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.

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.

A Case of Multifocal Neurological Symptoms

2018 ◽  
pp. 9-14
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Acute Disseminated Encephalomyelitis ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
High Dose ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Good Recovery ◽  
Related Disease ◽  
Demyelinating Disorder ◽  
Immune Mediated ◽  
Initial Onset ◽  
Adolescents And Adults

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating disorder characterized by multifocal involvement of the CNS and encephalopathy. Diagnostic criteria also require MRI lesions characteristic for demyelination and that new symptoms/findings not occur more than three months after initial onset. ADEM preferentially affects young children but also occurs in adolescents and adults. An antecedent infection or vaccine may be reported, but a clear causal relationship has not been established, and more often no clear provoking factor is identified. In addition to encephalopathy, patients present with symptoms reflecting multifocal CNS involvement such as optic neuritis, ataxia, hemiparesis, spinal cord syndrome, and seizures. Respiratory failure may occur. ADEM is typically treated with high-dose corticosteroids. Plasma exchange or intravenous immunoglobulin may also be used. The course is most often monophasic with good recovery. However, a subset of patients will go on to have recurrent disease, reflecting a diagnosis of multiple sclerosis, neuromyelitis optica spectrum disorder, or myelin oligodendrocyte glycoprotein related disease.

New therapeutic approaches for polymyositis and dermatomyositis

2011 ◽  
Vol 152 (39) ◽  
pp. 1552-1559 ◽  
Author(s):  
Katalin Dankó ◽  
Melinda Vincze
Keyword(s):  
Immunosuppressive Agents ◽  
Inflammatory Myopathy ◽  
Proximal Muscle ◽  
Therapeutic Approaches ◽  
First Line ◽  
New Therapeutic Approaches ◽  
Remission Period ◽  
Immune Mediated ◽  
Systemic Manifestations ◽  
Line Of Therapy

Inflammatory myopathies are chronic, immune-mediated diseases characterized with progressive proximal muscle weakness. They encompass a variety of syndromes with protean manifestations. The aims of therapy are to increase muscle strength, prevent the development of contractures, and to manage the systemic manifestations of the disease. This is a complex treatment which requires routine and wide knowledge. The most important task is to recognize the disease and guide the patient to immunologic center. Although the first line of therapy continues to include corticosteroids, there are a multitude of agents available for treating patients with myositis. There are several different immunosuppressive agents which may be applied alone or in combination with each other, as well as an increasing number of novel and exciting biologic agents targeting molecules participating in the pathogenesis of inflammatory myopathy. Physiotherapy and rehabilitation in the remission period may significantly improve the functional outcome of patients with these disorders. Orv. Hetil., 2011, 152, 1552–1559.

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 Acute Disseminated Encephalomyelitis Following Typhoid Fever: A Case Report

2014 ◽  
Vol 9 (4) ◽  
pp. 55-58
Author(s):  
R Adhikari ◽  
A Tayal ◽  
PK Chhetri ◽  
B Pokhrel
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Typhoid Fever ◽  
Demyelinating Disease ◽  
Acute Disseminated Encephalomyelitis ◽  
Medical Sciences ◽  
Cerebellar Syndrome ◽  
Immune Mediated ◽  
The Central Nervous System

The involvement of central nervous system in children with typhoid fever is common. Acute disseminated encephalomyelitis is a rare immune mediated and demyelinating disease of the central nervous system that usually affects children. We report a 7-year-old child with typhoid fever who developed acute cerebellar syndrome due to acute disseminated encephalomyelitis.Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 55-58 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10237

scholarly journals P.049 Medically refractory longitudinally extensive transverse myelitis successfully treated with cyclophosphamide induction

2016 ◽  
Vol 43 (S2) ◽  
pp. S32-S33
Author(s):  
LJ Baxter ◽  
S Chen ◽  
JM Burton
Keyword(s):  
Respiratory Failure ◽  
Plasma Exchange ◽  
Immune Therapy ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
High Dose ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Intravenous Methylprednisolone ◽  
Intensive Care Admission ◽  
Extensive Transverse Myelitis

Background: Longitudinally extensive transverse myelitis (LETM) is a demyelinating condition that is associated with diseases such as neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis, collagen vascular disease, or can be idiopathic. LETM can be severe enough to cause quadraparesis, marked sensory dysfunction, and respiratory failure. Rarely, these patients are unresponsive to conventional immune therapy. Methods: We report two cases of severe LETM with acute development of quadraparesis and respiratory failure requiring intensive care admission and failure to respond to high-dose corticosteroids, plasma exchange, IVIg and rituximab. Disease cessation and ultimately, significant recovery, was achieved after an 8-day cyclophosphamide induction. Results: A 21 yo female with antibody positive NMOSD and a 19 yo male with idiopathic LETM remained quadraparetic and ventilator dependent with active MRIs despite multiple courses of intravenous methylprednisolone, plasma exchanges, and in the NMOSD patient, IVIg and a 4-week course of rituximab. Both patients ultimately improved significantly and are now ambulatory with subsequent cyclophosphamide induction. Conclusions: In patients with severe LETM of presumed immune origin, who fail to respond to corticosteroids and plasma exchange, cyclophosphamide induction should be considered. This agent provides a more robust immunosuppressive response and can be induced rapidly. Cyclophosphamide effects and supportive evidence are further discussed.

scholarly journals Anti-HMGCR myopathy presenting with acute systolic heart failure

2019 ◽  
Vol 12 (5) ◽  
pp. e230213 ◽  
Author(s):  
Mitchell Pitlick ◽  
Floranne Ernste
Keyword(s):  
Heart Failure ◽  
Immunosuppressive Agents ◽  
Systolic Heart Failure ◽  
High Dose ◽  
Proximal Muscle ◽  
Immune Mediated ◽  
Improved Outcomes ◽  
Rare Side Effect ◽  
Autoimmune Myopathy ◽  
Statin Use

Necrotising autoimmune myopathy (NAM) is an immune-mediated myopathy that may be associated with statin use, malignancy or an autoimmune connective tissue disease, but it can also be idiopathic. Anti-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) myopathy is an extremely rare side effect of statin use, occurring in approximately 2–3 out of every 100 000 patients who use statins. Patients typically present with subacute proximal muscle weakness and creatine kinase levels >10 times the upper limit of normal. The diagnosis is suggested by muscle biopsy showing necrotic fibres with minimal inflammation along with positive anti-HMGCR antibodies. Treatment nearly always requires multiple immunosuppressive agents, the earlier use of which is associated with improved outcomes. Reports of statin-induced NAM leading to heart failure are limited. We present the case of a 69-year-old woman with statin-induced NAM who presented with acute systolic heart failure. Early initiation of high-dose corticosteroids and IVIG resulted in significant improvement in her symptoms.

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