Report of Cases.\p=m-\Case1.\p=m-\A41/2-year-old boy underwent total correction of transposition of the great vessels. A right hemiparesis occurred after postoperative Italian
anoxia. He recovered with minimal residua. At age 11 years, he had a subarachnoid
hemorrhage (SAH). Computerized tomography of the brain showed a left frontal lobe hematoma subadjacent to frontoparietal atrophy. Cerebral angiography demonstrated moyamoya of the left middle cerebral artery. He has maintained recovery for three years. Case 2.—Hemiparesis developed during a painful crisis in an 8-year-old black girl with sickle cell disease. A technetium Tc 99m radionuclide brain scan demonstrated a wedge-shaped uptake in the region of the left middle cerebral artery. She recovered almost completely. Six years later, she had SAH. Computerized tomography an showed left parietotemporal atrophy and a fresh hemorrhage in the subadjacent basal ganglia. Cerebral angiography demon¬ strated moyamoya of the left middle cere¬ bral artery. Her recovery has been sus¬ tained for 2Vè years.
Comment.—The typical angiograph¬ ie appearance of moyamoya has been described in several disease entities.2"6 However, moyamoya has been asso¬ ciated with sickle cell disease in only two previous reports7'" and with congenital heart disease in only one prior case." Although the two patients described here are not unique, they emphasize the need for careful inves¬ tigation of cerebrovascular events in children regardless of the underlying process.
Moyamoya generally presents
as
subarachnoid hemorrhage in adults and strokes in children and only rarely as a seizure disorder in children.4 The occurrence of SAH in children with moyamoya has been previously noted in only three Japanese children7 and in one patient of Solomon et aP at age 23 years, 12 years after her initial hemiparesis. Neither of the patients described here were hypertensive, had a coagulopathy, or an acute change in their primary disease prior to their SAH. The previously reported cases and these two children suggest that children as well as adults with moya¬ moya are at risk for SAH. The period of risk extends at least until adult¬ hood. The follow-up-care of children with moyamoya should be concerned with this specific possibility.
Carl J. Crosley, MD Dept of Neurol State University of New York
Upstate Medical Center Syracuse, NY 1. Suzuki
J, Takaku A: Cerebrovascular
"moyamoya" disease:
Disease
showing abnormal
net-like vessels in base of brain. Arch Neurol
20:288-299, 1969.
2. Taveras JM: Multiple progressive intracranial arterial occlusions: A syndrome of children and young adults. Am J Roentgenol 106:235-268, 1969. 3. Solomon EG, Hilal SK, Gold AP, et al: Natural history of acute hemiplegia of childhood. Brain 93:107-120, 1970. 4. Schoenberg BS, Mellinger JF, Schoenberg DG: Moyamoya disease in children. South Med J 71:237-241, 1978. 5. Stock JA, Nigro MA, Mishkin MM, et al: Occlusion of large cerebral vessels in sickle cell anemia. N Engl J Med 287:846-849, 1972. 6. Numaguchi Y, Balsys R, Marc JA, et al: Some observations in progressive arterial occlusions in children and young adolescents. Surg Neurol 6:293-300, 1976. 7. Nishimoto A, Takeuchi S: Moyamoya disease, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam, North Holland Publishing Co, 1972, vol 12, pp 352-383.
Visuospatial Judgment To the Editor.\p=m-\Inreference to the article "Visuospatial Judgment," by Benton et al in the Archives (35:364\x=req-\ 367, 1978), the authors emphasize that defective performance occurred in the right cerebral lesion cohort only in patients with posterior or indeterminate lesions. What percentage of the left cerebral lesions occurred posteriorly or indeterminately? Is it possible that a number of patients with left cerebral lesions placed posteriorly or indeterminately had to be excluded from testing because they were too dysphasic to understand the instructions for the test? This point may partially explain the surprisingly normal results that occurred among the left cerebral cohort. Sana Louis Bloch, MD Dept of Neurol Albert Einstein Coll of Med Bronx, NY 10461 In
Reply.\x=req-\
point.
