Sneddon's Syndrome presenting with topographic disorientation.

[sneddon syndrome]

A 32 -year -old woman was admitted to our department for a progressive difficulty in topographic orientation , confirmed by an extensive battery of neuropsychological tests . All biochemical and immunological examinations of blood and cerebrospinal fluid and a cerebral MRI were normal , but a technetium-99 m-ethyl cysteinate dimer-single photon emission CT (Tc-99 m ECD -SPECT ) scan showed bilateral parietal hypoperfusion . The patient refused to undergo other examinations , but 14 months later she returned to hospital for diffuse cutaneous livedo reticularis over her trunk and legs . This time the MRI showed small frontoparietal cortical -subcortical abnormalities suggestive of arterial ischemic infarctions . We made a diagnosis of Sneddon 's Syndrome (SNS ). SNS is characterized by the association of livedo reticularis and cerebrovascular disease ; non-dermatological onset is extremely uncommon . To our knowledge , this is the first description of a patient presenting with cognitive impairment only and observed at such an early stage of the disease that a cerebral MRI was normal .

Diseases
Validation

Diseases presenting "small frontoparietal" symptom

sneddon syndrome

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