PMID- 21775733
OWN - NLM
STAT- MEDLINE
DCOM- 20110930
LR  - 20211020
IS  - 1526-632X (Electronic)
IS  - 0028-3878 (Print)
IS  - 0028-3878 (Linking)
VI  - 77
IP  - 5
DP  - 2011 Aug 2
TI  - Progressive encephalomyelitis with rigidity and myoclonus: glycine and NMDA 
      receptor antibodies.
PG  - 439-43
LID - 10.1212/WNL.0b013e318227b176 [doi]
AB  - BACKGROUND: The syndrome of progressive encephalopathy with limb rigidity has 
      been historically termed progressive encephalomyelitis with rigidity and 
      myoclonus (PERM) or stiff-person syndrome plus. METHODS: The case is presented of 
      a previously healthy 28-year-old man with a rapidly fatal form of PERM developing 
      over 2 months. RESULTS: Serum antibodies to both NMDA receptors (NMDAR) and 
      glycine receptors (GlyR) were detected postmortem, and examination of the brain 
      confirmed an autoimmune encephalomyelitis, with particular involvement of 
      hippocampal pyramidal and cerebellar Purkinje cells and relative sparing of the 
      neocortex. No evidence for an underlying systemic neoplasm was found. CONCLUSION: 
      This case displayed not only the clinical features of PERM, previously associated 
      with GlyR antibodies, but also some of the features associated with NMDAR 
      antibodies. This unusual combination of antibodies may be responsible for the 
      particularly progressive course and sudden death.
FAU - Turner, M R
AU  - Turner MR
AD  - Oxford University Nuffield Department of Clinical Neurosciences, John Radcliffe 
      Hospital, Oxford, UK.
FAU - Irani, S R
AU  - Irani SR
FAU - Leite, M I
AU  - Leite MI
FAU - Nithi, K
AU  - Nithi K
FAU - Vincent, A
AU  - Vincent A
FAU - Ansorge, O
AU  - Ansorge O
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20110720
PL  - United States
TA  - Neurology
JT  - Neurology
JID - 0401060
RN  - 0 (Antibodies)
RN  - 0 (Receptors, Glycine)
RN  - 0 (Receptors, N-Methyl-D-Aspartate)
SB  - IM
CIN - Neurology. 2011 Aug 2;77(5):414-5. PMID: 21775740
MH  - Adult
MH  - Antibodies/*blood
MH  - Brain/pathology
MH  - Encephalomyelitis/*blood/complications/pathology
MH  - Humans
MH  - Male
MH  - Muscle Rigidity/*blood/complications/pathology
MH  - Myoclonus/*blood/complications/pathology
MH  - Receptors, Glycine/*immunology
MH  - Receptors, N-Methyl-D-Aspartate/*immunology
MH  - Spinal Cord/pathology
PMC - PMC3146306
EDAT- 2011/07/22 06:00
MHDA- 2011/10/01 06:00
PMCR- 2012/01/27
CRDT- 2011/07/22 06:00
PHST- 2011/07/22 06:00 [entrez]
PHST- 2011/07/22 06:00 [pubmed]
PHST- 2011/10/01 06:00 [medline]
PHST- 2012/01/27 00:00 [pmc-release]
AID - WNL.0b013e318227b176 [pii]
AID - WNL202799 [pii]
AID - 10.1212/WNL.0b013e318227b176 [doi]
PST - ppublish
SO  - Neurology. 2011 Aug 2;77(5):439-43. doi: 10.1212/WNL.0b013e318227b176. Epub 2011 
      Jul 20.