Manish Ranjan

Isolated cranio-spinal involvement of Rosai-Dorfman disease: case report

Rosai-Dorfman disease is a rare non-neoplastic lymphoproliferative condition. It commonly affects individuals between third and fifth decades, most common presentation being in the form of massive painless cervical lymphadenopathy with fever, weight loss and malaise. Isolated intracranial involvement is rare seen in less than 5% of patients with extranodal involvement. We present a patient with isolated contiguous cranio-spinal involvement who presented to us with remitting symptoms.

Granulomatous and lymphocytic hypophysitis - are they immunologically distinct?

Hypophysitis includes three histopathologically distinct entities – granulomatous, lymphocytic and xanthomatous forms. Etiopathogenesis and the immunological differences among these is not well characterized. This study aims to explore the immunopathogenesis of granulomatous and lymphocytic forms of hypophysitis. Demographic, clinical, endocrine function and radiological features of 33 histologically confirmed cases of hypophysitis were reviewed. Immunophenotyping of inflammatory component was performed in 13/33 cases. Visual disturbances (46%), headache (36%), polyuria/polydipsia (6%), menstrual disturbance (6%) and galactorrhoea (6%) were the frequent presenting symptoms. Endocrine abnormalities were noted in 11/18 cases evaluated (61%). Hypothyroidism was the most common endocrine abnormality (33.33%) followed by hyperprolactinaemia (22%) and hypocortisolism (16.66%). On neuroimaging, sellar mass with variable contrast enhancement was observed. On histology, granulomatous hypophysitis (GH) was more common (84.84%) than lymphocytic hypophysitis (LH) (15.15%). In GH, the infiltrate had almost equal proportions of CD3+ T cells and CD68+ histiocytes. Cytotoxic T cells (CD8+) predominated [CD4:CD8 < 1]. CD20+ B cell component ranged from <5% to 50%. Fibrosis, necrosis and giant cells accompanied GH. LH in contrast, had CD4+ T‐helper cell predominance [CD4: CD8 > 1]. CD68+ histiocytes constituted <20% and CD20+ B cells, 5–40% of the infiltrates. In conclusion, GH revealed cytotoxic T cell and histiocyte rich infiltrate in contrast to CD4+ T‐cell predominance in LH suggesting that the two forms have distinct immunological mechanisms in evolution, an autoimmune process in LH and type IV hypersensitivity response in GH.

Kümmell's disease - uncommon or underreported disease: A clinicopathological account of a case and review of literature.

Kümmells disease is a rare form of vertebral body osteonecrosis, which develops as a delayed post-traumatic event. It is infrequently reported in literature and to the best of our knowledge, has not been reported from India. We describe the clinical, radiological, and pathological features of a case occurring in a 60-year-old man and relevant brief review of the literature of this rare disease. Its close resemblance to more commonly occurring bony tuberculosis poses a diagnostic dilemma particularly in developing country like India, where tuberculosis is endemic. Awareness of this entity, though rare, is essential to avoid unnecessary diagnostic work up and treatment.

Fourth ventricular ependymoma with a distant intraventricular metastasis: Report of a rare case

Ependymoma is one of the uncommon tumors of the central nervous system (CNS) in the adult age group. These tumors have a distinct propensity for metastasis, both within and outside the CNS. However, dissemination at the time of first presentation and retrograde dissemination of the tumor is rare. We report the case of a patient with fourth ventricular anaplastic ependymoma who presented with left lateral ventricular metastasis which was anatomically different from the primary tumor. We describe the clinic-pathological detail of the patient and discuss the probable pathophysiological basis for this rare presentation and its significance in management of the patient.

Factors predicting progression of low-grade diffusely infiltrating astrocytoma

BACKGROUND Low-grade diffuse astrocytoma (DA) is considered benign tumor (World Health Organization [WHO] grade II), but it has an inherent tendency for malignant progression, which is quite variable. AIM To identify malignant progression in an individual case of DA, we studied the clinico-radiological and immunohistochemical factors and correlated with progression of DA at a dedicated tertiary level neurosciences centre NIMHANS, Bangalore, India. PATIENTS AND METHODS Consecutive adult patients who had undergone tumor decompression for lobar supratentorial DA at our institute from 1994 to 1998 were retrospectively selected and followed up for clinico-radiological progression. The clinico-radiological and histomorphological features were studied. With the use of immunohistochemistry, proliferation index [MIB-1 labeling index (LI)], p53 protein expression, microvessel density (MVD) count [assessed using anti-CD34 antibody] were analyzed and correlated with progression-free survival (PFS) Results: There were 13 patients. Mean age was 34 years. The most common presenting symptom was seizures. The median follow-up was 54 months. There were four recurrences, with median interval of 75 months. Eight patients received radiotherapy. Younger patients (<40 years), seizure as the presenting symptom and postoperative radiotherapy were associated with longer PFS, while gemistocytic morphology (>20% gemistocytic cells), MVD value >20 correlated with shorter PFS, albeit statistically insignificant. MIB1 LI did not correlate with recurrence pattern. Moreover, p53 LI > 10% correlated with early progression (P = 0.04). CONCLUSION Our study highlights some of the clinical, histological and immunohistochemical parameters that predict progression on DA. Validation on a larger sample may be useful to plan appropriate treatment in an individual case.

