What is nephrogenic systemic fibrosis (NSF)? Nephrogenic systemic fibrosis previously known as nephrogenic fibrosing dermopathy, is characterized by progressive fibrosis and thickening of the skin (similar to scleroderma), which is particularly painful, and also fibrosis in other organs (e.g., pleura, diaphragm). A rare but debilitating or fatal fibrosing condition that most often affects the skin …
What is POEMS syndrome? The acronym POEMS refers to • P olyneuropathy • O rganomegaly • E ndocrinopathy • M onoclonal protein • S kin involvement POEMS syndrome stands for: • P olyneuropathy: a demyelinating sensorimotor peripheral neuropathy is seen in 100% of patients. At least 50% of patients develop severe muscle weakness. • O rganomegaly: hepatomegaly, splenomegaly, or lymphadenopathy develops in 50%. • E ndocrinopathy: 67% have or develop at least …
What is transverse myelitis? Transverse myelitis is an inflammatory process that is localized over several segments of the cord and functionally transects the cord. It may occur as an infectious or parainfectious illness or as a manifestation of multiple sclerosis, neuromyelitis optica, or other autoimmune process. Incidence of Transverse myelitis Transverse myelitis is extremely rare …
What is Reyes syndrome? Reyes syndrome is a rare, acute noninflammatory encephalopathy that primarily affects children and adolescents. A correlation between the disease and a preceding viral infection (especially influenza and varicella) treated with salicylates has been reported, although other toxic, metabolic, or hypoxic insults may play roles in the pathogenesis. The clinical manifestations of …
What is hemicrania continua (HC) Hemicrania continua (HC) is a rare disorder that may have a prevalence of up to 1% of the population. HC is more common in females than males, 1.6:1. The onset is often during the third decade of life with a range from the first to seventh decades. The pain is …
Sarcoidosis -15 Interesting Facts Sarcoidosis is a multisystem granulomatous disease with widely varied presentation. In the United States, onset is typically in young or middle-aged adults, with higher prevalence in the black and female subpopulations Presentations with the most diagnostic specificity include asymptomatic bilateral hilar adenopathy, acute onset of Löfgren syndrome (ie, fever, arthralgia, erythema …
What is Stiff Person Syndrome (SPS) Rare neurologic disorder characterized by severe muscle rigidity Stiff Person Syndrome is a fluctuating motor disturbance characterized by persistent muscular stiffness due to coactivation of agonist and antagonist muscles with superimposed spasms. As with tetanus, generalized spasms can be precipitated by movements, noise, touch, or emotional stimulation Unlike tetanus, …
Stiff Person Syndrome (SPS) – Interesting Facts, Symptoms, triggering factors Read More »