Subarachnoid hemorrhage after an ischemic attack due to a bacterial middle cerebral artery dissecting aneurysm: case report and literature review.

A 78-year-old woman suffered sudden-onset left hemiparesis. There were no remarkable infectious findings. Computed tomography (CT) demonstrated a low-intensity area supplied by the right middle cerebral artery (MCA). The diagnosis was cerebral ischemia and she was conservatively treated with hyperosmotic fluids. Two days after the ischemic stroke she suddenly became comatose. CT showed diffuse subarachnoid hemorrhage (SAH) in the basal cistern associated with a right intra-Sylvian and a right frontal subcortical hematoma. Three-dimensional (3D)-CT angiography demonstrated occlusion of the M2 portion of the right MCA. Four days after the ischemic onset she died of brain herniation. Autopsy revealed arterial dissection in the intermediate membrane of the right MCA bifurcation and occlusion of the M2 portion of the thrombosed right MCA. Gram staining showed remarkable bacterial infection in the thrombus. SAH after an ischemic attack due to MCA dissection is extremely rare. We suspect that bacterial infection was involved in the formation of her fragile dissecting aneurysm.

Disseminated infection and pulmonary embolization of Cunninghamella bertholletiae complicated with hemophagocytic lymphohistiocytosis.

A 22-year-old Japanese woman was diagnosed with hemophagocytic lymphohistiocytosis and subsequently was treated with etoposide and cyclophosphamide. On Day 22, multiple nodular lesions appeared in the bilateral lungs. Neither the administered antibiotics nor the antifungal agent were effective, and she died suddenly of respiratory failure on Day 35. An autopsy revealed disseminated zygomycosis and a pulmonary infarction due to the embolization of an angioinvasive fungus, which was later identified as Cunninghamella bertholletiae using in situ hybridization of 18S rRNA. C. bertholletiae is aggressive as well as resistant to antifungal agents. This rare species should therefore be taken into consideration as a potential causative agent of zygomycosis.

Monitoring twenty-six chronic myeloid leukemia patients by BCR-ABL mRNA level in bone marrow:a single hospital experience.

Chronic myeloid leukemia (CML) is caused by the BCR-ABL oncogene. The Philadelphia chromosome (Ph) from a reciprocal translocation, t(9;22) (q34;q11) causes a fusion gene, BCR-ABL, that encodes a constitutively active tyrosine kinase. Treatment of CML by imatinib is effective to control the tyrosyl phosphorylation of the protein related to the cell signaling. BCR-ABL mRNA is overexpressed in the minimal residual disease (MRD), known as an early sign of relapse. Between December 2005 and June 2008, we measured BCR-ABL mRNA levels in the bone marrow (BM) from patients by quantitative real-time polymerase chain reaction (RQ-PCR) in Aomori Prefectural Central Hospital. Eighty-six samples from 26 patients were collected. Among the 26 CML patients, 11 patients (42%) were in the pretreatment group. Seven (64%) of the 11 patients achieved complete molecular response (CMR). In the post-treatment group consisting of the remaining 15 patients, 9 (60%) patients achieved CMR. The patients receiving imatinib at a dose over 300 mg per day required 13 (6-77) months [median (range)] to achieve CMR. On the other hand, the patients receiving a dose below 300 mg per day required 29.5 (11-84) months [median (range)]. When BCR-ABL mRNA was detected during the treatment course of patients with CMR, careful observation of BCR-ABL mRNA was useful for tracking the clinical course of patients. In conclusion, the BCR-ABL mRNA level was useful for monitoring the clinical course in 26 patients with CML.

Pilocytic astrocytoma with histological malignant features without previous radiation therapy--case report.

Pilocytic astrocytoma sometimes transforms to a malignant type, and previous radiation therapy is considered to be a key factor. We report a case of pilocytic astrocytoma with histological malignant features without previous radiation therapy. A 21-year-old man presented a sudden onset of severe headache. Neuroimaging had detected a cystic mass in the posterior fossa at the age of one year without therapeutic intervention. On admission, computed tomography depicted a brain tumor in the posterior fossa with cystic components, intratumoral hemorrhage, and upward herniation. Urgent surgery was performed, and histological examination revealed some features of pilocytic astrocytoma but also broad necrosis, high cellularity, and MIB-1 labeling index of more than 20%. The histological diagnosis was pilocytic astrocytoma with malignant features. This tumor had continued a benign clinical course for 20 years, but had eventually transformed to a malignant type. Therefore, pilocytic astrocytoma may undergo spontaneous malignant transformation during its natural clinical course.

