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Table 4 Neurological signs suggestive of Kennedy’s disease

From: The French national protocol for Kennedy’s disease (SBMA): consensus diagnostic and management recommendations

Site of involvementNeurological signs
Peripheral motor neuron involvement limited or diffused to the four limbs- Fasciculations
- Cramps
- Amyotrophy
- Predominant motor deficit in the lower and proximal limbs,
- Decrease or abolition of osteo-tendinous reflexes
Progressive peripheral bulbar involvement- Atrophy of the tongue and fasciculations that may extend to the lips, chin, or perioral area (often with a myokymic appearance).
- Dysarthria, with potentially a discreet nasal voice
- Dysphagia
Sensory involvement- Most often subclinical and distal
Absence of evidence of central motor neuron involvement- Absence of spasticity, clonus, exaggeration and / or diffusion of reflexes, or Babinski’s sign
Postural tremor of the upper limbs