Skeletal muscle fibers produce B-cell stimulatory factors in chronic myositis.

Introduction: We aimed to identify B-cell-mediated immunomechanisms in inclusion body myositis (IBM) and polymyositis (PM) as part of the complex pathophysiology.

Materials And Methods: Human primary myotube cultures were derived from orthopedic surgery. Diagnostic biopsy specimens from patients with IBM (n=9) and PM (n=9) were analyzed for markers of B cell activation (BAFF and APRIL) and for chemokines that control the recruitment of B cells (CXCL-12 and CXCL-13).

Early Ectopic Recurrence of Craniopharyngioma in the Cerebellopontine Angle.

Ectopic recurrence of craniopharyngioma in the cerebellopontine angle after surgical resection of a suprasellar craniopharyngioma is rare. Thus, only 5 cases were reported with a delay ranging between 4 and 26 years after removal of the primary tumor. We report a unique case of ectopic recurrence of craniopharyngioma in the cerebellopontine angle, which occurred at only 4 months after surgical resection of the primary tumor.

Delayed distant spinal metastasis in thymomas.

Study Design: A case report.

Objective: To demonstrate 2 cases of delayed distant spinal metastasis from a thymoma with comprehensive immunohisto-chemical analysis.

Summary Of Background Data: Thymoma is a rare slow-growing cancer arising in the mediastinum.

Outcome of surgical decompression of spinal mass lesions in non-Hodgkin's lymphoma and plasmacytoma.

Objective: Surgical treatment for spinal mass lesions due to non-Hodgkin's lymphoma (NHL) or plasmacytoma is necessary only in rare instances. The purpose of this study was to investigate long-term outcome and quality of life of surgery combined with postoperative chemotherapy or radiochemotherapy.

Methods: The data of patients, who underwent spinal surgery for mass lesions in a 10-year periods were reviewed, identifying 10 patients with a histopathological diagnosis of NHL or plasmacytoma.

Mitogen-activated protein kinase-activated protein kinases 2 and 3 regulate SERCA2a expression and fiber type composition to modulate skeletal muscle and cardiomyocyte function.

The mitogen-activated protein kinase (MAPK)-activated protein kinases 2 and 3 (MK2/3) represent protein kinases downstream of the p38 MAPK. Using MK2/3 double-knockout (MK2/3(-/-)) mice, we analyzed the role of MK2/3 in cross-striated muscle by transcriptome and proteome analyses and by histology. We demonstrated enhanced expression of the slow oxidative skeletal muscle myofiber gene program, including the peroxisome proliferator-activated receptor gamma (PPARγ) coactivator 1α (PGC-1α).

Familial hypertrophic cardiomyopathy: functional effects of myosin mutation R723G in cardiomyocytes.

Familial Hypertrophic Cardiomyopathy (FHC) is frequently caused by mutations in the β-cardiac myosin heavy chain (β-MyHC). To identify changes in sarcomeric function triggered by such mutations, distinguishing mutation effects from other functional alterations of the myocardium is essential. We previously identified a direct effect of mutation R723G (MyHC723) on myosin function in slow Musculus soleus fibers.

Circulating endothelial cells as potential diagnostic biomarkers in primary central nervous system vasculitis.

Objective: Histological evidence is considered the only proof of primary central nervous system vasculitis (PCNSV). However, brain biopsy is often omitted or delayed because of the invasiveness and possible complications of the procedure. Circulating endothelial cells (CEC) were shown to be elevated in patients with active antineutrophil cytoplasmic antibody-associated vasculitis.

Pipestem capillaries in necrotizing myopathy revisited.

Pipestem-capillaries in necrotizing myopathy, have been reported as a feature of a distinct type of myopathy. Here, we analyze four muscle biopsy specimens from patients exhibiting endomysial fibrosis associated with pipestem capillaries using histological and electronmicroscopic techniques. However, only one case displayed all of the originally described features, including necrotic fibres, capillary thickening and lack of a significant lymphocytic inflammation, while one case exhibited striking capillary pathology with minimal necrosis and absence of inflammation, and the other two cases were accompanied by additional pathological features.

Progressive osteolytic calvarial lesions in children after minor head injury.

Objective: Osteolytic lesions of the skull in children have a broad differential diagnosis including congenital, inflammatory and neoplastic lesions. Progressive osteolysis of the skull secondary to head trauma is rare and has been poorly characterized.

Methods: The pediatric database at our hospital was screened for children with osteolytic lesions who had a previous mild head injury without fracture or dural tears.

Bilateral sphenoorbital hyperostotic meningiomas with proptosis and visual impairment: a therapeutic challenge. Report of three patients and review of the literature.

Objective: Bilateral hyperostotic sphenoorbital meningiomas are extremely uncommon. Due to extensive infiltration of the orbits and the frontotemporal skull base, often only a subtotal tumor resection is feasible. Thus far, no treatment algorithms have been suggested for this rare tumor entity.

Spontaneous regression of an intracerebral lymphoma (ghost tumor) in a liver-engrafted patient.

Posttransplantation lymphoproliferative disorder involving the central nervous system is a rare and serious complication associated with solid organ transplantation. We report a liver transplant recipient who noticed unbalance, dizziness, and headache 30 months after transplantation. Magnetic resonance imaging (MRI) showed a space-occupying lesion in the corpus callosum and adjacent parenchyma of the left hemisphere.

