ABSTRACT
Sneddon syndrome (SS) is characterized by livedo racemosa, recurrent ischemic strokes, and often progressive vascular dementia. Treatment options for SS center on either anticoagulation or immunosuppression to prevent strokes and to dissipate the skin findings, with these modalities based historically on the presence or absence of antiphospholipid antibodies (APA) respectively. However, few effective treatments have been reported to reverse the cognitive decline in SS. We report a case of a woman with seronegative SS (absence of APA) with cognitive decline who demonstrated objective and subjective improvements in her memory and emotional functioning after treatment with cyclophosphamide.
Subject(s)
Cyclophosphamide/therapeutic use , Emotions/drug effects , Immunosuppressive Agents/therapeutic use , Memory/drug effects , Nervous System Diseases/drug therapy , Nervous System Diseases/psychology , Sneddon Syndrome/drug therapy , Sneddon Syndrome/psychology , Attention/drug effects , Cerebral Angiography , Executive Function/drug effects , Female , Humans , Injections, Intravenous , Learning , Magnetic Resonance Angiography , Magnetic Resonance Imaging , Male , Nervous System Diseases/etiology , Neuropsychological Tests , Skin Diseases/drug therapy , Skin Diseases/etiology , Sneddon Syndrome/complications , Verbal LearningABSTRACT
Sneddon's syndrome is a rare disease defined by the presence of ischemic cerebrovascular events associated with livedo reticularis. We report a retrospective study of fifteen cases, thirteen women and two men, mean age of 37.93+/-9.77 years. All patients presented one or more cerebral infarcts. Six patients had dementia. Brain magnetic resonance imaging showed several cortical infarcts with white matter involvement. Cerebral angiography performed in all patients, showed a distal arteriopathy in twelve and thrombosis of the right carotid internal artery in one. One patient had antiphospholipid antibodies. Ten patients were treated with antiplatelet agents and five with anticoagulants. The course was favorable in eight patients and stationary in three. Four patients had several recurrent infarcts, one when anticoagulants were discontinued, one taking an anti-sludge-platelet agent and two who were not initially taking any treatment.
Subject(s)
Sneddon Syndrome/pathology , Adult , Antibodies, Antiphospholipid/analysis , Anticoagulants/therapeutic use , Carotid Artery, Internal/pathology , Cerebral Angiography , Cerebral Infarction/etiology , Cerebral Infarction/pathology , Cerebral Infarction/prevention & control , Dementia/etiology , Female , Humans , Intracranial Thrombosis/etiology , Intracranial Thrombosis/pathology , Intracranial Thrombosis/prevention & control , Magnetic Resonance Angiography , Male , Middle Aged , Platelet Aggregation Inhibitors/therapeutic use , Recurrence , Retrospective Studies , Sneddon Syndrome/drug therapy , Sneddon Syndrome/psychologyABSTRACT
A 24-year-old man with livedo racemosa and psychiatric disturbances, manifesting as low intelligence (IQ 80) and delusions, had anti-cardiolipin antibody and showed shortening of the fingers and toes. A skin biopsy of the livedo lesion revealed endoarteritis obliterans, being compatible with Sneddon's syndrome. MRI of the brain demonstrated multiple infarction and moderate cortical atrophy. A single photon emission tomography of the brain showed a marked reduction of the blood flow in the front-temporal lobe. These findings might relate to the psychiatric disturbance. After intravenous administration of cyclophosphamide and the start of oral prednisolone, the anti-cardiolipin antibody level decreased and his physical condition improved. However, a low dose of haloperidol is still necessary to maintain his mental condition.
Subject(s)
Sneddon Syndrome/diagnosis , Adult , Antibodies, Anticardiolipin/blood , Brain/pathology , Delusions , Fingers/pathology , Humans , Magnetic Resonance Imaging , Male , Skin/pathology , Sneddon Syndrome/pathology , Sneddon Syndrome/psychology , Toes/pathologySubject(s)
Brain/pathology , Dementia/etiology , Sneddon Syndrome/diagnosis , Adult , Biopsy , Cerebral Angiography , Cerebral Infarction/diagnosis , Diagnosis, Differential , Female , Humans , Magnetic Resonance Imaging , Neuropsychological Tests , Sneddon Syndrome/physiopathology , Sneddon Syndrome/psychologyABSTRACT
The results of a neurological, neuropsychological and MRI study of the brain in 21 patients (aged 18-59 years) with Sneddon's syndrome are reported. The predominant findings were marked neuropsychological deficits in two-thirds of the patients. While sensorimotor deficits after stroke in these patients had a good prognosis, neuropsychological deficits persisted. Of the 21 patients, 14 were incapable of gainful employment, 10 because of severe cognitive dysfunction.