BRAIN AND LANGUAGE 7, 62-73 (1979) Left Thalamic Hemorrhage with Dysphasia: A Report of Five Cases ARDEN F. REYNOLDS,* PAUL T. TURNER,* A. BASIL HARRIs,t GEORGE A. OJEMANN,~ AND LARRY E. DAVIS+ *University of New Mexico, tllniversity of Washington, and $CJniversity of New Mexico A specific type of “thalamic speech” is being recognized with increasing frequency. Paucity of spontaneous speech, fading vocal volume, anomia, perseveration, and neologisms, with intact comprehension and word repetition, characterize the speech disorder associated with thalamic lesions. Nine cases of left thalamic hemorrhage and speech disturbance have been reported previously. This report details the speech, neurological, and neuroradiological findings in five additional cases of thalamic hemorrhage with dysphasia. INTRODUCTION The role of the dominant thalamus in speech is becoming increasingly recognized. Acute effects of thalamic hemorrhage were well summarized by Fisher (1959). These effects include aphasia, ocular findings, and a predominance of sensory over motor deficits of the right side. Case reports of speech effects from thalamic hemorrhage are unusual because frequently the lesion causes coma, negating speech examination. Nine cases of thalamic hemorrhage with aphasia have been reported in the literature (Bugiani, Conforto, & Sacco, 1969; Ciemins, 1970; Mohr, Walters, & Duncan, 1975; Samarel, Wright Sergay et al., 1976). This report details an additional five cases. CASE REPORTS Case 1 A 61-year-old male with preexisting hypertension and alcoholism presented with a fluent aphasia, anomia, frequent perseverations, and neologisms. He could repeat verbal input. No record of spontaneous speech or vocal volume was made. Neurological examination revealed normal ocular motility and visual fields, diminution of all sensory modalities on the right side, a mild right hemiparesis, and right Babinski. A CT Requests for reprints should be sent to: Arden F. Reynolds, Divison University of Arizona Health Sciences Center, Tucson, AZ 85724. 62 0093-934X/791010062-12$02.00/0 Copyright All rights @ 1979 by Academic Press. Inc. of reproduction in any form reserved. of Neurosurgery, THALAMIC HEMORRHAGE AND DYSPHASIA 63 FIG. I. Case 1. CT scan. There is a hematoma in the posterolateral left thalamus with extension into the internal capsule. (computerized tomogram) scan (Fig. 1) revealed a left thalamic hemorrhage in the posterior thalamus with a small extension into the internal capsule. The patient developed delirium tremens which lasted 2 weeks. After this period his speech and right sensory and motor deficits rapidly improved. He was discharged with only minor sensory deficits on the right. He underwent formal neuropsychological testing on his 20th hospital day and again 1 month later. Over this interval, his Wechsler adult intelligence scale improved two standard deviations on both the verbal and performance scales. Rapid finger oscillation and grip strength were unchanged over this interval and were impaired on the right. The Porch Index of Communicative Ability (Porch, 1971) rated the patient at the 59th percentile 3 weeks after his hemorrhage and at the 71st percentile 10 days later. Testing at 3 weeks revealed normal motor substrate for speech. One year later the patient denied any language difficulties. He remained hypertensive. He could read and write fluently, without error. Graphesthesia and stereognosis were decreased on the right. 