CLINICAL INVESTIGATION

Microscopic polyangiitis complicated by oculomotor nerve palsy

Yuri Hiramatsu • Takuya Kotani • Tohru Takeuchi •

Takuji Kurimoto • Shigeki Makino •

Toshiaki Hanafusa

Received: 25 March 2012 / Accepted: 3 October 2012 / Published online: 11 December 2012

� Japanese Ophthalmological Society 2012

Abstract

Background Microscopic polyangiitis (MPA) is a necro-

tizing vasculitis of the small vessels. Among the nerve

lesions of MPA, the incidence of multiple mononeuritis is

high, but cranial nerve palsy is rarely reported.

Case A female patient with oculomotor nerve palsy

associated with MPA.

Observations The 68-year-old patient was admitted to

our hospital with a high fever, numbness and weakness of

the extremities, and muscle weakness. Multiple mononeu-

ritis and purpura were observed. The urine was positive for

occult blood and protein and the creatinine level was

1.2 mg/dL, indicating renal impairment. The levels of

C-reactive protein (15.5 mg/dL) and myeloperoxidase-

antineutrophil cytoplasmic antibody titers (600 ELISA

units) were elevated. MPA was diagnosed, and 45 mg/day

prednisolone was initiated. On the fifth day after the

initiation of treatment, the patient suddenly developed

diplopia and blepharoptosis of the left eye. Anisocoria and

decreased light reflex as well as limited supraduction,

infraduction, and adduction were also observed in the eye.

Left oculomotor nerve palsy was diagnosed. The palsy

gradually improved with continued prednisolone treatment.

Conclusions We encountered a rare case of MPA com-

plicated by oculomotor nerve palsy.

Keywords Oculomotor nerve palsy � Microscopic

polyangiitis

Introduction

Microscopic polyangiitis (MPA) is a necrotizing vasculitis

that affects the small vessels of mainly the kidneys, skin,

lungs, and peripheral nerves. Among the nerve lesions of

MPA, the incidence of multiple mononeuritis is high

(56 %) [1], but cranial nerve palsy is rarely reported. We

report a rare case of MPA complicated by peripheral

oculomotor nerve palsy, which gradually improved with

corticosteroid treatment.

Case report

A 68-year-old woman developed a high fever and numbness

and weakness of the extremities in February 2011 and was

admitted to our hospital on April 12, 2011. Physical

examination on admission showed a fever (37.8 �C) and

muscle weakness and purpura in the upper and lower

extremities. Impaired surface sensation in the left fingers

and in the dorsal and plantar areas of the feet as well as

impaired vibratory sensation of the left leg was observed.

Urinalysis showed occult blood, protein, and granular casts.

The white blood cell count was 11,030 lL (neutrophils,

79 %). Albumin, creatinine, and C-reactive protein levels

were 2.5 g/dL, 1.2, and 15.5 mg/dL, respectively.

Myeloperoxidase-antineutrophil cytoplasmic antibody

(MPO-ANCA) was positive (600 ELISA units). Other

disease-specific autoantibodies were negative. Chest com-

puted tomography (CT) scans revealed calcification of the

bilateral superior lungs. Peripheral nerve conduction tests of

Y. Hiramatsu � T. Kotani � T. Takeuchi (&) � S. Makino �T. Hanafusa

Department of Internal Medicine (I), Osaka Medical College,

Daigaku-Machi 2-7, Takatsuki, Osaka 569-8686, Japan

e-mail: t-takeuchi@poh.osaka-med.ac.jp

T. Kurimoto

Department of Ophthalmology, Osaka Medical College,

Daigaku-Machi 2-7, Takatsuki, Osaka 569-8686, Japan

123

Jpn J Ophthalmol (2013) 57:221–224

DOI 10.1007/s10384-012-0221-9

the limbs showed multiple mononeuritis. MPA was diag-

nosed according to the diagnostic criteria [2]. Initiation of

treatment was prioritized over conducting a biopsy con-

sidering the rapidly progressive muscle weakness due to

multiple mononeuropathy. Isoniazid (200 mg/day) was

initiated on the second hospital day to prevent reactivation

of pulmonary tuberculosis, and prednisolone (45 mg/day)