Peripheral pulmonary papillary adenocarcinoma with prominent cilia: report of a rare case that was difficult to diagnose preoperatively.

BACKGROUND: In pulmonary cytology, the existence of cilia is considered cytologic evidence of benign cells because it is generally considered that cilia could not be identified by light microscopic observation of pulmonary adenocarcinoma. However, we encountered a rare exceptional case of pulmonary adenocarcinoma with cilia. CASE: A 55-year-old woman with bloody sputum was admitted. Computed tomography revealed a lung tumor. Although transbronchial brushing cytology showed atypical cells suggestive of malignancy, some atypical cells had cilia, so we could not diagnose them as cancer cells. After antibiotic therapy, the tumor was surgically excised. Imprint cytology showed similar atypical ciliated cells. Histologically, the tumor was diagnosed as papillary adenocarcinoma with cilia and diffuse pleural dissemination was observed. Electron microscopic observation identified cilia. The patient died due to aggravation of cancer, which was confirmed on autopsy. CONCLUSION: This rare case of peripheral pulmonary papillary adenocarcinoma with cilia could not be diagnosed as cancer cell on cytology. Pulmonary papillary adenocarcinoma with cilia should be considered in the differential diagnosis of atypical cells in pulmonary cytology.

Anterior cranial fossa dural arteriovenous fistula with bilateral cortical drainers--case report.

A 58-year-old man presented with sudden onset of severe headache. Computed tomography demonstrated subarachnoid hemorrhage and right acute subdural hematoma. He had no neurological deficits. Cerebral angiography showed an anterior cranial fossa dural arteriovenous fistula (AVF) supplied by the bilateral ethmoidal arteries. A fistula was suggested on the right side, and the dural AVF drained into the superior sagittal sinus via the bilateral frontal cortical veins. Venous varix was observed at both drainage sites. Bifrontal craniotomy with right-side dural incision was performed and the fistula was interrupted. Postoperative angiography demonstrated a persistent fistula draining into the left cortical vein. Nineteen days later, bifrontal craniotomy with left-side dural incision was performed and the draining vein was completely coagulated with the aid of intraoperative angiography. Postoperatively, there was no detectable residual fistula. He was discharged without neurological deficits 2 weeks after surgery. The present case of anterior cranial fossa dural AVF with bilateral cortical drainers shows that drainer occlusion at two points may be needed for complete obliteration of the drainers because the fistulous connection may not be simple.

Efficacy of middle meningeal artery embolization in the treatment of refractory chronic subdural hematoma.

BACKGROUND: There are no established treatment procedures for repeatedly recurring chronic subdural hematoma (CSH). In this study, we discussed the efficacy of middle meningeal artery (MMA) embolization in preventing recurrence of CSH. METHODS: We performed superselective angiography of MMA in four patients who suffered from repeated recurrence of CSH. After angiography, we performed embolization of MMA with endovascular procedure. RESULTS: In all cases, superselective angiography of MMA revealed diffuse abnormal vascular stains that seemed to represent the macrocapillaries in the outer membrane of CSH. In all the patients, there were no recurrences or enlargements of CSH after the embolization of the MMA. CONCLUSION: MMA embolization can be an effective adjuvant procedure in preventing the recurrence of CSH.

Clinical availability of skin biopsy in the diagnosis of Parkinson's disease.

To determine whether skin biopsy is practically useful in the premortem diagnosis for Parkinson's disease (PD), we examined Lewy pathology in the skin of the chest wall and leg, obtained from 6-mm punch biopsies, using phosphorylated alpha-synuclein antibody in 20 patients with clinically diagnosed PD. Abnormal accumulation of alpha-synuclein was found in the chest skin of two (10%) of 20 patients, but not in the leg. Although skin biopsy combined with a conventional immunohistochemistry for alpha-synuclein is not sufficient as a diagnostic tool, we could firstly demonstrate Lewy pathology in premortem tissue. The skin remains to be a promising tissue to be examined for the premortem diagnosis of PD.

