Hime Suzuki

Pathophysiological consideration of medullary streaks on FLAIR imaging in pediatric moyamoya disease

OBJECTIVE Medullary streaks detected on fluid-attenuated inversion recovery (FLAIR) imaging have been considered to be reflected ischemic regions in pediatric moyamoya disease. The purpose of this study was to evaluate these medullary streaks both clinically and radiologically and to discuss associated pathophysiological concerns. METHODS The authors retrospectively reviewed data from 14 consecutive pediatric patients with moyamoya disease treated between April 2009 and June 2016. Clinical and radiological features and postoperative imaging changes were analyzed. In 4 patients, hyperintense medullary streaks on FLAIR imaging (HMSF) at the level of the centrum semiovale were detected. RESULTS The HMSF were coincident with hyperintense medullary streaks on a T2-weighted image, though they were not completely coincident with the vasculature on either a T2*-weighted image or contrast-enhanced CT. Analysis revealed significantly higher values in terms of MR angiography scores, number of flow voids of the basal ganglia, and the presence of the medullary artery in the group with HMSF than in those without. In contrast, the presence of white matter damage was significantly less frequent in the HMSF group. All HMSF disappeared after surgery, and the mean apparent diffusion coefficient at the same level was significantly reduced postoperatively. CONCLUSIONS Although HMSF should be associated with collateral circulation in moyamoya disease, other factors may be involved, including stagnated cerebrospinal fluid or vasogenic edema that is relevant to the impaired state of the white matter. Findings in this study provide insight into the pathophysiological basis of the perivascular space in moyamoya disease.

Germinoma Mimicking Tumefactive Demyelinating Disease in Pediatric Patients

Cases of intracranial germinoma with granulomatous reaction are rare, so a pathological diagnosis of this disease is difficult. In this report, we describe the case of a 13-year-old boy with a bilateral thalamic germinoma which initially mimicked tumefactive demyelinating disease with inflammation, based on the clinical symptoms, imaging results and histology of a biopsy specimen obtained endoscopically. Upon examination of the cerebrospinal fluid, oligoclonal bands were detected. Although his symptoms and radiological findings improved following steroid pulse treatment, they worsened dramatically almost 1 year after the first surgery and even after an additional steroid pulse treatment. Prompted by the clinical course, a second biopsy was performed, and a pathological examination of the specimen showed a two-cell pattern. The diagnosis was changed to intracranial germinoma. After chemotherapeutic treatment with etoposide and cisplatin, the patients symptoms and radiological findings dramatically improved. We conclude that it is very challenging to distinguish germinomas with a granulomatous reaction due to other inflammatory diseases, especially when only small specimens can be obtained. Stereotactic or endoscopic biopsies should be performed using samples from several different points even if the lesions are associated with eloquent brain regions.

Occipital Artery to Middle Cerebral Artery Bypass in Cases of Unavailable Superficial Temporal Artery

In neurosurgery, extracranial-to-intracranial (EC-IC) bypass surgery is necessary for patients who have undergone surgery in which the superficial temporal artery (STA) was already used for a different bypass procedure or was damaged. Here we report our experience with EC-IC bypass using the occipital artery (OA) in patients in whom the STA was unavailable, and discuss the technical considerations and pitfalls. Five patients with ischemic-onset moyamoya disease and atherosclerotic disease were included. Two patterns of skin incisions were planned according to the OA pathway and recipient artery location. In one of these methods, a skin incision is made above the OA, and a craniotomy is performed under this incision after OA dissection. In the other method, a skin incision is made above the OA to enable its dissection, and a craniotomy is performed via a separate skin incision. No major perioperative complications developed in any of the 5 cases, and bypass patency was confirmed in all patients. There was a significant difference between the preoperative and postoperative asymmetry ratios of the mean transit time values. Our findings suggest that OA-to-middle cerebral artery (MCA) bypass is a simple and effective technique in patients in whom the STA was already used or was damaged by previous intracranial revascularization or craniotomy. This procedure could be an alternative to STA-MCA bypass in patients without an available STA.

