ABSTRACT
Background: Immunoglobulin (Ig)G4-related disease is a systemic fibroinflammatory disease characterized by dense infiltration of IgG4-positive plasma cells in the affected tissue(s) with or without elevated plasma levels of IgG4. Hypophysitis itself is a very rare disease with reported prevalence in the operative specimens are around 0.2-0.88%. IgG4-related hypophysitis (IgG4-RH) may account for a substantial percentage of cases previously regarded as idiopathic hypophysitis. Methods: This study is a registry-based, retrospective, and cohort study from a tertiary care hospital in North India. The medical records and clinical data of biopsy-proven and suspected IgG4-RH patients registered were retrospectively analyzed. Treatment outcome of cases was also explored during this analysis. Results: Two thousand and six sellar area space-occupying lesions have been operated-on since 2006, among them only four patients had IgG4-RH on histopathological specimen. One case was diagnosed on clinical suspicion. Mean age of the patients was 31.8 ± 6.32 years. Most frequent presenting complaint was headaches. Extracranial manifestations were present in four patients. The most common pituitary dysfunction was cortisol deficiency. 18 F-fluorodeoxyglucose positron emission tomography (18F FDG PET) was helpful in three cases for diagnosis of hypophysitis and other organ involvement. Classical histological findings with storiform fibrosis, obliterative phlebitis seen in two cases, and IgG4-positive plasma cell infiltration were positive in four cases. Surgery was the primary modality of treatment in all four cases. Only one patient received steroids as a primary therapeutic modality. Conclusion: IgG4-RH is rare. High index of suspicion is required to diagnosis the case precisely. FDG PET is helpful in diagnosing hypophysitis and extrapituitary lesions.
ABSTRACT
Pediatric glioblastoma (pGBM) is a rare entity accounting for only approximately 3% of all childhood brain tumors. Treatment guidelines for pGBM have been extrapolated from those in adult glioblastoma. Rarity of pGBM and underrepresentation of pediatric population in major studies precludes from defining the ideal treatment protocol for these patients. Maximum safe resection is performed in most of the cases followed by postoperative radiotherapy in children over 3 years of age. Benefit of temozolomide is unclear in these patients. Here, we present the clinicopathological details and outcome of six pGBM patients treated at our institute in 2018-2019.
ABSTRACT
Human nocardiosis is primarily an opportunistic infection affecting immunocompromised patients, however, one-third of them are immunocompetent. CNS involvement is less commonly reported and associated with a grave prognosis. The majority of these patients are organ transplant recipients on immune suppressants. In the recent past, association of Nocardia asiatica with brain abscess has been reported in a few cases. We are reporting a case of isolated cerebellar abscess caused by N. asiatica in an immune-compromised adult with a review of relevant literature. A 53-year-old male presented with complaints of headache and vomiting for 14 days. There was no previous history of any comorbid illness. During presentation, he was having gait ataxia and radiology showed the right-sided cerebellar multiple lesions. Further hematological investigations revealed the patient to be HIV positive. The abscess was tapped and the pus culture showed Nocardia species. Antibiotics were started as per sensitivity and the patient did well at 3-month follow-up. Though rare, Nocardia should be kept as a differential in brain abscess patients. Owing to the different antimicrobial sensitivity patterns among Nocardia species, both appropriate speciation and susceptibility testing of uncommon species such as N. asiatica are required for their successful treatment.
ABSTRACT
Background: Skull base lesions are still considered surgically challenging and primary gamma knife radio surgery (GKRS) is gaining popularity. However the effectiveness of GKRS may be overrated especially in lesions with indolent course. Case Description: We report a case of chondrosarcoma, mimicking a trigeminal schwannoma treated with upfront radio surgery. Relatively lower dose was administered in view of proximity to the brainstem. The patient was asymptomatic and the size of the lesion remained static for over a decade. This was misinterpreted as effectiveness of GKRS. The lesion grew after a decade necessitating surgery. Conclusion: With popularity of upfront GKRS, suboptimal but maximal safe radiation dose is usually prescribed for lesions close to critical structures like brainstem. In these cases the long indolent natural course of the pathology, as in the case of chondrosarcoma may be misconstrued as success of radiosurgery. An extended follow up beyond this static period is necessary before concluding its effectiveness.
ABSTRACT
BACKGROUND: The minimally invasive approach to distal anterior cerebral artery (DACA) aneurysms has not gained much acceptance due to difficulties associated with the conventional frontal paramedian approach. The more proximal basal interhemispheric approach, however, necessitates extensive dissection of soft tissues. We describe a novel minimally invasive median supraorbital keyhole craniotomy with a basal interhemispheric approach for clipping a ruptured DACA aneurysm. METHODS: A 62-year-old patient presented with subarachnoid hemorrhage. Computed tomography angiography revealed a DACA aneurysm. The surgical technique involved a keyhole craniotomy made via an eyebrow incision extending between the supraorbital notches, and flush with the anterior cranial fossa. The dura was opened at the anterior part, the falx was cut, an interhemispheric dissection was carried out, adequate proximal control was obtained, and the aneurysm neck was dissected and clipped. A relevant review of the literature was carried out. RESULTS: The patient recovered well, with no residual aneurysm or forehead numbness, with good cosmesis. Compared with the previously described "keyhole unilateral interhemispheric" approaches, our technique has less likelihood of encountering bridging veins; easier cisternal cerebrospinal fluid release, making it feasible even in swollen brain; better proximal vascular control; and trajectory toward the neck rather than dome. CONCLUSION: The median supraorbital keyhole approach is a minimally invasive technique sufficient for clipping most DACA aneurysms, with easier access, better proximal control, and good cosmesis.
