TABLE 1.

Clinical and Radiologic Summary of 16 Patients With Moyamoya Syndrome and Associated Down Syndrome

Patient No.Age at Presentation, yGenderAssociated AbnormalitiesClinical PresentationTreatmentPostoperative Follow-up Period, moComplicationsClinical OutcomeModified Rankin Scale Score
15MSlipped R femoral head, VSD, B undescended testicles, repeated tonsillitis, repeated sinusitisR hemiparesis and speech difficulties with some recoveryStaged B pial synangiosis with B frontal burr holes plus ASA115NoneMild persistent R hand weakness2
219FHirschsprung’s disease, repeated conjunctivitis, repeated otitis externaR UE paresis with speech disturbance 1 y ago; recent L hemiparesisB pial synangiosis plus ASA128NoneImproved L LE strength but now wheelchair bound and totally dependent for ADL5
313MNoneL hemiparesis with almost complete recoveryStaged B pial synangiosis with B frontal burr holes104NoneMild persistent L hemiparesis2
46FAcute lymphocytic leukemia, AV canal, repeated pneumoniaL facial droop; 1 mo later, R facial droop, R hemiparesis, and decreased speech productionB pial synangiosis with B frontal burr holes plus infant ASA113L subacute SDH on POD 48Persistent R hemiparesis and speech difficulties3
514FHypothyroidism, NIDDM, repeated pneumonia, repeated urinary tract infectionsR hand weakness with speech disturbance; partial recoveryB pial synangiosis with B frontal burr holes plus infant ASA78NoneNeurologically intact0
62FAV canal, hydrocephalus, acute lymphocytic leukemia, tethered cordL hemiplegia with partial recovery; 1 wk later, R hemiplegia and R visual field deficitB pial synangiosis plus ASA68NonePersistent spastic quadriparesis; walks with gait trainer3
74FPDA, hyperthyroidism, duodenal atresiaR UE tremors; gait instabilityB pial synangiosis with L parietal burr hole plus ASA78L subacute SDH and seizure on POD 54Neurologically intact0
88MIntussusception, repeated otitis media, tonsillitis, and pneumoniaL hemiparesis with recovery; 1 y later, R hemiparesisStaged B pial synangiosis with L frontal burr hole and R parietal burr hole plus infant ASA146NoneNeurologically intact0
929FRepeated upper respiratory infectionsR hemiparesis and speech difficultiesStaged L STA-MCA bypass and then R pial synangiosis plus ASA42R hemiplegia with new L MCA territory infarction after direct bypassNeurologically intact0
105FASD, patent foramen ovaleL hemiparesis with complete resolution; 3 mo later, R hemiparesis; 1 mo later, L hemiparesisStaged B pial synangiosis plus ASA25NoneNeurologically intact0
117FPDA, ADHD, celiac diseaseR UE paresisStaged B pial synangiosis plus infant ASA23New-onset seizures 1 y postoperativelyNeurologically intact0
122MASD, VSDR hemiparesis with complete resolutionB pial synangiosis with B frontal burr holes plus infant ASA112NoneNeurologically intact0
1313MAV canalR focal seizures; 1 mo later, R hemiplegia, aphasia, and swallowing difficulties with gradual improvementR pial synangiosis plus ASA14New-onset R hemispheric seizures 5 mo postoperativelyMild persistent R hemiparesis and behavioral problems3
1412FAV canal, ureteral stenosis, repeated tonsillitisL hemiparesis with almost complete recoveryB pial synangiosis plus ASA21NoneMild persistent L hemiparesis2
153MPatent foramen ovale, repeated pneumonia and otitis mediaL hemiplegia with almost complete recoveryR pial synangiosis; L temporalis muscle and deep temporal artery synangiosis plus infant ASA9NoneImproved L-sided spasticity1
1615FAtlantoaxial instability, occipitocervical instability, hyperthyroidism, repeated otitis mediaR hemiplegia and speech difficulties; 2 mo later, R UE paresis and speech difficulties; 1 mo later, L hand weaknessB pial synangiosis plus ASA6NoneNANA
  • ASD indicates atrial septal defect; VSD, ventricular septal defect; ASA, aspirin; R, right; L, left; B, bilateral; UE, upper extremity; NA, not available; LE, lower extremity; AV, arteriovenous; SDH, subdural hematoma; POD, postoperative day; NIDDM, non–insulin-dependent diabetes mellitus; STA, superficial temporal artery; MCA, middle cerebral artery; PDA, patent ductus arteriosus; ADHD, attention-deficit/hyperactivity disorder.