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Acute neurological symptoms of Moschcowitz disease - case report

Anetta Lasek-Bal1, Katarzyna Kosarz-Lanczek1, Zofia Kazibutowska1

  • 1Department of Neurology, Medical University of Silesia Hospital No. 7, Professor Leszek Giec Upper Silesian Medical Centre, Katowice, Poland.

Neurologia I Neurochirurgia Polska
|August 30, 2014
PubMed

Insights

Thrombotic thrombocytopenic purpura (TTP) is a rare clotting disorder causing organ damage. Early diagnosis and treatment are crucial for survival, as TTP can rapidly progress to fatal outcomes.

Area of Science:

  • Hematology
  • Neurology
  • Pathology

Background:

  • Thrombotic thrombocytopenic purpura (TTP), also known as Moschcowitz disease, is a rare thrombotic microangiopathy.
  • TTP causes microvascular occlusion and ischemic dysfunction, frequently affecting the brain and leading to neurological symptoms.

Observation:

  • A case of a 34-year-old female with severe thrombocytopenia, anemia, and rapid neurological decline into coma is presented.
  • Brain imaging was normal, complicating the initial diagnosis of TTP.
  • Despite prompt steroid therapy and plasma exchange, the patient experienced a fatal course with multiple organ failure.

Findings:

  • TTP diagnosis can be challenging, especially with atypical presentations or inconclusive neuroimaging and laboratory results.
  • The presented case highlights the severe neurological manifestations and high mortality associated with TTP.
  • Hematological analysis was key in diagnosing TTP in this patient.

Implications:

  • TTP requires early and aggressive intervention; historically, its case fatality rate was approximately 90% before modern treatments.
  • Prompt diagnosis and vigorous treatment, including plasma exchange, significantly improve recovery chances in TTP.
  • This case underscores the need for heightened clinical suspicion for TTP in patients with unexplained neurological deterioration and hematological abnormalities.
Keywords:
Moschcowitz diseaseNeurologic signThrombotic purpura

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