There
Dr Bloch raises is
the
a
possibility of
good a
bias
selection when patients with right and left hemisphere lesions are compared on one or another performance. Some patients with severe receptive language disorder either fail to grasp nonverbal pantomimed (as well as verbal) instructions or they apparently forget the instructions in the course of task performance. This also may occur with nonaphasic patients with massive right hemisphere disease and with demented patients. In our study, five nonaphasic patients with right hemisphere lesions were excluded from the study because they failed to respond appropriately to the easy practice items preceding the test. Of the 48 patients with left hemisphere disease, nine had anterior lesions of whom six were aphasic, 20 in
case
posterior lesions of whom 14 were aphasic, and 19 patients had lesions that either could not be confidently
had
localized or extended into both the anterior and posterior regions, 18 of these were aphasic. Two of the three moderately defective performances (score of 17 to 18) in the left hemi¬ sphere group occurred among the six nonaphasic patients with posterior lesions. Both patients had occipital lobe disease. The other moderately defective performance (score, 18) was made by an aphasie patient with a lesion of indeterminate locus. All the 14 aphasie patients with posterior lesions and the six aphasie patients with anterior lesions performed ade¬ quately. It seems clear that aphasie disorder, even of the Wernicke type, was not substantially related to per¬ formance level. Most of the aphasie patients in the study were also given other nonverbal tests assessing constructional praxis,1 pantomime recognition,'-' and sound recognition.3 A substantial proportion of them performed defectively on one or more of these tests, which contrasts with their adequate performance on the visuospatial test. The presence of other visual or auditory cognitive defects in these aphasie patients with left hemisphere disease suggests a dissociation in performance according to the nature of the task rather than a substantial bias in case selection. Arthur L. Benton, PhD Nils R. Varney, PhD Kerry des. Hamsher, PhD Dept of Neurol Univ Hospitals Iowa City, Iowa 1. Benton AL: Visuoconstructive disability in with cerebral disease. Doc Ophthalmol 34:67-76, 1973. 2. Varney NR: Linguistic correlates of pantomime recognition in aphasic patients. J Neurol Neurosurg Psychiatr 41:564-568, 1978. 3. Benton AL: Problems of test construction in the field of aphasia. Cortex 3:32-58, 1967.
patients
Moyamoya
Disease
To the Editor.\p=m-\Iread with interest the report in the Archives by Schoenberg et al (34:511-512, 1977) of a 7\x=req-\ year-old girl with moyamoya disease who had seizures and headache, which the authors state is a rare phenomeI think some comment is non. warranted. Analysis of a series of 111 patients by Nishimoto and Takeuchi1 resulted in their finding that convulsions and headaches were relatively common. Of 73 patients younger than 16 years old, seizures were an initial symptom in 15 and the primary reason for seeking
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medical attention in all but one of these. In a single year at Duke, I cared for four children in whom this diagnosis was proved. Seizure was the initial symptom in one girl who experienced prolonged focal convulsions at the ages of 13 and 22 months, two years prior to the development of repeated ischemic events at the age of 4 years. Treatment with anticonvulsants, although appearing effective after her first two seizures, had no effect on her subsequent episodes, and the diagno¬ sis was made angiographically at the age of 7 years and 3 months. These two patient groups would support an inci¬ dence of seizures in more than 20% of the children with this problem, hardly
qualifying as a rare phenomenon. Despite this argument, from the data presented in the authors' descrip¬
tion, I have serious doubts that the girl's episodes represented true cere¬ bral seizures. On only one occasion is it mentioned that "motor symptoms" were present, by which I assume that focal clonic activity is meant. All other symptoms described are negative phe¬ nomena, including alteration of con¬ tact with the environment, aphasia, and focal paresis of unclear duration. It is difficult to accept these episodes as either inhibitory seizures, which are reported but are associated with spike and wave discharge on EEG,2 or as postictal events with no clear ictus demonstrated electrically or clinically.
