Case Report: Clinical, MRI Imaging and Histopathology of Acute Necrotizing Hemorrhagic Leukoencephalitis (ANHLE) (P04.013) (original) (raw)

Neurology, 2013

Abstract

OBJECTIVE: To describe clinical, imaging, histopathological characteristics, treatment options and outcome in a patient with ANHLE. BACKGROUND: ANHLE is a rare often fatal disease and few cases with histopathological and MRI correlation has been reported in literature. ANHLE can be hyper acute frequently fatal form of ADEM. The etiology and pathogenesis of this disease remain unknown. Although some viruses have been reported as causative agents, it is now believed that this disease is likely immune-mediated or metabolic. DESIGN/METHODS: A previously healthy 29 year old Syrian left handed male lived in Dubai 4 month prior to presentation as an emergency with few days9 history of ophthalmoplegia, ataxia, and generalized tonic colonic seizure and progressed rapidly to coma and quadriplegia and subsequently required intubation and mechanical ventilation. RESULTS: Initial MRI of the Brain showed bilateral basal ganglia, thalamic and brainstem well-defined T2 high signals and T1 mixed signals non enhancing lesions, and multiple smaller superficial white matter similar lesions involving parietal regions. Further extensive diagnostic workup with metabolic, hematologic, hormonal, vasculitic, para neoplastic, viral, neurophysiology and immunology studies was negative. CSF analysis was repeatedly normal. Brain biopsy and histopathological studies performed by neuropathologist in UK showed necrotizing grey matter lesions within sulcal cortex accompanied by capillary proliferation and endothelial hyperplasia and vaculation of white matter . These findings raised many diagnostic possibilities including ANHLE. Patient was initially treated with high dose steroids, followed by IVIG and plasma exchange. Patient recovered but remained with mild residual cognitive dysfunction and limbs spasticity. Follow up MRI showed that the lesions almost disappeared. CONCLUSIONS: Early diagnosis with high index of suspicion for ANHLE is required. Early and aggressive treatment of ANHLE may increase the chances of survival and improve the outcome even before the diagnosis is confirmed with brain biopsy which is often needed. Disclosure: Dr. Inshasi has nothing to disclose. Dr. Almadani has nothing to disclose. Dr. Alrukn has nothing to disclose. Dr. Noori has nothing to disclose. Dr. Abdool has nothing to disclose. Dr. Thakre has nothing to disclose.

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