64 REYNOLDS ET AL. Case 2 A 40-year-old male Navajo acutely developed dysphasia, supranuclear paralysis of upward gaze, and sensory and motor deficits of the right side. A CT scan (Fig. 2) revealed a left thalamic hemorrhage with extension of the clot into the posterior third ventricle. He became obtunded, and an external ventriculostomy was placed. His level of consciousness, paralysis of upward gaze, and motor deficit promptly improved. He required placement of a ventriculo-peritoneal shunt to remain alert. An arteriogram revealed a vascular tumor confined to the thalamus as the etiology of this hemorrhage. The patient, although fluent in both Navajo and English before this event, could only speak Navajo after the hemorrhage. The interpreter characterized the Navajo he spoke as containing perseverations and nonsense syllables. He exhibited spontaneous speech. He received a course of radiation therapy for his tumor and his clinical state remained stable for the 4 months of follow-up, i.e., severe dysphasia with mild right sensory deficits. FIG. 2. Case 2. CT scan. There is extensive hemorrhage in the left thalamus. THALAMIC HEMORRHAGE AND DYSPHASIA 65 Case 3 A 23-year-old male acutely developed left-sided headache and sensory loss on the right side. He had a paucity of spontaneous speech with fading vocal volume. In addition, anomia, perseveration, and neologisms were present. He also had supranuclear paralysis of upward gaze, a right sixth nerve paresis, diminution of all senses on the entire right side, and a mild right hemiparesis. An arteriogram revealed a vascular blush in the left pulvinar. A ventriculogram showed elevation of the floor of the left trigone (Fig. 3) and filling defects in the posterior third ventricle. Ventriculostomy drainage rapidly improved the paralysis of upward gaze and right hemiparesis. The diagnosis of thalamic hemorrhage secondary to cryptic arteriovenous malformation was made. Language comprehension appeared intact. On a standard objectnaming test, there was a 31% error rate with marked perseveration. These perseverations often were words or phrases completely unrelated to the test material. Number reading was mildly impaired. Simple arithmetic calculations, especially those involving the tens place, were severely impaired (Ojemann, Blick, & Ward, 1969). Repeat language testing 18 FIG. 3. Case 3. Lateral positive contrast ventriculugram. The floor of the left trigone is elevated and irregular. The floor of the right trigone is smooth. The posterior third ventricle fails to fill, probably representing clot. 66 REYNOLDS ET AL. days later revealed clearing of anomia and marked improvement in simple calculations. One month later the only clinical residual deficit was a mild central seventh paresis. Repeat ventriculography was normal. Eight months later repeat angiography was normal. A Reitan battery at 1 year post-ictus was administered. The results were as follows: The patient demonstrated a mild degree of aphasia with a slight increase in errors while reading aloud. The patient also demonstrated a mild impairment in flexibility and alertness in dealing with language symbols. In addition, his right hand performance was slightly impaired. Case 4 A 37-year-old male with preexisting hypertension and diabetes mellitus experienced the acute onset of confusion, aphasia, and right hemiparesis. The patient had no spontaneous speech and responded to verbal questions with garbled words and occasional meaningful words. He followed commands. He had conjugate paralysis of upward gaze, right fifth, seventh, and twelfth nerve paresis, profound right sensory loss, and mild right hemiparesis. Angiography revealed left-to-right shift of the internal cerebral vein. Ventriculography revealed elevation of the floor of the left trigone, with filling defects in both the left trigone and posterior third ventricle (Fig. 4). Ventriculostomy drainage did not lead to improvement. The patient did not improve over 8 weeks and was transferred to a chronic care facility. Case 5 A 51-year-old hypertensive female had the acute onset of headache and rapidly became comatose. A lumbar puncture revealed elevated pressure with bloody fluid. A ventricular catheter was placed, and she rapidly became responsive. She had paralysis of conjugate upward gaze, right hemisensory diminution to all modalities, and a right hemiparesis. The only notation of her speech abnormality was that of dysphasia. An arteriogram revealed left-to-right shift of the internal cerebra1 vein in a square fashion (Fig. 5). After the ventriculostomy catheter was removed she became obtunded and required a ventriculo-atria1 shunt. One year later she was contacted and refused to return for neuropsychological testing or examination. However, she denied speech problems or difficulty with sensation or motor power. DISCUSSION The clinical pattern of left thalamic hemorrhage is easily recognized by three cardinal features. First, is the predominance of sensory over motor deficits on the right body side. Second, is the impairment of ocular THALAMIC HEMORRHAGE AND DYSPHASIA 67 FIG. 4. Case 4. Anteroposterior positive contrast ventriculogram. There is irregular elevation and lateral displacement of the floor of the left trigone. In addition, the superior third ventricle is shifted from left to right. mobility, with conjugate paralysis of vertical gaze being common. Third, is the presence of a speech disorder (Fisher, 1959). The speech disorder has been characterized by the following: paucity of spontaneous speech with fading voice volume, anomia, perseveration and neologisms, with intact comprehension and word repetition (Mohr et al., 1975; Samarel et al., 1976). Cases 3 and 4 demonstrated all of these features. Case 5 was not examined by any of the authors, and no notation was made other than dysphasia. In Case 1, no notation of spontaneous speech or vocal volume was made, but anomia, perseveration, and neologisms were present, with intact comprehension and word repetition. 68 REYNOLDS ET AL. FIG. 5. Case 5. Towne arteriogram, venous phase. The internal cerebral vein is shifted from left to right in a square fashion. Case 2 exhibited spontaneous speech with normal vocal volume. Of interest is the permanent loss of ability to speak English with retention of aphasic Navajo. The speech abnormalities improved in three of the five patients over a time period of weeks to months. However, minimal residual deficits were detected at 1 year in the only patient in which formal speech testing was done. In the cases reported in the literature, improvement occurred in two of three cases. In the other cases, death, coma, or lack of observation precluded evaluation of the natural history of the speech disorder (Mohr et al., 1975). The clinical management of these patients optimally should include making the diagnosis on clinical grounds and confirming it with a CT scan. If the patient has an altered level of consciousness, moderate hemiparesis, or paralysis of conjugate upward gaze, a trial of ventricular drainage THALAMIC HEMORRHAGE AND DYSPHASIA 69 seems indicated, as three of four patients so treated improved. Two patients required permanent ventricular shunts. Additional modes of therapy would include corticosteroids, control of hypertension, and correction of metabolic abnormalities. Preexisting conditions were present in all patients thus far reported. The most common preexisting condition was hypertension, present in 9 of 14. Diabetes mellitus was present in 2, an arteriovenous malformation in 1, a glioma of the thalamus in I, and leukemia in 1 (Tables 1 and 2). Evidence for the role of the thalamus in speech is twofold. First, autopsy data from six patients with thalamic hemorrhage and aphasia revealed that aphasia can occur without cortical lesions (Table 2). In addition, CT scans have also demonstrated that thalamic lesions without cortical lesions have occurred in patients with speech disorders in an additional three patients-Cases 1 and 2, and Samarel’s case (1976). The second major area of evidence for the role of the thalamus in speech is those patients undergoing thalamotomy, during which no cortical lesions are made. Acute dysphasia, as judged by clinical examination, occurs in 34-42% of patients after left thalamotomy (Bell, 1968; Selby, 1967). Pathologically, the lesions in those patients with dysphasia were limited to the left ventrolateral nucleus or globus pallidus (Herman, Turner, Gillingham, et al., 1966; Samra, Riklan, Levita et al., 1969). Lesions confined