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Is Cerebral Arteritis the Cause of the Landau-Kleffner Syndrome? Four Cases in Childhood with Angiographic Study

Published online by Cambridge University Press:  18 September 2015

Ignacio Pascual-Castroviejo ,
V. López Martín ,
A. Martínez Bermejo  and
A. Pérez Higueras
Ignacio Pascual-Castroviejo*
Affiliation:
Departments of Paediatric Neurology and Neuroradiology, Hospital Infantil “La Paz”, Madrid, Spain
V. López Martín*
Affiliation:
Departments of Paediatric Neurology and Neuroradiology, Hospital Infantil “La Paz”, Madrid, Spain
A. Martínez Bermejo*
Affiliation:
Departments of Paediatric Neurology and Neuroradiology, Hospital Infantil “La Paz”, Madrid, Spain
A. Pérez Higueras*
Affiliation:
Departments of Paediatric Neurology and Neuroradiology, Hospital Infantil “La Paz”, Madrid, Spain
*
Servicio de Neurología Infantil, Hospital “La Paz”, Paseo de la Castellana 261, Madrid 28046, Spain
Servicio de Neurología Infantil, Hospital “La Paz”, Paseo de la Castellana 261, Madrid 28046, Spain
Servicio de Neurología Infantil, Hospital “La Paz”, Paseo de la Castellana 261, Madrid 28046, Spain
Servicio de Neurología Infantil, Hospital “La Paz”, Paseo de la Castellana 261, Madrid 28046, Spain
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Abstract:

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Four children with Landau-Kleffner syndrome were studied over a six year period. They presented with acquired aphasia, epilepsy, and focal or generalized EEG discharges which were exacerbated during sleep. In addition, cerebral angiography demonstrated isolated arteritis of some branches of the carotid arteries in all cases. Computed tomographic and magnetic resonance images were normal. Nicardipine in a dose of 1 to 2 mg/kg/day, added to conventional anticonvulsant drugs provided effective supplementary control of seizures, of paroxysmal EEG discharges, and of language and behavioural disturbances, even several years after the onset of the disorder and in patients whose response to other medications, including steroids, had been poor. Interruption of nicardipine administration was followed by relapse of the language disorder. Repeat angiography was performed in all four patients and showed recanal-ization of obstructed vessels in two cases. Focal cerebral vasculitis may be the pathogenesis of the Landau-Kleffner syndrome and calcium channel blockers such as nicardipine may be effective and specific therapy.

Type
Articles
Information
Canadian Journal of Neurological Sciences , Volume 19 , Issue 1 , February 1992 , pp. 46 - 52
Copyright
Copyright © Canadian Neurological Sciences Federation 1992

