Epilepsia, 30(5):569-572, 1989 Raven Press, Ltd., New York 8 International League Against Epilepsy Acquired Epileptic Aphasia (the Landau-Kleffner Syndrome) Due to Neurocysticercosis Enrique Otero, Sergio Cordova, Fabian Diaz, Imelda Garcia-Teruel, and Oscar H. Del Brutto Division de Neurologia, Instituto Nacional de Neurologia Y Neurocirugia, Mexico, D.F. Summary: A healthy 7-year-old boy developed a Ianpage disorder associated with clinical seizures and a paroxysmal EEG. Computed tomography and magnetic resonance imaging revealed a small cysticercus deep in the left Sylvian fissure. We postulate a cause and effect relationship between the parasitic cyst and the clinical man- ifestations supported by the strategic location of the cyst and the critical age at which this lesion developed. This case provides evidence that the syndrome of acquired epileptic aphasia may be explained in terms of an unilateral structural brain lesion. Key Words. EpilepsyAphasibNeurologic diagnosislanguage. The syndrome of acquired epileptic aphasia, first described by Landau and Kleffner (1957), is a childhood disorder characterized by acute or progressive loss of previously normal language function associated with a paroxysmal EEG (Dreifuss et al., 1985). Clinical seizures of various types, although not invariably present, occur in a large number of cases (Toso et al., 1981). The language disorder is not a true aphasia but a verbal auditory agnosia with secondary loss of speech (Rapin et al., 1977). Electrical abnormalities typically include bilateral spikes and spike-and-wave discharges with temporal lobe predominance (Shoumaker et al., 1974). The course and prognosis of acquired epileptic aphasia is variable; while positive correlation between the severity of the language disorder and EEG abnormality is usually found, correlation with clinical seizures is less striking (Landau and Kleffner, 1957). Seizures and electrical abnormalities usually respond to appropriate antiepileptic drug therapy (Mantovaniand Landau, 1980); however, the language disorder may persist despite good seizure control (van Harskamp et al., 1978). Bishop (1985) suggested that the longterm prognosis for language is related to age of onset; the younger the child the worse the prognosis. The pathogenesis of this syndrome is controversial. An autoimmune chronic encephalitis involving temporal lobes has been suggested (Worster- Drought, 1971) but not proved (Cole et al., 1988). Most authors consider that bilateral dysfunction is necessary to explain the language disorder and the EEG abnormality (Beaumanoir, 1985; Bishop, 1985; Cole et al., 1988). We report a patient with acquired epileptic aphasia whose neurodiagnostic studies revealed a single subarachnoid cysticercus in the left Sylvian fissure, near the cortical areas concerned with language. CASE REPORT A 7-year-old right-handed boy was evaluated because of a 1-year history of language difficulty and a seizure disorder. Pregnancy and delivery were unremarkable. The child was considered to have normal speech and comprehension. At age 6, he had several brief episodes of loss of awareness and responsivenessassociated with automatic movements of the hands; such episodes had an abrupt onset and termination and recurred more than 20 times per day. Two months later, his parents became aware that he did not respond to calls, although he still talked well. The school teacher noticed that the patient did not comprehend stories when they were read, and it was considered that he had a hearing defect. As the patient’s comprehension diminished, his speech production worsened; according to his mother, the speech was telegraphic. The patient pronounced “Uobeta” instead of “bottella” (battle), “manzan” instead of ‘‘manmna” (apple), and Received May 1988; revision accepted January 1989. Address correspondenceand =print requests to Dr. 0. H.Del Brutto at P.O. Box 3734,Guayaquil, Ecuador, South America. 569 E. OTERO ET AL. 570 “cani” instead of “ferrocanil” (railroad). In addi- tion, he omitted several articles and prepositions in sentences; he said “el va casa” instead of “el va a la casa” (he goes home). The articulation of consonants was normal, and he did not make consonant substitutions. Four months later, there was a 1week period during which he did not respond to any oral commands and his speech practically disappeared. After this time, language improved, but he still had a decrease in spontaneous speech and poor response to oral commands. He continued to have brief episodes of loss of awareness associated with automatic movements of the hands, although these were less frequently noticed. On admission, the neurologic examination was normal with the exception of an indifferent right plantar response. During the examination, hyperventilation provoked a brief episode of loss of awareness and automatic shaking of the hands. Neuropsychological testing showed a mild delay in response and sentence formulation with decreased comprehension for spoken language. WISC yielded a verbal IQ of 89, and performance IQ of 87. Audiometry did not indicate any hearing loss. EEG showed pseudorhythmic 2.5-3 Hz biphasic sharp and slow wave complexes over both anterior and mid-temporal regions with clear left predominance (Fig. 1); the background activity was diffusely slow. Lumbar puncture yielded a clear cerebrospinal fluid (CSF) with normal cytochemical analysis. A head computed tomographic (0scan revealed a small cysticercus in the left temporal lobe (Fig. 2A). Magnetic resonance imaging (MRI) showed a subarachnoid cystic lesion deep in the left Sylvian fissure (Fig. 2B). The patient was treated with 15 mg/kg valproate per day. In addition, a course of the anticysticercal drug albenda- F FIG. 1. EEG recordina showina sharo and slow wave complexes over ttie left‘ centrotemporal regions with contralateral transmission. DISCUSSION This patient fulfills clinical and electrographic criteria of acquired epileptic aphasia (Landau and Kleffner, 1957; Mantovani and Landau, 1980; Beaumanoir, 1985; Sawhney et al., 1988). He was a previously healthy boy who developed a language