Interdisciplinary Prevention and Management of Wound-Related Complications in Extracranial-to-Intracranial Bypass Surgery

Extracranial-to-intracranial (EC-IC) bypass surgery may be necessary in patients with moyamoya disease and other ischemic conditions. However, there is a potential risk of wound-related complications in some cases. In this study, we report our approach to the prevention of wound-related complications in EC-IC bypass. Technical considerations and pitfalls of surgery are also discussed. This study included 89 patients with ischemic-onset moyamoya disease and atherosclerotic disease who underwent 108 superficial temporal artery (STA)-to-middle cerebral artery bypass procedures. Our study emphasized 3 major features. First, 3-dimensional simulation imaging was used to confirm STA anatomy. Second, the STA was meticulously dissected on the epigaleal layer to protect the galeal layer. Third, scalp skin ischemia was taken into consideration at each step until skin closure. There was no neurologic morbidity or mortality in this series. There were 2 cases of major wound-related complications requiring plastic surgical intervention, and 4 cases of minor complications that were treated conservatively. In major complication cases, the scalp defect was treated with pedicle flap reconstruction. In EC-IC bypass surgery, interdisciplinary management involving neurosurgery, plastic surgery, and radiology should reduce wound-related complications and achieve safe surgery.

Surgical Anatomy of Rats for the Training of Microvascular Anastomosis

BACKGROUND Microvascular anastomosis is an essential procedure in neurosurgery, but the opportunity to perform the surgery has gradually decreased for neurosurgeons. Therefore, training is necessary for obtaining and maintaining the skills required for the procedure. We describe the detailed anatomy of cervical and femoral regions in rats and discuss the advantages for practicing microvascular anastomosis. METHODS Cervical regions of Sprague-Dawley rats were dissected under intraperitoneal anesthesia. The step-by-step anatomic description was documented using a high-resolution charge-coupled device image sensor and recording systems. Using this model, temporal occlusion time and patency were measured, and these measures were compared between the trainee and trainer groups. The number of times the training needs to be completed to attain competency in the bypass procedure was estimated. RESULTS After exposing the carotid triangle, a half-ring was created by end-to-side anastomosis. Anastomosis was performed at the common carotid artery using the contralateral side of the carotid artery as a graft. The cutoff value for the temporal occlusion time was 79.3 minutes in the receiver operating characteristic curve based on a target temporal occlusion time for beginners determined during the training. CONCLUSIONS Using a living animal model, a trainee has the opportunity to learn not only anastomotic techniques but also hemostatic control as well as overcoming mental strain during surgery. Living animal models are important in training because the fidelity of a living animal model is superior to nonliving models. Applying training using a half-ring model contributes to safe and efficient surgery.

Influence of hemodynamics on enlarged perivascular spaces in atherosclerotic large vessel disease

ABSTRACT Objectives: Enlarged perivascular spaces (EPVS) are often observed in small vessel disease on T2-weighted images. However, their role in ischemic conditions caused by cerebral large vessel disease remains unclear. We evaluated EPVS in patients with hemodynamic compromise associated with atherosclerotic large vessel disease and aimed to identify the pathophysiology of EPVS. Methods: We examined 28 adults with atherosclerotic large vessel disease. EPVS numbers in the basal ganglia and the centrum semiovale were assessed. For each affected hemisphere, the total numbers of EPVS were compared with those on the unaffected side. EPVS in the impaired hemodynamics group were compared with those in the unimpaired hemodynamics group. Moreover, EPVS were compared in the presence/absence of large stroke. Results: The number of EPVS was significantly increased on the affected side in the centrum semiovale (p = 0.023), particularly in the impaired hemodynamics group (p = 0.006). Moreover, in the small stroke subgroup of the impaired hemodynamics group, the number of EPVS was significantly increased on the affected side (p = 0.002), although this number was insignificant in the large ischemic stroke subgroup. Conclusions: The number of EPVS was increased in patients with atherosclerotic large vessel disease with hemodynamic compromise and decreased in the presence of a large stroke. EPVS might act as fluid absorbers in a hemodynamically compromised state until the occurrence of an ischemic stroke.

Development of moyamoya disease after non-herpetic acute limbic encephalitis: A case report

We report a case of moyamoya disease (MMD), which developed after non-herpetic acute limbic encephalitis (NHALE) associated with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody. The patients mother had a history of MMD. No vascular lesions were identified at the time of the NHALE. Nine years later, the patient visited our hospital due to memory disturbances and repeated transient ischemic attacks affecting the right limb. Diffusion-weighted magnetic resonance imaging revealed scattered areas of signal hyperintensity, and the patient was ultimately diagnosed with MMD based on angiography. Revascularization surgery was performed on the left side, where cerebral blood flow was impaired on 123I-N-isopropyl-p-iodoamphetamine single photon emission computed tomography. Postoperatively, the patient was discharged with a normal neurological examination. NHALE associated with LGI1 antibodies is an autoimmune disease. Although autoimmune disease is the most frequent finding other than atherosclerosis in quasi-MMD, this is the first report of NHALE associated with anti-LGI1 antibodies mimicking quasi-MMD. Inflammation and angiogenesis may contribute to the development of MMD, in addition to genetic background.

