Cerebral-perfusion-based single-photon emission computed tomography (SPECT) staging using NeuroGam® in patients with moyamoya disease.

BACKGROUND AND PURPOSE: Cerebral angiography (CA) is the gold standard for moyamoya disease (MMD) staging and diagnosis, but CA findings are not well correlated with clinical symptoms. The purpose of this study was to establish novel cerebral-perfusion-based staging for MMD that is well correlated with clinical symptoms. MATERIALS AND METHODS: From 2010 to 2015, regional cerebrovascular reserve (rCVR) was examined by single-photon emission computed tomography (SPECT) using NeuroGam® (Segamicorp, Houston, TX, USA) in 30 patients (17 women, 13 men; 60 hemispheres; mean 42.0 years old [range 5-60 years old]) with MMD, which was diagnosed by CA and magnetic resonance angiography (MRA). Brain CT or brain magnetic resonance imaging (MRI) was used to evaluate neurological conditions such as transient ischemic attack (TIA), cerebral hemorrhage, and cerebral infarction. A novel staging system for MMD was developed by combining findings from CA, MRI, and SPECT with NeuroGam®. RESULTS: Our novel staging system was strongly associated with clinical symptoms. Twenty-two hemispheres out of 60 were categorized as stage I, 24 hemispheres were categorized as stage II, and 14 hemispheres were categorized as stage III. Hemispheres with higher scores exhibited a higher incidence of clinical symptoms. These findings indicate that cerebral-perfusion-based staging is predictive of MMD clinical symptoms. CONCLUSION: Perfusion-based SPECT staging correlates well with clinical symptoms and may be a reliable alternative to the Suzuki staging by CA.

Intracerebral Hemorrhagic Infarction after Cranioplasty in a Patient with Sinking Skin Flap Syndrome.

The sinking skin flap syndrome (SSFS) is a rare complication after a large craniectomy. Hemorrhage infarction after a cranioplasty is a very rare complication with only 4 cases to date. We report a case of the patient who underwent an autologous cranioplasty to treat SSFS that developed intracerebral hemorrhage infarction. A 20-year-old male was admitted to our emergency department with stuporous mentality. Emergent decompressive craniectomy (DC) have done. He had suffered from SSFS and fever of unknown origin (FUO) since DC. After 7 months of craniectomy, cranioplasty was done. After 1 day of surgery, acute infarction with hemorrhagic transformation involved left cerebral hemisphere. We controlled increased intracranial pressure by using osmotic diuretics, steroid and antiepileptic drugs. After 14 day of surgery, he improved neurological symptoms and he had not any more hyperthermia. Among several complication of large cranioplasty only 4 cases of intracerebral hemorrhagic infarction due to reperfusion injury has been reported. In this case, unstable autoregulation system made brain hypoxic damage and then reperfusion and recanalization of cerebral vessels resulted in intracerebral hemorrhagic infarction. 7 month long FUO was resolved by cranioplasty.