Sunithi Mani

Decompressive craniectomy in cerebral venous thrombosis: a single centre experience

Background Cerebral venous thrombosis (CVT) is an important cause for stroke in the young where the role for decompressive craniectomy is not well established. Objective To analyse the outcome of CVT patients treated with decompressive craniectomy. Methods Clinical and imaging features, preoperative findings and long-term outcome of patients with CVT who underwent decompressive craniectomy were analysed. Results Over 10 years (2002–2011), 44/587 (7.4%) patients with CVT underwent decompressive craniectomy. Diagnosis of CVT was based on magnetic resonance venography (MRV)/inferior vena cava (IVC). Decision for surgery was taken at admission in 19/44 (43%), within 12 h in 5/44 (11%), within first 48 h in 15/44 (34%) and beyond 48 h in 10/44 (22%). Presence of midline shift of ≥10 mm (p<0.0009) and large infarct volume (mean 146.63 ml; SD 52.459, p<0.001) on the baseline scan influenced the decision for immediate surgery. Hemicraniectomy was done in 38/44 (86%) and bifrontal craniectomy in 6/44 (13.6%). Mortality was 9/44 (20%). On multivariate analysis (5% level of significance) age <40 years and surgery within 12 h significantly increased survival. Mean follow-up was 25.5 months (range 3–66 months), 26/35 (74%) had 1 year follow-up. Modified Rankin Scale (mRs) continued to improve even after 6 months with 27/35 (77%) of survivors achieving mRs of ≤2. Conclusions This is the largest series on decompressive craniectomy for CVT in literature to date. Decompressive craniotomy should be considered as a treatment option in large venous infarcts. Very good outcomes can be expected especially if done early and in those below 40 years.

Clinicopathologic correlates of giant pituitary adenomas.

Giant adenomas comprise a clinical/therapeutic subset of pituitary adenomas that pose a surgical challenge. The study population consisted of 28 patients who had giant pituitary adenomas, which are defined as tumors with a diameter greater than 5cm. Clinically, five tumors (18%) were endocrinologically functional and 23 (82%) were not. During surgery, one tumor was radically excised, four were subtotally excised, 12 were partially excised, and 11 were biopsied. All of the tumors showed typical histological features of pituitary adenoma. Of the 23 clinically non-functional adenomas, 18 were gonadotrophic tumors, four were null cell adenomas and one was a silent corticotroph adenoma. The MIB-1 labeling indices ranged from 0.1% to 2.0%. The mean topoisomerase labeling index was 0.75%. Microvessel density ranged from 0.42% to 5.55%, and there was moderately intense immunostaining for vascular endothelial growth factor. The present study found giant adenomas to be invasive but slow growing, histologically benign and often gonadotrophic in subtype.

Leflunomide with low-dose everolimus for treatment of Kaposi’s sarcoma in a renal allograft recipient

Current treatment of Kaposis sarcoma is reduction of immunosuppression with or without addition of mammalian target of rapamycin inhibitors (mTORi). Akt signalling plays a central role in oncogenesis of Kaposis sarcoma. We describe a case of multifocal Kaposis sarcoma in a renal allograft recipient, which showed unsatisfactory early response to immunosuppression reduction along with everolimus therapy but completely resolved after adding leflunomide. mTORi impair Kaposis sarcoma oncogenesis by inhibiting mTOR downstream from the Akt signalling. Leflunomide inhibits Akt phosphorylation. This synergistic effect may be beneficial in treatment of Kaposi sarcoma and needs to be explored in trials.

IgG4-related disease with sinonasal involvement: A case series.

We present the imaging findings in two cases of IgG4-related disease involving the sinonasal region in the pediatric age group. Imaging findings in IgG4-related disease affecting the nasal cavity and paranasal sinuses have been rarely reported in literature. The diagnosis is made by a combination of clinical, imaging, and histopathologic findings. Radiologists should be aware of the imaging findings of this condition to ensure early diagnosis and treatment.

