Rahul Lath

Rosette-forming glioneuronal tumors: a report of two cases.

Rosette-forming glioneuronal tumor, a mixed glial and neuronal tumor, is a relatively new entity in tumors of the central nervous system, included in 2007 classification published by World Health Organization (WHO). It was initially described to occur in and around the fourth ventricle; however, recent case series have reported other locations also. Their occurrence in supratentorial and spinal locations has recently been reported. We report two cases of rosette-forming glioneuronal tumors, one in the midbrain and one in a suprasellar location, and review the literature.

Decompressive surgery for severe cerebral venous sinus thrombosis

BACKGROUND Cerebral venous sinus thrombosis (CVST) is one of the common causes of stroke in young people. Mortality in CVST, in addition to progressive thrombosis, is related to elevated intracranial pressure causing transtentorial herniation. The role of decompressive surgery in CVST is not well established. AIMS We report our experience with decompressive surgery in CVST and review the literature. SETTINGS AND DESIGN This is a retrospective study carried out in the Stroke Unit of a multispeciality tertiary care hospital in south India. MATERIALS AND METHODS The medical records of patients admitted with the diagnosis of CVST between December 2003 and July 2009 were reviewed. The clinical presentation, etiology, medical management, indications for surgery and outcomes were assessed for patients undergoing decompressive surgery. The sensorium was assessed using the Glasgow Coma Scale (GCS), while the outcome was assessed using the modified Rankin scale (mRS). Descriptive statistics were used as appropriate. RESULTS One hundred and six patients were admitted with the diagnosis of CVST during the study period. Eleven patients (10%) underwent decompressive surgical procedure. Indications for surgery included a low GCS at admission with large infarct on the computed tomography scan, mass effect and midline shift, clinical and radiological signs of transtentorial herniation, deterioration in the sensorium in spite of anti-edema measures and postthrombolysis hematoma. Eight patients (73%) had a good outcome while three patients (27%) died. Of the patients who died, two had a low GCS and bilaterally nonreactive pupils before the surgery while one had thrombosis of the deep venous system. CONCLUSION Decompressive surgery for patients with large cerebral venous infarcts is a life-saving procedure. Patients with CVST who develop clinical and radiological features of transtentorial herniation either at presentation or during the course of medical management may benefit from decompressive surgery.

Olfactory ensheathing cell tumor

Olfactory ensheathing cells (OECs) are found in the olfactory bulb and olfactory nasal mucosa. They resemble Schwann cells on light and electron microscopy, however, immunohistochemical staining can distinguish between the two. There are less than 30 cases of olfactory groove schwannomas reported in the literature while there is only one reported case of OEC tumor. We report an OEC tumor in a 42-year-old male and discuss the pathology and origin of this rare tumor.

Desmoplastic fibroma of the frontal bone

Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. We report a case of desmoplastic fibroma of the frontal bone in a young male.

Exosomes as a biomarker platform for detecting epidermal growth factor receptor–positive high-grade gliomas

OBJECTIVE High-grade glial brain tumors are often characterized by an elevated expression of the tumorigenic epidermal growth factor receptor variant III ( EGFRvIII). The authors sought to establish a clinically adaptive protocol as a noninvasive diagnostic tool for EGFRvIII detection through serum exosomes. METHODS Purity of serum exosome/RNA was confirmed by electron microscopy and flow cytometry and through an RNA bioanalyzer profile. EGFRvIII amplification was initially established by semiquantitative polymerase chain reaction in tumor tissues and exosomes. Diagnostic performance of EGFRvIII transcript in tissue versus exosome was determined using a 2 × 2 clinical table approach. Overall survival was determined using Kaplan-Meier analysis. RESULTS The EGFRvIII transcript was detected in 39.5% of tumor tissue samples and in 44.7% of their paired serum exosome samples; 28.1% of biopsy tumors coexpressed wild-type EGFR and EGFRvIII. Tissue EGFRvIII amplification served as the reference-positive control for its paired serum expression. The overall clinical sensitivity and specificity of semiquantitative exosome EGFRvIII polymerase chain reaction detection assay in serum were 81.58% (95% CI 65.67%-92.26%) and 79.31% (95% CI 66.65%-88.83%), respectively. Age, sex, tumor location, and side of the body on which the tumor was located had no effect on the detection rate of exosomal EGFRvIII transcript. EGFRvIII expression either in exosomes or tissue correlated with poor survival. CONCLUSIONS The authors established a serum-based method for detection of EGFRvIII in high-grade brain tumors that might serve as an optimal noninvasive method for diagnosing EGFRvIII-positive high-grade gliomas.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report and review of literature

