Fulminant Central Plus Peripheral Nervous System Demyelination without Antibodies to Neurofascin.
BACKGROUND
Combined central and peripheral nervous system demyelination is a rare and poorly described phenomenon. Recently, anti-neurofascin antibodies were reported to be positive in 86% of these patients in a Japanese cohort. Yet, there seems to be a clinical, radiological, and serological heterogeneity among these patients. In this report, our aim is to describe characteristics of our patients with this entity and compare with others in the literature.


METHODS
We report clinical, electrophysiological, radiological, and laboratory characteristics of five patients with both multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy from our institutional database containing 1890 MS patients.


RESULTS
Three patients presented with extensive, active demyelination of both central nervous system and peripheral nervous system with hypertrophic peripheral nerves. Plexuses, trunks, division and cords were involved in the process. Oligoclonal band was negative. Conduction block was not detected. Corticosteroid treatment was not adequate. Others had a slowly progressive clinical course. Serum anti-neurofascin antibody was negative. Review of the literature revealed similar cases with active disease, early-onset hypertrophic peripheral nerves, and central demyelination, in addition to other cases with an insidious course.


CONCLUSIONS
Patients with combined central and peripheral demyelination form a spectrum. Some patients may have an antibody-mediated syndrome with or without anti-neurofascin antibodies and others seem to represent a coincidence.
1Hacettepe University Medical Faculty,Department of Neurology,Ankara,Turkey.
Atay
A
Vural
2Hacettepe University Medical Faculty,Department of Radiology,Ankara,Turkey.
Rahşan
R
Göçmen
1Hacettepe University Medical Faculty,Department of Neurology,Ankara,Turkey.
Aslı Tuncer
AT
Kurne
2Hacettepe University Medical Faculty,Department of Radiology,Ankara,Turkey.
Kader Karlı
KK
Oğuz
1Hacettepe University Medical Faculty,Department of Neurology,Ankara,Turkey.
Çağrı Mesut
ÇM
Temuçin
1Hacettepe University Medical Faculty,Department of Neurology,Ankara,Turkey.
Ersin
E
Tan
1Hacettepe University Medical Faculty,Department of Neurology,Ankara,Turkey.
Rana
R
Karabudak
3Ludwig Maximilians University,Institute of Clinical Neuroimmunology,Munich,Germany.
Edgar
E
Meinl
1Hacettepe University Medical Faculty,Department of Neurology,Ankara,Turkey.
Sevim
S
Erdem Özdamar
D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D015815:Cell Adhesion Molecules / Q000276:immunology*; D015897:Comorbidity; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000097:blood* / Q000453:epidemiology / Q000473:pathology*; D009414:Nerve Growth Factors / Q000276:immunology*; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000097:blood* / Q000453:epidemiology / Q000473:pathology*; D055815:Young Adult
D001323:Autoantibodies; D015815:Cell Adhesion Molecules; C054305:NFASC protein, human; D009414:Nerve Growth Factors
Multiple sclerosis; anti-neurofascin antibody; chronic inflammatory demyelinating polyradiculopathy; magnetic resonance imaging