scholarly journals Hepatic epithelioid hemangioendothelioma associated with acute disseminated encephalomyelitis by a possible paraneoplastic process

Rare Tumors ◽  
2020 ◽  
Vol 12 ◽  
pp. 203636132097701
Author(s):  
Adem Aktas ◽  
Daniel Probst ◽  
Brian Van Tine ◽  
Kathryn Marlow
Keyword(s):  
Paraneoplastic Syndrome ◽  
Brain Biopsy ◽  
Acute Disseminated Encephalomyelitis ◽  
Acute Onset ◽  
Epithelioid Hemangioendothelioma ◽  
Low Grade ◽  
Vascular Neoplasm ◽  
Demyelinating Disorder ◽  
The Central Nervous System ◽  
Work Up

Epithelioid hemangioendothelioma (EHE) is a low-grade, malignant vascular neoplasm that frequently involves the liver, lungs, bone, and soft tissue. Although not commonly associated with a paraneoplastic syndrome, paraneoplastic syndromes in the setting of EHE have been reported. Acute disseminated encephalomyelitis (ADEM) is an acute, autoimmune, demyelinating disorder of the central nervous system that most commonly occurs after an infection or vaccination. We present the case of a 23 year old female who developed the acute onset of fevers, tremors, right sided hemiplegia, global aphasia, and incontinence of urine and stool. MRI demonstrated findings consistent with a demyelinating disorder and brain biopsy confirmed the diagnosis of ADEM. The patient’s work up revealed multiple liver lesions which were biopsy proven EHE. This case report discusses the diagnosis and treatment of two concurrent rare disease processes and the possible association of the processes via a paraneoplastic syndrome.

scholarly journals Acute Disseminated Encephalomyelitis (ADEM): A Demyelinating Disease with Specific Morphological Features

2021 ◽  
pp. 106689692199356
Author(s):  
Fleur Cordier ◽  
Lars Velthof ◽  
David Creytens ◽  
Jo Van Dorpe
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Clinical Case ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
The Central Nervous System

Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated inflammatory and demyelinating disorder of the central nervous system. Its characteristic perivenular demyelination and inflammation aid in the differential diagnosis with other inflammatory demyelinating diseases. Here, we present a clinical case of ADEM, summarize its histological hallmarks, and discuss pitfalls concerning the most important neuropathological differential diagnoses.

Acute Disseminated Encephalomyelitis

2012 ◽  
Vol 27 (11) ◽  
pp. 1408-1425 ◽  
Author(s):  
Gulay Alper
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Protein Levels ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
Elevated Protein ◽  
Demyelinating Lesions ◽  
Diagnostic Difficulties ◽  
The Central Nervous System

Acute disseminated encephalomyelitis is an immune-mediated inflammatory and demyelinating disorder of the central nervous system, commonly preceded by an infection. It principally involves the white matter tracts of the cerebral hemispheres, brainstem, optic nerves, and spinal cord. Acute disseminated encephalomyelitis mainly affects children. Clinically, patients present with multifocal neurologic abnormalities reflecting the widespread involvement in central nervous system. Cerebrospinal fluid may be normal or may show a mild pleocytosis with or without elevated protein levels. Magnetic resonance image (MRI) shows multiple demyelinating lesions. The diagnosis of acute disseminated encephalomyelitis requires both multifocal involvement and encephalopathy by consensus criteria. Acute disseminated encephalomyelitis typically has a monophasic course with a favorable prognosis. Multiphasic forms have been reported, resulting in diagnostic difficulties in distinguishing these cases from multiple sclerosis. In addition, many inflammatory disorders may have a similar presentation with frequent occurrence of encephalopathy and should be considered in the differential diagnosis of acute disseminated encephalomyelitis.

Demyelinating disorders of the central nervous system

2010 ◽  
pp. 4948-4963
Author(s):  
Siddharthan Chandran ◽  
Alastair Compston
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Demyelinating Disorder ◽  
System P ◽  
The Central Nervous System ◽  
Demyelinating Disorders ◽  
Brain And Spinal Cord

Clinicians suspect demyelination when episodes reflecting damage to white matter tracts within the central nervous system occur in young adults. The paucity of specific biological markers of discrete demyelinating syndromes places an emphasis on clinical phenotype—temporal and spatial patterns—when classifying demyelinating disorders. The diagnosis of multiple sclerosis, the most common demyelinating disorder, becomes probable when these symptoms and signs recur, involving different parts of the brain and spinal cord. Other important demyelinating diseases include post-infectious neurological disorders (acute disseminated encephalomyelitis), demyelination resulting from metabolic derangements (central pontine myelinosis), and inherited leucodystrophies that may present in children or in adults. Accepting differences in mechanism, presentation, and treatment, two observations can usefully be made when classifying demyelinating disorders. These are the presence or absence of inflammation, and the extent of focal vs. diffuse demyelination. Multiple sclerosis is prototypic for the former, whereas dysmyelinating disorders, such as leucodystrophies are representative of the latter....