Threshold and distribution of afterdischarges with electrical cortical stimulation

OBJECTIVE The present study aimed to investigate the threshold and distribution of afterdischarges (ADs) with cortical electrical stimulation for functional brain mapping. METHOD We retrospectively analyzed data from 11 patients with medically intractable epilepsy who underwent 50-Hz cortical electrical stimulation for functional mapping followed by resection. These patients became seizure free for more than six months. The threshold and distribution of ADs induced by the stimulation were evaluated. RESULTS The median threshold was 6 mA (range: 2-15 mA) for the frontal lobe, 8 mA (3-15 mA) for the temporal lobe, 6 mA (2-15 mA) for the parietal lobe, and 6 mA (4-12 mA) for the occipital lobe. No significant interlobar differences were observed in AD thresholds. No significant differences were noted between within and outside epileptogenic zones. The distribution of ADs, remote spread was observed in all patients, reflecting fronto-parieto-temporal connections, as well as contiguous spread. The stimulation of premotor areas, the inferior parietal lobule, supplementary motor area, and basal temporal areas appeared to induce ADs in remote cortices. CONCLUSION While no locational differences were observed in AD thresholds, each brain region showed a characteristic pattern for AD spread. Remote AD spread needs to be considered for safe functional mapping.

Geometrical Complexity of Cortical Microvascularization in Moyamoya Disease

BACKGROUND Dilatation of the microvascular diameter is recognized in moyamoya disease and referred to as microvascularization. The purpose of this study was to characterize the cortical microvascularization in moyamoya disease using imaging analysis, and to explore the developmental mechanism of the collateral network around the cortical surface. METHODS A total of 20 hemispheric sides of 14 patients with moyamoya disease were included in this study. From the intraoperative images, cortical surface images were extracted, and binary images were subsequently created. Then the ratio of the microvessels of the brain surface (vascular fraction; VF) and the box-counting fractal dimension (Db) values were calculated. The VF and Db values in the moyamoya disease group were then compared with those in atherosclerotic disease and nonischemic disease groups, and assessed in terms of clinical and radiologic factors. RESULTS VF was significantly higher in the moyamoya disease group compared with the atherosclerotic disease group, and Db was significantly higher in the moyamoya disease group compared with the atherosclerotic disease and nonischemic disease groups. In the moyamoya disease group, VF showed a moderate correlation with magnetic resonance angiography (MRA) score. Moreover, Db was significantly higher in the pediatric patients, in the presence of ischemic symptoms, and in the presence of ivy sign, and Db showed a moderate correlation with MRA score and cerebral blood flow in moyamoya disease. CONCLUSIONS In the patients with moyamoya disease, the cortical microvascularization exhibited increased Db and dilatation of the pial arteries. In moyamoya disease, cortical microvascularization is associated with clinical and radiologic factors. This microvascularization might be a compensatory mechanism in the ischemic condition in moyamoya disease.

The Involvement of Sensory-motor Networks in Reflex Seizure

Reflex seizures are epileptic events triggered by specific external stimuli, or less commonly, internal mental stimuli. Understanding the characteristics of reflex seizures is important to elucidate the mechanisms underlying network abnormalities in epileptic conditions. This report details a patient with medically intractable reflex seizures provoked by sensory stimuli to the patient’s right foot. Single-photon emission computed tomography (SPECT) during the seizure induced by sensory stimulation showed hyperperfusion in broad sensory-motor networks (dorsal column-medial lemniscus pathway, left thalamus, bilateral postcentral gyri and posterior parietal cortices, left supplementary motor area (SMA), and left paracentral lobule) and left caudateputamen. The irritative zones and ictal onset zone were localized to the left medial frontoparietal (SMA, anterior and middle cingulate gyrus, and paracentral lobule) and lateral posterior parietal cortices, as evidenced by amelioration of reflex seizures following intracranial electroencephalography and surgical resection of these areas. The neuroradiological and electrophysiological findings in our case study illustrate that the mechanism of reflex seizures may be associated with hyperexcitability of the broad sensory-motor networks, including the basal ganglia. Disconnection of these networks is necessary to treat reflex seizures.