Patients with moyamoya disease often complain of headache before surgery, after surgery, or in both periods, and the symptom is one of the major complaints in moyamoya disease.
Preoperative headache was considered to be related to hypoperfusion because it has been reported that headaches could disappear after revascularization surgery [5] . Such phenome- non is especially documented after indirect pial synangiosis for pediatric moyamoya disease.
It was considered to be a sort of migraine or to be derived from hemodynamic changes, while the exact cause was undetermined [6] . Seol and colleagues reviewed 204 consecutive surgi- cal cases with pediatric moyamoya disease, and found that preoperative headache was pres- ent in 21.6% (44 of 204) of the patients and was one of their major symptoms [7] . Postoperative headache was seen in 63% (28 of 44) of the patients with preoperative head- ache, and in 6.3% (10 of 160) of those without preoperative headache. Notably, the aggrava- tion on postoperative MR imaging or single-photon emission computed tomography scan did not correlate with this symptom, suggesting that progressive recruitment and redistribution
Table 1 Onset-type of 962 patients with moyamoya disease
Onset-type Number of patients
Transient ischemic attack (TIA) 353 (37%)
Cresiendo TIA 63 (7%)
Cerebral infarction 165 (17%)
Intracranial hemorrhage 186 (19%)
Headache 57 (6%)
Seizure 29 (3%)
Asymptomatic 32 (3%)
Others 13 (1%)
Unclear 64 (7%)
112 R. Shirane and M. Fujimura
of blood flow, as well as the hemodynamic compromise, could be the cause of headaches in patients with moyamoya disease [7] . The author performed direct revascularization surgery (superficial temporal artery–middle cerebral artery anastomosis) for 13 patients with signifi- cant headache with apparent hemodynamic compromise. As a result, 11 of 13 (84.6%) patients showed marked improvement of their symptom after surgery. A 14-year-old female was admitted to our clinic suffering from significant headache and weakness on her left lower extremity 6 years after successful direct/indirect revascularization during her child- hood. Medication was not effective for her headache, and single-photon emission computed tomography scan demonstrated decreased cerebral blood flow at the vascular territory of right anterior cerebral artery (Fig. 1 ), while direct/indirect revascularization was well visual- ized by MR angiography (Fig. 2 ). Then she underwent right occipital artery–posterior cere- bral artery anastomosis as the salvage surgery [8] . Her weakness and headache completely disappeared after surgery, indicating that direct revascularization surgery improved her cerebral hemodynamics and could thereby relieve headache. In light of the hypothesis that progressive recruitment of indirect pial synangiosis stimulates trigeminal nerve and thus causes headache, direct revascularization surgery may have more potential benefit for improving headache by rapid improvement of cerebral blood flow and ameliorating the demand for the development of pial synangiosis.
Conclusion
Headache is one of the common clinical presentations of moyamoya disease. Patients with moyamoya disease may complain of headache before and after revascularization surgery.
Although the underlying mechanism and optimal treatment of such headache are undetermined, the decreased cerebral blood flow, and the progressive recruitment and redistribution of blood flow are thought to be a cause of headache in patients with moyamoya disease.
The revascularization surgery, direct revascularization surgery in particular, may have potential benefit for improving headache by relieving the hemodynamic compromise.
Fig. 1 N -isopropyl- p -[ 123 I] iodoamphetamine single-photon emission computed tomography in a 14-year-old female presenting with significant headache and weakness on her left lower extremity, indicating her cerebral blood flow at the vascular territory of right anterior cerebral artery was markedly compromised
113 Headache in Moyamoya Disease
References
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2. Oki K, Suzuki N (2007) In: Hashimoto N (ed) Report by the Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) pp. 4–5
3. Yoshida Y, Yoshimoto T, Shirane R et al. (1999) Clinical course, surgical management, and long-term outcome of moyamoya patients with rebleeding after an episode of intracerebral hemorrhage: an extensive follow-up study. Stroke 30:2272–2276
4. Kuroda S, Hashimoto N, Yoshimoto T et al. (2007) Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease. Results of multi-center survey in Japan. Stroke 38:1430–1435 5. Matsushima Y, Aoyagi M, Niimi Y et al. (1990) Symptoms and their pattern of progression in
childhood moyamoya disease. Brain Dev 12:784–789
6. Scott RM, Smith JL, Robertson RL et al. (2004) Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg 100(2 Suppl Pediatrics):142–149 7. Seol HJ, Wang KC, Kim SK et al. (2005) Headache in pediatric moyamoya disease: review of 204
consecutive cases. J Neurosurg 103(5 Suppl Pediatrics):439–442
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Fig. 2 A 14-year-old female presenting with significant headache and weakness on her left lower extremity. Magnetic resonance (MR) angiography, 6 years after direct/indirect revascularization surgery for ischemic-onset moyamoya disease, indicates that both direct and indirect anastomosis were well demonstrated
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Introduction
Moyamoya disease is an idiopathic cerebrovascular disease characterized by progressive steno-occlusion of the arteries of the circle of Willis, accompanied by collateral vessel formation in the basal ganglia [1, 2] . Involuntary movements are relatively rare symptoms of this condition, and their frequency is estimated to range from 3 to 6% [3– 5] . However, the incidence could be higher, if limb shaking, a specific type of transient ischemic attack (TIA), is also included. In this chapter, we focus on moyamoya disease-induced involuntary movements, and the patient characteristics, symptoms, underlying mechanisms, and treatment of this condition are discussed.