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Smouldering myeloma is a disease classified as intermediate in a spectrum of step-wise progressive diseases termed plasma cell dyscrasias.In this spectrum of diseases, a clone of plasma cells secreting monoclonal paraprotein (also termed myeloma protein or M protein) causes the relatively benign disease of monoclonal gammopathy of undetermined significance.
They are less likely than multiple myeloma patients to have lytic bone lesions. In several studies of patients with either form of plasma cell leukemia, the disease was associated with clonal IgG in 28% to 56% of cases, IgA in 4% to 7% of cases, and a light chain in 23% to 44% of cases; 0-12% of patients had no myeloma protein.
Life expectancy may be shortened by respiratory complications arising from weakness of the muscles that aid breathing and swallowing. It was first described in four patients by Vucic and colleagues [ 3 ] working at the Massachusetts General Hospital in the United States ; subsequent reports from the United Kingdom, [ 4 ] Europe and Asia [ 5 ...
Distal spinal muscular atrophy type 1 (DSMA1), also known as spinal muscular atrophy with respiratory distress type 1 (SMARD1), is a rare neuromuscular disorder involving death of motor neurons in the spinal cord which leads to a generalised progressive atrophy of body muscles.
The impact on life expectancy depends on the individual condition, [9] but is usually severe without treatment. [1] [3] It's estimated only 25–29% of people affected survive to adulthood, and only 10% to the age of 50. [1] The median life expectancy is around 9 years, and the average life expectancy is 16.3 years. [1]
Dementia may occur when neurodegenerative and cerebrovascular pathologies are mixed, as in susceptible elderly people (75 years and older). [2] [5] Cognitive decline can be traced back to occurrence of successive strokes. [4] ICD-11 lists vascular dementia as dementia due to cerebrovascular disease. [1]
The average lifespan after the onset of symptoms in patients with MSA is 6–10 years. [4] Approximately 60% of patients require a wheelchair within five years of onset of the motor symptoms, and few patients survive beyond 12 years. [4] The disease progresses without remission at a variable rate.
The fifth revision of the Diagnostic and Statistical Manual of Mental Disorders gives Lewy body disease as the causative subtype of dementia with Lewy bodies, and Parkinson's disease as the causative subtype of Parkinson's disease dementia. [10] Dementia with Lewy bodies is marked by the presence of Lewy bodies primarily in the cortical regions ...