SENSORY NEURONOPATHIES
1. Paraneoplastic with anti-Hu antibodies
- Clinical Features
- 80% women
- Onset
- Painful paresthesias & dysesthesias
- Asymmetric & distal
- Sensory loss
- All modalities
- Proximal & Distal; Asymmetric; Painful
- Ataxia; Pseudoathetosis
- Motor: Normal
- Course
- Progression over days to 2 months
- Then plateau
- Other syndromes (70%)
- Associated neoplasms: Often small & slow growing
- Strongest association with small-cell lung cancer
- Others (rare): Breast; Ovary; Renal; Esophagus; Testis
- Laboratory features
- Electrodiagnostic
- Absent sensory potentials
- Normal motor studies
Neurons (PNS & CNS); Adenohypophesis; Adrenal cortex; Retina
- Low titers of IgG vs Hu associated with neoplasm with no neurologic signs
- High titers of IgG vs Hu associated with neoplasm and paraneoplastic syndrome
- Hu antigen
- Hu is a family of 35 to 40 kDA neuronal nuclear proteins
- One, HuD
, is an RNA-binding protein (Chromosome 1p34)
- Others: HuC; Hel-N1
- Serum M-Protein: Never
- CSF: Protein & cells increased; Oligoclonal bands
- Pathology: Dorsal root ganglion inflammation & neuronal loss: Patchy
- Rule out: cis-platinum toxicity
- Treatment: None
2. Sjögren's syndrome
- Clinical Features
- Sensory loss
- All modalities
- Proximal & Distal
- Ataxia
- No CNS signs
- Sicca syndrome
- Electrodiagnostic
- Absent sensory potentials
- Normal motor studies
- Serum autoantibodies: IgG vs SS-A and SS-B
- Treatment: None
3. Pyridoxine intoxication
- Acute; High dose (100 - 200 g)
- Chronic (0.2 - 10 g/day) produces reversible distal axonopathy
4. Doxorubicin (animals only, no human cases described)
5. Localized: Herpes zoster
6. Acute sensory neuronopathy
- Idiopathic ? GBS-like syndrome
- Autonomic system may be involved
- Rare patients may have preserved tendon reflexes
- ? central branch of sensory neuron damaged rather than cell body
- ? preserved Ia afferents to motor neurons
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5/31/97