Coma in thrombotic thrombocytopenic purpura

Department of Neurology, Erasmus Medical Center, Rotterdam, The Netherlands.
Case Reports 11/2010; 2010(nov22 1). DOI: 10.1136/bcr.06.2010.3113
Source: PubMed


Thrombotic thrombocytopenic purpura (TTP) is characterised by a thrombotic, haemolytic microangiopathy leading to microvascular occlusion, haemolysis and ischaemic dysfunction of various organs including the brain. TTP may present with a variety of neurological symptoms, including headache, focal deficits, seizures and coma. The authors describe a 55-year-old man presenting with abdominal pain and rapidly progressive deterioration into coma without focal neurological deficits or seizures. A concomitant, transient, rapid increase in blood pressure raised the suspicion of a hypertensive crisis. Yet, our patient did not improve after vigorous treatment with antihypertensives. Brain imaging excluded a hypertensive leucoencephalopathy. Despite the absence of a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13) deficiency, the diagnosis idiopathic TTP was made after excluding secondary causes of TTP. Upon treatment with plasma exchange, corticosteroids and vincristin our patient gradually improved. On discharge to a rehabilitation centre he was awake and alert, had minor cognitive deficits and a mild proximal tetraparesis consistent with a critical illness poly(neuro)myopathy.

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    ABSTRACT: Thrombotic thrombocytopenic purpura (TTP, Moschcowitz disease) is characterized by thrombotic microangiopathy leading to microvascular occlusion and ischemic dysfunction of various organs including the brain. In the course of the rare disease most patients develop neurological symptoms of varying severity and characteristics. The case presented is that of a 34-year-old female patient with profound thrombocytopenia, anemia and rapidly progressive neurological deterioration into coma with normal result of brain imaging. TTP was recognized on the basis of hematological analysis. The initiated steroid therapy and plasma exchange failed to prevent the turbulent course of disease in the patient, who died exhibiting symptoms of multiple organ failure caused by thrombotic microangiopathy. TTP remains to be a diagnostic challenge, particularly in the case of atypical symptoms or when neuroimaging and laboratory results are inconclusive. Before using the corticosteroids and plasma exchange, TTP had a case fatality rate of approx. 90% (Podolak-Dawidziak, 2013). Nowadays recovery is possible when vigorous treatment is introduced early in the course of this disease.
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