Intracranial necrotizing granuloma caused by Cladophialophora bantiana.

The dematiaceous fungi are a group of pigmented hyphal yeasts best known as agents of chronic skin and subcutaneous infections. Extracutaneous disease is exceptional. We report herewith a case of necrotizing granuloma caused by one member of the group, Cladophialophora bantiana. This organism is fully capable of invading the nervous system of apparently immunocompetent hosts and in many cases does so in the absence of demonstrable foci of extraneural infection. It has also been reported to be the most frequently found causative organism in the central nervous system phaeohyphomycosis. C. bantiana has several older names in the literature including Clodosporium trichoides, C. bantianum and Xylohypha bantiana. Patients require treatment with a combination of medical and surgical therapy. Rarity of the case and the usefulness of a simple diagnostic method such as smear cytology, which lead us to the diagnosis, is highlighted by the report.

Hedgehog signaling pathway is active in GBM with GLI1 mRNA expression showing a single continuous distribution rather than discrete high/low clusters.

Hedgehog (Hh) signaling pathway is a valid therapeutic target in a wide range of malignancies. We focus here on glioblastoma multiforme (GBM), a lethal malignancy of the central nervous system (CNS). By analyzing RNA-sequencing based transcriptomics data on 149 clinical cases of TCGA-GBM database we show here a strong correlation (r = 0.7) between GLI1 and PTCH1 mRNA expression--as a hallmark of the canonical Hh-pathway activity in this malignancy. GLI1 mRNA expression varied in 3 orders of magnitude among the GBM patients of the same cohort showing a single continuous distribution-unlike the discrete high/low-GLI1 mRNA expressing clusters of medulloblastoma (MB). When compared with MB as a reference, the median GLI1 mRNA expression in GBM appeared 14.8 fold lower than that of the "high-Hh" cluster of MB but 5.6 fold higher than that of the "low-Hh" cluster of MB. Next, we demonstrated statistically significant up- and down-regulation of GLI1 mRNA expressions in GBM patient-derived low-passage neurospheres in vitro by sonic hedgehog ligand-enriched conditioned media (shh-CM) and by Hh-inhibitor drug vismodegib respectively. We also showed clinically achievable dose (50 µM) of vismodegib alone to be sufficient to induce apoptosis and cell cycle arrest in these low-passage GBM neurospheres in vitro. Vismodegib showed an effect on the neurospheres, both by down-regulating GLI1 mRNA expression and by inducing apoptosis/cell cycle arrest, irrespective of their relative endogenous levels of GLI1 mRNA expression. We conclude from our study that this single continuous distribution pattern of GLI1 mRNA expression technically puts almost all GBM patients in a single group rather than discrete high- or low-clusters in terms of Hh-pathway activity. That is suggestive of therapies with Hh-pathway inhibitor drugs in this malignancy without a need for further stratification of patients on the basis of relative levels of Hh-pathway activity among them.

Staging in giant vestibular schwannoma surgery: A two consecutive day technique for complete resection in basic neurosurgical setups.

INTRODUCTION: Vestibular schwannomas constitute 8% of all intracranial tumors. A majority of vestibular schwannomas are sporadic and unilateral. Giant vestibular schwannomas are seen in our country due to the late diagnosis and long duration of symptoms before diagnosis. These giant schwannomas are challenging to manage as most of the patients are having brainstem compression. MATERIALS AND METHODS: Twelve cases of a giant vestibular schwannoma were operated in our department between May 2011 and December 2012. Vestibular schwannomas with a maximal diameter of more than 4 cm were defined as a giant vestibular schwannoma. All the patients had a unilateral vestibular schwannoma. Performance status of all the patients were graded as per the Karnofsky performance score. Pre-operative assessment of 5(th), 7(th), 8(th) and lower cranial nerve status was done in all cases. Ventriculoperitoneal shunting was done pre-operatively in all cases. All patients were operated through retromastoid suboccipital craniectomy and retrosigmoid approach. These patients were operated in two stages in two consecutive days with overnight elective ventilation in ICU. Ultrasonic aspirator and nerve monitoring techniques were not used. RESULTS: Giant acoustic schwannomas can be safely resected completely by a staged resection on two consecutive days without any increased morbidity or mortality. This technique may be employed to achieve complete resection of such lesions without deterioration of facial nerve function in institutions which do not have advanced facilities like nerve monitoring or ultrasonic aspirator.

Bobbling head in a young subject.

Bobble-head Doll Syndrome is a rare and unique movement disorder found in children. Clinically, it is characterized by a to and fro or side to side movement of the head at the frequency of 2 to 3 Hz. It is mostly associated with cystic lesions around the third ventricle, choroid plexus papilloma, aqueductal stenosis and other rare disorders. An eleven year old child presented in the outpatient department with continuous to and fro movement of the head and declining vision for the last one month. MRI Scan showed a large contrast-enhanced lesion in the region of the third ventricle along with gross hydrocephalus. Ventriculo-peritoneal shunt was inserted and the movements of the head disappeared completely. Bobble-head doll syndrome is a rare condition and therefore this case is presented and the literature reviewed.