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Updated: Jun 24, 2026

Minimally Invasive Surgical Decompression of Occipital Nerves

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Published on: September 13, 2024

Sneddon's syndrome presenting with severe disabling bilateral headache.

Cinzia Cavestro¹, Luca Richetta, Enrico Pedemonte

¹Department of Neurology, Headache Center, Ospedale San Lazzaro A.S.L., Alba (CN), Italy. cicaves@tin.it

The Journal of Headache and Pain

|March 17, 2009

Summary

Sneddon's syndrome, a rare vascular condition, can cause severe headaches and livedo reticularis. This case highlights effective treatment with ticlopidine and pentoxifylline for symptom relief.

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Area of Science:

Neurology
Vascular Biology
Dermatology

Background:

Sneddon's syndrome is a rare vascular disorder characterized by livedo reticularis and recurrent ischemic strokes.
It results from endothelial proliferation occluding small arteries in the skin and brain.

Observation:

A 64-year-old woman presented with a 4-year history of severe, disabling headache and facial pain, initially misdiagnosed.
She also exhibited cognitive decline, severe livedo reticularis, myalgias, and stiffness.

Findings:

Diagnosis of Sneddon's syndrome was confirmed after excluding other conditions.
Treatment with acetylsalicylic acid, followed by ticlopidine and pentoxifylline, led to significant symptom improvement, including headache resolution.
Olanzapine provided temporary relief for associated psychiatric symptoms.

Implications:

Accurate headache classification is crucial for avoiding diagnostic delays in Sneddon's syndrome.
This case demonstrates an atypical presentation and the therapeutic efficacy of ticlopidine and pentoxifylline.
Highlights the importance of considering Sneddon's syndrome in patients with unexplained neurological and dermatological symptoms.