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Hemorrhagic Acute Diffuse Encephalomyelitis associated with Bordetella pertussis
Last modified: 2014-04-03
Abstract
Introduction:ADEM (Acute Diffuse Encephalomyelitis) is a demyelinating inflammatory white matter disease observed in any age group, especially in children under 10 years. Usually It hás a favorite prognosis, but sometime it can evolve into coma, convulsions and even death, mainly in cases of hemorrhagic ADEM.
Case report: Eight-months-old female, with one week lasting cough, presenting fever and focal seizures. Liquor had 2 leukocytes and 6,800 erythrocytes. She received ceftriaxone, phenobarbital, and phenytoin. Initial neurologic examination was normal. PCR was positive to Bordetella pertussis (BP). Brain CT (Computed Tomography) showed mixed lesions on the right fronto-parietal region, contrast enhancement, without mass effect. One week later, she presented left hemiparesis with no conscious compromise. Brain MRI (Magnetic Resonance Imaging) showed diffuse hypo and hyperintense lesions in grey and white matter, vasogenic edema and middle line deviation. She received pulse therapy with methyl prednisolone, but developed arterial hypertension, bradycardia, and conscious compromise, needing mechanical ventilation. A intracranial hypertension was installed, which was refractory to all therapeutic measures, such as manitol, hypersaline solution, dexamethasone, barbituric coma. Fifteen days later initial symptoms, cerebral death was diagnosed.
Conclusion: Although usually occurring as an auto-immune response after infectious diseases or post-vaccination procedures, ADEM remain with no clear etiology. Fortunately it is a rare situation. The hemorrhagic form of ADEM is even rarer, but can be lethal. In our case, BP was identified in a case that evolved into hemorrhagic ADEM. The possible cause-effect relation between this infection and neurologic outcome is controversial, but plausible.
Case report: Eight-months-old female, with one week lasting cough, presenting fever and focal seizures. Liquor had 2 leukocytes and 6,800 erythrocytes. She received ceftriaxone, phenobarbital, and phenytoin. Initial neurologic examination was normal. PCR was positive to Bordetella pertussis (BP). Brain CT (Computed Tomography) showed mixed lesions on the right fronto-parietal region, contrast enhancement, without mass effect. One week later, she presented left hemiparesis with no conscious compromise. Brain MRI (Magnetic Resonance Imaging) showed diffuse hypo and hyperintense lesions in grey and white matter, vasogenic edema and middle line deviation. She received pulse therapy with methyl prednisolone, but developed arterial hypertension, bradycardia, and conscious compromise, needing mechanical ventilation. A intracranial hypertension was installed, which was refractory to all therapeutic measures, such as manitol, hypersaline solution, dexamethasone, barbituric coma. Fifteen days later initial symptoms, cerebral death was diagnosed.
Conclusion: Although usually occurring as an auto-immune response after infectious diseases or post-vaccination procedures, ADEM remain with no clear etiology. Fortunately it is a rare situation. The hemorrhagic form of ADEM is even rarer, but can be lethal. In our case, BP was identified in a case that evolved into hemorrhagic ADEM. The possible cause-effect relation between this infection and neurologic outcome is controversial, but plausible.
Keywords
Hemorrhagic; Acute Diffuse Encephalomyelitis;Bordetella pertussis
References
Felippe Borlot et al, J Pediatr Neurocis. 2011 jan-jun; 6 (1): 48-51
i.Mader et al, neuropediatrics 2004; 35 (2): 143-146
Relig. Saenz R et al, An Pediatr (Barc). 2013 Apr; 78 (4): 234-40
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