Journal of Pediatric Epilepsy 2016; 05(04): 168-175
DOI: 10.1055/s-0036-1584914
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Kozhevnikov-Rasmussen Syndrome: A Historical Perspective Spanning Two Centuries

Tatyana Gitlevich
1   Department of Neurology, Albert Einstein College of Medicine and Montefiore Epilepsy Management Center, Bronx, New York, United States
,
Fred A. Lado
1   Department of Neurology, Albert Einstein College of Medicine and Montefiore Epilepsy Management Center, Bronx, New York, United States
,
Solomon L. Moshé
1   Department of Neurology, Albert Einstein College of Medicine and Montefiore Epilepsy Management Center, Bronx, New York, United States
› Institutsangaben
Weitere Informationen

Publikationsverlauf

21. Dezember 2015

09. März 2016

Publikationsdatum:
07. September 2016 (online)

Abstract

The Kozhevnikov-Rasmussen syndrome consists of a distinctive constellation of intractable, mainly focal, motor seizures, associated with relentlessly progressive hemiparesis and neuropsychological deterioration with cognitive and linguistic deficits. It dates back to the end of the 19th century and spans two continents. The main seizure type, epilepsia partialis continua, was first described by the Russian neurologist Kozhevnikov in 1894 and shortly after the eponym Kozhevnikov epilepsy was used both in Russian and in Western literature. The description of “focal seizures due to chronic localized encephalitis in children” by Rasmussen and his colleagues took place in 1958 and it was introduced as Rasmussen encephalitis. What began as a clinical observation became a complex syndrome affecting many age groups with a possible neuroimmunological etiology. Here we describe the historical perspectives underlying the establishment of the syndrome.

