Neuroimaging Abnormalities in Patients with Subacute Sclerosing Panencephalitis : Prospective Follow-up Study.

OBJECTIVE: This study aimed to assess the neuroimaging abnormalities and their progression in patients with Subacute sclerosing panencephalitis (SSPE) and identify clinical predictors of these imaging findings. METHODS: This prospective observational study evaluated clinical and neuroimaging features in patients with SSPE. Patients were categorized using Dyken's criteria, Jabbour's staging system, and the definition of fulminant SSPE. They underwent comprehensive clinical assessments, cerebrospinal fluid examination, Electroencephalogram (EEG), and Magnetic Resonance Imaging (MRI) scans. Treatment involved intrathecal interferon­α and antiepileptic medications. Functional disability was assessed using the modified Barthel index. Follow-ups were performed at 6 months, including reassessment of Modified Barthel Index (MBI) and Jabbour's staging and EEG and MRI scans. RESULTS: The mean age was 13.9 ± 6.7 years, with males comprising 81.5% (44/54) of the cohort. Fulminant SSPE was noted in 33% (18/54) of cases. Disease duration before presentation varied significantly between fulminant and non-fulminant forms (p = 0.001). Neuroimaging abnormalities were more prevalent in JS III stage patients, with diffuse cerebral atrophy being a significant finding (p = 0.011). Basal ganglia involvement correlated with movement disorders. The 6­month follow-up showed increased cerebral atrophy (p = 0.004). Increasing disease duration was an independent predictor of cerebral atrophy. An Intercomplex interval (ICI) of more than 10 minutes correlated with normal neuroimaging, 10 patients died within the study period, 8 of whom had fulminant SSPE. CONCLUSION: Parieto-occipital White matter hyperintensity (WMH) is the most prevalent and sensitive neuroimaging finding for the diagnosis of SSPE. Despite interferon treatment, cerebral atrophy progressed in both aggressive and fulminant SSPE. Increasing disease duration is an independent predictor of cerebral atrophy.

Rhino orbital cerebral mucormycosis in settings of COVID-19 infection: A case series of thirteen patients.

Rhino orbital cerebral mucormycosis (ROCM) is an important infectious disease encountered in large numbers in this recent post-COVID-19 era. An alteration in the defense immune system during COVID-19 illness; in the presence of uncontrolled hyperglycemia has led to the new epidemic of ROCM, especially in developing nations such as India. This case series of thirteen patients illustrates the various clinical presentations, laboratory parameters, imaging features and outcomes of patients with ROCM admitted to a tertiary care hospital in Northern India. In our case series, a total of 13 newly diagnosed cases of rhino-orbital-cerebral mucormycosis were studied. A history of COVID-19 illness was observed in seven cases (53.8%) with a mean duration of mucormycosis after 25 ± 3.6 days, the use of steroids during COVID-19 illness was seen in 5 cases (38.5%), and oxygen therapy was given in 4 cases (30.8%). A comorbid state in the form of diabetes mellitus was present in 12 cases (92.3%) with a mean duration of 16.69 months, with an important finding of seven cases (53.85%) having new-onset diabetes; hypertension was present in three cases (23.1%). Magnetic resonance imaging of paranasal sinuses showed involvement of multiple sinuses in all 13 cases (100%), including maxillary and ethmoidal sinuses, with frontal involvement in 12 cases (92.3%), sphenoidal involvement in 11 cases (84.6%), symmetric involvement in 9 cases (69.2%), mastoiditis in four cases (30.8%), maxillary space involvement in four cases (30.8%), and palatal involvement in one case (7.7%). On statistical analysis, there was a significant association of new-onset diabetes, optic neuropathy and high C reactive protein with blindness (P-value < 0.05) in our study. However, there were no statistically significant association for the involvement of nervous system in our study. Multispecialty approach treatment was given in the liposomal amphotericin B therapy in all the patients along with thorough endo-nasal debridement done in all cases, transcutaneous retrobulbar amphotericin B in six cases (46.2%) with exenteration done in seven patients (53.9%). At 3 months of follow-up, there was substantial clinical improvement in all cases. There should be definite emphasis on high suspicion of mucor clinically for early diagnosis and aggressive management at the initial state of diagnosis for better outcomes. The need for sustained proper glycemic control during the COVID-19 era along with judicious use of steroids and public awareness of early symptoms and manifestations of mucor can curb the magnitude of such potentially opportunistic epidemics to a substantial rate. New-onset diabetes mellitus, optic neuropathy and high C reactive protein (>50 mg/L) showed statistically significant association with blindness. The longer the infection remains undetected, the greater the devastation ROCM can impose, of which blindness is an important hazard.

Case Report: An Unusual Case of Subacute Sclerosing Panencephalitis with Distinctive Clinical and Neuroimaging Features.

Subacute sclerosing panencephalitis (SSPE) is a relentlessly progressive brain disorder with invariable mortality. Subacute sclerosing panencephalitis is common in measles-endemic areas. We report an unusual SSPE patient with distinctive clinical and neuroimaging features. A 9-year-old boy came with a 5-month history of spontaneously dropping objects from both hands. Subsequently, he developed mental decline, a loss of interest in his surroundings, decreased verbal output, and inappropriate crying and laughing along with generalized periodic myoclonus. On examination, the child was akinetic mute. The child demonstrated intermittent generalized axial dystonic storm with flexion of upper limbs, an extension of lower limbs, and opisthotonos. Dystonic posturing was more dominant on the right side. Electroencephalography revealed periodic discharges. Cerebrospinal fluid antimeasles IgG antibody titer was markedly elevated. Magnetic resonance imaging revealed marked diffuse cerebral atrophy, and periventricular T2/fluid-attenuated inversion recovery hyperintensity. T2/fluid-attenuated inversion recovery images also revealed multiple cystic lesions present in the region of periventricular white matter. The patient was given a monthly injection of intrathecal interferon-α. The patient is currently continuing in the akinetic-mute stage. In conclusion, in this report, we described an unusual case of acute fulminant SSPE in which neuroimaging demonstrated unusual multiple small discrete cystic lesions in the cortical white matter. The pathological nature of these cystic lesions currently is not clear and needs to be explored.