Neuromuscular

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PARANEOPLASTIC SYNDROMES 1

General principles

Anatomy
Neoplasm
  Associations
  Incidence
  Malignancy
Temporal
Types
  Endocrine
  Immune
    Antigen is tumor protein
      Intracellular
      Membrane
    Neoplasm produces antibody
    Unknown immune mechanism

Antibody testing
Syndromes

Autonomic
Central nervous system
Cerebellar
Limbic encephalitis
Muscle fiber activity
Myelopathy
Myopathy
Neuromuscular junction
Neuronopathy
  Sensory
  Motor
Neuropathy
Opsoclonus-Myoclonus
Visual loss
Specific antigens

Acetylcholine Receptor
  Muscle
  Neuronal
Amphiphysin
CARP 8
Ion Channel-related
  Ca++(P-type)
  Caspr2 (K+)
CRMP-5
Decorin
CV2
Hu
IgM antigens
Ma
Ma2 (Ta)
mGluR1
Muscle striations
  Titin
PCA-2
Recoverin
Ri
Tr
Yo



GENERAL PRINCIPLES

Anatomy
Diagnostic principles
Neoplasm
  Associations
  Incidence
  Malignancy
Temporal
Types
  Endocrine
  Immune
    Antigen is tumor protein
      Intracellular
      Membrane
    Neoplasm produces antibody
    Unknown immune mechanism


First associations of
polyneuropathy & cancer

SPECIFIC SYNDROMES 5

MYOPATHIES NEUROMUSCULAR JUNCTION DISORDERS NEURONOPATHY NEUROPATHY CENTRAL NERVOUS SYSTEM

Cerebellar
Limbic encephalitis
Myelopathy
Neuromuscular junction
Opsoclonus-Myoclonus
PLS
Stiffman Syndrome
Visual loss



VISUAL LOSS SYNDROMES: Paraneoplastic

Syndrome Symptoms Fundus Physiology Malignancy Antibody
Cancer-associated
   retinopathy
Night blindness
Photopsia
Visual loss: Sudden
Narrow retinal
 vessels
ERG: Absent Lung: Small cell
Breast
Renal cell
Recoverin
Acquired night
 blindness
Night blindness
Shimmering lights
Vitiligo
Narrow retinal
 vessels
ERG: Absent Melanoma Bipolar retinal
  neurons
Bilateral diffuse
 uveal melanotic
 proliferation
Visual loss: Rapid Pigment epithelial
 patches
Uveal tumors
Retinal detachment
Cataract
Scotopic
 response Δ
Lung: Non-small cell
Adenocarcinoma
 Ovary & GI
Cone dystrophy &
  Achromatopsia
Blindness after
 light exposure
Achromatopsia
Narrow retinal
 arteries
Cone
  response
Renal
Optic neuropathy Visual loss
 Sudden or progressive
Scotoma: Arcuate or
 Centrocecal
Papillitis
Disk pallor
VER: Delayed
ERG: Normal
Lung: Non-small cell
Multiple myeloma
IgG M-protein
Retinal
  ganglion cells

Paraneoplastic Sensory-Motor Neuromyopathy


OTHER CANCER-ASSOCIATED ANTIBODIES

Purkinje cell antibody 2 (PCA-2) 3
Antibody testing is performed at:

NEUROMUSCULAR CLINICAL LABORATORY: Washington University
Box 8111 - Neurology
660 South Euclid Avenue
St. Louis, MO 63110
Phone: 314-362-6981
Fax: 314-362-2826
e-mail: pestronka@neuro.wustl.edu

Athena Diagnostic Laboratories

Mayo Clinic


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Return to Neuromuscular syndromes

References
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2. NEJM 2000;342:21-27
3. Ann Neurol 2000;47:297-305, Ann Neurol 2017 Jan 11
4. Neurology 2000;54 S3:A37
5. Muscle Nerve 2000;23:1800-1818
6. Ann Neurol 2001;49:146-154, J Neurol Neurosurg Psychiatry 2009;80:412–416
7. Neurology 2003;60:230–234
8. Ann Neurol 2003; Online March, Brain 2003;126:1409-1418
9. Ann Neurol 2003;54:530–533
10. Ann Neurol 2004; Online September
11. Neurology 2011;76:2089-2095

1/23/2017