ABSTRACT
Moyamoya disease (MMD) is a cerebrovascular disorder involving stenosis of brain vessels. Brain perfusion SPECT in MMD demonstrates impaired perfusion in ischemic areas. We present a 6-year-old boy with MMD. The patient had numbness on the right arm and simultaneous electroencephalography changes while studying arms up on the table. To differentiate seizure and ischemic symptoms, brain perfusion SPECT studies were obtained when the patient was asymptomatic (SPECT-A) and during the symptoms-EEG changes (SPECT-B). SPECT-A showed perfusion defect on the right frontal cortex, hypoperfusion on the right parieto-occipital region and slightly increased perfusion on the left parietal cortex. SPECT-B displayed significant hyperperfusion on the left parietal cortex; hypoperfusion on the right parietal, right temporal, right parieto-occipital and left frontal cortex. Additionally, brain perfusion SPECT of the child's younger brother diagnosed with MMD showed decreased regional cerebral perfusion. Physiopathological mechanisms of our patient's SPECT findings and indications of brain perfusion SPECT in MMD were also discussed.
Subject(s)
Cerebral Cortex/diagnostic imaging , Moyamoya Disease/diagnostic imaging , Perfusion Imaging , Seizures/diagnostic imaging , Tomography, Emission-Computed, Single-Photon , Acute Disease , Arm/blood supply , Brain Ischemia/diagnosis , Brain Ischemia/diagnostic imaging , Brain Ischemia/etiology , Carotid Arteries/physiopathology , Cerebral Arteries/diagnostic imaging , Cerebral Arteries/pathology , Cerebral Cortex/blood supply , Cerebral Cortex/physiopathology , Cerebrovascular Circulation , Child , Collateral Circulation , Craniotomy , Cysteine/analogs & derivatives , Delta Rhythm , Diagnosis, Differential , Electroencephalography , Humans , Hypesthesia/etiology , Moyamoya Disease/complications , Organotechnetium Compounds , Posture , Radiopharmaceuticals , Seizures/diagnosis , Tomography, Emission-Computed, Single-Photon/methodsABSTRACT
La enfermedad de moyamoya (EdM) es una alteración cerebrovascular que implica la estenosis de los vasos cerebrales. La SPECT de perfusión cerebral en la EdM demuestra alteración de la perfusión en las zonas isquémicas. Presentamos el caso de un niño de 6 años con EdM que presentaba adormecimiento en el brazo derecho y alteraciones en el EEG cuando estudiaba con los brazos sobre la mesa. Para diferenciar entre crisis epiléptica y síntomas isquémicos se efectuaron estudios cuando el paciente estaba asintomático (SPECT-A) y durante los síntomas con cambios en el EEG (SPECT-B). La SPECT-A mostró defecto de perfusión frontal derecho, hipoperfusión parieto-occipital derecha y leve incremento de la perfusión en la corteza parietal izquierda. La SPECT-B mostró hiperperfusión significativa en la corteza parietal izquierda e hipoperfusión en el parietal, temporal y región parieto-occipital derechos y corteza frontal izquierda. Además, la SPECT de perfusión cerebral de un hermano menor del niño diagnosticado de EdM mostró disminución de la perfusión cerebral regional. Se discuten los mecanismos fisiopatológicos de estos hallazgos y las indicaciones de la SPECT de perfusión cerebral en la EdM(AU)
Moyamoya disease (MMD) is a cerebrovascular disorder involving stenosis of brain vessels. Brain perfusion SPECT in MMD demonstrates impaired perfusion in ischemic areas. We present a 6-year-old boy with MMD. The patient had numbness on the right arm and simultaneous electroencephalography changes while studying arms up on the table. To differentiate seizure and ischemic symptoms, brain perfusion SPECT studies were obtained when the patient was asymptomatic (SPECT-A) and during the symptoms-EEG changes (SPECT-B). SPECT-A showed perfusion defect on the right frontal cortex, hypoperfusion on the right parieto-occipital region and slightly increased perfusion on the left parietal cortex. SPECT-B displayed significant hyperperfusion on the left parietal cortex; hypoperfusion on the right parietal, right temporal, right parieto-occipital and left frontal cortex. Additionally, brain perfusion SPECT of the child's younger brother diagnosed with MMD showed decreased regional cerebral perfusion. Physiopathological mechanisms of our patient's SPECT findings and indications of brain perfusion SPECT in MMD were also discussed(AU)