References

1. Landau, WM, Kleffner, FR.Syndrome of acquired aphasia with convulsive disorder in children. Neurology 1957; 7: 523–530.CrossRefGoogle ScholarPubMed
2. Cole, AJ, Andermann, F, Taylor, L, et al. The Landau-Kleffner syndrome of acquired epileptic aphasia: unusual clinical outcome, surgical experience, and absence of encephalitis. Neurology 1988; 38:31–38.CrossRefGoogle ScholarPubMed
3. Mantovani, JF, Landau, WM.Acquired aphasia with convulsive disorder: course and prognosis. Neurology 1980; 30: 524–529.CrossRefGoogle ScholarPubMed
4. Lou, HC, Brandt, S, Bruhn, P.Aphasia and epilepsy in childhood. Acta Neurol Scand 1977; 56: 46–54.CrossRefGoogle ScholarPubMed
5. Gascon, G, Victor, D, Lombroso, CT, et al. Language disorder, convulsive disorder and electro-encephalographic abnormalities. Arch Neurol 1973; 28: 156–162.CrossRefGoogle Scholar
6. Worster-Drought, C.An unusual form of acquired aphasia. Dev Med Child Neurol 1971; 13:563–571.CrossRefGoogle Scholar
7. Bishop, DVM.Age of onset and outcome in “acquired aphasia with convulsive disorders” (Landau-Kleffner syndrome). Dev Med Child Neurol 1985; 27: 705–712.Google ScholarPubMed
8. Holmes, GL, McKeever, M, Saunders, Z.Epileptiform activity in aphasia of childhood: an epiphenomenon? Epilepsia 1981; 22: 631–639.CrossRefGoogle ScholarPubMed
9. Deonna, T, Beaumanoir, A, Gaillard, F, et al. Acquired aphasia in childhood with seizures disorder: a heterogenous syndrome. Neuropadiatrie 1977; 8: 263–273.CrossRefGoogle Scholar
10. Deonna, T, Peter, C, Ziegler, AL.Adult follow-up of the acquired aphasia-epilepsia syndrome in childhood. Report of 7 cases. Neuropediatrics 1989; 20: 132–138.Google Scholar
11. Nakano, S, Okuno, T, Mikawa, H.Landau-Kleffner syndrome. EEG topographic studies. Brain Dev 1989; 11: 43–50.CrossRefGoogle ScholarPubMed
12. Ansink, BJJ, Sarphatie, H, van Dongen, HR.The Landau-Kleffner syndrome. Case report and theoretical considerations. Neuropediatrics 1989; 20: 170–172.CrossRefGoogle ScholarPubMed
13. Kellerman, K.Recurrent aphasia with subclinical bioelectric status epilepticus. Eur J Pediatr 1978; 128: 207–212.CrossRefGoogle Scholar
14. Rapin, I, Mattis, S, Rowan, AJ, et al. Verbal auditory agnosia in children. Dev Med Child Neurol 1977; 19: 192–207.CrossRefGoogle ScholarPubMed
15. Dulac, O, Billard, C, Arthuis, M.Aspects électrocliniques et évolutifs de l’épilepsie dans le syndrome aphasie-épilepsie. Arch Fr Pediatr 1983; 40: 299–308.Google Scholar
16. Dugas, M, Masson, M, Le Heuzey, MF, et al. Aphasie “acquise” de l’enfant avec épilepsie (syndrome de Landau et Kleffner): Douze observations personnelles. Rev Neurol 1982; 138: 755–780.Google Scholar
17. Shoumaker, RD, Bennett, DR, Bray, PF, et al. Clinical and EEG manifestations of an unusual aphasie syndrome in children. Neurology 1974; 24: 10–16.Google Scholar
18. McKinney, W, McGreal, DA.An aphasie syndrome in children. Can Med Assoc J 1974; 110: 637–639.Google Scholar
19. Rodriguez, I, Niedermeyer, E.The aphasia epilepsy syndrome in children: electroencephalographic aspects. Clin Electro-encephalogr 1982; 13: 23–35.Google ScholarPubMed
20. Toso, V, Moschini, M, Gagnin, G, et al. Aphasie acquise de l’enfant avec épilepsie. Trois observations et revue de la littérature. Rev Neurol (Paris) 1981; 137: 425–434.Google Scholar
21. Giovanardi Rossi, P, Pazzaglia, P, Frank, G.Afasia acquisita con anomalie convulsive nell’età evolutiva: estudio clinico, neuropsicologico ed elettroencefalografico di un caso. Riv Neurol 1976; 46: 130–162.Google Scholar
22. Hirsh, E, Marescaux, C, Maquet, P, et al. Landau-Kleffner syndrome: A clinical and EEG study of five cases. Epilepsia 1990; 31: 756–767.CrossRefGoogle Scholar
