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Inclusion body myositis (IBM) (/ m aɪ oʊ ˈ s aɪ t ɪ s /) (sometimes called sporadic inclusion body myositis, sIBM) is the most common inflammatory muscle disease in older adults. [2] The disease is characterized by slowly progressive weakness and wasting of both proximal muscles (located on or close to the torso ) and distal muscles (close ...
Inclusion body myopathy with early-onset Paget disease and frontotemporal dementia (IBMPFD), now more commonly referred to as multisystem proteinopathy (MSP), is an autosomal dominant condition caused by mutations in VCP, HNRPA2B1 or HNRNPA1; it is a multisystem degenerative disorder that can affect muscle, bone, and/or the central nervous system.
Multisystem proteinopathy (MSP) is a dominantly inherited, pleiotropic, degenerative disorder of humans that can affect muscle, bone, and/or the central nervous system.MSP can manifest clinically as classical amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), inclusion body myopathy (IBM), Paget's disease of bone (PDB), or as a combination of these disorders. [1]
It can also be associated with underlying cancer. The main classes of idiopathic inflammatory myopathy are polymyositis (PM), dermatomyositis (DM) (including juvenile, amyopathic, and sine-dermatitis form), inclusion-body myositis (IBM), immune-mediated necrotising myopathy (IMNM), and focal autoimmune myositis. [1]
Inclusion bodies of aggregations of multiple proteins are also found in muscle cells affected by inclusion body myositis and hereditary inclusion body myopathy. [1] Inclusion bodies in neurons may accumulate in the cytoplasm or nucleus, and are associated with many neurodegenerative diseases. [2]
Inclusion body myositis is a slowly progressive disease that produces weakness of hand grip and straightening of the knees. No effective treatment is known. (M60.9) Benign acute childhood myositis (M61) Myositis ossificans
A neuronopathy affects the cell body of a nerve cell in the peripheral nervous system. [5] Amyotrophic lateral sclerosis; Spinal muscular atrophy; Spinal muscular atrophy with respiratory distress type 1; Atypical motor neuron diseases; Dorsal root ganglion disorders
Autoimmune: Autoimmune disease is an abnormal immune response to specific body protein or other biomolecular target, such as one of the muscles. The three main types of idiopathic myositis (known as inflammatory myopathies ) that typically test positive for autoantibodies are dermatomyositis , polymyositis , and inclusion body myositis . [ 4 ]