No abstract available.
Adult ; Brain Ischemia/diagnosis/*etiology ; Cerebral Angiography ; Fatal Outcome ; Female ; Humans ; Hyperventilation/complications ; Moyamoya Disease/*complications/diagnosis/therapy ; Risk Factors ; Stroke/diagnosis/*etiology ; Thyroid Crisis/*complications/diagnosis/therapy

OBJECTIVETo study the clinical manifestations and neuroimaging characteristics of pediatric moyamoya disease.

METHODSThe clinical data of 17 children with moyamoya disease were retrospectively studied.

RESULTSThe onset age was between 3 and 14 years. The main clinical manifestations included motor weakness of extremities or hemiplegia, sensory disturbance and headache. Cranial CT or/and MRI examinations predominately showed cerebral infarct. Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) showed stenosis or occlusion at the terminus of the siphon portions of internal carotid arteries and proximal portions of anterior or middle cerebral arteries, and abnormal vascular networks at the base of brain.

CONCLUSIONSCerebral ischemia is main clinical manifestations in children with moyamoya disease, presenting motor weakness of extremities or hemiplegia, sensory disturbance and headache. DSA is essential to the diagnosis of the disease.

Adolescent ; Angiography, Digital Subtraction ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Moyamoya Disease ; complications ; diagnosis ; therapy ; Tomography, X-Ray Computed

It is well known that the electroencephalographic finding in patients with moyamoya disease demonstrates the characteristic "re-build up" phenomenon a few minutes after hyperventilation. To evaluate the usefulness of an electroencephalogram (EEG) in the postoperative management of children with moyamoya disease, we studied the presence or absence of improvement in the clinical, single photon emission computed tomography (SPECT) and EEG findings, before and after surgery. Twenty-two patients, who underwent indirect revascularization surgery for moyamoya disease, were included in our study. Clinical improvement was assessed as the disappearance or decrease of a transient ischemic attack or headache. The findings on the EEG and SPECT were considered improved when the re-build up phenomenon was absent and when there was improvement in the cerebrovascular reserve as a result of the acetazolamide challenge test. The statistical correlation analysis for both clinical and EEG improvement were consistent (kappa value=0.409, p< 0.05). However, the result from the clinical and SPECT improvement as well as that from EEG and SPECT improvement were not statistically significant. Our results suggest that EEG can be used as a noninvasive and simple follow-up test for moyamoya disease after indirect revascularization surgery if the hyperventilation procedure is effectively performed during EEG recording.
Tomography, Emission-Computed, Single-Photon/methods ; Postoperative Period ; Moyamoya Disease/*diagnosis/*therapy ; Models, Statistical ; Male ; Ischemic Attack, Transient/diagnosis ; Humans ; Headache/diagnosis ; Female ; Electroencephalography/*methods ; Child, Preschool ; Child ; Cerebral Revascularization ; Adolescent

We report here the case of a suprasellar cystic germ cell tumor (GCT) initially diagnosed as an arachnoid cyst. A 10-year-old boy experienced headache, dizziness, and diplopia, and was shown to have an approximately 2 cm suprasellar cyst. Two months after endoscopic third ventriculostomy was performed, a 5-6 cm cystic mass with an internal enhancing component was observed in the suprasellar cistern. Serum human chorionic gonadotropin levels were slightly increased in the serum and cerebrospinal fluid (55 and 162 IU/L, respectively) but were strikingly elevated in the cystic fluid (14,040 IU/L). The patient showed complete remission, with only a very small cystic lesion remaining after surgery, chemotherapy, and radiation treatment for a suprasellar mixed GCT. However, follow-up after treatment was complicated by moyamoya syndrome and cerebral infarction. GCT can be considered as a rare differential diagnosis in the case of a suprasellar cystic mass. Evaluation of tumor markers and close follow-up will be necessary.
Arachnoid* ; Central Nervous System Cysts* ; Cerebral Infarction ; Cerebrospinal Fluid ; Child ; Chorionic Gonadotropin ; Diagnosis, Differential ; Diplopia ; Dizziness ; Drug Therapy ; Follow-Up Studies ; Germ Cells* ; Headache ; Humans ; Male ; Moyamoya Disease ; Neoplasms, Germ Cell and Embryonal* ; Biomarkers, Tumor ; Ventriculostomy