Cerebrovascular injury in cryptococcal meningitis

Background Cryptococcal meningitis continues to be one of the common causes of chronic central nervous system infection worldwide. Individuals with cryptococcal meningitis can occasionally present with small vessel vasculitis causing infarcts primarily in the basal ganglia, internal capsule, and thalamus. Literature regarding patterns of cerebrovascular injury among patients with cryptococcal meningitis is scanty, and outcome following these vascular involvements is unknown. Aim To study the clinical profile, imaging findings, and details of vascular territory involved among patients admitted with cryptococcal meningitis and central nervous system infarct in a tertiary care center from India. And to compare the outcomes of patients of cryptococcal meningitis with or without central nervous system infarcts in terms of mortality and morbidity, Methodology A total of 151 patients with microbiologically proven cryptococcal meningitis over a time span of 11 years were retrospectively enrolled into the study. Of these, 66 patients met the inclusion criteria of having appropriate imaging of the brain. The presence of infarct in the imaging was analyzed by two independent radiologists. Patterns of central nervous system involvement and types of vascular injury were ascertained based on radiological parameters. Clinical parameters and outcomes of patients with and without infarcts were compared. Results Twenty (13%) of these patients had evidence of central nervous system infarcts on imaging. The mean age of patients with and without infarcts was 41 years and 38 years, respectively. Male predominance was present among both the groups. The presence of fever, neck stiffness, positive blood culture, and hydrocephalus in central nervous system imaging was similar among patients with or without infarct. Longer duration of illness, low sensorium at the time of presentation, low Glasgow Coma Scale score, presence of meningeal inflammation, cryptococcomas, and basal exudates in imaging were higher in patients with infarct. All the infarcts were of the lacunar type. Sixty percent of the cerebrovascular infarcts were acute in nature, 50% of these being multiple. Unilateral infarcts were seen in 70% of the patients. The most common site of infarct was the basal ganglia, others being distributed over the thalamus, frontal, temporal, parieto-occipital regions in the descending order. The presence of neurovascular involvement in the form of infarcts to the risk of morbidity and mortality had an odds ratio of 9.1 and 2.6, respectively. Conclusion Neurovascular involvement in chronic cryptococcal meningitis is a rare entity. These tend to present as multiple lacunar infarcts. Mortality and morbidity associated with these patients is higher when compared to patients who do not have infarcts. This result suggests that vascular injury plays a role in predicting outcome of patients with cryptococcal meningitis. Future studies are needed to understand the mechanism by which vascular events (infarcts) occur and result in poor outcome.

Recurrent craniospinal subarachnoid hemorrhage in cerebral amyloid angiopathy

Cerebral amyloid angiopathy (CAA) usually manifests as cerebral hemorrhage, especially as nontraumatic hemorrhages in normotensive elderly patients. Other manifestations are subarachnoid (SAH), subdural, intraventricular hemorrhage (IVH) and superficial hemosiderosis. A 52-year-old hypertensive woman presented with recurrent neurological deficits over a period of 2 years. Her serial brain magnetic resonance imaging and computed tomography scans showed recurrent SAH hemorrhage, and also intracerebral, IVH and spinal hemorrhage, with superficial siderosis. Cerebral angiograms were normal. Right frontal lobe biopsy showed features of CAA. CAA can present with unexplained recurrent SAH hemorrhage, and may be the initial and prominent finding in the course of disease in addition to superficial cortical siderosis and intracerebal and spinal hemorrhages.

Natural history of multiple sclerosis from the Indian perspective: Experience from a tertiary care hospital.

CONTEXT Multiple sclerosis (MS) has a spectrum of heterogeneity, as seen in western and eastern hemispheres, in the clinical features, topography of involvement and differences in natural history. AIM To study the clinical spectrum, imaging, and electrophysiological as well as cerebrospinal fluid (CSF) characteristics and correlate them with outcome. SETTINGS AND DESIGN Retrospective analysis of MS patients during a period of 20 years. SUBJECTS AND METHODS Cases were selected according to recent McDonalds criteria (2010), They were managed in the Department of Neurology, Christian Medical College, Vellore. STATISTICAL ANALYSIS USED Chi-square and Fishers exact tests were used for categorical variables. Multiple binary logistic regressions were done to assess significance. Kaplan-Meier curves were drawn to estimate the time to irreversible disability. RESULTS A total of 157 patients with female preponderance (55%) were included. The inter quartile range duration of follow-up was 9.1 (8.2, 11) years for 114 patients, who were included for final outcome analysis. Relapsing remitting MS (RRMS) (54.1%) was the most common type of MS seen. RRMS had a significantly better outcome (odds ratio: 0.12, 95% confidence interval: 0.02-0.57, P = 0.008) compared to progressive form of MS (primary progressive, secondary progressive). The Expanded Disability Status Scale score of patients at presentation and at final follow-up was 4.4 ± 1.31 and 4.1 ± 2.31, respectively. During the first presentation, polysymptomatic manifestations like motor and sphincteric involvement, incomplete recovery from the first attack; and, during the disease course, bowel, bladder, cerebellar and pyramidal affliction, predicted a worse outcome. CONCLUSION A high incidence of optico-spinal presentation, predominance of RRMS and a low yield on cerebrospinal fluid (CSF) studies are the major findings of our study. A notable feature was the analysis of prognostic markers of disability.

