Neurological disorders demonstrate remarkable specificity in afflicting precise brain regions. They are most frequently sporadic in origin and they are devastating because of the progressive impairment of cognitive and/or motor functions. Our studies have found that some neurological diseases may actually reflect a dysfunction of the immune system in which highly specific autoantibodies are generated that bind neuronal glutamate receptors and directly affect their function in normal neurotransmission. Understanding at the molecular level how the immune system participates in neurological disease will ultimately lead to more effective therapeutic approaches to some of these disorders with presently unknown etiology. NEURO SCIENTIST 4:373-379, 1998
Lange KW, Riederer P.Glutamatergic drugs in Parkinson's diseaseLife Sci1994;55:2067-2075
2.
Giacobini E.Nicotine acetylcholine receptors in human cortex: aging and Alzheimer's disease In: Lippiello PM, Collins AC, Gray AC, Robinson JH, editors. Biology of nicotine.New York: Raven Press, 1992 p 183-215
3.
Koningsmark VW , Weiner LPThe olivopontocerebellar atrophies. A reviewMedicine1970;4:227-241.
4.
Patrick J., Lindstrom J.Autoimmune response to acetylcholine receptorNature1973;180:871-872.
5.
Olsen, RW, Avoli M.GABA and epileptogenesisEpilepsia1997; 38:399-407.
6.
Choi DWGlutamate neurotoxicity and diseases of the nervous systemNeuron1988;1:623-634
7.
Olney JWExcitotoxic amino acids and neuropsychiatric disordersAnn Rev Pharmacol Toxicol1990;30:47-71.
8.
Monaghan DT, Bndges RJ, Cotman CWThe excitatory ammo acid receptors: their classes, pharmacology, and distinct properties in the function of the central nervous systemAnn Rev Pharmacol Toxicol1989;29.365-402.
9.
Hollmann M., Heinemann S.Cloned glutamate receptorsAnn Rev Neurosci1994; 17:31-108
10.
Rogers SW, Andrews PI, Gahring LC, et alAutoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitisScience1994;265:648-652
11.
Twyman RE, Gahnng LC, Speiss J., Rogers SWGlutamate receptor antibodies activate a subset of receptors and reveal an agomst binding siteNeuron1995;14:755-762.
12.
Gahnng LC, Twyman RE, Greenlee JE, Rogers SWAutoantibodies to neuronal glutamate receptors in patients with paraneoplastic neurodegenerative syndrome enhance receptor activationMol Med1995;1:245-253.
13.
Gahring LC, Twyman RE, Rogers SWAutoantibodies to glutamate receptor subunit GluR2 in non-familial ohvopontocerebellar degenerationNeurology1997;48:494-500.
14.
Rasmussen, TBChromc encephalitis and seizure: Historical introduction In: Andermann F, editor. Chronic encephalitis and epilepsy. Rasmussen's syndrome.Stoneham (MA) : Butterworth-Heinemann, 1991 . p. 1-4.
15.
Ogom H., Andermann F., Rasmussen TBThe natural history of chronic encephalitis and epilepsy; a study of the MRI series of forty-eight cases In Andermann F, editor. Chronic encephalitis and epilepsy: Rasmussen's syndrome.Stoneham (MA): Butterworth-Heinemann; 1991 . p. 7-35.
16.
Vining EP, Freeman JM, Pillas DJ, et alWhy would you remove half a brain? The outcome of 58 children after hemispherectomy— the Johns Hopkins experience: 1968 to 1996Pediatrics1997;100: 163-171
17.
DeBates MH, Vincent, A.Myasthenia gravis In. Bona CA, Siminovitch KA, Zanetti M, Theofilopoulos AN, editors. The molecular pathology of autoimmune diseases.Reading (UK): Harwood Academic Publishers; 1993. p 1-14.
18.
Karni A., Zisman E., Katz-Levy Y., et al Reactivity of T cells from seronegative patients with myasthenia gravis to T cell epitopes of the human acetylcholine receptorNeurology1997,19:1638-1642.
19.
Engel AG, Hutchinson DO, Nakano S., et al.Myasthenic syndromes attributed to mutations affecting the epsilon subunit of the acetylcholine receptorAnn NY Acad Sci1993;681:496-508.
20.
Andermann E. , Ogum H., Guttman RD, et al.Genetic aspects of chronic encephalitis In: Andermann F, editor. Chronic encephalitis and epilepsy: Rasmussen's syndrome.Stoneham (MA) : Butterworth-Heinemann ; 1991 . p. 167-175.
21.
