Endovascular Mechanical Thrombectomy for Basilar Artery Occlusion Caused by Thrombosis as an Initial Manifestation of Acute Myelogenous Leukemia: A Case Report.

We report a rare case of a basilar artery occlusion (BAO) caused by thrombosis as an initial magnification of acute myelogenous leukemia (AML) and performed mechanical thrombectomy (MT) to treat it. A 67-year-old female presented left hemiparalysis of her arm and right-sided blindness. Magnetic resonance imaging (MRI) and magnetic resonance angiography revealed acute infarction in the left occipital and anterior lobes of the cerebellum and incomplete BAO. Her blood test showed hyperleukocytosis with precursor cells and high levels of C-reactive protein, and we diagnosed AML and disseminated intravascular coagulation (DIC). We decided to treat conservatively with rapid rehydration and heparin, but three hours after admission, she suddenly lost consciousness. We performed acute MT with a direct aspiration first-pass technique (ADAPT). A white elastic embolus was aspirated, and DSA showed successful recanalization of the basilar artery. The next day, MRI revealed acute infarction in the midbrain and bilateral thalamus. The patient remained unconscious after MT and so chemotherapy to treat the acute leukemia could not be performed. The patient died of the primary disease 14 days after BAO. Thrombosis in association with AML is very rare disease and could occur in arterial vessels because of hypercoagulation, and this tendency may not respond to anticoagulation therapy. Although ADAPT might be performed safety without complications even in cases of DIC, indications for treatment with MT should be carefully considered in patients in whom hemorrhage is a possibility.

Double LVIS Jr. Stenting of a Ruptured Proximal Anterior Inferior Cerebellar Artery Aneurysm: A Case Report.

Objective: We report a case of ruptured aneurysm at the anterior pontine segment of the anterior inferior cerebellar artery (AICA) which re-ruptured after stent placement and was treated by overlapping stenting. Case Presentation: A 53-year-old woman presented with headache. CT demonstrated subarachnoid hemorrhage. DSA revealed no evident source of bleeding. On day 10, she complained of sudden headache and CT demonstrated re-bleeding. On repeated DSA, an aneurysm at the anterior pontine segment of the right AICA was found. An LVIS Jr. stent was deployed at the right AICA including the aneurysm. On postoperative day 23, the aneurysm ruptured again. Another LVIS Jr. stent was deployed at the same area. On day 56, she was discharged home without neurological deficit. Conclusion: Intracranial aneurysms not indicated for coil embolization or parent artery occlusion are difficult to treat. Overlapping stenting may be a treatment option for such aneurysms.

Unusual Presentations of Pediatric Skull Hemangiomas: Report of Two Cases.

BACKGROUND: Intraosseous hemangioma is a rare bone tumor, accounting for 0.7%-1.0% of all bone tumors. It can occur at any age, but only 9% of cases are younger than 10 years old. Although this tumor is usually slow-growing and clinically silent, we experienced 2 pediatric patients undergoing surgery for skull hemangioma who presented with uncommon clinical manifestations. CASE DECRIPTION: Case 1 was a 9-year-old boy who presented with sudden onset of headache and was referred to our hospital. Radiologic images revealed an osteolytic oval lesion in the right parietal bone and acute subdural hemorrhage in the right cerebral hemisphere. The right parietal lesion was removed surgically. The lesion was found to have grown into the dura and to be adherent to the pia matter. The removed lesion was histologically confirmed to be a hemangioma. Case 2 was an 8-year-old girl who was referred to our hospital with an elastic mass that had been slowly enlarging for 7 years. Radiologic images revealed an osteolytic oval lesion in the right parietal bone. Surgical removal was thus planned. The lesion was found to be attached to the dura, and we removed the lesion with the surrounding bone and attached dura. Histologic examination confirmed the lesion to be a hemangioma. CONCLUSIONS: Although skull hemangiomas show clinical heterogeneity, surgical removal is usually diagnostic and leads to good patient outcomes. On occasion, however, this tumor causes secondary changes in the dura, such that dural incision and dural plasty should be planned in advance of lesion removal.

