Moyamoya disease as a cause of subarachnoid hemorrhage in a Negro patient Case report
ROBERT P. MERIWETHER, M.D., HUGH G. BARNETT, II, M.D., AND DEAN H. ECHOLS, M.D.
Departments of Neurosurgery, Tulane University and Alton Ochsner Medical Foundation, New Orleans, Louisiana w' The authors describe a case of subarachnoid hemorrhage secondary to moyamoya disease in an adult Negro. To the authors' knowledge, this is the first such case reported. Moyamoya, the Japanese word for "puff of smoke," describes the characteristic angiographic appearance of the collateral arterial network associated with internal carotid artery occlusion. The cause is unknown. Treatment consists of supportive care; the prognosis is variable. KEY WORDS subarachnoid hemorrhage 9 moyamoya disease occlusion of internal carotid artery 9 collateral circulation 9
.UMEROUS reports during the last 15 years have described a syndrome of bilateral intracranial internal carotid artery occlusion associated with extensive intracranial collateral circulation. This was first described in detail in 1961 by Takeuchi; 7 in 1969 it was given the name "moyamoya," meaning "puff of smoke," by Suzuki and Takaku. 6 This term describes the angiographic appearance of the collateral arterial network that is characteristic of the disease. Moyamoya disease was first recognized in Japanese people, 1,~,4,8,7 but as noted by Lee and Cheung 2 in 1973, there have been several recent reports of its occurrence in Caucasians and Negroes. We are presenting a case of spontaneous subarachnoid hemorrhage
(SAH) occurring in a Negro man as a manifestation of moyamoya disease. To the best of our knowledge this is the first such reported case. Case Report A 50-year-old Negro man was admitted to the hospital on November 15, 1974, in a semicomatose condition. He had pin-point pupils, hyperactive reflexes in his lower extremities, and bilateral positive Babinski reflexes. Two days later neck stiffness was noticed and a lumbar puncture revealed grossly bloody spinal fluid. The patient was transferred to our neurosurgical service on November 22, 1974. J. Neurosurg. / Volume 44 / May, 1976
Moyamoya disease Examination. His mental status had improved to the degree that he could follow simple commands; however, he still had a stiff neck and a lateral deviation of the right eye. He was able to move all four extremities purposefully, but gait was not tested. There was no papilledema. Babinski reflexes were no longer present. Red blood cell count and erythrocyte sedimentation rates were within normal limits. Transfemoral three-vessel angiograms on November 26 (Figs. 1 and 2) revealed bilateral occlusion of the intracranial internal carotid arteries with an extensive collateral arterial network at the base of the brain. Retrograde filling of the pericallosal artery was detected upon injection of the right carotid artery (Fig. 2). Transdural anastomotic communications, that is, rete mirabile (Fig. 2 right) were most prominent in the right parietooccipital area. Both carotid injections revealed prominent meningeal vessels and ophthalmic arteries. Course. The patient's mental state gradually improved. He was not aphasic, and he walked with minimum assistance. Fourteen days later a second lumbar puncture revealed clear spinal fluid under normal pressure. A second angiogram on December 10 showed no change. The patient was subsequently discharged with a diagnosis of SAH secondary to moyamoya disease. He had no focal neurological deficit and was able to care
Fro. 1. Angiogram, left carotid injection, showing characteristic "puff of smoke" appearance created by anastomotic network in region of basal ganglia. Note prominent meningeal vessels and ophthalmic artery.
for himself with minimum supervision. No follow-up history was obtained. Discussion
Patients with the syndrome of moyamoya disease can be divided into two groups based
Fro. 2. Angiogram, right carotid injection. Left: Early phase. Right: Late arterial phase of right carotid injection demonstrating retrograde filling of pericallosal artery and prominent rete mirabile.
J. Neurosurg. / Volume 44 / May, 1976
R. P. Meriwether, H. G. Barnett, II and D. H. Echols upon their age. In patients younger than 20 years of age, which includes the majority of cases, the classic symptoms are weakness of one or more extremities, transient or permanent visual disturbances, or some form of seizure activity. In patients over 20 years of age the most common initial manifestation is SAH. The disease is slightly more common in women? ,4 The sine qua non of moyamoya disease is its angiographic appearance, including partial or complete occlusion of intracranial portions of both internal carotid arteries with extensive collateral circulation. Taveras 8 reported three types of collateral vessels including "end-to-end anastomoses on the surface of the cerebral hemisphere," as described by Vander Eecken,9 an extensive collateral network in the region of the basal ganglia, and transdural anastomoses between the external carotid circulation and internal carotid circulation, the classic rete mirabile. The term moyamoya describes the appearance of these collateral vessels in the region of the basal ganglia. The anterior and middle cerebral arteries are not usually visualized, but some major vessels may fill in retrograde fashion from the posterior circulation. This was the case with the pericallosal artery of our patient. The cause of moyamoya disease remains a mystery. Autopsy reports have been rare and inconclusive. Yada, et al., I~ suggested a genetic origin, and reported a long Y-chromosome in their patients. However, Nishimoto and Takeuchi'P report of 96 cases did not support Yada's findings but did seem to indicate some familial predisposition. An arteritic process was considered by Lee and Cheung~ but was rejected when their patients demonstrated normal blood counts, erythrocyte sedimentation rates, and autoimmune studies. Although it has been considered,5'8 infection has never been implicated as a causative agent. Treatment for moyamoya consists solely of supportive care, and anticoagulant therapy is
contraindicated. The prognosis for patients with the disease is varied. Suzuki and Takaku 8 have studied the progression of the disease in a young patient by repeated angiograms. The partial occlusion of the internal carotid arteries was reported to progress to completeness in a matter of months. Clinically, younger patients may experience periods of remission mingled with episodes of alternating hemiplegia, recurrent weakness, or seizure activity. Patients with SAH as the initial manifestation of moyamoya disease usually undergo slow partial recovery. References 1. Kudo T: Cited in ref. 2 2. Lee ML, Cheung EM: Moyamoya disease as a cause of subarachnoid hemorrhage in Chinese. Brain 96:623-628, 1973 3. Leeds NE, Abbott KH: Collateral circulation in cerebrovascular disease in childhood via rete mirabile and perforating branches of anterior choroidal and posterior cerebral arteries. Radiology 85:628-634, 1965 4. Nishimoto A, Takeuchi S: Abnormal cerebrovascular network related to the internal carotid arteries. J Neurosurg 29:255-260, 1968 5. Shillito J Jr: Carotid arteritis: a cause of hemiplegia in childhood. J Neurosurg 21:540-551, 1964 6. Suzuki J, Takaku A: Cerebrovascular "moyamoya" disease. Disease showing abnormal net-like vessels in base of brain. Arch Neuroi 20:288-299, 1969 7. Takeuchi K: Cited in ref. 2 8. Taveras JM: Multiple progressive intracranial arterial occlusions: A syndrome of children and young adults. Am J Roentgenol Radium Ther Nucl Med 106:235-268, 1969 9. Vander Eecken HM: Cited in ref. 8 10. Yada K, Suwa T, Ito N, et al: Cited in ref. 4
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J. Neurosurg. / Volume 44 / May, 1976