Predictors of Outcome Following Interventions for Ruptured Intracranial Aneurysms in an Emerging Health Institution in West Bengal: A 6-Year Experience.

BACKGROUND: Microsurgical clipping and endovascular coiling have remained over the past half-century, the main options for definitive treatment of ruptured intracranial aneurysms. However, recent advances in endovascular techniques have broadened their application stimulating much debate regarding the usefulness of microsurgical options. For locations with limited capacity or evolving endovascular service, however, microsurgical treatment offers not only cost-effective and durable options but also oftentimes the only option available for most patients. MATERIALS AND METHODS: This was a single institution questionnaire-based retrospective study from West Bengal India. It was performed on patients with aneurysmal rupture managed from June 2010 to October 2016. Data analysis was performed with SPSS for Windows, version 21. RESULTS: One hundred and ten patients were studied. The mean age was 50.8 years (standard deviation [SD] ±13.5) for females and 50.2 (SD ± 12.4) for males (P = 0.8112, t-test). Ninety (81.8%) patients received microsurgical clipping. Microsurgical options such as wrapping, bypass, and excision were performed for 9 (8.2%) patients. Coiling was performed for 11 (10%) patients. ACOM was the most common site for ruptured aneurysm. Ictus-intervention interval (χ2 = 10.034, P = 0.007) and multiple surgical procedures (χ2 = 8.9341, P = 0.003) were the significant outcome determinants. Overall, 72.7% of those treated had a good outcome (Glasgow Outcome Score of 4 and 5). The good outcome was higher among microsurgery groups (73.7%) compared to the coiling group (63.6%) but this difference did not achieve statistical significance (χ2 = 0.46 P = 0.4976). CONCLUSION: Microsurgical options achieved good outcomes in the management of ruptured aneurysms in our series. Their availability makes them first-line options for low-income settings.

Neurocutaneous Melanosis with Hydrocephalus and Dandy-Walker Variant.

Neurocutaneous melanosis (NCM) is one of the rare, congenital, noninheritable phakomatoses characterized by the presence of large and/or multiple congenital melanocytic cutaneous nevi associated with intracranial leptomeningeal melanocytosis. NCM usually presents before 2 years of age. So far 302 cases have been reported in literature. We report a case of NCM presenting with obstructive hydrocephalus and Dandy-Walker Variant in a young adult.

Nondysraphic cervicomedullary intramedullary lipoma.

Spinal cord lipomas are usually associated with spinal dysraphism and is most common in lumbosacral region. Spinal intradural lipoma is a rare condition accounting for less than 1% of spinal cord tumours and is most prevalent in cervicodorsal region. Intramedullary spinal cord lipoma of cervical spine not associated with spinal dysraphism is one of the rarest lesions. They usually present insidiously with slowly progressive myelopathic deficits. We present a case of nondysraphic intramedullary spinal cord lipoma with exophytic component and intracranial extension. A 30 year male patient presented with wasting of muscles of left shoulder and left forearm with resultant weakness for last two months and myelopathic signs. Magnetic resonance imaging revealed a T1 hyperintense , T2 hypointense lesion on the dorsal aspect of cervical spinal cord with intracranial extension and exophytic component. There was no contrast enhancement, fat suppression image indicated a lipoma. The patient underwent cervical laminectomy with foramen magnum decompression with subtotal resection of intramedullary lipoma. Histopathology examination confirmed the diagnosis of lipoma. Subtotal resection of intramedullary lipoma is a reasonable and acceptable surgical option considering the benign nature of lesion and high probability of neurological deterioration due to dense adhesion between lipoma and neural tissue.

Chronic Pseudomonas aeruginosa cervical osteomyelitis.

Pseudomonas aeruginosa is a rare cause of osteomyelitis of the cervical spine and is usually seen in the background of intravenous drug use and immunocompromised state. Very few cases of osteomyelitis of the cervical spine caused by pseudomonas aeruginosa have been reported in otherwise healthy patients. This is a case presentation of a young female, who in the absence of known risk factors for cervical osteomyelitis presented with progressively worsening neurological signs and symptoms.

Variant allele frequency enrichment analysis in vitro reveals sonic hedgehog pathway to impede sustained temozolomide response in GBM.

Neoplastic cells of Glioblastoma multiforme (GBM) may or may not show sustained response to temozolomide (TMZ) chemotherapy. We hypothesize that TMZ chemotherapy response in GBM is predetermined in its neoplastic clones via a specific set of mutations that alter relevant pathways. We describe exome-wide enrichment of variant allele frequencies (VAFs) in neurospheres displaying contrasting phenotypes of sustained versus reversible TMZ-responses in vitro. Enrichment of VAFs was found on genes ST5, RP6KA1 and PRKDC in cells showing sustained TMZ-effect whereas on genes FREM2, AASDH and STK36, in cells showing reversible TMZ-effect. Ingenuity pathway analysis (IPA) revealed that these genes alter cell-cycle, G2/M-checkpoint-regulation and NHEJ pathways in sustained TMZ-effect cells whereas the lysine-II&V/phenylalanine degradation and sonic hedgehog (Hh) pathways in reversible TMZ-effect cells. Next, we validated the likely involvement of the Hh-pathway in TMZ-response on additional GBM neurospheres as well as on GBM patients, by extracting RNA-sequencing-based gene expression data from the TCGA-GBM database. Finally, we demonstrated TMZ-sensitization of a TMZ non-responder neurosphere in vitro by treating them with the FDA-approved pharmacological Hh-pathway inhibitor vismodegib. Altogether, our results indicate that the Hh-pathway impedes sustained TMZ-response in GBM and could be a potential therapeutic target to enhance TMZ-response in this malignancy.

Primary isolated amyloidoma of the lumbar spine causing neurological compromise: case report and literature review.

OBJECTIVE AND IMPORTANCE: We describe a patient with cauda equina compression secondary to amyloidoma to alert other clinicians to this rare cause of a compressive epidural lesion. It is the fourth published report of primary lumbar amyloidoma causing neurological compromise. CLINICAL PRESENTATION: A 53-year-old, previously fit salesman presented with several years history of back pain and recent weakness, especially in the left leg. He also had numbness and tingling radiating down the left leg. On examination, the left knee jerk was diminished, and both ankle jerks were absent. Power was reduced to 4/5 in ankle dorsiflexion bilaterally. A magnetic resonance imaging scan of the lumbar spine revealed an extradural mass, compressing the theca at L3-L4. This was enhancing in T1-weighted images and had low signal intensity in T2-weighted images. There was no evidence of systemic amyloidosis or development of multiple myeloma. INTERVENTION: L3-L4 laminectomy was performed, with removal of the epidural mass. The patient had complete resolution of sciatica and regained normal power in both lower limbs. There was no evidence of any recurrence at 1-year follow-up. CONCLUSION: Lumbar epidural amyloidoma is an extremely rare cause of cauda equina compression. Clinical presentation can be nonspecific, and radiologically, it can be indistinguishable from a tumor. Diagnosis is made at histological examination of a Congo red-stained section under polarized light. Complete resection of the localized epidural amyloid mass is associated with a good prognosis.