scholarly journals A Case of Vanishing Metastatic Mass: Right Atrial Mass in the Setting of Primary Epithelioid Hemangioendothelioma of the Spine

2018 ◽  
Vol 11 (2) ◽  
pp. 534-540 ◽  
Author(s):  
Eshan Patel ◽  
P. Kancharla ◽  
B.K. Surapaneni ◽  
K. Hennrick ◽  
M. Goldfinger ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Epithelioid Hemangioendothelioma ◽  
The Body ◽  
Right Atrial ◽  
Low Grade ◽  
Vascular Neoplasm ◽  
Right Atrial Mass ◽  
Progression Of Disease ◽  
The Right ◽  
Age And Sex

Epithelioid hemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that is characterized as intermediate between benign hemangioma and high-grade angiosarcoma affecting 1 in 1,000,000 people worldwide. It has been described throughout the body with lung, liver, skin, and bone being the most frequent sites. Primary EHE of the spine has been reported in 56 cases so far with no correlation of age and sex. Our case highlights a rare clinical presentation, etiopathogenesis, diagnosis, and treatment of EHE of the spine with metastasis to the right atrium. This is the first documented case of EHE of the spine with metastatic spread to the heart treated with bevacizumab leading to resolution of the heart metastatic mass. Further studies are warranted to develop a treatment formula for this rare tumor, to consider combination chemotherapy and new adjuvant targeted immunotherapies to prevent progression of disease.

scholarly journals Silent Tumefactive Demyelinating Lesions and Radiologically Isolated Syndrome

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Ozgul Ekmekci ◽  
Cenk Eraslan
Keyword(s):  
Magnetic Resonance Imaging ◽  
Central Nervous System ◽  
Acute Disseminated Encephalomyelitis ◽  
Resonance Imaging ◽  
Demyelinating Disorder ◽  
Radiologically Isolated Syndrome ◽  
Demyelinating Lesions ◽  
The Central Nervous System ◽  
Granulomatous Diseases

Demyelinating lesions larger than 2 cm in diameter, with or without edema, are known as tumefactive demyelinating lesions (TDLs). They constitute a rare inflammatory demyelinating disorder of the central nervous system. TDLs are typically characterized by headaches, cortical symptoms such as aphasia, hemiparesis, hemisensory deficits, seizures, and changes in consciousness. TDLs may occur in patients with or without an established diagnosis of MS or may occur as the initial demyelinating event. They may also be observed during follow-up in patients with MS, neuromyelitis optica, acute disseminated encephalomyelitis, or other autoimmune diseases. Differential diagnosis includes brain tumors, abscess, granulomatous diseases, and vasculitis. In some cases, it may be very difficult to distinguish TDLs from a tumor, such that biopsy might be needed. However, no cases of asymptomatic TDLs have yet been reported in the literature. Hence, in this report, we present a case involving an asymptomatic TDL detected incidentally during magnetic resonance imaging in an 18-year-old man. The patient did not develop any symptoms during the 1-year follow-up period. During follow-up, the patient was diagnosed with a radiologically isolated syndrome. TDLs have not previously been identified as radiologically isolated syndrome. Thus, reporting similar cases in the future will help in further understanding this phenomenon.

scholarly journals Use of Cyclophosphamide in a Child With Fulminant Acute Disseminated Encephalomyelitis

2018 ◽  
Vol 5 ◽  
pp. 2329048X1875463
Author(s):  
Hana Ayed ◽  
Mohammed W. Chaudhary ◽  
Raidah AlBaradie ◽  
Ali Mir
Keyword(s):  
Intravenous Immunoglobulin ◽  
Immunosuppressive Agents ◽  
Acute Disseminated Encephalomyelitis ◽  
High Dose ◽  
Intravenous Methylprednisolone ◽  
First Line ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
Minimal Improvement ◽  
The Central Nervous System

Acute disseminated encephalomyelitis is an immune-mediated inflammatory demyelinating disorder of the central nervous system. The first-line treatment is usually high-dose intravenous methylprednisolone. Intravenous immunoglobulin and plasmapheresis have also shown to be beneficial. Immunosuppressive agents like cyclophosphamide have been used in adults with fulminant acute disseminated encephalomyelitis. We report a case of a 3-year-old boy with fulminant acute disseminated encephalomyelitis. Minimal improvement was seen with high-dose intravenous methylprednisolone, intravenous immunoglobulin, and plasmapheresis. Based on the reports of cyclophosphamide being used successfully to treat adult patients with fulminant acute disseminated encephalomyelitis, we used it in our patient who then showed dramatic and quick improvement. We suggest that if conventional treatment fails, cyclophosphamide could be tried in pediatric patients with fulminant acute disseminated encephalomyelitis.

