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Sensory Neuronopathies 14


Paraneoplastic Sensory Neuronopathy with Anti-Hu Antibodies 1

Anti-Hu Antibodies




Staining of neuronal nuclei
(Cerebellar granule &
Purkinje cells)

Sjögren's
Syndrome 2


Henrik Sjögren
Diagnostic Criteria for Sjögren's Syndrome
Criteria Definitions
Ocular symptoms Dry eyes every day for more than 3 months
Recurrent sensation of sand or gravel in the eyes, or
Use of tear substitutes more than three times a day
Oral symptoms Dry mouth, daily for more than 3 months
Recurrent or persistently swollen salivary glands, or
Use of liquids to aid in swallowing dry food
Ocular signs Positive Schirmer's test (<5 mm in 5 min), or
Rose Bengal score of ≥4 (van Bijsterveld system)
Histopathology Minor salivary gland (lip) biopsy: Focus score ≥ 1
Salivary gland involvement Positive: Salivary scintigraphy
    Parotid sialography, or
    Salivary flow (≤1.5 mL in 15 min)
Autoantibodies Antibodies: Ro (SS-A); La (SS-B)
  Also see: IgG vs FGFR3
Probable Sjögren's syndrome: 3 criteria present
Definite Sjögren's syndrome: 4 or more criteria present.

Sensory Neuropathy/Neuronopathy with serum IgG vs FGFR-3 13


Idiopathic Sensory Neuronopathy: Pan-sensory


Sensory Neuronopathy: Small fiber 11


Cerebellar Ataxia, Neuronopathy, Vestibular Areflexia Syndrome (CANVAS) 12

  Recessive or Sporadic
Limbic encephalitis

from A Kornberg

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References
1. Neurology 1998;50:652-657, Brain 2002;125:166-175, Brain 2003;126:1409-1418
2. Clin Rheumatol 1999;18:299-303, Best Pract Res Clin Rheumatol 2004;18:313-329
3. JNNP 1999;67:353-357
4. Neurology 2000;54:1008
5. Arthritis & Rheumatism 2000;43:1647-1654
6. J Rheumatol 2002;29:717-725
7. Neurology 2002;59:1113-1114
8. J Rheumatol 2003;30:1552-1557
9. Mod Rheumatol 2003;13:160-167
10. Muscle Nerve 2006;33:672–676, Neurology 2005;65:925-927
11. JNNP 2007 Oct 2
12. Ann N Y Acad Sci 2011;1233:139-147, Neurology 2014;82: Online March, Brain 2014; Online July
13. JNNP 2015; Online Jan
14. Muscle Nerve 2015 Oct 15

2/5/2016