Trigeminal Nerve Melanotic Schwannoma in Carney Complex.

Melanotic schwannoma (MS) is a rare peripheral nerve sheath tumor commonly found in the thoracic paraspinal region. It is present in an intracranial location rarely, with 18 out of 105 MS cases described in the literature. Trigeminal nerve was involved in only six of these cases. Fifty percent of psammomatous melanotic schwannoma (PMS) patients have Carney complex. Carney complex is an autosomal dominant disorder featuring peripheral nerve tumors (schwannomas), myxomas (heart, skin, and breast), skin pigmentation (lentigines and blue nevi), and endocrine tumors (adrenal, testicular, and pituitary). We present a case of left trigeminal nerve PMS as a part of Carney complex. Patient had diffuse lentiginosis. Magnetic Resonance Imaging revealed enhancing lesion along left trigeminal nerve with widened Meckel's cave and foramen ovale. Final diagnosis of PMS was derived on clinical, radiological, and histopathological findings with immunohistochemistry correlation. Key Message Trigeminal nerve can be rarely involved in melanotic schwannoma (MS). Knowledge of typical radiological features of MS is crucial for its diagnosis. Its association with Carney complex should be sought.

A real world multi center study on efficacy and safety of natalizumab in Indian patients with multiple sclerosis.

BACKGROUND: Natalizumab (NTZ) is increasingly being used in Indian multiple sclerosis (MS) patients. There are no reports on its safety and efficacy, especially with respect to the occurrence of progressive multifocal leukoencephalopathy (PML). OBJECTIVES: To describe the patient characteristics, treatment outcomes, and adverse events, especially the occurrence of PML in NTZ-treated patients. METHODS: A multicentre ambispective study was conducted across 18 centres, from Jan 2012 to Dec 2021. Patients at and above the age of 18 years treated with NTZ were included. Descriptive and comparative statistics were applied to analyze data. RESULTS: During the study period of 9 years, 116 patients were treated with NTZ. Mean age of the cohort was 35.6 ± 9.7 years; 83/116 (71.6%) were females. Relapse rate for the entire cohort in the year before NTZ was 3.1 ± 1.51 while one year after was 0.20±0.57 (p = 0.001; CI 2.45 -3.35). EDSS of the entire cohort in the year before NTZ was 4.5 ± 1.94 and one year after was 3.8 ± 2.7 (p = 0.013; CI 0.16-1.36). At last follow up (38.3 ± 22.78 months) there were no cases of PML identified. CONCLUSIONS: Natalizumab is highly effective and safe in Indian MS patients, with no cases of PML identified at last follow up.

Role of imaging in CNS infections.

Neuroinfections are seen in both adults and children. These can result in serious morbidity and if left untreated and/or associated with comorbidities can be life threatening. Cross-sectional imaging like computed tomography (CT) and magnetic resonance imaging (MRI) are advised by the clinicians for the diagnosing, confirmation of the diagnosis, assess any complications of the infection, and also for follow up. Though CT is the initial imaging investigation commonly asked by the clinician, due to its lesser soft tissue resolution, early brain changes may not be seen on CT. MRI has better soft tissue resolution with no ionizing radiation to the patient and helps in detecting the early signs of infection. Appropriate MRI, not only helps the radiologist to reduce the number of possibilities of the causative organism but also differentiates tumors from infection. However, CT is useful to assess the bony changes and also easily available and affordable cross-sectional imaging modality worldwide. The review summarizes the approach of the radiologist to central nervous system (CNS) infections and their typical imaging characteristic features.

Transient Ischemic Attack after Eating Spicy Foods in Children: Think of Moya Moya Disease.

Moya Moya disease is an important cause for childhood strokes and morbidity. An early diagnosis and treatment can prevent strokes and disability. Here we report the case of an eight-year-old boy who had transient episodes of headache and hemiparesis secondary to Moya Moya disease. He was misdiagnosed as hemiplegic migraine, seizure disorder, paroxysmal non-kinesigenic dyskinesia and dissociative disorder during the past three years. The diagnosis was significantly delayed as an important clinical cue was overlooked. A detailed history showed that symptoms were precipitated on eating spicy food. This clinical pointer prompted evaluation for Moya Moya disease. MRI and MR angiogram confirmed Moya Moya disease.

Efficacy and safety of rituximab in multiple sclerosis: Experience from a developing country.

BACKGROUND: Rituximab is increasingly being used in treatment of multiple Sclerosis (MS) in our centers due to its easy availability, efficacy and favorable side effect profile. Here we describe experience with rituximab over a period of 4 years from three MS centers from south India. METHODS: The data of MS patients who were treated with rituximab in three MS centers at Bangalore, India, from December 2015 to December 2019 were collected and evaluated with respect to relapse rate, EDSS score and adverse events. RESULTS: Over the four-year study period 118 MS patients were evaluated, 80 of whom were on rituximab. 58 (72%) had RRMS, 15 (19%) SPMS and 7 (9%) PPMS. Most patients (89%) received rituximab at a dose of 500 mg every 6-12 months. Nine patients (11%), all with progressive MS were on 1 gm to 2 gm every 6 months. Follow up ranged from 1 year to 3 years, with a median of 2 years. 56 (97%) RRMS patients had no relapses during follow up. EDSS score improved by a score of 0.5-2.0 in 68 (85%) patients, remained same in 10 (12.5%) and worsened in 2 patients (2.5%). Most patients (91%) tolerated rituximab infusions well. There were no opportunistic infections or neoplasms. CONCLUSION: Anti B cell therapy with rituximab appears effective, safe and affordable in the treatment of MS in developing countries like India with resource limited settings.

Demonstration of different histological layers of the pachygyria/agyria cortex using diffusion tensor MR imaging.

PURPOSE: Pachygyria/agyria are congenital brain malformations characterized by presence of a few broad, flat gyri with thickened cortex, resulting from arrest of neuronal migration in early gestation. We are hereby describing diffusion tensor imaging findings in different histological layers of lissencephaly cortex in two children. METHOD: DTI in addition to conventional MR imaging was performed in two children on a 3 T MRI and post-processed with vendor supplied software to generate the fractional anisotropy, mean diffusivity and trace maps. Tractography was also performed to identify presence of tracts in the thickened cortex. RESULTS: DTI demonstrated the dysplastic multilayered cortex in cases of pachygyria/agyria; the thickened fourth layer and superficial layer demonstrated high anisotropy on diffusion tensor imaging. CONCLUSION: DTI is a useful tool for identifying gross histological features of pachygyria-agyria complex. Superficial layer and thickened fourth layer demonstrate high anisotropy. Identification of anisotropy in the superficial layer has not been described in previous reports.