Power C., Poland SD, Blume WT, Girvin JP, Rice Gpa.Cytomegalovirus and Rasmussen's encephalitisLancet1990,336. 1282-1284.
22.
Jay V., Becker Le, Otsubo H., et alChronic encephalitis and epilepsy (Rasmussen's encephalitis): detection of cytomegalovirus and herpes simplex virus 1 by the polymerase chain reaction and in situ hybridizationNeurology1995;45:108-117
23.
Asher DM, Gajdusek DCVirology studies in chronic encephahtis In: Andermann F, editor. Chronic encephalitis and epilepsy Rasmussen's syndrome.Stoneham (MA): Butterworth-Hememann; 1991 . p 147-158.
24.
McLachlan RM , Levin S., Blume , WTTreatment of Rasmussen's syndrome with ganciclovirNeurology1996 ;47:925-928.
25.
Dabbagh O., Gascon G., Crowell J., Bamoggadam F.Intraventncular interferon-α stops seizures in Rasmussen's encephalitis A case reportEpilepsia1997;38:1045-1049
26.
Andrews PI, Dichter MA, Berkovic SF, Newton MR, McNamara JAPlasmapheresis in Rasmussen's encephalitisNeurology1996, 46:242-246.
27.
Hollmann M. , Maron C., Heinemann S.N-glycosylation site tagging suggests a three transmembrane domain topology for the glutamate receptor GluR1Neuron1994;13:1331-1343.
28.
Stern-Bach Y., Bettler B., Hartely M., Sheppard PO, O'Hara PJ, Heinemann SFAgonist selectivity of glutamate receptors is specified by two domains structurally related to bacterial ammo acid-binding proteinsNeuron1994; 13:1345-57.
29.
Carlson NG, Gahnng LC, Twyman RE, Rogers SWIdentification of ammo acids in the glutamate receptor subunit, GluR3, important for antibody-binding and receptor activationJ Biol Chem1997,272.11295-11301.
30.
Neuwelt EA, Frankel EPThe challenge of the blood-brain bamer In Neuwelt EA, editor. Implications of the blood-brain barrier and its manipulation Vol 1. New York: Plenum , 1989. p. 1-26.
31.
Streit WJ, Graeber MB, Kreutzberg GWFunctional plasticity of microglia: a reviewGlia1988;1:301-307.
32.
Wekerle H.Lymphocyte traffic to the brain In Pardridge WH, editor. The blood-brain barrier cellular and molecular biology.New York: Raven Press; 1993. p. 67-85.
33.
Zamvil SS, Steinman L.The T lymphocyte in expenmental allergic encephalomyelitisAnn Rev Immunol1990;8:579-621.
34.
Friedman A. , Kaufer D., Semer J., Hendler I., Soreq H., Tur-Kaspa I.Pyndostigmine brain penetration under stress enhances neuronal excitability and induces early immediate transcriptional responseNat Med1996;2:1382-1385.
35.
Rogers SW, Hughes T., Hollmann M., Gasic G., Denens E., Heinemann S.The characterization and localization of the glutamate receptor subunit, GluRl, in the rat brainJ Neurosci1991, 11:2713-2724
36.
Oztas B., Camurcu S., Kaya M.Influence of sex on the blood brain bamer permeability dunng bicuculline induced seizuresInt J Neurosci1992;65:131-139.
37.
Candore G., DiLorenzo G., Mansueto P., et al.Prevalence of organ-specific and non organ-specific autoantibodies in healthy centenanansMech Ageing Dev1997 ;94:183-190.
38.
Cunnigham M.Bactenal Antigen Mimicry In: Bona CA, Siminovitch KA, Zanetti M, Theofilopoulos AN, editors. The molecular pathology of autoimmune diseases.Reading (UK)Harwood Academic Publishers; 1993. p. 245-256
39.
Ehrenstein M., Isenberg D.Autoimmunity associated with infection : Leprosy, acute rheumatic fever, and Lyme disease Curr OpinImmunol1991;3:930-932
40.
Gahnng LC, Carlson NG, Rogers SWAntibodies prepared to neuronal glutamate receptor subunit 3 bind IFNα-Receptors: Implications in an autoimmune diseaseAutoimmunity1998 (in press).
41.
Mäkelä O., Karjalainen K.Inherited immunoglobulin idiotypes of the mouseImmunol Rev1977;34:119-138.
42.
Uccelli LY, Laxer KD, Jeong MC, et al.Local-clonal expansion in infiltrating T lymphocytes in chronic encephalitis of RasmussenJ Immunol1997,158:1428-1437.
