Endovascular treatment of unruptured aneurysm arising from duplicate origin of the middle cerebral artery - A case report and literature review.

Background: Duplicate origin of the middle cerebral artery (MCA) is a rare variation of MCA, often mislabeled as the fenestration of the M1 segment of MCA. Case Description: The authors treated an unruptured aneurysm, 8 mm in diameter, associated with a duplicate origin of MCA in a 42-year-old woman who underwent magnetic resonance imaging for transient vertigo. Clipping surgery was inapplicable due to the lack of space to insert clip blades between the neck and two origins of MCA. Under stent-assisted maneuver, the aneurysm sac was successfully obliterated using three coils, resulting in Raymond-Roy class 1 occlusion status. Digital subtraction angiography performed 3 months after the embolization showed complete obliteration of the aneurysm. So far, only 11 patients with aneurysms associated with duplicate origin of MCA have been reported. We performed a literature review of this very rare combination. The size of aneurysms ranged from 2 to 8 mm, with a mean of 5.2 mm. The neck of the aneurysm is mainly located at the corner between the inferior limb and the internal carotid artery. Ours is the youngest and has the largest aneurysm. Conclusion: Aneurysm can arise from duplicate origin of MCA, for which stent-assisted coiling may be an appropriate treatment modality.

Systemic Embolism Following Mechanical Thrombectomy for Acute Ischemic Stroke: A Case of Suspected Catastrophic Antiphospholipid Syndrome.

Objective: Catastrophic antiphospholipid syndrome (CAPS) is a disease characterized by a poor prognosis and a high mortality rate, leading to systemic thrombosis. Approximately two-thirds of CAPS cases are associated with conditions such as infections, malignancies, surgical interventions, and events linked to the disease activity of systemic lupus erythematosus (SLE). Herein, we present a case of CAPS with multiorgan ischemia following ischemic stroke. Case Presentation: In this case report, a 33-year-old woman with a history of SLE and prolonged steroid use manifested impaired consciousness. Detection of the right internal carotid artery (ICA) occlusion led to successful ICA recanalization through endovascular thrombectomy. Postoperatively, she experienced pulmonary embolism and renal infarction. Although antiphospholipid syndrome (APS) was suspected, APS-related antibodies were negative. Anticoagulation therapy was initiated, presuming corticosteroid-induced thrombosis. However, she developed multiorgan thrombosis, culminating in multiple organ failure. Based on her clinical course, a diagnosis of CAPS was established. Intensive care and plasma exchange therapy were instrumental in her recovery, and she was discharged with a modified Rankin Scale score of 4. Conclusion: When encountering multiorgan ischemia following ischemic stroke in a young adult patient with an autoimmune disease, the consideration of CAPS as a differential diagnosis is crucial, even if APS-related antibodies test negative.

Bilateral Internal Carotid Artery Hypoplasia with Craniofacial Anomalies: A Case of Suspected Treacher Collins Syndrome.

Internal carotid artery aplasia or hypoplasia above the cervical bifurcation is rare, occurring in less than 0.01% of the general population. Unilateral neurocristopathy complicated by unilateral internal carotid artery agenesis or hypogenesis has been reported, but bilateral internal carotid artery hypoplasia is rare and scarcely reported. Herein, we report a novel case of Treacher Collins syndrome complicated by bilateral internal carotid artery hypoplasia. A 94-year-old woman presented with complaints of headache and vomiting. Computed tomography revealed a subarachnoid hemorrhage and dysplasia of the bilateral zygoma, mandible, and external auditory meatus. The patient had severe hearing loss and visual impairment. Computed tomography angiography revealed bilateral internal carotid artery hypoplasia and multiple aneurysmal changes in the intracranial arteries. We diagnosed the patient with a ruptured anterior inferior cerebellar artery aneurysm and performed coil embolization. The patient's unique facial features were consistent with neurocristopathy, especially Treacher Collins syndrome. Developmental anomalies of neural crest cells can present as vascular abnormalities and craniofacial malformations. Special care is required for endovascular treatment and airway management in cases of neurocristopathy because of the specific craniofacial anomalies.

Efficacy and safety of the pterional keyhole approach for the treatment of anterior circulation aneurysms.