A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with the largest lesion in the temporal lobe.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system with a predilection for the hindbrain. Generally, lesions decrease in size with increasing distance from the hindbrain. We herein describe a case of CLIPPERS in a patient showing the largest lesions in the temporal lobe. A 49-year-old man consulted to our hospital with a 22-day history of a high fever and an abnormal visual field involving the left eye. Brain MRI showed a FLAIR hyperintense lesion in the right temporal lobe with punctate gadolinium enhancement. Several days later, lesions showing similar enhancement extended to the pons, medulla oblongata, and cerebellar hemispheres. Biopsy of the right temporal lobe lesion revealed perivascular lymphocytic infiltrates without any findings of demyelination, lymphoma, or glioma. He was diagnosed with CLIPPERS based on clinical, radiological, and histopathological findings. Clinical and radiological findings improved quickly after steroid therapy. CLIPPERS could represent larger lesions distant from the hindbrain showing punctate enhancement.

Treatment of a skull-base giant cell tumor with endoscopic endonasal resection and denosumab: case report.

A 34-year-old man with a 1-week history of diplopia was referred to the authors' hospital. Neurological examination revealed left abducens nerve palsy. Computed tomography showed a lesion in the left sphenoid sinus involving the medial wall of the left internal carotid artery (ICA) and osteolytic change at the clivus bordering the lesion. Magnetic resonance imaging demonstrated an extensive soft-tissue mass occupying the left sphenoid sinus. Surgical intervention by the endoscopic transnasal method allowed most of the lesion to be removed. Only the portion attached to the medial wall of the ICA was not removed. Postoperatively, the lesion was diagnosed as a giant cell tumor (GCT) and the patient received 120 mg of subcutaneous denosumab every 4 weeks, with additional doses on Days 8 and 15 during the first month of therapy. MRI a week after starting denosumab revealed shrinkage of the initially fast-growing residual tumor. The patient was discharged upon completion of the third denosumab administration. GCT is an aggressive stromal tumor developing mainly in young adults. Complete resection is recommended for GCT in the literature. However, size and location of the CGT often limit this approach. Various adjuvant treatments for skull base GCTs have been reported, including radiation and chemotherapy. However, the roles of adjuvant therapies have yet to be clearly defined. Denosumab, a monoclonal antibody, was recently approved for GCT in several countries. Denosumab may permit less invasive treatments for patients with GCTs while avoiding deleterious outcomes, and may also limit disease progression and recurrence.

Neurological symptoms in a patient with isolated adrenocorticotropin deficiency: case report and literature review.

BACKGROUND: Isolated adrenocorticotropic hormone (ACTH) deficiency is a pituitary disorder characterized by reduction only in the secretion of ACTH. Although the underlying mechanism remains to be elucidated, numbers of cases with this entity have been increasing. We experienced a case presenting with gait disturbance necessitating differential diagnosis from idiopathic normal pressure hydrocephalus (iNPH). CASE PRESENTATION: A 69-year-old female with a complaint of difficulty walking and suspected to have iNPH at a prior hospital was referred to our department. For the prior three years, she had suffered from a progressive gait disturbance. Magnetic resonance imaging (MRI) revealed global ventricular dilatation. The typical features of the gait in iNPH cases were all identifiable. Neuropsychological dementia scale tests showed deterioration. However, the major feature of a disproportionately enlarged subarachnoid-space on MRI was not obvious. The patient developed progressively worsening fatigue during hospitalization. Her symptoms resembled those of hypothalamic-pituitary tumor patients. Serum ACTH and cortisol levels were low. While corticotrophin releasing hormone stress tests showed no response, other stress tests using thyrotropin releasing hormone, luteinizing hormone releasing hormone, and growth hormone releasing hormone yielded normal responses, indicating a diagnosis of isolated ACTH deficiency. We initiated corticosteroid therapy, and her gait disturbance improved promptly. CONCLUSION: Isolated ACTH deficiency may have major significance to the differential diagnosis of iNPH. Early consideration of this entity is anticipated to facilitate making an early diagnosis.

