A useful operational definition of MSP is dominantly inherited degeneration that includes neurological involvement (either motor neuron disease or dementia) in combination with either distal myopathy or Pagetic bone degeneration. Most MSP patients present with weakness, and of these, approximately 65% present with motor neuron involvement. Although rare, MSP can also include involvement of cardiac, hepatic, visual, auditory, sensory, and autonomic systems. The histopathology of tissues affected by MSP includes ubiquitin-positive cytoplasmic inclusions of RNA-binding proteins, such as TDP-43, HNRNPA1, HNRNPA2B1, and other components of RNA granules.
Multisystem proteinopathy
Proteinopathy
Organ-limited amyloidosis
HIV-associated nephropathy
Hereditary motor and sensory neuropathy
Monoclonal gammopathy
Cardiac amyloidosis
AA amyloidosis