Keyhole surgery is partly replacing the standard pterional approach in patients undergoing surgery to treat aneurysms of the anterior circulation. We describe the pterional keyhole approach for the clipping of anterior circulation aneurysms and discuss the efficacy and safety of our keyhole craniotomy procedure. We treated 103 patients with 111 intracranial aneurysms by surgical clipping via the pterional keyhole approach and retrospectively compared the characteristics and clinical outcomes of the keyhole procedure and the standard pterional approach. We also compared the surgical results of the keyhole approach when the operator was an experienced neurosurgeon or a less experienced neurosurgeon guided by an experienced colleague. All keyhole operations were carried out successfully without enlargement of the craniotomy or a change to a different approach. The outcomes of the keyhole and the standard pterional approach in patients with subarachnoid hemorrhage were not significantly different. Favorable outcomes were obtained in patients with unruptured aneurysms treated by either experienced or less experienced surgeons. The pterional keyhole approach offers the same surgical possibilities as conventional pterional approaches for the treatment of anterior circulation aneurysms. It is safe and simple and yields favorable outcomes even if the operators are less experienced neurosurgeons. Careful patient selection and sufficient opening of the sylvian fissure are the key points for good outcomes and the prevention of intraoperative complications.

Clinical presentation and treatment of distal posterior inferior cerebellar artery aneurysms.

Aneurysms located at the distal portion of the posterior inferior cerebellar artery (PICA) are rare, and their clinical features are not fully understood. We report the clinical features and management of 30 distal PICA aneurysms in 28 patients treated during the past decade at Kagoshima University Hospital and affiliated hospitals. Our series includes 20 women and eight men. Of their 30 aneurysms, 24 were ruptured, and six were unruptured; there were 27 saccular and two fusiform aneurysms; one was dissecting. Their location was at the anterior-medullary (n = 4), lateral-medullary (n = 9), tonsillomedullary (n = 7), telovelotonsillar (n = 6), and cortical (n = 4) segment of the PICA. In 18 patients, angiographic features suggested hemodynamic stress including an absent contralateral PICA or ipsilateral anterior inferior cerebellar artery, termination of the vertebral artery (VA) at the PICA, and hyperplasia or occlusion of the contralateral VA. As three patients died before surgery, 27 aneurysms in 25 patients were surgically treated. Of these, 6 were unruptured aneurysms; 20 were clipped via midline or lateral suboccipital craniotomy, and 5 were embolized with Guglielmi coils; in one, the PICA flow was reconstructed by OA-PICA anastomosis, and in the other one, the PICA was resected. Of the 25 surgically treated patients, 22 (88%) had good outcomes. The predominant contributor to the development of distal PICA aneurysms is thought to be increased hemodynamic stress attributable to anomalies in the PICA and related posterior circulation. Both direct clipping and coil embolization yielded favorable outcomes in our series. However, considering the difficulties that may be encountered at direct clipping in the acute stage and the availability of advanced techniques and instrumentation, aneurysmal coiling is now the first option to address these aneurysms.

Efficacy and safety of key hole craniotomy for the evacuation of spontaneous cerebellar hemorrhage.

The efficacy and safety of cerebellar hemorrhage evacuation by key hole craniotomy and the importance of thorough evacuation and irrigation of the hematoma in the fourth ventricle to resolve obstructive hydrocephalus were assessed in 23 patients with spontaneous cerebellar hemorrhage (SCH) greater than 3 cm or with brainstem compression and hydrocephalus. A 5-cm elongated S-shaped scalp incision was made, and a 3-cm key hole craniotomy was performed over a cerebellar convexity area. The hematoma was immediately evacuated through a small corticotomy. The hematoma in the fourth ventricle was gently removed through the hematoma cavity, followed by thorough saline irrigation to release obstructive hydrocephalus. Patients classified retrospectively into favorable and poor outcome groups using the Glasgow Outcome Scale (GOS) scores of 4-5 vs. 1-3 showed significant differences with respect to the preoperative Glasgow Coma Scale, hematoma size and volume, and brainstem compression. Only 2 of the 23 patients required ventricular drainage and no postoperative complications were recorded. Patients treated by experienced and inexperienced surgeons showed no significant differences in the hematoma evacuation rate, postoperative GOS, and interval from skin incision to start of hematoma evacuation. Our simplified method of key hole craniotomy to treat SCH was less invasive but easy to perform, as even inexperienced neurosurgeons could obtain good surgical results. Thorough cleaning of the fourth ventricle minimized the necessity for ventricular drainage.

Dietary intake of seven B vitamins based on a total diet study in Japan.

