Childhood Stroke and Supraventficular Tachy a Figure I. Vijaya L. A t l u r u , MD*, Leon G. Epstein, b i D t, Electrocardtbgram ~n the da), o~ admti~!.;n demun strates widened QRS complex and heart rate o/240 / rain. and Norman Gootman, biD * Case Report Acquired hemiplegia in childhood is uncommon. All causes of stroke in adults should be considered in children, including atherosderotic cerebrovascular disease even though it is rare. In addition, children suffer from many conditions that may result in stroke. Unlike adults, a cardiac illness resulting in stroke is extremely unusual in an otherwise healthy child. We report a case of a four-year-old child; he presented with the sudden onset of a right hemiplegia associated with supraventricular tachycardia. Cerebral angiography indicated a left internal carotid artery occlusion. To our knowledge this is the first reported case of childhood stroke with supraventricular tachycardia (SVT). A 4*/2-year-old left-handed white boy was admitted to Nassau County Medical Center with right-sided weakness and lethargy. He was in good health until five days prior to admission when he began to have recurrent pain and vomiting. He was afebrile and had no history of trauma. On the night prior to admission he complained of headache. On awakening, he was found to have weakness of the right arm and leg and non-fluent aphasia. Pulse rate on admission was 280/min. Blood pressure was 80/40 mm Hg. Spinal tap revealed an opening pressure of 120 mm of H20 with no cells, protein of 20 mg/dl and a glucose of 61 mg/dl. A computed tomogram of his head was normal. He was transferred to the Nassau County Medical Center. Further history revealed that he was the product of a full term pregnancy and a normal vaginal delivery that was followed by normal developmental milestones. Physical examination on admission revealed a temperature of 37.60C, pulse rate of 240/min, respiratory rate of 32/min, and blood pressure of 80/50 mm Hg. Pulses were equal in Atluru VL, Epstein LG, and Gootman N. Childhood stroke and supraventricular tachycardia. Pediat Neurol 1985; 1:54-56. all extremities. Carotid pulsations were equally palpable bilaterally and no bruits were heard over the carotids. Auscultation of the chest was normal. The liver was palpable 4 cm below the right costal margin. He was lethargic but easily arousable. He had global aphasia. Positive neurologic findings included right central facial palsy and flaccid right hemiparesis. There was areflexia and an extensor plantar Introduction A majority of the cases of acquired acute hemiplegia in children are of undetermined etiology [1]. Cerebral arterial occlusive disease constitutes a major known etiologic factor. The pathogenesis of occlusion is usually not determined. Intracranial occlusion can result from structural changes, infection [2], or embolism. Children with cyanotic congenital heart disease and rheumatic fever have significantly increased risk of cerebrovascular occlusive disease. In an otherwise healthy child, tachyarrhythmia resulting in stroke, is uncommon. Our patient presented with acute onset of right hemiplegia associated with SVT. From the *Departments of Pediatric Neurology and Pediatrics; State University of New York at Stony Brook, Nassau County Medical Center; East Meadow, NY; and the tDepartment of Neurosciences; University of Medicine and Dentistry of New Jersey; Newark, NJ. 54 PEDIATRIC NEUROLOGY Vol. 1 No. 1 response on the right. Chest x-ray revealed cardiomegaly with normal vascular markings. Electrocardiogram showed SVT with widened QRS complex. The heart rate was 240/min (Fig 1), computed tomography of the head demonstrated only minimal compression of the left frontal horn. Other laboratory studies including white blood count, serum electrolytes, calcium, serum osmolality, creatine kinase, urinalysis, antinuclear antibodies, rheumatoid factor, prothrombin time, and partial thrombop!astin time were all normal. The platelet count was 4 lO,000/mm 3. Viral cultures revealed no growth. Intravenous edrophonium 7.3 mg, propranalol 0.1 mg, and digoxin 50/zg were administered with no response. Four hours later cardioversion was performed at 50 watt-see following which the heart rate decreased to 90/min. ECG documented a normal sinus rhythm with right bundle branch block. Communications should be addressed to: Vijaya L. Atluru, MD; Nassau County Medical Center; 2201 Hempstead Turnpike; East Meadow, NY 11554. Figure 2. A lateral projection of left carotid arteriogram on the day of admission shows complete occlusion of the internal carotid after. Four vessel cerebral angiography performed on the day of admission demonstrated extracranial occlusion of the left internal carotid artery (Fig 2). The left anterior cerebral artery originated from the right carotid circulation. On the second hospital day he was awake with non-fluent aphasia but able to follow commands well. On the fourth hospital day while on maintenance doses of digoxin and propranalol, he again