Epilepsia, 33( I):8487, 1992 Raven Press, Ltd., New Yolk 0 International League Against Epilepsy
Aphasia as the Sole Manifestation of Simple Partial Status Epilepticus Christine R. Wells, Douglas R. Labar, and Gail E. Solomon Department o j Neurology and Neurosciences, Division o j Clinical Neurophysiology, New York Hospital-Cornell University Medical Center New York, New York, U . S . A .
Summary: Aphasia due to simple partial status epilepticus is rare, particularly in the absence of a seizure history. N o previous report describes acute aphasia as the sole clinical manifestation of EEG-monitored status epilepticus, with prompt resolution with treatment. We report a 45-year-old man with a left temporal glioblastoma who acutely developed a global aphasia, during which an
EEG revealed continual repetitive sharp waves emanating from the left hemisphere. After injection of i.v. diazepam, the EEG seizure activity ceased, and the patient’s language output returned to preseizure levels. Key Words: Partial epilepsy-Temporal lobe-Aphasia-Status epilepticus-Simple partial seizures.
Ictal or postictal behavioral changes can include aphasia, speech arrest, or speech vocalization. Reversible aphasia as the sole manifestation of partial status epilepticus is rare, particularly without a history of prior seizures (Racy et al., 1980; Dinner et al., 1981; Rosenbaum et al., 1986). No previous reports describe such a phenomenon in a previously seizure-free patient, with the diagnosis confirmed by simultaneous EEG monitoring, that resolved with treatment. We describe such a patient.
rections to the driver. He walked 1.5 miles to his office, at which point his co-workers noted his language difficulty and brought him to the hospital. Examination revealed an alert and cooperative patient, physically intact except for a mild right pronator drift. On language testing, he could speak or repeat only one or two words at a time, and could not name objects. He followed one-step instructions but not more complex verbal commands. Serum PHT level was 14.7 pg/ml (therapeutic range 10-20 kg/ml). A 16-channel EEG revealed continuous stereotyped rhythmical repetitive 4-Hz sharp waves emanating from the entire left hemisphere, intermixed with rhythmic theta activity (Fig. 1A). Three milligrams of i.v. diazepam (DZP) were given over 1 min, and the EEG pattern changed to periodic lateralized epileptiform discharges (PLEDs) at 1 Hz, which lasted for 10 min (Fig. 1B). During the PLEDs, the exam was unchanged; the patient spoke only one or two words at a time and followed one-step commands. After an additional 4 mg of i.v. DZP, the PLEDs were replaced by slowing (Fig. 1C). Coincident with the disappearance of PLEDs, the patient abruptly became fluent, speaking in full intelligible sentences (“I’m well enough to go home now”). Although improved, some comprehension difficulties remained. He was given an additional 500 mg of oral PHT, and his language ability returned to near baseline by the next morning. He fully recalled detailed events during the episode of
-
CASE REPORT
A 45-year-old right-handed man had a left temporal lobe, pathologically confirmed glioblastoma multiforme, treated with partial surgical resection, whole-brain radiation (6100 cGy), and i.v. carmustine (BCNU) therapy. He received phenytoin (PHT) for seizure prophylaxis, and returned to executive-level work without difficulty. An occasional difficulty remembering personal names was his only complaint. Seven months postoperatively, on the day of a BCNU treatment, he noted sudden inability to speak. He hailed a taxi, but was unable to give di-
Received September 1990; revision accepted February 1991. Address correspondence to Dr. C. R. Wells at 500 Lilly Road NE, No. 2304, Olympia, WA 98506, U.S.A.
