er, is

Intracranial Hemorrhage With Vasculitis in Rheumatoid Arthritis

To the Editor.\p=m-\Edwardshas described patients with intracranial hemorrhage occurring with cerebral arteritis (Arch Neurol 34:549-555,1977). One of these cases was a subarachnoid two

hemorrhage secondary to amphetamine-induced necrotizing angitis. The

second was an intracerebral hematoma in association with intracranial arteritis and anticoagulant therapy in a patient with ulcerative colitis. They pointed out that intracranial hemorrhage with cerebral vasculitis is not well recognized. We published a case in the context of CNS vasculitis in rheumatoid disease,1 itself a rare entity in the literature. The hemorrhagic aspect of the vasculitis was not considered a remarkable feature in that report. In our case, four separate areas of the brain were involved by hemorrhage at at least two different times. The symmetry is remarkable. A review of the eight cases of CNS vasculitis in rheumatoid arthritis1-7 shows that in only one of these did hemorrhage occur.5 In this instance (case 2), the bleeding was associated with purulent meningitis and brain abscess. Our patient, then, is unique not only with regard to the myelopathy second¬ ary to vasculitis, but also in regard to the singular occurrence of hemor¬ rhage in frontal lobes arid pons asso¬ ciated only with vasculitis and no other predisposing factor, in the setting of rheumatoid disease. The association of vasculitis and hemor¬ rhage, however, does lend support to Edwards' observation of this concur¬ rence.

Peter

Watson, MD, FRCP(C)

Dept of Neurol Toronto Western

RL, Smyth CJ, Holt GW: Steroid and vascular lesions in rheumatoid arthritis. Arthritis Rheum 2:224-249, 1959. 5. Steiner JW, Gellbloom AJ: Intracranial manifestations in two cases of systemic rheumatoid disease. Arthritis Rheum 2:537-545, 1959. 6. Ouyang R, Michell DM, Rozdilsky B: Central nervous system involvement in rheumatoid disease: Report of a case. Neurology 17:1099-1105, 1967. 7. Ramos M, Mandybur TI: Cerebral vasculitis in rheumatoid arthritis. Arch Neurol 32:271-275, 1975. 4. Johnson

therapy

Cerebral Bone Marrow Embolus After Closed Chest Cardiac

Massage To the Editor.\p=m-\Thecomplications of closed chest cardiac massage (CCCM) have included pneumothorax, hemothorax, cardiac contusion, and pulmonary bone marrow-fat emboli. We report a case of bone marrow embolus in a cerebral artery after CCCM.

Report of a Case.\p=m-\A77-year-old man was admitted to the hospital because of progressive confusion, weakness, and an 18-kg weight loss. Examination showed signs of dehydration, cachexia, and confusion. Chest roentgenogram showed severe diffuse interstitial nodular pulmonary disease. Cardiopulmonary arrest occurred 17 hours after admission. Closed chest cardiac massage, intubation, and defibrillation were performed. Several intracardiac adrenaline injections were administered. Necropsy disclosed widespread miliary tuberculosis. A bone marrow embolus was found lodged in a cerebral artery (Figure).

Comment.\p=m-\The incidence of pulmonary bone marrow and fat emboli after CCCM is quite high.1 Bone marrow embolus of the brain, howevCerebral bone marrow embolus after closed chest cardiac massage.

Hospital

Toronto, Ontario, Canada M5T 2S8 1. Watson P, Fekete J, Deck J: Central nervous system vasculitis in rheumatoid arthritis. Can J

Neurol Sci 4:269-272, 1977. 2. Pirani CL, Bennett GA: Rheumatoid arthritis: A report of three cases progressing from childhood and emphasizing certain systemic manifestations. Bull Hosp Joint Dis 12:335-367, 1951. 3. Sokoloff L, Bunim JJ: Vascular lesions in rheumatoid arthritis. J Chronic Dis 5:668-687, 1957.

Downloaded From: http://archneur.jamanetwork.com/ by a Oakland University User on 06/02/2015

an extraordinary event. Of the four reported cases, one had a patent foramen ovale,- and the other three had no evidence of a right to left shunt.35 Two had severe osteoporosis with pathologic fractures; one had received intrathoracic cardiac mas¬ sage; and the fourth had probable vertebral fractures secondary to te¬ tanic-like muscular contractions asso¬ ciated with anaphylactic shock. Our patient had no evidence of a patent foramen ovale, cardiac septal defect, or pulmonary arteriovenous malfor¬ mation. The source of embolus in our case is clearly related to the ribs frac¬ tured during resuscitation. It must, therefore, be assumed that there was sufficient pulmonary vascular pres¬ sure to carry a small marrow embolus through the pulmonary capillaries to the heart. A speculative explanation for this would be the possible exis¬ tence of pulmonary precapillary anas¬

tomoses

functioning as a true venoar-

terial shunt durine shock. Uros Roessmann, MD Institute of Pathol Case Western Reserve Univ 2085 Adelbert Rd Cleveland, OH 44106 Lawrence E. Zarchin, MD Neurological Associates, PC 8301 Arlington Blvd Fairfax, VA 22031 1. Yanoff M: Incidence of bone marrow embolism due to closed chest cardiac massage. N Engl J Med 269:837-839, 1963. 2. Ghatak NR, Zimmerman HM: Cerebral bone marrow embolism. Arch Pathol 92:112-118, 1971. 3. Karlen A: Todesfall an Fett-knochemmar-$ kembolie und ur\l=a"\mienach "intraduraler" perabrodil myelographie. Acta Chir Scand 87:497\x=req-\ 512, 1942. 4. Rappaport H, Raum M, Horrell JB: Bone marrow embolism. Am J Pathol 27:407-433, 1951. 5. Pyun KS, Katzenstein RE: Widespread bone marrow embolism with myocardial involvement. Arch Pathol 89:378-381, 1970.

Childhood Moyamoya Presenting Subarachnoid Hemorrhage

as

To the Editor.\p=m-\Moyamoyawas originally thought to represent the roentgenographic expression of a congenital cerebrovascular anomaly indigenous to the Japanese.1 Subsequently, this syndrome has been reported as an acquired process in non-Japanese

adults and children.2-6 A recent review4 of moyamoya in children has documented more than 100 non-Japanese cases. I have cared for two children in whom the presentation of moyamoya again demonstrates its acquired nature and in whom the mode of presentation has rarely been noted in children.

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

Downloaded From: http://archneur.jamanetwork.com/ by a Oakland University User on 06/02/2015

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