Idiopathic inflammatory demyelinating diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

## Contents

* 1 Overview
* 2 Historical Perspective
* 3 Classification
* 4 Pathophysiology
* 5 Causes
* 6 Differentiating Idiopathic inflammatory Demyelinating Diseases from other Diseases
* 7 Epidemiology and Demographics
* 7.1 Age
* 7.2 Gender
* 7.3 Race
* 8 Risk Factors
* 9 Natural History, Complications and Prognosis
* 10 Diagnosis
* 10.1 Diagnostic Criteria
* 10.2 Symptoms
* 10.3 Physical Examination
* 10.4 Laboratory Findings
* 10.5 Imaging Findings
* 10.6 Other Diagnostic Studies
* 11 Treatment
* 11.1 Medical Therapy
* 11.2 Surgery
* 11.3 Prevention
* 12 References

## Overview

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Idiopathic Inflammatory Demyelinating Diseases (IIDDs), sometimes known as Borderline forms of multiple sclerosis[1], is a collection of multiple sclerosis variants, sometimes considered different diseases[2].

## Historical Perspective

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## Classification

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## Pathophysiology

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## Causes

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## Differentiating Idiopathic inflammatory Demyelinating Diseases from other Diseases

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The list of these diseases depends of the author, but usually are included:

* Optic-spinal MS and neuromyelitis optica (NMO or Devic's disease), which are currently considered variants of the same condition.
* Acute disseminated encephalomyelitis or ADEM, a closely related disorder in which a known virus or vaccine triggers autoimmunity against myelin.
* Balo concentric sclerosis, a pathological form.
* Schilder disease or diffuse myelinoclastic sclerosis: is an infrequent disease that presents clinically as a pseudotumoural demyelinating lesion; and is more common in children.[3][4]
* Marburg multiple sclerosis, pathological form, also known as malignant, fulminant or acute MS.

As MS is an active field for research, the list is not closed or definitive. For example, some diseases like Susac's syndrome (MS has an important vascular component [5]) or autoimmune variants of peripheral neuropathies like Guillain-Barré syndrome could be included.

Some authors also think that primary progressive multiple sclerosis should be considered a different entity from standard MS[6] [7]. Others maintain the opposite[8].

Finally, also a dual classification of these diseases has been proposed, according to the shape of edges of the scars, in MS-like and ADEM-like[9]

## Epidemiology and Demographics

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### Age

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### Gender

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### Race

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## Risk Factors

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## Natural History, Complications and Prognosis

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## Diagnosis

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### Diagnostic Criteria

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### Symptoms

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### Physical Examination

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### Laboratory Findings

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### Imaging Findings

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### Other Diagnostic Studies

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## Treatment

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### Medical Therapy

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### Surgery

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### Prevention

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## References

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1. ↑ "[Borderline forms of multiple sclerosis]". Rev. Neurol. (Paris) (in French). 157 (8-9 Pt 2): 929–34. 2001. PMID 11787357. Unknown parameter `|autho=` ignored (help)CS1 maint: Unrecognized language (link)
2. ↑ Wingerchuk DM, Lucchinetti CF (2007). "Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis". Curr. Opin. Neurol. 20 (3): 343–50. doi:10.1097/WCO.0b013e3280be58d8. PMID 17495631.
3. ↑ Garrido C, Levy-Gomes A, Teixeira J, Temudo T (2004). "[Schilder's disease: two new cases and a review of the literature]". Revista de neurologia (in Spanish). 39 (8): 734–8. PMID 15514902.CS1 maint: Multiple names: authors list (link) CS1 maint: Unrecognized language (link)
4. ↑ Afifi AK, Bell WE, Menezes AH, Moore SA (1994). "Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature". J. Child Neurol. 9 (4): 398–403. PMID 7822732.CS1 maint: Multiple names: authors list (link)
5. ↑ Minagar A, Jy W, Jimenez JJ, Alexander JS (2006). "Multiple sclerosis as a vascular disease". Neurol. Res. 28 (3): 230–5. PMID 16687046.CS1 maint: Multiple names: authors list (link)
6. ↑ Vukusic S, Confavreux C (2003). "Primary and secondary progressive multiple sclerosis". J. Neurol. Sci. 206 (2): 153–5. PMID 12559503.
7. ↑ IDressel A, Kolb AK, Elitok E, Bitsch A, Bogumil T, Kitze B, Tumani H, Weber F (2006). "Interferon-beta1b treatment modulates cytokines in patients with primary progressive multiple sclerosis". Acta Neurol. Scand. 114 (6): 368–73. PMID 17083335.CS1 maint: Multiple names: authors list (link)
8. ↑ Ebers GC (2004). "Natural history of primary progressive multiple sclerosis". Mult. Scler. 10 Suppl 1: S8–13, discussion S13-5. PMID 15218804.
9. ↑ Poser CM, Brinar VV (2004). "The nature of multiple sclerosis". Clinical neurology and neurosurgery. 106 (3): 159–71. PMID 15177764.

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