Global Aphasia Without Hemiparesis Daniel Tranel, PhD; Jos\l=e'\Biller, MD; Hanna Damasio, MD; Harold P. Adams, Jr, MD; Steven H. Cornell, MD \s=b\ Three patients acutely developed global aphasia, but did not manifest the typical accompanying right hemiparesis. Computed tomography and magnetic resonance imaging studies demonstrated that the patients had two discrete left hemisphere lesions, one in the anterior language cortices or language-related subcortical areas, and one in the posterior language cortices. Cerebral angiography showed that two patients had complete occlusion of the left internal carotid artery, and the third had an intraluminal "clot" in the supraclinoid portion of the left internal carotid, findings suggestive of an embolic etiology. Our cases indicate that global aphasia without hemiparesis predicts two discrete lesions and a particularly good recovery of speech and lan- guage. (Arch Neurol 1987;44:304-308) is characterized by fllobal aphasia impairment of speech pro¬ severe duction, auditory comprehension, and repetition. Hemiplegia is considered to be an almost constant component of the clinical presentation.1 The pres¬ ence of global aphasia is indicative of a large lesion in the left perisylvian and basal ganglia regions, an assump¬ tion supported by most studies for which anatomical data are available. Furthermore, the severity of global aphasia and the size of lesion in these Accepted for publication Oct 20, 1986. From the Departments of Neurology, Divisions of Behavioral Neurology (Drs Tranel and Damasio) and Cerebrovascular Diseases (Drs Biller and Adams), and Radiology, Section of Neuroradiology (Dr Cornell), University of Iowa, Iowa City. Reprint requests to Department of Neurology, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242 (Dr Tranel). locations are strongly associated.25 Recently, however, it has been noted that global aphasia can be associated with large lesions confined to prerolandic language areas.6 8 Finally, glob¬ al aphasia may even result from two discrete lesions, one affecting the anterior and the other involving the posterior left language areas.910 Van Horn and Hawes10 described three patients who developed global aphasia without hemiparesis, and suggested that this presentation was indicative of "embolie encephalopathy." We recently studied three patients who acutely developed global aphasia without hemiparesis as a result of cerebral infarctions. These patients were examined in both acute and chronic stages. Our findings support the conclusions that global aphasia without hemiparesis predicts two dis¬ crete left hemisphere lesions; predicts an unusually good recovery of speech and language; and may be an indica¬ tor of an embolie etiology. PATIENTS AND METHODS We evaluated three patients who, as a result of cerebral infarctions, developed global aphasia without hemiparesis. Two patients had computed tomography (CT) and neuropsychologic evaluation both in the acute phase (within 14 days of onset), and in the chronic phase (between three and ten months after onset), and the third patient received these studies acutely and again five weeks after onset. Magnetic resonance imaging (MRI) was also obtained for all patients. Cerebral angiography was performed during the acute phase of the illness. Computed tomography was obtained with a fourth generation scanner (Picker 1200 Sinerview). Transverse slices of 8 mm thickness were obtained. Magnetic reso¬ nance imaging was obtained using a scan¬ ner (Picker 0.5 Tesla Superconducting Magnetic Resonance Scanner). Anatomical Downloaded From: by a Thomas Jefferson University User on 06/24/2018 localization was obtained by analysis of CT and MRI according to the standard proce¬ dures of the Division of Behavioral Neurology's Neuroimaging Laboratory, University of Iowa, Iowa City. Patient identification in the films was masked. Investigators blind to the patients' behav¬ ioral status selected the appropriate tem¬ plate system for plotting the abnormal images and charted the position of those images on a standard template protocol.11 A transparency containing the boundaries of neural units of interest, in both gray- and white-matter structures, was then superimposed on the chart. Damage was assigned to the units that contained the abnormal images. A complete neuropsychologic evaluation included assessment of intellectual func¬ tioning (Wechsler Adult Intelligence Scale—Revised), verbal and visual memo¬ ry, perception, and orientation. Testing was conducted over several short sessions, commensurate with the patients' ability to cooperate and attend. Some tests were omitted or truncated, as necessitated by the patients' condition. Speech and lan¬ guage were assessed with the Multilingual Aphasia Examination12 and the Boston Diagnostic Aphasia Examination.15 Em¬ phasis was placed on the most salient parameters of speech and language, including fluency, articulation, naming, repetition, comprehension, and reading and writing. REPORT OF CASES Case 1.