 
  • References

  • 1 Bien CG, Schramm J. Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. Epilepsy Res 2009; 86 (2–3) 101-112
  • 2 Vein AA. The Moscow clinic for nervous diseases - walking along the portraits. J Hist Neurosci 2007; 16 (1–2) 42-57
  • 3 Vein AA, van Emde Boas W. Kozhevnikov epilepsy: the disease and its eponym. Epilepsia 2011; 52 (2) 212-218
  • 4 Andermann F , ed. Chronic Encephalitis and Epilepsy. Rasmussen Syndrome. Stoneham, MA: Butterworth-Heinemann; 1991. . “A particular Type of Cortical Epilepsy” (Epilepsia corticalis sive partialis continua), A.Ya. Kozhevnikov (Translated from Russian by David M. Asher). 245-261
  • 5 Bruns E. Anastomische und klinische Demostrationen. Neurol Zentralbl. 1895; 14: 47-48
  • 6 Orlovskij SB. Ein Fall von epilepsia partialis continua (Russ.) Medskoe Obozr. Sprimona 43. Neurol. Zbl. 1825; 526: 891-892
  • 7 Choroschko VK. Zur Differentialdiagnostik der Polyclonia epileptoides continua (Kozevnikov). Neurol Zbl. 1908; 27: 279-279
  • 8 Wilson G, Winkelman N. Partial continuous epilepsy with special reference to that produced by microscopic cortical lesions. Arch Neurol Psychiatry 1924; 11: 530-542
  • 9 Omorokov L. Die Kojewnikkoffsche epilepsie in Sibirien. Z Ges Neurol Psych 1927; 107: 487-496
  • 10 Gulyaeva SE. Kozhevnikov's epilepsy and tick-born viral encephalitis: history of research, pathogenesis issues [in Russian]. Pa Med J 2004; 4: 21-25
  • 11 Davidenkov SN. Clinical Lectures on Nervous Diseases [in Russian]. Leningrad: Medgiz; 1957: 81-106
  • 12 Rasmussen T, Olszewski J, Lloydsmith D. Focal seizures due to chronic localized encephalitis. Neurology 1958; 8 (6) 435-445
  • 13 Dubeau F, Andermann F, Wiendl H, Bar-Or A, Rasmussen's encephalitis (chronic focal encephalistis). In: Engel Jr. J, Pedley TA. (eds) Epilepsy: A Comprehensive Textbook. Vol. 3. Philadelphia, PA: Lippincott Williams & Wilkins; 2008: 2439-2440
  • 14 Oguni H, Andrmann FF, Rasmussen TB. The natural history of the syndrome of chronic encephalomyelitis and epilepsy: a study of the MNI series of forty-eight cases. In: Anderman F, ed. Chronic Encephalitis and Epilepsy. Rasmussen's Syndrome. Stoneham, MA: Butterworth-Heinemann; 1991: 7-36
  • 15 Wieser HG. Epilepsia Partialis Continua. 2005. Available at www.ilae-epilepsy.org
  • 16 Andermann F, Lugaresi E, Dvorkin GS, Montagna P. Malignant migraine: the syndrome of prolonged classical migraine, epilepsia partialis continua, and repeated strokes; a clinically characteristic disorder probably due to mitochondrial encephalopathy. Funct Neurol 1986; 1 (4) 481-486
  • 17 Rasmussen M, Sanengen T, Skullerud K, Kvittingen EA, Skjeldal OH. Evidence that Alpers-Huttenlocher syndrome could be a mitochondrial disease. J Child Neurol 2000; 15 (7) 473-477
  • 18 Lado FA, Legatt AD, LaSala PA, Shinnar S. Alteration of the cortical motor map in a patient with intractable focal seizures. J Neurol Neurosurg Psychiatry 2002; 72 (6) 812-815
  • 19 Singh BM, Strobos RJ. Epilepsia partialis continua associated with nonketotic hyperglycemia: clinical and biochemical profile of 21 patients. Ann Neurol 1980; 8 (2) 155-160
  • 20 Morres CA, Dire DJ. Movement disorders as a manifestation of nonketotic hyperglycemia. J Emerg Med 1989; 7 (4) 359-364
  • 21 Desbiens R, Berkovic SF, Dubeau F , et al. Life-threatening focal status epilepticus due to occult cortical dysplasia. Arch Neurol 1993; 50 (7) 695-700
  • 22 Peterus T, Huang KM, Chi EY, Shih CC, Su ML, Huang JS. Koshevnikov syndrome in a patient with MELAS plus syndrome: electron microscopic and neuroimage studies. Chin Med J (Engl) 1997; 110 (9) 726-730
  • 23 Misawa S, Kuwabara S, Hirano S, Shibuya K, Arai K, Hattori T. Epilepsia partialis continua as an isolated manifestation of motor cortical dysplasia. J Neurol Sci 2004; 225 (1–2) 157-160
  • 24 Longaretti F, Dunkley C, Varadkar S, Vargha-Khadem F, Boyd SG, Cross JH. Evolution of the EEG in children with Rasmussen's syndrome. Epilepsia 2012; 53 (9) 1539-1545
  • 25 Cheong JY, Wong C, Bleasel A, Varikatt W, Ng T, Dexter MA. Late onset Rasmussen's encephalitis with triple pathology. J Clin Neurosci 2009; 16 (12) 1677-1681
