|LETTER TO EDITOR
|Ahead of print publication
Isolated oculomotor nerve palsy: A rare initial presentation of granulomatosis with polyangitis
Debashis Maikap1, Amrita Pradhan2, Prasanta Padhan1
1 Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India
2 Department of Ophthalmology, S.C.B Medical College, Cuttack, Odisha, India
|Date of Submission||09-Oct-2021|
|Date of Acceptance||28-Nov-2021|
|Date of Web Publication||28-Mar-2022|
Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar - 751 024, Odisha
Source of Support: None, Conflict of Interest: None
An 80-year-old male presented with complaints of sudden onset, painless, left side drooping of the eyelid without vision loss for 3 days. He had a history of recurrent sinusitis with bloody nasal discharge for the last 2 years unresponsive to multiple antibiotics. He had no fever, headache, vomiting, giddiness, jaw or limb claudication, carotydynia, abdominal pain. He had no history of bronchial asthma, diabetes mellitus, hypertension, or pulmonary tuberculosis. On general examination, there was no scalp tenderness, skin rash, muscle weakness. Ocular examination revealed no red eyes, proptosis, or ocular tenderness. There was complete left ptosis, dilated nonreactive pupil with marked limitation of adduction, elevation, and depression suggestive of left 3rd cranial nerve palsy [Figure 1]a. There was no relative afferent pupillary defect. Slit-lamp examination showed peudophakia in both eyes. Other cranial nerve examination was normal. All peripheral pulses were palpable and there was no bruit. Systemic examination was normal. Laboratory investigation revealed raised acute-phase reactants (C-reactive protein [CRP]-18 mg/L, normal <6 mg/L and erythrocyte sedimentation rate [ESR]-58 mm in 1st h, normal <20 mm in 1st h). His complete blood count, liver function, and renal function tests were normal. His ANA by (indirect immunofluorescence), hepatitis B, hepatitis C, HIV were tested negative. His antiproteinase-3 antibodies (anti-PR3) and antimyeloperoxidase antibodies (anti-MPO) levels by ELISA were 124 AU/mL (normal range <5 AU/mL) and 3.56 AU/mL (normal range <5 AU/mL), respectively. His urine routine examination was normal. Contrast magnetic resonance imaging of the brain including orbit was unremarkable. Sinus computed tomography (CT) demonstrated pansinusitis. His thoracic CT scan was normal. In view of pansinusitis with cranial nerve involvement and raised PR3 ANCA, diagnosis of granulomatosis with polyangiitis (GPA) was made. He was started on IV methylprednisolone pulse therapy 500 mg per day for 3 days followed by prednisolone 1 mg/kg/day. Intravenous cyclophosphamide pulse therapy at a dose of 750 mg every 2 weeks for 3 doses followed by every 3 weeks was administered. On follow-up after 1 month, there was partial recovery of ptosis, pupillary reflex and eyeball movement recovered completely with normalization of inflammatory markers (ESR-12 mm in 1st h and CRP-2 mg/L) [Figure 1]b. His follow-up after 3 months revealed near-complete recovery of ptosis [Figure 1]c.
|Figure 1: (a) Complete left ptosis at the time of presentation. (b) Partial recovery of left ptosis after 1 month of treatment. (c) Near complete resolution of left ptosis after 3 months of treatment|
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GPA is a systemic vasculitis affecting small-to-medium vessels, classically affecting kidneys, upper and lower airways. It has been reported that sinonasal symptoms could appear as the initial clinical symptom in GPA. Common symptoms of nasal involvement include nasal obstruction or stuffiness, hyposmia or anosmia, septal perforation, epistaxix, or saddle nose deformity. Our patient had upper airway involvement.
Nervous system involvement is rare at the initial stages, but not uncommon during its entire clinical course. Although peripheral neuropathy is the most common neurological presentation, cranial nerve palsies are reported in GPA. Isolated cranial nerve palsies are reported in 8%–28% cases of GPA., However, cranial neuropathy as the first presentation of the disease preceding other organ involvement is reported rarely in GPA.
Drachman in 1962 reviewed neurological complications in 103 patients of GPA and found to have 56 patients had neurological involvement 3. He described 3 patterns of nerve involvement in GPA as follows, (a) granulomatous lesions encroaching on the nerves by direct invasion from nasal or paranasal granulomas, (b) vasculitis of the cranial nerves, and (c) granulomatous lesions involving vessel remote from the nasal granuloma. Orbital granulomatous masses tend to be more frequent, determining compressive cranial neuropathies in most of the cases. Granulomatous pachymeningitis can be associated with cranial neuropathies and polyneuritis cranialis is seen where the base of the skull is involved. The cranial nerves most commonly affected are II, VI, and VII. In our case, the mechanism of 3rd cranial nerve palsy may be due to vasculitis of vaso nervorum of oculomotor nerve leading to ischemic cranial neuropathy or result of the immune-mediated inflammatory process. Our patient responded dramatically to immunosuppression.
This case highlights 3rd cranial nerve palsy can be isolated initial presentation of GPA preceding any other organ disease which requires prompt recognition and treatment.
Written informed consent for publication was obtained from the patient.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
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Drachman DA. Neurological complication of Wegener's granulomatosis. Arch Neurol 1962;8:45.