—After an evening of heavy alco¬ hol consumption and subsequent vomiting, a 37-year-old right-handed man suddenly became unable to speak, except for saying "yes" and "goddamn." He complained of "numbness" of the right side of the face. Neurologic examination showed a discrete right facial weakness and a minimal right pronator drift (both of which resolved completely by the third day after onset). Strength was otherwise normal. Tendon reflexes were slightly brisker in the right upper extremity. Plantar responses were normal. Computed tomography showed asymmetry of the lateral ventricles, but no focal area of abnormal attenuation was seen. Angiography demonstrated an intraluminal filling defect in the supraclinoid portion of the left internal carotid artery. During the next three days, the patient was noted to utter only a few poorly artic¬ ulated words. Comprehension of conversa¬ tional speech was restricted to very simple commands. He could not name, repeat, read, or write. Six days after onset, CT with contrast enhancement showed two well-defined areas of infarction in the left hemisphere, one anterior (frontal lobe) and one posterior (parietal lobe). The ante¬ rior lesion involved the most superior part of the frontal operculum (area 44), and extended into the premotor region immedi¬ ately above (area 6). The posterior lesion involved part of the supramarginal gyrus (area 40), as well as the inferior part of the superior parietal lobule (areas 7 and 5). It extended inferiorly to involve the most posterior portion of the superior temporal gyrus (posterior area 22). Comprehensive neuropsychologic evalu¬ ation was completed within two weeks of the ictus. The patient continued to have severely nonfluent speech. He had virtual¬ ly no spontaneous speech production. In response to questions, he attempted to communicate. However, utterances were restricted to a few grunts and to one or two unintelligible phonemes. He was unable to name in either the visual, auditory, or tactile modalities. Repetition was not pos¬ sible. He could follow simple commands and showed some preservation of aural and reading comprehension of simple words and short phrases; however, aural and reading comprehension of more complex material was severely defective. He was severely agraphic, and had gestural aprax¬ ia. Nonverbal abilities, such as visual per¬ ception and two- and three-dimensional constructional praxis, were normal. Magnetic resonance imaging obtained five months after onset showed two dis¬ crete lesions in the left hemisphere, in the same locations as described before (Fig 1). At this time, follow-up neuropsychologic evaluation revealed remarkable recovery of speech and language (Table). His speech remained slightly effortful, but he pro¬ duced sentences of normal length, and there were no paraphasias. As shown in Table, nearly all formal linguistic func¬ tions had improved to the normal range. Case 2.—A 52-year-old right-handed man suddenly developed right-sided focal seizures and an inability to speak. On admission to the hospital, he was alert and oriented. He did not produce any intelligi¬ ble speech, but answered questions by nod¬ ding. He was able to follow a few very simple commands. He had mild right cen¬ tral facial paresis, and slight pronator drift on the right. There were no other signs of motor weakness. Tendon reflexes were slightly brisker in the right upper extremity and right knee. Plantar responses were normal. He had frequent episodes of right facial twitching. By the seventh day after onset, the facial weak¬ ness and pronator drift had disappeared. Computed tomographic scans obtained Fig 1.—Magnetic resonance imaging scan of patient 1 obtained five months after onset with T2-weighted image. Note two distinct areas of increased signal in left hemisphere. Speech and Language Characteristics of Patients 1 and 2 Patient 2 Patient 1 Characteristic Acute Severely Speech nonfluent; reduced Chronic Fluent, nonparaphasic to "yes" and a Acute Chronic Severely Essentially two fluent with occasional profanities paraphasias nonfluent; profanity Language Naming* 0/30 2/30 8/14 Sentence repetition* Aural 1/18 8/18 comprehension* Token test* 33/44 Readingt 4/10 Writing* Severely 9/10 Severely defective defective Gestural praxis Severely defective Controlled oral word association * * Multilingual Aphasia Examination. tBoston Diagnostic Aphasia Examination. Downloaded From: by a Thomas Jefferson University User on 06/24/2018 Normal Moderately Normal defective Severely defective Moderately defective long history of poorly controlled arterial hypertension and severe cerebral vascular disease. According to relatives, he began to evidence changes in behavior and mental status three years previously, with a gradual diminution of activities and mental acuity, and eventual retirement from work. In retrospect, these changes probably reflect the cumulative effect of multiple ischemie events; however, at no time did he show evidence of focal motor or sensory deficits. He then developed an acute onset of difficulty speaking, which prompted admission to our service. On admission, he showed severe global aphasia. The remainder of the neurologic examination was normal. Computed tomography performed three days after onset showed multiple infarcts located in the left posterior temporoparietal region, left basal ganglia, left prefrontal region, right corona radiata, and right posterior infracalcarine area. These lesions were also seen on MRI obtained the same day. Cerebral angiography performed five days after the onset of symptoms showed complete occlusion of the left internal carotid artery. The left middle cerebral artery filled through the ophthalmic col¬ laterals. Branch occlusions of the angular and posterior parietal branches of the left