to the dorsomedial left thalamus exhibited a dysphasia rate of 1 in 90. With formal language testing, anemic errors were found in 62% of patients 48 hr following left ventrolateral thalamic lesions (Ojemann, 1975a). This rate was significantly greater than the rate prior to thalamotomy or following right thalamotomy. These errors consisted of misnamings and perseverations. Lesions in the left internal capsule did not affect speech (Ojemann, 1975b). Electrical stimulation of the thalamus during tests of object-naming has evoked anomia in patients with electrodes in the anterior superior pulvinar and the posterior-medial-central portion of the ventrolateral nucleus of the left thalamus. Stimulation in the ventrolateral nucleus just anterior to this area has evoked perseveration, and slightly more anteriorly, results in repetition of the same wrong word (Ojemann, 1975b). During stimulation, comprehension remained intact, and speech remained fluent. These types of errors hsve not been evoked with right thalamic stimulation. Among the mechanisms proposed to explain the role of the thalamus in speech are the following: First, speech disturbance as a result of a general deterioration in neural function has been proposed (Brown, 1974; Geschwind, 1%7; Nielsen, 1946; Penfield & Roberts, 1959). Evidence against this view comes from Cases 2, 3, and 5, in which the generalized deficits cleared with ventriculostomy drainage while the speech deficits remained. Additionally, speech deficits occur following left, but not right, thalamic SPW Perseveration Paucity of speech Dysphasia DM HT HT 4 5 1 32,500 39,000 Angiogram Angiogram Ventriculogram Angiogram Ventriculogram CT 120,ooo 42,500 CT Diagnosis - CSF (RBC/mm3) Ventricular shunt Marked improvement Ventriculostomy Minimal improvement Ventriculostomy Marked improvement Ventricular shunt Moderate improvement Marked improvement Treatment sequelae (1Abbreviations: AVM = arteriovenous malformation; DM = diabetes mellitus; HT = hypertension; Spug = supranuclear paralysis of upward gaze; R-HH = right homonymous hemianopsia; CT = computerized tomogram: CSF = cerebrospinal fluid; RBC = red blood cells. Spug R-HH SPWZ Anomia Perseveration AVM 3 Normal Perseveration Paraphasia SPWS Glioma 2 Ocular findings Speech deficit Perseveration Paraphasia HT Predisposing factors 1 Case number TABLE SUMMARY OF CURRENT CASES~ F 3 R i2 2 p Absent spontaneous speech Perseveration Paraphasia HT Samarel et al. (1976) Moderate improvement R-HH CT-hemorrhage in posterior half of left thalamus L-thalamic hemorrhage anterior, ventrolateral, centromedian, anterior limb, internal capsule (’ Abbreviations: DM = diabetes meltitus; HT = hypertension: R-HH = right homonymous hemianopsia; CT = computerized tomogram. Death, 10 months - -.I 2 v) 5 2 Dysarthria HT Mohr et al. (1975) Hemorrhage L-thalamus and internal capsule Death, 1 day Slurred Leukemia is u L-thalamic hemorrhage medial, dorsal, and pulvianr Death, 72 days Mohr et al. (1975) R-HH Paraphasia Partial aphasia HT Mohr et al. (1975) Refused g E F R E Death, 32 days L-IJJ Paraphasia Perseveration HT DM Mohr et al. (1975) - Marked improvement i2 E Normal HT Mohr et al. (1975) 2 F L-thalamic hemorrhage to posterior commissure L-thalamic hemorrhage Paraphasia Perseveration HT Ciemins (1970) Death, 22 days Death, 13 days Autopsy or CT findings L-thalamic hemorrhage ventroposteriolateral and lateral posterior Perseveration - Ciemins (1970) Miosis Unreactive pupils - Sequelae Death, 3 years Paraphasia HT Bugiani et al. (1969) Ocular findings Expressive aphasia Speech deficit Predisposing factors Reference TABLE 2 SUMMARY OF REPORTEDCASES" 72 REYNOLDS ET AL. lesions (Ojemann, 1975a), and then only in a discrete region of left thalamus (Ojemann, 1975b). Another proposed mechanism is that of “conduction” aphasia, which follows lesions in the fiber tracts running through the insBIg from the posterior temporal association area to Broca’s area (Benson, Sheremata, Bouchard, et al., 1973). Without