Moyamoya disease concurrent with Graves' disease treated by direct bypass: clinical features and treatment strategies.
BACKGROUND
Moyamoya disease (MMD) concurrent with Graves' disease (GD) is rare. There is no guideline about optimizing thyroid hormones and the appropriate timing of surgical treatment for MMD with GD.


METHODS
We encountered eight patients with MMD and GD presenting with cerebral ischemia who were treated by direct bypass. Thyroid hormones [free thyroxin (fT4) and free triiodothyronine (fT3)], thyroid-stimulating hormone (TSH), and TSH receptor antibody (TRAb) were measured sequentially. After thyrotoxic conditions were medically optimized, revascularization surgery was performed by superficial temporal artery-middle cerebral artery (STA-MCA) double bypass in all cases. Clinical outcomes were estimated by modified Rankin scale (mRS) at discharge and 3 months after surgery.


RESULTS
In six patients with thyrotoxicosis, the fT4, fT3, and TRAb (range) at the onset of cerebral ischemia were 4.81-10.30 pg/ml, 13.08-31.90 pg/ml, and 3.5-83.8 IU/l, respectively. At surgery, mean (range) fT3 and fT4 were optimized to 3.02 (1.01-4.87) pg/ml and 1.09 (0.41-1.68) ng/dl, respectively. In the thyrotoxic cases, it took 70-310 days (mean, 142 days) to optimize thyroid hormones before surgery. There was no neurological aggravation after surgery, and outcome was excellent at 3 months with mRS scores ≤2 in all cases.


CONCLUSIONS
For MMD concurrent with GD, optimizing thyroid hormones followed by STA-MCA double bypass was successful to prevent cerebral ischemic events.
Department of Neurosurgery, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.
Bikei
B
Ryu
Takakazu
T
Kawamata
Koji
K
Yamaguchi
Akitsugu
A
Kawashima
Masami
M
Ono
Yoshikazu
Y
Okada
D000328:Adult; D002548:Cerebral Revascularization; D005260:Female; D006111:Graves Disease / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D020768:Middle Cerebral Artery / Q000601:surgery; D009072:Moyamoya Disease / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D013699:Temporal Arteries / Q000601:surgery; D013963:Thyroid Hormones / Q000097:blood
D013963:Thyroid Hormones