84
APHASIA AND STATUS EPILEPTICUS
D
A
85
C
FIG. 1. A: (EEG) during aphasia, illustrating regularly repetitive 4-Hz sharp wave activity from the left hemisphere, constituting electrographic status epilepticus. B: Periodic lateralized epileptiform discharges seen transiently following i.v. diazepam (DZP), without associated language improvement. C: EEG after treatment with DZP. Epileptiforrn activity was replaced by left hemispheral slowing, and language function improved.
status epilepticus (such as shopping, hailing a cab, walking 30 blocks to his office, and undergoing an EEG), and was aware of his previous difficulty speaking. A magnetic resonance imaging scan revealed interval deterioration, with enlargement of the left temporal glioblastoma. DISCUSSION Previous reports based on clinical observations (Penfield and Rasmussen, 1949; Bingley, 1958; Se-
rafetinides and Falconer, 1969) and video EEG studies (Koerner and Laxer, 1988; Gabr et al., 1989) have detailed language dysfunction due to seizures originating from left temporal lobe foci. These studies and several other case reports illustrate the difficulty of correlating language testing with EEG findings and response to therapy, either because of impaired consciousness during the seizure (i.e., complex partial) (Knight and Cooper, 1986; Racy et al., 1980), or because of lack of response to therapy
Epilepsiu, Vol. 33, N o . I, 1992
C . R . WELLS ET AL.
(Hamilton and Matthews, 1979; Dinner et al., 1981; Primavera et al., 1988). The temporal coincidence of our patient’s aphasia and electrographic seizure patterns indicates that aphasia was the manifestation of an ictal event, which resolved when treated with an i.v. antiepileptic drug (AED). In previous reports, aphasic status epilepticus was considered to be a manifestation of complex partial status epilepticus (DePasquet et al., 1976; Hamilton and Matthews, 1979; Racy et al., 1980; Dinner et al., 1981; Knight and Cooper, 1986; Rosenbaum et al., 1986; Primavera et al., 1988). In contrast, our case represents simple partial status epilepticus rather than complex partial status epilepticus since there was no alteration in consciousness (Commission, 1981). Although formal neuropsychological evaluation of higher cortical functions was not carried out during the ictus, the patient was capable of initiating and carrying out complicated volitional behaviors (hailing a cab, walking 1.5 miles on the streets of downtown New York), and following nonverbal commands during the basic neurological examination and the EEG, and he could recall events during the ictus. Thus, the patient demonstrated preserved responsiveness and recall (meeting the accepted operational definition for preserved consciousness during a seizure), and the seizure could be classified as simple partial (Commission, 1981). The normal level of consciousness allowed detailed correlation of the findings on language exam with EEG before, during, and after treatment. During the initial electrographic seizure component, the patient was globally aphasic. When i.v. DZP converted the EEG pattern to PLEDs, the patient remained globally aphasic; thus, this should be considered continuing ictal activity (Brenner and Schaul, 1990). When the PLEDs were replaced by slowing, the patient returned to near baseline language function, thus indicating termination of the ictus. It may be hypothesized that the DZP produced neuronal inhibition by activating postsynaptic membrane chloride channels via the benzodiazepine receptor, and thus initially converted the continuous ongoing electrographic ictal pattern to an intermittent epileptiform pattern (PLEDs), and subsequently terminated the seizure. A preexisting PHT level of 14.7 pg/ml may have acted to prevent local spread of the discharge and a secondary generalized seizure. As suggested by Rosenbaum et al. (1986), isolated epileptic aphasia may represent an artifact of partial seizure control. Because acute aphasia more commonly results from a transient ischemic attack, stroke, or perhaps
Epilepsia, Vol. 33, No. 1 , 1992
even a cerebral hemorrhage, recognizing the possibility of an ictal event in a patient without a previous seizure history can be difficult; we are aware of only three such reported cases (Racy et al., 1980; Dinner et al., 1981; Rosenbaum et al., 1986). However, considering seizure in the differential diagnosis can lead to a diagnostic EEG and prompt AED therapy. Acknowledgment: We thank Dr. Fred Plum for a critical review of this manuscript, and Dr. Frank Petito for permission to report his patient.
