Neuromuscular

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NEUROMUSCULAR CLINICAL LABORATORY

Contact Information
Box 8111 - Neurology
660 South Euclid Avenue
St. Louis, MO 63110
Phone: 314-362-2406
Fax: 314-362-3413
e-mail: pestronka@neuro.wustl.edu

Instructions on sending
  Antibody testing
, or
  Biopsies: Muscle or Nerve
Phone: 314-362-2406

Laboratory certifications
    CAP Lab # 19233-16
    Medicare Provider # 26-8235
    CLIA Certificate # 26D0652044
    New York State 3499
    California COS 00800679
    Florida 800028650
Testing at Washington University
  Laboratory: Specific tests
  Requisition forms & Information
    Antibodies
      Sending samples: Instructions
    Biopsies: Muscle, Nerve & Skin
      Muscle instructions
        Freezing; Other
      Nerve instructions
        General; Surgery;
        Freezing; Other technical
      Skin instructions

Antibodies
  General principles
  Classification
  Binding to oligosaccharides
    Glycoproteins & Glycolipids
  Specific types
  Testing methods
  Survey




Charcot's clinic
Charcot's clinic


Testing at Washington University Neuromuscular Laboratory

Antibodies Muscle biopsies Nerve biopsies Neuromuscular Pathology Training Program

LAB TESTING IN NEUROMUSCULAR DISORDERS
Antibodies Biopsies WU Testing
IgM vs
  Decorin (BJ)
  Disaccharides
    NS6S
    TS-HDS
  Glycolipids
    GalC
    GalNAc-GD1a
    GALOP
    GD1a
    GD1b
    GM1
    GM1b
    GM2
    NP9
    SGPG
    Sulfatide
  Heparan sulfate
  MAG
  Neurofilaments
  Tubulin
IgG vs
  Acetylcholine receptors
    Muscle
    Neuronal (α3)
    Other
  NMJ
    LRP4
    MuSK
  Channels
    Calcium
    Potassium complex
      Lgi1
      Caspr2
  Paranodal
    Caspr
    Contactin-1
    Neurofascins
      140; 155
  Cortactin
  CRMP-5
  DPPX
  FGFR-3
  FHL1
  GAD
  Glutamate &
    GABA Receptor


  Glycolipids
    GalC
    GM1
    GD1a
    GalNAc-GD1a
    Disialosyl
      GD1b
      GQ1b
      GT1a
    Lysoganglioside-GM1
    Sulfatide
  HMGCR
  Mitochondria
  MPP1
  Muscle striations
  Neuronal glia
    SOX1
  Neuronal nuclei
    Hu
    Ri
  NT5C1A
  Purkinje cells
    PCA-2
    Tr
    Yo
Antibodies: General
Testing methods
Polyspecific IgM & IgG
IgG4
Connective tissue disorders
  Myositis-associated
  Myositis-overlap
  Other antibodies
  Vasculitis
Diabetes
M-proteins
Myopathies
Myositis
Neuropathies
NMJ disorders
  Myasthenia Gravis
  LEMS
Oligosaccharide antigens
Paraneoplastic
Stiffman
Transglutaminase
Muscle

Nerve

Skin
Antibodies
  Requisition
  Instructions

Biopsies
  Requisition
Laboratory testing at Washington University, St. Louis


Neuromuscular Autoantibodies: Classification & Features

Antibody Antigen Disease
Class Location Type Pathogenic
properties
Features Antigen
Examples
Neuromuscular
Disease Examples
Associations,
Systemic
Common
treatments
IgG Serum Polyclonal Often Protein
  Cell surface
    membrane
Receptors: AChRs
  Muscle; Neuron
Ca++ ion channels
K+ channel complex
Myasthenia gravis
Autonomic PN
LEMS
Isaac's
Neoplasms
  Thymoma
  Adeno Ca
  Small cell lung
Corticosteroids
T-cell suppressants
IVIg
Plasma Exchange
IgG Serum Polyclonal Possible Carbohydrate
  Glycolipid
Gangliosides
  GM1
  GQ1b
  GD1b

AMAN
Miller-Fisher
Sensory, Acute
Infection,
    Prodromal
Disease often
    monophasic
IgG Serum Polyclonal Few Protein
  Intracellular
tRNA synthetases
SRP
ANCA
FGFR3
IMPP
Polymyopathy, immune
Vasculitis
Neuropathy, Sensory
Organ system
  disease, or
Neoplasm, or
None
Corticosteroids
T-cell suppressants
Rituximab
IgG Serum + CSF Polyclonal No Protein
  Intracellular
Hu (Neuron nuclei)

CRMP5
Neuronopathy
  Sensory; Autonomic
Polyneuropathy
Neoplasms
  Small cell lung
  Gynecologic
Screen for & Treat
  Associated neoplasm
IgG4 Serum Polyclonal Possible Protein
  Cell surface, or
  Extracellular
MuSK
Neurofascins
  140
  155
Contactin-1
Lgi1
Caspr2
MG-MuSK
PN
  Sensory ataxia, subacute
  Demyel +: Tremor; CNS
Demyel PN: IVIg resistant
Encephalopathy ± PN
Neuromyotonia; Autonomic
None Rituximab (q 1-6 mo)
Corticosteroids
IgM Serum Monoclonal
  or
Polyclonal
Possible Carbohydrate
  Glycolipid or
  Glycoproteins
GM1
MAG
TS-HDS
GD1b
Motor PN
Demyelinating PN
Sensory PN
Sensory PN, Ataxic
Neoplasms
  M-protein
Rituximab (IgM ↓)
Intravenous Ig
  (Conduction block)
Cyclophosphamide
IgM, IgG,
  or IgA
Serum Monoclonal Some Not identified Polyneuropathy POEMS
Cryoglobulins
Waldenström's
Radiate; Chemo; Stem cell

