Presenting symptoms | Other disorders to consider |
Ocular (50%) | Brainstem and cranial nerve lesions (including Horner syndrome), thyroid ophthalmopathy, oculopharyngeal muscular dystrophy, chronic external ophthalmoplegia (mitochondrial disease) |
Bulbar (15%) | Brainstem and multiple cranial nerve lesions, motor neuron disease, obstructive or malignant lesion of the nasal and oropharynx |
Limb weakness (<5%) | Motor neuron disease, chronic inflammatory demyelinating polyneuropathy (CIDP) and other motor neuropathies, multiple radiculopathies, Lambert-Eaton myasthenic syndrome, myopathies |
Isolated neck (uncommon) | Motor neuron disease, inflammatory myopathy, paraspinous myopathy |
Isolated respiratory (rare) | Motor neuron disease, acid maltase deficiency, polymyositis |
Distal limb (rare) | Motor neuron disease, CIDP and other motor neuropathies, distal myopathies |