The present study estimated the dietary intake of seven B vitamins using a total diet study (TDS) in Japan. The daily intake of vitamins estimated by TDS was calculated based on the mean contents of vitamins in 18 food groups, and the amount of food intake in the Nation Health and Nutrition Survey in Japan, 2006. The estimated daily intake of these vitamins for all ages was 22.8 mg NE/d for niacin, 7.4 µg/d for vitamin B(12), 146 µg/d for folic acid, 4.52 mg/d for pantothenic acid, 1.06 mg/d for riboflavin, and 1.44 mg/d for pyridoxine. The estimated daily intake of the vitamins of niacin, vitamin B(12) and pyridoxine exceeded the dietary reference values for adults aged 18-29 y. The estimated daily intake of these vitamins by TDS was higher than the daily intake reported in the National Health and Nutrition Survey in Japan, 2006. There was a strongly positive correlation between the intake levels estimated by TDS and those reported in the National Health and Nutrition Survey. This suggests that TDS is an effective dietary survey for estimating the dietary intake of water-soluble vitamins. Therefore, when being determined by TDS, the estimated daily intake of biotin was 51.0 µg/d for all ages.

Cranial surgery without head shaving.

OBJECTIVE: Based on a series of 632 patients who underwent craniotomy without head shaving, we report the efficacy and safety of our simplified procedure and document the usefulness of the electrosurgical scalpel. METHODS: After brushing a chlorhexidine-alcohol solution onto the craniotomy site, the hair was parted from the incision line and fixed with adhesive paper drapes. In recent cases, electrosurgical scalpels were used for scalp- and subcutaneous dissection. At the end of surgery, the wound was closed in the usual manner, taking care that no hair was in the wound and the hair and wound were rinsed with clean water in the operating room. We did not apply disinfectant for postoperative wound care, rather, the hair was shampooed on the 2nd, 4th, and 6th postoperative day. RESULTS: Among 632 patients who underwent cranial surgery without head shaving, only 7 (1.1%) developed postoperative wound infections. None of the 34 patients who underwent craniotomy using the electrosurgical scalpel developed wound infections. CONCLUSIONS: Our simplified cranial surgery without head shaving does not increase the risk of wound infection. Because the use of the electrosurgical scalpel for skin and soft tissue dissection minimizes bleeding, the probability of wound infection appears to be reduced.

Effects of biotin deficiency on embryonic development in mice.

OBJECTIVE: The purpose of this investigation was to determine the effects of biotin deficiency on maternal metabolism and embryonic development in pregnant mouse dams. METHODS: The pregnant mice were randomly assigned to one of three dietary groups and given a biotin-deficient diet, biotin-supplemented diet, or biotin-control diet during gestation. On days of gestation (dgs) 0, 4, 8, 12, and 16, organic acids including 3-hydroxyisovaleric acid in urine were discovered by high-performance liquid chromatography, and the biotin level in the serum and urine was determined by a bioassay. On dg 18, fetuses were examined for morphologic development. RESULTS: In the biotin-deficient group, biotin excretion in urine decreased on dg 4 and was subsequently below the lower limit, whereas the urinary concentration of 3-hydroxyisovaleric acid increased after dg 12. In contrast, the biotin concentration in urine significantly increased on dgs 4, 8 and 12 in the biotin-supplemented group, but decreased on dg 16 in the biotin-supplemented and biotin-control groups. The urinary excretion of pyruvic acid in the biotin-deficient group was significantly higher than that in the biotin-supplemented group throughout the entire gestation. These concentrations in urine significantly increased on dg 16 compared with dg 0. The inhibition of embryonic development and external malformations such as cleft palate (100%), micrognathia (100%), and micromelia (91.4%) were also detected in biotin-deficient fetuses. CONCLUSION: These findings indicated that, as the requirement of biotin increases during gestation and/or embryonic development, a large amount of biotin is necessary for maintaining normal reproductive performance during the late stage of gestation.

[Biotin deficiency in amino acid formula nutrition for an infant with milk protein allergy].

We reported a 4-month-old girl with biotin deficiency caused by amino acid formula. Two weeks after birth, she was diagnosed as having a milk protein allergy. After switching to amino acid formula from usual formula, her symptoms and laboratory findings became normal. About three weeks after the beginning of amino acid formula, she developed intractable skin erosions around the eyes, mouth, neck, and anogenital area. By measuring concentrations of some trace elements, she was diagnosed as having a biotin deficit, because of the organic aciduria and undetectable serum biotin concentration. Her serum biotinidase level was normal. Upon administration of oral biotin supplementation, all her symptoms and laboratory findings were dramatically improved. Since amino acid formula contains very few biotin, we should pay attention to biotin deficiency when infants receiving amino acid formula.

Transplacental transport and tissue distribution of biotin in mice at midgestation.