was initiated on the third hospital day. On the eighth hos-

pital day, the patient suddenly developed diplopia and

blepharoptosis of the left eye. The best-corrected visual

acuity of each eye was 1.0. The intraocular pressure was

11 mmHg OD and 12 mmHg OS. Left exotropia and

hypertropia at near were 10 and 3 prism diopters (PD),

respectively, and at distance they were 16 and 6 PD,

respectively. Anisocoria (left, 4 mm; right, 2 mm) in room

light and a decreased direct and indirect light reflex in the

left eye were observed. Supraduction, infraduction, and

adduction in the left eye were limited (Fig. 1a). The Hess

chart showed a contracted left chart and an expanded right

chart. The left eye showed exotropia with limited adduction,

supraduction, and infraduction. Regarding the vertical eye

movement, no significant deviation was present owing to

the symmetric involvement of supraduction and infraduc-

tion (Fig. 2a). The Hess chart supported the disturbance of

eye movement. Whole-brain gadolinium-enhanced mag-

netic resonance imaging (MRI), magnetic resonance angi-

ography (MRA), and contrast-enhanced cranial CT scans

showed no abnormalities. Regarding the image findings, no

difference was observed in the left and right oculomotor

nerves. Left oculomotor nerve palsy due to ischemic vas-

culitis was diagnosed. The left oculomotor nerve palsy

gradually improved with continuing prednisolone treatment

(Figs. 1b, 2b).

Comments

Oculomotor nerve palsy complicated by ANCA-associated

vasculitis (AAV) can be caused by the pathologic mecha-

nism of ischemic vasculitis, mesencephalon involvement,

pseudotumor of the orbit, and hypertrophic cranial pachy-

meningitis. Seishima et al. [3] reported a case of a 66-year-old

woman who had MPA complicated by oculomotor nerve

palsy due to ischemic vasculitis. The palsy gradually

improved over six months of systemic corticosteroid and

oral cyclophosphamide treatment. In AAV other than

MPA, five cases of Churg–Strauss syndrome (CSS)

showing oculomotor nerve palsy have been reported [4–8].

Fig. 1 Course of left

oculomotor nerve palsy.

a Blepharoptosis and limited

supraduction, infraduction, and

adduction of the left eye were

observed (eighth hospital day).

b The blepharoptosis and

limited movements of the left

eye improved after steroid

therapy (30th hospital day)

222 Y. Hiramatsu et al.

123

The pathologic mechanisms were central oculomotor nerve

palsy due to mesencephalon infarction in one case [4] and

peripheral ischemic vasculitis in two cases [5, 6], but were

unclear in the other two cases owing to the absence of

pathologic descriptions. Nishino et al. [9] observed

oculomotor nerve palsy in 2 of 324 patients with Wegener’s

granulomatosis (WG; 0.6 %), and the pathology was a

pseudotumor of the orbit. Sakurazawa et al. [10] reported a

case of WG with oculomotor nerve palsy due to hyper-

trophic cranial pachymeningitis.

Fig. 2 Hess chart of the case with oculomotor nerve palsy. a Left chart showed limited supraduction, infraduction, and adduction. b These

limitations in eye movement showed improvement after steroid therapy (55th hospital day)

Oculomotor nerve palsy with MPA 223

123

Comprehensive assessment based on ophthalmic and

imaging tests is needed to determine the lesion site in

oculomotor nerve palsy. The present case involved ipsi-

lateral blepharoptosis, pupil dysfunction, and eye move-

ment disturbance unaccompanied by trigeminal or

abducent nerve palsy. MRI and MRA revealed no lesions

in the brain stem and other intracranial areas such as the

cavernous sinus, orbit, and subarachnoid space. Compli-

cations such as diabetes mellitus, systemic lupus erythe-

matosus, temporal arteritis, and other systemic diseases

were absent. Thus, ischemic vasculitis caused by MPA

probably induced the oculomotor nerve palsy. Typically,

oculomotor nerve palsy due to vascular disease is not

accompanied by pupil dysfunction, and responds to ste-

roids. In this case, the ischemic vasculitis was severe and

active despite the initiation of steroid therapy, possibly

causing the oculomotor nerve palsy with pupil dysfunction.

In conclusion, we encountered a second case of MPA

complicated by oculomotor nerve palsy, and ischemic

vasculitis was suggested as the pathologic mechanism.

When MPA is complicated by cranial nerve palsy such as

oculomotor nerve palsy due to ischemic vasculitis, the

vasculitis should be adequately treated by immunosup-

pressive therapy, such as corticosteroids, in the early stage.

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