Similar symptoms have been observed in all four of the Duke patients, and all had the character of transient is¬ chemie attacks. The one EEG reported by the authors was done during the episode associated with motor symptoms and showed only slowing with focal accen¬ tuation, a pattern not unheard of during seizure but certainly one more compatible with cerebral ischemia. The associated clinical seizure on that occasion is then more likely a response to hypoxia. A similar EEG pattern has been seen in two of my own four patients who had no seizure history and in whom focal paroxysmal slow¬ ing on EEG could be elicited easily with hyperventilation, even in the absence of clinical symptoms. This seems to represent an imbalance of blood flow through abnormal vessels with aberrant response to certain stimuli (unpublished data). I would add that the response to phenytoin is not reasonable proof of seizure in a notoriously variable disease. The idea that cerebrovascular insuf¬ ficiency produces headache is not a new concept. All of the four children seen by me suffered vascular-type
headaches both with and without clin¬ ical neurologic changes, as did several patients in the series of Taveras.3 In the Japanese series, headache was a cardinal symptom in 14 of the 73 children studied. This may take the form of childhood migraine, but many patients have atypical features if closely considered. Despite these reservations, I concur with the authors' conclusion that vascular disease of the moyamoya type is more common in children than has previously been considered in the English literature. Although I dis¬ agree that the case presented is very atypical, it serves to point up some of the features that should make this disease suspect in a child. The condi¬ tion needs to be considered in certain children with seizures as part of their problem. To these I would add chil¬ dren with atypical forms of vascular headache, particularly those with the
hémiplégie variety. Angi¬ ography of children in larger centers is a fairly safe and acceptable proce¬ dure and deserves more use by those of us dealing with pediatrie neurologic disease. The importance lies in mak¬ ing the diagnosis, allowing then for therapeutic considerations. Cervical sympathectomy for symptomatic re¬
uncommon
lief of ischemie attacks from this condition presently shows some prom¬ ise.4 More particularly, however, ad¬ vances in cerebrovascular microsur¬ gery may soon offer a means of abort¬ ing a future devastating neurologic insult in these children. Theodore R. Sunder, MD Pediatric Service Naval Regional Med Ctr Camp Pendleton, CA 1. Nishimoto A, Takeuchi S: Moyamoya dis-
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. 2. Hansen P: Hemiparetic seizures. Read before the Child Neurology Society meeting, Monterey, Calif, 1976. 3. Taveras J: Multiple progressive intracranial occlusions: A syndrome of children and young adults. AJR 106:235-268, 1969. 4. Suzuki J, Takaku A, Kodama N, et al: An attempt to treat cerebrovascular moyamoya disease in children. Child's Brain 1:193-206, 1975. ease:
Reply.\p=m-\ We appreciate Dr Sunder's thoughtful comments concerning our
In
article. His letter concentrates on three major points: (1) the frequency of seizures as the initial symptom of childhood moyamoya disease, (2) whether the stereotyped spells described in our report represent seizures or transient ischemic attacks, and (3) whether these spells were secondary to underlying vascular dis-
We shall attempt to address each of these issues. After studying the patient described in our report, we made what we consider to be a fairly exhaustive review of the literature available to us. Searches of the Index Medicus and the Medlars /Medline computerized bibliographic system of the National Library of Medicine were carried out. Each article so obtained was reviewed for details of the cases reported as well as for possible additional references. Unfortunately, neither the review appearing in the Handbook of Clinical Neurology2 and mentioned by Dr Sunder nor Dr Sunder's unpublished series was thus disclosed. In the analysis by Nishimoto and Takeuchi2 of 73 patients with moya¬ moya younger than 16 years of age referred to by Dr Sunder, convulsive seizures were reported to be one of the "initial symptoms" in 15 patients and one of the "cardinal symptoms" in 14. On the basis of the figures quoted by Nishimoto and Takeuchi, individual, patients must have had multiple "ini¬ tial and cardinal symptoms." Conse¬ quently, if one were to calculate a percentage distribution for each of the initial symptoms as Dr Sunder did for seizures, one would come up with a total percentage in excess of 100%. Furthermore, it is unclear what the authors of this series meant by initial symptoms and cardinal symptoms. For example, "mental retardation" is listed as an initial symptom; we presume that mental retardation rep¬ resents a residual disability following some acute neurologic event. Dr Sun¬ der interprets the results of the Japa¬ nese study to mean that "... seizures were an initial symptom in fifteen and the primary reason for coming to medical attention in all but one of these." This is based on the assump¬ tion that the 14 patients for whom seizures are listed as a cardinal symp¬ tom all had seizures as an initial symp¬ tom. This may not be the case, howev¬
ease.
er.
