13.10 Recurrent painful ophthalmoplegic neuropathy

Identification: Female patient, 32 years old, with recurrent episodes of orbital pain and diplopia for 3 years. History of Present Illness: Current Acute Episode: Pain: Location: Left retro-orbital and supraorbital Characteristic: Constant, with deep pressure (7/10 VAS) Lancinating exacerbations (9/10) with eye movements Duration: 5 days Neurological Deficit: Complete ptosis External ophthalmoplegia (cranial nerves III and VI affected) Areactive mydriasis Previous History: 4 similar episodes in the last 3 years (2-3/year) Spontaneous remission in 4-8 weeks Previous brain MRI without alterations Physical/Neurological Examination: Ocular Motility (Left Eye): Complete limitation of elevation, adduction, and abduction Preservation of depression (cranial nerve IV intact) Pupils: Anisocoria (Right Eye 4mm, Left Eye 6mm) Direct and consensual photomotor reflex absent on the left Sensory: Hypoesthesia in ipsilateral V1 Complementary Exams: Orbital Magnetic Resonance Imaging with Contrast (3T): Thickening and enhancement of the left intracavernous oculomotor nerve Absence of vascular compression or expansive lesions Cerebrospinal Fluid (lumbar puncture): Leukocytes: 8 cells/mm³ (lymphocytosis) Proteins: 65 mg/dL Negative oligoclonal bands Serologies: ANCA, ANA, anti-MOG, and anti-AQP4 negative