Spontaneous haemorrhage into a large abducens nerve schwannoma: a case report.

Purpose: Abducens schwannomas are rare tumors that are not known to present with acute haemorrhage. We present a case of a 59 year-old female on warfarin who presented acutely with a sudden onset headache, nausea and photophobia. Neuroimaging revealed an acute haemorrhage into a lesion that entered Dorello's canal and was consistent with an abducens nerve schwannoma.Materials and methods: The patient's case notes, imaging, histology and operative recording were reviewed retrospectively to compile this case report.Results: The tumor was resected via a retro-mastoid approach with sacrifice of the abducens nerve. Removal of the haematoma intra-operatively provided more space in the surgical corridor to facilitate resection. Final histological examination confirmed the diagnosis of schwannoma and the patient made a good post-operative recovery.Conclusion: We conclude that accurate pre-operative radiological diagnosis can facilitate surgical planning and removal of haematoma at an early stage during the operation can create space and facilitate resection. Furthermore, abducens schwannoma should be considered in the differential diagnosis of any heamorrhagic cerebello-pontine angle lesion.

Case report of intraventricular schwannoma.

We describe the case of a 52 year old male presenting with subacute headache. Cranial imaging suggested haemorrhage into a parietal, partially intraventricular, space occupying lesion. The radiology was interpreted to be most consistent with a glioblastoma. The lesion was near totally resected. The histopathology was a WHO grade 1 schwannoma.

A Rare Case of Concurrent Herpes Simplex Encephalitis and Glioblastoma Multiforme.

Herpes encephalitis superimposed on an intracranial malignancy has previously been described mainly in the context of malignancy imitating infection or in the postoperative setting after neurosurgical intervention. We report a rare case of de novo presentation of concurrent herpes encephalitis and glioblastoma. A 63-year-old man presented with status epilepticus and subsequent magnetic resonance imaging (MRI) brain showed a right temporal enhancing lesion with mass effect. He underwent a craniotomy and debulking of this lesion, which on subsequent histology was positive for herpes simplex virus (HSV) antigens and HSV DNA was confirmed by polymerase chain reaction analysis. The sample however also had some hypercellular areas with atypical astrocytes. Our patient recovered well from surgery and was eventually commenced on acyclovir albeit with a delay of 3 weeks due to the initial diagnostic dilemma. However, he re-presented with lethargy and confusion a further 3 weeks later and an MRI scan showed recurrence of the temporal lesion with MR spectroscopy more suggestive of high-grade glioma. He, therefore, underwent a further debulking surgery and the histology revealed a WHO Grade 4 glioblastoma with some residual areas of inflammation. A diagnosis of 2 co-existing pathologies namely HSV encephalitis and glioblastoma was thus reached. Unfortunately, due to poor performance status, he could not undergo chemo-radiotherapy and died 8 months after presentation. Immuno-modulators, expressed locally and globally in glioma patients, are likely to render them susceptible to infections. There are an increasing number of reports of HSV encephalitis in the glioma setting postoperatively. However, we report a de novo presentation which has only been recognized once before in the 1970s. Recognition of HSV encephalitis in glioma patients in the de novo and also the postoperative context is important for commencing early treatment and preventing poor outcomes.

Management of elderly patients with glioblastoma-multiforme-a systematic review.

The management of elderly patients with glioblastoma-multiforme (GBM) remains poorly defined with many experts in the past advocating best supportive care, in view of limited evidence on efficacy of more aggressive treatment protocols. There is randomised evidence (NORDIC and NA-O8 studies) to support the use of surgery followed by adjuvant monotherapy with either radiotherapy (RT) using hypofractionated regimes (e.g. 36 Gy in 6 fractions OR 40 Gy in 15 fractions) or chemotherapy with temozolomide (TMZ) in patients expressing methylation of promoter for O6-methylguanine-DNA methyltransferase enzyme. However, the role of combined-modality therapy involving the use of combined RT and TMZ protocols has remained controversial with data from the EORTC (European Organisation for Research and Treatment of Cancer)-NCIC (National Cancer Institute of Canada) studies indicating that patients more than 65 years of age may not benefit significantly from combining standard RT fractionation using 60 Gy in 30 fractions with concurrent and adjuvant TMZ. More recently, randomised data has emerged on combining hypofractionated RT with concurrent and adjuvant TMZ. We provide a comprehensive review of literature with the aim of defining an evidence-based algorithm for management of elderly glioblastoma-multiforme population.

The Spectrum of C9orf72-mediated Neurodegeneration and Amyotrophic Lateral Sclerosis.

The discovery that a hexanucleotide repeat expansion in C9orf72 is the most numerous genetic variant of both amyotrophic lateral sclerosis and frontotemporal dementia has opened a rapidly growing field, which may provide long hoped for advances in the understanding and treatment of these devastating diseases. In this review we describe the various phenotypes, clinical and pathological, associated with expansion of C9orf72, which go beyond amyotrophic lateral sclerosis and frontotemporal dementia to include neurodegeneration more broadly. Next we take a step back and summarize the current understanding of the C9orf72 expansion and its protein products at a molecular level. Three mechanisms are prominent: toxicity mediated directly by RNA transcribed from the repeat; toxicity mediated by dipeptide repeat proteins translated from the repeat sequence; and haploinsufficiency resulting from reduced transcription of the C9orf72 exonic sequence. A series of exciting advances have recently described how dipeptide repeat proteins might interfere with the normal role of the nucleolus in maturation of RNA binding proteins and in production of ribosomes. Importantly, these mechanisms are unlikely to be mutually exclusive. We draw attention to the fact that clinical and pathological similarities to other genetic variants without a repeat expansion must not be overlooked in ascribing a pathogenic mechanism to C9orf72-disease. Finally, with a view to impact on patient care, we discuss current practice with respect to genetic screening in patients with and without a family history of disease, and the most promising developments towards therapy that have been reported to date.