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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)

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

  Replication factor C subunit 1 (RFC1) ; Chromosome 4p14; Recessive
Limbic encephalitis

from A Kornberg

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14. Muscle Nerve 2015 Oct 15