Moyamoya Disease in Black Adults Phinehas Z. Makoyo, MD, PhD Nashville, Tennessee

A 40-year-old hypertensive black female, who suddenly developed aphasia, lethargy, and a right hemiparesis, and a 42-year-old nonhypertensive black male, who suddenly developed intractable headache, drowsiness, and vomiting, were found by angiography to have moyamoya disease. This condition is characterized by a decreased caliber of the internal carotid arteries and bilateral occlusion of the anterior and middle cerebral arteries with visualization of an extensive collateral network of tortuous blood vessels of the rete mirabile type at the base of the brain.

Introduction Moyamoya disease was first described in the Japanese literature between 1956 and 1963, and later appeared in Western literature. 1-4 The first cases reported were all in Japanese patients residing in Japan or Japanese-Americans residing in the United States,5 but the condition has now been reported in non-Japanese-Americans.6'7 It has also been described in European patients.4'8-'0 More recently the condition has been discovered in British patients"1 and Dumas et alt2 have reported occurrences in 42 black children. Although this disease was originally thought to be limited to Oriental patients, the author will present what to his knowledge are the first two cases of moyamoya disease in adult blacks. Both patients were seen in a community hospital during a five-year period.

Case Reports Case 1 A 40-year-old black female (known to be a hypertensive for many years), who had been on a program of weight

From the Department of Surgery, Meharry Medical College, Nashville, Tennessee. Requests for reprints should be addressed to Dr. Phinehas Z. Makoyo, Department of Surgery, Meharry Medical College, Nashville, TN 37208.

reduction, salt restriction, and antihypertensive medication (methyldopa 250 mg three times a day), was admitted to Stanford Hospital on January 30, 1975. One month prior to admission, her blood pressure was recorded at 130/80 mmHg, with a pulse rate of 72/min; weight was measured at 72 kg and height was 173 cm. On the day of admission, while sitting in a beauty parlor, she suddenly developed numbness of the right side of her face, followed by rightsided weakness associated with loss of speech and lethargy. Physical examination at the time of admnission disclosed a temperature of 98.6F, a regular pulse of 80/min, respirations of 18/min, and blood pressure of 160/100 mmHg. She was a well developed, well nourished black female who was lethargic and aphasic, but was able to follow simple commands. Fundi and fields were normal and the pupils were equal, regular, and reactive to light. There was a right central facial paralysis, a right hemiparesis, and hemisensory syndrome. There was a Babinski sign present on the right. No bruits were heard in the head or neck, and the carotid pulses were equal and strong bilaterally. On the third hospital day a loud grade 3/4 bruit was heard on the right side of the neck beneath the angle of the jaw. Lumbar puncture revealed the following values: RBC, 1,056/cu mm; 6% polymorphonuclear leukocytes; 2% lymphocytes; and a protein of 51 mg/100 ml. The electroencephalogram

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979

showed a slow wave focus in the posterior portion of the left cerebral hemisphere; the brain scan on the fifth hospital day showed increased uptake in the left posterior parietal region. An aortic arch and bilateral carotid angiography was performed (Figures 1 and 2). There was bilateral decreased caliber of internal carotid artery at the level of siphons, left more than right. There was also bilateral occlusion of the middle cerebral arteries at their origin from the supraclinoid internal carotid arteries, with marked increase of both lenticulostriate groups and hypertrophy of the middle meningeal branches of the external carotid system bilaterally. The right posterior cerebral artery originated from the right carotid artery. The left posterior cerebral artery originated from the basilar artery. There was a mass of perforating vessels in the base of the brain. Collaterals had formed between the internal carotid system and branches of the external carotid, including the ophthalmic artery and the middle meningeal artery. The greatest amount of contrast medium was localized in a nonhomogenous hazy formation between the anterior and middle cerebral arteries, which consisted of a collection of tortuous and disorganized small blood vessels. Her hospital course was uneventful and her aphasia cleared rapidly. The right-sided weakness gradually improved with an intensive rehabilitation program. Eleven months later, she had 479

.

Figure 1. Case 1. Right carotid arteriogram (lateral view). Attenuation of internal carotid artery at the level of siphon with occlusion in the supraclinoid segment. Prominent lenticulostriate arteries and collateral arterial flow through external carotid and posterior cerebral collateral arterial channels

only limited use of the right arm, but could walk unassisted.