The danger inherent in generaliz¬ ing from a single series is also appar¬ ent in the chapter referred to by Dr
Sunder. The authors of that report conclude that moyamoya disease is rare among non-Japanese. It is only in an addendum that reviews literature in 1969 and 1970, that Nishimoto and Takeuchi conclude that the disease may be found with some frequency outside of Japan as well. Non-Japa¬ nese cases reported in these more recent articles are not included in the figures quoted by Dr Sunder. Our own review of childhood moya-
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anoxia. He recovered with minimal residua. At age 11 years, he had a subarachnoid
hemorrhage (SAH). Computerized tomography of the brain showed a left frontal lobe hematoma subadjacent to frontoparietal atrophy. Cerebral angiography demonstrated moyamoya of the left middle cerebral artery. He has maintained recovery for three years. Case 2.—Hemiparesis developed during a painful crisis in an 8-year-old black girl with sickle cell disease. A technetium Tc 99m radionuclide brain scan demonstrated a wedge-shaped uptake in the region of the left middle cerebral artery. She recovered almost completely. Six years later, she had SAH. Computerized tomography an showed left parietotemporal atrophy and a fresh hemorrhage in the subadjacent basal ganglia. Cerebral angiography demon¬ strated moyamoya of the left middle cere¬ bral artery. Her recovery has been sus¬ tained for 2Vè years.
Comment.—The typical angiograph¬ ie appearance of moyamoya has been described in several disease entities.2"6 However, moyamoya has been asso¬ ciated with sickle cell disease in only two previous reports7'" and with congenital heart disease in only one prior case." Although the two patients described here are not unique, they emphasize the need for careful inves¬ tigation of cerebrovascular events in children regardless of the underlying process.
Moyamoya generally presents
as
subarachnoid hemorrhage in adults and strokes in children and only rarely as a seizure disorder in children.4 The occurrence of SAH in children with moyamoya has been previously noted in only three Japanese children7 and in one patient of Solomon et aP at age 23 years, 12 years after her initial hemiparesis. Neither of the patients described here were hypertensive, had a coagulopathy, or an acute change in their primary disease prior to their SAH. The previously reported cases and these two children suggest that children as well as adults with moya¬ moya are at risk for SAH. The period of risk extends at least until adult¬ hood. The follow-up-care of children with moyamoya should be concerned with this specific possibility.
Carl J. Crosley, MD Dept of Neurol State University of New York
Upstate Medical Center Syracuse, NY 1. Suzuki
J, Takaku A: Cerebrovascular
"moyamoya" disease:
Disease
showing abnormal
net-like vessels in base of brain. Arch Neurol
20:288-299, 1969.
2. Taveras JM: Multiple progressive intracranial arterial occlusions: A syndrome of children and young adults. Am J Roentgenol 106:235-268, 1969. 3. Solomon EG, Hilal SK, Gold AP, et al: Natural history of acute hemiplegia of childhood. Brain 93:107-120, 1970. 4. Schoenberg BS, Mellinger JF, Schoenberg DG: Moyamoya disease in children. South Med J 71:237-241, 1978. 5. Stock JA, Nigro MA, Mishkin MM, et al: Occlusion of large cerebral vessels in sickle cell anemia. N Engl J Med 287:846-849, 1972. 6. Numaguchi Y, Balsys R, Marc JA, et al: Some observations in progressive arterial occlusions in children and young adolescents. Surg Neurol 6:293-300, 1976. 7. Nishimoto A, Takeuchi S: Moyamoya disease, in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology. Amsterdam, North Holland Publishing Co, 1972, vol 12, pp 352-383.
Visuospatial Judgment To the Editor.\p=m-\Inreference to the article "Visuospatial Judgment," by Benton et al in the Archives (35:364\x=req-\ 367, 1978), the authors emphasize that defective performance occurred in the right cerebral lesion cohort only in patients with posterior or indeterminate lesions. What percentage of the left cerebral lesions occurred posteriorly or indeterminately? Is it possible that a number of patients with left cerebral lesions placed posteriorly or indeterminately had to be excluded from testing because they were too dysphasic to understand the instructions for the test? This point may partially explain the surprisingly normal results that occurred among the left cerebral cohort. Sana Louis Bloch, MD Dept of Neurol Albert Einstein Coll of Med Bronx, NY 10461 In
Reply.\x=req-\
point.