23. Beaumanoir, A, Ravnik, I, Tassinari, CA.Le syndrome de Landau-Kleffner. In: Roger, J, Dravet, C, Bureau, M, Dreifuss, F, Wolf, P, eds. Les syndromes Epileptiques de L’Enfant et al L’Adolescent. London: John Libbey Eurotext Ltd, 1984; 185–195.Google Scholar
24. Otero, E, Cordova, S, Diaz, F, et al. Acquired epileptic aphasia (Landau-Kleffner syndrome) due to neurocysticercosis. Epilepsia 1989; 30: 569–572.CrossRefGoogle ScholarPubMed
25. Sawhney, I, Suresh, N, Dhand, U, et al. Acquired aphasia with epilepsy-Landau-Kleffner syndrome. Epilepsia 1988; 29: 283–287.Google ScholarPubMed
26. Billard, C, Autret, A, Laffont, F, et al. Electrical status epilepticus during sleep in children: a reappraisal from eight new cases. In: Sterman, M, Shouse, M, Passouant, P, eds. “Sleep and Epilepsy”. London: Academic Press, 1982; 481–490.Google Scholar
27. Lerman-Sagie, T, Statter, M, Lerman, P.Low erythrocyte zinc content in acquired aphasia with convulsive disorder (Landau-Kleffner syndrome). J Child Neurol 1987; 2: 28–30.CrossRefGoogle ScholarPubMed
28. Pascual-Castroviejo, I.Nicardipine in the treatment of acquired aphasia and epilepsy. Dev Med Child Neurol 1990; 32: 930 (letter).CrossRefGoogle ScholarPubMed
29. Ferris, EJ, Levine, HL.Cerebral arteritis: classification. Radiology 1973; 109:327–341.Google ScholarPubMed
30. Cupps, TR, Moore, PM, Fauci, AS.Isolated angiitis of the central nervous system. Prospective diagnostic and therapeutic experience. Am J Med 1983; 74: 97–105.Google ScholarPubMed
31. Craven, RS, French, JK.Isolated angiitis of the central nervous system. Ann Neurol 1985; 18: 263–265.CrossRefGoogle ScholarPubMed
32. Marescaux, C, Hirsch, E, Fink, S, et al. Landau-Kleffner syndrome: A pharmacologic study of five cases. Epilepsia 1990; 31: 768–777.CrossRefGoogle ScholarPubMed
33. Ravnik, I.A case of Landau-Kleffner syndrome: effect of intravenous diazepan. In: Roger, J, Dravet, C, Bureau, M, Dreifuss, FE, Wolf, P, eds. Epileptic syndromes in infancy, childhood and adolescence. London: John Libbey, 1985; 192–193.Google Scholar
34. Lerman, P, Lerman-Sagie, T.Early steroid therapy in Landau-Kleffner syndrome. In: Manelis, J, Bental, E, Loeber, JN, Dreifuss, FE, eds. Advances in epileptology. XVIIth International Epilepsy Symposium. New York: Raven Press, 1989; 330–332.Google Scholar
35. Meyer, FB, Anderson, RE, Sundt TM Jr, et al. Selective central nervous system calcium channel blockers: a new class of anticonvulsant agents. Mayo Clin Proc 1986; 61: 239–247.CrossRefGoogle ScholarPubMed
36. Morocutti, C, Pierelli, F, Sanarelli, L, et al. Antiepileptic effects of a calcium antagonist (nimodipine) on cefazolin-induced epileptogenic foci in rabbits. Epilepsia 1986; 27: 498–503.CrossRefGoogle ScholarPubMed
37. Binnie, CD, de Beukelaar, F, Meijer, JWA, et al. Open dose-ranging trial of flunarizine as add-on therapy in epilepsy. Epilepsia 1985; 26: 424–428.Google ScholarPubMed
38. Overweg, J, Binnie, CD, Meijer, JWA, et al. Double-blind placebocontrolled trial of flunarizine as add-on therapy in epilepsy. Epilepsia 1984; 25: 217–222.Google ScholarPubMed
39. Keene, D, Whiting, S, Humphreys, P, et al. Flunarizine as a supplementary medication in refractory childhood epilepsy: A double-blind crossover study. Can J Neurol Sci 1989; 16: 191–193.CrossRefGoogle ScholarPubMed
40. Pascual-Castroviejo, I.Tratamiento de la epilepsia rebelde con nicardipina. Drugs of Today 1988; 24 (Suppl 3): 191–201.Google Scholar
41. Maquet, P, Hirsch, E, Dive, D, et al. Cerebral glucose utilization during sleep in Landau-Kleffner syndrome: A PET study. Epilepsia 1990; 31:778–783.CrossRefGoogle ScholarPubMed