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of central nervous system with distinct clinical and radiological features. The etiopathogenesis of this rare entity remains to be understood. The histopathological findings closely resemble chronic inflammatory diseases like sarcoidosis and malignancies like lymphoma. With advancements in serology, immunopathology and radiology CLIPPERS is identified as a distinct entity that differs considerably in its clinical presentation, immunopathology, radiological findings and response to steroids. We describe a case that presented to us with progressive quadriparesis and lower cranial nerve deficits whose radiological and pathological findings were consistent with CLIPPERS. The patient had a good outcome with long term immunosuppression.

Postmenopausal osteoporosis: Our experience

Introduction: There is very little published literature about experience with osteoporosis treatment from our country. Materials and Methods: It is a retrospective analysis of first 50 patients enrolled in our clinic for osteoporosis. Postmenopausal women with T score of less than -2.5 or history suggestive fragility fracture with supportive bone mineral density (BMD) were included. Patients having hypercalcemia, abnormal renal function, myeloma and on long-term steroids were also excluded. Results: Nearly 34% subjects were below the age of 60 years, 47% of subjects were between 60 and 70 years, whereas 18% were above 70 years. Nearly 6% had family history of osteoporosis s or history of osteoporotic fractures. Nearly 20% subjects had fracture prior to starting of any treatment. A total of 86% (40/46) had evidence of Vitamin D (VD) deficiency. Nearly 80% of patients were treated with bisphosphonates, 12% were treated with injectable bisphosphonates, and 8% were treated with teriperatide. Nearly 16% patients had duration of more than 5 years of experience with bisphosphonates. Follow up BMD was available in 25 subjects. BMD had improved significantly in 68% of subjects. In 24% the BMD was stable (the change was less than least significant change (LSC)). In 8% BMD had shown a significant decline while being on treatment. Conclusion: Postmenopausal osteoporosis occurs in relatively younger women in our country. Majority of them are VD deficient. Oral bisphosphonates is the most common used drug; it is fairly well tolerated and effective.

Hyponatremia in neurological diseases in ICU

Hyponatremia is the commonest electrolyte disturbance encountered in the neurological and neurosurgical intensive care units. It can present with signs and symptoms mimicking a neurological disease and can worsen the existing neurological deficits. Hyponatremia in neurological disorders is usually of the hypo-osmolar type caused either due to the Syndrome of Inappropriate Secretion of Anti Diuretic Hormone (SIADH) or Cerebral Salt Wasting Syndrome (CSWS). It is important to distinguish between these two disorders, as the treatment of the two differ to a large extent. In SIADH, the fluid intake is restricted, whereas in CSWS the treatment involves fluid and salt replacement.

Arteriovenous fistula of the filum terminale misdiagnosed and previously operated as lower lumbar degenerative disease.

Filum terminale arteriovenous fistula (FTAVF) presenting as a cause of failed back surgery syndrome is a rare entity. We report a 48-year-old male patient who presented with clinical features of a conus medullaris/cauda equina lesion. He had upper and lower motor neuron signs in both the lower limbs with autonomic dysfunction. The patient was misdiagnosed and was operated twice earlier for lumbar canal stenosis and disc prolapse. After reviewing his clinical and radiological findings a diagnosis of FTAVF was made. He underwent surgery and there was a significant improvement in his neurological functions. We discuss the case and review the literature on FTAVFs.