Case 2 A 42-year-old non-hypertensive black male was admitted to Stanford Hospital on August 29, 1972, with intractable headache associated with drowsiness and vomiting of several hours duration. Physical examination at the time of admission showed a rectal temperature of 98.6 F, a regular pulse of 96/min, respirations of 20/min, and blood pressure of 210/96 mmHg. He was a well developed, well nourished black male in no acute distress. Fundi and fields were normal and the pupils were equal, 480

regular, and reactive to light. Nuchal rigidity was measured at 3 + but no focal neurological deficit was found. The possibility of a ruptured intracranial aneurysm was entertained. Lumbar puncture revealed the following values: RBC, 455,000/cu mm; WBC, 543/cu mm; 92% polymorphonuclear leukocytes; 8% lymphocytes; and a protein of 300 mg/100 ml. The EEG showed diffuse slowing of the basic frequency which was dominated by theta rhythms without evidence of focal findings; an echogram revealed no significant shift of midline. Bilateral carotid angiography was performed (Figures 3 and 4). The findings were similar to those described in Case 1 and noted in Figures 1 and 2. The patient's hospital course was one of continued improvement. He was discharged without neurologic deficit,

free of headache and fully ambulatory. He died three years later at another institution; necrospy findings were not specifi'c to cerebral vessels.

Comment The first patient presented with a classical picture of cerebrovascular accident in the distribution of the left middle cerebral artery. The second patient presented with subarachnoid hemorrhage that angiographically appeared identical to the vascular disorder described by Japanese authors under the term moyamoya disease. Suzuki and Takaku 13 first named this disorder "moyamoya" -the Japanese word for something hazy, like a

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979

Figure 2. Case 2. Left carotid arteriogram (lateral view). More pronounced attenuation of internal carotid artery in the siphon portion, and supraclinoid occlusion with reconstitution of anterior cerebral artery via collaterals. The lenticulostriate arteries are prominent just cephalad to dorsum sellae

Figure 3. Case 2. Left carotid arteriogram (lateral view). Supraclinoid internal carotid artery occlusion with changes similar to Case 1, and prominent external carotid collaterals JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979

-J 481

Cse Rghtcartidarteriogram (lateral view). Note changes similar to Figre .

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puff of smoke-referring to the appearance of the dilated torturous vessels at the base of the brain on angiography (Figures 1 and 2). Hoare and Keogh'" noted that the clinical course in those patients whose first symptoms occur in childhood is different from those in whom symptoms developed in adult life. The dominant symptomatology in children is weakness, which is usually of sudden onset with rapid recovery and frequent recurrence. Speech disturbance, seizures, headaches, and intellectual deterioration are also common. In adults, the predominant symptom is headache followed by disturbance of consciousness of speech disorders (Table 1).1-4.6,7,10-16 Examination often shows a hemiparesis or, less commonly, monoplegia. Cranial nerve palsies and disturbance of gait and speech are found. Subarachnoid 482

hemorrhage, although uncommon in children, is the most common finding in adults. "I Lumbar puncture is normal in cases that do not have subarachnoid hemorrhage. Electroencephalographic appearances are usually abnormal but non-specific.4,12,13 Four vessel angiography provides the diagnosis. The carotid arteries are narrowed, suggesting longstanding or congenital disease. There is occlusion of the internal carotid arteries above the level of the siphon, occlusion of the middle and anterior cerebral arteries, and, sometimes, occlusion of the posterior cerebral arteries. The cerebral circulation is maintained by collaterals. A mass of perforating vessels appears in the base of the brain. Further anastomosis occurs between the internal and external carotid system via leptomeningeal ves-

sels and rete mirabile. The rete mirabile is a tightly packed network of arterial vessels joining the internal and external carotid system directly across the subdural space. Galligioni et al'0 noted that a real and true rete mirabile is present in lower animals such as cats, goats, sheep, and pigs. Patient 1 presented with a stroke and a history of hypertension under good control. Out of 20 cases reported by Suzuki,13 eight had a strong family history of hypertension. There is no other mention in the literature as to the coexistence of moyamoya disease and hypertension. Case 2 presented with classical subarachnoid hemorrhage. The literature indicates that adults with moyamoya usually present with subarachnoid hemorrhage rather than a stroke syndrome. However, Suzuki'3 and Hoare and Keogh" had two adult

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979

Table 1. Cases of Moyamoya Disease Reported in the Literature

Date of Report

Authors of Series

Number of Patients

Children

1965-1966 1968

Kudo1 2 Nishimoto and Takeuchi3

146

146

96

73

1969

1969

Taveras6 Suzuki and

10

10

20

12

1 86 4

86

Adults

23

8

Takaku13

1970 1970 1971 1972 1973 1973 1974 1974 1979

Urbanek4 Solomon7 Galligioni'0 Picard14 Dumas12 LaMarec15 Rosengren16 Hoare"1 Makoyo*

