Cerebral Atheromatous Embolism Following Carotid Sinus Pressure M. Flint Beal, MD; Tae S. Park, MD; C. Miller Fisher, MD \s=b\ Complications of carotid sinus pressure, which are rare, include cardiac arrhythmias and cerebrovascular accidents. A 79-year-old man experienced a flaccid right hemiplegia one minute after left carotid sinus pressure. At necropsy, an atheromatous embolus occluded the left middle cerebral artery, resulting in hemorrhagic infarction in its vascular territory. Atheromatous embolism following carotid sinus pressure has not been previously documented. Most sudden stroke deficits following carotid sinus pressure are probably caused by this mechanism. (Arch Neurol 1981;38:310-312) '"The complications of carotid sinus pressure include untoward cardiac arrhythmias and sudden stroke defi¬ cits. We report what we believe to be the first pathologically proved exam¬ ple of cerebral atheromatous embo¬ lism resulting from carotid sinus pres¬ sure. REPORT OF A CASE A 79-year-old man was admitted for evaluation of dysuria and urinary frequen¬ cy. Past history included diet-controlled adult-onset diabetes, a myocardial infarct four years previously for which he received isosorbide dinitrate (Isordil) and digoxin, and right leg claudication. The blood pres¬ sure was 150/80 mm Hg, and the pulse rate was 80 beats per minute and regular. The carotid pulsations were full bilaterally, and no carotid bruits were heard. The prostate was enlarged and firm. A left inguinal Accepted for publication July 8, 1980. From the Departments of Neurology (Drs Beal and Fisher) and Neuropathology (Drs Beal, Park, and Fisher), Massachusetts General Hospital and Harvard Medical School, Boston. Reprint requests to Massachusetts General Hospital, Neurology Service, Boston, MA 02114 (Dr Fisher). hernia was present. The patient underwent transurethral resection of the prostate and a left inguinal hernia repair. Postoperatively, he experi¬ enced persistent sinus tachycardia, with a heart rate of 120 beats per minute, and pulmonary congestion. He was transferred to the coronary care unit, where an ECG showed loss of voltage in the anterior leads thought to represent a new myocardial infarct. The creatine phosphokinase level rose to 330 mU/dL. He had repeated bouts of supraventricular tachycardia, which were terminated by carotid sinus pres¬ sure. One week after the operation, the patient experienced paroxysmal atrial tachycardia, with a heart rate of 160 beats per minute. Massage of the left carotid sinus with firm pressure for approximately 4 s resulted in conversion to normal sinus rhythm. One minute later, he was observed to have a right hemiparesis and his speech was slurred. Neurologic examination four hours after the ictus showed a drowsy man with Cheyne-Stokes respirations. He followed simple commands. Speech was dysarthric. A visually presented key was identified as a quarter and a coin as a ring. Tactile identification of a comb was correct. The abnormal findings included a right homon¬ ymous hemianopsia, left gaze preference, a right central facial paresis, and a dense flaccid right hemiplegia. Painful stimuli produced right arm adduction and triple flexion of the right leg. Subsequently, the dense right hemiple¬ gia persisted but the patient could name and comprehend well. Further questioning disclosed that he had been a left-handed baseball pitcher in his youth, raising the possibility of right hemisphere dominance for speech. He died of sepsis two weeks after the ictus. Neuropathologic examination showed a large recent infarct in the left middle cerebral artery territory involving the anterior limb of the internal capsule, the inferior frontal gyrus, superior and middle temporal gyri, and parietal lobe (Fig 1). Petechial hemorrhages were scattered through the infarct. There was an ulcer¬ ated plaque of atherosclerosis with super¬ imposed fresh thrombus on the posterior wall of the left internal carotid artery just distal to the bifurcation (Fig 2). Stenosis was minimal. The left middle cerebral artery was occluded at its main bifurcation by a pale-red mass. Microscopic sections of the left middle cerebral artery revealed moderate athero¬ sclerosis of the stem without significant compromise of the lumen. At the bifurca¬ tion, the occluding plug extended 2 mm along the stem and 7 mm into the superior and inferior branches beyond the bifurca¬ tion. The intraluminal mass consisted of typical atheromatous material containing an abundance of cholesterol clefts. There was no endothelial reaction and the ap¬ pearance was diagnostic of embolism (Fig 3). The presence of capillary proliferation, foamy macrophages, and reactive astrocytes in the middle cerebral infarct was consistent with an age of two weeks. COMMENT The utility of carotid sinus pressure diagnostic and therapeutic proce¬ dure in cardiac arrhythmias is unques¬ tioned. The risk of carotid sinus pres¬ sure is generally regarded as extreme¬ ly small. Lown and Levine' noted only one brief episode of facial weakness during several thousand tests. SiglerJ tested 345 patients without encoun¬ tering untoward effects. Despite the small risk, complications can result in severe morbidity and death. Most ser¬ ious complications fall into the catego¬ ries of cardiac arrhythmias and per¬ manent neurologic deficits.' Among the arrhythmias that have been reported are asystole, ventricular tachycardia, and ventricular fibrilla¬ tion.'7 Death has resulted from arrhythmias in four instances."7 A neurologic complication was first as a Downloaded From: http://archneur.jamanetwork.com/ by a DALHOUSIE UNIVERSITY-DAL-11762 User on 05/18/2015 Fig 2.