REFERENCES Bingley T. Ictal mental symptoms of temporal lobe epilepsy. Acta Psych Neurol Scan Suppl 1958;120:95-9. Brenner RP, Schaul N . Periodic EEG patterns: classification, clinical correlation, and pathophysiology. J Clin Neurophysiol 1990;7:249-67. Commission on classification and terminology of the International League Against Epilepsy. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsiu 1981;2:489-501. DePasquet EG, Gaudin ES, Bianchi A, et al. Prolonged and monosymptomatic dysphasia status epilepticus. Neurology 1976;26:244-7. Dinner DS, Lueders H , Lederman R, et al. Aphasic status epilepticus: a case report. Neurology 1981;31:888-90. Gabr M, Liiders H , Dinner D, et al. Speech manifestations in lateralization of temporal lobe seizures. Ann Neurol 1989;25: 82-7. Hamilton NG, Matthews T. Aphasia: the sole manifestation of focal status epilepticus. Neurology 1979;29:745-8. Knight RT, Cooper J . Status epilepticus manifesting as reversible Wernicke’s aphasia. Epilepsiu 1986;27:301-4. Koerner M, Laxer KD. Ictal speech, postictal language dysfunction, and seizure lateralization. Neurology 1988;38:634-6. Penfield W, Rasmussen T. Vocalization and arrest of speech. Arch Neurol Psychiatry 1949;61:21-7. Primavera A, Bo G, Venturi S. Aphasic status epilepticus. Eur Neurol 1988 ;28:255-7. Racy A, Osborn MA, Vern BA, et al. Epileptic aphasia: first onset of prolonged monosymptomatic status epilepticus in adults. Arch Neurol 1980;37:419-22. Rosenbaum DH, Siege1 M, Barr WB, et al. Epileptic aphasia. Neurology 1986;36:822-5. Serafetinides EA, Falconer MA. Speech disturbance in temporal lobe seizures: a study in 100 epileptic patients submitted to anterior temporal lobectomy. Brain 1963;86:333-46.
RESUME L’aphasie est liCe a un etat de ma1 kpileptique partiel simple et rare, en particulier en I’absence d’antecedents de crises. I1 n’y a pas de cas publies qui decrivent l’aphasie comme seule manifestation clinique d’un etat de ma1 6pileptique surveil16 par EEG, disparaissant rapidement avec le traitement. Les auteurs rapportent l’observation d’un homme de 45 ans prCsentant un glioblastome temporal gauche qui a present6 une aphasie globale aigue; pendant cet Cpisode I’EEG a mis en evidence des ondes lentes angulaires continues, rtpktees, hemisphkriques gauches. Aprts injection de diazCpam intraveineux, I’activitC EEG critique a disparu et le langage du patient a recupere son niveau antCrieur. (P. Genton, Muuseilk)
APHASIA A N D STATUS EPILEPTICUS
87
RESUMEN
ZUSAMMENFASSUNG
L a afasia, como manifestacion de un status epilepticus parcial simple, ocurre raramente, particularmente, en ausencia de una historia de ataques. No hay informes previos que describa la fase aguda como la unica manifestacion de status epilepticus monitorizado con EEG y rapida resolucion con el tratamiento. Los autores describen un hombre con 45 atios que tenia un glioblastoma del lobulo temporal izquierdo que presento, de forma aguda, una afasia global durante la cual un EEG mostr6 ondas agudas repetitivas y continuas que se originaban en el hemisferior izquierdo. DespuCs de la inyeccion intravenosa de diacepam la actividad del EEG ces6 y el lenguaje expresivo del enfermo volvi6 a 10s niveles preataque.
Aphasie als Status epilepticus einfacher Partialanfalle ist selten, besonders bei Abwesenheit von Anfallen in der Vorgeschichte. Bisher gibt es keinen Bericht iiber eine Aphasie als einziges klinisches Zeichen eines irn EEG aufgezeichneten Status epilepticus mit prompten Ansprechen auf Therapie. Wir beschreiben einen 45 Jahre alten Patienten mit einem linkstemporalem Glioblastom, welcher akut eine globale Aphasie entwickelte. Im EEG fanden sich kontinuierlich repetitive sharpwaves im Bereich der linken Hemisphare. Nach intervenoser GAbe von Diazepam sistierte die Krampfaktivitat im EEG und die Sprache des Patienten kehrt zu ihrer ursprunglichen Funktion zuriick.
