection or pain, visual loss, or proptosis. Examination revealed complete right ptosis and limitation of supraduction in the right eye with no abnormalities of adduction, infraduction, or pupillary reactivity (Fig. 1). The red glass test revealed vertical diplopia that increased in upward gaze and disappeared in downward gaze. Other cranial nerves, including the trigeminal and motor, sensory, and cerebellar systems were normal. Brain MRI did not reveal abnormalities in the right superior orbital fissure/cavernous sinus, orbit, or periorbital area. However, diffusion-weighted images showed abnormal high-signal intensity in the right medial midbrain near the cerebral peduncle compatible with acute infarction (Fig. 2). Her symptoms gradually improved over 3 months.

FIG. 1

FIG. 1

FIG. 2

FIG. 2

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METHODS FOR LITERATURE REVIEW AND IMAGE OVERLAPPING

We conducted a review of the literature of similar clinical manifestations and collected MRI scans of these cases. This was performed by a web-based search for both English (www.ncbi.nlm.nih.gov/pubmed) and Korean (www.koreamed.org) publications using the following terms: oculomotor nerve; third cranial nerve; stroke; hemorrhage; ischemia; infarct; elevation; paresis, palsy; midbrain; mesencephalon. Our search was conducted up to July, 2014. Articles were selected using predetermined criteria. These criteria excluded reports that lacked original patient data, did not provide description of ocular motor symptoms, and did not indicate MRI findings. We identified 30 articles, 23 of which were excluded due to the presence of only experimental data (n = 12) and other associated ophthalmologic abnormalities, such as oscillopsia and horizontal gaze palsy (n = 11).

After a full-text review, we found 4 cases of monocular elevation palsy with ipsilateral ptosis in 3 (7–9). Four articles were excluded due to the lack of MR images. The demographics and clinical characteristics of the cases are shown in Table 1, and schematic diagrams of the MRI lesions appear in Figure 3.

TABLE 1

TABLE 1

FIG. 3

FIG. 3

We constructed an overlay image from each MRI scan containing the lesions using MRIcro software version 1.4 (www.mricro.com) (Fig. 4). The lesions of each patient were drawn manually onto transverse slices of the publicly available Montreal Neurological Institute brain, a T1-weighted template MRI scan, which is oriented to match the Talairach space. The template images containing the region of interest of each patient were summed up, and the overlapping area was shown in different colors according to the numbers of intersecting portions: 4 = red, 3 = green, 2 = blue, 1 = purple. One case with additional neurologic deficits due to a large midbrain stroke was excluded (8). The overlapping areas were primarily located in the midbrain between the red nucleus and cerebral peduncle. This area was correlated with previously reported diagram of third nerve fascicular topography (1) (Fig. 5).

FIG. 4

FIG. 4

FIG. 5

FIG. 5

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DISCUSSION

Our case highlights a rare manifestation of partial third nerve palsy characterized by monocular elevation palsy and ipsilateral ptosis due to midbrain infarction. Ischemia of the lateral portion of the fascicle that contains fibers innervating the superior rectus and levator palpebrae were affected in our patient.

Several previous reports (7–10) also support our findings and are summarized in Table 1. Most cases had a focal ischemic lesion of the third nerve fascicle in the anterior portion of the midbrain between the cerebral peduncle and the red nucleus (Fig. 3). A case of lateral midbrain infarct with ptosis and upgaze palsy was documented in a patient with hemiparesis and dysarthria (8). In this patient, autopsy confirmed involvement of the lateral fascicular fibers.

Most commonly, a superior division palsy of the third nerve is due to a lesion of the cavernous sinus/superior orbital fissure or orbit (3,8). Rarely, the cause may be an extra-axial ischemic neuropathy due to diabetes mellitus (6,11).

In summary, a partial third nerve palsy in the absence of long tract signs can be caused by a fascicular brainstem lesion. Furthermore, midbrain infarction involving the lateral portion of the third nerve fascicle may present with isolated monocular elevation paresis and ipsilateral ptosis.

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