Common molecular cytogenetic pathway in papillary tumors of the pineal region (PTPR).

Primary papillary tumors of the central nervous system and particularly the pineal region are rare. Papillary tumor of the pineal region (PTPR) is a recently described neoplasm that has been formally recognized in the 2007 World Health Organization Classification of Tumors of the Nervous System. Because of their rarity, further pheno- and genotypical observations as well as therapeutic experience are necessary to differentiate PTPR from other primary or secondary papillary tumors of this region.

Spontaneous recovery from progressive multifocal leukoencephalopathy in a patient with non-active sarcoidosis.

We report the case of a 50-year-old female patient with non-active sarcoidosis and no kind of immunosuppression, admitted to our hospital because of increasing confusion and focal neurological deficits. Initially a tumor, herpes encephalitis, or neurosarcoidosis were suspected, but surprisingly biopsy revealed progressive multifocal leukoencephalopathy, additionally confirmed by JC-positive PCR in cerebrospinal fluid. Cases of sarcoidosis and progressive multifocal leukoencephalopathy have been reported before.

Treatment of recurrent trigeminal neuralgia due to Teflon granuloma.

Recurrent trigeminal neuralgia after microvascular decompression (MVD) may be due to insufficient decompression, dislocation of the implant to pad the neurovascular contact, or the development of granuloma. Here, we report on our experience with Teflon granuloma including its treatment and histopathological examination. In a series of 200 patients with trigeminal neuralgia MVD was performed with Teflon felt according to Jannetta's technique.

Microtransplantation of dopaminergic cell suspensions: further characterization and optimization of grafting parameters.

Intracerebral transplantation of dopaminergic (DA) cells is currently further explored as a potential restorative therapy for Parkinson's disease (PD). However, before they can be considered for a more widespread clinical use a number of critical issues have to be resolved, including an optimized transplantation protocol. This study has been performed in a rat 6-hydroxydopamine model of PD and is based on the microtransplantation approach.

Exophytic giant cell glioblastoma of the medulla oblongata.

Giant cell glioblastoma is a rare variant within the spectrum of glioblastoma multiforme (GBM) tumors. A giant cell glioblastoma may be associated with a better prognosis than the common type of GBM after combined treatment involving tumor resection and radiochemotherapy. A giant cell glioblastoma may occur at various sites in the brain and spinal cord.

Cavernous angioma of the geniculate ganglion.

Intracranial extraaxial cavernous angiomas are rare vascular malformations. Their occurrence at the geniculate ganglion of the facial nerve within the temporal bone is exceptional. The authors describe a 35-year-old man who developed a slowly progressing facial palsy.

A case of multiple primary tumors of the anterior skull base.

We report a case of synchronous olfactory bulb meningioma and undifferentiated carcinoma of the nose and paranasal sinuses that involved and destroyed the anterior skull base and mimicked intracranial invasion by a carcinoma. The heterogeneity of tissue types in the skull base gives rise to a diverse variety of benign and malignant neoplasms which have totally different prognoses. Synchronous development of benign and malignant primary tumors both originating from and involving the skull base at the same location is very rare and may cause confusion for both the skull base surgeon and neuroradiologist.

Erythropoietin and erythropoietin receptor expression in vestibular schwannoma: potential role in tumor progression.

Hypothesis: Hypoxia-inducible factor (HIF)-1alpha, erythropoietin (Epo), Epo receptor (EpoR), and bcl-2 are expressed in both sporadic unilateral vestibular schwannomas (VSs) and those associated with neurofibromatosis Type 2, and the expression data correlate with clinicopathological tumor features including microvessel density and Ki-67-labeling index.

Background: Erythropoietin expression is regulated by the transcription factor, HIF-1alpha. Erythropoietin signaling via EpoR results in stimulation of cell proliferation and elevated expression of the antiapoptotic protein, bcl-2, and then inhibition of apoptosis.

Transition from meningeal melanocytoma to primary cerebral melanoma. Case report.

The authors describe the first case of an intracranial transition of a melanocytoma into a primary malignant melanoma within a short time. A 37-year-old woman presented with progressive brainstem syndrome due to a tumor, originally diagnosed and treated 12 years earlier, that extended from the petroclival area to the anterior craniocervical junction. The histological workup following subtotal tumor resection of the initial tumor had revealed the typical features of a fibrous melanocytic meningioma without increased proliferation.

Late metastasis of breast adenocarcinoma into internal auditory canal and cerebellopontine angle: case report.

A case of metastasis of breast adenocarcinoma into the internal auditory canal (IAC) and cerebellopontine angle (CPA) is presented, which appeared 16 years after primary tumor had been treated by surgery and radiation therapy. The 66-year old patient was considered cured from the primary disease, when she started with a rapidly developing hearing loss and intermittent facial palsy. Magnetic resonance image (MRI) displayed an intra- and extracanalicular tumor mass, which radiologically resembled a vestibular schwannoma.

[The value of radiotherapy in treatment of meningeal melanocytoma].

Background: Meningeal melanocytoma is described as rare benign lesion with a high risk of recurrence. There are no well-substantiated treatment recommendations in the literature. Only case reports have been published by now.