A Novel Case of Primary Conus Medullaris Epithelioid Glioblastoma with Gliosarcomatous Differentiation.

Intramedullary location is seldom seen in spinal cord neoplasms. Ependymomas and astrocytomas comprise the vast majority of these intramedullary lesions. Primary spinal origin is rarely seen in gliosarcomas. No epithelioid glioblastomas have been reported in the spine. We describe the case of an 18-year-old male who presented with symptoms suggestive of a spinal mass lesion. Magnetic resonance imaging revealed a homogeneous intradural-intramedullary lesion involving the conus medullaris. Biopsy of the lesion showed a unique morphology comprising gliosarcoma and epithelioid glioblastoma differentiation, supported by relevant immunohistochemistry. The prognosis of such an entity is expected to be poor. However, the presence of mutant BRAF V600E, as seen in the current case, and the availability of targeted therapy against it are expected to improve the prognosis.

Metastases from renal cell carcinoma-Report of three unpredictable cases and literature review.

The capacity to metastasize after long periods of discovery and resection of the primary renal lesion or to present as metastasis with undisclosed primary lesions grant renal cell carcinomas (RCC) a formidable and unpredictable behavior. We report three unusual cases of metastasis from RCC. The first patient presented with metastatic clear cell adenocarcinoma in the right shoulder muscles, with unknown primary and revealed an undetected clear cell right RCC on radiology. The second patient presented with a hemorrhagic nasal metastasis of RCC. A history of nephrectomy for RCC, 10 years prior to the development of nasal metastasis was elicited. The third patient presented with axillary and later abdominal wall metastasis of papillary RCC. He had a history of RCC 3 years prior to the discovery of metastasis. RCC has the propensity to appear as a metastatic carcinoma with unknown primary, requiring exclusion of several diagnostic pathologic entities. The appearance of metastasis many years after disease quiescence is challenging and clinicians need to be sensitized to the need for long-term surveillance in RCC. Though the prognosis remains poor, immune checkpoint inhibitors are currently the modalities of choice in such cases.

Pathological, ultrasonographic, and electrophysiological characterization of clinically diagnosed cases of pure neuritic leprosy.

A subset of neuritic form of leprosy, called pure neuritic leprosy (PNL), seen in a minority of leprosy patients, is characterized by peripheral neuropathy without skin lesions and an absence of acid-fast bacilli on skin smears. Patients with PNL are often started on drug therapy without confirmation of diagnosis. We, therefore, did a prospective study of clinically diagnosed PNL patients with correlation of ultrasonographic and biopsy findings. A total of 100 consecutive patients with PNL, diagnosed according to the consensus case definition, were included in the study. All patients underwent nerve conduction study, peripheral nerve ultrasonography, and sural nerve biopsy. Multiple mononeuropathies were present in 75% of cases, mononeuropathy in 18%, and polyneuropathy in the remaining 7%. Compared to clinical examination, ultrasonographic assessment of the peripheral nerves was not only better at the detection of thickening but also helped in characterization of their fascicular architecture, echogenicity, and vascularity. A total of 32 cases were confirmed on nerve biopsy, out of which 75% had demonstrable lepra bacilli. Cranial nerve involvement, presence of trophic ulcers, and bilateral thickening of the great auricular nerve were significantly associated with the positivity of lepra bacilli. A significant improvement in the disability score happened after multidrug therapy. A comprehensive electrophysiologic, ultrasonographic, and histological evaluation may be helpful in establishing a diagnosis of PNL with greater confidence, while ruling out other non-leprosy diagnoses.

The spectrum of malignancies presenting with neurological manifestations: A prospective observational study.

INTRODUCTION: A neurological consultation is needed in nearly 45% of patients suffering from cancer. The present study was planned to evaluate the clinical, radiological and histopathological spectrum of patients with an underlying malignancy and presenting with a neurological complaint. MATERIALS AND METHODS: We prospectively evaluated all patients provisionally diagnosed either with a primary or secondary malignancy of the brain on the basis of clinical, radiological and/or histopathological features. RESULTS: A total of 155 patients were enrolled from a total of 4893 admissions done from January 2015 to July 2016. The common presenting symptoms were headache, back pain and paraparesis. Around 26% of patients presented with an altered sensorium, 19.4% with seizures and 21% had at least one cranial nerve involvement. The most common site of involvement was the brain noted in 49.7% of patients. Primary malignancies constituted 78 cases (50.7%) while secondary malignancies included 77 cases (49.3%). Magnetic resonance imaging (MRI) revealed 92 (59.4%) intra-axial lesions and 59 (38.1%) extra-axial lesions, with five cases having both. The most common diagnoses were intra-cerebral metastases and glioblastoma (intra-axial), and vertebral metastases and meningioma (extra-axial). Histopathological confirmation was obtained in 59 patients (38.1%) with 12 primary and 47 secondary lesions. Ten (6.45%) patients had an unknown primary with secondary metastases. The three most common histopathologically confirmed diagnoses were adenocarcinoma lung, plasma cell dyscrasia and adenocarcinoma prostate. CONCLUSION: Primary neurological consultations with an unknown primary are common hence a high index of suspicion can prevent an inadvertent delay in the diagnosis and appropriate treatment of a malignant lesion. Developing a neuro-oncology register may help us in gaining more insight into such situations.

Etiologic spectrum and prognosis in noncompressive acute transverse myelopathies: An experience of 80 patients at a tertiary care facility.

INTRODUCTION: We evaluated the spectrum of acquired demyelinating and inflammatory disorders in patients presenting with an acute transverse myelopathy. We also studied differences between an acute idiopathic transverse myelitis and myelitis resulting from other etiologies. MATERIALS AND METHODS: Eighty consecutive patients with acute transverse myelopathy were included. At inclusion, clinical profile, serum and cerebrospinal fluid parameters, brain and spinal cord magnetic resonance imaging, and visual evoked potentials were obtained. All patients were given methylprednisolone therapy. Patients were followed up for 6 months. Outcome was assessed using modified Barthel index. A modified Barthel index score of ≤12 indicated a poor prognosis. RESULTS: Majority (n = 49; 61.25%) of patients had idiopathic acute transverse myelitis. Eleven cases had neuromyelitis optica spectrum disorders (8 had anti-aquaporin antibody positivity). Multiple sclerosis was diagnosed in 7 cases. Eight cases had infectious or parainfectious myelitis. Longitudinally extensive transverse myelitis was noted in 66 (82.5%) patients. Seventeen patients had abnormalities in the brain. Majority of patients improved following methylprednisolone therapy. On univariate analysis, delay in administering methylprednisolone therapy, poor modified Barthel index at discharge, and extensive cord involvement were associated with severe residual disability. On multivariate analysis, delayed initiation of methylprednisolone was identified as a poor prognostic factor. CONCLUSION: A variety of inflammatory, infective, demyelinating, and autoimmune disorders present with acute transverse myelopathy. Early institution of methylprednisolone reduces the disability in these patients.