Serial angiography of dynamic changes of traumatic middle meningeal arteriovenous fistula: case report.

A 48-year-old woman suffered head trauma and presented with an acute epidural hematoma with a linear fracture of the right temporal bone across the middle meningeal groove. Initial angiography demonstrated no vascular abnormalities. Eight months later, she again suffered head trauma and computed tomography demonstrated traumatic subarachnoid hemorrhage. Right external carotid angiography revealed a middle meningeal arteriovenous fistula (AVF) which drained into the superficial sylvian veins via the sphenoparietal sinus. Serial angiography showed progressive dilation of the draining veins, but she refused surgical intervention and dropped out of our outpatient clinic. Fifteen years after the first head trauma, she presented with subarachnoid hemorrhage. Angiography demonstrated formation of venous aneurysms on the drainer of the AVF. The dilated superficial sylvian vein was removed together with the ruptured venous aneurysm. Histological examination of the drainer revealed an arterialized vein. The serial angiographic evaluations revealed dynamic changes of the traumatic middle meningeal AVF, including progressive dilation of the drainers, simplification of the drainage routes, and the formation of venous aneurysms, which presumably represents the entire natural course of traumatic middle meningeal AVF manifesting as hemorrhage. The present case of traumatic middle meningeal AVF with a deteriorating course suggests that surgical removal or embolization of the AVF is strongly indicated if follow-up angiography shows dilation of the drainers, which implies increased shunt flow.

WT1 mRNA level in peripheral blood is a sensitive biomarker for monitoring minimal residual disease in acute myeloid leukemia.

The Wilms' tumor gene 1 (WT1) encodes a transcription factor that is involved in normal cellular development and cell survival. WT1 mRNA is overexpressed in the minimal residual disease (MRD) of patients with hematopoietic malignancy patients, particularly acute myeloid leukemia (AML). MRD represents the condition with the low levels of leukemia cells in the bone marrow and is known as a sign of recurrence. In hematopoietic malignancies, definition of remission is based on the lack of MRD at submicroscopic level. Between December 2005 and June 2008, we started to measure WT1 mRNA levels in the peripheral blood (PB) from patients by quantitative real-time PCR in Aomori Prefectural Central Hospital. Three hundreds and eight samples from 95 patients were evaluated. The patients included AML (55 patients), acute lymphoblastic leukemia (11), myelodysplastic syndrome (20), malignant lymphoma (5), chronic myeloid leukemia (1), prostatic carcinoma (1), and leukopenia (2). Among the 55 AML patients, 21 patients were pretreated with remission induction therapy. In the clinical course of 21 patients, timely therapeutic approaches could be started for relapse by the early detection of WT1 mRNA overexpression before the morphological findings were apparent. Monitoring WT1 mRNA is helpful to identify patients at high-risk relapse. High overall survival rate (71.2%, 15/21, median: 24.6 months, range 1.1-35.6 months) was achieved in 3 years. The overall survival rate of 34 post-treatment patients was 61.7% (median: 23.5 months, range 0.13-126.5 months after treatment start). In conclusion, the WT1 mRNA level is a sensitive biomarker for monitoring MRD.

[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.

Hippocampal sclerosis with four-repeat tau-positive round inclusions in the dentate gyrus: a new type of four-repeat tauopathy.

Hippocampal sclerosis is defined as selective neuronal loss and gliosis of the hippocampus with heterogeneous etiologies, including neurodegenerative tauopathies. We report a 78-year-old woman who presented with depression, in whom postmortem examination revealed almost complete loss of neurons with gliosis in the subiculum and CA1-3 regions of the hippocampus and abundant neuronal cytoplasmic inclusions in the dentate gyrus. The inclusions were round, slightly basophilic and argyrophilic, resembling Pick bodies. However, they were Gallyas- and 4-repeat tau-positive, and 3-repeat tau- and ubiquitin-negative. To our knowledge, the histopathological features in this case were different from those in hippocampal sclerosis or 4-repeat tauopathies reported previously. It is likely that this case is a new variant of 4-repeat tauopathy presenting with hippocampal sclerosis.