Diffusion tensor imaging in evaluation of posterior fossa tumors in children on a 3T MRI scanner

Context: Primary intracranial tumors in children are commonly located in the posterior fossa. Conventional MRI offers limited information regarding the histopathological type of tumor which is essential for better patient management. Aims: The purpose of the study was to evaluate the usefulness of advanced MR imaging techniques like diffusion tensor imaging (DTI) in distinguishing the various histopathological types of posterior fossa tumors in children. Settings and Design: DTI was performed on a 3T MRI scanner in 34 untreated children found to have posterior fossa lesions. Materials and Methods: Using third party software, various DTI parameters [apparent diffusion coefficient (ADC), fractional anisotropy (FA), radial diffusivity, planar index, spherical index, and linear index] were calculated for the lesion. Statistical Analysis Used: Data were subjected to statistical analysis [analysis of variance (ANOVA)] using SPSS 15.0 software. Results: We observed significant correlation (P < 0.01) between ADC mean and maximum, followed by radial diffusivity (RD) with the histopathological types of the lesions. Rest of the DTI parameters did not show any significant correlation in our study. Conclusions: The results of our study support the hypothesis that most cellular tumors and those with greater nuclear area like medulloblastoma would have the lowest ADC values, as compared to less cellular tumors like pilocytic astrocytoma.

Primary pyogenic spinal epidural abscess: How late is too late and how bad is too bad? – A study on surgical outcome after delayed presentation

Spinal epidural abscess is a rare clinical entity with considerable morbidity. Even with prompt diagnosis and treatment, many patients are left with persistent residual neurological deficits. The present study details the outcome in 23 patients of primary pyogenic spinal epidural abscess, addressing the outcome following late presentation at a neurological facility. At presentation only 2 patients had relatively preserved neurological status. Eleven patients were paraplegic. All the patients underwent laminectomy and evacuation of abscess. A good functional outcome was observed in almost half of the patients, and there was a significant reduction in the number of the patients with severe disability. Factors influencing the outcome are described in this study.

Endoscopic intervention for the fourth ventricular neurocysticercal cyst: What should be the optimum therapeutic approach?

Abstract Objective. Fourth ventricular neurocysticercal cyst (FVNCC) usually presents with acute hydrocephalus, requiring surgery. Though endoscopic intervention is preferable, there is no consensus on the method and/or the extent of endoscopic intervention. We share our experience of endoscopic intervention for FVNCC and propose an algorithm of management. Method. We reviewed the clinicoradiological details of consecutive patients, who underwent endoscopic intervention for the FVNCC at our institute from 1998 to 2009. Details of cyst excision, endoscopic intervention(s), cerebrospinal fluid (CSF) diversion (internal and external), complications and the outcome were analyzed. Results. There were 21 patients. Cyst could be totally excised in 13 patients, while two patients had only partial excision of cyst. Excision of cyst could not be done in five patients due to ependymitis/adhesion, intraventricular hemorrhage (IVH) and poor visibility of CSF due to hazy CSF. One patient only underwent endoscopic third ventriculostomy (ETV), as cyst excision was not planned in view of calcified FVNCC. Three patients had only cyst excision, as the sole endoscopic intervention, while 12 patients underwent ETV along with excision. Two patients had symptomatic periaqueductal injury with partial recovery (one each with rigid and flexible scope). The mean available follow-up was 22 months. Shunt was avoided in 90% of patients. All patients who are available for follow-up are asymptomatic, irrespective of the type of endoscopic intervention and extent of cyst excision status. Conclusion. Relieving the acute symptomatic hydrocephalus by endoscopic internal CSF diversion (ETV) should be the primary therapeutic goal. Cyst excision (partial or total) should be attempted, only where feasible. The rigid scope is safe and satisfactory for the endoscopic intervention for FVNCC.

Cerebellar tuberculoma presenting as haematoma--a case report and pathophysiological consideration.

Intracranial tuberculoma represents the most common mass lesion in neurotuberculosis. We report an unusual case of tuberculoma with haemorrhage presenting clinically as a cerebellar haemorrhage. Cranial CT scan revealed a peripherally enhancing cerebellar mass with haemorrhage and obstructive hydrocephalus, requiring urgent surgical evacuation of the haematoma. Histopathological examination revealed tuberculoma with features of phlebitis. In view of the pathological evidence of phlebitis in the lesion, it could represent an immune-mediated acute haemorrhagic leucoencephalitis in response to tubercular antigens.