Rasmussen’s encephalitis

GC: n

S: NCBI – https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4005780/ (last access: 17 June 2017); The Lancet – http://www.thelancet.com/journals/laneur/article/PIIS1474-4422(13)70260-6/abstract (last access: 20 March 2018).

N: 1. – Rasmussen (pn): Theodore Brown Rasmussen was an American neurologist, born April 28, 1910, Provo, Utah; died January 23, 2002, Calgary, Canada.
– encephalitis (n): Encephalitis, plural encephalitides, from Greek enkephalos (“brain”) and itis (“inflammation”), inflammation of the brain. Inflammation affecting the brain may also involve adjoining structures; encephalomyelitis is inflammation of the brain and spinal cord, and meningoencephalitis is inflammation of the brain and meninges (the membranes covering the brain).
2. Symptoms: Typically, affected individuals develop focal seizures that may progress to near continuous seizures termed epilepsia partialis continua (EPC). EPC is characterized by a rapid, rhythmic succession of contractions and relaxations of a muscle or muscle group (myoclonus), particularly of the arms, legs, and face, that may occur singularly or in a repetitive, continuous series. In Rasmussen this occurs consistently on one side of the body opposite the side of the inflammation.
Most affected children will exhibit progressive paralysis of one side of the body (hemiparesis) and if the seizures continue developmental disabilities. In many cases, the development of physical and mental abilities of affected children may cease (developmental arrest). In addition, affected children may lose previously acquired physical and mental abilities (developmental regression). Some affected children may exhibit degeneration (atrophy) of one side of the brain and/or progressive confusion, disorientation, and deterioration of intellectual abilities (dementia).
3. Causes: The exact cause of Rasmussen encephalitis is not known. Most researchers now suspect that Rasmussen encephalitis is an autoimmune disorder following review of the tissue involved under the microscope. In autoimmune disorders, the body’s natural defenses (antibodies) fight its own tissue, mistaking it for foreign organisms for no apparent reason.
Some researchers believe that Rasmussen encephalitis may be triggered by an unidentified infection such as influenza, measles, or cytomegalovirus.
4. Cultural Interrelation: We could mention episode 18 Body and Soul from the TV series (2004-2012) House M. D. (season 8).

S: 1. WNMDT – http://www.whonamedit.com/doctor.cfm/2423.html (last access: 17 June 2017); EncBrit – https://www.britannica.com/science/encephalitis (last access: 17 June 2017). 2 & 3. NORD – https://rarediseases.org/rare-diseases/rasmussen-encephalitis/ (last access: 13 June 2017). 4. http://house.wikia.com/wiki/Lue (last access: 20 March 2018).

SYN: chronic encephalitis and epilepsy, chronic localized (focal) encephalitis, epilepsy, hemiplegia and intellectual disabilities, Rasmussen syndrome (RE).

S: NORD – https://rarediseases.org/rare-diseases/rasmussen-encephalitis/ (last access: 13 June 2017)

CR: encephalitis, epilepsy.