—Fresh thrombus is superimposed on atherosclerotic plaque on posterior wall of left internal carotid artery. Fig 1.—Horizontal whole brain section showing hemorrhagic infarction in left middle cerebral artery territory. Fig 3—Section of left middle cerebral artery just below its bifurcation, showing occlusion by atheromatous embolus con¬ taining multiple cholesterol clefts (hema¬ toxylin-eosin, original magnification X27). reported by Marmor and Sapirstein" in 1941. Their patient, a 53-year-old man, underwent testing for hypersen¬ sitive carotid sinuses following nor¬ mal neurologic examination results. The left carotid sinus was compressed for 30 s with no reaction. After com¬ pression of the right carotid sinus, there was immediate slowing of the pulse followed in a few seconds by generalized clonic twitchings and un¬ consciousness for 10 to 15 s. He recov¬ ered completely, but after a few min¬ utes became unresponsive and aphas¬ ie. The right leg was paralyzed, the right toe was upgoing, and forced grasping was present in the right hand. Subsequently, paralysis of the right arm and left leg developed. Death occurred four days after the ictus. Neuropathologic examination demonstrated thrombosis of both an¬ terior cerebral arteries accompanied by hemorrhagic infarction of the medial aspects of both frontal lobes. The thrombosis was thought to have been precipitated by slowing the pulse rate and the fall in blood pressure produced by stimulation of the carotid sinus. This was the only pathologically studied case in the literature. A number of clinical observations have been reported. Askey" pooled the experience of the California Heart Association in 1946 and found that there had been six instances of hemi¬ plegia immediately after carotid sinus stimulation performed diagnostically and one instance following chiroprac¬ tic massage. The deficits resolved within 24 hours in two patients, but persisted from one week to several months in five patients before good recovery was made. The deficits were attributed to reflex vasoconstriction of the ipsilateral carotid. Zeman and Siegal"1 described an 83-year-old man who underwent "routine testing of the carotid sinus." A few minutes after the test, a flaccid paralysis of the right arm developed, which per¬ sisted on examination one year later. Both Levine" and Harvey and Roñ¬ an'- have noted single cases of hemi¬ plegia following carotid sinus mas¬ sage, but further details were not provided. Calverly and Millikan"" Downloaded From: http://archneur.jamanetwork.com/ by a DALHOUSIE UNIVERSITY-DAL-11762 User on 05/18/2015 described a total of seven patients who experienced contralateral paraly¬ sis following manipulation of the caro¬ tids in which there was no attempt to elicit a carotid sinus reflex. Clinically, our patient was similar to those described in previous reports in that within one minute of left carotid massage, he experienced the sudden onset of a right hemiplegia. Patholog¬ ically, a large atheromatous embolus occluded the left middle cerebral artery with resultant hemispheral infarction. It is likely that most of the previous cases of hemiplegia asso¬ ciated wnth carotid sinus massage were also caused by atheromatous emboli. A patient described by David et al'"' underwent surgical exploration of the internal carotid artery; however, it was elected not to open the artery because of normal blood flow mea¬ surements. A postoperative hemiple¬ gia resulted from an atheromatous embolus occluding the middle cerebral artery. In addition, cholesterol emboli were found in the retinal arterioles. The large size of the embolus in our patient is in contrast to spontaneous cerebral cholesterol crystal emboli that involve small blood vessels in the range of 15 to 200 µ , resulting in patches of infarction usually less than 1 cm in greatest dimension.'"'7 At times, the infarcts can be confluent, resulting in larger infarcts, particu¬ larly in the arterial border zones.'* The largest reported spontaneous athe¬ romatous embolus occluded the right posterior cerebral artery 3 cm beyond its origin."' The manner in which carotid sinus massage is carried out is difficult to standardize because digital pressure is not measurable. The pressure must not be so great as to occlude the circulation in the internal carotid artery lest cerebral ischemia result directly. One authority has recom¬ mended that the pressure be enough to indent a tennis ball with one palpat¬ ing finger.'