Medical Comics as Tools to Aid in Obtaining Informed Consent for Stroke Care.

Informed consent has now become common in medical practice. However, a gap still exists between doctors and patients in the understanding of clinical conditions. We designed medical comics about "subarachnoid hemorrhage" and "intracerebral hemorrhage" to help doctors obtain informed consent intuitively, quickly, and comprehensively.Between September 2010 and September 2012, we carried out a questionnaire survey about medical comics with the families of patients who had suffered an intracerebral or subarachnoid hemorrhage. The questionnaire consisted of 6 questions inquiring about their mental condition, reading time, usefulness of the comics in understanding brain function and anatomy, pathogenesis, doctor's explanation, and applicability of these comics.The results showed that 93.8% responders would prefer or strongly prefer the use of comics in other medical situations. When considering the level of understanding of brain function and anatomy, pathology of disease, and doctor's explanation, 81.3%, 75.0%, and 68.8% of responders, respectively, rated these comics as very useful or useful.We think that the visual and narrative illustrations in medical comics would be more helpful for patients than a lengthy explanation by a doctor. Most of the responders hoped that medical comics would be applied to other medical cases. Thus, medical comics could work as a new communication tool between doctors and patients.

Strategies to prevent positioning-related complications associated with the lateral suboccipital approach.

The lateral positioning used for the lateral suboccipital surgical approach is associated with various pathophysiologic complications. Strategies to avoid complications including an excessive load on the cervical vertebra and countermeasures against pressure ulcer development are needed. We retrospectively investigated positioning-related complications in 71 patients with cerebellopontine angle lesions undergoing surgery in our department between January 2003 and December 2010 using the lateral suboccipital approach. One patient postoperatively developed rhabdomyolysis, and another presented with transient peroneal nerve palsy on the unaffected side. Stage I and II pressure ulcers were noted in 22 and 12 patients, respectively, although neither stage III nor more severe pressure ulcers occurred. No patients experienced cervical vertebra and spinal cord impairments, brachial plexus palsy, or ulnar nerve palsy associated with rotation and flexion of the neck. Strategies to prevent positioning-related complications, associated with lateral positioning for the lateral suboccipital surgical approach, include the following: atraumatic fixation of the neck focusing on jugular venous perfusion and airway pressure, trunk rotation, and sufficient relief of weightbearing and protection of nerves including the peripheral nerves of all four extremities.

[Munchausen syndrome, a factitious injury, presenting brain abscess and intraventricular hemorrhage: a case report].

Munchausen syndrome is a factitious disorder. Patients sometimes inflict injury on themselves in order to assume a sick role. The authors report a patient with Munchausen syndrome suffered from brain abscess, reopened wound and intraventricular hemorrhage. A 64-year-old male was admitted to our hospital after head injury. CT and MR imaging revealed a mass with surrounding edema in the right frontal lobe. The mass was surgically removed, and diagnosed as brain abscess. During the surgery, the authors noticed a small bone defect in the frontal bone above the brain abscess; therefore, we considered that head injury just concerned this lesion. There were no particular clues leading to other possible pathologies. After the first surgery, the patient presented atypical seizures several times. Once we discharged him from our hospital, we hospitalized him again because the wound had reopened. A subsequent operation was needed, and we removed the bone flap which we considered the origin of the infection. After the second surgery, he stabbed a nail into his head where the bone had been removed due to the previous surgery, and presented intraventricular hemorrhage. The hemorrhage decreased in size through non-surgical treatment and he was referred to the psychiatry department under a diagnosis of Munchausen syndrome. Diagnosis of this entity is difficult and often made at the later stage of hospitalization, because patients present a variety of complaints and clinical symptoms, which are hardly proved factitious. Early consideration of this syndrome will offer an early and accurate diagnosis, and is mandatory for a good prognosis.

