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Published on: March 10, 2021
Sneddon's Syndrome presenting with topographic disorientation
M Marianetti1, C Mina, P Marchione
1Department of Neurology and ORL, Viale dell'Università 30, Sapienza University, 00185 Rome, Italy. m.marianetti@virgilio.it
Summary
Sneddon's Syndrome (SNS) can initially present with cognitive impairment only, preceding characteristic skin and MRI findings. Early diagnosis is crucial for managing this rare cerebrovascular disorder.
Area of Science:
- Neurology
- Dermatology
- Radiology
Background:
- Sneddon's Syndrome (SNS) is a rare condition characterized by livedo reticularis and cerebrovascular disease.
- Non-dermatological onset, particularly cognitive impairment, is exceptionally uncommon in SNS.
Observation:
- A 32-year-old woman presented with progressive topographic disorientation, confirmed by neuropsychological testing.
- Initial investigations including blood tests, cerebrospinal fluid analysis, and cerebral MRI were normal.
- Technetium-99m-ECD-SPECT scan revealed bilateral parietal hypoperfusion.
Findings:
- Fourteen months later, the patient developed diffuse livedo reticularis.
- Follow-up MRI showed frontoparietal cortical-subcortical abnormalities indicative of arterial ischemic infarctions.
- Diagnosis of Sneddon's Syndrome was established based on clinical and imaging findings.
Implications:
- This case highlights the possibility of Sneddon's Syndrome presenting solely with cognitive deficits in its early stages.
- It underscores the importance of considering SNS in patients with unexplained cognitive impairment, even with normal initial MRI.
- Early detection through advanced imaging like SPECT may aid in timely intervention for Sneddon's Syndrome.
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