(A. Portera-Sanchez, Madrid)
(C. G. Lipinski, HeidelbergiNeckurgemund)
Epilepsiu, Vol. 33, NO. 1, 1992
Aphasia as the Sole Manifestation of Simple Partial Status Epilepticus Christine R. Wells, Douglas R. Labar, and Gail E. Solomon Department o j Neurology and Neurosciences, Division o j Clinical Neurophysiology, New York Hospital-Cornell University Medical Center New York, New York, U . S . A .
Summary: Aphasia due to simple partial status epilepticus is rare, particularly in the absence of a seizure history. N o previous report describes acute aphasia as the sole clinical manifestation of EEG-monitored status epilepticus, with prompt resolution with treatment. We report a 45-year-old man with a left temporal glioblastoma who acutely developed a global aphasia, during which an
EEG revealed continual repetitive sharp waves emanating from the left hemisphere. After injection of i.v. diazepam, the EEG seizure activity ceased, and the patient’s language output returned to preseizure levels. Key Words: Partial epilepsy-Temporal lobe-Aphasia-Status epilepticus-Simple partial seizures.
Ictal or postictal behavioral changes can include aphasia, speech arrest, or speech vocalization. Reversible aphasia as the sole manifestation of partial status epilepticus is rare, particularly without a history of prior seizures (Racy et al., 1980; Dinner et al., 1981; Rosenbaum et al., 1986). No previous reports describe such a phenomenon in a previously seizure-free patient, with the diagnosis confirmed by simultaneous EEG monitoring, that resolved with treatment. We describe such a patient.
rections to the driver. He walked 1.5 miles to his office, at which point his co-workers noted his language difficulty and brought him to the hospital. Examination revealed an alert and cooperative patient, physically intact except for a mild right pronator drift. On language testing, he could speak or repeat only one or two words at a time, and could not name objects. He followed one-step instructions but not more complex verbal commands. Serum PHT level was 14.7 pg/ml (therapeutic range 10-20 kg/ml). A 16-channel EEG revealed continuous stereotyped rhythmical repetitive 4-Hz sharp waves emanating from the entire left hemisphere, intermixed with rhythmic theta activity (Fig. 1A). Three milligrams of i.v. diazepam (DZP) were given over 1 min, and the EEG pattern changed to periodic lateralized epileptiform discharges (PLEDs) at 1 Hz, which lasted for 10 min (Fig. 1B). During the PLEDs, the exam was unchanged; the patient spoke only one or two words at a time and followed one-step commands. After an additional 4 mg of i.v. DZP, the PLEDs were replaced by slowing (Fig. 1C). Coincident with the disappearance of PLEDs, the patient abruptly became fluent, speaking in full intelligible sentences (“I’m well enough to go home now”). Although improved, some comprehension difficulties remained. He was given an additional 500 mg of oral PHT, and his language ability returned to near baseline by the next morning. He fully recalled detailed events during the episode of
-
CASE REPORT
A 45-year-old right-handed man had a left temporal lobe, pathologically confirmed glioblastoma multiforme, treated with partial surgical resection, whole-brain radiation (6100 cGy), and i.v. carmustine (BCNU) therapy. He received phenytoin (PHT) for seizure prophylaxis, and returned to executive-level work without difficulty. An occasional difficulty remembering personal names was his only complaint. Seven months postoperatively, on the day of a BCNU treatment, he noted sudden inability to speak. He hailed a taxi, but was unable to give di-
Received September 1990; revision accepted February 1991. Address correspondence to Dr. C. R. Wells at 500 Lilly Road NE, No. 2304, Olympia, WA 98506, U.S.A.
