PMID- 22759321
OWN - NLM
STAT- MEDLINE
DCOM- 20130610
LR  - 20211021
IS  - 1742-2094 (Electronic)
IS  - 1742-2094 (Linking)
VI  - 9
DP  - 2012 Jul 3
TI  - Cytotoxic CD8+ T cells and CD138+ plasma cells prevail in cerebrospinal fluid in 
      non-paraneoplastic cerebellar ataxia with contactin-associated protein-2 
      antibodies.
PG  - 160
LID - 10.1186/1742-2094-9-160 [doi]
AB  - OBJECTIVE: The purpose of this paper is to report a patient with otherwise 
      unexplained cerebellar ataxia with serum antibodies against contactin-associated 
      protein-2 (CASPR-2) and provide a detailed description of the composition of 
      cellular infiltrates in the cerebrospinal fluid (CSF) compared to the peripheral 
      blood (PB). CASPR-2 antibodies strongly labeling axons of cerebellar granule 
      neurons have recently been identified in sera from nine patients with otherwise 
      unexplained progressive cerebellar ataxia with mild to severe cerebellar atrophy. 
      DESIGN: This is a report of a single case. METHODS: The study methods used were 
      neurologic examination, magnetic resonance imaging, fluorodeoxyglucose positron 
      emisson tomography, lumbar puncture and multicolor flow-cytometry. RESULTS: A 
      23-year-old Caucasian male presented with a two-year history of a progressive 
      cerebellar and brainstem syndrome. Magnetic resonance imaging (MRI) showed 
      pronounced cerebellar atrophy, especially of the medial parts of the hemispheres 
      and the vermis. Cerebral fluorodeoxyglucose positron emission tomography 
      (FDG-PET) showed pronounced hypometabolism of the whole cerebellum. CASPR-2 
      antibodies were detected in the serum but not the CSF, and none of the staging 
      and laboratory assessments revealed other causes of progressive cerebellar 
      degeneration. Interestingly, flow-cytometry of the CSF as compared to the PB 
      showed increased fractions of CD138+ plasma cells as well as human leukocyte 
      antigen (HLA)-DR+ CD8+ T cells suggesting that both B cells and CD8+ T cells were 
      preferentially recruited to and activated within the CSF- (and putatively central 
      nervous system (CNS)-) compartment. CONCLUSION: We confirm the association of 
      CASPR-2 serum antibodies with cerebellar ataxia and provide the first evidence 
      for a combined humoral and cellular immune response in this novel 
      antibody-associated inflammatory CNS disease.
FAU - Melzer, Nico
AU  - Melzer N
AD  - Department of Neurology, Inflammatory Disorders of the Nervous System and 
      Neurooncology, University of Munster, Albert-Schweitzer Campus 1, Munster 48149, 
      Germany. nico.melzer@ukmuenster.de
FAU - Golombeck, Kristin S
AU  - Golombeck KS
FAU - Gross, Catharina C
AU  - Gross CC
FAU - Meuth, Sven G
AU  - Meuth SG
FAU - Wiendl, Heinz
AU  - Wiendl H
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20120703
PL  - England
TA  - J Neuroinflammation
JT  - Journal of neuroinflammation
JID - 101222974
RN  - 0 (Autoantibodies)
RN  - 0 (CNTNAP2 protein, human)
RN  - 0 (Membrane Proteins)
RN  - 0 (Nerve Tissue Proteins)
RN  - 0 (SDC1 protein, human)
RN  - 0 (Syndecan-1)
SB  - IM
MH  - Autoantibodies/*biosynthesis/cerebrospinal fluid
MH  - Cerebellar Ataxia/*cerebrospinal fluid/diagnosis/immunology
MH  - Humans
MH  - Male
MH  - Membrane Proteins/cerebrospinal fluid/*immunology
MH  - Nerve Tissue Proteins/cerebrospinal fluid/*immunology
MH  - Plasma Cells/immunology/*metabolism
MH  - Syndecan-1/*cerebrospinal fluid/immunology
MH  - T-Lymphocytes, Cytotoxic/immunology/*metabolism
MH  - Young Adult
PMC - PMC3464990
EDAT- 2012/07/05 06:00
MHDA- 2013/06/12 06:00
PMCR- 2012/07/03
CRDT- 2012/07/05 06:00
PHST- 2012/05/09 00:00 [received]
PHST- 2012/06/20 00:00 [accepted]
PHST- 2012/07/05 06:00 [entrez]
PHST- 2012/07/05 06:00 [pubmed]
PHST- 2013/06/12 06:00 [medline]
PHST- 2012/07/03 00:00 [pmc-release]
AID - 1742-2094-9-160 [pii]
AID - 10.1186/1742-2094-9-160 [doi]
PST - epublish
SO  - J Neuroinflammation. 2012 Jul 3;9:160. doi: 10.1186/1742-2094-9-160.