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Acute neurological symptoms of Moschcowitz disease—Case report
Affiliations- Department of Neurology, Medical University of Silesia Hospital No. 7, Professor Leszek Giec Upper Silesian Medical Centre, Katowice, Poland
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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.
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.
Keywords
Neurologic sign, Moschcowitz disease, Thrombotic purpura
About this articleTitle
Acute neurological symptoms of Moschcowitz disease—Case report
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
296-298
Page views
266
Article views/downloads
349
DOI
10.1016/j.pjnns.2014.07.002
Bibliographic record
Neurol Neurochir Pol 2014;48(4):296-298.
Keywords
Neurologic sign
Moschcowitz disease
Thrombotic purpura
Authors
Anetta Lasek-Bal
Katarzyna Kosarz-Lanczek
Zofia Kazibutowska
