Disseminated Aspergillosis in a Patient With Neurosarcoidosis: Persistent Contrast Enhancement in CNS Despite Prolonged Antifungal Treatment: A Case Report.

A 56-year-old Caucasian man was diagnosed with definite neurosarcoidosis after he presented with progressive bilateral lower extremity weakness and dysesthesia. He was started on a combination immunosuppressant regimen of dexamethasone, methotrexate and infliximab. Two months into treatment with immunosuppressants, he developed devastating disseminated aspergillosis which clinically stabilized with aggressive antifungal treatment however had a protracted radiological course despite prolonged anti-fungal treatment for over two years. Interestingly, he remained in remission from neurosarcoidosis off immunosuppression during the same period. This case emphasizes need for vigilance for fungal infections in patients treated with combination immunosuppressive therapy particularly TNF-α inhibitors such as infliximab.

Development of Expert-Level Classification of Seizures and Rhythmic and Periodic Patterns During EEG Interpretation.

BACKGROUND AND OBJECTIVES: Seizures (SZs) and other SZ-like patterns of brain activity can harm the brain and contribute to in-hospital death, particularly when prolonged. However, experts qualified to interpret EEG data are scarce. Prior attempts to automate this task have been limited by small or inadequately labeled samples and have not convincingly demonstrated generalizable expert-level performance. There exists a critical unmet need for an automated method to classify SZs and other SZ-like events with expert-level reliability. This study was conducted to develop and validate a computer algorithm that matches the reliability and accuracy of experts in identifying SZs and SZ-like events, known as "ictal-interictal-injury continuum" (IIIC) patterns on EEG, including SZs, lateralized and generalized periodic discharges (LPD, GPD), and lateralized and generalized rhythmic delta activity (LRDA, GRDA), and in differentiating these patterns from non-IIIC patterns. METHODS: We used 6,095 scalp EEGs from 2,711 patients with and without IIIC events to train a deep neural network, SPaRCNet, to perform IIIC event classification. Independent training and test data sets were generated from 50,697 EEG segments, independently annotated by 20 fellowship-trained neurophysiologists. We assessed whether SPaRCNet performs at or above the sensitivity, specificity, precision, and calibration of fellowship-trained neurophysiologists for identifying IIIC events. Statistical performance was assessed by the calibration index and by the percentage of experts whose operating points were below the model's receiver operating characteristic curves (ROCs) and precision recall curves (PRCs) for the 6 pattern classes. RESULTS: SPaRCNet matches or exceeds most experts in classifying IIIC events based on both calibration and discrimination metrics. For SZ, LPD, GPD, LRDA, GRDA, and "other" classes, SPaRCNet exceeds the following percentages of 20 experts-ROC: 45%, 20%, 50%, 75%, 55%, and 40%; PRC: 50%, 35%, 50%, 90%, 70%, and 45%; and calibration: 95%, 100%, 95%, 100%, 100%, and 80%, respectively. DISCUSSION: SPaRCNet is the first algorithm to match expert performance in detecting SZs and other SZ-like events in a representative sample of EEGs. With further development, SPaRCNet may thus be a valuable tool for an expedited review of EEGs. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that among patients with epilepsy or critical illness undergoing EEG monitoring, SPaRCNet can differentiate (IIIC) patterns from non-IIIC events and expert neurophysiologists.

Anti-Neurofascin Antibodies Associated with White Matter Diseases of the Central Nervous System: A Red Flag or a Red Herring?

