Sneddon's syndrome: a cause of cognitive decline in young adults.

[sneddon syndrome]

To review the clinical , psychometric , laboratory , and radiologic findings of 6 patients with Sneddon 's syndrome (SS ) who presented with cognitive dysfunction rather than stroke .Case series . All patients fulfilled were diagnosed as SS based on the co -occurrence of livedo racemosa and neurologic disease . Patients presenting with clinical stroke were excluded .Patients presented with cognitive complaints at an early age and all noted skin lesions from 6 months to 10 years before onset of cognitive symptoms . Associated systemic disorders included hypertension and seizures . Laboratory evidence of a hypercoagulable condition was identified in 4 of 6 cases . Brain MRI scans demonstrated atrophy , especially in parieto-occipital regions and cerebral blood flow on brain SPECT scan was reduced in a similar distribution .Patients with SS can develop dementia without antecedent clinical stroke . While the specific pathogenic mechanism of dementia in SS remains speculative , the disease predominantly injures brain tissue in vascular "watershed " territories .

Diseases
Validation

Diseases presenting "seizures" symptom

alexander disease

alpha-thalassemia

cadasil

canavan disease

child syndrome

classical phenylketonuria

coats disease

cohen syndrome

cowden syndrome

erdheim-chester disease

familial hypocalciuric hypercalcemia

familial mediterranean fever

gm1 gangliosidosis

hirschsprung disease

homocystinuria without methylmalonic aciduria

kabuki syndrome

kallmann syndrome

krabbe disease

lamellar ichthyosis

legionellosis

locked-in syndrome

lymphangioleiomyomatosis

malignant atrophic papulosis

monosomy 21

neonatal adrenoleukodystrophy

oligodontia

phenylketonuria

proteus syndrome

pyruvate dehydrogenase deficiency

scrub typhus

sneddon syndrome

wolf-hirschhorn syndrome

x-linked adrenoleukodystrophy

zellweger syndrome

This symptom has already been validated