Dermoid tumor with diastematobulbia

BACKGROUND Dermoid tumors are rare congenital lesions and account for 0.2% to 1.8% of all intracranial tumors. Dermoid tumor with diastematobulbia is very rare. CASE DESCRIPTION We report a dermoid tumor in an adult female with an unusual location and morphology. The lesion had 2 major components with different material within the cysts. The posterior part of the dermoid had presented on the floor of the fourth ventricle and had split the pons into 2 distinct halves. The anterior part of the lesion was situated in the prepontine cistern and encircling the anterior half of the brainstem. The lesion was radically excised, and the postoperative images showed return of the 2 halves of the pons to appose each other in the midline. CONCLUSION This case report demonstrates the anatomical peculiarities of this extremely unusual presentation of a dermoid cyst with diastematobulbia. Total excision of the lesion through a dorsal approach resulted in a good outcome.

Role of penumbra mechanical thrombectomy device in acute dural sinus thrombosis

Background: In dural venous sinus thrombosis (DVST), the mortality ranges 5–30%. Deep venous system involvement and septic dural sinus thrombosis have a higher mortality rate. In acute occlusion, collateral flow may not be established, which may result in significant edema and mass effect. Endovascular interventions may be considered as a treatment option in appropriate high-risk patients with DVST. Materials and Methods: Eight patients with magnetic resonance imaging (MRI)-confirmed dural sinus thrombosis, who did not respond to the conventional standard medical treatment, were subsequently treated with mechanical thrombectomy using the Penumbra System®. In all cases, medical treatment including anticoagulants were continued following the procedure for a minimum period of 1 year. Results: Recanalization of the dural sinus thrombosis was achieved in all 8 cases. There were no immediate or late endovascular-related complications. One death occurred due to an unrelated medical event. At 6 months, there was notable improvement in the modified Rankin Score (mRS), with 5/8 (62%) patients achieving mRS of 2 or less. The follow-up ranged between 3 months and 26 months (mean: 14.5 months), and there were no new neurological events during the follow-up period. Conclusion: Cerebral venous sinus thrombosis is a rare but life-threatening condition that demands timely diagnosis and therapy. In cases of rapidly declining neurological status despite standard therapy with systemic anticoagulation and anti-edema measures, mechanical thrombectomy could be a lifesaving and effective option. In this study, good outcomes were observed in the majority of patients at long-term follow up.

Siblings with fucosidosis

Fucosidosis is a rare lysosomal storage disorder due to deficiency of fucosidase enzyme, with around 100 cases reported worldwide. Here, we describe the clinical and imaging features in two siblings with fucosidosis. An 8-year-old girl presented with global developmental delay, followed by regression of acquired milestones from 3 years of age with bipyramidal, extrapyramidal involvement, coarse facies, telangiectatic lesions, dysostosis multiplex, characteristic magnetic resonance imaging finding along with undetectable levels of the fucosidase activity, which confirmed the diagnosis. Younger sibling has mild developmental delay with autistic traits with no neuroregression until now. He also has undetectable level of fucosidase enzyme activity and is being considered for stem cell transplantation. New case reports would expand the clinical spectrum, early diagnosis and help formulating appropriate therapy. Early diagnosis is crucial and hence sibling screening can be done, and those in the presymptomatic stage can undergo hematopoietic stem cell transplantation, which is potentially curable.