The New England Journal of MedicineA 67-Year-Old Woman with Alternating Sixth Cranial Nerve Palsy

A Case Challenge from The New England Journal of Medicine

In this detailed case study from the Massachusetts General Hospital, the perplexing symptoms of a 67-year-old woman presenting with headache, retro-orbital pain, and a series of dermatological and ophthalmological findings that defy straightforward diagnosis are discussed. The patient’s journey through the healthcare system, encompassing multiple hospital visits and an array of diagnostic tests, highlights the challenges and intricacies of medical diagnosis in the face of complex, overlapping symptoms. This case emphasizes the importance of a holistic approach to patient care, integrating detailed patient history, laboratory data, and imaging findings to navigate towards a diagnosis.

Key Points:

• A 67-year-old woman presented with headache, retro-orbital pain, and a history of rash after starting hydrochlorothiazide, which improved upon switching to amlodipine and using topical triamcinolone.
• Initial MRI showed edema and enhancement of the optic-nerve sheath, sparing the nerve. Subsequent imaging revealed similar symptoms in the opposite eye, with stranding of the intraconal fat.
• The patient’s medical history includes Graves’ disease with orbitopathy, hypothyroidism, hypertension, hyperlipidemia, and a history of breast cancer, highlighting the complexity of her medical background.
• Laboratory findings were largely normal, including tests for systemic inflammation (ESR, CRP) and screenings for various infections and autoimmune conditions.
• Elevated IgG4 level was noted, which could indicate a potential underlying condition not yet diagnosed in this context.
• The patient’s symptoms and imaging findings suggest a complex interplay of systemic and local factors, necessitating a broad differential diagnosis.
• Treatment with intravenous methylprednisolone was initiated, leading to partial symptom relief, suggesting an inflammatory component to her condition.

IgG4-related disease, a condition that may present with diverse symptoms including organ enlargement, nodules, and a variety of systemic manifestations, was only formally recognized in the early 21st century? This relatively new diagnostic entity underscores the evolving nature of medical knowledge and the importance of ongoing research in identifying and treating complex diseases.

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