Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory disorder of the central nervous system. We describe a previously healthy 2-year-old boy with ADEM, who exhibited high fever, lethargy, and recurrent seizures at 25 days after H1N1 influenza vaccination. To our knowledge, there has been only one report of ADEM following the 2009 H1N1 influenza vaccine, although such vaccination is accompanied with optic neuritis apart from this case. Thus, this is the first case of ADEM without optic neuritis, following the 2009 H1N1 influenza vaccination. Although vaccine-associated ADEM remains rare, the increasing number of influenza vaccinations might increase the incidence of ADEM. We still need to pay attention to the occurrence of ADEM and treat patients with steroid therapy.
"None NS Recovered completely Yang et al. 2010  4 40 Y, M Infection ADEM 1 m None Oseltamivir Valproic acid Plasmapheresis Methylprednisolone Severely disabled state Fugate et al. 2010  5 38 Y, M Vaccination ADEM 10 d None Methylprednisolone Recovered completely Denholm et al. 2010  6 19 Y, F Vaccination ADEM 21 d None Methylprednisolone Persistent paresthesia Denholm et al. 2010  7 22 Y, M Infection ADEM 3 d None J-globulins Methylprednisolone Oseltamivir Recovered completely Wang et al. 2011  8 77 Y, F Vaccination TM 1 d Rectal cancer Immunoglobulins Methylprednisolone Severe neurological damage Sato et al. 2011  9 5 Y, M Vaccination ADEM 2 d None Corticosteroids Unknown Fernandes and Marchiori 2011  10 14 Y, M Infection TM 2 w None NS Recovered completely Landau et al. 2011  11 6 Y, M Infection ADEM 2 d None Oseltamivir Immunoglobulins Acyclovir Midazolam Phenytoin Recovered completely Yildizdas et al. 2011  12 34 m, M Vaccination ADEM 5 d None Dexamethasone Recovered completely Lee et al. 2011  13 13 Y, M Vaccination TM 6 d None Methylprednisolone Physical therapy Acupuncture (Moderate??) neurological damage Gui et al. 2011  14 44 Y, M Vaccination TM 1 m None Methylprednisolone Recovered completely Korn-Lubetzki et al. 2011  15 ?, F Vaccination TM 4 d None ? Unknown Arcondo et al. 2011  16 36 Y, M Vaccination ADEM & GBS 10 d None Methylprednisolone Persistent mild weakness and numbness of the distal extremities Hoshino et al. 2012  17 60 Y, M Infection ADEM 25 d None Osteltamavir Methylprednisolone 1 g 5 d Prednisolone 60 mg Recovered completely Athauda et al. 2012  18 2 Y, M Vaccination ADEM 25 d None Methylprednisolone Recovered completely Fujii et al. 2012  19 33 Y, F Vaccination ADEM 15 d None Methylprednisolone IV Prednisolone PO Recovered completely Maeda and Idehara 2012  20 7 Y, M Infection ADEM 5 d None Acyclovir Ceftriaxone Oseltamivir Methylprednisolone Prednisone Immunoglobulin IV Anarthria Quadriparesis Ozkale et al. 2012  21 52 Y, M Vaccination TM 7 d None ? Vieira et al. 2012  22 26 Y, F Vaccination ADEM 5 d Hypertension Acyclovir Corticosteroids Plasmapheresis Decompressive craniotomy Drainage of cerebrospinal fluid Persistent vegetative state Van Ussel et al. (This Study) "
[Show abstract][Hide abstract] ABSTRACT: Objectives:
To illustrate that acute, even dramatic, demyelination of the central nervous system and encephalitis can occur after viral, i.e., influenza A/H1N1 vaccination or infection.
Patients and methods:
We describe a case of encephalitis/acute disseminated encephalomyelitis associated with vaccination against influenza A/H1N1 and review the available literature.
We report a case of a 26-year-old female who developed symptoms of acute encephalitis 5 days after vaccination against the pandemic 2009 A/H1N1 influenza. MRI of the brain showed confluent T2-hyperintense signal intensity changes in the deep white matter which further confirmed the diagnosis of encephalitis/acute disseminated encephalomyelitis. Despite therapy with immunoglobulins and corticosteroids, her persistent vegetative state continued. In light of the dramatic cause of this case, we reviewed all 21 other previously reported cases of central nervous system demyelination related to H1N1 vaccination and/or infection.