- Some clinicians massage with the thumb, others with the fin¬ gers. The movement may be rotatory or along the direction of the artery. Most authorities recommend that massage be applied for no more than 5 s at a time.' The literature usually does not describe the actual massage that caused a hemiplegia. Anyone wdio is familiar with the appearance at autopsy of the ragged, ulcerated atheromatous plaque that may be present exactly in the carotid sinus will be led to use the gentlest effective manipulation, avoiding ex¬ cessive backwards pressure during massage. The presence of a carotid bruit bespeaks a large plaque and warning. However, our patient did not have a bruit. serves as a References tion during carotid sinus stimulation. N Engl J Med 1963;268:1338-1342. 4. Meredith HC, Beckwith JR: Development of ventricular tachycardia following carotid sinus stimulation in paroxysmal supraventricular tachycardia. Am Heart J 1950;39:604-606. 5. Alexander S, Wong GP: Fatal ventricular fibrillation during carotid sinus stimulation. Am J Cardiol 1966;18:289-291. 6. Greenwood RJ, Dupler DA: Death following carotid sinus pressure. JAMA 1962;181:605-609. 7. Hilal H, Massumi R: Fatal ventricular fibrillation after carotid sinus stimulation. N Engl J Med 1966;275:157-158. 8. Marmor J, Sapirstein MR: Bilateral thrombosis of anterior cerebral artery following stimulation of a hyperactive carotid sinus. JAMA 1941;117:1089-1090. 9. Askey JM: Hemiplegia following carotid sinus stimulation. Am Heart J 1946;31:131-137. 10. Zeman FD, Siegal S: Monoplegia following carotid sinus pressure in the aged. Am J Med Sci 1947;213:603-607. 11. Levine SA: Clinical Heart Disease, ed 4. Philadelphia, WB Saunders Co, 1951, p 393. 12. Harvey WP, Ronan JA: Bedside diagnosis of arrhythmias. Prog Cardiovasc Dis 1966;8:419\x=req-\ 445. 13. Calverly JR, Millikan CH: Complications of carotid manipulation. Neurology 1961;11:185-189. 14. Millikan CH: The pathogenesis of transient focal cerebral ischemia. Circulation 1965;32:438\x=req-\ 450. 15. David NJ, Klintworth GK, Friedberg ST, et al: Fatal atheromatous cerebral embolism associated with bright plaques in the retinal arterioles. Neurology 1963;13:708-713. 16. Sturgill BC, Netsky MG: Cerebral infarction by atheromatous emboli. Arch Pathol 1963;76:189-196. 17. Soloway HB, Aronson SM: Atheromatous emboli to central nervous system. Arch Neurol 1964;11:657-667. 18. Beal MF, Williams RS, Fisher CM, et al: 1. Lown B, Levine SA: The carotid sinus: Clinical value of its stimulation. Circulation Cholesterol embolism as a cause of transient ischemic attacks and cerebral infarction. Neurol- 1961;23:766-789. ogy 1980;30:444. 2. Sigler LH: Clinical observations on the carotid sinus reflex. Am J Med Sei 1933;186:110-128. 3. Porus RL, Marcus FI: Ventricular fibrilla- 19. Sayre GP, Campbell DC: Multiple peripheral emboli in atherosclerosis of the aorta. Arch Intern Med 1959;103:799-806. Focal EEG Abnormalities in Heidenhain's Variant of Jakob-Creutzfeldt Disease Anthony J. Furlan, MD; Charles E. Henry, PhD; Patrick J. Sweeney, MD; Hiroshi Mitsumoto, MD \s=b\ Serial EEGs obtained during a sixweek period from a patient with Heidenhain's variant of Jakob-Creutzfeldt disease demonstrated periodic complexes confined to the occipital regions that at no time became generalized. The focal character of the discharges correlated with the site of maximal disease in the occipital cortex, suggesting that cortical damage is a necessary substrate for the production of periodic complexes in Jakob-Creutzfeldt disease. (Arch Neurol 1981;38:312-314) complexes are the most "periodic * characteristic EEG as¬ abnormality sociated with Jakob-Creutzfeldt dis¬ ease. Although usually generalized, such complexes may be focal either initially or throughout the course of the illness. We report the case of a patient with Heidenhain's variant of Accepted for publication July 8, 1980. From the Department of Neurology, Cleveland Clinic and Cleveland Clinic Educational Foundation. Reprint requests to Department of Neurology, Cleveland Clinic, 9500 Euclid Ave, Cleveland, OH 44106 (Dr Furlan). Jakob-Creutzfeldt disease in whom serial EEGs obtained during a sixweek period demonstrated periodic complexes confined to the occipital regions. REPORT OF A CASE A 62-year-old woman complained of headaches, visual loss, and abdominal pain in July 1976. On Aug 20, 1976, she had a transient episode of blindness followed by two major motor seizures that began focally on the right. The patient had been confused and blind since that time. Her speech became unintelligible and she failed to recognize family members. Formed Downloaded From: http://archneur.jamanetwork.com/ by a DALHOUSIE UNIVERSITY-DAL-11762 User on 05/18/2015