Autoantibodies against nodal and paranodal proteins, specifically anti-neurofascin antibodies (ANFAs), have been recently described in central and peripheral nervous system demyelinating disorders. We retrospectively reviewed the charts of six individuals evaluated at our Multiple Sclerosis Program who tested positive for serum ANFAs on Western blot. We describe these patients' clinical and diagnostic findings and attempt to identify features that might guide clinicians in checking for ANFAs. In our series, the women-to-men ratio was 2:1. At presentation, the median age was 60 years (range 30-70). The clinical presentation was pleiotropic and included incomplete transverse myelitis (n = 3), progressive myelopathy (n = 1), recurrent symmetric polyneuropathy (n = 1), and nonspecific neurological symptoms (n = 1). Atypical features prompting further workup included coexisting upper and lower motor neuron features, older age at presentation with active disease, atypical spinal cord MRI features, and unusual cerebrospinal fluid findings. The serum ANFAs panel was positive for the NF-155 isoform in five patients (IgM n = 2; IgG n = 2; both n = 1) and the NF-140 isoform in two (IgG n = 2). Larger studies are needed to assess the relevance of ANFAs in demyelinating nervous system diseases, their impact on long-term clinical outcomes, and associated therapeutic implications.

Lymphomatoid papulosis in a patient treated with glatiramer acetate and the glatiramoid Glatopa for multiple sclerosis: A case report.

A 48-year-old Caucasian woman with history of multiple sclerosis (MS) presented with erythematous papulonodular lesions in her extremities and trunk. She was being treated with glatiramer acetate (GA) for the past 10 years and the glatiramoid, Glatopa, for 2 years prior to this presentation. A skin biopsy showed CD30+ lymphoproliferative disorder consistent with lymphomatoid papulosis (LyP). Three weeks after stopping Glatopa, her skin lesions were improved. It remains unclear whether GA's or Glatopa's capability to alter T-cell differentiation, may have a link with LyP. This case report is a reminder to be vigilant for skin lesions in patients with MS.

Driving with drug-resistant and controlled seizures from a patient's perspective: Assessment of attitudes and practices.

BACKGROUND: Driving restrictions in epilepsy are intended to safeguard public and personal safety; however, these limitations inhibit socialization, restrict employment, and reduce self-esteem in patients with seizures. A large proportion of patients with seizures continue to drive, and factors leading to noncompliance with driving regulations are poorly understood. Thus, the patients' perspective on driving safety is not incorporated into the existing counseling tools on driving safety in epilepsy. The present study assessed social, economic, and psychological perceptions related to driving restrictions in patients with refractory and pharmacotherapy-controlled seizures at the single epilepsy center and identified impediments for safe driving. METHODS: Data were obtained from an anonymous survey completed by 25 adult patients in the presurgical group (PG) with refractory epilepsy and 46 patients in the ambulatory group (AG) with confirmed epilepsy which did not meet criteria for refractoriness. The questionnaire (administered via Research Electronic Data Capture (REDCap)) addressed seizure and driving history, knowledge of driving restrictions, and social consequences of losing driving privileges. RESULTS: Eighty-seven percent of all responders experienced seizures with alteration of awareness; however, 34% of patients continued to drive during the time when they were legally restricted, and 6% had accidents related to seizures. All responders reported their seizure status accurately to the treating physician, and 93% understood state-based driving restrictions. The median time from the last seizure was shorter, and the duration of last driving restriction was longer in the PG compared with the AG (1 vs. 20weeks, and 12 vs. 24weeks, respectively). Despite that, the proportions of patients driving at the time of survey were not significantly different between the two groups. Nearly 80% of all patients stated that driving restrictions reduced their quality of life, and 70% believed that these restrictions carry a social stigma. Employment was chosen to be the most affected by driving restrictions from a list of four social domains by the majority of patients in both groups. Notably, the employment rate was 26% higher in the AG compared with the PG. The lack of public transportation was regarded as a hurdle by more than 60% of patients in each group with greater than two-thirds of patients relying on other drivers for transportation. CONCLUSIONS: These findings suggest that patients with refractory and pharmacotherapy-controlled seizures are similarly likely to drive a vehicle, disregarding a practitioner's advice and state restrictions. The lack of public transportation is a shared constraint and likely leads to reduced compliance with driving regulations. Driving restrictions carry social stigma and limit the employment of patients with epilepsy, regardless of the refractory seizure status.