Idiopathic inflammatory demyelinating diseases
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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
Classification
Pathophysiology
Causes
Differentiating Idiopathic inflammatory Demyelinating Diseases from other Diseases
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
Age
Gender
Race
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Criteria
Symptoms
Physical Examination
Laboratory Findings
Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Prevention
References
- ↑ “[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) - ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Minagar A, Jy W, Jimenez JJ, Alexander JS (2006). “Multiple sclerosis as a vascular disease”. Neurol. Res. 28 (3): 230–5. PMID 16687046.
- ↑ Vukusic S, Confavreux C (2003). “Primary and secondary progressive multiple sclerosis”. J. Neurol. Sci. 206 (2): 153–5. PMID 12559503.
- ↑ 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.
- ↑ Ebers GC (2004). “Natural history of primary progressive multiple sclerosis”. Mult. Scler. 10 Suppl 1: S8–13, discussion S13-5. PMID 15218804.
- ↑ Poser CM, Brinar VV (2004). “The nature of multiple sclerosis”. Clinical neurology and neurosurgery. 106 (3): 159–71. PMID 15177764.
© 2026 MyEClinic – IFTM Institut für Telematik in der Medizin GmbH