open access

Vol 48, No 4 (2014)
Case reports
Submitted: 2014-04-05
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Acute neurological symptoms of Moschcowitz disease—Case report

Anetta Lasek-Bal, Katarzyna Kosarz-Lanczek, Zofia Kazibutowska
DOI: 10.1016/j.pjnns.2014.07.002
·
Neurol Neurochir Pol 2014;48(4):296-298.

open access

Vol 48, No 4 (2014)
Case reports
Submitted: 2014-04-05

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.

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Keywords

Neurologic sign, Moschcowitz disease, Thrombotic purpura

About this article
Title

Acute neurological symptoms of Moschcowitz disease—Case report

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 48, No 4 (2014)

Pages

296-298

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

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