- Lesion: Motor cortex and ant horn spinal cord
- U or L: Both
- Muscles: All, distal and proximal.
- Pyramidal features: May predominate.
Progressive bulbar palsy PBP (10%)
- Lesion: CN 9-12. Often progresses to ALS.
- U or L: --
- Muscles: Tongue, palate, pharyngeal.
- Pyramidal features: May be present.
Progressive muscular atrophy PMA (10%)
- Lesion: Ant horn cells only
- U or L: LMN only
- Muscles: Distal limb muscles before proximal.
- Pyramidal features: --
Primary lateral sclerosis PLS
- Lesion: Betz cells in the motor cortex.
- U or L: Mainly UMN.
- Muscles: Spastic leg weakness and pseudobulbar.
- Pyramidal features: --
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