disorder associated with clinical seizures and a paroxysmal EEG. The language disorder was interpreted from formal neuropsychological testing as a verbal auditory agnosia, and the paroxysmal EEG showed sharp and slow wave complexes with temporal lobe predominance. We interpreted the seizures as absences because of the characteristics (Loiseau, 1985); nevertheless, these episodes may well correspond to complex partial seizures which have also been described in patients with acquired epileptic aphasia (Toso et al., 1981). In addition, our patient had neurocysticercosis documented by CT findings of a cystic lesion that decreased in size after a trial with anticysticercal drugs (Sotelo and Del Brutto, 1987). While the characteristic CT finding in neurocysticercosis is the presence of multiple cystic lesions, the finding of a single lesion does not exclude Z - F Fa-F, T4 -c4 Epilepsio, Vd.30, No.5. I W zole was given. During the first days of albendazole therapy, he experienced headaches which subsided with common analgesics. At the end of the trial, a CT scan showed reduction in size of the cyst. The patient remained asymptomatic, the language disorder improved, and seizures disappeared. Repeat EEG while receiving valproate showed only minor background slowing without sharp and slow wave complexes. The patient was discharged on a program of speech therapy. Follow-up at 6 months showed no recurrence. ; ACQUIRED EPILEPTIC APHASIA 571 FIG. 2. Plain CT scan (A) and TP-weighted MRI (B) showing a noncalcified subarachnoid cysticercus in the left Sylvian fissure (arrows). the diagnosis, since several cases with a single intracranial cyst have been reported (RodriguezCarbajal et al., 1983; Del Brutto and Sotelo, 1988). Likewise, the absence of CSF abnormalities should not be taken as proof against the diagnosis, since many patients with single lesions have localized inflammatory responses without CSF changes (Sotelo et al., 1985; Rosas et al., 1986; Del Brutto et al., 1988). We postulate that neurocysticercosis caused the syndrome of acquired epileptic aphasia in our patient. The cause-and-effect relationship between these two conditions is supported by the particular location of the parasitic cyst causing chronic irritation of adjacent brain parenchyma near the cortical areas concerned with language. Further spread of the epileptic discharges explains the bilateral EEG abnormality which was, nevertheless, more marked on the left (Fig. 1). Neurocysticercosis is a well-recognized cause of both epilepsy and focal neurologic deficitsincluding language disorder (Sotelo et al., 1985; Del Brutto and Sotelo, 1988). However, the particular combination of language disorder and seizures in such a way that resembles the syndrome of acquired epileptic aphasia has not been described in patients with this parasitic disease of the central nervous system. The critical age at onset and the strategic location of the cyst could explain this patient’s uncommon clinical picture. Several controversies exist about the etiology of the syndrome of acquired epileptic aphasia (Cole et al., 1988). It has often been considered that a lowgrade autoimmune encephalitis is the cause of this disorder; however, Cole et al. (1988) failed to demonstrate encephalitis in the temporal lobes of two patients with acquired epileptic aphasia. Sawhney et al. (1988) stated that “appropriate investigations” have ruled out tumors and vascular malformations as the cause of this syndrome; however, it is important to note that in only a minority of such patients were CT or MRI performed. On the other hand, Rapin et al. (1977) reported a patient with acquired epileptic aphasia whose angiogram showed a small angiomatousmalformation contined to the left parietal cortex which accounted for the clinical manifestations;further follow-up of this patient with MRI failed to document such malfonnation (Rapin, 1989) stressing the fact that even sophisticated neurodiagnostic studies may show no gross cerebral abnormalities in patients with acquired epileptic aphasia. Our case provides evi- E@epsia. Vd.30. No.J. I989 E. 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An unusual form of acquired aphasia in children. Dev Med Child Neurol 1971;13:56>71. R f i s m Un petit garcon de 7 ans, en bonne sant6, a present6 un trouble du langage asswit5 B des crises cliniques et B des paroxysmes EEG. La scanographie et I’IRM ont m i s en evidence un petit kyste de cysticercose dans la profondeur de la scissure sylvienne gauche. Les auteurs pensent qu’il existe une relation de cause effet entre le kyste parasitaire et les manifestationscliniques, en se fondant sur la localisation significative du kyste et sur I’&e critique auquel cette lesion s’est dtvelopp6e. Ce cas fournit la preuve qu’un syndrome d’aphasie Cpileptique acquise peut4tre expliquC par I’existence d‘une lesion ctrebrale structurale unilaterale. (P. Genton, Marseille) RESUMEN Un niiio sano de 7 aios de edad desarroll6 un trastorno del lenguaje asociado a ataques clinicos y a un EEG paroxistico. Una tomografia Computarizada y U M Resonancia Magnetics revelaron un pequefio cisticerco localiido en las profundidades de la cisura de Silvio izquierda. Se postula una relacih causaefecto entre el quiste parasitic0 y las manifestaciones clinicas debido a la localizaci6nestrategica del quiste y a la edad critica en la que esta lesi6n se produjo. Este caso aporta evidencia de que el sindrome de afasia epilCptica adquirida puede ser explicad0 en terminos de una lesi6n cerebral estructural unilateral. (A. Portera-Sanchez, Madrid) ZUSAMMENFASSUNG Ein bis dahin gesunder Junge von 7 Jahren entwickelte eine Sprachstonmg, welche mit klinischen Anfaen und paroxysmalen EEG-Verbderungen einherging. CT und NMR entdeckten eine kleine Cystizercus-Zyste tief in der Linken Sylvischen Furche. Wu postulieren einen kausalen Zusammenhang zwischen klinischem Bild und Zyste aufgrund ihrer Lage und des kritischen Erkrankungsalters. Dieser Fall unterstiitzt die Ansicht, das erworbene Aphasie-Epilepsie-Syndrom beruhe auf einer strukturellen Hirnlaesion. (C. G. Lipinski, Heidelberg-Neckargemiind)