title: Acute neurological symptoms of Moschcowitz disease - case report.
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.
authors.affiliation: Department of Neurology, Medical University of Silesia Hospital No. 7, Professor Leszek Giec Upper Silesian Medical Centre, Katowice, Poland. Electronic address: alasek@gcm.pl.
authors.forename: Anetta
authors.initials: A
authors.lastname: Lasek-Bal
authors.affiliation: Department of Neurology, Medical University of Silesia Hospital No. 7, Professor Leszek Giec Upper Silesian Medical Centre, Katowice, Poland.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Kosarz-Lanczek
authors.affiliation: Department of Neurology, Medical University of Silesia Hospital No. 7, Professor Leszek Giec Upper Silesian Medical Centre, Katowice, Poland.
authors.forename: Zofia
authors.initials: Z
authors.lastname: Kazibutowska
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D009102:Multiple Organ Failure / Q000209:etiology*; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D013256:Steroids / Q000627:therapeutic use*
chemical_list: D013256:Steroids
keywords: Moschcowitz disease; Neurologic sign; Thrombotic purpura