Cavernous angioma associated with arteriovenous malformation of the brain--case report.

A 61-year-old man presented a rare case of cerebral cavernous angioma associated with cerebral arteriovenous malformation (AVM) manifesting as headache. Magnetic resonance imaging showed an AVM in the right occipital lobe and a cavernous angioma in the left middle fossa. The patient underwent left frontotemporal craniotomy with total resection of the left parasellar intra-axial tumor. One month after the first operation, he underwent right occipital craniotomy with total resection of the right occipital AVM. The histological diagnoses were cavernous angioma and AVM, respectively. Immunostaining for angiogenic growth factors and structural proteins revealed different expression patterns of alpha-smooth muscle actin in these structures. Expression of structural proteins may reflect differences in their pathogenesis.

[A case of posttraumatic forehead cavernous angioma].

Cavernous angiomas of the bone are rare tumors. Skull cavernomas are even less frequent. Most cavernous angiomas of the bone are congenital tumors. In a review of the literature, we found only one case report of de novo generation of a skull cavernous angioma. We present a case of a 25-year-old woman who had experienced a head injury, and 7 years later exhibited a skull tumor at the exact region of the injury. We performed tumor resection and cranioplasty. Follow-up examinations revealed no recurrence or neurological defects. Pathological findings showed a cavernous angioma-like lesion with some atypical details. We finally diagnosed the lesion as a de novo cavernous angioma. Our case suggests that fine injury may result in de novo generation of bone cavernomas.

[A successful surgical case of a hypothalamic hamartoma with gelastic seizure: a case report].

Gelastic seizure is a rare form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. Surgical treatment of hypothalamic hamartomas is associated with a high risk of complications because of the close vicinity of adjacent structures such as the optic tracts and mammillary bodies. This case was an 11-year-old girl who presented with gelastic seizure and complicated partial seizure. She developed gelastic seizure at a frequency of 10 bouts per day. She was found to have an elliptical mass close to the left hypothalamus. The signal intensity on magnetic resonance imaging (MRI) was consistent with hypothalamic hamartoma. The patient underwent surgical resection by a subtemporal approach. Pathological findings confirmed the diagnosis of hypothalamic hamartoma. Postoperative MRI demonstrated that the hypothalamic hamartoma was successfully resected. Twenty four months after surgery, complicated partial seizure in this patient has improved to Engel's class 1a and gelastic seizure has improved to Engel's class IIIa.

Multiple isolated sinus dural arteriovenous fistulas associated with antithrombin III deficiency--case report--.

A 55-year-old man presented with a rare case of multiple isolated sinus dural arteriovenous fistulas (AVFs) associated with antithrombin (AT) III deficiency manifesting as sudden onset of headache and gait disturbance. Increased arterial shunting flow had caused intraventricular hemorrhage after incomplete repeated transarterial embolization procedures for dural AVFs. Multiple isolated sinus dural AVFs were located in the anterior superior sagittal sinus (SSS) and transverse sinus, which were completely embolized by direct packing of the isolated sinuses via the SSS. The development of dural AVF is complicated and associated with a number of factors, such as congenital abnormality, head trauma, craniotomy, radiation, hematological abnormality, and sinus thrombosis. Hematological abnormality is a risk factor of sinus thrombosis. In the present case, the multiple isolated sinus dural AVFs might have resulted from the aggravation of multiple dural AVFs and the coagulative tendency due to AT III deficiency. Direct sinus packing should be considered if transvenous catheterization is difficult or fails.

Gradual deterioration of brainstem cavernous angioma associated with hemophilia--case report.