middle cerebral artery were also noted. Neuropsychologic testing was conducted ten days after admission. His speech was severely nonfluent, and he showed pro¬ found deficits in most aspects of linguistic functioning. He did produce some short utterances of two or three words. These contained paraphasse errors, but no signif¬ icant articulatory disturbance. Naming and repetition were not possible. He showed marked impairment of aural and reading comprehension, even for simple material such as single words and short phrases. He was agraphic, with frequent paragraphic errors especially when copy¬ ing written text. Five weeks later, he showed slightly improved speech fluency. He produced longer utterances with frequent paraphasic errors, but continued to have little suc¬ cess in naming, repetition of short sen¬ tences, or comprehension of verbal materi¬ al. Also, he showed moderate compromises in areas of nonverbal, visuospatial cogni¬ tive functioning. These deficits, less severe than those in the verbal sphere, were inter¬ preted as due to the right hemisphere man had a Fig 2.—Magnetic resonance imaging scan of patient 2 obtained two days after onset with T2-weighted image. Note two distinct areas of increased signal in left hemisphere. the day of onset showed a lesion in the parietal region, and an ill-defined area of possible infarct in the left frontal lobe. Electroencephalography demonstrated rare, left frontal high amplitude and rhythmic delta paroxysms. Cerebral angiography showed complete occlusion at the origin of the left internal carotid and a small residual stump. The left middle cere¬ bral artery filled through retrograde flow from the ophthalmic artery. It did not receive collateral flow from the right inter¬ nal carotid or from the posterior circula¬ tion. Magnetic resonance imaging performed at this time showed two discrete lesions in the left hemisphere (Fig 2). A frontal-lobe lesion involved the most anterior and supe¬ rior portion of the frontal operculum (area 45 and white matter undercutting area 44), and extended into prefrontal areas 46 and 9. The other lesion was in the parietal lobe, and involved the inferior and posterior portions of the inferior parietal lobule (areas 40 and 39). Laboratory evaluation of speech and lan¬ guage was started the day after the event, and continued for several days. It revealed severe impairments in nearly all aspects of speech and language. Spontaneous speech was limited to only two words, both pro- fanities. Even these were uttered with extreme effort and frequent initial stutter¬ ing. The patient could not name or repeat. He had severe alexia and agraphia, and gestural apraxia of the right arm. Compre¬ hension was limited to very simple, onestep commands (eg, "make a fist"). He also showed constructional apraxia and im¬ paired right-left discrimination. Visual perception was relatively intact. He improved slightly during the first week in the hospital, the most notable recovery taking place in the domain of comprehen¬ sion of conversational speech. Follow-up neuropsychologic evaluation ten months after the stroke showed excel¬ lent recovery of speech and language (Ta¬ ble). He had occasional pauses and wordfinding difficulties in spontaneous speech, but formal linguistic assessment demon¬ strated normal or near-normal levels of performance in most areas tested. Com¬ puted tomography performed at this time showed a well-defined frontal lobe lesion in the same area as described before, but extending higher into prefrontal regions in the dorsolateral sector (areas 9 and 8). The parietal lobe lesion was visible as an area of atrophy in the inferior parietal lobule (areas 40 and 39). Case 3.—A 51-year-old right-handed Downloaded From: by a Thomas Jefferson University User on 06/24/2018 lesions. Computed tomography performed at this time showed three distinct areas of infarcì in the left hemisphere (Fig 3). One was in the dorsolateral prefrontal cortex (areas 46, 9, 8). Another was in the basal ganglia, and involved the head of the cau¬ date nucleus, part of the anterior limb of the internal capsule, and the paraventricular white matter. The third was in the posterior portion of the superior temporal gyrus (posterior area 22 and 37) and the inferior parietal lobule (areas 40 and 39). A neuropsychologic follow-up examina¬ tion was performed four months after the initial event. His speech at this time was essentially fluent, although there were fre¬ quent pauses and hesitations. Paraphasias common, especially in spontaneous speech and naming tasks. He remained unable to name (nearly all paraphasic responses) or repeat, but aural and reading comprehension were significantly im¬ proved. There were also very significant improvements in gestural and construc¬ tional praxis. Although nearly all of his scores continued to fall in the "impaired" range, he generally showed a very good profile of recovery of verbal communica¬ tion abilities, relative to the severe global aphasia he manifested acutely. were COMMENT Our patients acutely developed a syndrome of global aphasia without the expected hemiparesis, caused by cerebral infarction. The lesions asso¬ ciated with global aphasia can vary in both extent and location. There is, however, a strong association between the typical clinical picture of global aphasia and involvement of the entire left perisylvian region. These lesions result