Hurst Disease: A Case Report and Literature Review

1UMass Memorial Medical Center, Worcester, MA

Meeting: Hospital Medicine 2011, May 10-13, Dallas, Texas.

Abstract number: 429

Case Presentation:

A 32‐year‐old woman presented to the emergency department with 2 days of diplopia, worsening headache, pharyngitis, and lethargy. She also reported at that time 1 month of abdominal pain, nausea, vomiting, and diarrhea. She denied any recent travel, sick contacts, or chemical exposure. On the evening of admission, she described dysphagia and hydrophobia and was subsequently noted to be delirious with an abnormal neurological exam. Physical exam was notable for: asymmetric pupils, the left pupil was not reactive to light, and she had left eye ptosis as well as an asymmetric facial grimace with the left side being weak. CT scan of the head demonstrated an enlarged right lateral ventricle, the midline was displaced to the left by 3 mm, and there was concern for tonsillar her‐niation. After the findings of the CT were reported, she was intubated for airway protection and a frontal ventriculostomy was performed. Subsequent imaging showed hemorrhage in the right lateral ventricle, right basal gangliathalamic region, and ventricular system. Brain biopsy revealed acute necrosis and inflammation, multifocal hemorrhages and necrotizing vasculitis. Serology, cerebrospinal fluid, and tissue specimens were negative for any acute infectious process. Despite optimal medical management and surgical decompression, the patient developed excessively elevated intracranial pressure and was declared brain dead on day 11 of hospitalization. Autopsy showed severe hemorrhagic necrosis of the right medial basal ganglia, thalamus, and choroid plexus. Microscopic examination showed necrotizing vasculitis of small veins and venules, fibrinoid necrosis, and mononuclear infiltrate as well as ring‐and‐ball hemorrhages surrounding the affected vessels. These findings are consistent with a diagnosis of Hurst disease.

Discussion:

Hurst disease, also known as acute hemorrhagic leukoencephalitis, is a rare disease of the central nervous system. It is a rapidly progressive severe inflammatory process that leads to neurological deficits and respiratory depression. The disease may be preceded by an upper respiratory tract infection, Epstein–Barr virus, or Mycoplasma infection. The pathogenesis is thought to be an intense immune response to the antigens present, which leads to central nervous system (CNS) demyelination. Diagnosis is usually made by CT or MRI of the brain, which shows a mass lesion in the white matter with increased intracranial pressure. Pathological findings are characteristic and include fibrinoid necrosis and confluent ring‐and‐ball hemorrhages surrounding affected vessels, and demyelination surrounding scattered affected vessels. Early recognition of the disease and initiation of therapy is very important to increase the chance of survival. Systemic corticosteroids and plasma exchange therapy have shown promising results.

Conclusions:

Hurst Disease is a rare disease of the CNS that requires early diagnosis and prompt initiation of treatment to optimize chances of survival. The purpose of this abstract is to increase general awareness of this fatal disease.

Disclosures:

S. Wheeler ‐ none; P. Georges ‐ none

To cite this abstract:

Wheeler S, Georges P. Hurst Disease: A Case Report and Literature Review. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 429. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/hurst-disease-a-case-report-and-literature-review/. Accessed September 18, 2019.

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