The available data suggest that even severe central nervous system demyelination i.e. acute encephalitis/disseminated encephalomyelitis and transverse myelitis may very rarely be associated with vaccination against novel influenza A/H1N1 or with A/H1N1 infection itself.
[Show abstract][Hide abstract] ABSTRACT: A wide variety of inflammatory diseases temporally associated with the administration of various vaccines, has been reported in the literature. A PubMed search from 1979 to 2013 revealed seventy one (71) documented cases. The most commonly reported vaccinations that were associated with CNS demyelinating diseases included influenza (21 cases), human papilloma virus (HPV) (9 cases), hepatitis A or B (8 cases), rabies (5 cases), measles (5 cases), rubella (5 cases), yellow fever (3 cases), anthrax (2 cases), meningococcus (2 cases) and tetanus (2 cases). The vast majority of post-vaccination CNS demyelinating syndromes, are related to influenza vaccination and this could be attributed to the high percentage of the population that received the vaccine during the HI1N1 epidemia from 2009 to 2012. Usually the symptoms of the CNS demyelinating syndrome appear few days following the immunization (mean: 14.2 days) but there are cases where the clinical presentation was delayed (more than 3 weeks or even up to 5 months post-vaccination) (approximately a third of all the reported cases).
In terms of the clinical presentation and the affected CNS areas, there is a great diversity among the reported cases of post-vaccination acute demyelinating syndromes. Optic neuritis was the prominent clinical presentation in 38 cases, multifocal disseminated demyelination in 30, myelitis in 24 and encephalitis in 17. Interestingly in a rather high proportion of the patients (and especially following influenza and human papiloma virus vaccination-HPV) the dominant localizations of demyelination were the optic nerves and the myelon, presenting as optic neuritis and myelitis (with or without additional manifestations of ADEM), reminiscent to neuromyelitic optica (or, more generally, the NMO-spectrum of diseases). Seven patients suffered an NMO-like disease following HPV and we had two similar cases in our Center. One patient with post-vaccination ADEM, subsequently developed NMO.
Overal, the risk of a demyelinating CNS disease following vaccination, although non-negligible, is relatively low. The risk of onset or relapse of CNS demyelination following infections against which the vaccines are aimed to protect, is substantially higher and the benefits of vaccinations surpass the potential risks of CNS inflammation. This does not in any way exempt us from “learning” the lessons taught by the reported cases and searching new and safer ways to improve vaccination techniques and increase their safety profile.
[Show abstract][Hide abstract] ABSTRACT: Objective:
To describe the clinical and neuroimaging features of a young female patient with acute disseminated encephalomyelitis associated with anti-aquaporin-4 antibodies.
The patient had mild encephalopathy 14 days after influenza vaccination. Cerebrospinal fluid analysis revealed an increased cell count and a marked increase in myelin basic protein. Magnetic resonance imaging (MRI) demonstrated multiple lesions in the juxtacortical white matter. The patient was diagnosed with acute disseminated encephalomyelitis and treated with methylprednisolone pulse therapy. She recovered in 1 month. However, right retrobulbar optic neuritis appeared 2 months after discharge, and serum anti-aquaporin 4 antibodies were measured with a cell-based assay.
Anti-aquaporin 4 antibodies were present in the patient's serum. She was treated with a prolonged course of oral prednisolone. The patient was negative for serum anti-aquaporin 4 antibodies 8 months after the second clinical event, and prednisolone was discontinued 13 months after the second clinical event. Serum anti-aquaporin 4 antibodies remained negative 4 months after the discontinuation of prednisolone. There was no evidence of relapse at 9 months after discontinuation of steroids.
This case will expand the spectrum of anti-aquaporin-4 antibody-related central nervous system disorders. The measurement of anti-aquaporin 4 antibody may be considered in patients with a clinical diagnosis of acute disseminated encephalomyelitis and a second clinical event within a short interval.
Brain and Development 05/2014; 37(3). DOI:10.1016/j.braindev.2014.04.013 · 1.88 Impact Factor
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