1 42 3 1 2 2

1

1

3

42 3 1

1

-

2 2

*This report

patients with stroke syndrome; the author saw one such adult case. There is no known effective treatment for moyamoya disease. Several attempts have been made using cerebral vasodilators with apparent improvement; however, long-term prognosis is unknown.10 Most patients appear to deteriorate initially and then remain static.' 2'fi6'0 In Nishimoto's and Takeuchi's series,3 66 out of 73 patients had recurring hemiplegia without deterioration in the pediatric age group. None of the 23 adults deteriorated. The etiology of moyamoya disease is unknown. There is no reported evidence of atherosclerosis, autoimmune disease, temporal arteritis, cerebral embolism, or coagulopathies in cases studied.6 Most investigators believe that it is a progressive arterial disease. '"3 The cause of the arterial disease is unknown. Others believe that it is congenital.4'9"10 In some series3 cases have been reported in siblings. Others"'"2 maintain that the disease has multifactorial etiology and is not a single disease entity, but instead is a group of diseases in which the normal collateral circulation via circle of Willis

is occluded. Therefore, other collaterals develop, and their appearance is the same regardless of the etiology of the occlusion. Both Chang et al'7 and Poor and Gacs'8 have reported an association between bilateral dissecting aneurysms of the carotid arteries and moyamoya disease in three patients.

Literature Cited 1. Kudo T: Juvenile occlusion of circle of Willis. Clini Neurol 5:607-627, 1965 2. Kudo T: Spontaneous occlusion of the circle of Willis: A disease apparently confined to Japanese. Neurology 18:485-496, 1968 3. Nishimoto A, Takeuchi S: Abnormal cerebrovascular network related to the internal carotid arteries. J Neurosurg 29:255-260, 1968 4. Urbanek K, Farkova H, Klaus E: Nishimoto-Takeuchi-Kudo disease: Case report. J Neurol Neurosurg Psychiatry 33:671673, 1970 5. 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 6. Taveras JM: Multiple progressive intracranial arterial occlusions: A syndrome of

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 71, NO. 5, 1979

children and young adults. Am J Roentgenol Radium Ther Nucl Med 106:234-268, 1969 7. Solomon GE, Hilal SK, Gold AP, et al: Natural history of acute hemiplegia of childhood. Brain 93:107-120, 1970 8. Simon J, Sabouraud 0, Guy G, et al: Un cas de maladie de Nishimoto: A propos d'une maladie rare et bilaterale de la carotide interne. Rev Neurol 119:376-383, 1968 9. Lepoire J, Tridon P, Montaut J, et al: Angiomes cerebraux: Malformations angiomateuse arterio-arterielles du systeme carotidien. Neurochirurgie 15:5-18, 1969 10. Galligioni F, Andrioli GC, Marin G, et al: Hypoplasia of the internal carotid artery associated with cerebral pseudoangiomatosis: Report of four cases. Am J Roentgenol 112:251-262, 1971 11. Hoare AM, Keogh AJ: Cerebrovascular moyamoya disease. Br Med J 1:430-432, 1974 12. Dumas J, Girard PD, Konate S, et al: Occlusions carotido-sylviennes et moyamoya chez 1'enfant noir. Bull Soc Med Afr Noire Lang Fr 1:67-71, 1973 13. Suzuki J, Takaku A: Cerebrovascular "moyamoya" disease: Disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288-299, 1969 14. Picard L, Andre JM, Weber M, et al: Stenoses multiples des arteres cerebrales de moyen calibre et reseaux capillaries anormaux (moyamoya): Problemes nosologiques. Sem Hop Paris 48(28):2049-2055, 1972 15. LeMarec B, Henry JF, Signargout J, et al: Le moyamoya ou maladie de Nishimoto: A propos de trois cas. Ndiatrie 2:133-148, 1973 16. Rosengren K: Moyamoya vesselscollateral arteries of the basal gangliamalignant occlusion of the anterior cerebral arteries. Acta Radiol [Diagn] 15:145-151, 1974 17. Chang V, Rewcastle NB, Harwood-Nash DCF, et al: Bilateral dissecting aneurysms of the intracranial carotid arteries in an 8-year-old boy. Neurology 25:573-579, 1975 18. Poor G, Gacs G: The so-called moyamoya disease. J Neurol Neurosurg Psychiatry 37:370-377, 1974

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Moyamoya disease in black adults.

Moyamoya Disease in Black Adults Phinehas Z. Makoyo, MD, PhD Nashville, Tennessee A 40-year-old hypertensive black female, who suddenly developed aph...
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