  • 26 Tobias SM, Robitaille Y, Hickey WF, Rhodes CH, Nordgren R, Andermann F. Bilateral Rasmussen encephalitis: postmortem documentation in a five-year-old. Epilepsia 2003; 44 (1) 127-130
  • 27 Chiapparini L, Granata T, Farina L , et al. Diagnostic imaging in 13 cases of Rasmussen's encephalitis: can early MRI suggest the diagnosis?. Neuroradiology 2003; 45 (3) 171-183
  • 28 Ben-Zeev B, Nass D, Polack S , et al. Progressive unilateral basal ganglia atrophy and hemidystonia: a new form of chronic focal viral encephalitis. Neurology 1999; 51 (Suppl. 01) 58-58
  • 29 Bancaud J, Bonis A, Trottier S, Talairach J, Dulac O. L'épilepsie partielle continue: syndrome et maladie. Rev Neurol (Paris) 1982; 138 (11) 803-814
  • 30 Engel Jr J ; International League Against Epilepsy (ILAE). A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia 2001; 42 (6) 796-803
  • 31 Oguni H, Andermann F, Rasmussen TB. The syndrome of chronic encephalitis and epilepsy. A study based on the MNI series of 48 cases. Adv Neurol 1992; 57: 419-433
  • 32 Bien CG, Widman G, Urbach H , et al. The natural history of Rasmussen's encephalitis. Brain 2002; 125 (Pt 8) 1751-1759
  • 33 Bien CG, Urbach H, Deckert M , et al. Diagnosis and staging of Rasmussen's encephalitis by serial MRI and histopathology. Neurology 2002; 58 (2) 250-257
  • 34 Bien CG, Granata T, Antozzi C , et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain 2005; 128 (Pt 3): 454-471
  • 35 Panayiotopoulos CP. Kozhevnikov-Rasmussen syndrome and the new proposal on classification. Epilepsia 2002; 43 (8) 948-949 , author reply 949
  • 36 Panayiotopoulos CP. The Epilepsies. Seizures, Syndromes and Management. Oxfordshire, UK: Bladon Medical Publishing; 2005: 186-192
  • 37 Berg AT, Berkovic SF, Brodie MJ , et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia 2010; 51 (4) 676-685
  • 38 Rogers SW, Andrews PI, Gahring LC , et al. Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis. Science 1994; 265 (5172) 648-651
  • 39 Watson R, Jiang Y, Bermudez I , et al. Absence of antibodies to glutamate receptor type 3 (GluR3) in Rasmussen encephalitis. Neurology 2004; 63 (1) 43-50
  • 40 Hart YM, Cortez M, Andermann F , et al. Medical treatment of Rasmussen's syndrome (chronic encephalitis and epilepsy): effect of high-dose steroids or immunoglobulins in 19 patients. Neurology 1994; 44 (6) 1030-1036
  • 41 Bien CG, Schramm J. Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. Epilepsy Res 2009; 86 (2–3) 101-112
  • 42 Watson R, Jepson JE, Bermudez I , et al. Alpha7-acetylcholine receptor antibodies in two patients with Rasmussen encephalitis. Neurology 2005; 65 (11) 1802-1804
  • 43 Takahashi Y, Mori H, Mishina M , et al. Autoantibodies and cell-mediated autoimmunity to NMDA-type GluRepsilon2 in patients with Rasmussen's encephalitis and chronic progressive epilepsia partialis continua. Epilepsia 2005; 46 (Suppl. 05) 152-158
  • 44 Tziperman B, Garty BZ, Schoenfeld N , et al. Acute intermittent porphyria, Rasmussen encephalitis, or both?. J Child Neurol 2007; 22 (1) 99-105
  • 45 Ohmori I, Ouchida M, Kobayashi K , et al. Rasmussen encephalitis associated with SCN 1 A mutation. Epilepsia 2008; 49 (3) 521-526
  • 46 Alvarez-Barón E, Bien CG, Schramm J, Elger CE, Becker AJ, Schoch S. Autoantibodies to Munc18, cerebral plasma cells and B-lymphocytes in Rasmussen encephalitis. Epilepsy Res 2008; 80 (1) 93-97
  • 47 Schmalbach B, Lang N. New hope for Rasmussen encephalitis?. Discov Med 2009; 8 (42) 130-132
  • 48 Rassner MP, van Velthoven-Wurster V, Ramantani G, Feuerstein TJ. Altered transporter-mediated neocortical GABA release in Rasmussen encephalitis. Epilepsia 2013; 54 (3) e41-e44
  • 49 Owens GC, Erickson KL, Malone CC , et al. Evidence for the involvement of gamma delta T cells in the immune response in Rasmussen encephalitis. J Neuroinflammation 2015; 12: 134 DOI: 10.1186/s12974-015-0352-2.
  • 50 Varadkar S, Bien CG, Kruse CA , et al. Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurol 2014; 13 (2) 195-205
  • 51 Vora N, Cross J, Robinson R , et al. Epilepsy Surgery in Rasmussen Syndrome. Epilepsy Curr 2012; 12 (Suppl. 01) 241-242