Korean J Med.  1999 Jan;56(1):119-123.

A case of Thrombotic thrombocytopenic purpura - hemolytic uremic syndrome presenting with cortical blindness confirmed by MRI

• Lim GJ
• Kim YE
• Suh KD
• Jeong YS
• Kim JD
• Lee JH
• Koo DY
• Lee TY
• Sin YH
• Park YK

Abstract

Thrombotic thrombocytopenic purpura (TTP) and the related hemolytic uremic syndrome (HUS) are disorders characterized by thrombocytopenia, microangiopathic hemolytic anemia, a variable degree of impairment of renal function and fluctuating neurological symptoms, which are thought to be due to platelet activation and subsequent formation of thrombi in the microcirculation. The fact that there was no clear-cut clinical and laboratory features that differentiate HUS from TTP has lead to view these two syndromes as a clinical continuum. Microvascular thrombosis is the typical lesion and closely related with endothelial injury and platelet activation. Pathologic alterations of the brain parenchyma are mainly manifested by small multiple infarcts. Numerous cases of CNS complications of these syndromes have been evaluated by using CT, but few reports have mentioned the MR findings. We experienced a case of TTP-HUS that had clinical features of cortical blindness and the brain lesion was confirmed by MRI showing cerebral infarct at the occipital area but it was reversible course. So we report this case with a brief review of literature.

Keyword

thrombotic thrombocytopenic purpura; Hemolytic uremic syndrome Cortical blindness; MRI

MeSH Terms

Anemia, Hemolytic
Blindness, Cortical*
Brain
Hemolytic-Uremic Syndrome*
Magnetic Resonance Imaging*
Microcirculation
Platelet Activation
Purpura, Thrombotic Thrombocytopenic*
Thrombocytopenia
Thrombosis