Acute Hemorrhagic Encephalomyelitis Research Articles

Bilateral Cerebellar Hemorrhages: An Atypical Presentation of Acute Hemorrhagic Encephalomyelitis

Bilateral Cerebellar Hemorrhages: An Atypical Presentation of Acute Hemorrhagic Encephalomyelitis

Reply to Kim et al. Acute Hemorrhagic Encephalomyelitis in the Context of MOG Antibody-Associated Disease. Comment on “Chen et al. Rapid Progressive Fatal Acute Hemorrhagic Encephalomyelitis. Diagnostics 2023, 13, 2481”

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Acute Hemorrhagic Encephalomyelitis in the Context of MOG Antibody-Associated Disease. Comment on Chen et al. Rapid Progressive Fatal Acute Hemorrhagic Encephalomyelitis. Diagnostics 2023, 13, 2481.

The study by Chen et al. of a 56-year-old man diagnosed with acute hemorrhagic encephalomyelitis (AHEM) had a significant impact on us. The authors provided a comprehensive account of their diagnostic journey and emphasized the need to differentiate myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) from AHEM. However, recent research suggests that AHEM may not be an isolated entity, but rather a phenotype within MOGAD. The patient's clinical presentation included MRI brain lesions characteristic of MOGAD in addition to hemorrhagic abnormalities. These findings raise the possibility that AHEM in this case represents a MOGAD phenotype. In conclusion, it is important to recognize the potential association between AHEM and MOGAD, especially when distinct MOGAD brain MRI patterns are present, as in this case.

Rapid Progressive Fatal Acute Hemorrhagic Encephalomyelitis.

Acute hemorrhagic encephalomyelitis (AHEM) is the most severe form of acute disseminated encephalomyelitis (ADEM). Patients with AHEM usually have unfavorable outcomes with high mortality rate. We reported a middle-aged male, who was diagnosed with AHEM and died 35 days after admission even under intensive immune therapy. Clinical courses were recorded and serial MR images were demonstrated to illustrate the rapidly changes in brain parenchyma. By highlighting these aspects, we hope to provide valuable insights for future studies and potential advancements in the management of AHEM.

Acute hemorrhagic encephalitis in pediatric patient: a case report

Acute hemorrhagic encephalomyelitis (AHEM) is considered a rare presentation of acute disseminated encephalomyelitis (ADEM) and is challenging to manage. ADEM is a rare illness with an incidence of 0.2–0.4 per 100,000 children annually. Here we discuss a case report of a 3-year-old patient who presented with the clinical features of ADEM. On neuroimaging patient had a rare form of ADEM as hemorrhagic encephalitis. AHEM incidence is very low in the pediatric age group with poor prognosis. With the use of immunosuppressants and immunoglobulin, the survival of the patient was possible.

Case Series: Acute Hemorrhagic Encephalomyelitis After SARS-CoV-2 Vaccination

We present three cases fulfilling diagnostic criteria of hemorrhagic variants of acute disseminated encephalomyelitis (acute hemorrhagic encephalomyelitis, AHEM) occurring within 9 days after the first shot of ChAdOx1 nCoV-19. AHEM was diagnosed using magnetic resonance imaging, cerebrospinal fluid analysis and brain biopsy in one case. The close temporal association with the vaccination, the immune-related nature of the disease as well as the lack of other canonical precipitating factors suggested that AHEM was a vaccine-related adverse effect. We believe that AHEM might reflect a novel COVID-19 vaccine-related adverse event for which physicians should be vigilant and sensitized.

Acute hemorrhagic encephalomyelitis – Rare presentation of pediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2: A case report

Acute hemorrhagic encephalomyelitis (AHEM) is a rare form of acute disseminated encephalomyelitis (ADEM) characterized by a fulminant course, poor response to immunomodulation, and unfavorable outcomes. Early clinical and radiological suspicion can enable aggressive use of immunotherapy, thereby improving outcomes. Pediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a novel disease, with protean clinical manifestations being reported from all over the world. Although multiple neurological manifestations of PIMS-TS have been reported previously, AHEM has been less commonly reported. We describe a case of PIMS-TS with AHEM and highlight very rare presentation of this novel disease.