Biotin is a water-soluble vitamin which functions as a coenzyme of carboxylases in glucose and amino acid metabolism and fatty acid synthesis. Biotin is also essential for maintaining reproductive function. Biotin deficiency during gestation induces cleft palate, micrognathia and limb hypoplasia in mouse fetuses at near term. Maternal biotin deficiency is severely tetatogenic in mammals. However, the relationship between abnormal morphogenesis and biotin deficiency is not sufficiently clear. This study was conducted to elucidate the mechanism of biotin transport from dams to embryos and the nutritional roles of biotin in ICR mice. Pregnant mice were given either a biotin-deficient or biotin-supplemented diet, and biotin and biotinidase activity were determined in dams and fetuses. It became evident that biotin was supplied from dams to growing embryos during morphogenesis. In particular, a large amount of biotin was transported to palates and mandibles on days 12-15 of gestation. The transportation of biotin to fetuses differed among fetal growth periods and organs. These results suggest that biotin is an essential nutrient and may play an important role in embryonic growth.

Biotin deficiency affects the proliferation of human embryonic palatal mesenchymal cells in culture.

It has recently been demonstrated that pregnancy in women may cause mild biotin deficiency without any clinical signs. However, the teratogenicity of biotin deficiency in humans has not been well investigated. On the other hand, our previous studies have shown that maternal biotin deficiency induces many kinds of malformations, such as cleft palate, micrognathia, and micromelia, in all animal fetuses. However the mechanism for cleft palate induction under biotin-deficient conditions is unknown. Therefore, to investigate the possible mechanisms for cleft palate induction in embryos, we investigated the effects of biotin deficiency on human embryonic palatal mesenchymal (HEPM) cells in culture in this study. HEPM cells were cultured in biotin-deficient and biotin-physiological (control) media for 5 wk. The proliferative availabilities of HEPM cells in the biotin-deficient state were significantly lower after wk 2 of culture (41.3% of the control). Biotin concentrations in biotin-deficient cells were drastically lower after wk 1 of culture, whereas those in the control cells remained at almost the same level. Biotinidase activities were also lower in biotin-deficient cells. Holocarboxylases in biotin-deficient cells were fewer after the first week of culture and were almost undetectable after wk 2. The amount of biotinylated histones in the nuclei of biotin-deficient cells was lower than in the control cells. This suppressed proliferation of mesenchymal cells may delay or inhibit the growth of palatal processes in embryos and thus it may partially contribute to the mechanisms for cleft palate induction.

Roles of biotin in growing ovarian follicles and embryonic development in domestic fowl.

It is well known that biotin deficiency causes morphological anomalies in hatchlings of fowl. An abundance of biotin in the yolk, therefore, is greatly required for maintaining reproductive functions. Although there is growing evidence for the molecular significance of the vitamin in the processes of the reproductive system in mammals, little is known about its use and roles in fowl's yolk. This review focuses on studies on the roles of biotin in the ovarian follicles and embryos of domestic fowl. Recent studies from our laboratory showed an analysis of the mechanism of biotin supply from the maternal body to follicles, or the yolk using the eggs of domestic fowl. In growing ovarian follicles, biotin was incorporated into the follicles, particularly in a large amount immediately before ovulation. Biotin incorporated from the early stage to the growth stage of ovarian follicles was stored in the protein-binding form and became free biotin again immediately before ovulation. This may be because the biotin necessary for the maintenance of embryonic growth and development should be used immediately after fertilization. In embryos, free biotin in the yolk increased shortly after fertilization. A large amount of free biotin was incorporated into the embryo at the age of 3-4 d. Biotin supply to the embryo differed among embryonic growth stages and organs, suggesting involvement in the formation of each tissue and organ. Thus, a large amount of biotin was utilized for embryonic growth, suggesting its important role in normal embryonic growth. These findings show that biotin is an essential nutrient and may play a major role in the normal morphogenesis of embryos in domestic fowl.

Subarachnoid hemorrhage from a ruptured anterior cerebral artery aneurysm caused by polyarteritis nodosa. Case report.

Polyarteritis nodosa (PAN) is a rare systemic necrotizing arteritis that involves small- and medium-sized arteries in various organs. Although aneurysm formation in visceral arteries is a typical finding in PAN, intracranial aneurysms are much less common, and only a few cases of aneurysm rupture associated with this disease have been documented. In this paper, the authors report on a ruptured PAN aneurysm of the anterior cerebral artery; the lesion was trapped and resected. On histological examination, extensive fibrinoid necrosis and an inflammatory infiltration of leukocytes were seen in the aneurysm wall. To the authors' knowledge this is the first report of subarachnoid hemorrhage from a histologically confirmed PAN aneurysm.