cortical lesions, these patients are unable to repeat auditory input. Our patients, as well as Mohr’s and Samarel’s patients, could also repeat auditory input (Bohr, 1975; Samarel et al., 1976). More recently, it has been proposed that the left thalamus is involved in attention mechanisms that gate storage and retrieval of both long-term and short-term verbal memory (Ojemann et al., 1969; Ojemann, 1975a, 1975b, 1977). These mechanisms can be altered by electrical stimulation of the left ventrolateral thalamus. Stimulation during input enhances later recall and increases the accuracy of naming items presented during stimulation in a preexisting aphasia (Ojemann, 1975a, 1975b). Difficulties in focusing attention and the resultant problems with recent memory would explain the wide fluctuation in performance and the apparent perseverations of unrelated verbal material, the “hallmarks” of thalamic speech (Luria, 1977). Left thalamic mechanisms also seem to be involved in coordinating the respiratory substrate of motor speech (Ojemann, 1977). This factor may explain the mutism of Case 4 (Bell, 1968). REFERENCES Bell, D. S. 1968. Speech functions of the thalamus inferred from the effects of thalamotomy. Bruin, 91, 619438. Benson. D. F., Sheremata, W. A., Bouchard, R., et al. 1973. Conduction aphasia.Archives of Neurology, 28, 339-346. Brown, J. W. 1974. Language, cognition and the thalamus. ConJiniu Neurologica, 36,33-60. Bugiani, 0.. Conforto, C., & Sacco, G. 1969. Aphasia in thalamic hemorrhage. Lancer, 1052. Ciemins. V. 1970. Localized thalamic hemorrhage, a cause of aphasia. Neurology, 20, 776-782. Fisher. C. M. 1959. The pathologic and clinical aspects of thalamic hemorrhage. Transac- tions of rhe American Neurological Association, 84, 56-59. Geschwind, N. 1967. Discussion on cerebral connectionism and brain function. In F. L. Darley & C. H. Milliken (Eds.), Bruin mechanisms underlying speech and language. New York: Grune & Stratton. Pp. 71-72. Herman, K., Turner, J., Gillingham, F., et al. 1966. The effect of destructive lesions and stimulation of the basal ganglia on speech mechanism. Conjnia Neurologica, 27, 197-207. Luria, A. R. 1977. On quasi-aphasic speech disturbance in lesions of the deep structures of the brain. Brain and Language. 4, 432-459. Mohr, J. P., Walters, W. C., & Duncan, G. W. 1975. Thalamic hemorrhage and aphasia. Bruin and Language, 2, 3-17. Nielsen, J. M. 1946. Agnosia, apraxia, aphasia: Their value in cerebral localization. New York: Harper & Row (Hoeber). Ojemann, G. A., Blick, K., & Ward, A. A., Jr. 1969. Improvement and disturbance of short THALAMIC HEMORRHAGE AND DYSPHASIA 73 term verbal memory during human ventrolateral thalamic stimulation. Transactions of the American Neurological Association, 94, 72-75. Ojemann, G. A. 1975a. Subcortical language mechanisms. In H. Whitaker & H. A. Whitaker (Eds.), Studies in neurolinguistics. New York: Academic Press, Vol. 2, pp. 103-138. Ojemann, G. A. 1975b. Language and the thalamus: Object naming and recall during and after thalamic stimulation. Brain and Language, 2, 101-120. Ojemann, G. A. 1977. Asymmetrical function of the thalamus in man. Annals of the New York Academy of Science, 299, 380-396, 1977. Penfield, W.. & Roberts, L. 1959. Speech and brain mechanisms. Princeton, N.J.: Princeton University Press. Porch, B. E. 1971. Multidimensional scoring in aphasia testing. Journal of Speech und Hearing Research, 14, 776-792. Samarel, A., Wright, T. L., Sergay, S., et al. 1976. Thalamic hemorrhage with speech disorder. Transactions of the American Neurological Association, 101, 283-285. Samra, K., Riklan, M., Levita, E., et al. 1969. Language and speech correlates of anatomically verified lesions on thalamic surgery for parkinsonism. Journal of Speech and Hearing Research, 12, 5 10-540. Selby, G. 1967. Stereotaxic surgery for the relief of Parkinson’s disease. II. an analysis of the results of a series of 303 patients (413 operations). Journalof Neurological Science, 5, 343-375.