Clinical study of children with cryofibrinogenemia: a retrospective study from a single center

BackgroundThis study aimed to evaluate the demographic, clinical features, laboratory data, pathology and other survey in pediatric patients with cryofibrinogenemia.MethodsA 12-year retrospective chart review identified eight pediatric patients at Mackay Memorial Hospital, Taipei, Taiwan.ResultsThe female-to-male ratio was 3:1. The mean age at symptom onset and of diagnosis was 10.3 ± 4.6 years and 12.3 ± 4 years, respectively. One child (12.5%) had primary cryofibrinogenemia. The common symptoms were purpura, arthralgia, and muscle weakness (100%). On laboratory examination, cryofibrinogen was positive in all patients. All patients had increased anti-thrombin III while 87.5% and 62.5% had abnormal protein S and protein C, respectively. All eight also complained of neurologic symptoms. One had vertebral artery narrowing, two showed increased T2-weighted signal intensity on the thalamus or white matter, and one had acute hemorrhagic encephalomyelitis on brain magnetic resonance imaging.ConclusionsThis study reports on the presentations of cryofibrinogenemia, which is rare in children. Most cases are associated with autoimmune disease and have severe and complex presentations. Central nervous system involvement is common.

A Case of Acute Hemorrhagic Encephalomyelitis associated with Mycoplasma Pneumoniae Infection

A Case of Acute Hemorrhagic Encephalomyelitis associated with Mycoplasma Pneumoniae Infection

Acute Hemorrhagic Encephalomyelitis Following Pneumococcal Meningitis in a 19 Years Old Girl

Acute Hemorrhagic Encephalomyelitis Following Pneumococcal Meningitis in a 19 Years Old Girl

P.067 Steroid responsive life threatening acute hemorrhagic encephalomyelitis (AHEM) in a child with sickle cell disease (SCD)

Background: AHEM is a rare form of acute disseminated encephalomyelitis (ADEM) characterized by fulminant encephalopathy with hemorrhagic necrosis and most often fatal outcome. Methods: A case report and review of literature. Results: A 6-year-old girl known SCD presented an acute demyelinating syndrome (ADS) with diplopia due to unilateral fourth nerve palsy. She received (20mg/kg/day for 5 days) of IVMP (intravenous methylprednisolone). Two weeks after steroid weaning, she presented right hemiplegia. Brain MRI showed a left frontal necrotico-hemorrhagic lesion and new areas of demyelination. She showed signs of herniation and underwent craniotomy. Investigations ruled out vascular and infectious process in both events. The deterioration stopped concomitantly to the initiation of IVMP. She was discharged with a longer oral wean. Later, she was diagnosed with Crohn’s disease and primary sclerosing cholangitis. We considered AHEM as the most plausible diagnosis based on the clinical and radiological presentation, the preceding ADS, the exclusion of other etiologies, and the response to IVMP including resolution of non-necrotic lesions. Similar - but fatal - AHEM has been reported in 2 SCD patients. Conclusions: In any ADS occurring in the context of SCD and/or auto-immune condition, we recommend to slowly wean steroids, and to closely monitor the patient after weaning to quickly treat any recurrence with IVMP.

Acute hemorrhagic encephalopathy - diagnostic challenges in a pediatric case

Background Acute hemorrhagic encephalomyelitis (AHEM) is considered a rare form of Acute disseminated encephalomyelitis (ADEM), due to acute cerebral vasculitis. The symptomatology consists in similar neurological findings, (meningismus, headache, seizures, multifocal neurologic signs, asymmetrical neurological deficits and coma) with rapid onset of encephalopathy and biphasic evolution. Although previous respiratory disease was registered days before this condition, establishing etiology is quite a challenge, viruses being incriminated.

