Acute Hemorrhagic Leukoencephalitis: Multimodal Diagnosis and Treatment

We report a case of a previously healthy 26-year-old male, admitted to the intensive care unit due to cerebral edema. The patient was admitted 3 days earlier to the neurological department because of sudden onset of headache and vision disturbances. Brain magnetic resonance imaging (MRI) on admission revealed an extended right parietooccipital lesion with marginal contrast enhancement and extension towards the corpus callosum. The patient quickly deteriorated, became comatose and was intubated and transferred to the intensive care unit. In order to control intracranial hypertension, decompressive craniectomy was decided which also allowed for a brain biopsy. According to clinical, radiological and laboratory data, acute disseminated encephalomyelitis was the working diagnosis. The patient was treated with high dose corticosteroids and plasmapheresis sessions and gradually improved and transferred to a rehabilitation center. Brain biopsy showed acute hemorrhagic leukoencephalitis or Hurst disease, a variant of acute disseminated encephalomyelitis. I N t r O D U c t I O N Acute fulminant brain dysfunction may derive from infectious, inflammatory, vessel-obstructive, vessel-inflammatory, demyelinating, neoplastic, metabolic or toxic processes. Causative spectrum is quite wide to be timely worked up when danger for life is present. Clinical evaluation, blood testing, brain computed tomography and lumbar puncture constitute the standard emergency diagnostic sequence. From the physician’s perspective, narrowing the differential diagnosis to subgroups of entities with common treatment plan is highly desirable. Early recruitment of detailed brain imaging, i.e., brain parenchyma magnetic resonance imaging (MRI) together with arteriography and venography protocols (MRA, MRV) should be viewed as mandatory in this context. On the contrary, specific laboratory investigations in blood and cerebrospinal fluid are often either not available or not informative in the early stage. In cases with ambiguous findings, targeted brain biopsy always remains an option. cAse repOrt Department of Critical Care Medicine and Pulmonary Services, Department of Neurology, Department of Neurosurgery, Laboratory of Pathology, Evagelismos Hospital, Athens, Greece HOSPITAL CHRONICLES 2014, 9(3): 202–207