84
APHASIA AND STATUS EPILEPTICUS
D
A
85
C
FIG. 1. A: (EEG) during aphasia, illustrating regularly repetitive 4-Hz sharp wave activity from the left hemisphere, constituting electrographic status epilepticus. B: Periodic lateralized epileptiform discharges seen transiently following i.v. diazepam (DZP), without associated language improvement. C: EEG after treatment with DZP. Epileptiforrn activity was replaced by left hemispheral slowing, and language function improved.
status epilepticus (such as shopping, hailing a cab, walking 30 blocks to his office, and undergoing an EEG), and was aware of his previous difficulty speaking. A magnetic resonance imaging scan revealed interval deterioration, with enlargement of the left temporal glioblastoma. DISCUSSION Previous reports based on clinical observations (Penfield and Rasmussen, 1949; Bingley, 1958; Se-
rafetinides and Falconer, 1969) and video EEG studies (Koerner and Laxer, 1988; Gabr et al., 1989) have detailed language dysfunction due to seizures originating from left temporal lobe foci. These studies and several other case reports illustrate the difficulty of correlating language testing with EEG findings and response to therapy, either because of impaired consciousness during the seizure (i.e., complex partial) (Knight and Cooper, 1986; Racy et al., 1980), or because of lack of response to therapy
Epilepsiu, Vol. 33, N o . I, 1992
C . R . WELLS ET AL.
(Hamilton and Matthews, 1979; Dinner et al., 1981; Primavera et al., 1988). The temporal coincidence of our patient’s aphasia and electrographic seizure patterns indicates that aphasia was the manifestation of an ictal event, which resolved when treated with an i.v. antiepileptic drug (AED). In previous reports, aphasic status epilepticus was considered to be a manifestation of complex partial status epilepticus (DePasquet et al., 1976; Hamilton and Matthews, 1979; Racy et al., 1980; Dinner et al., 1981; Knight and Cooper, 1986; Rosenbaum et al., 1986; Primavera et al., 1988). In contrast, our case represents simple partial status epilepticus rather than complex partial status epilepticus since there was no alteration in consciousness (Commission, 1981). Although formal neuropsychological evaluation of higher cortical functions was not carried out during the ictus, the patient was capable of initiating and carrying out complicated volitional behaviors (hailing a cab, walking 1.5 miles on the streets of downtown New York), and following nonverbal commands during the basic neurological examination and the EEG, and he could recall events during the ictus. Thus, the patient demonstrated preserved responsiveness and recall (meeting the accepted operational definition for preserved consciousness during a seizure), and the seizure could be classified as simple partial (Commission, 1981). The normal level of consciousness allowed detailed correlation of the findings on language exam with EEG before, during, and after treatment. During the initial electrographic seizure component, the patient was globally aphasic. When i.v. DZP converted the EEG pattern to PLEDs, the patient remained globally aphasic; thus, this should be considered continuing ictal activity (Brenner and Schaul, 1990). When the PLEDs were replaced by slowing, the patient returned to near baseline language function, thus indicating termination of the ictus. It may be hypothesized that the DZP produced neuronal inhibition by activating postsynaptic membrane chloride channels via the benzodiazepine receptor, and thus initially converted the continuous ongoing electrographic ictal pattern to an intermittent epileptiform pattern (PLEDs), and subsequently terminated the seizure. A preexisting PHT level of 14.7 pg/ml may have acted to prevent local spread of the discharge and a secondary generalized seizure. As suggested by Rosenbaum et al. (1986), isolated epileptic aphasia may represent an artifact of partial seizure control. Because acute aphasia more commonly results from a transient ischemic attack, stroke, or perhaps
Epilepsia, Vol. 33, No. 1 , 1992
even a cerebral hemorrhage, recognizing the possibility of an ictal event in a patient without a previous seizure history can be difficult; we are aware of only three such reported cases (Racy et al., 1980; Dinner et al., 1981; Rosenbaum et al., 1986). However, considering seizure in the differential diagnosis can lead to a diagnostic EEG and prompt AED therapy. Acknowledgment: We thank Dr. Fred Plum for a critical review of this manuscript, and Dr. Frank Petito for permission to report his patient.