Pediatric Inflammatory Diseases

Acute post-infectious immune disorders include Acute Disseminated Encephalomyelitis (ADEM) and its variants such as Acute Hemorrhagic Encephalomyelitis (AHEM), acute necrotizing hemorrhagic leukoencephalitis (ANHLE) of Weston Hurst, multiphasic and recurrent ADEM. Acute Necrotizing Encephalopathy of Childhood (ANE or ANEC) represents a dramatic event, consequent to viral infections, especially Influenza-A, and is now considered different from ADEM. ADEM and variants are classically described as uniphasic syndrome occurring in association with an immunization or vaccination (postvaccine encephalomyelitis) or systemic viral infection (parainfectious encephalomyelitis). However, multiphasic forms are not rare. Pathologically, there is perivascular inflammation, edema, and demyelination within the CNS. Clinical features are focal or multifocal neurologic disorder following exposure to virus or receipt of vaccine. The onset of the CNS disorder is usually rapid and include encephalopathy ranging from lethargy to coma, seizures, and focal and multifocal signs reflecting cerebral and spinal cord involvement. The mortality rate is estimated at 10 to 30 percent, with complete recovery rates of 50 percent cited. Poor prognosis is correlated with severity and abruptness of onset of the clinical syndrome. Multifocal CNS lesions are generally evident on MRI that can be similar from those observed in MS.

Pathology of acute disseminated encephalomyelitis

Abstract Acute disseminated encephalomyelitis (ADEM) is an acute, monophasic neurologic syndrome that occurs after vaccination against various viruses and after many viral infections and rarely occurs again in the same patient. Weston Hurst disease or acute hemorrhagic encephalomyelitis (AHE) is a hyperacute ADEM variant that shares many pathological similarities with ADEM. ADEM clinically and neuropathologically faithfully reflects the animal model of experimental allergic encephalomyelitis (EAE), and animal studies have provided us with new insights into pathogenesis of this disorder. Although there is much controversy whether ADEM is a separate disease or just one of possible manifestations of multiple sclerosis, there are clear histopathological characteristics that support ADEM as a separate disease This mini review will focus on pathological characteristics of ADEM emphasizing differences from other types of idiopathic demyelinating diseases.

Acute hemorrhagic encephalomyelitis in childhood: Case report and literature review

Acute disseminated encephalomyelitis (ADEM) is an inflammatory immune-mediated disorder which is more common in pediatric patients. The clinical setting is characterized by a rapid onset of encephalopathy and multifocal neurological features. Acute hemorrhagic encephalomyelitis (AHEM) is considered a rare form of ADEM. This report shows a 2-year-old patient who presented with the classical features of ADEM and after 8 weeks developed severe neurological worsening. The second magnetic resonance image (MRI) showed hemorrhagic lesions. Differences in prognosis between ADEM and AHEM justify the investigation of AHEM whenever a patient has neurological recrudescence in a known patient of ADEM.

Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients.

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the CNS. Few pediatric series have been published, with retrospective and short-term follow-up studies. To describe a cohort of pediatric patients with ADEM and to determine whether clinical and neuroimaging findings predict outcome. A prospective study was conducted between March 1988 and July 2000 on 84 consecutive children with ADEM at the National Pediatric Hospital "Dr. J. P. Garrahan." Mean age at onset was 5.3 +/- 3.9 years, with a significant male predominance. Sixty-two patients (74%) had a preceding viral illness or vaccination. Acute hemiparesis (76%), unilateral or bilateral long tract signs (85%), and changes in mental state (69%) were the most prominent presenting features. Four MRI groups were identified: ADEM with small lesions (62%), with large lesions (24%), with additional bithalamic involvement (12%), and acute hemorrhagic encephalomyelitis (2%). Of the 54 children whose CSF samples were analyzed, none showed intrathecal oligoclonal bands. The use of high-dose corticosteroid treatment, particularly IV methylprednisolone, was associated with good recovery and resolution of MRI lesions. After a mean follow-up of 6.6 +/- 3.8 years, 90% of children showed a monophasic course, and 10% a biphasic disease. Eighty-nine percent of patients show at present Expanded Disability Status Scale scores of 0 to 2.5. Eleven percent have disability scores of 3 to 6.5. Childhood acute disseminated encephalomyelitis is a benign condition, affecting boys more frequently. No association was found between MRI groups and disability. Disability was related to optic nerve involvement at presentation. Even in relapsing cases, the distinction between acute disseminated encephalomyelitis and MS was possible on the basis of long-term clinical and neuroimaging follow-up and the absence of oligoclonal bands in CSF.