developed SVT. He did not respond to Ftgure ~. An unenhanced CT fcan stay month¢ later shows low densities in the left deep temporal region and the posterior limb o[ the internal capsule with mild ddation o/Cthe lj~¢ilateral frontal horn. repeated doses of edrophonium; however, successful cardioversion was again accomplished. Cardiac echocardiogram, sector scan, and been reported following paroxysmal atrial tachycardia cardiac catheterization revealed no pathology. The child gradually [8]. recovered his neurologic function. He had normal speech and was ambulating well. Repeat CT scan revealed old infarcts deep in the left temporal region and in the posterior limb of the internal capsule (Fig 3). Eighteen months after the initial episode the child had bruits over both carotids and over both orbits, left greater than right. Repeat four vessel cerebral angiography demonstrated narrowing of the cervical portion of the left internal carotid artery with complete block distai to the origin of the left opthalmic artery. The child continues to do well in regular school despite a mild residual hemiparesisand remains in normal sinus rhythm. Second, this child may have had a congenital anomaly of the vessel with relative narrowing. Such anomalies have been described in neurofibromatosis [7]; however, this child and his family had no findings of this condition. This malformation could have acted in concert with generalized cerebral ischemia resulting from the tachyarrythmia to produce focal ischemic damage. Tachyarrythmias have been reported to cause hypotension and symptoms of generalized transient ischemia in the form of dizziness and syncope in adults Discussion A variety of possible causes of carotid occlusion can be postulated in our patient. First, cardiac arrythmia may have resulted in congestive heart failure with poor myocardial contraction and mural thrombus formation followed by embolism to the cerebral circulation. In spite of the normal cardiac catheterization findings and the lack of specific findings consistent with embolic process in the cerebral angiogram, mural thrombus remains a possibility. Peripheral arterial embolism has [5]. A third consideration is that of a systemic vasculitis affecting both the cerebral and cardiac vessels resulting in a cerebrovascular accident and tachyarrythmia. Arteritis, both systemic and local [1,2,6], is an accepted cause of cerebral arterial occlusion. The angiographic findings in our patient are similar to prior reports of local inflammation associated with nasopharyngeal infection resulting in proximal carotid occlusion; however, no findings of diffuse vasculitis were seen. Atluru et al: Stroke and Supraventricular Tachycardia 53 Our patient did have signs and symptoms of systemic illness prior to his stroke but laboratory studies did not define a specific infectious etiology or provide evidence of disorders associated with vasculitis. The last possibility is that of primary (usually idiopathic) occlusive disease of the internal carotid artery [1]. This situationmight be analogous to the occurrence of arrhythmias following subarachnoid hemorrhage due to a cerebral aneurysm. On the basis of the angiographic finding, carotid occlusion of the idiopathic type seems a more likely explanation than that of a malformation of the cerebral vessel, vasculitis, or emboli. The subsequent narrowing of the internal carotid artery proximal to the occlusion is believed to be due to decreased blood flow [1] and is not specific for any etiology. Internal carotid artery occlusion secondary to trauma was considered but no history of trauma was elicited. It is obvious from the preceding discussion that a precise cause and effect relationship cannot be established between SVT and childhood stroke in the above described patient. 56 PEDIATRIC NEUROLOGY Vol. 1 No. 1 The authors thank Dr. Platon J. Collipp for tti~i~al review of the manuscript, andJanice Wisniewski for secretarial assistance References [1] Hilal S, Soloman G, Gold A, et al. Primary cerebral arterial occlusive disease in children. Part 1, Acute acquired hemiplegia. Neuroradiol 1971;99:71-86. [2] Bickerstaff ER. A etiology of acute hemiplegia in childhood. Brit MedJ 1964;2:82-87. [3] Lundberg A. Paroxysmal atrial tachycardia in infancy: Long term follow up study of 49 subjects. Pediatrics 1982;70:638-642. [4] G-arson A Jr. Supraventricular tachycardia, In: Gillette P and Garson A Jr, eds. Pediatric cardiac dysarrhythmias. New York: Gruhn and Stratton, 1981;177-253. [51 Van Durme JP. Tacharrhythmias and transient cerebral ischemic attacks. Am HeartJ 1975;89:538-540. [6] Harwood-Nash DG, McDonald P, Argent W. Cerebral arterial disease in children: An angiographic study of 40 cases. Am J Roentgenol 1971 ; 111:672-686. [7] Levisohn PM, Mikhael MA, Rothman SM. Cerebrovascular changes in neurofibromatosis. Dev Med & Child Neurol 1978;20:789-793. [8] Mody G, Folger GM. Peripheral arterial embolism following paroxysmal atrial tachycardia. AmJ Dis Child 1973; 126:520-522.