moya disease

surveyed

25

published

reports and yielded 205 patients.3 In

only three instances were seizures clearly stated to be the initial symp¬

a child younger than 15 years old. On the basis of the series of Nishimoto and Takeuchi, it would seem that perhaps the Japanese expe¬ rience with this phenomenon differs from that reported in the periodicals included in the National Library of Medicine's bibliographic compila¬ tions. With regard to the nature of the spells occurring in the patient de¬ scribed in our case report, Dr Sunder questions whether they were seizures or transient ischemie attacks. The spells usually occurred in the evening when the patient was drowsy. In the patient, left-sided head pain, often associated with nausea or vomiting, would typically develop. These head¬ aches might occur in the absence of any further symptoms. At the start of a spell, the patient characteristically described an aura consisting of a "feeling of strangeness and fear." When asked to be more specific, she said, "I know I am Lisa, but don't feel like Lisa." Following this sensation, she would suddenly cry out for no

tom in

apparent reason, stare straight ahead,

and appear to lose contact with her surroundings. She would again be¬ come aware of her environment in less than one minute, but was commonly left with a mild aphasia, right hemi¬ paresis, and right facial droop. Al¬ though the language and motor prob¬ lems usually lasted less than five minutes, occasionally they would con¬ tinue for several hours. Typically, the headache would persist for several hours. When asked what she could recall about these spells, she would remember the headache and "strange feeling" and would next remember the difficulty speaking and the mild weakness. She had no recollection of intervening events. In our own experi¬ ence, the presence of an aura, staring, and loss of contact with one's surroundings are much more charac¬ teristic of a seizure phenomenon and have not been observed by us with transient ischemie attacks unassociated with seizure activity. Informa¬ tion concerning the results of electro¬ encephalography and the response to treatment with anticonvulsants were included in our report for complete¬ ness and were not intended to serve as proof of the presence of a seizure

disorder. We would agree with Dr Sunder that what we interpreted as seizures most likely had an underlying cere-

brovascular origin. We reported this because we were unaware of any other child in whom a seizure disorder and vascular headaches were the only manifestations of moyamoya disease and who was under close medical observation for a period of three years. One might argue that in anoth¬ er five years, other neurologic symp¬ toms may develop in this patient. Although this may certainly be the case, we have no way of predicting such an outcome. We appreciate Dr Sunder's interest in our article and are pleased that he has added his own experience to the literature. It is only through such additional reports that we will have a better indication of the frequencv of this phenomenon. Bruce S. Schoenberg, MD, MPH Natl Institute of Neurological and Communicative Disorders and Stroke Bethesda, Md James F. Mellinger, MD Section of Pediatric Neurology Dept of Neurology Mayo Clinic Rochester, Mn case

1.

Schoenberg BS, Mellinger JF, Schoenberg

et al: Moyamoya disease presenting as a seizure disorder: A case report. Arch Neurol 34:511-512, 1977. 2. Nishimoto A, Takeuchi S: Moyamoya disease: Abnormal cerebrovascular network in the cerebral basal region, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. New York, Elsevier Publishing Co, 1971, vol 12, pp 352-383. 3. Schoenberg BS, Mellinger JF, Schoenberg DG: Moyamoya disease in childhood: Case series and review of the literature. South Med J, in press.

DG,

Paroxysmal

Atrial

Tachycardia

To the Editor.\p=m-\Thereport in the Archives by Rush et al titled "Paroxysmal Atrial Tachycardia and Frontal Lobe Tumor" (34:578-580, 1977) suggests that a 49-year-old male patient had symptoms secondary to paroxysmal atrial tachycardia (PAT) and that the PAT was secondary to seizure activity related to a frontal lobe glioma. Unfortunately, this report gives no documentation that the patient had PAT and, in addition, there is no evidence that his symptoms were in any way related to a change in cardiac function. Certainly, these symptoms cannot be explained by a pulse rate of 110 beats per minute. It is possible that the patient's spells could have been partial seizures with an associated mild sinus tachycardia. Figure 1 showing four channels of EEG monitored during one of the patient's spells is small, making interpretation difficult. However, I see no clear electrical seizure activity,

this possibility cannot be ruled out from the sample. Although unusual at age 49, it is entirely possi¬ ble that the change shown in the EEG is a response to hyperventilation. It seems to me that the origin of the patient's spells remains obscure. An additional point is the EEG in Fig 3, which is reported to show a spike "almost exclusively localized to [the] right nasopharyngeal area." In fact, the EEG (Fig 3) suggests that the spike is distributed widely over the surface leads on the right and to the left frontopolar lead as well as the ear (presumably the right ear) and does not appear at the nasopharyn¬ geal leads at all. James A. Zeese, MD Dept of Neurol Univ Minnesota Univ Hospitals Minneapolis, MN 55455

although

In Reply.\p=m-\ Dr Zeese's letter questions the diagnosis of PAT and its association with seizure activity related to a frontal lobe glioma. Electrocardiographically, and clinically, the episodes were of sudden onset and offset.

Electrocardiographic telemetry, a sample of which was deleted by the editor, because of limited space, documented a pulse rate of 160 beats per

minute. It is a fact that, at the time of the ECG telemetry illustrated in the article, the cardiac lead was not available and the pulse rate palpated by an observer was 110 beats per minute; it is probable that this reading was obtained at the termination of the seizure and thus does not reflect the cardiac rate during the actual event itself. Hyperventilation was never observed in this patient. The patient's symptoms corresponded temporally with the rapid heartbeat, and subsequent to surgery he has not been troubled with either changes in cardiac rate or other complaints. We agree that the statement "almost exclusively localized to [the] right nasopharyngeal area" states the case too strongly even though the tumor itself was located in the right frontopolar area. In short, we think we have adequately docu¬ mented the association of a tumor with epileptiform activity and it is highly likely that EEG and ECG abnormalities were causally related. Jeffery L. Rush, MD Bruce A. Everett, MD Alan H. Adams, MD John A. Kusske, MD VA Hosp 5901 E Seventh St Long Beach, CA 90822

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Paroxysmal atrial tachycardia.

moya disease surveyed 25 published reports and yielded 205 patients.3 In only three instances were seizures clearly stated to be the initial symp...
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