There
Dr Bloch raises is
the
a
possibility of
good a
bias
selection when patients with right and left hemisphere lesions are compared on one or another performance. Some patients with severe receptive language disorder either fail to grasp nonverbal pantomimed (as well as verbal) instructions or they apparently forget the instructions in the course of task performance. This also may occur with nonaphasic patients with massive right hemisphere disease and with demented patients. In our study, five nonaphasic patients with right hemisphere lesions were excluded from the study because they failed to respond appropriately to the easy practice items preceding the test. Of the 48 patients with left hemisphere disease, nine had anterior lesions of whom six were aphasic, 20 in
case
posterior lesions of whom 14 were aphasic, and 19 patients had lesions that either could not be confidently
had
localized or extended into both the anterior and posterior regions, 18 of these were aphasic. Two of the three moderately defective performances (score of 17 to 18) in the left hemi¬ sphere group occurred among the six nonaphasic patients with posterior lesions. Both patients had occipital lobe disease. The other moderately defective performance (score, 18) was made by an aphasie patient with a lesion of indeterminate locus. All the 14 aphasie patients with posterior lesions and the six aphasie patients with anterior lesions performed ade¬ quately. It seems clear that aphasie disorder, even of the Wernicke type, was not substantially related to per¬ formance level. Most of the aphasie patients in the study were also given other nonverbal tests assessing constructional praxis,1 pantomime recognition,'-' and sound recognition.3 A substantial proportion of them performed defectively on one or more of these tests, which contrasts with their adequate performance on the visuospatial test. The presence of other visual or auditory cognitive defects in these aphasie patients with left hemisphere disease suggests a dissociation in performance according to the nature of the task rather than a substantial bias in case selection. Arthur L. Benton, PhD Nils R. Varney, PhD Kerry des. Hamsher, PhD Dept of Neurol Univ Hospitals Iowa City, Iowa 1. Benton AL: Visuoconstructive disability in with cerebral disease. Doc Ophthalmol 34:67-76, 1973. 2. Varney NR: Linguistic correlates of pantomime recognition in aphasic patients. J Neurol Neurosurg Psychiatr 41:564-568, 1978. 3. Benton AL: Problems of test construction in the field of aphasia. Cortex 3:32-58, 1967.
patients
Moyamoya
Disease
To the Editor.\p=m-\Iread with interest the report in the Archives by Schoenberg et al (34:511-512, 1977) of a 7\x=req-\ year-old girl with moyamoya disease who had seizures and headache, which the authors state is a rare phenomeI think some comment is non. warranted. Analysis of a series of 111 patients by Nishimoto and Takeuchi1 resulted in their finding that convulsions and headaches were relatively common. Of 73 patients younger than 16 years old, seizures were an initial symptom in 15 and the primary reason for seeking
Downloaded From: http://archneur.jamanetwork.com/ by a Georgetown University Medical Center User on 05/25/2015
medical attention in all but one of these. In a single year at Duke, I cared for four children in whom this diagnosis was proved. Seizure was the initial symptom in one girl who experienced prolonged focal convulsions at the ages of 13 and 22 months, two years prior to the development of repeated ischemic events at the age of 4 years. Treatment with anticonvulsants, although appearing effective after her first two seizures, had no effect on her subsequent episodes, and the diagno¬ sis was made angiographically at the age of 7 years and 3 months. These two patient groups would support an inci¬ dence of seizures in more than 20% of the children with this problem, hardly
qualifying as a rare phenomenon. Despite this argument, from the data presented in the authors' descrip¬
tion, I have serious doubts that the girl's episodes represented true cere¬ bral seizures. On only one occasion is it mentioned that "motor symptoms" were present, by which I assume that focal clonic activity is meant. All other symptoms described are negative phe¬ nomena, including alteration of con¬ tact with the environment, aphasia, and focal paresis of unclear duration. It is difficult to accept these episodes as either inhibitory seizures, which are reported but are associated with spike and wave discharge on EEG,2 or as postictal events with no clear ictus demonstrated electrically or clinically.