A 49-year-old man presented with a brainstem cavernous angioma associated with hemophilia manifesting as gradual progression of neurological deficits over a period of 2 weeks. Computed tomography revealed a hematoma and perifocal edema on the left side of the pons, and T(2)-weighted magnetic resonance imaging revealed a hemosiderin rim around the lesion with venous malformation. The neurological deficits deteriorated despite conservative treatment, so surgery was performed 2 weeks after admission, after supplementary therapy of factor IX. The hematoma and anomalous vascular component were entirely removed without intractable bleeding. The postoperative course was uneventful and his neurological symptoms improved. The histological diagnosis was cavernous angioma. Six months after onset, he was doing well. Surgery can be effective for the treatment of hemorrhagic cavernous angioma associated with hemophilia after initiation of supplementary therapy with coagulation factor.

Intracranial peripheral-type primitive neuroectodermal tumor.

A 15-year-old man presented with headache. Magnetic resonance (MR) imaging revealed a large extraaxial tumor with cyst at the right frontotemporal region. The solid part of the tumor was homogeneously enhanced on T(1)-weighted MR imaging after injection of gadolinium. Digital subtraction angiography of the external carotid artery revealed sunburst appearance corresponding to the tumor, which was fed by the right middle meningeal artery. His headache worsened and computed tomography revealed enlargement of the tumor and intracystic hemorrhage, so emergent operation was performed. At surgery, the tumor strongly adhered to the dural membrane, and was obviously extraaxial. The tumor and cyst were gross totally removed. The attachment site at the dura mater was resected. Histological examination showed solid growth of small round cells with uniform round nuclei and minimal cytoplasm. Immunohistochemical staining showed the cells were positive for MIC-2 (CD99). The MIB-1 labeling index was 53%. The histological diagnosis was peripheral-type primitive neuroectodermal tumor (pPNET). Following surgery, radiation therapy and chemotherapy were given. Ewing's sarcoma and pPNET form a family of small round cell tumors arising in the bone or soft tissue. MIC-2 is a useful marker in the differential diagnosis. Good prognosis may be attained if complete surgical excision of intracranial pPNET is achieved.

[Clinical investigation of cerebral infarction following head and neck injury].

The diagnosis of cerebral infarction following head and neck injury is difficult because of the lucid interval, which causes high mortality and morbidity. We report six patients with cerebral infarction following head and neck injury, whom we treated from 1993 to 2005. Only one patient suffered from cerebral infarction following direct neck injury. At the time of injury three patients had prodromal symptoms and the others had no symptoms. Three patients were diagnosed with artery-to-artery embolism based on the dissection of the internal carotid artery in the neck, two patients, from the dissection of the vertebral artery in the neck, and one patient was diagnosed with Wallenberg's syndrome. The overall patient outcomes at discharge as measured by using the Glasgow Outcome Scale were as follows: moderately disabled in one, severely disabled in three, vegetative survival in one, and death in two patients. No patient had been administered a screening test for neck vessel injury, indicating the difficulty in diagnosis. Prodromal symptoms, due to not only direct but also indirect neck injuries indicate the possibility of neck vessels injury, and hence, we should develop a method that enables the early detection of lesions caused by neck vessel injury.

[Orbital schwannoma developing from the superior branch of the oculomotor nerve--case report].

Orbital schwannoma accounts for 1-6% of all orbital tumors and it is not easy to identify the origin of the tumor because of the anatomy of the orbit. A case of orbital schwannoma developing from the superior branch of the oculomotor nerve is reported. A 74-year-old female was admitted to our hospital with the chief complaint of left exophthalmus. MR imaging revealed a space-occupying lesion in the left orbit. Operation was performed through the transcranial approach. It was noticed that the tumor was developing from the nerve that runs into the superior rectus muscle or the levator palpebrae superioris muscle, and it was diagnosed as a schwannoma on frozen section histology during surgery. The tumor was successfully removed and, postoperatively, no additional defects occurred. Careful consideration of microsurgical anatomy is essential for surgery of orbital schwannoma especially when it's necessary to cut the nerves.