REFERENCES Bingley T. Ictal mental symptoms of temporal lobe epilepsy. Acta Psych Neurol Scan Suppl 1958;120:95-9. Brenner RP, Schaul N . Periodic EEG patterns: classification, clinical correlation, and pathophysiology. J Clin Neurophysiol 1990;7:249-67. Commission on classification and terminology of the International League Against Epilepsy. Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsiu 1981;2:489-501. DePasquet EG, Gaudin ES, Bianchi A, et al. Prolonged and monosymptomatic dysphasia status epilepticus. Neurology 1976;26:244-7. Dinner DS, Lueders H , Lederman R, et al. Aphasic status epilepticus: a case report. Neurology 1981;31:888-90. Gabr M, Liiders H , Dinner D, et al. Speech manifestations in lateralization of temporal lobe seizures. Ann Neurol 1989;25: 82-7. Hamilton NG, Matthews T. Aphasia: the sole manifestation of focal status epilepticus. Neurology 1979;29:745-8. Knight RT, Cooper J . Status epilepticus manifesting as reversible Wernicke’s aphasia. Epilepsiu 1986;27:301-4. Koerner M, Laxer KD. Ictal speech, postictal language dysfunction, and seizure lateralization. Neurology 1988;38:634-6. Penfield W, Rasmussen T. Vocalization and arrest of speech. Arch Neurol Psychiatry 1949;61:21-7. Primavera A, Bo G, Venturi S. Aphasic status epilepticus. Eur Neurol 1988 ;28:255-7. Racy A, Osborn MA, Vern BA, et al. Epileptic aphasia: first onset of prolonged monosymptomatic status epilepticus in adults. Arch Neurol 1980;37:419-22. Rosenbaum DH, Siege1 M, Barr WB, et al. Epileptic aphasia. Neurology 1986;36:822-5. Serafetinides EA, Falconer MA. Speech disturbance in temporal lobe seizures: a study in 100 epileptic patients submitted to anterior temporal lobectomy. Brain 1963;86:333-46.
RESUME L’aphasie est liCe a un etat de ma1 kpileptique partiel simple et rare, en particulier en I’absence d’antecedents de crises. I1 n’y a pas de cas publies qui decrivent l’aphasie comme seule manifestation clinique d’un etat de ma1 6pileptique surveil16 par EEG, disparaissant rapidement avec le traitement. Les auteurs rapportent l’observation d’un homme de 45 ans prCsentant un glioblastome temporal gauche qui a present6 une aphasie globale aigue; pendant cet Cpisode I’EEG a mis en evidence des ondes lentes angulaires continues, rtpktees, hemisphkriques gauches. Aprts injection de diazCpam intraveineux, I’activitC EEG critique a disparu et le langage du patient a recupere son niveau antCrieur. (P. Genton, Muuseilk)
APHASIA A N D STATUS EPILEPTICUS
87
RESUMEN
ZUSAMMENFASSUNG
L a afasia, como manifestacion de un status epilepticus parcial simple, ocurre raramente, particularmente, en ausencia de una historia de ataques. No hay informes previos que describa la fase aguda como la unica manifestacion de status epilepticus monitorizado con EEG y rapida resolucion con el tratamiento. Los autores describen un hombre con 45 atios que tenia un glioblastoma del lobulo temporal izquierdo que presento, de forma aguda, una afasia global durante la cual un EEG mostr6 ondas agudas repetitivas y continuas que se originaban en el hemisferior izquierdo. DespuCs de la inyeccion intravenosa de diacepam la actividad del EEG ces6 y el lenguaje expresivo del enfermo volvi6 a 10s niveles preataque.
Aphasie als Status epilepticus einfacher Partialanfalle ist selten, besonders bei Abwesenheit von Anfallen in der Vorgeschichte. Bisher gibt es keinen Bericht iiber eine Aphasie als einziges klinisches Zeichen eines irn EEG aufgezeichneten Status epilepticus mit prompten Ansprechen auf Therapie. Wir beschreiben einen 45 Jahre alten Patienten mit einem linkstemporalem Glioblastom, welcher akut eine globale Aphasie entwickelte. Im EEG fanden sich kontinuierlich repetitive sharpwaves im Bereich der linken Hemisphare. Nach intervenoser GAbe von Diazepam sistierte die Krampfaktivitat im EEG und die Sprache des Patienten kehrt zu ihrer ursprunglichen Funktion zuriick.
(A. Portera-Sanchez, Madrid)
(C. G. Lipinski, HeidelbergiNeckurgemund)
Epilepsiu, Vol. 33, NO. 1, 1992
