Atypical inflammatory demyelinating syndromes of the CNS

  title={Atypical inflammatory demyelinating syndromes of the CNS},
  author={Todd A Hardy and Stephen W Reddel and Michael Harry Barnett and Jacqueline A Palace and Brian G. Weinshenker},
  journal={The Lancet Neurology},
Atypical Inflammatory Demyelinating Syndromes of the Central Nervous System
  • T. Hardy
  • Psychology, Medicine
    Contemporary Clinical Neuroscience
  • 2019
The difficulties in diagnosing and distinguishing between conditions such as acute disseminated encephalomyelitis, tumefactive demyelination, Balo’s concentric sclerosis, Marburg's multiple sclerosis, and Schilder's diffuse myelinoclastic sclerosis are discussed.
Balo’s concentric lesions with concurrent features of Schilder’s disease in relapsing multiple sclerosis: neuropathological findings
An autopsy case of middle-aged female with a chronic history of MS newly relapsed with atypical demyelinating lesions, which showed concurrent features of BCS and SD is described and the overlapping features between these two variants are discussed.
Pseudotumoral Demyelinating Lesions: A Presentation of Acute Disseminated Encephalomyelitis
A 35-year-old male patient hospitalized in the department of neurology for a rapidly progressive onset of encephalopathy and polyfocal neurological deficits, with pseudotumoral lesions shown on brain MRI is presented.
Differential imaging of atypical demyelinating lesions of the central nervous system
In this review, the main pattern of presentation of atypical inflammatory demyelinating diseases is discussed, with particular attention on the differential diagnosis and how to adequately define the correct etiology.
The spectrum of immune-mediated and inflammatory lesions of the brainstem
Clinical features and MRI clues that help to distinguish among the different brainstem conditions are focused on.
Silent Tumefactive Demyelinating Lesions and Radiologically Isolated Syndrome
A case involving an asymptomatic TDL detected incidentally during magnetic resonance imaging in an 18-year-old man, who was diagnosed with a radiologically isolated syndrome and did not develop any symptoms during the 1-year follow-up period.
Biomarkers in Rare Demyelinating Disease of the Central Nervous System
Progress in biomarker research with respect to rare neuroinflammatory disease of the CNS is described, which may facilitate timely diagnosis and proper disease management, preventing disease exacerbation due to misdiagnosis and false treatment.
Description of a Tumefactive Demyelinating Lesion Studied with Advanced Magnetic Resonance Techniques: A Clinical Report and Review of Literature
The case of a 17-years-old girl with a single TDL suddenly enlarging even under steroid therapy is reported, which poses a diagnostic challenge as it enlarged, showed areas of restricted diffusion and high perfusion.


Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis
This review will discuss acute management as well as chronic immunotherapies in acquired pediatric demyelination and important efficacy and safety concerns regarding the use of these therapies in children and young adults.
The pathological spectrum of CNS inflammatory demyelinating diseases
The clinicopathologic features of these CNS inflammatory demyelinating disorders are reviewed and recent advances in understanding their immunopathogenesis are discussed.
Acute demyelinating disease. Classification and non‐invasive diagnosis
Localized proton magnetic resonance spectroscopy (MRS) performed in one case revealed a pattern typical of acute demyelination suggesting potential for a replacement of biopsy in the future.
Atypical idiopathic inflammatory demyelinating lesions: prognostic implications and relation to multiple sclerosis
Most types of atypical idiopathic inflammatory demyelinating lesions can be classified according to previously suggested radiologic characteristics and confer a relatively low risk of further clinical attacks, except for ring-like lesions and the combination with MS-typical lesions.
Pathology of demyelinating diseases.
The pathological features of these central nervous system inflammatory demyelinating disorders are reviewed and neuropathological studies that have yielded novel insights into potential mechanisms involved in the formation of the demYelinated lesion are discussed.
Exploring the overlap between multiple sclerosis, tumefactive demyelination and Baló’s concentric sclerosis
The similarities between typical MS, TD and BCS cases are explored, the potential insights that intermediate or overlapping phenotypes may contribute towards an understanding of MS immunopathogenesis are reflected, and whether these atypical forms of demyelination should be classified as separate demYelinating diseases.
Baló's concentric sclerosis
MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain
It is suggested that IIDLs reported as atypical in the literature can be segregated into several distinct subtypes based on their MRI appearance, which may be useful for the differential diagnosis and for a future classification.
Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome
This review comprehensively evaluates the current knowledge and controversies that surround ADEM, with special consideration of the distinction between ADEM and other demyelinating diseases such as multiple sclerosis.