from a large infarction in the territory of the left middle cerebral artery. Damage involves all or most of the areas known to be related to speech and language: the frontal operculum (areas 44 and 45), the posterior half of the superior temporal gyrus (areas 41, 42, and posterior area 22), the posterior region of the middle and inferior temporal gyri (area 37), the supramarginal and angular gyri (ar¬ eas 40 and 39), and deep structures, including the basal ganglia and insu¬ la. Commonly, damage involves part of the motor cortices, or the subcortical white matter where corticobulbar fibers cross, or both. Thus, global aphasia and right-sided motor deficits typically occur together, although the latter may be relatively transient in a few cases.14 Our cases, however, had two dis¬ crete lesions. One of these was in anterior language cortices or in the subcortical gray-matter areas known to be related to language dysfunc¬ tion,1516 and one was in the posterior language cortices. The primary motor cortices were intact. A similar local¬ ization of lesions in a case of global aphasia was noted previously in a description from this laboratory.9 The pathological events that lead to such double and discrete areas of damage are of special interest. Van Horn and Hawes10 described three patients who developed global aphasia without hemiparesis, all of whom had two separate lesions in the left hemi¬ sphere. The authors concluded that this clinical picture was indicative of an "embolie encephalopathy." Our cases are also consistent with the notion of an embolie pathophysiology. Fig 3.—Magnetic resonance imaging scan of patient 3 obtained five weeks after onset without contrast. Note three distinct areas of low density in left hemisphere. Two patients had complete occlusion of the left internal carotid artery, and the third had an intraluminal "clot" in the supraclinoid portion of the left internal carotid artery. A final point of importance is the issue of recovery. Poor recovery from global aphasia is a ubiquitous finding in extant research.51721 A comparison between recoveries from different types of aphasia shows that patients with global aphasia invariably fare the poorest.18·20·21 Sarno and Levita19 noted that the greatest changes in the evolution of global aphasia may occur between the sixth and 12th months after onset. Nevertheless, these patients remained severely defective in communication skills 12 months after onset, and were still classified as global aphasies. Intensive speech Downloaded From: by a Thomas Jefferson University User on 06/24/2018 therapy does not appear to signifi¬ cantly improve this poor recovery.18'9 In sharp contrast, the three cases described here showed very good recovery of speech and language. This was particularly evident in patients 1 and 2, in whom speech became fluent, repetition became normal, and aural comprehension only showed deficits with complex material. Agraphia and gestural apraxia also resolved. Pa¬ tient 3 also improved substantially, although not as completely. By ten months after onset, none of the three patients continued to meet diagnostic criteria for global aphasia. Patients 1 and 2 showed only minimal signs of residual aphasia, not sufficient to qualify for formal classification; patient 3 could be classified as having a residual Broca's aphasia. It is interesting to speculate as to the possible reasons for this unchar¬ acteristic recovery. A major differ¬ ence between our patients and the traditional patient with global apha¬ sia is the extent of involvement of language-related areas. Typically, as elaborated above, there is extensive destruction of most of the perisylvian and basal ganglia regions, including the frontal operculum, posterior supe¬ rior temporal gyrus, inferior parietal lobule, insula, and motor cortices. However, our patients had only cir¬ cumscribed involvement of some of these areas. Thus, although the initial insults rendered these areas suffi¬ ciently dysfunctional to produce a pic¬ ture of global aphasia, the amount of undamaged language-related territo¬ ry probably allowed for this otherwise unusually complete recovery of speech and language. In short, the presentation of global aphasia without hemiparesis predicts two discrete lesions in the anterior and posterior left hemisphere lan¬ guage areas, possibly of an embolie etiology. This syndrome also seems to indicate the possibility of an unusual¬ ly good recovery of verbal communica¬ tive abilities. This study was supported in part by National Institute of Neurological and Communicative Diseases and Stroke (Bethesda, Md) grant POI NS 19632. References 1. Geschwind N: Current concepts: Aphasia. N Engl J Med 1971;284:654-656. 2. Benson DF, Geschwind N: The aphasias and related disturbances, in Baker AB, Baker LH (eds): Clinical Neurology. New York, Harper & Row Publishers Inc, vol 1, 1971. 3. Naeser MA, Hayward RW: Lesion localization in aphasia with cranial computed tomography and the Boston Diagnostic Aphasia Examination. Neurology 1978;28:545-551. 4. Naeser MA, Hayward RW, Laughlin SA, et al: Quantitative CT scan studies in aphasia: I. Infarct size and CT numbers. Brain Lang 1981; 12:140-164. 5. Kertesz A, Harlock W, Coates R: Computer tomographic localization, lesion size, and prognosis in aphasia and nonverbal impairment. Brain Lang 1979;8:34-50. 6. 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