Rituximab; Chemo

Print

AUTOANTIBODIES AND IMMUNE POLYNEUROPATHIES

Introduction

AUTOANTIBODIES with BINDING TO OLIGOSACCHARIDES:
  Glycoproteins or Glycolipids

GalNAc-GD1a ganglioside
GALOP antigen
GD1b ganglioside
GD1a ganglioside
GD3 ganglioside
GM1 ganglioside
    Co-GM1; GM1; NP-9
GM1b ganglioside
GM2 ganglioside
GQ1b ganglioside
GT1a ganglioside
Heparan sulfate
Lysoganglioside-GM1
MAG
NS6S
SGPG
Sulfatide
    GALOP antigen
TS-HDS




Anti-GM1 Ganglioside Antibodies

Galβ1-3GalNAcβ1-4Galβ1-4Glcβ1-1'Ceramide
                  3
                  |
           Neu5Acα2
GM1 ganglioside
Galβ1-3GalNAcβ1-4Galβ1-X
                  3
                  |
           Neu5Acα2
Target epitope: GM1


Anti-GM1 antibodies: Clinical associations Testing for anti-GM1 antibodies

Anti-Sulfatide antibodies

SO4-3Galactose-X
Target epitope: Sulfatide


Anti-MAG antibodies

SO4-3Glucuronate-X
Target epitope: MAG & SGPG


Anti-SGPG antibodies

SO4-3GlcAβ1-3Galβ1-4GlcNAcβ1-3Galβ1-4Glcβ1-1'Ceramide
SGPG
SO4-3Glucuronate-X
Target epitope: SGPG & MAG


Anti-GQ1b & GT1a antibodies

                Galβ1-3GalNAcβ1-4Galβ1-X
                 3                3
                 |                |
Neu5Acα2-8Neu5Acα2                |
                 Neu5Acα2-8Neu5Acα2
Target epitope: GQ1b
                Galβ1-3GalNAcβ1-4Galβ1-X
                 3                3
                 |                |
Neu5Acα2-8Neu5Acα2                |
                           Neu5Acα2
Target epitope: GT1a



Anti-GD1b antibodies

Galβ1-3GalNAcβ1-4Galβ1-4Glcβ1-1'Ceramide
                  3
                  |
 Neu5Acα2-8Neu5Acα2
GD1b ganglioside
             Y-4Galβ1-X
                 3
                 |
Neu5Acα2-8Neu5Acα2
Target epitope: GD1b


Anti-GM1b antibodies

      Galβ1-3GalNAcβ1-4Galβ1-4Glcβ1-1'Ceramide
       3                
       |                
Neu5Acα2
GM1b ganglioside


Anti-GD1a antibodies

      Galβ1-3GalNAcβ1-4Galβ1-4Glcβ1-1'Ceramide
       3                3
       |                |
Neu5Acα2         Neu5Acα2
GD1a ganglioside
      Galβ1-X
       3
       |
Neu5Acα2
Target epitope: GD1a


Anti-GalNAc-GD1a & GM2 ganglioside antibodies

GalNAcβ1-4Galβ1-X
           3
           |
    Neu5Acα2
Target epitope:
  GalNAc-GD1a and GM2


Anti-Galactocerebroside (GalC) antibodies 39

Gal-Ceramide
Galactocerebroside


Anti-Heparan Sulfate antibodies



GALOP Syndrome



Anti-Decorin (BJ) antibodies


OTHER NEUROLOGICAL- or NEUROPATHY-ASSOCIATED AUTOANTIBODIES



Anti-Tubulin Antibodies



Anti-Hu antibodies



Anti-Yo antibodies, IgG



Lysoganglioside-GM1 (Lyso-GM1) antibodies



M-phase phosphoprotein-1 (Mphosph1; MPP1 ) antibodies 36



Polyspecific antibodies



Polyneuropathies associated with serum M-proteins



DETECTION OF ANTIBODIES: METHODOLOGIES

General principles

Enzyme-linked immunosorbent assays (ELISA)
Immunocytochemistry
Immunofixation
Thin layer chromatography (TLC)
Western blot



Antibody Testing Methods: General Principles



ELISA Methods



Western Blotting Methods



Immunocytochemical Methods


Thin Layer Chromatography Methods


Immunofixation Electrophoresis Methods



Sydenham chorea


Glutamate & GABA Receptor Antibodies

Antibody Age & Sex Syndrome Neoplasm
NMDAR Age
  37%<18 years
  5%>44 years
Sex: 80% female
Panencephalitis
Psychiatric at onset
Ovary teratoma (35%)
  Young fertile women
    1244 years(5560%)
AMPAR Age: 2387 years
Female predominant
Limbic encephalitis
Psychosis, rare
Thymoma
Small cell lung
Breast
GABAAR Age: 363
Male predominant
Limbic encephalitis
Seizures, refractory
None
GABABR Age: 1685 years
Male = Female
Limbic encephalitis
Seizures, refractory
Small cell lung
mGluR1 Age: 1965 years
Cerebellar degeneration Hodgkin's lymphoma
mGluR5 Age: 1546 years
Male = Female
Limbic encephalitis
Hodgkin's lymphoma
Also see
  GAD
  Lgi1
  Caspr2

Alan Pestronk MD: Neuromuscular Pathology Certification


Muscle pathology



Nerve Pathology


Neuromuscular Pathology


Neuromuscular Pathology Training Program



Neuromuscular Laboratory Certifications

Print


College of American Pathologists (CAP)



CLIA



New York



California


Florida

Return to Neuromuscular Home Page

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7/17/2017