Acute Disseminated Encephalomyelitis as a Complication of Scrub Typhus in Children

2018 ◽  
Vol 14 (05) ◽  
pp. 264-266
Author(s):  
Soumya Roy ◽  
Subroto Chakrabartty
Keyword(s):  
Medical Literature ◽  
Scrub Typhus ◽  
Acute Disseminated Encephalomyelitis ◽  
Pediatric Case ◽  
Rehabilitation Care ◽  
Demyelinating Disorder ◽  
The World ◽  
The Central Nervous System ◽  
Clinical Pictures

AbstractScrub typhus is a re-emerging threat throughout the world. The spectra of clinical pictures and possible complications are both varied and being increasingly reported. Acute disseminated encephalomyelitis (ADEM) is an infrequent demyelinating disorder of the central nervous system that is precipitated by infections and immunizations. There are only two documented cases of ADEM-associated scrub typhus published in the medical literature. We report the first pediatric case wherein a 5-year-old boy presented with high fever, altered sensorium, seizure, quadriparesis, and neck rigidity. He was ultimately diagnosed as a case of scrub typhus with ADEM. After treatment with intravenous doxycycline and methylprednisolone, the fever subsided but residual neurodeficits, such as inability to sit, stand, and talk, as well as bowel bladder dysfunctions persisted. He was subsequently transferred to long-term rehabilitation care.

scholarly journals Coexistence of Two Rare Sarcomas: Primary Leiomyosarcoma of Bone and Epithelioid Hemangioendothelioma of the Liver

Sarcoma ◽  
2008 ◽  
Vol 2008 ◽  
pp. 1-3 ◽  
Author(s):  
E. Gonzalez-Billalabeitia ◽  
M. Quintela-Fandino ◽  
I. Alemany ◽  
G. López-Alonso ◽  
A. Ruiz-Ollero ◽  
...  
Keyword(s):  
Osteolytic Lesion ◽  
Distal Tibia ◽  
Excisional Biopsy ◽  
Epithelioid Hemangioendothelioma ◽  
Medical Attention ◽  
Low Grade ◽  
Abdominal Ct ◽  
Metastatic Lesions ◽  
Work Up ◽  
Pathological Confirmation

A 33-year-old woman sought medical attention for a painful swelling of the left ankle. Plain radiographs revealed an osteolytic lesion involving the left distal tibia. An excisional biopsy provided the diagnosis of leiomyosarcoma in the tibia. A staging work-up was performed and an abdominal CT showed 4 liver hypodense lesions in both lobes with peripheral contrast enhancement. A liver biopsy confirmed the diagnosis of epithelioid hemangioendothelioma of the liver. No association between these two entities has been described before. This case introduces the importance of the pathological confirmation of apparent metastatic lesions in low grade sarcomas and provides a review of the literature of both tumours.

Central brain herniation secondary to fulminant acute disseminated encephalomyelitis: implications for neurosurgical management

2006 ◽  
Vol 105 (3) ◽  
pp. 472-474 ◽  
Author(s):  
Raymond F. Sekula ◽  
Edward M. Marchan ◽  
Parviz Baghai ◽  
Peter J. Jannetta ◽  
Matthew R. Quigley
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cerebral Edema ◽  
Acute Disseminated Encephalomyelitis ◽  
Small Subset ◽  
Brain Herniation ◽  
Demyelinating Disorder ◽  
Central Brain ◽  
The Central Nervous System ◽  
Neurosurgical Intervention

✓ Acute disseminated encephalomyelitis (ADEM), also known as postinfectious encephalomyelitis, is an immunologically mediated demyelinating disorder affecting the central nervous system that typically occurs after infection or vaccination. The prognosis of ADEM is generally favorable. In a small subset of patients with ADEM, however, fulminant cerebral edema requiring neurosurgical intervention will develop. Few recommendations are available to help the neurosurgeon in dealing with such cases. In this report, the authors present the case of a patient with ADEM in whom central brain herniation developed secondary to medically intractable cerebral edema. The authors review the salient features of the disease and suggest a role for neurosurgeons in cases of fulminant ADEM.

Immunomodulation in Multiple Sclerosis by Phytotherapy

2020 ◽  
Vol 16 (1) ◽  
pp. 28-36
Author(s):  
Maryam Bahrami ◽  
Ghasem Mosayebi ◽  
Ali Ghazavi ◽  
Ali Ganji
Keyword(s):  
Multiple Sclerosis ◽  
Nigella Sativa ◽  
Boswellia Serrata ◽  
Immunomodulatory Agents ◽  
Therapeutic Benefits ◽  
Demyelinating Disorder ◽  
Sensory Impairments ◽  
The Central Nervous System ◽  
Pomegranate Peel Extract ◽  
Peel Extract

Multiple sclerosis is a chronic inflammatory and demyelinating disorder of the central nervous system (CNS) that can cause cognition, mobility, and sensory impairments. Studies have shown that the immune system through inflammation and autoreactive T cells are involved in the progression of MS. The present article aimed to review the potent anti-inflammatory, antioxidant, and immunomodulatory agents that could modulate the immune response in MS. In herbal medicine, various medicinal plants including Olive, Silybum marianum, Grape, Pomegranate peel extract, Nigella sativa, Turmeric, Green tea, Aloysia citrodora, Boswellia papyrifera, Boswellia serrata, Ruta graveolens, and Andrographis paniculata are known with therapeutic benefits in MS patients through immunoregulation and reduction of major symptoms.

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