Similar symptoms have been observed in all four of the Duke patients, and all had the character of transient is¬ chemie attacks. The one EEG reported by the authors was done during the episode associated with motor symptoms and showed only slowing with focal accen¬ tuation, a pattern not unheard of during seizure but certainly one more compatible with cerebral ischemia. The associated clinical seizure on that occasion is then more likely a response to hypoxia. A similar EEG pattern has been seen in two of my own four patients who had no seizure history and in whom focal paroxysmal slow¬ ing on EEG could be elicited easily with hyperventilation, even in the absence of clinical symptoms. This seems to represent an imbalance of blood flow through abnormal vessels with aberrant response to certain stimuli (unpublished data). I would add that the response to phenytoin is not reasonable proof of seizure in a notoriously variable disease. The idea that cerebrovascular insuf¬ ficiency produces headache is not a new concept. All of the four children seen by me suffered vascular-type
headaches both with and without clin¬ ical neurologic changes, as did several patients in the series of Taveras.3 In the Japanese series, headache was a cardinal symptom in 14 of the 73 children studied. This may take the form of childhood migraine, but many patients have atypical features if closely considered. Despite these reservations, I concur with the authors' conclusion that vascular disease of the moyamoya type is more common in children than has previously been considered in the English literature. Although I dis¬ agree that the case presented is very atypical, it serves to point up some of the features that should make this disease suspect in a child. The condi¬ tion needs to be considered in certain children with seizures as part of their problem. To these I would add chil¬ dren with atypical forms of vascular headache, particularly those with the
hémiplégie variety. Angi¬ ography of children in larger centers is a fairly safe and acceptable proce¬ dure and deserves more use by those of us dealing with pediatrie neurologic disease. The importance lies in mak¬ ing the diagnosis, allowing then for therapeutic considerations. Cervical sympathectomy for symptomatic re¬
uncommon
lief of ischemie attacks from this condition presently shows some prom¬ ise.4 More particularly, however, ad¬ vances in cerebrovascular microsur¬ gery may soon offer a means of abort¬ ing a future devastating neurologic insult in these children. Theodore R. Sunder, MD Pediatric Service Naval Regional Med Ctr Camp Pendleton, CA 1. Nishimoto A, Takeuchi S: Moyamoya dis-
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. 2. Hansen P: Hemiparetic seizures. Read before the Child Neurology Society meeting, Monterey, Calif, 1976. 3. Taveras J: Multiple progressive intracranial occlusions: A syndrome of children and young adults. AJR 106:235-268, 1969. 4. Suzuki J, Takaku A, Kodama N, et al: An attempt to treat cerebrovascular moyamoya disease in children. Child's Brain 1:193-206, 1975. ease:
Reply.\p=m-\ We appreciate Dr Sunder's thoughtful comments concerning our
In
article. His letter concentrates on three major points: (1) the frequency of seizures as the initial symptom of childhood moyamoya disease, (2) whether the stereotyped spells described in our report represent seizures or transient ischemic attacks, and (3) whether these spells were secondary to underlying vascular dis-
We shall attempt to address each of these issues. After studying the patient described in our report, we made what we consider to be a fairly exhaustive review of the literature available to us. Searches of the Index Medicus and the Medlars /Medline computerized bibliographic system of the National Library of Medicine were carried out. Each article so obtained was reviewed for details of the cases reported as well as for possible additional references. Unfortunately, neither the review appearing in the Handbook of Clinical Neurology2 and mentioned by Dr Sunder nor Dr Sunder's unpublished series was thus disclosed. In the analysis by Nishimoto and Takeuchi2 of 73 patients with moya¬ moya younger than 16 years of age referred to by Dr Sunder, convulsive seizures were reported to be one of the "initial symptoms" in 15 patients and one of the "cardinal symptoms" in 14. On the basis of the figures quoted by Nishimoto and Takeuchi, individual, patients must have had multiple "ini¬ tial and cardinal symptoms." Conse¬ quently, if one were to calculate a percentage distribution for each of the initial symptoms as Dr Sunder did for seizures, one would come up with a total percentage in excess of 100%. Furthermore, it is unclear what the authors of this series meant by initial symptoms and cardinal symptoms. For example, "mental retardation" is listed as an initial symptom; we presume that mental retardation rep¬ resents a residual disability following some acute neurologic event. Dr Sun¬ der interprets the results of the Japa¬ nese study to mean that "... seizures were an initial symptom in fifteen and the primary reason for coming to medical attention in all but one of these." This is based on the assump¬ tion that the 14 patients for whom seizures are listed as a cardinal symp¬ tom all had seizures as an initial symp¬ tom. This may not be the case, howev¬
ease.
er.
The danger inherent in generaliz¬ ing from a single series is also appar¬ ent in the chapter referred to by Dr
Sunder. The authors of that report conclude that moyamoya disease is rare among non-Japanese. It is only in an addendum that reviews literature in 1969 and 1970, that Nishimoto and Takeuchi conclude that the disease may be found with some frequency outside of Japan as well. Non-Japa¬ nese cases reported in these more recent articles are not included in the figures quoted by Dr Sunder. Our own review of childhood moya-
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