Osteochondroma of the sella turcica presenting with intratumoral hemorrhage.

A 29-year-old man presented with a primary sellar turcica osteochondroma manifesting as intratumoral hemorrhage mimicking pituitary apoplexy. The patient suffered sudden onset of headache concomitant with vision loss in the left eye. Radiography and computed tomography detected destruction and calcification of the sellar turcica. Magnetic resonance imaging revealed a heterogeneously enhanced suprasellar mass that had elevated and compressed the optic chiasm. The preoperative diagnosis was hemorrhagic pituitary adenoma, craniopharyngioma, meningioma, or chordoma based on the signal heterogeneity of the lesion. To relieve the symptoms and make a definitive diagnosis, surgical removal via a basal interhemispheric approach was carried out. The tumor was not totally removed because of tight adhesion to the pituitary stalk, but postoperative ophthalmological examination revealed improvement of the visual disturbance. The histological diagnosis was osteochondroma based on the presence of mature chondrocytes and osteomatous tissue. Osteochondroma should be included in the differential diagnosis of tumors with acute hemorrhage in the sella turcica.

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.

Ectopic recurrence of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease): a case report.

Lhermitte-Duclos disease is a rare clinical entity characterized by slow deformation of the cerebellar lesion. A 53-year-old woman presented with Lherimitte-Duclos disease manifesting as ataxic gait, occipital headache, and loss of consciousness. Magnetic resonance imaging demonstrated striated and laminar pattern lesions in the right cerebellar hemisphere and vermis. She underwent subtotal removal of the vermis compressing the brainstem, but the cerebellar hemisphere appeared normal and was preserved. Histological findings were consistent with Lherimitte-Duclos disease. Two years later, magnetic resonance imaging revealed enlargement of the right hemispheric lesion. Intraoperative appearance of the hemisphere demonstrated marked enlargement of the folia. Subtotal removal was performed. Histological examination showed no malignant findings. Lhermitte-Duclos disease may recur, and should be carefully followed up as a low-grade neoplasm, not as a hamartomatous disorder.

Miliary brain metastasis presenting with dementia: progression pattern of cancer metastases in the cerebral cortex.

We report an autopsy case of an 82-year-old woman with progressive dementia due to miliary brain metastasis from lung adenocarcinoma. The patient presented with dementia 5 months prior to death and suddenly died of pulmonary hemorrhage. Postmortem examination revealed normal appearance of the brain. However, there were numerous foci of cancer metastasis in all parts of the brain on light microscopic examination. The carcinoma cells were located in the perivascular (Virchow-Robin) space and did not invade to the brain parenchyma. The carcinoma cells were also found in the subpial space. In the cerebral cortex, foci of metastasis appeared to spread in the following way: tiny foci of metastasis initially occur in the middle cortical layer, then spread to all layers through the perivascular space, and finally reach the subpial space and subcortical white matter. Although the junction between gray and white matter is a preferred site for usual brain metastasis, middle cortical layer was considered to be the initial site for metastasis in our patient. The perivascular pial sheath plays an important role for the development of miliary brain metastasis.

[Intracranial granulocytic sarcoma in a patient with acute myeloid leukemia].

Granulocytic sarcoma (GS) is extramedullary tumor composed of immature leukemic cells. GS is presenting usually as a complication during the course of hematologic neoplasm, such as acute myeloblastic leukemia as well as myeloproliferative and myelodysplastic syndrome. The tumor was also called chroloma based on the green color of the tumorous mass. Central nervous system manifestations of GS are extremely rare. We report a 41-year-old man with acute leukemia type M7, who developed GS in the right occipital lobe after complete remission was achieved. Operative findings revealed the color of the hard tumor was greenish, which suggested the tumor was chroloma. Histological findings showed the tumor was GS. The majority of reported cases of GS in acute myeloid leukemia were M2 FAB classification and have chromosome translocation. Our patient was M7 FAB classification, not have specific chromosome translocation. GS occurrence in AML: M7 patient was extremely rare. This is the first case report of AML: M7 with GS in the central nervous system.